My  husband Ryan and I have been together for 11 years and married for  almost 10 of those years. Unlike a lot of CF couples, we did not meet  online as one might think. In 2011, Ryan was working full time at his  family’s physical therapy clinic as an office manager and I worked full  time as an apartment manager in Austin, Texas. I had been through quite a  rough year, physically and mentally. I was divorced at 28 years old and  my health had started to really decline, mostly because I struggled  with compliance. I lost a lot of weight and my lung function started to  dip. Right before Christmas in 2011, I was hospitalized for three weeks  because I was extremely sick—I was coughing up blood and my lung  functions tanked.

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I was a  part of a patient advocacy group in 2011 at our CF clinic’s hospital. It  consisted of adult CF patients and parents of children with CF, in  addition to people from our care team. At this time, the cross-infection  guidelines were not as strict. Our clinic formed this group and  recruited us. I became close friends with Tabitha (Tabby), a mother of a  child with CF, Noah. She told me that she had another good friend,  Ryan, whom she met at other CF events. He was going through a divorce  and needed a friend. He was dealing with some of the issues I had dealt  with the prior year and his health was starting to take a hit. She asked  me to give him a call. I knew of Ryan but did not know him personally  at the time—Austin was still a fairly small CF community back then. We  became friends in the fall of 2011 and mostly talked on the phone and on  Facebook as meeting in person was frowned upon by clinic.

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A mutual  friend of ours, Amberlyn, passed away in November, 2011, after Ryan and  I became friends. It hit us both hard. Amberlyn was extremely loved in  our community and generally. She started a non-profit called Amber’s  Angels. Volunteers would deliver gifts to kids in the hospital at  Christmas and other times. We cried with each other on the phone.  Debbie, Amberlyn’s mom and a good friend of ours, held a memorial  concert for her at a local honky-tonk. Country music artists who knew  and loved her sang in her memory. The event raised money for Amber’s  Angels. Tabby and Debbie were best friends and the four of us made plans  to go. I told Ryan I would see him there. I didn’t think too much of  it, really. Ryan walked through the door and we saw each other for the  first time. We immediately had chemistry. He asked me to dance, but he  asked other girls, too. We danced three or so feet apart and moved our  heads away from each other; however, we were holding hands, too, so it  is sort of comical now to think we were trying to stay safe. From that  night on, we kept making up excuses to see each other, staying three  feet apart. After a couple weeks, it was impossible not to admit that we  were in love. I was in love with someone I had never even kissed! It  was strange and refreshing. We talked about it at length and what we  were going to do. We were both the happiest we had ever been, especially  after everything we had gone through with our previous relationships.

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We  looked at each other’s clinic reports and sputum cultures and discussed  with what bacteria our lungs were colonized. We both mainly grew  Staphylococcus aureus and we both had only grown Pseudomonas a handful  of times each in our lives. We both told each other that if we had  anything that was detrimental, such as B. cepacia or MRSA, we would not  be able to continue with our growing relationship. We both felt that  life was too short and we decided to dive in! We married on September  21, 2013, on a beach in Port Aransas, Texas.

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Our  doctors and nurses were surprisingly very supportive. Ryan and I were  active in the CF community and my health improved a lot being with him  and becoming 100% compliant. His health did, too. We were both still  working and enjoying married life when I dropped the B word on him. Oh,  boy, did I want a baby badly! Ryan is five years younger than I and he  was not quite ready for a baby. In fact, I’m sure he thought I was a tad  crazy at this point. I have always just thought I could do it all. I’ve  always loved children and being a mom was my dream. Ryan finally came  around to it and we saw a fertility doctor. We knew we didn’t want to  pass on CF. We spoke with our CF team and I was cleared to have a baby.  After a little while, I realized my body could not handle a pregnancy  Instead, we found a surrogate. I went through IVF and had my eggs  retrieved in June of 2014. Thankfully, I was very fertile at the age of  31 and we got seven embryos. The first and only embryo transfer into our  surrogate took place in October of 2014! Ryder Boone was born on June  11, 2015.

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My  health had begun deteriorating between 2014-2019. Some would say  motherhood and its responsibilities took a toll on my health and others  would say it’s just part of getting older with CF. I would argue both of  these reasons contributed to the decline in my health. To be perfectly  honest, it started even before Ryder was born. I came down with the flu  in December of 2014. It was the first year that I realized the flu shot  did not always work. I was hospitalized and very afraid. Our surrogate  was 12 weeks into her pregnancy, and I was truly thankful I was not. My  health never completely recovered after that hospitalization.

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I  required more IV antibiotics and hospital trips. I was not too happy  about the whole situation, as I had planned to continue working after he  was born. Instead, my doctor and I came to the realization that I  should not go back to work so I medically retired. I had two months to  wallow and then I had a newborn baby to take care of. Ryan had just  started a new job as an IT tech and was very busy. I was worn out but  blissfully exhausted in my new role of both CF patient and mom.

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By the  time Ryder was two years old, I was in and out of the hospital routinely  and it was taking a toll on my health. I was at a point in my health  that if I didn’t do something I was afraid I would not live long. My  allergies and asthma were horrible in Austin and it just exacerbated the  issues in my lungs. I told Ryan that we should move to Colorado Springs  as I had googled best places to live with allergies and asthma and then  cross-referenced those cities with accredited CF care centers.

I had  heard many wonderful things about the National Jewish adult CF clinic in  Denver, Colorado, and I was ready for a change. So off we went! We  lived in a B&B for a while to see if it was going to work out.  During that time, I saw the CF clinic in Denver and my body was in worse  shape than I thought. They immediately admitted me to the hospital. It  was the week of Thanksgiving and our short-term lease was about to end.  After some consideration and talks with the doctors and our family, it  was decided we needed to stay in Colorado. I was stuck in the hospital  alone for Thanksgiving and the three weeks following while Ryan and  Ryder went back to Texas to get our house packed up and sold. I came  home for five days at the beginning of December, only to have to be  rushed back to Denver because I was so ill. I spent Christmas and my  birthday two days later in the hospital away from my family and everyone  I knew. It was one of the darker periods in my life. Thankfully, my mom  flew in to be with me. My Dad and the rest of our family helped Ryan  get moved. We reunited as a family on December 28, 2017, and moved into  another short-term rental in Manitou Springs. I was on IV antibiotics at  home and still pretty sick.

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2018 was  another rough year for me health wise. I was in the hospital six times  that year. In 2019 my health saw some stability, but my lungs were still  severely damaged. I was being hospitalized every two to three months.  The visits were not as critical as previous times—my CF team had found a  decent antibiotic cocktail that worked for me. The National Jewish CF  Care Team were my saving grace. In October 2019, Ryan and I went to see  our doctor for our quarterly CF clinic visit. I always pack a bag  because I never know when I will be admitted, and we live a couple hours  away from the hospital. The visit was uneventful for both of us, which,  for me, is rare. While we were at the appointment, Ryan was on Facebook  and saw that the newest genetic modulator, Trikafta, had been approved  by the FDA. It was not projected to come out until March of 2020. We  were all in shock and so happy. Ryan had been on the drug through a  clinical trial and had already seen wonderful results. He gained weight  and his health was very stable. He recovered from viruses and a bad  sinus surgery quicker than usual. Unfortunately, he did not have a huge  increase in lung function.

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By  November 2019, my lung function was down to 44%, and I couldn’t get it  over 50% unless I had just been on a round of IV antibiotics. I felt  unwell most of the time and I slept a lot. I used oxygen at night and  during light exercise. It was hard to play with Ryder and keep up with  simple house chores. My husband and family helped us out a lot during  this time. My doctor referred me to a transplant clinic.

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I felt  defeated at first. The Cystic Fibrosis Foundation changed the way they  do transplant evaluations and they wanted us to see transplant clinics  earlier, if possible, so that we could be on their radar and get  information. My doctor explained that since my health is so up and down  it would not be a bad idea. I had anxiety about this and expressed to  her that I was worried about what would happen to me if I was really  sick and we did not have a plan. I think she thought this visit would  help ease some of my worries.

