My  name is Angeline and I have cystic fibrosis. What a difficult thing to  admit to myself even though it’s been more than six years since my  diagnosis. I still love to live in denial by taking a “healthy day” and  pretending I’m not sick by skipping my treatments. Yes, I am aware of  the flawed logic. I wasn’t diagnosed until I was 31 years old, so doing  treatments is not something that I am accustomed to. I just acquired a  Hill-Rom Vest a few months ago; before that I just had nebulizer  treatments and an Acapella device for airway clearance. Even that seemed  to devour too much time. But I am getting ahead of myself. Let’s start  from the beginning.

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Some  people who are diagnosed late in life have never heard of CF, but not  me! I was hospitalized when I was six months old because I was so  little. The doctors were worried because I wouldn’t eat and I couldn’t  gain weight. The doctors thought I had cystic fibrosis. I was later told  that children with cystic fibrosis died when they were teenagers, but I  was so lucky because it turned out that I didn’t have it. Throughout my  childhood, I came down with pneumonia frequently and I coughed  routinely. I told people I had allergies or asthma, although I had never  been diagnosed with either one of those.

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Well, at  31 I found out that the doctors had done two sweat tests—one was  positive and the other was borderline. Rather than trying to figure out  why, they sent me home on a high-calorie formula. I suspect the doctors  decided not to pursue a diagnosis back then to protect my family from  being denied insurance as they were self-insured dairy farmers.

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Having  expected a long and fulfilling life, I obtained a Bachelor’s Degree in  Nursing and I got married. Shortly after, we had a daughter. However, we  divorced after five years. I later remarried a widower who had four  children with his late wife. I grew up with 12 biological siblings, so  his four children were just part of the package deal. We decided to have  a daughter of our own and then decided one more baby couldn’t hurt. We  ended up having twin boys. So, at the time of my diagnosis at 31, I was a  mother to eight children, two of which were nine-month-old twin boys.

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When I  found out I had an incurable, chronic, life-limiting illness I was  devastated. I was overwhelmed, to say the least. The guilt was  unbearable. Had I known I had a life-threatening illness would I have  lived life differently? Would my husband have ever considered marrying a  woman who was, at the very least, living with an illness that required  constant vigilance and, at the worst, left him yet again a widower, now  with even more children? I also felt vindicated: after all the years of  doctor’s visits and illnesses, only to be told it was another cold, I  finally had answers—cystic fibrosis.

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My first  visit to a cystic fibrosis clinic did not go how I expected. My husband  and I loaded up our twins and drove in to find out how long I had left  to live. But when we got there, the visit was much longer than the 20  minutes I initially anticipated. In hindsight, it was poor planning to  take the twins to what ended up being a three-hour long visit. Sadly,  the clinic answered almost none of my questions. Rather, the doctor  asked me nearly every question known to man and then they asked me to  cough into a cup. I had spent my whole life avoiding coughing in front  of people and not spitting out my mucus—I wasn’t about to start now.  They took x-rays and then said they would call. So, I went home and  waited for what felt like forever. When my doctor finally called with  x-ray results, I quickly asked about my life expectancy. She told me the  average age and what factors might influence that number, but that  ultimately, it was hard to predict. I hung up the phone and began  researching on the internet. I joined some Facebook groups to learn more  and connect with others. Overall, I felt completely lost. In  retrospect, I should have made a list of questions for my first CF  clinic visit, but I had no idea what to expect at the time. I thought it  would be more like a cancer diagnosis—you’re told you have cancer, how  long to live, and what treatment options are available. That is what I  was ready for and I thought it would happen in 20 minutes, maybe 30  minutes, tops. And I am a nurse! I am not sure what other people go in  thinking, but I was not ready for this.

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Let’s  fast forward to my first hospitalization: it was at Thanksgiving and it  didn’t go well. I still had very young children at home who were having a  hard time with mom being gone from home. In my denial, I mistakenly  assumed I would never be hospitalized for cystic fibrosis. I had  mentioned it during one clinic visit and it was waived off as a future  possibility only if things got bad. And then, out of the blue (OK, maybe  not that out of the blue), I got super sick. I waited until I was  really, really sick. My doctor had no clue what bug was making me so  ill. I was hospitalized for 10 days and now, in hindsight, I can tell  you that I should have remained in the hospital for at least 14 days. I  was discharged early because a doctor came to see me and found me hiding  in the bathroom, on the floor, crying my eyes out. I wasn’t eating or  sleeping, and my anxiety level was through the roof. It was such a shock  to me being in the hospital for such a long period of time. Prior to  this hospitalization, I had only been hospitalized to deliver my babies.  But the length of time for this stay and not knowing ahead of time what  to expect was very hard for me to accept. And there is the  obvious—being in the hospital completely sucks. You have no control, no  privacy; people continually come and go from your room, effectively  disrupting everything; you feel terrible physically; and you’re away  from your usual comfort items. On top of all that, I felt guilty for  being away from my responsibilities.

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The next  hospitalization was still hard, but better overall. This time, I cried  out in the open rather than in the bathroom. At this point, I had three  years of a CF diagnosis under my belt and I knew a little something  about how to deal with it but still very little about being hospitalized  for CF. Most patients leave after a couple of days in the hospital—why  did I need to be there for two weeks? I was not as sick as the first  time, so why did they need to keep me for so long now that I could  breathe and talk at the same time? During this admission, I found the  nurse case manager and asked why I needed to stay for so long. I told  her that I had been diagnosed three years prior and that even though I  myself was a nurse, I had no clue what was going on with my care. As a  nurse, I had worked in the operating room, in home-health settings, and  in case management. I had no experience working with other CF patients.  Despite having researched cystic fibrosis, it still made no sense to me.  It’s one thing to know something based on research and an entirely  different thing to live it. Everyone who walked into the hospital room  assumed this wasn’t my first rodeo. The nurse case manager was very  understanding and passed my concerns along to my care team. She brought  me a book that explained what being hospitalized with cystic fibrosis  really looks like. She also asked the social worker to come talk to me.  It still wasn’t perfect, but, for the first time in a long time, I took a  deep breath.

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Not  unsurprisingly, I met my catastrophic out-of-pocket maximum that year.  That’s what my insurance calls it, anyway. $5,500 per year. That only  includes the things that the insurance counts. Somehow, I still met the  criteria for having had a catastrophic year. It’s not hard to see why.  Copays for this, a percentage of that, hospitalizations here, and daily  medications everywhere. I thought catastrophes were supposed to be a  once-in-a-lifetime event, not a yearly expense that we have to plan for  and pay. When do the so-called catastrophes stop? They don’t. I keep  getting hospitalized. I keep getting sick. I keep needing medication,  antibiotics, doctor’s visits, and lab work. So, as I merrily count down  the days until Christmas, I also feverishly schedule everything I can  into the end of the year while there are no copays and no out-of-pockets  costs since I’ve already hit the maximum. I continually hope that I can  have a small break from the “catastrophe” that is my new normal.

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It’s  nice to tell my story and reflect on some of the things that have  happened to me. I would have loved a “What to expect now that you know  you have CF” book and I would still love a “if you have this happen, it  means this” book or a “these are all the organizations that could help  you and how” book. When I got a sweat test, my husband thought I was  crazy. He was sure there was no way I could be diagnosed with cystic  fibrosis as an adult. But here I am. I no longer fit into my old world.  That world has changed out of necessity. Some of my friends seem to  understand what a big change this diagnosis has been, and they have  continued to travel this road with me, but others have fallen by the  wayside. I am not a great fit in the cystic fibrosis world, either. Some  people, who have been diagnosed for longer, find it hard to be friends  with people who are new to the world of CF and are still trying to  understand all of it. I know I am still trying to figure it out. I am  the new kid in a class that has been together since they were in  kindergarten and it is hard to join the club. Especially since it’s a  club with such strong bonds. I am much more comfortable with my cystic  fibrosis diagnosis now. In the end I was never told when I was dying,  but with each bug I have grown, with each PFT I take, and with each  antibody I react poorly to, I calculate what “we’ll see” is turning into  and I worry that I won’t be able to fulfill the commitments I made to  be a mother to these eight children and to grow old with a man who has  already been a widower once. I no longer ask the question “how long  until cystic fibrosis kills me?” Instead, I try to focus on living in  the moment and refuse to allow the future to hold me hostage any  longer.

Angeline  Chase is 37 years old and has CF. She lives in Kaysville, Utah. She  likes to listen to audiobooks, watch Asian melodramas on Netflix,  garden, hike, play with animals (especially her little dog Millie),  tickle babies, and has taken up playing a Nintendo switch game  (adventure fit ring) for exercise.

CF Roundtable readers have submitted important questions related to the law in the  past three months. People with CF who receive Social Security disability  benefits have many questions about eligibility. Many people with CF are  considering a return to work when COVID-19 risk reduces sometime in the  future. Anyone considering a return to work should make sure they  understand the Social Security rules related to work and continuing  benefits or continuing Medicare or Medicaid before returning to work.

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If you have questions about your own situation, please email the CF Legal Information Hotline at CFLegal@sufianpassamano.com or call 1-800-622-0385. Nothing in this article is meant as legal advice and is only meant to be legal information.

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Question  1: I have lost my job and cannot afford to elect health insurance  coverage through COBRA. How can I purchase a policy on the Affordable  Care Act Website?

Answer: If a person is in need of health insurance, the person can go to www.healthcare.gov to see what policies are available in his/her area. The open enrollment  period for purchasing a policy ended on December 15, 2020; however, a  person can get special enrollment if that person meets one of the  special enrollment reasons that allow enrollment with no exclusion for a  pre-existing condition. If a person loses their insurance coverage,  that person has 60 days to purchase a policy on www.healthcare.gov.  That person can also purchase a policy from a specific insurance  company, but that person will not have access to possible help with  premiums, which is only available to those who enroll in a plan through www.healthcare.gov.

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Question 2: Why is my Supplemental Security Income (“SSI”) benefit reduced by 30 percent because my parents pay my rent?

Answer: A  person who receives SSI benefits must have low income and low assets.  SSI also has rules that consider who pays for a person’s housing and  food. If a person on SSI is unable to pay their share of rent and food  in addition to certain other expenses, then Social Security reduces the  SSI check by 30 percent each month. This is called the Social Security  SSI Household Deduction.

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A person  must have a written agreement to pay their share of household expenses  (rent, food, electricity, utilities, and garbage fees) if the person  wants to avoid the SSI Household Deduction. The written agreement is  typically submitted to the Social Security Administration (“SSA”) during  the SSI application process. The written agreement must contain certain  language. If the person who receives SSI benefits cannot pay their  share of household expenses, then the SSA will use the SSI Household  Deduction to reduce the monthly SSI benefit check.

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For  example, if the SSI recipient lives with their mother and the rent is  $2,000 then their share of rent is $1000. In most states, the maximum  SSI benefit is $783 a month. The person in this example would not be  able to pay their share of household expenses because their share of  rent alone is over the amount the person will receive from SSI. Social  Security would reduce the SSI check by 30 percent. California and New  York also add state money to the federal SSI amount of $783, so, if a  person lives in California or New York and receives SSI benefits, the  total amount the person will receive when the state supplement is added  is higher.

If a  person cannot initially pay their share of household expenses and then  moves and is then able to pay their share of household expenses, the  person can submit a rental agreement to Social Security to try to have  the SSI Household Deduction removed.

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The CF  Social Security Project will have funding from the CF Foundation to  assist with Social Security SSI Household Deduction issues for some  individuals with CF starting January 2, 2021. Please contact the CF  Social Project for more information at CFLegal@sufianpassamano.com.

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Question  3: I receive SSI benefits and I am thinking of taking a part-time job.  Will I be jeopardizing my SSI benefits and Medicaid benefits?

Answer:  People with CF who have never worked or have not worked enough to  qualify for Social Security Disability benefits are only eligible for  SSI benefits. SSI recipients must adhere to all of the asset and income  restrictions set out in the Social Security regulations. If a person  goes over the low-income asset and income criteria, the person may lose  both the cash SSI benefit and the Medicaid coverage that SSI recipients  receive.

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In the  13 states that have not expanded Medicaid eligibility for low-income  adults under the Affordable Care Act, one of the only ways to be  eligible for Medicaid as an adult is to be on SSI. Therefore, it is  extremely important for people with CF on SSI who live in those 13  states to make sure they maintain eligibility for SSI.

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If a  person who receives SSI benefits works part time, Social Security will  deduct $1 from the monthly benefit check for every $2 earned from work  activity. So, if a person earns $400 from work activity in one month,  then the person receives a deduction in the SSI amount of $200 for that  month. Typically, Social Security finds out about the work income after  the SSI benefit has been sent to the person, thus triggering an  overpayment in a future month.

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The  Social Security Plan to Achieve Self-Support (“PASS”) program allows a  person to work part time and save money for a work goal and the work  earnings do not reduce the SSI check.

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Generally,  Social Security work rules limit work activity to 20 hours or less per  week and making no more than $1,260 per month from work activity. If the  person is self-employed, the monthly work amount limit is $910 per  month before taxes are taken out of the work earnings.

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Question  4: I receive Social Security Disability Insurance (“SSDI”) benefits. I  have stopped working part time due to the COVID-19 risk. Does my SSDI  check increase?

Answer:  An SSDI benefit amount does not increase if a person is no longer  working part time. If a person has been receiving SSDI and works part  time making under $1,260 a month (before taxes are taken out of the  check) or $910 a month if self-employed, the SSDI check remains the same  if the person stops working part time.

SSI is the Social Security benefit that reduces the monthly SSI benefit by the amount of any monthly work income. s

Beth  is 55 and has CF. She is an attorney who focuses her law practice on  disability law and is the Treasurer of USACFA. Her contact information  is on page 2. You may contact her with your legal questions about  CF-related issues at:

CFLegal@sufianpassamano.com.

We  are well into nearly a year since we sheltered in place with COVID-19.  Many of us CFers have been extra careful about going out. Many of us  have adapted. We have more Zoom events than we know what to do with. We  have found ways to occupy our time: cooking, reading, exercising,  browsing YouTube, working, or pursuing hobbies.

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When I  was a child, my parents had an old wooden frame that looked much older  than it was hanging in the bathroom. The top line read in calligraphy,  “The Serenity Prayer.” I don’t know where it came from and my parents  were never part of the recovery movement, where this prayer is most  famous. I never understood what it meant as a kid. But now, as an adult,  I have truly appreciated its words: God grant me the serenity to accept  the things I cannot change, courage to change the things I can and  wisdom to know the difference.” Isn’t this particularly relevant for  living with CF? And living through a pandemic?

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This  article is about the spirit of acceptance. Acceptance means  understanding something is what it is. The pandemic. Having CF. A  dysfunctional family. A loss of any kind. Only after we fully accept  what is, can we move toward doing something about it. This includes  grieving and experiencing the emotional and spiritual fallout of what  is.

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As a  grief counselor, I see so many people who protest the death of their  loved one. The disbelief and denial are natural early on in loss; people  simply don’t want what is. They want what they used to have. And it is a  long, slow, hard process to accept fully—intellectually and  emotionally—that their loved one is not coming back and this is the life  they have now.

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In  Buddhism, there is the story of the two arrows. The first arrow is the  thing that hurts when it hits us. That could be a cancer diagnosis, a  divorce, the death of a loved one, or an unmet goal. The disappointment  is a blow; the unexpected interruption stings. The second arrow is what  hits us next. The second arrow is our reaction or response to the first  arrow. It can be anger, regret, sorrow, grief, fear, guilt, denial,  depression, or self-inflicted numbing or self-blame. The first arrow is  pain. Pain is inevitable in life—we cannot avoid it. The second arrow is  suffering. Suffering is our attitude toward our pain. Suffering is  optional. And so, our life mission is to come to terms with our  suffering.

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In a  recent support group, we talked about COVID-19 and sheltering in place.  One woman said, “There’s nothing I can do!” Another said, “I have so  many choices in what I can do!” It is interesting that the exact same  situation brings such different responses. I’ve had a chance to practice  my responses to another health scare that has come up lately. I can  freak out, I can get down, I can deny it. Instead, I am practicing to  just let it be. I will face what I need to face and deal with it. I’ll  find the wisdom to know what I can control and surrender what I can’t.

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Sometimes  acceptance implies passivity. There is a Japanese word, shikataganai,  which means “it can’t be helped.” Sometimes this has been used to  describe Japanese culture as weak or indifferent. Rather, it’s a Zen  philosophy to save energy by acknowledging what is and using that energy  for things that can be changed. When a challenge comes my way, I sure  would like to save energy!

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Acceptance  does not mean resignation, cowering under oppression, or giving up. We  all have a survival instinct; accepting we have a problem is the first  step toward cultivating motivation for problem solving. This is the  American way. To survive with CF, we must possess creativity and  ingenuity when it comes to figuring out alternatives or solutions. The  development of modifier drugs is a prime example of not accepting this  disease as inevitably fatal; thousands of people have worked really hard  over the years to find drugs to treat it.

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One of  the great strengths of the human spirit is defiance. To me, defiance is  determination. It’s raising your fists in the air after accepting what  is and proclaiming your power to persevere. Michelle Compton, a dear  friend and beloved member of the CF community, wrote a poem about  defiance of end-stage CF: “I am coming to terms with my life…I am coming  to terms with my death—and finding beauty in both. I am not trapped. I  refuse to be frightened. I will not be conquered.” She describes a  freedom in acceptance. What an awesome attitude! If I were Michelle, I’d  be so proud of myself for arriving at this realization. In some ways,  acceptance and defiance can raise confidence and self-love.

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And  acceptance does not mean we have to like what we have to accept. We can  hate what is, but soften around it. Accepting that our choices are  limited right now during this pandemic or accepting that we have to go  on disability or need a transplant invites us to examine what is in  front of us. Acceptance leads to awareness. Similarly, awareness also  leads to acceptance. When we look at our lives, we realize this may not  be the life we want but it’s the one life we have. Acceptance can mean  less drama and more harmony in your inner life as well as with  relationships. Imagine if we accepted people for who they are! Life  could be easier. The Bible says, “For God gave us a spirit not of fear  but of power and love and self-control.” (2 Timothy 1:7) Acceptance will  bring solace to our spirits. May you find its power.

Isa Stenzel Byrnes is 49 years old and has CF. She lives in Redwood City, California. She is 17 years post-lung transplant.

What  a year 2020 has been. I am so tired of P and P (pandemic and  politics!). The pandemic has worn us down for most of this year and  there is no end in sight. I am tired of having to stay home, having to  wear a mask, having to wear gloves, having to keep six feet between  myself and anyone else, and just having the concern about a vicious  virus. The politics just kept getting more and more irritating. I think  we could do better by using the method that is used in the United  Kingdom where campaigning is limited to about six weeks prior to an  election. Wouldn’t that be enough time to let all of us know all that we  need to know about any candidate? Oh well, this is the system by which  we live.

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At least  I am feeling well. Trikafta seems to be working well for me and I am so  grateful for the good feelings I am enjoying. I haven’t always felt  this well. When I was very young, I felt really rotten most of the time.  No doctors would believe my mother when she would tell them of my  health troubles. If they didn’t see it with their own eyes, it just  couldn’t be so. Since we lived out in the country, and I rarely was able  to get to the doctor’s office while I was feeling so ill, they just  discounted all of my mother’s reports.

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Despite  my frequent bouts of lung infections and pneumonia, no one believed my  mother when she talked with them about the status of my health. I missed  a lot of school. Every time some “bug” went around the school, I was  sure to get it. It seemed as if I always had an ear infection, a cold,  or worse. I was teased for missing so much school and my parents were  rebuked for allowing me to stay home when I “should have been in  school.” I remember one classmate’s mother told me that it was a good  thing I wasn’t her daughter because she wouldn’t have let me stay home  from school for a “runny nose.” To myself, I thought it was lucky for me  that she wasn’t my mother!

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By the  time I was 12, I was losing ground with my health. I was getting sicker  and sicker. When I was in the hospital, some doctors decided that I  should be tested for CF, even though I was “too old to have it.” They  did what were called duodenal drainages. This entailed putting a tube  through my nose and down the esophagus, into my stomach and into the  duodenum at the other end of the stomach. The only problem was that my  nasal passages were too small to allow the tube, with its steel  perforated ball, to pass into my esophagus, so it had to go down through  my mouth. The goal was to ascertain if I was producing the correct  amounts of digestive enzymes. I would have to lie on my right side, with  the tube hanging out of my mouth and draining into an emesis basin that  was placed on a chair next to the bed. This procedure was started at  about 7 a.m. and continued all day until almost dinner time. Of course, I  was not allowed to eat or drink anything while the tube was in. Since  the result was less than what it should have been, the whole procedure  was repeated the next day. This went on for a week.

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They  never got the results they wanted, so they decided to try something that  was quite new back then—a sweat chloride test. Back then, these were  done differently from how they are done now. My back was cleansed with  acetone and a piece of gauze about eight inches square was put on my  back and covered with a large square of plastic that was taped down  completely. The combination was left on for several hours. This also was  repeated for a few times. Since I was allergic to the tape that was  used, my back had blisters and was rather sore after that.

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It  turned out that my sweat chloride was extremely high. The doctors  couldn’t believe that I could have such a high sweat chloride and still  be alive at 12 years of age. They didn’t think that I could survive for  long because I was “so old.” They didn’t bother with many treatments,  since I was “so old.” I didn’t even have to do nebulizer treatments or  sleep in a mist tent. I am so happy that I didn’t have to sleep in a  tent. I think that, if I had slept in that wet environment, I might have  gotten pneumonia more often than I did.

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My own  private doctors couldn’t quite believe that I had CF. What little they  knew about it meant that children with CF couldn’t live to 12 years of  age. They would say that I had a “fibrocystic lung condition.” Their  denial did not help me with my acceptance of the diagnosis. In their  defense, I will say that they had attended medical school in the 1920s  and early ’30s so cystic fibrosis was not something that they studied in  school. After all, CF wasn’t identified until 1938 and it was thought  to be a disease only in infants.

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Having a  diagnosis didn’t change the attitudes of people outside of our family.  They didn’t get it and were proud of their ignorance. At least my own  family did get it and did their best to keep me well and active.

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At the  start of the school year after my diagnosis, a doctor at the medical  school where I was diagnosed sent a letter to my school telling them  that I wasn’t allowed to participate in any physical activities. That  doctor had never even seen me and did not know what would be best for  me, but I had to comply with the edict in the letter. At least I was  allowed to keep up my normal activity at home. I still participated in  ballet and acrobatics, or tumbling as it was called then, and kept very  active. I am sure that activity was much better for me than inactivity.  With all of my movement I was able to cough up the junk in my lungs and  was able to keep breathing.

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Fortunately  for me, at that time I didn’t have too many bad bugs in my lungs.  Mostly, I cultured only Staphylococcus aureus. It was somewhat sensitive  to the antibiotics that were around at that time. There were more  antibiotics available in 1956, when I was diagnosed, and they were not  quite as expensive as the earlier ones.

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It  seemed to me that the docs tried each new antibiotic as they came out.  Some of them had some long-term effects that were not positive. Some of  them caused me gastric symptoms, others caused damage to my teeth, and  still others seemed to do very little.

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I was  able to get through the eighth grade without missing a single day of  school. I realize that for many people that would be no big deal. For  me, it was a very big deal since I had missed the equivalent of three  years of school in my first seven years. My eighth-grade year was the  first time that Asian flu had come around. Only two of us in my class  failed to contract that flu and both of us were the only two with  perfect attendance.

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High  school was another case all together. I started out okay, without  missing any school until January. I was sick for a week or two then. I  missed a little time here and there until the end of third term. I was  studying for finals and suddenly was very tired. I told my mother that I  was going to take a nap and I asked her to wake me in an hour. She  tried to wake me after an hour and was unable to rouse me. She kept  trying for about three hours. My breathing was okay and I seemed to be  sleeping comfortably, so she let me sleep.

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The next  morning, when she still could not wake me, she called my doctor. He  said to let me sleep, as long as I was breathing okay and didn’t seem to  be getting congested. I slept for about three days. At that point, my  parents took me to the doctor. He could find nothing wrong that would be  causing me to sleep. He decided that I was exhausted and needed rest. I  missed the final 10 weeks of that school year. After a few weeks, the  school district sent a tutor to help me with my studies. She tried to  help me, but I was so weak that I couldn’t do much work. Even though I  missed a quarter of that year, I still was able to start my sophomore  year with my class.

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The rest  of my time in high school was much less dramatic and I graduated with  my class. I then went on to nursing school and eventually got a job in a  hospital. I worked in hospitals until I was in my 30s. Since I kept  picking up viruses and infections, my doctors wanted me to stop working.  They were fairly certain that I would be exposed to many fewer ills if I  stayed out of hospitals. They were correct.

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I  applied for Social Security Disability Insurance but was denied. I  appealed and tried a couple more times, but I was denied each time. I  decided that living without the money I was used to earning was much  better than dying from some rotten bug. We had to adjust our budget to  manage without my salary, but we made it and I have lived long enough to  collect on the money that I paid into Social Security.

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I am 76  years old and doubt that I would have lived this long if my mother had  not been so insistent about getting the doctors to pay attention and  treat me for cystic fibrosis. So, even though I was not diagnosed until I  was 12 years old, I believe that it was better late than never.

Please stay well and be happy.

Kathy s

Kathy  is 76 and has CF. She and her husband, Paul, live in Gresham, OR. She  has been a Director, Treasurer and President of USACFA, as well as a  past Editor of CF Roundtable. You may contact her at krussell@usacfa.org.

Welcome  to the first official entry in my “Pearls of Wisdom” column! If you’ve  been reading CF Roundtable regularly, you probably spotted the teaser in  our Fall 2020 issue for this column. So, you know that I’ll be focusing  on lessons learned in my journey as a medical educator and public  health program evaluator living with cystic fibrosis. I can’t think of a  better way to get started than to share with all of you some lessons  I’ve learned about my own CF in the context of my personal and family  history! Like many other things about me, such as my sexuality and  gender, my experiences of both ethnic identity and CF diagnosis have  been complex—and characterized by feeling “in between” different ways of  being.

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In this  issue of CF Roundtable, we’re sharing many different stories about the  diverse experiences of late diagnosis in our adult community. These  include several narratives from patients of color, some of whom have  very diverse racial and ethnic backgrounds and some who can trace their  families’ origins to a few specific places. Across these stories, we can  easily see how the experiences of adult patients who are Black, Brown,  and/or Indigenous have been shaped by racist stereotypes of who can and  cannot have CF. Likewise, we’re featuring several narratives from ethnic  minority patients within the broader community of white adults with CF.  These stories also illustrate complexities in the diagnosis and  treatment journey that can wind up reinforcing other forms of  discrimination and harm.

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Both  racial and ethnic inequality—and the intersection of the two—persist in  the diagnosis and management of CF. The diverse stories in this issue of  CF Roundtable demonstrate the urgent need to address inequity and  injustice in all aspects of both pediatric and adult care. For people  with CF and other progressive diseases, time is often the most valuable  resource we have. An early diagnosis and early treatment can make  tremendous differences in both the quality of our lives and the quantity  of living we can do. So, when people who already experience racial  and/or ethnic marginalization struggle even to get diagnostic testing  for CF, those existing hardships get exacerbated by lack of access to  proper care. In this way, late diagnosis itself can both illustrate  systemic inequality within the CF community and deepen the harms done by  those disparities.

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Like  racism and ethnocentrism themselves, disparities in diagnosis and  treatment begin to do harm the moment a child comes into the world. The  newborn screening that has enabled many white patients born in hospitals  to access quality CF care from their earliest days of life only tests  for a few dozen of the most common CFTR gene mutations. Because CF can  be caused by over 2,300 known variants on the CFTR gene, a lot of us get  missed by that early screening! And of course, this screening was not  even available when many of us who are now adults were born. I was five  years old when the CFTR gene was first discovered and remain the only  known CF patient in the US with my specific CFTR variants! Although I am  racially white—meaning that I am read as white in most situations and  thus experience white privilege—newborn screening would have led my  family to believe that something other than CF was causing my health  challenges.

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Sweat  testing did not help much either, largely because my providers could not  manage to collect enough sweat for a valid sample! I am not good at  sweating; even in very hot environments I will only sweat a little on my  brow line. The fluid that comes out of the skin along my hairline is so  corrosive it will eventually eat through the band on a hat. But,  because the process for collecting sweat sample uses only the skin on  the arms, I never did manage to produce a useful sample—not at age four  when I was originally tested for CF and not even at age 33, when the  tests were repeated with modern methods for research purposes. This was  around the same time I learned what CFTR variants I actually had.  Unsurprisingly, I did not have any copies of either the 508Fdel or other  common CF-causing mutations.

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This is  where my heritage and lineage become relevant. A total of three people  contributed to my physical and cultural history. There’s my mother, whom  I am related to both genetically and socially. Her mother was German  and Danish; her father was Tuscarora and Scottish. So, I knew I had some  Indigenous lineage and heritage from her side of the family.  Culturally, I am Polish on my father’s side—his family lived in Poland  until the early twentieth century. My Slavic ethnicity(1) and heritage  have deeply influenced my social and cultural consciousness. However, my  father and I are not related genetically because I was conceived using  sperm from an anonymous donor.

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I’ve  often referred to this third person simply as “the donor” but have  increasingly come to understand them as something more. Connecting with  members of that part of my genetic tree has made that other part of my  history very real and present for me. Over the past few years, thanks to  genetic testing and a lot of detective work, I’ve been able to learn  about the other side of my genealogy. My biological father seems to be  mostly of Creek descent, from the Natchez region within the Creek  nation. The family also traces a lot of their lineage back to Egypt; a  few Sicilian traders joined the family later on and settled in what is  now central Mississippi. Most of that family—and my half-brother from  the same donor—are still living in the area today.

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So why  does all this matter for my journey with CF? Well, like many patients  with multiracial lineage and/or multiethnic backgrounds, I don’t have  any common CFTR mutations. This means my road to diagnosis was more  complex even after the CFTR gene was discovered, and although I had  presented pretty textbook CF symptoms from my earliest days of life. I  got a tentative diagnosis of CF at age five after my tiny, invalid sweat  samples came back borderline. But this was not confirmed until I got my  complete CFTR genes sequenced at age 33. The time I lost in the interim  will never be given back to me; sometimes I still struggle emotionally  with this. These days, though, I don’t know if I would want to go back  and do anything differently. Too much else in my life—too much that is  good and meaningful and fulfilling—has come from my own struggles in  trying to get a conclusive diagnosis and appropriate care.

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Foremost  among these positives is my unique and nuanced ability to advocate for  racial and ethnic justice in CF diagnosis and treatment. Patients like  me are presently making up for lost time without the benefit of  transformational drugs like Trikafta, because our CFTR mutations do not  qualify us to take modulators. Some of us who may be modulator eligible  have also missed our chance to benefit from these therapies because the  damage our CF has already done to major organs like our livers and  kidneys is already too extensive to allow us to safely take these drugs.

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If you  think my story of not getting a conclusive diagnosis until age 33 sounds  harrowing, consider all the patients with darker skin or other more  strongly racialized features who have had to fight even harder for an  even longer time to get diagnosed and access appropriate care. Several  of my personal heroes in the CF community were in their 50s and 60s when  they were diagnosed. Think about the injustice of that, and also about  the tremendous strength and determination it takes to survive that long  with CF without any guideline based care services. And think about how  all of those hardships intersect with other forms of injustice.

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As a  multiethnic white adult with CF who has survived a lot—and learned a lot  along the way about my own multiracial lineage and how that may have  contributed to some of the struggles I faced in getting a conclusive  diagnosis—I can use my voice constructively to educate, advocate, and  uplift. Really examining how we diagnose CF—and how we intentionally  transform diagnosis approaches and services for patients of marginalized  racial and ethnic backgrounds—will be a vital priority in this time of  so many exciting changes in adult care. Making late diagnosis a thing of  the past will not be possible without robust and sustained attention to  racial and ethnic oppression. By actively focusing on justice for  racial and ethnic minority patients, we can ensure that every person  with CF gets the opportunity to become an adult patient and share our  wisdom with those who are just starting out on this complex journey.

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(1) Ethnicity  is multifaceted. It comes from lineage, experience, socialization,  acculturation, etc. So, although I am not genealogically Slavic, I am  ethnically Slavic because I was raised by a Polish American parent with  all of their customs and history.

Dr. Alexandra “Xan” Nowakowski is 37 years old and has CF. Xan is a director of CF Roundtable, in  addition to being a medical sociologist and public health program  evaluator. They currently serve as an Assistant Professor in the  Geriatrics and Behavioral Sciences and Social Medicine departments at  Florida State University College of Medicine. They also founded the  Write Where It Hurts project (www.writewhereithurts.net)  on scholarship engaging lessons from lived experience of illness and  trauma with their spouse, Dr. J Sumerau. You can find their contact  information on page 2.

Q: I have chronic sinusitis because of my CF. What’s new in the treatment of CF-related sinus problems?

A:  Patients who have cystic fibrosis have a very high incidence of chronic  sinusitis—by some measures, over 90%. Historically, CF-related chronic  rhinosinusitis (“CRS”) has been very difficult to control. Many of our  CF Roundtable readers and their friends have undergone multiple sinus  surgeries and some may still have sinus problems. Moreover, your sinuses  can serve as “reservoirs” for the types of bacteria, like Pseudomonas,  that can then infect the lungs. Typical symptoms of CRS include nasal  obstruction or congestion, nasal discharge, sinus pain or pressure, and  decreased sense of smell. Interestingly, many patients with CF do not  have sinus symptoms, even if their sinuses are chronically infected.

But we  have some GOOD NEWS!  Over the last several years, rhinologists (ear,  nose, and throat doctors who subspecialize in complex sinus surgery)  have developed and studied new and better ways to treat sinuses. A  combination of the proper surgery and the proper post-operative  management can really help patients feel better and keep the sinuses  healthy.

And  there’s more! Early data shows us that the new CF modulator medications  are great for sinus problems, too. As many of our readers know, there  are four cystic fibrosis transmembrane conductance regulator (“CFTR”)  modulators: ivacaftor (Kalydeco), lumacaftor/ivacaftor (Orkambi),  tezacaftor/ivacaftor (Symdeko), and elexacaftor/tezacaftor/ivacaftor  (Trikafta). Our CF Center at Columbia University published a study in  the Journal of Cystic Fibrosis demonstrating that patients taking  Trikafta have a huge improvement in sinus symptoms. Several other CF  Centers have since published similar data. Be sure to speak to your CF  doctor about these options.

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Have more questions? Send your CF ENT questions to us at articles@usacfa.org

Dr. David Gudis is an Ear, Nose, and Throat doctor at Columbia University Irving Medical Center in New York City.

A  few months ago, I gave a talk at the North American Cystic Fibrosis  Conference (“NACFC”) about the importance of integrating sexual and  reproductive health care into CF clinics. After my talk, I received an  email from my pediatric CF nurse, Mary Helmers. In her more than 36  years treating CF patients at Stanford Children’s Hospital (first as an  inpatient nurse, then as nurse coordinator of the adult and now  pediatric clinic), she watched CF transition from a fatal, pediatric  disease to a more manageable chronic condition with many different  manifestations that led to more adult and late-childhood diagnoses.

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Mary  wanted my thoughts on discussing CF-related infertility and future  family goals with her adolescent patients. As we talked, she divulged  that she has a personal reason for wanting to make sure her male  patients understand they will likely need assisted reproductive  technology (“ART”) to have children before they find out the hard  way—her husband was diagnosed with CF subsequent to their infertility  diagnosis. I have known Mary since my own delayed diagnosis, in 1998,  when I was a very sick nine-year-old girl, but I never knew late  diagnosis was also close to her personally. I asked if I could interview  her and her husband, Tom, and they readily agreed.

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Before I  present the interview, I want to give a short background on CF and  infertility. Because 95% of people with CF born with male organs are  born without the vas deferens (the two tubes that transport sperm out to  the penis during ejaculation), they are clinically deemed “infertile.”  This condition is called congenital bilateral absence of the vas  deferens (“CBAVD”). This is distinct from being diagnosed as “sterile”  because most still produce sperm, they just need science to help them  get it out to create an embryo. There are many different ARTs that can  help people with CBAVD have biological children. Testicular sperm  aspiration (“TESA”) and microscopic epididymal sperm extraction  (“MESA”), referenced below, are two methods of extracting sperm.  Intracytoplasmic sperm injection (“ISCI”) is a method of injecting a  single sperm directly into a mature fertilized egg to create an embryo.  During the early 1990s, around the time Tom was diagnosed, many adults  were being diagnosed with CF due to CBAVD when they were unable to have  biological children.

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When were you diagnosed with CF and how did you find out?

TH: I  was 34 years old when diagnosed through UC Irvine Fertility Clinic when  my wife and I were going through fertility treatment to have children. I  had already been diagnosed with CBAVD, and, at the time of the  diagnosis, I did not know it correlated to CF. A physician from Italy  was doing his fellowship in reproductive medicine and was involved in a  research study looking at males with CBAVD and the correlation to CF. I  had lab work drawn for genetic analysis and I was found to be homozygous  for R117H.

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Were there any signs you might have CF prior to your diagnosis?

TH: Yes,  I recall getting bronchitis starting in my teens and throughout  adulthood, as well as having very salty sweat. My baseball caps were  always white from my sweat. I had a productive cough with illnesses and  tended to cough more in the early morning upon waking, especially when  taking a shower (the steam made me cough). I also noticed my mucus was  pasty and thicker.

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How did you feel about the diagnosis?

TH: I  was surprised. Then, I started thinking about it more and realized that I  did have symptoms, and I knew that because I was around people with CF  through Mary’s work. I realized I was salty and had a cough, and those  correlated with CF.

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Can you describe your experience with infertility testing?

TH: Mary  and I were attempting to have children and did not have success in  getting pregnant despite not using protection for six months. We waited  another six months and went to see Mary’s ObGyn. She ordered a semen  analysis for me and lab work only for Mary. Her doctor decided that  doing a semen analysis first was the most cost-effective and least  invasive option. The results came back—azoospermia, meaning no sperm was  detected in the semen sample.

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I was  then referred to a local urologist who performed a transrectal  ultrasound, which confirmed my CBAVD. He referred us to a Stanford  University urologist who told us that we had two options: adopt or use  donor sperm. Our local urologist gave Mary the number of a physician at  UCLA who encouraged us to see two fertility doctors, Drs. Sherman Silber  and Ricardo Asch, at UC Irvine who had some success with pregnancies in  couples where the men had been diagnosed with CBAVD.

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Can you describe your experience and the process you took to conceive biological children?

TH: Our  experience was definitely one no one would probably believe. At UC  Irvine, after two rounds of IVF, each of which required a MESA surgery  for me and a round of infertility drugs, injections, etc., for Mary, we  had no success. Shortly after our second attempt at UC Irvine, we  learned that Dr. Asch had used eggs and embryos from his patients  without their consent and gave them to other women. It was a nightmare  for the fertility world.

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Dr.  Silber then took a group of patients to Brussels, Belgium, to access  ICSI, a newer fertility technology not available in the U.S. After  another MESA surgery for me, many rounds of injections and medications  for Mary, and ICSI to fertilize the egg, we had a successful pregnancy.  We had Matthew, our oldest son (who is now 26), in September 1994. We  later went back to Belgium for a frozen embryo transfer (“FET”) with no  success. We then headed back to California where I had another MESA  surgery and Mary had more medications and injections. John, our youngest  child (who is now 23), was born in July 1997 as a result of a FET.

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We went  through nine rounds of fertility treatments and ultimately, we were  lucky to have had Matt on our fourth attempt and John on our seventh  attempt. Mary did all of the research and never gave up hope. She would  not take “no” for an answer and, were it not for her persistence, we  would not have our two healthy, biological children, Matt and John.

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What do you wish reproductive health care personnel knew about CF?

TH: That  all men with CBAVD should have genetic testing for cystic fibrosis and,  if they test positive, additional testing for any mutations. Their  partner must also get genetic testing prior to proceeding with fertility  treatment. Healthcare personnel should also be aware that some men may  have atypical CF but should still be tested, even if they may not appear  to have CF or experience symptoms indicative of CF.

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How does CF affect your everyday life right now?

TH: I do  not feel it impacts my everyday life. I am aware of it, but I don’t do  treatments unless I get sick and, even then, I may just take an  antibiotic.

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Are you eligible for modulators?