Ryan and  I each have only one copy of the most common CF mutation, DF508. My  second copy is a rare mutation, E60X, which is a nonsense mutation and  rather severe. Because neither one of us has two copies of DF508, we  have never been eligible for any of the prior modulators. After finally  getting insurance approval and copay assistance, I was able to start  Trikafta on Friday, November 8, 2019. I was excited, but not expecting  too much.

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My  health and life changed on day five of Trikafta. I started coughing up  tons of mucus and then I started to breathe again. I honestly could not  remember the last time I could take a deep breath like that. It woke me  up in the middle of the night. I was in shock and could not go back to  sleep. I decided to do my PFTs with my home spirometry to check my lung  function. I blew a 56% FEV1, which was a 12% jump in four days! I  started crying. My PFTs had been hovering around 41-50% over the last  year, and rarely at 50%. My numbers had dropped to the lower 30s with  lung exacerbations. I was in disbelief but felt elated. I posted this on  my Facebook page: “It is such an exciting time in CF and I’m so happy  for my CF friends that are eligible! I cannot wait until children can  benefit from this and I pray that their lung function stays high or  stabilizes so they will never have to endure the things we have. I have  hope for my future.” I did recognize the grief I felt for my friends who  remained ineligible and hoped that CFF had plans for them as well.

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I  attended my transplant clinic appointment the day after this health  breakthrough. I was turned down for a transplant because everything  looked stable. My oxygen saturation was good and my lungs actually  sounded fairly clear. I was told to go home and continue the new miracle  drug I had begun. The physician said she hoped I would never need a  transplant; however, I would still be monitored to make sure.

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I just  turned 40 last December! I am living the dream. I have far exceeded the  age my parents were told that children with CF lived to. When I was  diagnosed at 10 years old, children were expected to only live until  they were twelve.

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There  were some scary times following the Trikafta boom, as we all endured the  COVID-19 pandemic in 2020. We were fortunate to have just received  Trikafta prior to this new virus. We were deathly afraid, but somehow  managed to stay COVID-19 free for two years. When we did get it,  thankfully it was very mild for us. We both got the flu and rhinovirus  and fared better than we would have prior to Trikafta.

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Our son  Ryder is now seven years old and in the second grade. He is in his  second year of Scouts.  Watching him grow up is painful and joyous at  the same time. It’s just going by too quickly! We enjoy hanging out with  him and talking about life. We moved into our little farmhouse on  acreage in April of 2020. We can see beautiful mountain ranges (Pikes  Peak and Sangre de Cristos) from our front porch. All of the years we  saved and saved have paid off. We are both on disability now, and it can  be hard at times, but we make it work and live rather frugally. We have  one horse, one labradoodle, Stormy, and a new poodle puppy named  Trooper. We had goats for a bit, and that was crazy but fun! Our “first  born” Moogie, our 11-year-old husky, passed away in September of 2022.  He is missed every day, and this year has been difficult, especially for  my husband.

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Ryan  stays busy with ranch chores, fixing our vehicles that forever seem to  have problems, and solving all of our IT issues. He is still very active  and hasn’t gained a whole lot of weight on Trikafta because of it. I,  on the other hand, have gained 30 pounds! It is uncomfortable to me and I  am working on body image issues. However, I am still incredibly  grateful. I would rather be a little fluffy but alive! I have become a  pretty decent cook and baker. I enjoy entertaining our friends and  family and filling their bellies.

I am  rarely hospitalized now; usually just once a year, and almost always  after a virus, not just a CF exacerbation. I decided to go back to  “school” last summer to obtain my paralegal certificate. The Claire’s  Place Work Proudly program sponsored me in an online program with Boston  University. It was a 14-week long program. I loved it and hope to get a  part-time remote job in the near future. I am working out the kinks  with how to keep my medical benefits and go back to work without risking  my health.

We are  sort of lost on what to do next due to this drastic change in our lives.  We may actually have to plan for living a long time now! It is not  something we are set up for. We still have to do breathing treatments,  and CF is still a big part of our lives.

However, we get much more time to live “normally” now than ever before.

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Becoming  CF grandparents is a reality that could very well happen now, even if  those grandbabies are fur babies. Ryder says he’s going to have lots of  dogs and no kids, so we shall see!

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This  article is for Noah and Tabby. Noah passed away the summer of 2018  waiting for a double lung and liver transplant. He was only 10 years  old. I will forever be thankful to you for inspiring your mother to get  involved, as I would not have my little boy or my husband without you. I  hope my faith will someday be as strong as yours.

Amanda  Boone is 40 years old and has CF. She lives in Colorado Springs, CO,  with her husband, Ryan, who also has CF. They have a son, Ryder, and two  pups, Stormy and Trooper.

If  you have questions about laws related to Social Security benefits,  Medicaid, Medicare, health insurance, employment, and education rights,  you can contact the CF Legal Information Hotline at CFLegal@sufianpassamano.com or 1-800-622-0385 to set up a time to speak to an attorney. All calls  are confidential and there is no cost to the caller. The CF Legal  Information Hotline (CFLIH) is funded by the CF Foundation, but CFLIH  employees are not employed by the CF Foundation. The CFLIH is now in its  25th year.

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The  information provided in this article is only meant to be general  information. A person should verify the type of benefit they receive and  review the Social Security rules to make sure that Social Security work  income and asset criteria are followed. Nothing in this article is  meant to be a guarantee that a person will be eligible for SSDI, SSI, or  any other government program.

In the  past three months, CF Roundtable readers have sent many questions  related to the ability to work part time and still receive Social  Security Disability benefits. There have also been questions about  Medicare and requirements for low-income Medicare plans that provide  coverage at a reduced premium with assistance for some Medicare copays.

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Question:  Does a person who receives Social Security Disability (SSDI) benefits  have to work less than 20 hours a week and make less than a certain  amount per month in work earnings, or do you only have to meet one of  those requirements?

Answer:  In order to maintain eligibility for SSDI benefits, a person cannot work  more than 20 hours a week and cannot make more than a certain amount  per month from work activity. In 2023, the maximum amount a person on  SSDI can make per month is $1,470 before taxes are taken out of the work  check.

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There is  a possibility that Social Security will provide up to nine trial work  months to go over the allowable monthly income amount. However, Social  Security uses a lower number to trigger use of a trial work month. Once  the trial work months are used and a person goes over the maximum  monthly amount of $1,470, the person can lose SSDI benefits. This is a  very confusing rule and a person should make sure they understand when a  trial work month is used.

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If a  person is self-employed, the work earnings amount per month is lower and  there are a variety of ways Social Security determines work income if a  person is self-employed. In addition, a person’s medical condition must  still meet or equal the medical criteria listed in the Social Security  Listing.

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Question: Does the value of a car owned by a person who receives SSDI benefits count in terms of eligibility for SSDI benefits?

Answer:  SSDI eligibility is not affected by assets, so the value of a car is not  counted when determining eligibility for SSDI. It is important to know  which type of Social Security benefit you receive as the asset rules are  different for SSDI and SSI.

Question:  Does the value of a car owned by a person who receives Supplemental  Security Income (SSI) benefits count in terms of eligibility for SSI  benefits?

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Answer:  If a person is receiving SSI benefits, the person must have low assets  and low income. This is different than the eligibility rules for SSDI. A  single person who receives SSI benefits has a maximum asset limit at  all times of $2,000.

The  value of one car is not counted toward the SSI asset limit. The car is  owned by a person if the title of the car is in their name. If the title  of the car is in a parent’s name, then the person on SSI is not  considered the owner. However, the value of a second car is counted  toward the SSI maximum asset limit for those receiving SSI. For example,  if a person who receives SSI owns a car that is worth $5,000 and owns a  second car that is worth $2,000, then the $5,000 car will not count  toward the $2,000 SSI asset limit. The second car will be counted and  its value of $2,000 will mean the person cannot have any other assets  except the person can own one house.