TH: Yes,  I was in the Vertex 770-110 trial back in 2013 and then took Kalydeco  for a bit, but I ultimately decided there was no benefit in me taking  it, so I chose to stop.

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Were there any signs Tom had CF prior to diagnosis?

MH: To  be honest, it was a running joke for us at first. I used to say, “If I  didn’t know better, I’d think you have CF…in fact, I would say you have  CF” since I was a nurse coordinator in a CF clinic. Tom would laugh it  off and insist he didn’t have CF. His sweat tasted very salty and he had  a pronounced cough, especially in the morning upon awakening. He shared  with me that he had frequent bronchitis as a teen and into adulthood.

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How did you feel about your husband being diagnosed with the disease you treat?

MH: It  was definitely weird, but I think a part of me always knew he did have  CF. I just could not figure out how he did, since he did not present  like all my other patients who, at that time in the late 80s, were very  sick.

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When we  went to UC Irvine for fertility treatments, we were asked to participate  in a research study. The fellow explained, “We are doing a study to see  the correlation between CBAVD and men with CF. I am not sure you know  anything about this disease but we would like to test your blood to see  if you are a carrier of a gene for CF.” I said, “Yes, I know about CF. I  am the nurse coordinator for a CF center!”

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Immediately  following this, I called my colleague, Dr. Rick Moss, and asked him if  he was familiar with the correlation of CF and CBAVD and whether he  could evaluate Tom. Needless to say, Tom was worked up at Stanford since  this was all new to them as well. Dr. Jeff Wine, a researcher at  Stanford, had a field day with this in his research lab—being a  homozygote for R117H is 1:1,000,000 odds!

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How did the diagnosis change the way you treat your CF patients?

MH: I  think it was beneficial to the adult CF men under my care at the clinic.  I was aware of the fertility issues which CF men exhibited. I knew the  questions to ask the fertility specialist, I knew what they could  expect, and I knew the cost and potential outcomes of the treatment  options. It enabled me to give the adolescent and adult CF men whom I  cared for better information on male infertility. I know I did counsel  several adult males, a handful of whom had their own children because of  my advice. I felt like it was meant to be—that I had this experience so  I could help my patients. At the very least, they would know that if  they wanted to have children they could and if not, that was OK, too.  That, in itself, was priceless. I know it was for us.

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How has the process of having biological children changed for CF men, from the time your children were conceived to now?

MH: All  treatments are now more TESA/ICSI (non-surgical procedure) rather than  MESA/ICSI (surgical procedure), and there is no more IVF—all embryos are  developed though the ICSI procedure. The fertility clinics know about  atypical males with CF and much more about CBAVD and its correlation to  CF. Advances in fertility treatments are also better and more fertility  clinics pre-screen for CF mutations now, either the basic or extended  panel. Not all 2,000 plus mutations are checked without an insurance  fight, but it is a start!

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What do you think is important for male CF patients to know about CF-related male infertility (CBAVD)?

MH: Most  importantly, it’s important they know that they are not sterile and  they can have their own child if that is something they want.  Unfortunately, I still come across articles stating CF men are sterile,  when really, they are infertile.

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I would  reiterate the importance of knowing the right questions to ask so no one  feels taken advantage of by fertility clinics. It is also important to  make sure their partner gets a full sequencing/mutation analysis to  ensure they do not have a child with CF. If their partner is a carrier,  then I would recommend pre-screening embryos (a/k/a PGD) to check for CF  prior to implantation of the embryos into the partner. If this is  something they can afford or chose to do, it gives them the options but  does not mean they need to follow through with it. We were not given all  the options and I had to fight to get answers. Options are always good.

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On a  more personal note, when I order a semen analysis for a patient, I teach  both adolescent and adult males that they might only have a small  amount of liquid in the container. When Tom gave his sample, the lab  tech came out, pointed to a full container, and said “Excuse me, sir, we  need this much.” It was another person’s sample that filled the  container. Tom’s barely filled the bottom circumference of his  container. It’s unfortunate that the lab tech embarrassed him.

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What are some of the questions you suggest asking at fertility clinics?

MH: Questions to make sure it is a reputable fertility clinic

How long have you been in existence? How long have you been doing male infertility treatments?

Have you had any complaints or violations at the clinic?

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Genetic Testing

Will you test my partner for CF?

Which panel do you use? Can I get the whole panel?

Does my  insurance cover the full panel? Some insurance companies may only cover  the basic panel; however, you can fight that with a prior authorization  since your partner has CF. It may require a letter from your physician  to get the full panel approved.

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Procedures

What are your success rates for men with CBAVD?

Which treatment has the most success: MESA/ICSI or TESA/ICSI

As a cautionary note, ensure they are using ICSI rather than IVF as that is now the protocol for CBAVD

Molly  Pam lives in San Francisco, CA, with her husband Adam and betta fish  Sally. They have been learning bridge during COVID-19 lockdowns. If you  want to play bridge with them online, email Molly at mpam@usacfa.org.

Grace  recently joined the CF Roundtable board with plans to interview other  adults with CF, to learn their (your) stories, and to share them through  our blogs. It made perfect sense to interview her first! Throughout her  young life (she’s 22 years old), Grace has demonstrated such  perseverance and determination, especially during her college years when  she battled severe infections and yet kept up with her courses. Be  prepared to be energized by her strength and conviction. As a bonus,  read about her somewhat humorous experience during one of her  hospitalizations and how it ended up being one of her higher moments.  She’s now enrolled in law school to enhance her advocacy for others.  Please welcome Grace Knight, our newest star. Spotlight please!

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Where do you live?

I am from Tyler, TX, but am attending law school at the University of Texas in Austin where I will be living for three years.

When were you diagnosed?

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I am  homozygous for 508Fdel and was diagnosed when I was a year and a half.  When I was born, I was a normal size, but over the next few months I  didn’t gain any more weight. My mom had a baby book for parents and in  it she read about CF. She was convinced that I had it, but the doctors  refused to test me. She was told by many people that I was fine, and she  needed to calm down, but she kept pushing. Eventually, I was tested and  my parents found out that I had CF.

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What was your major in college?

My major  in college was English with a concentration in creative writing. I  always knew I wanted to major in English, but I added the creative  writing later. During my freshman year I took a creative writing class  with a professor who inspired me. I ended up having to go into the  hospital for six weeks and he worked around the syllabus to allow me to  still participate in the class from my hospital room. I was incredibly  challenged by the class and decided I wanted to focus on that throughout  my college career.

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What challenges have you had during college?

In my  freshman year of college, I was infected with Mycobacteria abscessus.  This bacterial infection was terrifying and it took us a very long time  to figure out what was causing the dramatic downfall in my health. When I  started college, my lung function was 80% and, by January, it had  dropped to 39%. I was exhausted by the time I was hospitalized in  February. I had just transitioned to adult care, so my doctor was fairly  new. He spent a lot of time blaming me for my own illness, even though  there was no way I could have prevented this bacterial infection. I am  very diligent with all of my medications, and there were many times when  I would contact my care team with concerns about my health and would  never get a response. I was in the hospital in Philadelphia for two  weeks with pneumonia. When they finally discharged me, I had recovered a  little bit of my lung function, but I was still really sick. When I had  a follow-up appointment the next week, my doctor told me there was  nothing else he was going to do to help me. He gave up. I was incredibly  angry and frustrated because I was willing to do anything to get better  and he just walked away. At that point, my mom and I decided that it  was best for me to go to another doctor, one that another CF patient had  recommended. This new doctor was in Denver and the next week we flew  out there. I was still going to school during this time, and I was able  to complete my classes online through Skype while I was in the hospital.  Once I got to Denver, the new doctor knew right away that I had  mycobacteria from the CT scan. The culture grew straight off the  bacterial slide! He said it was one of the most aggressive forms of M.  abscessus he had ever seen. The treatment for this bacteria would be  four weeks in the hospital, three months of IV antibiotics after that,  followed by nine months of oral antibiotics. It was an intense cocktail,  and, when August eventually came, I ended up taking a medical leave  from school because I was still so weak. I eventually returned to Penn a  semester later, but my lung function would not recover until two years  later after starting Trikafta. This entire experience shaped most of my  college years. I spent a lot of time catching up from the semester I had  lost. I joined clubs as a junior, when everyone else joined as  freshmen. I went to summer school the following summers, when everyone  else did internships. I was scared, angry, and frustrated by the  limitations my disease held over me, and the worries I carried around  were very different from those of my classmates. This is not to say that  I didn’t have a good time in college, because I definitely did. This is  to say that going to college with CF is incredibly tough, and that  graduating from college with CF is a feat worthy of being celebrated.

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How did the college clinic experience affect you?

School  has always been my happy place, so, when I had to leave it because of my  health, I really struggled. Because I came so close to not surviving, I  was convinced that the next cold I got would take me out completely. I  was really scared and it took me a long time to heal from that trauma. I  improved my outlook by staying busy with things I was interested in and  loved doing. This reminded me of all the good things in life. About a  year after M. abscessus treatment, my friends and I ran a half-marathon.  Completing that race gave me a lot of control over my disease. I didn’t  feel so hopeless anymore and I ran another one a few months later.  Pushing my body to the extreme reminded me that even if CF took me down a  little, it certainly would not win.

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How have CFTR modulators worked for you?

Because  of my mutation, I have been on Orkambi, Symdeko, and Trikafta. I even  did the clinical trial for Orkambi and I gained so much weight that I am  certain I had the drug. However, after being on Orkambi for a while, I  started experiencing chest tightness and major hair loss. I ended up  stopping the medication because there were more side effects than  benefits. Once Symdeko came out I started on that one, too. I didn’t see  much improvement, but it’s possible I got over colds a little quicker. I  didn’t have any side effects so I just stayed on it until Trikafta came  out and then I switched. I have been on Trikafta since November, 2019,  and it has been a dream. I feel incredible and it is almost like I don’t  even have CF anymore. At my doctor’s recommendation I still do all my  breathing treatments, etc., but my quality of life has greatly improved.  I have gained about five to seven pounds, rarely cough, and am able to  breathe so much easier. It has changed my life.

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What are your current health issues?

I still  have digestive issues and am taking the same amount of enzymes as I did  before Trikafta. I tried taking less enzymes once and it was not good  (you know what I mean). However, I don’t have as much discomfort in my  stomach and don’t have to eat as much throughout the day. About two  years before starting Trikafta, I had my fifth sinus surgery, so my  sinuses were still doing well when I started the drug and have continued  to stay clear.

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How does your joining CF Roundtable as a director fit into your passion for writing?

I can’t  remember the first time I encountered CF Roundtable, but it has always  been a huge source of information and support for me. Growing up, I only  knew a few people with CF so being able to read an entire newsletter  from CF patients was incredibly valuable. CF Roundtable has also helped  me financially as I am a past recipient of the Lauren Melissa Kelly  scholarship. I studied English and creative writing in college, so  becoming a CF Roundtable director aligns perfectly with my passion for  writing.

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What pearls can you share regarding the demands of CF and classes?

In  college I found that knowing my priorities was really helpful. My health  always came first because without that you can’t do much of anything.  After that came my classes and friends. When I was sick, I made a lot of  sacrifices with regards to my social life and I made it a point to have  strong relationships with my professors so they would be accommodating  to my needs. I have found that if professors know your name and know you  truly care about the material, they will be much more open to helping  you if you have to go into the hospital, etc. For me this meant going to  office hours as often as I could, being present in every class unless I  was sick, and being incredibly open with them about my disease. When I  did eventually get sick, I always went to them with a plan so all they  had to do was say yes or no, instead of asking them to think of a  solution. I always loved school and going to class so, even when I was  sick, I found my joy in academics.

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What inspired you to go into law?

Not to  be cliché, but it was definitely my experience with CF. Advocacy is an  important part of the patient experience. You have to stand up for your  own health, and, if you can’t, then you need someone who can do it for  you. When I was sick my freshman year, my mom stood up for me when the  doctors didn’t listen. Being a lawyer means being a professional  advocate. It is your job to have your client’s best interest in mind and  get them the best outcome. After years of advocating for my own life, I  decided I wanted to become a lawyer to help others advocate for theirs.

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How do your friends deal with your having CF?

My  friends are incredibly understanding of my disease. They came to visit  me when I was in the hospital during freshman and junior year and they  are always encouraging me to keep fighting. When I can’t go out with  them because I am sick, they completely understand and have never made  me feel different because of my disease.

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Favorite things to do?

I enjoy playing the violin, writing, running, going to museums, and listening to music.

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Do you have an amusing CF story?

Last  summer when I was in the hospital, I was on IV colistin among other  drugs and it made me feel terrible. I didn’t want to eat and was  struggling to keep my energy up. The nurse asked me if I wanted some  medicine to help me eat and I said, yes, please. He didn’t tell me what  it was, just that I could take it before each meal. I started taking it  and I think it helped me eat, but then I would be so tired and lazy  afterward. The next day, about an hour or so after taking the medicine, I  started feeling really weird and started hallucinating. My heart rate  skyrocketed and I felt super anxious and paranoid. The doctor rushed in  and was like WHAT IS HAPPENING? We had to do an EKG and then I had to  wear a heart monitor for 24 hours to make sure I was OK. Turns out, the  medicine they gave me to help me eat was actually just straight THC, and  I’m pretty sure I was tripping from it. I had never done marijuana  before so the whole thing was traumatizing but thinking about how I was  and the whole hospital freaking out really makes me laugh.

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Who or what inspires you?

My  family—their patience, strength, and support—are so inspiring in the  face of such a debilitating disease. It is because of them that I am  still alive today.

Jeanie  Hanley is 58 and has CF. She is a Director and the Past President of  USACFA. Andrea Eisenman is 56 and has CF. She is a Director of USACFA  and is both the Webmaster and Executive Editor of CF Roundtable. Their contact information is on page 2.

If you would like to be interviewed for “In The Spotlight,” please contact either Andrea or Jeanie.

According  to societal standards, I should be married by 25, complete with two  kids, a white picket fence, and a successful career. As if this were the  picturesque life to strive for and anything outside of that wasn’t  considered “good.” However, my life with CF has been anything but  predictable—or plannable, for that matter. There has never been a real  sense of certainty. I’ve learned that fighting what is robs us of joy  and the peace we seek. Accepting the life we’ve been given and finding  the good in it is the best that we can and should do.

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Society  has sold the lie that we are in control of our lives. Consequently, if  our lives don’t go as planned, we freak out. We are used to order,  schedules, and routines. When that doesn’t happen, we are unable to  deviate and adapt. This, in turn, leads to increased emotional  outbursts, irrational thought processes, and complete internal  shutdowns.

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On the  flipside, those of us with CF have learned that we can’t truly envisage  what may happen next. We are seasoned professionals in the world of  unpredictability. We hit every curveball out of the park. It’s what we  do. We can roll with the punches. We’re adaptable. And we’re better for  it.

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We don’t  have the luxury of being set in our schedules, unable to deviate from  the norm. If we’re not flexible, we spend our whole lives wishing for  something that is out of reach: perfection. In reality, though,  perfection is an unattainable goal—for everyone. We hold the illusion of  control with a white-knuckled grip for fear of what will happen if  we’re not in charge. Tuning into what is underneath the pain of not  knowing what will happen next is necessary for growth and healing.

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The  stages between sitting in the unknown and acceptance of the present is  where the tension resides. We all go through different seasons of life  that bring us through various struggles or celebrations. This ebb and  flow between the seasons is a constant throughout our entire lives. We  never have that moment of arrival where everything is perfectly in  alignment. We wish away seasons, that turn into years, that turn into  the course of our lives. Presence in each season is pertinent. We can’t  wait until (fill in the blank) happens before we start living. Life is  being lived right now. We either partake or remain in that place of  longing for something different.

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That’s  not to say it’s not OK to dream, have goals, and tentatively plan the  future. The difference is being content in the season that we are in no  matter the circumstances. It’s not easy. It hurts. It’s hard. And our  hearts long for things to be different. But we have to find ways to be  here, right now, in this space wholeheartedly.

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No, I  didn’t get the life I had envisioned so long ago. And I’m so thankful to  God that I didn’t. I would have never chosen to have my chest sawed in  half, say goodbye to my lungs, and hello to new ones; but, oh, how I  wouldn’t change one thing if given the choice. The greatest gift is  contentment. The ability to find ways to cope and be OK with however  things turn out brings peace.

I’ve had  to come to terms with death more times than I care to count throughout  my journey in life. I’m sure that you have, too. I’ve had to grieve the  life I had envisioned and heal those deep wounds. I’ve learned that  being bitter about circumstances doesn’t change them; rather, it makes  them so much harder to muscle through. Adapting the mindset of becoming  better from what we’re going through has afforded me the opportunity to  learn from any season that I’m in and to be grateful that I am still  here to experience it.

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We have  to learn how to accept what is and be all right with it, somehow,  someway. Yes, we grieve the life we thought we would have. We have to  feel those feelings and sit with the magnitude of letting go of what we  had pictured for our lives. Next, we need to heal that part of us. It’s  important to give attention to our process, whatever that looks like for  each of us. Then, we can step into wholehearted living. Loving the life  we have been granted is essential for cultivating a good life.

Together  we can provide the path that leads to contentment. We all have the  ability within us. If we give ourselves the tools, support, and  encouragement, we can allow ourselves to live differently; moreover, we  can encourage others to do the same. Let us become people who embrace  the life we’ve been granted. In doing this, we will pave the way for  others in their journey toward true joy in the face of any  circumstance.

Lara  Govendo is 34 years old and has CF. She lives in Vermont as a wild,  adventure enthusiast who holds a Master’s Degree in Mental Health  Counseling. She writes about living out loud and develops educational  programs to restore hope to those in need. Thanks to her double lung  transplant in 2017, you can now find Lara traveling on the regular,  exploring the glorious outdoors, and belly laughing with her loves. You  can find her online at www.laragovendo.com (and on Facebook and Instagram) at “Lungs4Lovey.” Her contact information is on page 2.

I’ve  spent more hours than I’m willing to admit in front of my laptop  thinking about ways to discuss CF family dynamics while my 19-year-old,  half-blind, and deaf Yorkie snores next to me. To be honest, I reckon my  relationship with Zappy, in many ways, mirrors the relationship between  me and my family.

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Before I  get into that, however, let me tell you a little about Zappy, formally  known as Zaphod Beeblebrox, otherwise known as, the President of Life,  the Universe, and Everything in The Hitchhiker’s Guide to the Galaxy (by  Douglas Adams). I thought he’d live up to the name by rising above his  circumstances. Fortune never did shine on that ole boy from birth. He  spent his formative years in an abandoned trailer in Apalachin, NY, with  a cruel owner who ran an illegal puppy mill and cooked meth to make  ends meet. It’s worth noting that the town he and I both grew up in  gained national infamy for three things: it was the site of the largest  mafia bust in history (November 15, 1957); it is located in a region  known for being the meth capital of the country; and is the last stop on  the heroin highway (on the southern bank of the Susquehanna River).  With such notoriety, it may come as no surprise that Apalachin is  located in the poorest, most rural, county with the highest poverty rate  in New York State.

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In the  midst of my divorce and bankruptcy in my late 30s, I was trying  unsuccessfully to adopt a friendly shelter dog when I came across an  article in the Sun Bulletin about some very sick Yorkies at a Tioga  County shelter in need of emergency adoption. My heart broke when I read  that Zappy (f/k/a Reilly at the shelter) and three other dogs were  abandoned when their owner was arrested and failed to tell the  authorities that she was living in an abandoned trailer with 24 Yorkies  in southern Apalachin. By the time a dirt biker came across the trailer  in an overgrown field two weeks later, only four of them remained.

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In order  to make it, Zappy developed incredible survival skills—breaking into  cabinets, escaping his holding pen, leaping onto the counter to drink  from the clogged kitchen sink, as well as cannibalizing his fellow  captives, all of which made him a terrible dog by most standards. In  fact, he was so traumatized by the experience that it took weeks before  he’d get out from behind my couch, and it took months before he’d let me  pick him up without trying to bite me. But he survived and, 15 years  later, he’ll occasionally lean against my leg while he naps on the  couch. However, the trauma left some terrible scars: the constant desire  to be alone in the dark, the urge to fight any dog in sight, consistent  barking at any and everything, excessive thirst, regular vomiting, and  pooping/peeing everywhere.

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Unlike  my family, who had no idea what they were getting into before I was  diagnosed with CF, I knew exactly what I was getting into with Zappy and  chose him, anyway. As hard as it is to hear, especially for CF  patients, a recent study found that 90% of children who tested positive,  in utero, for CF were aborted—families would, given the choice, choose  not to have children with CF if they knew what they were getting into  ahead of time.

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Nevertheless,  once a CF sweat test comes back positive, families generally respond to  the devastating news either by becoming CF-focused such that CF becomes  the raison d’être for a family’s existence or by hunkering down to try  to survive the disease while doing as many normal family things as  possible amidst juggling CF emergencies and treatment.

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Like  Zappy, I was, by most standards, a terrible human (infant) from the  moment my mom brought me home. Like most infants, I refused to take naps  or sleep at night; I was covered in my own diarrhea most of the time,  which I liked to smear all over everything; I cried all the time; I had a  peculiar habit of climbing out of my crib and balancing on the  handrail; and, to top all that off, I was sick—I required hours of chest  PT, mist tent cleaning, and room cleaning every day. Most likely, upon  my diagnosis, my mom had to grieve the healthy baby she had wished for.  Fortunately, when I was diagnosed, my family fought hard and initially  was very CF-focused. I was featured in CF promotions, spoke at CF  fundraisers, and made appearances on local TV and/or radio shows to  raise awareness about the then little-known disease that was the number  one genetic killer in the U.S. However, like many families, they  eventually burned out, perhaps when they realized all their efforts  wouldn’t add a day to my life or they couldn’t afford to keep neglecting  their other children, so they gradually shifted to survival mode. It’s  important to note that many families, in fact, don’t survive intact.  Parental divorce rates are 20% higher in CF families, and CF patient’s  siblings often cut themselves off from their family as they grow older  due to jealousy for parental attention; feeling neglected, especially in  childhood; or lack of attention, particularly during critical  milestones or personal achievements (e.g., birthdays, sporting events,  and other extracurricular activities).

In my  case, as I got older, my drinking escalated, run ins with the law  increased, my dad got sick with cancer and eventually died, and my  brothers went off to college and had their own families. Thus, CF became  even less of a priority for my family. To illustrate, in the last 20  years, I’ve had 32 hospitalizations. My mom visited just once, while on  her way to my brother’s house.

Similarly,  when I first met Zappy, I tried everything I could to correct his  learned behaviors but after too many failed attempts, I shifted into  more of a Zappy-survival mode: the constant battle to overlook his  overwhelmingly annoying qualities to meet my responsibilities as his  owner until he passes. Also, I’m somewhat ashamed to admit that since I  picked up my Siberian Husky puppy, Patton, four years ago, I’ve become  way more attached to him, rather than to Zappy, because he loves to play  and always wants to snuggle.

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Having  these two dogs has given me the perspective to better understand,  empathize, and accept my own family dynamics living in a health-centric  world with healthy family members. Thus, I’m not surprised that, when my  family does get together, certain family members try to hide impending  colds because they’re loath to change their plans, particularly if it  means disappointing their children or preventing them from seeing  grandma. On the flip side, particularly in the eyes of older family  members, I have to be okay with mom preferring to see her grandchildren  rather than her son, just as I would rather wrestle with Patton than  blot Zappy’s pee.

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Normally,  I have a rule against giving advice. As the leader of the CF Fighters  for Recovery and Freedom group, I’m often asked for advice and demur  because I strive to empower group members to find their own solutions  and follow their own paths. However, I will make this rare exception on  how to deal with family-of-origin issues:

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Do not  “should” on yourself to go home or visit your family. Only you can  determine if you are socially distancing out of self-preservation (e.g.,  fear of COVID-19 or flu) or socially isolating out of your own  dysfunction.

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If you  have to, keep your visits home short to mitigate risk of exposure and/or  stress that may result from being around certain family members. It’s  better to make a brief appearance and leave on a positive note than wait  until things have soured and leave feeling down.

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Similarly,  if home is far away and you have to stay a couple of days, carve out  plenty of time away from the family dynamics to relax, breathe, and  destress.

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Many CF  patients benefit from individual or group counseling to process family  and life issues, particularly in the winter months when frigid weather,  fear of illness, and family issues abound.

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Above  all else, if it’s emotional wellness that you desire, your objective  lies in finding peace and acceptance with your family of origin, whether  through direct communication or emotional processing in the form of  counseling or self-help. This is true no matter whether your role in the  family requires a lot of patience (like Zappy), or very little patience  (like Patton), or whether your family is CF-centric or in CF survival  mode. Remember, it’s never too late to begin emotional healing, so  there’s nothing preventing you from starting or restarting your journey  today. Good luck, Godspeed, and bon voyage! s

Mark  is 51 years old and lives in Albany, NY, with his wife, MaryGrace, and  stepson. He holds a Master of Arts in Psychology from Marywood  University and a Master of Public Administration from Syracuse  University. Mark has worked for six years in the New York Governor’s  Division of Budget and currently works full time at the Department of  Health. He is the President of CF Vests 4 Life Foundation. He and his  wife love cycling, church ministry, and riding their motorcycles. You  can follow them on their YouTube channel, “Breathing Grace,”and/or join  him at the CF Fighters for Recovery and Freedom group (Wednesday nights  at 7:00 p.m. ET).

I  found myself so angry—not angry because I found out that I have cystic  fibrosis at 33 years old, amidst an unprecedented pandemic, but angry  because I always had a suspicion that I have it. None of the doctors I  had seen in my 33 years had figured it out. Maybe I’d still have a gall  bladder? Maybe my kidneys might be in better condition? Maybe I would  have less damage in my lungs? Oops, I’m being pessimistic again—it’s  hard not to get lost in the “what if” cycle.

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I had  never met a doctor who could solve my mucus problem. Every primary care  doctor from birth to 33 blamed it on asthma, allergies, sinus  infections, bronchitis, and/or pneumonia. All could be solved with new  rounds of inhalers and antibiotics every few weeks and, if I was lucky,  every few months or even a whole year. At 23, in between what had become  normal lung infections, I was physically sicker than I had ever been.  Every time I ate, I broke out in a sweat, I screamed in pain, and I  couldn’t keep anything down. My doctor said there’s no way you’re  pregnant and you’re too young to need your gallbladder removed. This  particular doctor we saw made me take a pregnancy test on the spot. When  he didn’t believe the result, he ordered a blood test. Ironically, the  doctor’s nurse practitioner had prescribed me birth control a year prior  because I had lost too much weight and couldn’t gain it back. Not  unsurprisingly, he was wrong about the pregnancy speculation and my  gallbladder came out shortly thereafter.

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Countless  times I heard, “You have indigestion. Don’t eat these foods and your  mucus will go away.” It didn’t. I also heard, “Have this surgery and  your sinuses will be fixed, then your mucus will go away” or “Take these  allergy shots every day for 30 days and you’ll have relief for up to  six months.” My primary doctors said, “Come in as soon as you have any  symptoms so it won’t turn into pneumonia again.” They were all wrong and  I had lost hope. I stopped telling doctors how I felt and, for a brief  period, stopped going to the doctor entirely (I don’t recommend this). I  remember when someone told me that my cough sounded like I had a yeast  overgrowth from having too much sugar in my diet. That was a new one!  Everyone was willing to offer their unsolicited advice: “don’t drink  milk,” “only drink room temperature water,” “only drink ice cold water,”  “clean up your diet,” “chew gum,” and “don’t chew gum.” I stopped  telling my family and friends how I was feeling. I was so tired of  people asking if I was sick that I made a sarcastic joke about it: “I  don’t get sick.” No one I knew coughed like me. No one I knew was sick  like me. No one I knew had this much struggle, took this much time off  work, and was this tired every day.

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Shutting  down came easy to me. I have never been one to divulge my emotions, my  personal life, or my health. I have treated doctor’s appointments like  business transactions (“I’m good, how are you?”), skipping right over  the long list of symptoms I’ve had over the last three months to take  the spotlight off me. I’ve even spent sessions trying to get my  therapists to talk about themselves so that I wouldn’t have to talk  about me. Money not well spent.

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I hid my  cough as best as I could, but anyone who knows this cough knows that  it’s impossible to hide. Eventually, my cough was too much to bear.  Another few years on and off antibiotics every three months and I was  researching all of the time. What could be making me this sick? For  years I had wondered if I had cystic fibrosis. Based on my research it  had to be CF or primary ciliary dyskinesia. Or possibly early-stage  heart failure according to a Washington Post story I had read. I finally  asked my doctor if I could be tested for cystic fibrosis. He told me  that I would be sicker, that I would have been diagnosed as a baby, and  that I wouldn’t have made it this far. Maybe he’s right, I thought,  maybe it is just seasonal allergies.

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Finally,  in the spring of 2020, I was tested with a genetic testing kit. The  person reading the test asked, “Has anyone told you that you have cystic  fibrosis before?” She explained that I had two mutations that were not  common and that I should go to the cystic fibrosis clinic as soon as  possible to confirm. The first few minutes of my first clinic  appointment confirmed my suspicions but having the suspicion still  couldn’t prevent the shock from my new diagnosis. I was scared because I  had already researched CF and had read the doomsday articles on the  internet that told me I only had five years left to live. Cue the  emotional breakdown.

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It’s now  been about nine months since my diagnosis. My clinic has been so  incredible. This community has accepted me and my “CF light” with open  arms. Despite my rare mutations, I qualified for Kalydeco and started  doing two breathing treatments per day. Finally, my cough is (mostly)  gone. I still can’t believe it. I made a promise to myself that I will  not dismiss my symptoms anymore. I take notes, I ask questions, I go  over every single symptom with my clinic. I was so afraid of finding out  that my only condition was hypochondria that I didn’t fight for the  best care. Some of the best advice I’ve been given from the cystic  fibrosis community is to put myself first, advocate for myself, to be  the annoyingly detailed and thorough patient because no one else is  going to do it for you. And to stay off the life expectancy side of the  internet.

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I wish I  had more to say, I wish I had all the answers. The truth is that I  don’t. I’m still finding out all that’s “wrong” with me. I know I’m not  the only person to be diagnosed during COVID-19 because unfortunately  many of us weren’t tested as babies. To be honest, the pandemic might  have made the adjustment easier for me. It’s hard to feel insecure in a  mask when everyone else is wearing one, too. My hope is that anyone else  who has been recently diagnosed uses the resources available: connect  with a peer, attend the conferences, talk with your clinic, talk to your  social worker, and talk to your dietician. It’s hard to not shut  everyone out, but I know that’s what I need to do in order to accept it  myself.

Megan  Felch is 33 years old and has CF. She lives in Dallas, TX. Megan was  raised in Pennsylvania, studied economics at Penn State, and works in  commercial real estate. Megan likes to spend her free time drawing  portraits in charcoal, exercising, playing with her rescued bulldog,  Winston, and torturing her boyfriend, Nathan. She can be reached by  email at mcfelch@gmail.com.

According to the doctors who diagnosed me in 1956, I shouldn’t be here today; I shouldn’t have lived past the age of 16.

I was  born on a typical day in June—the fourth boy, in what would eventually  be a family of six children. As an 8-pound 9-ounce baby, I was seemingly  the picture of health. It wouldn’t be long before my parents realized  that I was anything but that.

My  childhood was not like that of others. I was chronically ill and  regularly diagnosed with everything from allergies to colds to asthma  and bronchitis to pneumonia. Finally, after years of misdiagnoses and  struggling with health issues, I was diagnosed with cystic fibrosis via a  sweat test at the age of 11.

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I  started regular breathing treatments, but it wasn’t an easy process  since I began at such a late age. My siblings and friends would be  outside playing on sunny, beautiful spring days in our Brooklyn  neighborhood (where I used to be pre-diagnosis) while I followed a  regimented, daily routine of nebulizers and digestive enzymes. Long ago,  the enzymes were in powder form that I had to mix with juice and hold  my nose just to get them down. And don’t get me started on the postural  draining regimen—my mother and brothers had to pound on my back and  chest twice daily to loosen chest congestion. Some days it was painful  to the point that I felt more like a punching bag than a little boy.

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Despite  all of this—and probably because of it—I became a fighter. I chose to  never be defined by my disease, but by my character and resilience. I  stayed as active as possible, integrating exercise into daily routine,  which led to my becoming a student athlete through college.

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I  excelled in college, graduating cum laude, and moved on to a successful  career in the fashion industry, all while no one knew I had CF. Once the  disease inevitably caught up with me, I moved on to volunteer at the  Boomer Esiason Foundation, where I am able to place my drive and focus  on helping others with cystic fibrosis.

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In 2012,  I tackled getting a double lung transplant like I do anything else—I  brought a positive attitude and the determination to get back to living  my life the way I want to live it as quickly as possible.

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Looking  back, being diagnosed later in my childhood may have been a blessing. I  lived 11 years without cystic fibrosis being a constant part of my life  and I believe that ultimately led to the way I dealt with it. I knew it  was a part of me; I knew I had to be vigilant, aggressive, and always  compliant with my treatments; but I didn’t have to let it be the  defining factor in my life.

A cystic  fibrosis diagnosis, even a late one, is not a death sentence. It is a  license to live a life you never imagined through perseverance. You  never give the disease control; you take control over it. If you’re  struggling with it, remember my story, the struggles I faced all those  years ago, and become a survivor. For me, the dreaded, predicted age of  16 came and went—and I was still here, thriving. Don’t get me wrong, it  was never easy and still isn’t. But the life I have been able to live is  worth every moment of the fight.

Jerry  Cahill is 64 years old and has CF. He lives in Brooklyn, NY. He loves  biking, jogging, weight training, and coaching high school pole  vaulting. You can watch his documentary, Up For Air, here: https://youtu.be/p35qG-Bjwig.

I  grew up in a large family with five brothers and three sisters and was  the eighth child in the pack. To top off the enormity of my parents’  caring for such a large household, my sister Theresa, who was a year  older than me, was always sick with bronchitis, sinusitis, and  pneumonia. She was one of the primary reasons that, at an early age, I  wanted to become a doctor—to find answers to this mysterious respiratory  disease, one that doctors couldn’t adequately treat or sufficiently  diagnose. In my preadolescence, I started having the very same symptoms  she had. My hope to someday figure this out took on a new dimension and  became even more urgent. But, I was very young. How much could I do  except study hard, so I could eventually get into medical school.

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Our care  alternated between our family doctor, pulmonologists, and specialists  at academic centers. Theresa, who was the sickest, was tested once for  cystic fibrosis. The doctor told my mom that the sweat chloride result  was not high enough and added that testing was really an academic  exercise only, not a realistic consideration, since CF was not found in  the Hispanic population (circa early 1970s).

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Despite  two more siblings (making four of us in total) developing recurrent  bronchitis, pneumonia, and GI problems, the possibility of cystic  fibrosis was not revisited in my youth. Over the years, my siblings and I  had been worked up for many diseases; none panned out. Instead, we were  given a generic diagnosis of an unusual form of asthma and allergies  because my brother had tested positive for allergies; yet none of us  improved much on the suggested asthma therapies.

During  the first year of medical school, my fellow students and I were often  guinea pigs for our professors’ research. For one protocol, we all had  to cough onto agar plates that, on mine, grew Pseudomonas. The professor  used its sweet-smelling odor as a class-learning experience. I looked  healthy enough on the outside so he brushed it off as a contaminant. If  only I knew then what I know now.

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When I  brought up the possibility of my family members having CF to other  professors, they quickly dismissed it and attributed it to “medical  student syndrome” where students think they are experiencing the  symptoms of the disease being studied. A few also blatantly stated I was  the wrong skin color (olive)/ethnicity (Mexican-American) and/or too  old to have CF at 24 years old. By the time of my med school graduation,  despite the contrary responses from my professors, the seed was firmly  planted and sprouting that cystic fibrosis could explain my siblings’  and my symptoms.

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As our  symptoms progressed to include hemoptysis, Theresa and I were then  diagnosed with bronchiectasis and allergic bronchopulmonary  aspergillosis (“ABPA”). While the diagnosis fit better, it didn’t  explain everything (e.g., Theresa’s GI symptoms, my brother’s  infertility, and the fact that neither my brother nor oldest sister  suffered from the symptoms of ABPA, but did have recurrent pancreatitis  and recurrent respiratory infections).

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During  my pediatric residency, I had treated a handful of kids with cystic  fibrosis. One was Black while the others were non-Hispanic white. The  possibility of CF grew as I realized that not all cases followed the  textbook description, but could affect different races and cultures. The  gene had recently been discovered (1989) and CF genetic testing was in  its nascent stages. There was no way to prove we had CF since our sweat  chloride tests were in the normal range. Although my suspicions were  strong, there was also a seed of doubt. Even so, I believed that I was  getting closer to the answers to our diagnostic dilemma.

I  decided to further my medical training by subspecializing in allergy,  asthma, and immunology and this helped me on my quest to further define  our illness. During my two years in the subspecialty, I had access to  many specialists—not only allergists, but geneticists, pulmonologists,  infectious disease doctors, and others. Many noted my chronic cough and  assumed I was the “typical doctor” and not compliant with my asthma  meds. If I only had a nickel for every time someone said “doctor, heal  thyself,” I’d have pockets overflowing with coins.

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The good  news is that my time in the allergy subspecialty would ultimately pay  off. It began when I attended a national medical conference. As I  perused the program, a presentation about cystic fibrosis caught my eye.  It was starting soon, so I ran over to participate. Excited to make it  (and out of breath), I quickly entered the room. Immediately, I realized  this was not meant for allergists-in-training like me. It was a small  group discussion of 15 prominent CF Center directors, like Drs. Bonnie  Ramsey and Richard Moss, who were running the meeting. A waiter was  serving breakfast and gestured for me to take the one seat available.  Hesitantly, I took it. It was a relief that the group didn’t kick me  out, but, in fact, welcomed me after introductions, even though I was  not a CF director. This meeting was the turning point and led to the  answers I craved.

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Besides  getting a delicious breakfast, I learned about the different classes of  CF mutations, which was groundbreaking information at the time. The  presenters also explained how each mutation class affected different  organs in severity and conferred age-related differences. After the  discussion ended, I presented my family to Dr. Moss from Stanford, who  listened intently, asked a few questions, then encouraged me to get the  family tested with the six CFTR mutation analysis that was available at  that time. I’m forever grateful for his thoughtfulness and  encouragement, which was the first I’d ever received.

Upon my  return, a geneticist I had worked with agreed to test my family, if only  to prove to me to give up this quest for the possibility of CF. At  Christmas time, I took the buccal (inner cheek) scrapings of my parents  and all siblings. The results showed that my three siblings and I who  had chronic respiratory and GI symptoms had one 508Fdel mutation.  Another sibling, with no symptoms, carried the same mutation as did my  mother. Like so many past doctors, the geneticist brushed it off as part  of the CFTR mutation carrier frequency in the general population. But  his ulterior motive of trying to convince me of my folly fell through,  as it only convinced me to forge on. I knew the low sweat chloride threw  everyone off and even I had a trickle of a doubt. I looked too healthy  on the outside even with my chronic, junky cough and many other  symptoms. But, like the Stanford professor, I needed someone who would  truly give weight to my words and think outside the box.

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During  my second year of allergy training, I became even sicker, taking massive  doses of oral steroids and receiving intramuscular shots of tobramycin  and oral ciprofloxacin to control the bacterial Pseudomonas. My  allergist colleague treating me had confirmed ABPA, but also diagnosed  “mucoviscidosis” a catch-all term meaning an illness with thick mucus.  Mornings were the worst for symptoms, yet I always went to work,  inhaling albuterol and Mucomyst in the car, no matter how fatigued I  was. One gloomy morning, there was a Grand Rounds talk on rare diseases,  where a scientific paper was discussed describing two sisters in their  twenties who were newly diagnosed with CF. They both had normal sweat  chloride tests and were diagnosed by a research lab that was conducting  rare CFTR mutation analyses. I felt this was the ticket! As soon as I  had a breather (pun intended) that day, I contacted the principal  investigator and postdoctoral fellow who wrote the paper. They readily  agreed to analyze my family’s genetics. The day turned bright and filled  with hope.

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Thank  goodness for the holidays during which I could take advantage of having  all my family together (yet again!). I drew their blood and sent it off  for testing. Two months later, the postdoc called me to say they had  found the other mutation, D1152H (a class 4 mutation) and that, indeed,  we did have CF. As an aside, he asked if I had Ashkenazi roots since  D1152H had been found exclusively in this population. I didn’t know it  at the time, but, years later, an ancestry analysis would confirm that  we did have these roots.