Question: Does a spouse’s income and assets affect eligibility for SSI?

Answer:  Yes. If a person, who only receives SSI benefits then gets married, then  the spouse’s income and assets will affect eligibility for SSI  benefits. A family of two can only have $3,000 total in assets at any  given time. In addition, the spouse will have a limit on how much income  the spouse can make from work activity.

Understanding  the effect of marriage on eligibility for SSI benefits is important. In  11 states in the U.S. there is no access to Medicaid for adults unless  the adult receives SSI benefits.

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If a  person lives in a state that has not expanded Medicaid to low-income  adults, then it is very important to make sure marriage will not result  in the loss of Medicaid if the person with CF gets married. Sometimes  marriage to a spouse who has access to health insurance means that a  loss of SSI or Medicaid will not be a problem. But if the spouse does  not have access to health insurance that will provide good coverage, the  person with CF should assess how they will access coverage for care and  treatment.

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Question: If I have a low-income Medicare plan do I need to keep income and assets below a certain amount?

Answer:  If a person has Medicare and is on a low-income Medicare plan, then  assets, work income, benefit amount and spouse’s assets and work income  may be considered when determining eligibility for the plan. Different  Medicaid low-income plans have different rules that can be found at www.Medicare.gov.

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Other  government programs may count assets differently than Social Security  when determining eligibility such as food assistance or housing  assistance programs. A person should check the asset criteria for any  federal or state program that is available for people who have low  income and low assets.

Beth  Sufian is 57 years old and has CF. She is an attorney who focuses her  law practice on disability law and is the Vice President 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.

I  have struggled with body loathing all my life. This morning, in a flash  of awareness, it occurred to me that I love my body. Really? Am I  lying? Well, in this journey toward spiritual wholeness, I do. I love my  body. It is my truth. I commit to the conviction that I must love my  body. How do we love a body that doesn’t always work?

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Much of  my body loathing has had to do with CF-induced appearance changes,  discomfort and pain, or not being able to do what I wanted to do. My  body is so darn high maintenance; I’d rather be doing most anything else  than taking care of my body’s endless demands.

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I am 19  years post-lung transplant and my body has responded the right way to  the immunosuppressive drugs all these years, which has spared me from  rejection. In suppressing my immune system, I am now living with  metastatic lung cancer. In exchange for being a long-term survivor, my  lungs are slowly diminishing, both by cancer and rejection caused by  reducing immunosuppression. In exchange for reaching the age of 50, my  joints and muscles are tight and achy. I have vision, gut, sinus, bone  issues, brain fog, and, my nemesis, diabetes. The list goes on. It is  just my body; it is a lot. But everyone’s got something, right?

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I know  many people with cystic fibrosis naturally feel frustrated by the work  it takes to care for the demands of a CF body. I try to take care of my  body with all I’ve got, but, to be honest, at this stage of my life I  triage my needs all the time—I exchange time and energy for quality of  life. My CF body fits into all the other parts of my life that make  demands to live a rich, fulfilled, engaging life.

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And yet,  there is so much in this body still deserving of love. This body is my  vessel that allows my spirit and soul to be housed so I can experience  the world, be of service to others, and be in relationship with so many  other loving bodies out there. It may only allow me to perform at around  60% capacity, not 100%, to fulfill my spiritual and mental desires, but  it’s still functional. This body is still working well enough. I am  still able to move, speak, think, plan, laugh, and do things I enjoy  with less intensity and speed. Wow. There is vitality.

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I  recently applied for disability and submitted pages upon pages of my  medical records. What a humbling experience. On paper, I look like a  royal medical trainwreck. The health history and medication list are  literally multiple pages long. Someone sitting at a social security  office must’ve been very compassionate—I was approved in two weeks’  time. I was shocked!

And then  I felt an overwhelming sense of gratitude. I was rewarded with  disability benefits because this body was ill enough to qualify. I am  grateful that I am mostly functioning relatively well; I am living more  than dying. And, before disability, I was managing a stressful job and a  time-consuming medical regimen. I was exercising hard. This body  climbed mountains, took me around the world, and helped me race in the  pool at the Transplant Games. What a glorious miracle this was! But I  cannot love my body only when it is well. That’s conditional love. Our  spiritual charge from God is to engage in loving kindness with all  beings, which includes unconditional love. It is hard to do! But our  bodies deserve unconditional love, too. So even with chemotherapy, I  love my body because it can still function more than I thought it could.  It is taking a toll and my weakness and fatigue are getting worse, but  I’m still here. My fierce and life-loving willpower and discipline,  cultivated by a lifetime with CF, help to fuel this body still, with  tenderness and patience. I’m loving my body for how well it is  tolerating the treatment, how it keeps going despite the assault of  poison on top of everything else. I love this body for responding to the  medications that I’m on and for switching to whole fat dairy and  actually gaining weight! It is working. I’m blessed to have started this  cancer journey with a reservoir of stamina and strength.

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I look  around at the waiting room of my hospital clinics and am amazed to see  so many people struggling with their bodies: some are old; some are  morbidly obese; some are barely walking; some are bald, cancer patients;  some are lung transplant patients on oxygen; and some barely stand up  from their wheelchairs. I know everyone has a relationship with their  own bodies. If we despise and curse our bodies when they aren’t working  well, we are spending a lot of emotional energy that could otherwise be  used to love what we can, whom we can, for as long as we can.

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So how  do we give love to our bodies? We listen to it. When we are tired, we  rest; hungry, we eat; thirsty, we drink. We put our hand on our hearts  and can talk to it, encourage it to recover. We can touch and caress the  parts that hurt or, even better, get a massage! We can stretch and do  yoga and breathe out the tension. We can watch the IV drip into our  veins and have faith and trust the body will respond. We can nourish it  with wholesome foods and water. We can do as much movement as is  comfortable for the body we are housed in, so we circulate our blood and  all its healing chemicals. We move slower. We can laugh at it and with  it, lovingly. We can accept this is the body we’ve got. And we abstain  from comparing this body to the perceived healthy, ideal body out there.  All perfect bodies will, at some point, change and fail.

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I am so  inspired by my dear friend who recently died of complications from CF  and her lung transplants. This remarkable author and patient advocate,  Tiffany Christensen, wrote about the importance of loving her body by  being patient and giving it space and time for healing. She wrote, “One  week and a day after the placement of my PD catheter, an amazing and  wondrous thing happened: I felt better. I was not in so much pain and I  felt like ‘myself.’ My body had done what it always has done, healed  from the medical wounds, but not on the timeline I had demanded. The  body is funny like that. It doesn’t listen to reason, bargaining, or  commands. It heals in its own time and in its own way. I know this after  49 years of chronic illness and, yet, once again, I betrayed my body  with my need to meet an intellectual expectation of healing rather than  the magical, peaceful, beautiful process that it is.” Amen, Tiffany. Her  wisdom outlives her body.

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This  body deserves love. It is a living creature. I believe it houses a  spirit of its own, that is in connection with my larger spirit. Both  spirits have to engage in collaborative magic. Much like plants and  animals are happier when we talk to them with loving words, so, too, are  our bodies. There is much spiritual healing happening when we love our  broken bodies deeply and fully.

I love  my body. I hope you, too, can say that to yours. Whether your body is  stronger with modulator drugs and you can push harder or do more with  your body or whether you continue to endure the spectrum of symptoms of  CF, may you find your own ways to give it love and talk to it lovingly.  Body, mind, and spirit are beautifully woven together and self-care to  one of that triad is self-care to all three.

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To end, I share with you an inspirational quote that has uplifted me for many years.

Birthright

“Despite  illness of body or mind, in spite of blinding despair or habitual  belief, who you are is whole.  Let nothing keep you separate from the  truth. The soul, illumined from within, longs to be known for what it  is. Undying, untouched by fire or the storms of life, there is a place  inside where stillness and abiding peace reside. You can ride the breath  to go there. Despite doubt or hopeless turns of mind, you are not  broken. Spirit surrounds, embraces, fills you from the inside out.  Release everything that isn’t your true nature. What’s left, the  fullness, light and shadow, claim all that as your birthright.”

—Danna Faulds

Isa  Stenzel Byrnes is 50 years old and has CF. She lives in Redwood City,  California, with her husband, Andrew. She is 19 years post-lung  transplant.

When  we announced our Focus Topic on CF and the Path to Parenting, I spent  some time thinking about what I would write as my column for this issue.  I don’t have children and have known for my entire life that I do not  want to become a parent by any means. But after giving it some thought, I  realized that my life as a childfree person with CF still affords  plenty of insight about family planning and parenthood in the context of  our shared health experiences. After all, my own family’s unique path  to parenting may well explain why I have CF.

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Let’s  rewind a bit. I was born in 1983 as the result of a science experiment  at the University of Mississippi Medical Center. Too perfect, right—how  many scientific faculty members at medical schools can claim that as our  origin story? I was made in a lab before ever growing up in one. And  yes, show and tell at school was always interesting. But before I was  busily spending my free time dissecting brains and dipping slides to  develop images of how cells were growing and moving around, I was a  little clump of rapidly growing tissue myself. My parents were able to  make me because they had some help from an anonymous sperm donor who  contributed samples to the research study they joined.

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My  parents, who have no history of CF in either of their families, were  both fertile but could not conceive a baby together. This phenomenon was  becoming more widely recognized by scientists in the early 1980s.  Reproductive health scholars took interest in exploring the relatively  new technology of artificial insemination to see if it would reliably  benefit histoincompatible couples like my parents. One of these studies  just happened to be located at the same medical school where they held  faculty positions. Three trials later, they got me—and an eventual crash  course in a disease they’d only known about previously from their work.

What we  still don’t know—and may never find out—is whether I would have had CF  regardless of whether my parents had done the artificial insemination  study or pursued in vitro fertilization as an alternate option. CF is  frequently hereditary, but not always. Every genetic mutation, whether  CF-related or not, started out as a spontaneous one. Sometimes these  mutations happen during a parent’s life, making it possible to pass the  altered gene to offspring. Other times they happen while a child is  developing in the womb.

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We don’t  know whether my CFTR mutations came from my mom and biological father.  But because I have double expression of the same extremely rare  mutation, it’s likely that neither of them passed it to me and that  something that happened during my mother’s pregnancy would explain how  my own CFTR genes wound up coded differently. Before I was born, my  parents didn’t know I had CFTR gene mutations or that I would have CF  clinically. I joined the world in December of 1983—nearly six years  before the CFTR gene was even discovered, let alone when any genetic  testing to detect potential CF in developing babies became available.

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Being  born via donor conception in the early 1980s posed some drawbacks. Laws  about mandatory information sharing for families of children conceived  via anonymous sperm donation missed my birth cohort by two years. People  born after 1985 get access automatically to details about their  background and origins that I spent decades seeking out. For me—and for  my half-brother who does not have CF—there are always more questions  than answers.

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At the  same time, I feel glad that my parents didn’t know before I was born  that I would have CF. If they had known, I might never have gotten to  live at all. We’ve spoken frankly about the fact that my mother and  father would have considered aborting the pregnancy had they known I  would have this disease. I’m not sure what choice they would ultimately  have made and neither are they. And I don’t think my own opinions about  the inherently eugenic elements of choosing to terminate a pregnancy  because of chronic disease should supersede the essential human right of  people to self-determine about gestation and childbirth. I may not  agree with the specifics of someone’s reasoning when choosing abortion,  but I will defend their right to get one no matter what.

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And  although it would be easy for me as a childfree person to say “if people  don’t want to deal with the possibility of a sick child, they should  just adopt” as if the word “just” belonged in that sentence, I cannot do  so. I’ve known for most of my life that there is no “just” when it  comes to expanding a family. Indeed, my parents tried for years to adopt  before finally giving up because even getting an interview would have  taken another 10 years or more. I can’t pretend to understand firsthand  the heartbreak of that experience—or of learning once they had me that I  would live with a life-threatening disease that forced all of us to  reckon constantly with my mortality.

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So what  have I learned about pathways to parenting from being a childfree person  with CF whose life began with donor conception? Lots—and it all comes  back to the vital importance of supporting people in their individual  family planning journeys. Many people with CF now find themselves having  options they’d never anticipated for expanding their families.

Apart  from just living longer thanks to better overall care, plenty of people  in the adult community are thriving on CFTR protein modulators. Drugs  like Trikafta can’t replace a missing vas deferens—the tube that allows  sperm to get into the seminal fluid before someone ejaculates—in people  born without one. Folks with testicles who have two copies of the DF508  mutation often don’t have a vas deferens. But in people with vaginas,  often taking a modulator will thin out the cervical mucus—the wet  substance that lubricates the canal—enough for sperm to pass through  into the uterus and fertilize an egg. Producing children through sexual  reproduction is now much easier for many folks with CF thanks to  modulators!

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Options  for assisted reproduction have also expanded since I was born. I’ve seen  many folks with CF use either artificial insemination or in vitro  fertilization to have children. Accessing these services has become  easier and more affordable than in times past, although we still have  miles to go in achieving true justice on that front. IVF also offers the  option of intentionally selecting eggs that do not carry any CFTR gene  mutations, and testing in advance for other genetic diseases. Whether  parents use this information to plan intentionally for how they will  meet their kids’ medical needs or select the specific sample they want  to use to make an embryo based on this information, it’s a huge  advantage.

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Many  folks with CF are also sharing joyfully these days about their adoption  and fostering journeys. With visibility increasing all the time for our  adult community—and better information circulating about what people  with CF can do with our lives as we continue to live longer and thrive  more—it’s becoming easier for prospective parents who want to open their  hearts to children who are already in the world to welcome kids into  their lives. I have to say, of all the stories I’ve seen where people  wanted to be parents and then actually got to do so, these make me smile  the biggest. I still think the kid who didn’t get to grow up with my  parents because they had me instead lost out. Then again, I’m terribly  biased.

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Finally,  it’s important as we think about all the ways people with CF can now  have kids that we remember to support members of our community who don’t  want that life. Some of us realize we’re childfree as adults; some of  us have known since we were still young children ourselves. Either way,  it’s vital for our loved ones to affirm our desire not to raise  children. As my own parents always told me, it’s better to regret not  having children than to regret having them. I’ve never regretted being  childfree for a moment—I always knew becoming a parent wasn’t my journey  in life.

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Likewise,  I always appreciated the insight I gained from my lack of interest in  having children. People who would pressure me to change my mind usually  changed their tune quickly when they learned I had a fatal genetic  disease. Ableism remains as deeply ingrained in our society as ever. Yet  I always had the full support of my parents in following the path that  would best for me—whether it led me toward parenting or brought me  nowhere near it. And being a little older and wiser these days, I deeply  appreciate what being childfree allows me to give with the time and  energy I would otherwise spend on my own children. Supporting my fellow  adult CF community members on their parenting journeys remains a joyful  part of that always.

Dr. Alexandra “Xan” Nowakowski is 39 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 Associate 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.

Hi,  there! My name is Penny, and I am a 16-year-old miniature schnauzer. I  am named after my parents’ home states (PA [Penn] and NY). Pretty  creative, I guess. There is another miniature schnauzer in my  neighborhood, also named Penny, because she came from PA. People get us  confused all the time, which makes no sense to me since I am obviously  cooler and better looking.

I came  from my grandparent’s dog, Nellie. Mama picked me out after sitting on  our puppy blanket for a LONG time. I don’t know why it took so long; I  sat there staring at her the entire time while she played with my  sister, who proceeded to poop right next to Mama! I thought that was  funny. My sister was trying to tell her not to pick her in no uncertain  terms! The rest, as they say, is history.

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I had a  hard time adjusting from rural life to city life. I didn’t like all the  cars going by on the street, since my grandparents live in a quiet,  rural area, where I did not see many cars. We three started hanging out  on the back patio and grassy common area, and slowly moved from there to  walking on the sidewalks. No sweat! I met a lot of friends (especially  boyfriends) that way, too! Now Daddy’s whole family calls me “The City  Dog”. BOL (Bark Out Loud)!