The  postdoc thought I’d be sad, but I was ecstatic. I finally had the name  of the disease that plagued my family. I had proof and a diagnosis that  fit. My sister Theresa and I were so excited and had to celebrate that  night. We couldn’t have been more eager to start receiving the proper  treatments and therapies.

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Officially  I was diagnosed at a CF center by an excellent USC CF pulmonologist who  was surprised, but knew about adult diagnoses in CF. I was 33 and his  first adult-diagnosed patient. My oldest sister and brother would be  treated at USC too, but not until ten years later. For them, the  diagnosis was more difficult to accept and it prevented them from  receiving proper care.

At my  first CF evaluation, the pulmonologist immediately hospitalized me. This  is when my practical introduction into CF began. No medical textbook  could prepare me for it and I initially felt like a deer in headlights.  Even though I was very familiar with hospital routines, it hadn’t been  as a patient, only as a doctor, and I wasn’t used to feeling helpless or  dependent on anyone. Learning good hospitalization care and routines  from the perspective of a patient took several stays and lots of help  from my CF care team, hospital staff, and other patients hospitalized  with CF, whom I could easily socialize with back then. With the proper  diagnosis and treatments of regular tune-ups, daily therapies and, very  recently, CFTR modulators like Trikafta, my health markedly improved.

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I  believe that had I and my siblings been born today, that either CF or  CF-related metabolic syndrome (“CRMS”) would have been diagnosed at  birth through newborn screening. A definitive diagnosis of CF would have  then been given in early childhood when our symptoms began, rather than  as 30-something adults. There still will be adults in their late 30s  and older who, like me, will be diagnosed late in life because they  missed out on newborn screening which began 30 to 40 years ago  (depending on the U.S. state). I’m hopeful that late-diagnosis journeys  like mine will be a thing of the past.

To this  day, I feel fortunate to have had access to physician specialists and  researchers who readily offered their expertise and helped me to achieve  the correct diagnosis years in advance of the comprehensive CFTR  mutation analysis that would take many more years. The physicians who  dismissed my suspicions also figure prominently in my mind and taught me  an important lesson: to not fall into the trap of only thinking “inside  the box.”

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Dealing  with my late-diagnosis journey, hospitalizations, and CF therapies have  given me a greater understanding and perspective of the health struggles  of others and ramped up my advocacy as a doctor. Reading insightful  books on personal CF journeys like Power of Two were life changing as I  realized I shared many experiences with those who were diagnosed at  birth. This led to my becoming involved with national CF organizations  like USACFA (that produces this CF Roundtable) and CFRI, where I was  able to forge deeper connections with those in the CF community.  Together these connections have eased the journey through CF, and for  this, I’m very grateful.

Jeanie  is 58 years old and has CF. She is a director and former president of  USACFA. She lives in Los Angeles and welcomes your comments. Her contact  information is listed on page 2.

I was diagnosed with CF when I was 20 years old. Yes, I was a sick child; but  I was child with allergic reactions, so most of my ailments were  attributed to allergies. When I coughed up blood, it was blamed on the  feather pillows in the hotel room, wearing a wool sweater, or having  dust-collecting stuffed animals on my bed. It was normal for me to be on  antibiotics for most of the winter. In March, my parents would throw me  and my three siblings in the car and we would drive from Toronto,  Ontario, Canada, where we lived, to the saltwater beaches of Florida.  Hypertonic saline was not an official therapy at that time, but it  usually did the trick and we all returned healthy, tanned, and happy!

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Ironically,  it was my sister’s illness that led to the CF diagnosis. She was always  healthy but started to cough up blood when she was 24 years old.  Doctors tested her for every possible illness, ranging from cancer to  tuberculosis. They eventually did a sweat chloride test, confirming the  CF diagnosis. Testing for all of us immediately followed. My brother;  however, was not tested as he was in the process of adopting children  and did not want CF on his records. Testing confirmed my CF diagnosis.  Later, after my brother adopted his two children, he too was diagnosed  at age 35. This explained his infertility.

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My CF  diagnosis came while I was a student at the University of Waterloo. I  was so happy to have a diagnosis—now I could get the proper antibiotics  rather than throwing broad-spectrum antibiotics at my CF bacteria. I  carried on as usual—studying hard, running track and cross country, and  experiencing all that university had to offer. I was very healthy when I  was healthy. My main challenge was returning to baseline when I was  ill, but a solid dose of antibiotics always worked.

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I  believe that had I been diagnosed as an infant I would have been  sheltered. Instead, I had a very normal upbringing as a child and young  adult. I loved sports and gradually transformed from a varsity runner  into a competitive triathlete while at university. When I graduated, I  became a math and computer science teacher. I balanced teaching with my  passion to swim, bike, and run competitively. Seven years into my  teaching career, I resigned and pursued a career in sports. CF created  challenges, but managing my infections wasn’t an obstacle. I often raced  with an active CF bacterial infection, but my love of triathlon  overshadowed my day-to-day dealings with CF.

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The St.  Mike’s Adult CF Clinic was instrumental in managing my CF and,  specifically, my lung health. Often, I would call from abroad while  racing in Hawaii, New Zealand, or South America, getting advice as to  whether to start a course of antibiotics, increase my inhalers, resume  Tobramycin or the like. But my doctors never told me that I could not  race or train. They knew that exercise made my lungs stronger and helped  me clear mucus. Ultimately, they knew that my body and lungs would  dictate my pace and my effort, and so I was not in any danger.  Ironically, it was my primary CF physician, Dr. Tullis, who suggested I  resign from teaching to pursue sports full time. I was frequently sick  trying to manage a full-time teaching career with training and racing on  the world stage. She reminded me that I could always come back and be a  teacher—now is the time to do sport. I would bet a million dollars that  she has never had that conversation with another patient!

The CF  team was also realistic. While they did deal the hard realities when  needed, they never discouraged my training. One January, while away at a  training camp in Florida, I was advised to return home when Pseudomonas  showed up in my sputum. Dr. Tullis wanted to eradicate the bacteria  quickly and I needed to get on a Tobramycin routine as soon as possible.  Meanwhile, during another exacerbation, the doctors offered to bring an  exercise bike to my hospital room to increase my sputum production  since physiotherapy alone wasn’t working. At that time, my lung function  had steadily dropped from 100% to 55% over the course of six months. I  desperately wanted to avoid IV antibiotics, but I relented and spent the  next five days in hospital followed by five weeks at home on IVs. I was  40 years old and that was my first and last hospitalization. That is  how good my CF clinicians are at keeping me healthy.

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I was  blessed with an amazing career in sports. I raced for 20 years—10 of  those as a full-time professional athlete. I raced 33 Ironman races (3.8  km swim, 180 km cycling, and a 42.2 km run) and won 11 of them. But my  real gift is CF. I don’t think I would have had that success in sports  without it. And that is a bold statement coming from an endurance  athlete whose lungs are her engine. But each time I raced, I had purpose  to bring hope to families with children with CF. As a parent, all you  need to know is that there is one person thriving in spite of CF and  that is hope! And I could be that hope. So, during the difficult and  discouraging moments of my nine-hour races, I always had a reason to  finish and do my best. I made a promise to myself to be the best person  on that start with CF. With a mission statement like that, I could not  fail!

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All any  of us can do is our best with the deck of cards we are dealt. Regardless  of wellness, we must embrace who we are and love our whole self,  including loving our CF. When you are compassionate with yourself, you  can lead with your whole heart and reach your full potential, whether in  life, business, school or sports.

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Yes, CF  has become my superpower despite a collapsed lung, IV antibiotics,  inhaled therapies, and not being able to have children of my own. I have  had more complications from CF in the last 10 years than in my first 40  years. I am grateful that, at 52 years old, I am very much alive, well,  and maintaining a lung function around 88-90%

Let’s all make CF our superpower and open up our lives and hearts to the incredible possibilities.

Lisa  Bentley is 52 years old and has CF. She originates from Toronto,  Canada. She is an 11-time Ironman Champion, speaker, and coach. Lisa is  also the author of An Unlikely Champion. You can find out more about her  and order her book at www.lisabentley.com. You can follow her on Instagram at lisa_bentley123.

9,357  days. That’s how many days I lived my life not knowing what was wrong  with me. So many times, everyone said, “Why do you stay sick so much?”  So many of my teachers believed I was playing hooky: There’s no way one  person can be sick this often.” All I could say in reply was, “I have a  low immune system.”

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Having a  diagnosis to put with my illness is like finally finding out the gender  of your baby. You know you have something in there. You feel it, you  know it’s something, but that confirmation just makes everything real.  Most people would be devastated to hear those two words: cystic  fibrosis. But for me, it was a sigh of relief. I finally had a name to  put on my symptoms. I finally had a reason why I stayed so sick. I  wanted to go back to all of my teachers and say, “I told you I wasn’t  faking!”

My life  has changed drastically since that day. I now have breathing treatments,  antibiotics, peripherally inserted central catheter (“PICC”) lines,  hospital stays, and tons of doctor appointments. Before, when I would  get a cold, it was just that, a cold. Now, the moment I get a small  sniffle, I have to report to the doctor for antibiotics immediately.  There is no cold too small, no sniffle too short. I have to be careful  where I go, whom I’m around, and what I do.

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I wonder  often what my life would have been like had I been diagnosed early.  What would be different? Would I have ever had my child? Would I have  ever gone to college, met my husband, and got married? It’s hard not to  wonder what if. My life is a constant battle of pacing myself. Some days  I feel like I could run a marathon (although there aren’t many of those  days). Some days I feel like getting out of bed is going to put me into  the grave. It’s like walking a tightrope between what I can do and what  I want to do. Will what I do today put me in the hospital tomorrow? Is  that runny nose just a cold or the start of a Pseudomonas infection?

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How many  people have questioned me about whether I’m really as sick as I say?  How many people have said I could not have cystic fibrosis because I’m  not underweight? How many times have I felt like the world is against me  and no one cares?

The  answer is too many to count. I feel like a prisoner in my own body and  mind. It’s crazy to say, but I almost wish I could look sick just so  people would believe that I really am. It’s crazy to even speak those  words, but it’s true. We live in such a superficial society where if you  cannot see it, it cannot possibly be true. If someone has a broken arm,  they get people to sign their cast. But with an invisible illness,  there’s no cast. There’s no sympathy. There’s only judgement and  heartache because our pain is on the inside and not the outside.

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Invisible  illnesses are real. And the emotional toll they take is far worse than  any of the pain we feel. So, when someone tells you they are sick,  believe them. Ask them more about their illness. Study it yourself.  Saying “you don’t look sick!” seems like a compliment, but it isn’t. It  is a slap in the face and essentially, you are calling them a liar. You  may mean well, you may not realize you are passing judgment, but you  are. And the difference in “I didn’t know that, how are you doing?” and  “you don’t look sick; I would have never known” could be the difference  in a bad day and a good day for that person.

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It’s  okay to cut off toxic family members or friends. It’s okay to cry. It’s  okay to not be okay. But just know, late diagnosis doesn’t make you any  less sick than the people who have known from the very beginning. If  I’ve learned anything, it’s that my story can help others who are  teenagers and struggling with no name to their illness. That alone is  enough for me to believe that everything happens for a reason. I am so  thankful that, even though it was late, I now know that I wasn’t just  “being weak” or it wasn’t “all in my head.” Keep searching, keep  fighting—you’re worth it. And if you’re a parent of a child who is sick  and you know deep down something is wrong, keep fighting. Never give  up.

Victoria  Greene is 28 years old and has CF. She lives in Milton, Florida. She is  an aspiring grant writer and mother of a child with autism. She loves  concerts and photography. You can reach her via email at toriw8674@yahoo.com.

Ever  since I can remember, I have been dealing with gastrointestinal issues.  In elementary school, I complained of stomachaches to my parents. I had  recently transitioned to a new school at that time, so my parents,  understandably, wrote it off as nerves. Only, the stomachaches never  stopped.

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Fast  forward to transitioning to a new middle school. I began to notice  bloating that accompanied the stomachaches. Aside from the abdominal  discomfort, I was seemingly healthy. It was easy to blame the bloating  on hormones and adolescent body changes, but I started to feel like  something might seriously be wrong.

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In high  school, it was progressively worse. The stomach discomfort and bloating  were increasingly painful. I began to feel nauseated on occasion. I  struggled with diarrhea and constipation constantly. I was never  overweight but, in search of an answer, I blamed it on my diet. I was  making a conscious effort to eat healthy foods (most of the time). The  diet did not help.

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The day  of my senior prom I was so excited to wear the light blue prom dress I  had somehow convinced my mom to buy me. I was maybe in third period when  I started feeling dizzy, hot, and nauseated. I knew I couldn’t make it  through the school day, so I begged my principal to let me go home early  and still allow me to go to prom that night. He was so kind and  understanding. He reluctantly agreed, but I could see the genuine  concern on his face.

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I looked  at myself in the mirror as soon as I got home. I looked pale and  something was off about the whites of my eyes. I didn’t pay close  attention to it because I was so tired. When I woke up from my nap, I  began the process of getting ready for my senior prom. I did my makeup,  my mom did my hair, I threw on my dress, and I was off!

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Unfortunately,  the magic was short-lived. Not even two hours into the night I began to  overheat, feel nauseated, and so incredibly tired again. I thought I  was going to faint in front of my entire graduating class. I apologized  to my date and called my dad to pick me up. I was devastated.

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I seemed  to be getting worse every day, so my mom scheduled a doctor’s  appointment that following Monday. The doctor confirmed my skin and eyes  were jaundiced. Not surprisingly, the lab tests confirmed my liver  enzymes were elevated. They scheduled more tests to look at my  gallbladder, but ultimately ruled it out as the culprit. During the two  weeks of missed school and in between doctor’s appointments, my entire  body itched. It was so bad that I actually scratched myself raw. I had  no appetite and an unquenchable thirst. When I wasn’t drinking water, I  was sleeping. Thankfully, I started to feel better around week three. I  was so eager to go back to school—I felt like I had missed so much. My  doctor wrote it off as an unidentified virus that attacked my liver and  that was it.

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Except,  it wasn’t. I continued having GI issues. I continued feeling sick. It  was a part of my daily life except some days were worse than others.  Year after year, it got worse.

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Fast  forward to age 23. I worked at my dream job at a great company with lots  of opportunities. I had worked so hard to get there. I tried to avoid  allowing my sickness to get in the way, but at times it was unavoidable.  Again, I was attempting to heal myself with diet, with exercise, and by  reducing stress (as much as possible given I was working 10-12-hour  days). On this particular morning, I could barely get myself into work. I  had skipped dinner the night before and breakfast the morning of. When  lunchtime rolled around, I ordered a healthy sandwich on whole-grain  bread. It sent me over the edge. I began feeling dizzy and nauseated.  Sound familiar? I aggressively swung open the glass doors, ran by our  office security, and shut myself in the sick room where I hovered over a  trash can and hoped for the sickness to pass. When I realized it wasn’t  going away, I walked to my desk, grabbed my purse, and quickly exited  the building without telling a soul. I drove myself home and called my  mom who knew this was out of character for me and urged me to go to  urgent care. On my way out the door, I was hallucinating. I blamed it on  my contacts shifting, got in my car, and drove myself to urgent care.

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I busted  through the doors hoping the staff could see me right away. That wasn’t  the case. I took a seat where I patiently waited to see a doctor. By  this point, I was drenched in sweat and hallucinating yet again. The  windows appeared to be waving like blacktop does on a hot summer day,  but it was the dead of winter in Ohio.

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They  called my name and took me back to see the doctor who gave me  anti-nausea tablets and asked if I felt better. I didn’t. He took my  temperature and immediately called an ambulance. I spent three days in  the hospital. I was diagnosed with IBS and gastroparesis. I imagine this  sounds familiar to many late-diagnosed CFers.

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Life  went on. I continued to focus on my career. Yet, every year I grew  sicker and sicker. Five years ago, when I met my now husband, I told him  I knew something was wrong with my health. He believed me and he stood  by me when even my doctor of over 12 years refused to believe anything  was wrong. I begged for more tests. I begged for help.

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Fast  forward to age 28. It’s the morning of our wedding and I felt sick. I  think I was running on adrenaline, because it was, nevertheless, one of  the best days of my life. The morning after returning home from our  honeymoon in St. Lucia, I woke up and I knew something was seriously  wrong. There was so much pain in my upper abdomen. I was bloated beyond  belief. I snuck out of bed so I wouldn’t wake my husband. I began the  process of scheduling a virtual doctor’s appointment for that day,  hoping it was as simple as a prescription they could send to the  pharmacy. The pain worsened and I decided it was best to go to my normal  doctor in person. When my husband woke up I told him something was  wrong, and I was going to the doctor. He offered to drive me there and,  as the exit approached, I told him to keep driving because I thought I  was dying. He sped to the emergency room. It felt like my insides were  tearing apart. I couldn’t feel my extremities and apologized to him and  told him I loved him because I genuinely thought that was my last day on  earth.

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The  emergency room doctors confirmed I was suffering from pancreatitis. They  explained that drinking is typically the cause and told me I probably  had too much to drink on our honeymoon. I was treated like an alcoholic  or a typical junkie with pancreatitis even though my toxicology test  showed nothing was in my system. I was treated this way even after  explaining, in chronological order, my past medical episodes to the  medical staff. No one documented what I said. No one believed me.

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Over the  next year and a half, I continued to experience bouts of pancreatitis.  It became a dark cloud that hovered over me. I was in a constant state  of fear wondering when I would be balled up in the fetal position and  crying in excruciating pain for days on end. I was treated like an  addict when I asked for pain medication. I truly cannot describe the  pain and agony that accompanies pancreatitis. It is, by far, the worst  pain I have ever endured.

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I knew  there was an underlying cause. I knew it was related to my past medical  episodes. All I needed was for a doctor to listen to and believe me. I  sat across from my doctor during my current bout of pancreatitis, which  lasted over three weeks. I went over my previous test results from prior  medical episodes, insisting they were all related and begging him to  help me get to the bottom of it. He told me all of it was “normal” for a  20-something-year old and asked me if I had ever considered anxiety  meds.

That was  our last appointment. I mustered the courage to challenge my doctor’s  opinion. I found the confidence to leave him in search of a doctor who  would help me. And, that is exactly who I found and exactly how I was  diagnosed. By this point, I had permanent sinus damage and scar tissue  covered 10 percent of my pancreas. Because of this new doctor, I didn’t  suffer further damage. Because of this doctor, I was able to take  Trikafta, which I like to refer to as a miracle medicine. This  medication has turned my life around. Thankfully, I haven’t experienced a  bout of pancreatitis since I started taking it. I have more energy and I  feel so much better. I laugh more, smile more, and every day is a gift.

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Cystic  fibrosis, for me, was hearing “you don’t look sick” so many times, I  lost count. It was sleeping with a heating pad velcroed around my waist  for three years straight, even though the outside world didn’t see that.  A large portion of my paychecks went toward medical bills I could  barely afford in my early 20s, even though no one knew that was why I  was broke. CF meant countless doctor’s appointments and a lot of normal  test results that very few saw. It meant canceling plans at the last  minute because I felt sicker than on the day I had agreed to go out with  friends and family. CF meant calling off work at the last minute  because I knew I couldn’t mask my symptoms that day. It was working an  eight-hour day during an active bout of pancreatitis, despite no one  having a clue.

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Cystic  fibrosis for me was also being my own best advocate. It was never giving  up even when I felt hopeless. It was me finding my voice, believing in  myself, learning my strength, and being the captain of my own ship.  Cystic fibrosis has many different faces and I am one of them. I’m  relieved to have a diagnosis and I’m so incredibly grateful there was a  medication available to me that truly gave me a second lease on life. I  will never take it for granted.

Danielle  Lassak is 30 years old and has CF. She lives in OH. She’s a wife, a dog  mom, a cat mom, and an IT project coordinator. She enjoys decorating,  cooking, and traveling! She can be reached via email at Danielle.Lassak@gmail.com.

I  can’t imagine a life without cystic fibrosis. Sometimes, it boggles my  mind to think that, for nine years, I was a relatively healthy kid. That  is, until I developed relentless walking pneumonia at eight years old.  My pediatrician couldn’t figure out what was going on and I was getting  sicker.