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When I  was younger, our next-door neighbors took care of me during the day. I  loved that because I got to play with their two kids AND I got to visit  with my boyfriend Rocky! We grew up together, and it was so sad when  they moved. I missed him for a while, and I didn’t understand why he  wasn’t around anymore. After that, two really nice women (Kate and Mary)  with their own dog-walking business took care of me. They walked me for  a half hour some days, and other days I was with them all day  (something called doggie day care) and went on all their walks with  them. There were days I walked five or six miles. Talk about being fit! I  met more friends and boyfriends! Not that I’m boy crazy, but I kind of  am. Then something called COVID-19 came, and they stopped walking me and  my friends. But the good news was I had Mama and Daddy home with me all  day every day! Daddy is usually upstairs working, but Mama is  downstairs with me. I see her doing something called “breathing  treatments” in the morning and at night. I usually sleep through those  since they are boring to watch, but it seems like she feels better after  doing them. Go Mama! Mama also sometimes has a nurse come in to do an  infusion. I really don’t understand what’s going on, so I sit on my bed  right next to her and watch the whole time, just in case.

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Getting  old is not for the faint of heart, am I right? I lost my hearing about  two years ago; I am not sure why. I do not mind thunderstorms or  fireworks as much now, so that’s good. I also have weakness in my back  legs, and I don’t have very good balance anymore. I started slipping on  the hardwood floors on our main level, so I now wear socks that have  slip proof bottoms. They work great, but I do like to take them off  sometimes. I like to keep my parents on their toes! I also wear a diaper  because I have some bladder control issues, but they are very pretty  with colorful flowers on them (so they tell me, anyway, since I don’t  see colors). I got a new harness that covers my whole body, which helps  Mama and Daddy keep me balanced when we go out for walks. The new  harness also has a handle on it that helps Mama guide me or carry me up  the stairs. Daddy just carries me up the stairs, but Mama likes to hold  on to the banister since she has balance issues, too. I also have dry  eyes, so I see a special veterinarian for that.

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I just  started acupuncture therapy. I really like it. I get treats during the  sessions! I LIVE for treats! I will do ANYTHING for a treat, even a  bath! Not that I’m food motivated or anything. Can we just talk about  peanut butter for a second? Delicious, am I right? My parents put my  pills in peanut butter so it’s easy for me to take them. Do I care? No!  Because peanut butter IS THE BEST! Sorry I got a little distracted there  for a second. What was I writing about? Oh, yeah, acupuncture. I do  feel like I am walking better even after just a couple of sessions. The  vet said it usually takes around four or five treatments to notice a  difference.

My  parents really do take very good care of me, I must say. I love them  very much, and I know they love me. There is nothing better than that,  not even treats!

Colleen  Adamson is 54 and has CF. She and her husband Scott got Penny in 2007  when she was only 10 months old. She is the love of their lives.

Spring  is here and, even though the days are longer, we may still have that  transition time between cold and warmer days. There is a bit of comfort  here with the orzo and cream, but the lemon, asparagus, and peas bring  in some fresh elements. This is a vegetarian dish, but you can add  roasted chicken breast or thighs for protein. I call this dish the quick  version of risotto. You do not have to stand over your stove stirring  for 30 minutes but you get a similar result to risotto with the use of  orzo. This one-pot dish comes together pretty quickly, which is great  when you aren’t feeling your best, but still want a healthier option  besides takeout or a frozen meal. The key to this recipe is to prep  everything before you start cooking. If you do that, all of it comes  together quite quickly in the end. I have put all prep work in the  ingredient list to give you a head start.

Creamy lemon orzo with asparagus and peas

Yield: 4 servings

Prep time: 10 minutes

Total time: 30 minutes

Ingredients:

2 tbsp olive oil

1 medium to large onion, finely chopped

2-3 garlic cloves, minced

1 cup of orzo

2-3 cups of vegetable or chicken stock

1 lemon, zested and juiced

½ cup frozen peas

7-8 asparagus spears, ends chopped off and cut into half-inch pieces

½ cup grated parmesan cheese

¼ cup heavy cream

1 tbsp chopped fresh herbs (dill, parsley, thyme, or basil all work)

salt and pepper to taste

Preparation:

Step 1:

On  medium heat add olive oil in a medium pot. Add onion and a pinch of  salt. Sauté for 5 minutes or until the onion is soft. Add chopped garlic  and cook for another minute.

Step 2:

Add orzo  and stir until all orzo is coated in oil. Add 2 cups of stock and cook  for the number of minutes it says on your orzo package. I recommend  cooking until al dente.

Step 3:

As the  orzo nears the last 3-4 minutes of cooking, add peas and ½ inch  asparagus pieces. Add zest of 1 lemon and its juice. Add the parmesan  cheese and stir. Test to make sure your asparagus is cooked through  after four minutes or continue cooking until you can pierce a fork  through it. If you notice the liquid evaporating too quickly before the  orzo is done cooking, add a bit more stock to ensure the orzo cooks and  the dish doesn’t become dry.

Step 4:

Add salt  and pepper to taste and pour in the heavy cream. Stir to combine and  cook for another minute to let the orzo soak in the cream just a bit.  Top with fresh herbs of your choice. s

Maggie  Williamson is 35 years old and has cystic fibrosis. She received a  double lung transplant in 2014. She now lives in the U.K. with her  British husband, Tom, and their Bengal cat, Charlie. You can find her  and all of her cooking delights on Instagram @justasprig

Every  time I turn around, I have a new skin cancer. Just a side-effect of  post-transplant life, which is mainly due to being immunosuppressed in  order to keep my non-native lungs from being attacked by my body’s  roving “security” system. And because my immune system is not as active  as non-transplanted people’s, I run the risk of infections, fungus, and  cancers—mainly skin cancer (although, I have had lymphoma, but that is  for another time).

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Alright,  full disclosure: I ran around as a child on the beach with no hat on—I  hated them. And I was raised by sun-worshipping Austrians who had light  eyes, fair skin, and constellations of freckles encompassing their  exposed skin. Nobody knew the harmful damage that sun exposure could  wreak on people later in life. I was the first generation in my family  to use sunscreen instead of baby oil and mercurochrome while out in the  sun.

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Not  surprisingly, my family had a history of skin cancers for the obvious  reasons. I sometimes went with my mom and grandmother for their skin  check appointments. This was not foreign to me. I saw them get skin  cancers removed almost monthly. They wore the white bandages from lesion  removal by scalpel or freezing off by liquid nitrogen. Then Mohs  surgery was developed and widely put to use. This new technique was  developed by Frederic E. Mohs, M.D. Mohs surgery improves patients’  outcomes by ridding them of all remaining cancer in one surgical visit  instead of having to repeatedly come back to get more tissue excised.  The Mohs procedure focuses on just the cancer cells—instead of taking  wide and deep swathes of skin, only cancerous growth with a clear margin  around it is carefully removed. The area is stitched up rather than  leaving a gaping hole to slowly heal. Before Mohs, the sites where I had  many skin cancers removed would resemble a gunshot wound because excess  skin was removed as well as the cancer. I have one of these gems on my  shoulder from a squamous cell carcinoma removal.

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From my  early sun exposure and my immune status, I had four Mohs surgeries on my  scalp about ten years post-transplant, one on my ear, and others  removed without Mohs. Thankfully I was introduced early on to a talented  Mohs surgeon who now does all my skin cancer surgeries. She or my  regular dermatologist can biopsy and confirm the skin lesion is either  basal, squamous, or melanoma. However, Mohs is not used for removing  melanoma—doctors still use the old “dig deep and gouge it out”  technique. I only had one of these procedures, thankfully. Otherwise, I  see a regular dermatologist who is aware of my immune-compromised  situation and she knows I need a skin check every three to four months  to get things in their nascent stage. She will biopsy suspicious skin  lesions or moles that look like they are changing shape or color. Once  the report returns as positive for cancer, I see my dear Mohs surgeon.

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The next  part of this story might be upsetting to some. It still freaks me out  and I went through it. I am usually the one who notices a bump or an  elevated area of flaky skin and I go to get it biopsied. I saw a dreaded  bump on my eyelid right near the lash line. I tried to get in to see my  regular dermatologist but, because she changed hospitals, I was  considered a new patient and could not get in quickly. I did get in  quickly to my Mohs surgeon. I showed her the small bump and she said it  is probably nothing, a benign growth, but because it is you, we should  be thorough and biopsy it. The worst part was the biopsy process. She  had to inject my eyelid with lidocaine to numb it to slice off the  smallest area to send to the lab. I apologized and told her that I am  fine with other parts of my body being cut, jabbed or sliced, but get  near my eye and it is going to be a fight. So, I was quite squirmy as  she drew near with the dreaded lidocaine syringe. She assured me she is  way worse when it comes to her eyes, and her nurse nodded in agreement.  It was nice to be understood. I tried to breathe deeply through it all.

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I  learned with relief it was a benign growth. However, after I finally saw  my regular dermatologist, she looked at it and the bump was still  slightly there and flaky so she sprayed it with liquid nitrogen, which  was not pleasant to say the least. About five days later, the scab that  formed from the nitrogen freezing fell off. It was finally smooth.  Unfortunately, about two months later, the bump was back as if nothing  had been removed, frozen, or biopsied. I thought, no way, not cancer. I  just could not deal with the thought of having to get another injection  on my eyelid so I let it go for a week. Then, it started getting bigger.  A white head formed on it and it kept getting bigger. I was asking  people what they thought it was the entire time. Many thought it was a  cyst. I thought, ok, so this can be lanced and I will be fine. I emailed  my dermatologist and was eager to see her because in 15 days, the  “cyst” had quadrupled in size.

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Part of  me dreaded investigating this as I feared it would require another  eyelid injection or I’d develop an infection from the cyst bursting. Way  far back in the depths of my brain, I feared cancer. “THIS IS CANCER!”  it screamed. I could not see my dermatologist for two weeks and when I  finally got there, she said, “I cannot touch that. You need to see an  ophthalmologic surgeon or a Mohs surgeon.” She said I should go see the  surgeon who does my Mohs surgeries. I called her office and sent photos  of my eyelid via text. They saw me an hour later and biopsied it. She  said it was not a cyst and probably a basal or squamous cell carcinoma  and that I would not only need Mohs but also an ocular plastic surgeon  to repair the eyelid after she removed the growth.

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She  recommended three different ocular surgeons. Even though she removed the  majority of the growth for biopsy purposes, the cancer was growing back  rapidly. It was very upsetting and made me very afraid of what lay  ahead. Once I had the biopsy report five days later, I started calling  the surgeons. With some cajoling and telling the surgeon’s office  manager that the cancer is on my eyelid, that it was growing rapidly,  and that I am immune suppressed, I was able to see him quickly. I had to  go into serious self-advocator mode, meaning, aggressively pushy. The  first doctor I met with was my Mohs surgeon’s favorite. When I met him  and heard his plan of action, I was certain he was the right surgeon. I  got a good feeling about him when he declared, “This has to be removed  ASAP.” He put me on his surgery schedule for three days later.

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It was  then arranged with my Mohs surgeon’s office. They fit me in the same  day. I was to get Mohs on Monday morning at 8 a.m. and, once the margins  were clear, run uptown to get the plastic surgery in the operating  room. I reported early to my Mohs appointment. I knew it would be  several hours and passes of the scalpel to get this area cancer free.  Because I was not going to be sedated for this process, I took an Ativan  to calm myself. My face was draped. A giant opaque contact lens was  placed in my eye. She numbed me and then she began cutting. What she cut  was then sent to her lab on the premises to check for cancer-free  margins. This happened two more times and the third time was the charm.  Even though I was numb, on the last round, I heard the scissors clip and  knew it was a section of my lashes. It was 10:30 a.m. and I was  bandaged up with sight only in one eye.

I could  not eat anything that morning because I was going to be sedated for my  second surgery—thank goodness! I was brought in to the operating room  rather quickly, saw the ocular plastic surgeon and his associate, who  explained they may need to do a skin graft, which they would take from  behind my ear, most likely. The Mohs surgeon had sent him a photo of my  eyelid so he knew what he was dealing with before I got there. My  surgery was going to take two or three hours due to the skin graft.

As luck  would have it, since I am 58 and never had an eye lift, the ocular  surgeon was able to take my extra (sagging) skin near my brow bone and  move it to cover the area on my eyelid. No skin graft was needed.  Because the Mohs surgeon had left a hole in my lid, the ocular surgeon  removed a slice or section of my lid where the hole was and then  stitched the two sections together. The affected left eye opening is now  slightly smaller than my unaffected right eye. The left is also  perkier, instead of sagging closed like my right. He did a fabulous job.  Unless up close, you cannot see the carnage. That is not to say I did  not suffer depression for several weeks after from the trauma of those  two surgeries. I felt defeated. But eventually I knew I was fortunate to  have the care of two great surgeons who made it their mission to cure  me.

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I have  gone back three times since surgery to check in with the ocular plastic  surgeon. He is pleased with how it is healing. I am missing a section of  lashes in the middle. Thankfully my vision is not impaired and I get to  live to tell this tale. I was told by a nurse that this kind of cancer  can spread by traveling up through the nasal passages to the brain. They  had actually seen this in another patient.

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My  dermatologist, the Mohs surgeon, and then the ocular plastic surgeon all  basically said the same thing to me: “Thank goodness you got this  addressed quickly!” I wish I had not been so afraid of an eyelid  injection and moved faster. Had I to do it all over again, I would not  have hesitated as I did. This is a cautionary tale to those who are  immunosuppressed. Do not put off any suspicious bumps or lesions.  Mountains quickly form out of mole hills! You will be happier if you act  swiftly.

Andrea  Eisenman is 58 and has CF. She lives in New York, NY, with her husband  Steve and dogs, Willie, Roscoe, and new girl, Trixie. Andrea is the  Executive Editor for USACFA. She enjoys cooking new recipes, playing  pickle ball, biking, tennis when possible, and staying active as her  health allows. Her contact information is on page 2.

Readers,  I’m thrilled to introduce my friend and fellow community advocate Jes  Davis! I first met Jes through Andy Lipman’s initial CF Warrior Project  book and the social media groups he created for participants to connect  with each other. Since then, we’ve stayed connected and worked together  on several advocacy initiatives focusing on racial and intersectional  justice within and beyond the CF community. When they’re not organizing  for Black Lives Matter, Jes continues to build their career as a  professional actor and filmmaker. And they still find time to do an  occasional modeling shoot, making still photos come alive with shining  confidence. They maintain a lively and thoughtful social media presence  across multiple platforms offering sneak peeks at their latest projects.  Jes is a courageous advocate, a master storyteller, a generous friend,  and a fashion icon. I jumped at the chance to celebrate their  contributions to the adult CF community with an interview!

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Age: 33

Home: Oakland, CA

What should our readers know about you?

CF is  like my partner in crime. I spent years demonizing it because that’s how  the world perceives disease. But if I die, the CF dies; it needs me to  sustain itself. So it’s kind of a working relationship. I don’t know if  other patients feel connected to that sentiment; to me it’s stronger  than thinking you have to destroy part of yourself. Society sees disease  as something to conquer and defeat. For me the “defeating” aspect is  seeing illness as a partner. That’s how you stop it having such agency  over your life.

How did your CF first get diagnosed?