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I  remember the first time I heard the words cystic fibrosis. I was sitting  in an examination room with both of my parents, and a doctor we had  never met walked in holding a picture of my lungs, indicating the right  one had collapsed. He told my parents, “This to me looks like cystic  fibrosis.” I later learned things must be pretty serious if the  radiologist walks into your room. Within a few weeks, I was being tested  at my local children’s hospital, Le Bonheur, for sis-tick  fy-bro-sis—wondering why I had two diapers wrapped around my now sweaty  arms. It was my first sweat test in a hospital that would later become  sacred to my healing and where I would undergo many more sweat tests. I  was never admitted to the hospital throughout this process, a decision  that I now think was probably a poor one. But I recovered well at home  with oral antibiotics and the new medications and treatments to heal my  lungs.

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I  remember the day we found out I have CF. I remember sitting in the car  with my mom, asking her if I was a survivor, like in Destiny Child’s new  song, knowing with some granule of intuition that my life was on the  cusp of a massive transition. I know my diagnosis came as a shock to my  dad and was followed by a long period of grief and mourning. But my mom  has a different story. My mom is an immigrant from Venezuela, the  daughter of a former head nurse of the tuberculous unit in her city. My  mother grew up surrounded by and immersed in the world of not only  medicine, but pulmonology. Sputum cultures, x-rays, bronchiectasis, and  lung inflammation were all familiar terms, reminiscent of a childhood  4,000 miles away. I think this background helped empower my mom to  advocate for her daughter—a skill set she homed in on the first weeks  after my diagnosis. Like I said, I was never admitted to the hospital  when diagnosed, despite a collapsed lung and pneumonia. In fact, the  pulmonologist I first saw made a poor impression on my mom when they  remarked we were unlikely to be able to afford the Vest. From there, my  parents found a new doctor to treat me.

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My new  doctor wasn’t trained in pulmonology, rather in allergy and immunology.  However, his father was one of the pioneering doctors in CF medicine. We  had met him serendipitously and I believe it was because of him that I  fared incredibly well for the next five years. But, as we all know,  cystic fibrosis is unpredictable and shameless. I started having massive  hemoptysis and soon surpassed the scope of his practice. It was  terrifying not knowing what to do next—the memories from my diagnosis  haunted my mom and me. However, my pediatrician (another Venezuelan mom)  pushed for an admission at the children’s hospital.

And so, I  was back in the place where I was diagnosed, even sicker than when I  first went with a collapsed lung and double lung pneumonia. This time, I  was sitting at the edge of life and death multiple times a week. And,  as luck would have it, the hospital had just hired a new chief of  pulmonology—a well renowned man from Kentucky (“Dr. S.”).

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Without a  doubt, Dr. S. saved my life. We quickly became friends, despite the  fact that I was the sickest I’d ever been. This relationship has endured  past my pediatric days. The three of us—Dr. S., my mom, and I—learned  to advocate for my body. Between Dr. S. and my mom, I learned how to use  my voice, to not fear asking questions, and to foster a love for  anatomy and experimental medicine.

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It’s  funny, I had always hated my children’s hospital because of how I was  treated at diagnosis. Life, however, has a funny way of working out.  Now, Le Bonheur has become a place that is, to this day, sacred and  critical to my healing.

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Remember  how I said that this hospital would become a place for many, many sweat  tests? I still go there, at 27 years old, because it is the site for  many drug trials. I think in the last two years, I have had over 15  sweat tests, this time without diapers!

I am  amazed at how my story has taken different turns and twists. I think of  myself as that skinny, sick nine-year-old child with a collapsed lung,  wondering if I am a survivor. I also think of myself today: many sweat  tests later, with curves and hips for days, and two powerfully  functioning lungs. Who would have thought? I think of the hospital that  diagnosed me and healed me, many times over the last 17 years. And, I  think, yes, baby Luisa! You are a survivor and, more importantly, you  have persevered with some of the kindest and most resilient folks at  your side.

Luisa  is 27 years old and has CF. She lives in Memphis, Tennessee. Through  her writing and rambling, she injects her perspective as a Hispanic  woman and as a lover of life and stories in all her interactions. Luisa  is an avid coffee lover and worldwide adventurer. In her downtime, she  practices Portuguese and takes selfies with raccoons. Follow her on  Instagram at @ladilla93 or email her at luisapalazola@gmail.com.

I  was diagnosed with cystic fibrosis in 1992 at 24 years old. While I was  growing up, doctors had always treated me for bronchitis. I started  having more severe respiratory symptoms when I was 19. In 1988, I had  pleurisy and pneumonia. It wasn’t until years later that my illness  reached a dire state.

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I had  gotten to the point where I wanted to find out what I had so that I  could be treated appropriately. My mom and dad took me to some good  doctors to try and figure out what was wrong with me. I went to an  allergy specialist and a lung specialist—both doctors told me to get out  and live my life because they thought I was going to die young. This  was a very frustrating and depressing time for me—I just kept getting  sicker, until I found a doctor who really cared about me. This doctor  had me do a sweat chloride test, which turned out to be negative. He  sent my case before a group of doctors who determined that if I did have  CF, it was only a mild case. I tried to check into Vanderbilt  University Medical Center, but they didn’t have an adult CF clinic at  the time. When I went to Memphis, I saw a doctor who had been treating  people with CF. They ordered genetic testing—CF showed up in the  results. I’m glad I didn’t learn that I had a terminal illness until I  was an adult. I think it would have made it more difficult growing up if  I had known that I had a serious disease. The only resources I had were  just a few people with CF whom I had met while going back and forth to  Memphis.

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Eventually,  in 1995, I went back to Vanderbilt where they had just started an adult  CF clinic. I met some friends who, unlike me, had gotten diagnosed  early in life. Vanderbilt had what they called “CF days.” They had  families get together twice a year. Dr. Preston Campbell was one of the  doctors there; the whole team was awesome. He had invited the CEO of the  CF foundation to attend. The foundation gave various educational  sessions that were very helpful.

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I’m  fortunate to have a mild case and to not have been as sick for as long  as most CFers. I didn’t let CF dictate who I was. I stayed above it by  working my butt off. I could not go to bed wheezing. I would get up and  do my Vest, hypertonic-saline, and flutter as much as it took. However,  CF took a toll. Around January 2012, I became very sick and I was  eventually listed for transplant. I was on the transplant list for 18  days, receiving a double lung transplant on May 30, 2012, at 44 years  old.

I feel  very grateful to have met my donor’s family. He was 22 when he passed  away. My donor was married with two beautiful girls. We were blessed to  have spent Christmas with his family in 2018.

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I have a  different philosophy than what I’ve heard other people with CF say. For  some, it makes them cherish life more. Personally, I hate CF! I would  not choose to have it. I would hope that I could have a zest and care  for life without it and still have a close relationship with God.

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Making  new friends has been the biggest resource for me. I enjoy attending the  virtual conferences. I love talking to people who have CF and have had a  double lung transplant. It seems like we have a deeper connection than  before I got transplanted. My advice for newly diagnosed patients is to  keep regular appointments, commit yourself to staying on top of CF, and  don’t worry about your PFTs—it’s just a number. Make as many CF friends  as possible. Love, laugh, and be your own normal. Become a child of  Jesus, putting your faith in Him; I wouldn’t be here without Him. Be  your own advocate. Keep the doctors in check, even if you have to be a  pain in the butt! I had one doctor out of at least 20 who understood my  fight for life. She happened to be the only CF doctor who visited me  after my transplant.

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CF can  be lonely. It’s hard to find someone to love you along with the demands  of CF. I went through a bunch of heartaches before I finally met my  wonderful wife—beautiful Alli, who is a nurse. God couldn’t have put me  with anyone better. We met in 2008 and got married on May 30, 2013,  exactly one year after my transplant. Now we get to celebrate my new  life and our new life together on the same anniversary. I also adopted  her son Hayden, whom I am very close to and love with all my heart.

I hope  my story helps give a different insight into how CF affects me and  connects us to each other. Having CF has changed my life. I cherish life  more after having had my transplant. When you’re faced with death, it  should change your perspective on life. It has given me more of an  affinity for people who suffer and are sick. Life is precious. Live it  one day at a time!

Darryl  Collins is 52 years old and has CF. He lives in McMinnville, Tennessee,  with his wife, Alli, and son Hayden. Prior to his double lung  transplant in 2012, he taught physical education. Currently, Darryl is  an adjunct professor at MTSU and has coached girls’ and boys’ basketball  for 17 years. He enjoys being active and has been an enthusiastic  participant in the Transplant Games of America. You can reach him by  email at collinsd1968@gmail.com.

The  information provided below is only one individual’s personal experience  and is not meant to be medical advice. Please contact your CF care  center if you have any concerns about COVID-19 or other CF-related  illnesses.

While I  downplayed the symptoms in my head, reality crept in. A 102-degree fever  plus body aches and fatigue equaled a sickening dread in the pit of my  stomach. My husband tested positive for COVID-19 and, as he came back  into the house with his mask on to grab clothes in order to  self-quarantine in a different location, I couldn’t help but wonder if  this would be the last time we would see each other. It was an emotional  departure as our six-year-old daughter and I stood sobbing on the front  porch. Turns out, we were able to reunite two days later when I came  down with similar symptoms and tested positive for COVID-19 as well. It  hit us so fast. Suddenly, our anxiety and worries if we would survive  overshadowed the chore of what to fix for dinner. Would this tear apart  our family? Would my daughter grow up without her mom or dad or both? I  needed to snap out of it and had a pep talk with myself, vowing to focus  on the positive and draw from that energy. Listen up self! There will  be no panic!

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My  daughter was not tested per the doctor’s recommendations as she didn’t  have symptoms; thus, we quarantined and monitored her condition.  Thankfully, my daughter remained mostly asymptomatic except for a dry  cough. So, on the first day after my diagnosis, I climbed a tree with  her. Why? Because she asked me to—because it was my way of taking  control and because it’s what a parent does for their child. It could  have been my last chance to ever climb a tree with my daughter, and I  was not going to miss the opportunity. I will tell you that it took  every ounce of energy to climb the tree, and all of my joints and  muscles hurt beyond belief—worse than any flu aches and pains. However, I  did it and it felt good to breathe in the fresh air, listen to the  birds, and watch the butterflies. As we talked and played make believe, I  savored every moment. We were in the present enjoying life.

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It has  been over a month since the first day of symptoms. I am pleasantly  surprised at how well both my husband and I have recovered. Neither one  of us had to go to the hospital and our daughter has remained  unaffected. Although I am not back to my baseline and do not know  whether my lung functions will suffer any long-term repercussions, I do  feel strong and energetic. So, I wanted to share a list of things I did  to heal and promote recovery from COVID-19.

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1. Water—I drank water like a camel preparing for a trip to Timbuktu.

2.  Sleep—the fatigue is real. Sleeping was key the first few days. I’ve  heard that the fatigue tends to linger off and on for months afterwards.

3. Upon  the advice of my CF team, I ordered a pulse oximeter online and  monitored my peripheral capillary oxygen level (SpO2) regularly. Any  reading below 90 is not normal. An SpO2 reading below 88 can be  dangerous and might warrant a trip to the hospital.

4. I  continued taking 50 mg of zinc every day. Zinc is a mineral with  antiviral properties that helps strengthen the immune system. Zinc is a  preventative and essential nutrient—your body does not produce or store  zinc on its own, yet it is needed for many metabolic processes.

5. Vitamin C to help my immune system.

6. Whole  food dietary supplements that contain concentrated fruit and veggie  extracts. This is a normal regimen for our family. When I run out, there  is a noticeable energy deficiency. Ideally, we should have doubled our  doses; however, we were running low and I wanted to make it last so all  our bodies would benefit during Covid-19 infection.

7. ADEK vitamins—continued normal regimen of one vitamin per day.

8.  Buried Treasure ACF Liquid Rapid Immune Support—this is an  over-the-counter, immune-boosting supplement. I discovered this a few  years back and always take it when I first feel the onset of a cold.  There are over 15 different natural ingredients, so, if you are  considering this product, make sure to discuss with your doctor.

9. Per  doctor’s orders, I doubled my existing nebulizer treatments—albuterol,  3% hypertonic saline, and Pulmozyme. I also continued my normal course  of inhaled antibiotics, which happened to be Tobi that month.

10. Increased airway clearance.

11. Continued use of 500mg Azithromycin three times a week

12. Tylenol for my throbbing headaches, which were compounded by any screen time whatsoever.

13.  Movement—after two days of lying around and sleeping, I realized it only  made the body aches worse and my lungs felt congested. Even though I  had no energy, walking for short periods of time gave some relief. There  is a delicate balance between activity and resting during illness—both   are needed.

14. Miso  soup and sweets were the only things that tasted good. I was able to  maintain a small amount of taste until the eighth day. Smell was  completely gone by the sixth day. My appetite was zero from the onset of  symptoms.

15.  Sinus rinses with the addition of Alkalol every other day. Alkalol is an  over-the-counter saline solution containing essential oils and natural  extracts (eucalyptus, spearmint, and menthol, to name a few) that act as  a mucous solvent helping to clear nasal passages for sinusitis,  post-nasal drip, and allergies.

16.  Trikafta—the drug that without a doubt gave my body the extra boost to  recover and most likely prevented hospitalization. The heartfelt  gratitude I feel for the timeliness of this modulator is indescribable. I  remain hopeful for the 10% of our CF community to have a modulator  medication available as soon as possible.

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While  taste and energy have returned, sadly my sense of smell is not back. It  was diminished before COVID-19 due to chronic sinusitis and multiple  sinus surgeries; however, now it is worse. I did not completely realize  this until my daughter and I did a turtle nest dig with the UCF turtle  guide well after recovery from COVID-19. After turtle nests hatch, they  are dug up to count the number of eggs that have hatched, among other  things. As you can imagine, this is a stinky process. While the guide  and my daughter were turning their heads away from the repugnant smell, I  could not smell anything. COVID-19 often causes anosmia, a temporary  loss of smell. So far, my olfactory senses haven’t returned but c’est la  vie; if that is the extent of long-term damage, I accept this small  loss with grace and humility.

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Looking  back, past CF exacerbations have left my body more debilitated than  COVID-19. Unfortunately, not everyone has been so lucky, those with and  without CF. This time, there were no feelings of trepidation that my  time on earth was ending. However, as I write, my good friend who does  not have CF, has been diagnosed with COVID-19 and is fighting for her  life in the ICU. With a heavy heart this leads me to search science for  cause and effect of this strange virus. It has affected so many lives in  an odd variety of ways.

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All of  this leads back to balance. A popular theory based on genetic analysis  reveals horseshoe bats as the origin of this novel coronavirus. Wildlife  trade, markets, urbanization, and farming threaten biodiversity around  the world. History has shown us that all of these foster opportunities  for viruses to become zoonotic diseases. Mass clearing of forests,  tremendous amounts of fertilizer and pesticides continually feeding into  aquifers, harmful farming practices, and construction on wetlands are  throwing ecosystems out of balance causing an alarming rate of species  extinction. We, too, are becoming imbalanced, resulting in an unhealthy  society, both physically and mentally. This is a wake-up call to look  within and rediscover personal equilibrium in the hope of spreading a  more cohesive, sustainable way of life in order to heal each other and  our home—planet earth. It all seems so daunting, much like cystic  fibrosis can be; however, not impossible if we continue to persevere,  making one small change at a time. As Francis J. Braceland said, “[w]e  can be sure that the greatest hope for maintaining equilibrium in the  face of any situation rests within ourselves.”

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Author  update: As of day 41, Sonya received a negative COVID-19 test. Her  antibody tests came back positive on day 50. Her pulmonary lung function  tests only dropped by three percent. She still has not recovered her  sense of smell. Most importantly, her great friend has walked out of the  hospital and is on the road to recovery!

Sonya  Ostensen is 45 years old and has CF. She lives in Melbourne, Florida,  with her husband and daughter. She received her Bachelor’s in Science in  Environmental Sustainable Resource Management from Ohio State  University. She loves to travel with her family and experience new  cultures, and she has a passion for wildlife rehabilitation. Her  favorite activities include gardening, baking, walking the beach, and  climbing trees with her beautiful daughter. Sonya is a CF Roundtable director. Her contact information is on page 2. You may also message her through Facebook at Facebook.com/Sonya.Ostensen

This article is not intended to be medical advice or curative. Please always talk to your doctor before starting new treatments.

Cussing  has helped me with so many CF problems. Seriously, just yelling  “$&*(!!” at the top of my lungs to my backed-up bowels has literally  blasted my way out of a sticky situation many a time. With such splashy  results, I wanted to be sure I shared my trick with the CF community.  You would be surprised just how many problems get solved by insulting  your mucus plugs’ ancestry. Why, just yesterday, I was sitting on the  couch, and I insinuated that a mucus plug’s mother was unworthy of love,  and—pop!—I coughed it up! Another time, when my liver enzyme levels  were high while I was a teenager on IV antibiotics, I expressed my  desire for my CF-related liver disease to put various suggestive objects  into its inflamed bile ducts. The next thing I knew, my laboratory  results were credited as a medical mystery.

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The most  memorable time cussing has helped me fight CF was an episode of  coughing up blood. I could not stop for the life of me. I had gone to  the hospital because of an earlier bout of hemoptysis after an  increasingly frequent series of gory episodes had concerned me and my  medical team. As night loomed outside, I lay myself down to sleep and  saw all the lights from various medical equipment reflected in the  window. Just as I set myself horizontal, I could feel bubbles gurgling  in my chest, and I immediately sat upright again, coughing. I grabbed a  pink, kidney-shaped bowl and spat blood. The thing with coughing up  blood is how easy it is to keep coughing up more, however much you know  it’s not good, it’s not right, and desperately want it to stop. As I  spat again and again into the bowl, I thought to myself “this blood  couldn’t get laid in a textile-free bar! #$%@ you! Your mother urinates  like a fire hydrant.” Suddenly, the blood stopped. My FEV1 skyrocketed. I  have no idea why this works, but it just does.

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My first  experience with this has been with bowel obstructions. They have also  presented me with many an opportunity to practice this trick. When I was  12, I had such an intense series of obstructions, I had been going back  and forth from the hospital back to my home almost every week. Each  time I went back, the doctors put their exciting enemas to work. I often  felt such pain that I would cry out. It was only when I begun using  language best known for peeling bananas that I noticed my stays at the  hospital would be shortened. I began to experiment. I found  “*#&$!!!” to be far more effective than “#&$*!!” while “@#&$  $#@$ ^@#%!!!” could be used with great results. My poor mother required  quite the explanation, but, once she understood, she joined me in my  quest for health. Even my nurses, upon understanding what we were doing,  would vigorously chant “#&$*!!” with my mother while I strained on  the toilet. The resounding echoes of our cussing in the bathroom would  soon be joined by a productive splash and evolve into cheers of joy.

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I must  warn you, sometimes this works unexpectedly well. I caution you of a  time I was riding my bike. Another rider rudely cut in front of me  without warning, and I had to swerve mightily to keep myself balanced. I  assumed only someone with a bowel obstruction could behave so rudely in  their rush to get home. With that in mind, I kindly informed this other  rider in quite colorful language that his mother has the wit of a  “@#&$ing” flea. He turned his face to me (I presume to thank me)  when his eyes suddenly widened in astonishment and his eyebrows vanished  under his helmet. I looked at him in confusion until I noticed the  pants clasped tight around his respectable bottom had suddenly turned a  much darker shade. Soon, something frightening began to drip through.  The poor fellow—I had not waited for him to at least hop onto a toilet! I  could only console myself with the fact that he no longer suffered from  intestinal distress. What a lesson we both learned that day.

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With  this newfound knowledge, I hope you, dear reader, have a new tool to  face CF. Remember that practice makes perfect, and, when your friends  join in, you can accomplish so much. Your health, I hope, will reach new  heights, and you will soon feel an unending peace. #&$* you,  #&$* your health, and have a wonderful day!

Devin  Wakefield is 29 years old and has CF. He lives in Seattle, WA. In his  free time, he likes to run, hike, and backpack. His contact information  is on page 2.

For  most of us with CF, it has become a part of our daily lives and  responsibilities. We have to do our treatments, go to doctor  appointments, and sometimes even be hospitalized. So, we have to learn  to make our treatments a part of our daily routine. The life of a  student who has CF is very different from that of the average student.  However, we must learn to adapt and overcome to progress and continue  our lives.