I was  three years old at diagnosis, even though I showed symptoms at birth. My  CF wasn’t diagnosed right away because of racism. My mother works in  the pharmaceutical industry; she has a wealth of medical science  knowledge. She recognized certain symptoms like fat intolerance and  tailored meals for me and my brother accordingly. But most doctors we  saw were white, cisgender, heterosexual men who didn’t take kindly to  being challenged by a Black woman. When my mom changed my brother’s  diaper in the clinic, the nurse recognized immediately that the smell  wasn’t normal. She’d been trying to tell them exactly this and getting  dismissed! Obviously, it shouldn’t be that hard to be heard so getting  diagnosed was not a great experience. We were still met with racism  afterwards, even from our CF specialist. Someone called Child Protective  Services on my mom because my brother was struggling to gain  weight—nobody had diagnosed his diabetes. The medical industry wants to  blame Black patients rather than understand disease in each person. This  kind of arrogance is rampant.

Can you tell us about your racial justice work?

My  community organizing work hit a stride during the George Floyd protests.  During 2020 I also connected with some CF patients who are also Black  and generally of color, like some Indigenous and Latin folks who don’t  have Black heritage specifically. I heard their stories about being in  doctors’ offices, their relationship to the CF community, and having to  explain the disease. There’s still a lot of dismissal—not just from  doctors, but from people in general—for Black people with diseases that  aren’t associated with our race. My activism emerged from the racism I  endured in clinic and school. That emboldened me in taking more of a  stance. People want to stifle movements because advocacy makes them  uncomfortable. They’re afraid of change, and of you as an activist. It’s  both a sword and shield when we are advocating for change. I’m always  confronting people who are complacent—because complacency is complicity.

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What are you working on right now?

The film  that I’m producing right now is called POV. It’s told from the  perspective of a feminine person on a date with a masculine person. This  is going to be purely from the feminine perspective because we always  get the masculine perspective on how people relate to the dating world  and those spaces. We’re going to highlight how objectifying these  perspectives can become. People don’t realize how much porn influences  the objectification of a lot of bodies, particularly more feminine ones.  These same perspectives often appear in movies outside of porn itself,  with things like shot angles and composition, because people respond to  them. We wanted to do something that flips this dynamic and shows how a  masculine person can also be objectified by a feminine view. I like  watching porn and find empowerment in saying that openly. A core goal of  the film is empowering femmes in a sexual space while also seeking  accountability from mascs.

How did your acting and filmmaking career get started?

Parents  fear sickness from other kids—even if someone’s cough isn’t contagious.  Having that isolation forced on me, I embraced being alone so it became  enriching. My brother also has CF; I had some camaraderie. But I’ve  always loved watching movies because I didn’t need friends to do that.  Movies became a coping mechanism for CF exacerbations or depressive  symptoms or anything else. I moved into acting and writing as well; when  I was a kid I did a Little Caesar’s pizza commercial. My love of  performing came from these early experiences where I could “take off” my  CF and dive into a different reality. Over time I became passionate  about using my body to create. This helped me see it wasn’t broken or  damaged just because I had a chronic illness.

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What drew you to working in the arts?

I acted  out the death scene from Bambi as a kid. Having CF means confronting  your mortality at a very early age. When you have a chronic illness you  get told you’re not invincible—and that you could die, possibly soon.  This drew me to the tragic elements in performing. So what I’ve learned  from having CF helps me in some ways, but I still have to fight for my  roles. The industry remains racist and I don’t audition as much as my  white peers do. I feel like we’re on the precipice of changing that with  more inclusion and diversity. But we still have to fight virtue  signaling and performative inclusion. People need fully fledged  characters instead of flat ones who only exist as “check marks” for  diversity. As an actor of color, I often have to create my own work. The  upside is that I now have a platform to share my experience my way.

How do you prepare for a new acting role?

It  changes from role to role. As a society we have very black and white  thinking, which has marginalized so many people. Gray areas are often  uncomfortable for people to acknowledge. So when I approach a character,  I always like to see the gray—I feel like there’s more information in  those areas. Humanity is gray; it’s not all good. I look at emotional,  mental, and physical aspects and how those all bleed into one another.  And I focus on creating different sensory inputs for the audience rather  than relying entirely on my body or voice. I want to be inclusive with  my performance. In the process, I’m dismantling this white cishet male  ideal of the method actor, which is very predatory. There’s no “right”  way to pursue a character—only how you feel and how you’re making the  audience feel.

Have you ever declined a role because of health challenges?

I don’t  think so, but on the other hand I’ve done lots of stubborn things to  avoid being hospitalized. For example, in high school I was  force-feeding myself so that I could stay on the track team. My doctors  at the time didn’t even see this as something negative. They just  praised me for “being so compliant” and “working so hard” instead of  thinking about what I needed. I spent so much time and energy appeasing  my white male doctors. Now I do things that make me comfortable and  promote others doing the same. I’ve pushed my physical limits for  certain roles, though. I’ve taken roles where I have to smoke a  cigarette or stay up all night. I didn’t want CF to limit my choices—and  I wanted to prove to myself that I could do it. I’ve turned down roles  before, but usually for other reasons; maybe I didn’t like the story or  the character.

What do you feel proudest of in your activism work?

People  rallied when I got passionate about feeding the community and started a  community fridge. They came together and protected that effort. I saw  people get inspired and passionate; I met folks who were excited to  help. I organized a clothing drive. I organize school drives and  resource drives for communities that are underserved and  neglected—predominantly ones that are home to Black and Indigenous  people of color. Feeling guilty about not making it to every action  remains a struggle—but I go back to the idea of gray areas. You can’t be  everywhere all the time or expect that of yourself as an activist. I  try to give myself as much grace as possible so that I can work smarter  and not harder.

Tell us more about your own journey with CF.

I’ve  always had a very tiny physique. I got picked on in school and people  thought I had an eating disorder. Being small was ridiculed because it  was also coveted, especially in the performing arts. I think we all  should be able to exist in our bodies without societal judgment. CF can  contribute to that, depending on where you fall physically. Everyone in  my family is tall except me and my brother. All those nutritional issues  probably stunted our growth. So I have a height insecurity; I’m always  in heels. People think I’m taller but I’m just in stilts all the time! I  feel like the odd one out.

Do you have a dream role you’d like to play?

I used  to want to play Josephine Baker. Her struggle was like mine; she was a  black sheep who never fit in. She had to travel abroad to find a  community that really enjoyed her performance and expression. Being a  Black femme in the South who was lighter-skinned, and seeing the  hypocrisy in colorism, was also relatable for me. But nowadays I’m drawn  less to biopics and more to roles that are physically demanding. I’ve  always wanted to do an action role! Lately I’ve been submitted for a  couple of Marvel Cinematic Universe productions.

What helps you cope with your CF?

The  biggest thing helping me cope is knowing I’ve survived thus far—meaning  I’ve outlasted the idea that CF was all I’d ever be or do. When I feel  more defeated or depressive, I always appreciate not being defined just  as a CF patient. At the same time CF can be invisible, which makes  people question whether it’s really a fatal disease. I think of it as a  badge of honor and an important part of me rather than something to  destroy. But I do have survivor’s guilt and sometimes I’m distant from  the CF community because of this. It’s painful when you form bonds and  then people die. I’m working on that in therapy. How can I be involved  and still protect myself emotionally? How do I not feel like I’m  abandoning my community while also not forcing community? Right now that  means advocating when I have capacity and resting when I don’t.

What advice would you give your younger self?

I was a  different person before 2020. I’ve always been very rebellious,  anti-establishment, and anarchist. But before that year, part of me was  still flirting with the idea of the status quo and trying to fit in. I  see this in the people I dated and befriended, and in my working  relationships. These days I really protect and value my comfort,  integrity, and values above everything else. So I would tell my younger  self “Don’t look back. Keep going forward.” As a society, our  projections of our past hold us back—especially when we try to make the  past our present.

Do you have a funny CF story?

My mom  had this bag of “nasty capsules”—enzymes that she would find on the  floor. She’d keep them in her purse; whenever my brother and I didn’t  want to do treatments she’d threaten us with those! She’s a science  person, pragmatic and good at problem solving. That running joke about  the nasty capsules was genuinely helpful. My mom and dad used “tough  love” and dark humor to show me and my brother we could be  self-sustaining. To this day I usually do well at remembering to take my  meds—including Trikafta which has reduced my treatment burden. I have  my own little tricks, like keeping enzyme capsules in an Altoids tin so  they’re easy to carry.