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Around  my third year of high school, I started having major issues with my  liver and, after about two months in the hospital, I was placed on the  transplant list. I had some issues throughout my last two years of high  school and missed some days here and there but got through without major  issues. However, when I was applying for college, I realized I needed  to choose my college carefully as it could mean needing to change  doctors, having to find a new liver transplant center, and a new  pulmonary doctor, along with all my other specialists. This could  potentially mean complications in my care as my doctors wouldn’t know my  case as well as my current team.

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So, I  had a big decision to make when choosing my college while considering  the added pressures on my health. For this reason, I applied to colleges  within four hours of my transplant center (four hours being the time  they give you to get to the hospital after you get the call that they  have an organ). Toward the end of my senior year, I had more health  issues and realized that I needed to stay right where I was currently  living. For this reason, I applied to my local community college and  took classes online. I felt sick at random times, and this allowed me  the flexibility I needed for study and work.

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The next  year or two, I continued studying at a community college while taking  care of my health. I did well but, quite frankly, I did not perform up  to my standards. I tried putting more time in and worked harder, but it  only helped a little. About this time, my transplant clinic called to  let me know they had a liver ready for me. I arrived at the clinic and,  about five to ten minutes later, I was being rolled into the operating  room. After my transplant, I contacted the school and withdrew from the  semester in the full expectation that I would be back the following  semester to continue my classes.

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Unfortunately,  this was not the case. I had various complications post-transplant and  was in and out of the hospital every few days. These stays sometimes  lasted a day or two and other times a few months. In the end, I was in  and out of the hospital for about two years, first for my liver and the  issues that arose after my transplant, then later for issues subsequent  to both my hospital stays and my resultant condition. My lungs and  kidneys took a hit in part from all the medications I had been given. It  took a while to regain my health and stay out of the hospital for more  than a month or two.

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After I  was able to stay out of the hospital for a bit, I started thinking about  how I would be able to continue my studies. It became clear that I  needed a way to continue studying without having to leave every time I  was admitted to the hospital. For this reason, I decided to continue  studying in an online learning format. However, the community college I  was in before my transplant required in-person testing. With my health  being very volatile and requiring often lengthy hospital visits every  few months, I needed to find a way to continue school remotely. That’s  when I found Penn State World Campus, which allowed me to work toward my  degree through a fully online experience. I could do my work and take  my tests online even when I was in the hospital.

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For a  while, I did OK and did not have many hospital admissions. However, I  was eventually admitted and had to do my schoolwork while in the  hospital. At first, it was difficult to find the time to do my work  without being disturbed by the parade of doctors and other healthcare  providers coming in and out my room. So, I started looking at the times  during which they visited and managed to find the time to study. At  first it was a bit difficult, but I got used to my schedule and soon I  was able to do everything without much trouble. The lack of energy we  all feel at times is even more likely to happen with chronic illness. It  is important to push through without overdoing it. When I was sick, I  often rested while doing my schoolwork.

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Because I  was able to step back and examine my responsibilities—health, studies,  and daily life—I was able to make a schedule that worked for me. My  enhanced organizational abilities have helped me to be very close now to  successfully completing my studies. In the end, you could say that my  health complications have had a positive impact on my studies because I  learned to develop tools that will help me in the future. Indeed, I have  kept up good grades no matter what difficult situations have come my  way. In the end, my experience with health complications and school has  helped. I have learned how to be better organized and adjust to  difficult schedules that change often. It has been overwhelming at  times, but I was also provided with tools that can help me in my future.  I have learned that it’s possible to continue working on my goals  without sacrificing my health. Since being hospitalized, I have  experienced different situations that have affected my studies, yet I  have managed to maintain good grades and have been able to learn from  the experience.

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I think  that it is important for students like me to know that while your CF can  have an impact on your student life, there are ways to work around it  and meet your needs. Each person’s situation is different and will  present different challenges. However, if you put your mind to it, you  will be able to find a way to overcome any difficulty. Many of us may  feel overwhelmed by our treatment needs, medications, and feeling sick.  This may lead to us forget things in any of these areas, such as taking  medicine. Having a good outlook about your needs is a good place to  start so as not to neglect anything.

Students  with CF have lots of pressures and responsibilities; however, we also  have experience with facing difficult situations. It is a good idea to  channel this experience in your schoolwork and daily lives.

Daniel  Gonzalez is 24 years old and has CF. He lives in Cedar Park, TX, and is  currently working toward his Bachelor’s in Business Administration  through Penn State World Campus. He is a previous winner of the Higher  Education Scholarship (f/k/a The Lauren Melissa Kelly Scholarship)  offered through CF Roundtable. Daniel  is a volunteer for CF Roundtable. He enjoys spending time with friends,  playing video games, and relaxing. His contact information is on page  2.

“What  do you want to be when you grow up?” With twinkling eyes, little Ashley  would answer, “I want to be on Broadway.” Looking back, I get a chuckle  at how big I could dream. Many things have changed over the years, and I  have grown up a bit since that time, but the one thing that hasn’t  changed is how big I dream. There have and will continue to be different  obstacles to face as I continue to work toward my dream.

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When I  started my first semester of college in 2016, I was told by a doctor  that 85% of cystic fibrosis college students will decline in health,  drop out of college, or even take more than four years to graduate. I  remember this doctor distinctly asking me, “Will you be the 85% that  fails or the 15% that succeeds?” I looked him in the eye and said, “15%,  of course.” I wanted to prove to that doctor and to others that I can  be successful despite living with cystic fibrosis. After finishing two  years of community college, I transferred to my dream university. I was  excited for all that the school would have to offer and was eager to  begin preparing for my career. Unfortunately, due to the air quality, my  lung function went downhill, and I was only able to stay at that  university for one year. I felt like I had let everyone down—I had  become that 85%. Since that time, I wish I could have given my younger  self this advice: “No, you did not meet that 15% standard of that past  doctor, but you have succeeded in your own 15%. Being a college student  with cystic fibrosis is a huge accomplishment. It does not matter if you  finish school in four years or seven. It is okay to go at your own  pace—15% is continuing to go for your dream through all the obstacles  you have had to face. You should be so proud of yourself!”

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I am  currently finishing my general education at Monterey Peninsula College  and will be transferring to California State University of Monterey Bay  in spring 2021. I will be studying to obtain my Bachelor’s Degree in  Cinematic Arts. I would like to have the opportunity to work for film  companies that inspire and make a difference in the community. I would  also like to create a company in the future assisting businesses with  the resources to grow through marketing and media. I am incredibly  thankful to have received the Higher Education Scholarship (f/k/a The  Lauren Melissa Kelly Scholarship), offered through CF Roundtable, as it  will assist in allowing me to continue my education as a cinematic arts  major.

Ashley  Wilson is 23 years old and has CF, CF-related diabetes, and CF liver  disease. She lives in central coast California. Ashley is currently  pursuing a Bachelor’s Degree in Cinematic Arts and Technology and hopes  to share others’ stories through documentaries. Outside of school,  Ashley enjoys surfing, adventures with friends, and working as a YouTube  creator. She also is a producer and contributor on various podcasts.  You can find her personal YouTube channel at Ashley’s Roses and her  podcast at Blooming Roses.

The  CF Roundtable has presented two awards to members of the CF community  for over 20 years. Readers submit nominations and the CF Roundtable  board votes on who receives the award.

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The  Jacoby Angel Award is named in memory of Dr. Jack Jacoby who was both an  adult with CF and a CF physician at the St. Vincent’s CF Center in New  York City for more than 15 years. The Jacoby Angel Award recognizes a  person who has followed in the footsteps of Dr. Jacoby by dedicating  themselves to helping others.

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On  October 18, 2020, CF Roundtable presented both the Jacoby Angel Award  and the Founders Award during the CF Roundtable virtual Hope for the  Future event.

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Terry  Wright is the recipient of the 2020 Jacoby Angel Award. Terry Wright is  the President and founder of the National Organization of African  Americans with Cystic Fibrosis, an organization that works to help  engage, educate, and raise awareness of CF in both the African American  and the broader community. His efforts have already directly increased  the number of African American adults who are now being diagnosed with  CF in the U.S.

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Terry  was misdiagnosed for more than 54 years. He suffered numerous health  issues due to an incorrect assumption in the medical field that African  Americans do not have CF. After his diagnosis Terry could have focused  on himself but instead decided to work to make sure no one else would  have to suffer because of a misdiagnosis. Terry’s CF Roundtable blog  post this past spring chronicling his journey to his CF diagnosis has  helped educate the CF community on issues African Americans with CF face  in the United States.

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Terry  has spent his life helping others. He has been actively involved in his  community, volunteering for many local organizations and working to help  others even though he himself was facing serious health issues. He is a  dual-certified Master Gardner and Naturalist. He had a 30-year career  as a certified personal trainer. In January 2020, Terry was awarded a  CFF Impact Grant, becoming the first recipient from Arkansas.

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Previous  recipients of the Jacoby Angel Award include Robyn Petras, Susan  Burroughs, Michele Compton, Jerry Cahill, Pammie Post, Isabel Stenzel  Byrnes, and Dr. Paul Quinton.

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The  Founders Award recognizes a person, either with or without CF, who has  made an outstanding contribution to the adult CF community. The award  was named in honor of the group of adults with CF who founded the U.S.  Adult CF Association (“USACFA”) and created CF Roundtable. They worked  tirelessly to bring information to the adult CF community at a time when  there was no internet and no efforts to connect adults with CF in order  to provide information and support.

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Martha  Markovitz is the recipient of the 2020 Founders Award. Martha has been a  social worker at the University of Southern California CF Adult Care  Center for 40 years. Before there was a CF Adult Center, she served as a  social worker at the CF Pediatric Center. Martha is known as the “Queen  of Social Workers.” She has been a mentor to hundreds of social workers  who work in CF and has imparted her knowledge of how to help people  with CF. She has been a leader in advocating for adult care when there  were very few speaking out for the needs of adults. Martha has  personally led the way in advocating for assistance with insurance  issues, access to medical treatments, and implementing standards of care  for CF Centers to follow, which has made access to CF adult care better  for thousands of adults with CF.

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Martha  is known for meeting her patients where they are and figuring out ways  to help them even if it takes her hundreds of hours. She has a heart of  gold and retired at the end of October 2020. Martha has been a pioneer  in adult care, and the lives of all adult with CF are better off because  of her tireless commitment to making the lives of people with CF  better.

Past  recipients of the Founders Award have included Lisa McDonough, Dr. Jim  Yankaskas, Darlene Hello, Dr. Jerry Nick, James Passamano, Dr. Jennifer  Taylor-Cousar, and Cathy Chacon.

Hi! My  name is Daniel Gonzalez. I am 24 years old and was diagnosed with CF  when I was two years old, before I can even remember. I was born in  Saltillo, Coahuila, in Mexico and moved to Austin, Texas, when I was  about eight years old to receive better care for my CF. I currently have  my Associate’s Degree in Business Administration from Penn State and am  currently working toward my bachelor’s degree.

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Over the  past few years, I have learned more about the difficulties that come  with CF and how varied these can be. It has given me a new perspective  into how CF is different for every single person. Hearing about how each  person deals with their own challenges has made me realize that we all  share some similar experiences despite our differences. This means that  we can learn from each other’s experiences.

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The  realization that we can learn from one another led me to look for new  ways to learn about others’ experiences and the different organizations  that help those with CF. This became my reason for choosing to work with  USACFA for a school project. As I discovered more about the  organization and its purpose, I realized that I wanted to get more  involved. I applied for and was awarded the LMK scholarship in the fall  of 2020. After volunteering with treasurer duties, I decided to  officially join the board.

I enjoy  spending time with my friends. We usually pass the time by playing  billiards (although I am not very good) and video games. I like to play  all types of video games that allow me to experience different  adventures and stories. I also enjoy watching a wide variety of TV shows  and listening to music.

Terry G.  Wright is the president and cofounder of the National Organization of  African Americans with Cystic Fibrosis (“NOAACF”), a 501(c)(3)  organization with a mission to connect individuals with CF, help build  diverse communities, and, through its national platform, raise CF  awareness within the African American community and beyond.

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At the  age of 54, Terry was unexpectedly diagnosed with cystic fibrosis. He has  also experienced extensive bacterial infections and recurrent fungal  infections including sinusitis, bronchitis, pneumonia, aspergillus, and  Burkholderia multivorans. Terry has also endured chronic pancreatitis as  a side effect of his CF diagnosis.

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Terry is  the recipient of a 2020 Impact Grant from the Cystic Fibrosis  Foundation as well as being the first person from Arkansas to receive  this honor. He is also a recipient of the 2020 Jacoby Angel Award, the  highest award presented by the U.S. Adult Cystic Fibrosis Foundation  (“USACFA”), publishers of CF Roundtable.

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Terry  likes to plant seeds of life, love, and hope to help bring change for  the better for cystic fibrosis patients, their families, and caretakers.  He is an Arkansas dual-certified Master Gardener and Master Naturalist.  He was elected the 2016 Pulaski County Master Gardner of the Year award  winner and was a finalist for the 2017 Arkansas Master Gardener of the  Year. He completed the standard curriculum in Permaculture Design in  2018. He has also been honored to formerly serve on the North Little  Rock Green Committee and Commission on Environmental Efficiency. He has  an impressive 38-plus-year career as a certified personal fitness  trainer.

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Today,  he wholeheartedly utilizes his deep-rooted passion for gardening,  nature, agriculture, horticulture, fitness, nutrition, and health to  help individuals from all walks of life to achieve the best in health!  As a consequence of his own life-altering health challenges, coupled  with the numerous challenging health issues he has witnessed firsthand  in the lives of others, Terry is fully committed to utilizing his  expertise in gardening, agriculture, horticulture, nutrition, and  fitness to help others combat medical issues and achieve a better  quality of health and life. Terry lives in North Little Rock, Arkansas,  with his wife and Butterbean, Dr. Michele R. Wright.

PRESS RELEASES

In CF, Certain Factors Could Predict More Severe Hemoptysis, Study Finds

Factors such as diabetes, a specific type of fungal infection, and previous mild to moderate episodes of hemoptysis (coughing up blood) may help predict the risk of massive hemoptysis in people with cystic fibrosis (CF). Older age, advanced lung disease, Pseudomonas aeruginosa lung infections, CF-related diabetes, portal hypertension (high blood pressure in the liver’s portal vein), and liver cirrhosis have all been described as risk factors for hemoptysis in CF. But it is still unclear which factors could predict the development of massive hemoptysis in CF patients who have experienced mild or moderate episodes in the past. To investigate which factors could lead to massive hemoptysis, a group of researchers did a retrospective analysis of the clinical data of all adult CF patients who had a history of at least one mild to moderate hemoptysis episode. Massive hemoptysis was defined as a volume of coughed blood greater than 240 mL in 24 hours, or more than 100 mL over two or more days. Coughed blood leading to airway obstruction or changes in blood flow was also considered a symptom of massive hemoptysis.

Mild to moderate hemoptysis was defined as an episode resulting in a volume of coughed blood between 5 and 240 mL. The findings suggest that “BPA, metabolic disorders and recurrent mild-to-moderate haemoptysis may predict MH [massive hemoptysis] occurrence in CF patients with at least one mild-to-moderate haemoptsis episode in the past.

https://tinyurl.com/y38l2lbm

 

Study: Screening For Hearing Loss In CF Patients Feasible With Tablet-Based Tests

Tablet-based hearing tests can accurately screen for hearing loss in people with chronic lung diseases such as cystic fibrosis (CF) without specialist supervision. This finding suggests that tablets may be an inexpensive and practical addition to programs that monitor patients for signs of hearing loss. Life-threatening bacterial infections are common among patients with chronic lung diseases such as CF. When caused by certain microbes known as gram-negative bacteria, patients often are prescribed repeated treatments with aminoglycoside antimicrobial medicines, such as azithromycin, which can lead to hearing loss — a process referred to as ototoxicity (also known as “ear poisoning”). Hearing loss can be minimized through early detection of its symptoms, but developing effective outpatient programs to monitor for these presents challenges. Conducting a hearing test — audiometry — in a sound booth is costly, time-consuming, and requires a trained audiologist. To address this challenge, a team analyzed the accuracy of tablet and web-based hearing tests. These tests could be performed by non-specialists in outpatient settings. Web- and tablet-based results were compared to those from a hearing booth. Results from the tablet-based test correlated well with those from the hearing booth. The tablet test correctly detected most cases of hearing loss (high test sensitivity) and it proved to be especially accurate in detecting instances of no hearing loss (high test specificity). Although the web-based test proved better at accurately identifying patients who truly had hearing loss, it was less sensitive overall and more likely than the tablet-based test to identify a patient as not having hearing loss when, in actuality, they did. Both tests outperformed the Hearing Handicap Inventory for Adults questionnaire — a standard self-reported measure of hearing loss — across nearly all measures. Furthermore, tablet-based tests were associated with a good user experience, with participants reporting a high preference for them, followed by the booth-based and web-based formats. This study highlights the significant [hearing-related] impact of antimicrobial prescribing in adults with CF, and presents data highlighting the applicability of tablet-based audiometry as a practical screening tool with high accuracy that can be used within integrated ototoxicity monitoring programmes in chronic lung disease to identify ototoxicity at an early stage.

https://tinyurl.com/yyxnuten

 

Self-Management Intervention Supports Treatment Adherence In Adults With Cystic Fibrosis

A self-management intervention aided adults with cystic fibrosis in achieving greater adherence to inhaled medications, habit strength, lower perceived treatment burden and higher BMI over 12 months compared with usual care. However, results demonstrated no significant difference in exacerbations or FEV1 between the intervention and usual care. Since untreated adherence for nebulized therapy in adults is about 30% a Study Team developed a self-management intervention, described as underpinned by behavioral science therapy and with input from patients with cystic fibrosis to improve adherence to inhaled medications. The current study aimed to evaluate effectiveness of the intervention compared with usual care. The primary outcome of the study was exacerbation and secondary outcomes included adherence, habit strength, BMI and FEV1.

At 52 weeks, the level of adherence in the intervention group improved to about 52.9%.  However, researchers observed no significant difference in exacerbations between the intervention and the usual-care group or FEV1. BMI slightly increased, habit strength significantly increased, and Cystic Fibrosis Questionnaire Revised (CFQ-R) treatment burden significantly declined.

https://tinyurl.com/y58e9686

 

S. Aureus Found Mostly in Lung Mucus, Not Tissue, Pig Model Shows

Using pig lungs as a model, scientists discovered that Staphylococcus aureus preferentially colonizes the mucus circulating there rather than organ tissue itself. According to the researchers, these findings may open new ways of treating such lung infections, including treatment-resistant infections, enabling a lower amount of prescribed antibiotics against S. aureus than is now normally given. Antibiotics often fail to fully eliminate lung disease and its symptom, leading some to question whether antibiotic use is best possible treatment for S. aureus. The bacteria appear to localize in mucus plugs in the lumens of bronchioles. Bronchioles, one of the smallest lung airways, are connected to the alveolar ducts that house the alveoli. Researchers found that when S. aureus colonizes pig lungs it tends to form aggregates in mucus, rather than invading the organs’ tissue and forming abscesses. Unlike previous reports in mice, S. aureus in pig lungs were not seen to invade the animals’ lung tissue. Instead, these bacteria seemed to concentrate and form large aggregates in the artificial CF mucus that had been added to the organs. No abscesses were observed. According to the team, these findings may provide valuable insights into the effects S. aureus has on the lungs of CF patients, which could lead to new and more effective treatments for infections caused by these bacteria.

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Twincer May Be Better Alternative To Nebulizers For CF Colistin Treatment

Twincer, a dry powder inhaler (DPI), was shown to be a more appealing approach for the inhalation of colistin when compared with a nebulizer.Using a nebulizer, a small device that turns liquid medicine into a mist, is the most common method of administering inhaled antibiotics as a treatment for CF patients infected with the bacteria Pseudomonas aeruginosa. DPIs are a more patient-friendly option because they have a much shorter administration time, are more stable, do not require an external power source or refrigeration, and are three to six times more efficient. Notably, it takes about 1–2 minutes per dose to administer colistin with Twincer, compared with 10-20 minutes with a nebulizer. Twincer is a disposable inhaler for a single use that allows the delivery of relatively high doses of antibiotics. Researchers did a real-life evaluation study, with a small number of patients, which showed that colistin dry powder inhalation using the Twincer is a patient friendly alternative to nebulization, with no significant differences found in the clinical outcome regarding lung function decline and exacerbation rates. The researchers acknowledged several limitations to their study, especially its small sample size. Thus, further studies are needed to confirm these findings.

https://tinyurl.com/yy5aascm

 

Intravenous Vs Oral Antibiotics For Eradication Of Pseudomonas Aeruginosa In Cystic Fibrosis (TORPEDO-CF): A Randomised Controlled Trial

Researchers undertook this multicentre, parallel group, open-label, randomised controlled trial to compare intravenous ceftazidime and tobramycin vs oral ciprofloxacin in terms of effectiveness and safety in the eradication of Pseudomonas aeruginosa in patients with cystic fibrosis. Findings showed that intravenous antibiotics, in comparison with oral therapy, failed to provide sustained eradication of P. aeruginosa in a greater proportion of patients with cystic fibrosis and was more costly. The intravenous group had fewer hospitalisations during follow-up compared with the oral group, but this conferred no benefit over oral treatment since intravenous eradication often needs hospitalisation. In the light of these findings, the use of intravenous antibiotics to eradicate P. aeruginosa in cystic fibrosis was not supported.

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Delafloxacin—A Novel Fluoroquinolone For The Treatment Of Ciprofloxacin-Resistant Pseudomonas Aeruginosa In Patients With Cystic Fibrosis

This investigation was undertaken to test in vitro susceptibility of delafloxacin against a population of P. aeruginosa isolated from adult cystic fibrosis (CF) patients. In addition, delafloxacin and ciprofloxacin in vitro susceptibilities against CF P. aeruginosa were compared. Experts also evaluated where delafloxacin may add advantage in treating CF P. aeruginosa. Findings revealed greater activity of delafloxacin vs ciprofloxacin, given similar breakpoints of these fluoroquinolones. With sensitive isolates, equal effectiveness was displayed by both delafloxacin and ciprofloxacin, however, the value of delafloxacin was observed with more resistant isolates to ciprofloxacin. While ciprofloxacin was suggested to be employed as the first line fluoroquinolone for treating CF P. aeruginosa, experts also concluded that delafloxacin has potential in treating ciprofloxacin-resistant P. aeruginosa.

https://tinyurl.com/yytnoehc

 

Inhaled Tobramycin Effectively Treats Initial P. Aeruginosa Infections In CF

Inhaled tobramycin alone works as well at eradicating an initial infection by Pseudomonas aeruginosa bacteria in people with cystic fibrosis (CF), and its use in combination with an oral fluoroquinolone, such as ciprofloxacin or levofloxacin, appears to be redundant. The study results also suggest that inhaled tobramycin is as effective in a real-life setting as in clinical studies, validating current trial-based treatment guidelines.

https://tinyurl.com/y4evgysq

 

Bronchitol Inhalation Powder Approved In US For Adult CF Patients

The U.S. Food and Drug Administration (FDA) has approved the dry inhalation powder Bronchitol (mannitol) as a maintenance treatment to be used along with other therapies for improving lung function in adults with cystic fibrosis (CF). The therapy is expected to be launched in the U.S. market in March 2021. Bronchitol’s active ingredient, mannitol, is a sugar alcohol that is able to attract water. When directed to the lungs, using a small dry powder inhaler device, mannitol draws water into the airways and moisturizes the mucus, making it easier for patients to expel the mucus. By increasing mucus clearance, Bronchitol can help improve lung function and quality of life for people with CF. It’s the only inhaled dry powder indicated to be used along with other treatments to improve lung function in adults with CF. Bronchitol offers a portable and discreet option for CF management, with no routine cleaning or maintenance of the inhaler device required. Participants in three Phase 3 studies (NCT00630812, NCT00446680, NCT02134353) were randomly assigned to receive either Bronchitol or a placebo for 26 weeks, and entered an open-label treatment portion for an additional 26 weeks. The main goal of these trials was to determine improvements in lung function, measured by change from baseline in forced expiratory volume in one second (FEV1), over 26 weeks. Other measures of lung function and pulmonary exacerbations, or respiratory infections leading to worsening of lung function, were also examined over time. Overall, results showed a significant improvement in lung function in treated patients when compared with a placebo. The rates of pulmonary exacerbations also tended to be lower after Bronchitol treatment — though not significantly lower in all trials. While one of these trials examined Bronchitol in patients 6 and older, the safety and efficacy of this treatment in children and adolescents is still not clear. Thus, the current indication for Bronchitol is for clinically stable adults only. The most common adverse reactions, occurring in 3% or more of patients, include cough, hemoptysis, oropharyngeal pain, vomiting, bacteria sputum identified, pyrexia, and arthralgia. Before treatment initiation, however, patients are required to undergo a Bronchitol tolerance test. Here, healthcare providers give patients a first dose to evaluate if the treatment will cause bronchospasm (a sudden tightening in the walls of the airways) or a decrease in FEV1 and in oxygen blood levels. If any of these events occur, the treatment will not be prescribed.

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Translate Bio Resumes Enrollment And Dosing In Phase 1/2 Clinical Trial Of MRT5005 In Cystic Fibrosis

Translate Bio announced that enrollment and dosing in its Phase 1/2 clinical trial for MRT5005 in cystic fibrosis (CF) has resumed. In March 2020, the Company had announced a pause to enrollment and dosing in the clinical trial in response to the COVID-19 pandemic. MRT5005 is the first clinical-stage mRNA product candidate designed to address the underlying cause of CF by delivering mRNA encoding fully functional cystic fibrosis transmembrane conductance regulator (CFTR) protein to the lung epithelial cells through nebulization. MRT5005 is being developed to treat all patients with CF, regardless of the underlying genetic mutation, including those with limited or no CFTR protein. The FDA has granted MRT5005 Orphan Drug, Fast Track and Rare Pediatric Disease designation.

https://tinyurl.com/yxgk6a7t

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A. Fumigatus Lung Infections Linked To Age, Long-Term Antibiotic Use

Among fungal infections in CF, A. fumigatus is one of the most common. This fungus is known to cause allergic bronchopulmonary aspergillosis (ABPA) and acute lung infections. Co-infection of A. fumigatus with P. aeruginosa is also associated with more hospitalization and poorer lung function. Researchers analyzed data and found records of fungi in CF patients of all ages, with the infection rate of A. fumigatus rising until about age 49, after which it declined. Candida albicans and related species were the most common fungal species in patients’ lungs fumigatus and related species were the next most common species. They found that patients with chronic P. aeruginosa infection had significantly poorer lung function, as measured by forced expiratory volume in one second (FEV1), than those without. Patients without chronic P. aeruginosa infection but with at least one positive A. fumigatus infection also had significantly lower FEV1, implying that A. fumigatus may directly impair lung function. Those with at least two A. fumigatus-positive cultures also experienced significantly more pulmonary exacerbations requiring antibiotic treatment. These results show a decrease in A. fumigatus prevalence in CF patients older than 50 years. This might reflect that getting older with CF presupposes a milder CF disease or a better lung health status, which prevents A. fumigatus colonisation. Compared to patients without A. fumigatus-positive cultures, those with at least two had significantly lower body mass indices and significantly more ABPA, CF-related diabetes, and arthritis or other joint disorders.

https://tinyurl.com/y2ncttcd

 

FDA Clears AP-PA02 Phage Therapy Trial For CF P. Aeruginosa Infections

The U.S. Food and Drug Administration (FDA) cleared Armata Pharmaceuticals‘ investigational new drug (IND) application for a Phase 1b/2a clinical trial of AP-PA02 for the treatment of the Pseudomonas aeruginosa bacterial infections that are a hallmark of cystic fibrosis (CF). AP-PAo2, conversely, utilizes a cocktail of viruses called bacteriophages to attack the infecting bacteria. Because phages are specific to bacteria — they cannot infect the cells of the patients’ organs — they cause fewer side effects than antibiotics and can target even antibiotic-resistant microbes. AP-PA02 delivers phages specific to P. aeruginosa directly to the lungs via inhalation.

The new trial — called SWARM-P.a. — is a multi-center, double-blind, randomized, placebo-controlled, single ascending dose (SAD) and multiple ascending dose trial that will assess the safety and tolerability of AP-PA02 in CF patients with chronic pulmonary P. aeruginosa infections. Results from this study - SWARM-P.a. - to reflect the manner in which phage attack dangerous pathogens, will be the first clinical trial to evaluate a phage-based therapy as a potential treatment for Pseudomonas aeruginosa airway infections. This clinical trial will contribute to the evaluation of the potential of phage to combat multi-drug resistant infections, and potentially usher in a new era in the fight to develop alternatives to antibiotics. While the study will initially evaluate AP-PA02 in combination with standard antibiotics, the ultimate goal with this product candidate is to replace antibiotics as a front-line therapy.  AP-PA02 is a second-generation phage product candidate for P. aeruginosa that follows AP-PA01, the first phage product developed by Armata. AP-PA01 was shown to successfully treat a CF patient with resistant P. aeruginosa infection when used in combination with antibiotics. According to Armata, AP-PA02 offers significantly improved therapeutic features compared with AP-PA01.

https://tinyurl.com/y5s7tsf8

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https://tinyurl.com/y5s7tsf8

 

BiomX Testing Potential Therapy For CF Lung Infections

BiomX has unveiled a platform designed for more rapid and efficient development of phage therapy, which the company is using to test potential treatments, including one for Pseudomonas aeruginosa infections in people with cystic fibrosis (CF). BiomX focuses on bacteria-targeting therapies that use phages — viruses that can infect and kill bacteria cells. The company’s BOLT (BacteriOphage Lead to Treatment) platform is designed to allow for rapid development of phages that can target particular bacteria and move quickly into human studies. According to BiomX, the upcoming CF program aims to test the investigational therapy in 20 CF patients. Each individual will be treated for 10 days, and researchers will assess the treatment’s safety and efficacy in terms of bacterial counts, lung function, and quality of life.

https://tinyurl.com/yy462rb5

 

Patients With Cystic Fibrosis And Advanced Lung Disease Benefit From Lumacaftor/Ivacaftor Treatment

Several studies have assessed safety and efficacy outcomes for lumacaftor/ivacaftor therapy. This study reports on lumacaftor/ivacaftor’s impact on lung function, physical performance, and health-related quality of life (HRQOL) in a subpopulation of people with Cystic Fibrosis with advanced pulmonary disease who would not fulfill inclusion criteria for these other studies. This follow-up review examined lumacaftor/ivacaftor’s effect in a highly selected CF population. Inclusion criteria included low percent predicted forced expiratory volume in one second (ppFEV1), fast deteriorating ppFEV1, low body mass index (BMI), and difficult-to-treat infections. Primary endpoints included change in ppFEV1 slope, cardiopulmonary exercise testing (CPET), and all domains of the Cystic Fibrosis Questionnaire-Revised (CFQ-R). Secondary outcomes included change in ppFEV1, BMI Z-score, and sweat chloride concentration. The findings suggest that lumacaftor/ivacaftor reduces lung function decline, improves lung function, physical performance, and HRQOL to a greater extent in PWCF with severe lung disease than previously recognized.

https://tinyurl.com/y3538tsc

 

Alternative And Experimental Therapies Of Mycobacterium Abscessus Infections

Mycobacterium abscessus is a non-tuberculous mycobacterium notoriously known for causing severe, chronic infections. Treatment of these infections is challenging due to either intrinsic or acquired resistance of M. abscessus to multiple antibiotics. Despite prolonged poly-antimicrobial therapy, treatment of M. abscessus infections often fails, leading to progressive morbidity and eventual mortality. Clofazimine and rifabutin are known anti-mycobacterial antibiotics, repurposed for use against M. abscessus. Novel antimicrobials active against M. abscessus include delamanid, pretomanid and PIPD1 and the recently approved beta-lactamase inhibitors avibactam, relebactam and vaborbactam. Previously unused antimicrobial combinations, e.g. vancomycin–clarithromycin and dual beta-lactam therapy, have been shown to have synergistic effect against M. abscessus in experimental models, suggesting their possible use in multiple-drug regimens. Finally, engineered phage therapy has been reported to be clinically successful in a severe case of disseminated M. abscessus infection. While many of these experimental therapeutics have shown activity against M. abscessus in vitro, as well as in intracellular and/or animal models, most have little if any evidence of effect in human infections. Clinical studies of M. abscesssus treatments are needed to reliably determine the value of their incorporation in therapeutic regimens.

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Polyphor Receives Award Of Up To USD 3.3 Million From Cystic Fibrosis Foundation To Support Clinical Development Of Inhaled Antibiotic Murepavadin

Polyphor AG announced a funding agreement with the Cystic Fibrosis Foundation to advance clinical development of its novel class antibiotic, inhaled murepavadin, in cystic fibrosis (CF). Inhaled murepavadin is a highly potent and selective antibiotic against Pseudomonas aeruginosa, including multidrug resistant strains. The award will fund a Phase Ib/IIa clinical trial of inhaled murepavadin. The Phase Ib/IIa trial in adults with CF, assessing safety and tolerability (both overall and local) of ascending doses of inhaled murepavadin, is planned to be initiated in Q4 2021. Polyphor’s inhaled murepavadin is currently being developed as a precision antibiotic specifically targeting chronic Pseudomonas aeruginosa. An inhaled antibiotic could make it easier for someone with a Pseudomonas infection to take the drug from home. In addition, the drug, which targets the outer membrane of bacteria, specifically focuses on Pseudomonas, which might have certain advantages over broad-spectrum antibiotics. It is the first member of the Outer Membrane Protein Targeting Antibiotics (OMPTA), a novel class of antibiotics. If approved for commercial use, inhaled murepavadin would be the first new class of antibiotics for Gram-negative pathogens in the last 50 years. It would also be potentially the first agent to target specifically Pseudomonas aeruginosa bacteria.

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Matinas Biopharma Awarded Up To $3.75 Million From The Cystic Fibrosis Foundation To Support Development Of Oral Amikacin (MAT2501) For The Treatment Of NTM Infections In Cystic Fibrosis Patients

Matinas BioPharma Holdings, Inc. announced that it has been awarded up to $3.75 million from the Cystic Fibrosis Foundation (CFF). The award will support preclinical development of MAT2501, Matinas’ lipid nano-crystal (LNC) oral formulation of the broad-spectrum aminoglycoside amikacin, toward an indication to treat nontuberculous mycobacterial (NTM) lung disease. The CFF award will allow Matinas to rapidly advance the development of MAT2501 and will support preclinical in vitro and in vivo studies, along with several of the toxicology studies necessary to progress MAT2501 into Phase 2. MAT2501 is an oral version of the broad spectrum aminoglycoside antibiotic amikacin (encochleated amikacin) used to treat bacterial infections, including NTM and various drug-resistant, gram-negative bacteria. MAT2501 utilizes the Company’s proprietary LNC platform to achieve oral bioavailability, limit toxicity and enable targeted delivery to sites of infection. MAT2501 is designed to deliver high levels amikacin directly to the lungs, based on a lipid nano-crystal delivery technology that allows for a more targeted treatment, while improving the antibiotics’ safety and tolerability. Currently, amikacin can only be delivered by injection or through inhalation. IV and inhaled amikacin, however, are associated with major side effects including toxicity to the kidneys and ototoxicity or permanent hearing loss with long-term use. Matinas believes that MAT2501’s ability to orally deliver high levels of amikacin directly to the lung and without use-limiting toxicity, distinguishes it from all available therapies and could provide an important solution for patients and physicians.

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FDA Support Given To Advancing Potential Gene Therapy For CF

Spirovant Sciences‘ lead gene therapy candidate for select cystic fibrosis (CF) patients, SPIRO-2101, was given rare pediatric disease and orphan drug designations by the U.S. Food and Drug Administration (FDA) to support its development. SPIRO-2101 is an inhaled gene therapy, designed to replace the faulty cystic fibrosis transmembrane conductance regulator (CFTR) gene with a working copy. The therapy uses a harmless virus called adeno-associated virus, or AAV, to carry the genetic sequence of the healthy CFTR into patient lung cells, which can then produce a functional CFTR protein. SPIRO-2101 aims to treat CF patients with class 1 mutations or who cannot tolerate treatment with an existing CFTR modulator. The company estimates this covers about 10% of the total patient population. Class 1 mutations are those in which either no messenger RNA (mRNA) — the intermediate molecule between DNA and a protein — is made (class 1A), or in which the cell produces damaged mRNA that it cannot turn into a protein (class 1B). Current CFTR modulator therapies do not treat these mutations.

SPIRO-2101 has been engineered to target the cells of the outer layer of the airways, called epithelial cells, with high efficiency, allowing the therapy to be delivered where it is needed most.

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Potential Breakthrough For Cystic Fibrosis As Moscow Scientists Use Gene-Editing To Correct Part Of Mutation That Causes Disease

Scientists at Moscow’s Bochkov Medical Genetic Research Center have successfully corrected a mutation of the CFTR gene. Using genetic editing, they replaced the parts of the DNA that cause mutations. The researchers focused their efforts on the most common mutation, F508del. The scientists removed specific parts of the DNA of the mutated cells using the CRISPR/Cas9 gene-editing tool, replacing them with a healthy copy of the desired segment. The editing took place on a cell line known as CFTE29o-. The specialists managed to correct around three percent of the cell line, and about five percent of the stem cell culture. To cure a person of the disease, scientists believe around 10-15 percent needs to be edited. Despite the progress, the scientists note that the relatively low efficiency of genomic editing means that it cannot yet be used inside a patient’s body.

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Calithera Biosciences’ CB-280 Arginase Inhibitor Trial In Progress Poster Presented At The North American Cystic Fibrosis 2020 Virtual Conference

The randomized, double-blind, placebo-controlled, multiple ascending dose-escalation study (NCT04279769) is exploring CB-280 versus placebo in adults with cystic fibrosis and chronic infection with Pseudomonas aeruginosa who are stable on cystic fibrosis medications, including cystic fibrosis transmembrane conductance regulator (CFTR) modulators. The study is evaluating the safety, pharmacokinetics, pharmacodynamics and biological activity of four dose cohorts versus placebo. Enrollment in this study is ongoing and Calithera expects to share interim data in 2021. Preclinical study results suggest CB-280 significantly improved lung function and reduced Pseudomonas aeruginosa colony-forming units. Arginase inhibition with CB-280 resulted in improved central airway resistance in CFTR knockout mice, and decreased lung infection in wild type and DeltaF508-CFTR-expressing mice infected with Pseudomonas aeruginosa.

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Ionis’ Inhaled Antisense Medicine Demonstrates Potential As A Novel Treatment For Cystic Fibrosis

Ionis Pharmaceuticals, Inc. announced that data from a clinical trial of IONIS-ENAC-2.5Rx demonstrated a significant decrease in the expression of epithelial sodium channel (ENaC) in healthy volunteers. The study represents the first time an antisense medicine delivered directly to the lung via a nebulizer has shown a significant reduction in ENaC messenger RNA levels. IONIS-ENAC-2.5Rx is an investigational antisense medicine designed to reduce the expression of ENaC in the lung. ENaC is believed to be hyperactive in cystic fibrosis. The data demonstrate attractive tolerability and safety for IONIS-ENAC-2.5Rx with substantial target reduction and the convenience of once weekly administration.

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Envara Health Launches Medical Food To Address Fat Malabsorption In Cystic Fibrosis Patients

Envara Health has launched a clinically supported medical food called Encala. The new product is formulated to address fat malabsorption in patients with cystic fibrosis and other exocrine pancreatic diseases by providing both easily absorbable fat calories and improving the absorption of fat and nutrients in accompanying meals and beverages. A study found that children with cystic fibrosis and pancreatic insufficiency that had low dietary fat absorption saw significant improvement in fat absorption after three months of treatment with Encala, as well as increase plasma linoleic acid, a-linoleic acid, and total fatty acids. Compared to placebo, these subjects also saw improvements in height, weight, and body mass index. According to Envara, the structured lipid in Encala does not require PERT or digestion to be absorbed. Encala is neutral in taste, gluten-free, non-GMO, and plant-based. It is a source of highly absorbable long-chain fats, including fatty acids, and can be an alternative to medium-chain triglycerides derived from palm, coconut, or vegetable oils. Encala is recommended for use under medical supervision in adults and children over one year of age.

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Laura is 73 and has CF. She is a former director and President of USACFA. She and her husband, Lew, live in Northville, MI.