What would a perfect day look like for you?

Getting  up, taking my meds, minimal coughing all day. Maybe doing some rigorous  exercise. Spending time with my partner. And doing what I love to do,  which is creating. Whether that be my fashion designs, my visual art, or  my film work—just being able to create in an open and comfortable  space. Somewhere in there, I’ll go to the beach because I’m a  Californian. Much as I love New York, the beach will always be my home. s

Dr.  Alexandra “Xan” Nowakowski is 39 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.

If  you would like to be interviewed for “In The Spotlight,” please contact  Xan Nowakowski or Andrea Eisenman. Their contact information is on page  2.

My  husband and I got married, bought a fixer upper and made it ours. One  holiday, I presented a baby card to both sets of our parents.  “Pregnant?” they asked in excitement. “No,” I replied. I blurted out  that we had genetic testing and Arden isn’t a carrier, so we are able to  have biological children together. The testing cost us four thousand  dollars.

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A couple  years later, we decided it was time to grow our family. We took one  last trip—a two-week journey through France: Paris, Troyes, and  Champagne. Fitting for a last hurrah—exploring the wine and champagne  houses and toasting to our future. We arrived home and I stopped my  birth control. I switched from oral meds to insulin and changed a few  other meds per my doctor’s advice. I had spoken to my doctors and they  were on board. At the time I was given the ok to start trying, my lung  functions were at 83%.

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Not pregnant.

After a few months of trying, I tried figuring out my ovulation, got a tracking app and a few more months passed.

Not pregnant.

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I spoke to my doctor and they recommended making an appointment with a specialist. It can take a while; you can always cancel.

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I got  blood work, a sperm sample from Arden, and a hysterosalpingography (HSG)  test. The HSG test is an x-ray procedure used to look at your uterus  and fallopian tubes (yes, this hurts but only for 30 seconds). I read  somewhere that many get pregnant following an HSG as the dye somehow  cleans out your fallopian tubes. That was hopeful.

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Unexplained infertility.

The  doctor said we were lucky—we came in early, we were young, and we lived  in Massachusetts, where fertility coverage is mandated. The process  would be two IUIs, which are required by our insurance, and then, if  that doesn’t work, we would move onto IVF.

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We  decided to keep trying on our own. Another year passed. We explored  foster care and took the training but didn’t go any further. My  husband’s family had three trees come down through their home so they  moved in with us for a year. We went to therapy. Another degree was  completed. We traveled more, especially when I was ovulating. I tore my  knee in three places from an unfortunate jump in a bouncy house. I could  not drive and, because I worked an hour away, I was out of work for six  months. Eventually, we returned to the doctor.

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We had  to redo all the testing per insurance. We did three IUI’s. My husband  was praised for attending all my appointments as I was on my back having  a procedure done while my leg was broken.

Not pregnant.

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I  returned to work. We had our own gynecological egg hunt on Easter: 11  eggs; 10 mature and seven fertilize. We got four embryos. We opted for  preimplantation genetic testing, which tests the chromosomes and  supposedly reduces the risk of miscarriage. We paid $3,900 for this  extra testing. A few weeks went by and I got the call that three of the  four embryos were chromosomally normal. Three girls. Visions of three  twin cribs side by side in a warm nursery entered my mind.

A couple of months later, we did our first transfer. The call came two weeks later.

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Pregnant...wow, ok!

Our beta  numbers were excellent. They doubled and continued to rise until, at 11  weeks and three days, our baby no longer had a heartbeat.

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I  applied for jobs while bleeding in the bathtub as I was sick of staying  at a job an hour away just because I’d worked there long enough to feel  comfortable taking maternity leave. We spent the next couple of months  in despair. I went to therapy. I talked about getting an Australian  merle puppy. I read books about pregnancy loss and childlessness not by  choice. I spent multiple hours at appointments.

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We  decided to try again. This time there was no excitement, only anxiety.  The transfer happened. Our beta number was low. Only 41, while our first  transfer was over 200. I cried in the bathtub, thinking, “it didn’t  work.” But it did, and the number kept rising. We passed the twelve-week  appointment. We finally let our guard down after the anatomy scan but,  the next day, the world shut down due to COVID-19.

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I wish I  could say that our path became easier after that. Unfortunately, I  can’t. However, we now have two wonderful girls: Rose, who is two and a  half years old, and Magnolia, who is one. And we will not be getting a  merle puppy anytime soon.

My  advice is to prioritize your relationship with your partner—let them  into your world and find ways to continue to have fun together. Plan  things to look forward to. Therapy can be a helpful tool.

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The path  to parenthood for us started years before we were ready to physically  start trying. I never considered that we could have difficulty getting  pregnant. Both my medical team and I were always more concerned about my  health and how my body would experience pregnancy (my body did great).

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Infertility  is really common. In general, one in seven couples have difficulty  conceiving and one in four experience pregnancy loss. Unfortunately,  these things aren’t talked about, which leads to people feeling like  they are more alone in their experience. We told everyone about our  first pregnancy because we thought we were in the safe zone. I recommend  only telling people who can grieve with you as much as they can  celebrate in the early weeks. A benefit to people knowing is that they  shared their own experiences; ones I would never have known otherwise.

Katherine  Lockwood is 35 years old and has CF. She lives on Cape Cod with her  family. She recently published a children’s book, Why Me, Mama?, after  her daughter was born with an even rarer condition than her own, so that  all little ones with differences could see themselves represented in  children’s literature and know that they matter. Katherine works with  individuals and couples as a therapist and specializes in infertility  and disability issues. You can reach her at KatherineML22@gmail.com or acorncottagepress.com.

I  was born in 1984 and diagnosed with CF when I was 13 months old.  Growing up, I knew that one day, if I was lucky enough to still be  alive, I would love to become a mother. Starting at an early age, I  spent a large portion of my time in the hospital for CF exacerbations  and, because of this, I also knew about a lot of older CF women who got  pregnant and became moms, but, ultimately, lost their lives because the  pregnancy was very hard on their bodies. That scared me a lot. As I got  older, I also learned that there were many different paths one could  take to become a mother.

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I  started dating my now husband, Joey, in 2008. We met in 2001 at a  restaurant where we both eventually worked. We became really great  friends at first because our age difference was an issue at the time. He  is three years younger than me and, while that is not an issue now, it  was when we worked together at the restaurant as he was 14 and I was 17.  I knew that, in a few years, he was going to make someone really happy.  I ended up moving away to go to college and then nursing school. While I  was in nursing school, I lived at home again and worked a few nights a  week at the restaurant. Joey was now 20, almost 21. We went out a few  times and ended up dating. I knew he was the person I was going to marry  someday. We had talks about the future and we both wanted kids. We had  conversations about how it might not be the best for me to carry a baby,  if I could even get pregnant, and that we might have to look into  different avenues to becoming parents. From the beginning this was never  an issue with Joey. He wanted me around for the long haul, so if  getting pregnant would somehow jeopardize that, he was willing to try  something else.

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In 2011,  I was told that I needed to be evaluated for a double lung transplant. I  decided that for me, personally, a chance at transplant and extra years  with my family and Joey were worth all the risks that came along with  it. I was on the transplant list for six and half months and was  transplanted in November of 2011. The transplant was a success—I felt  amazing, like I had a brand-new lease on life. In 2012, Joey proposed.  Our wedding was set for October 2013. At the end of 2012, I was admitted  to hospital with a virus and ended up on life support, ECMO, and  dialysis. The doctors didn’t know if I would make it, but I did. I came  off all life support and slowly recovered.

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I had  started seeing a reproductive endocrinologist a few years prior to this  because I always had really painful menstrual cycles, and I usually got  sick every month right before my period came. Our wedding was fast  approaching and I made it clear that I had always wanted to be a mom, so  my reproductive endocrinologist suggested I freeze my eggs because of  all my medical issues. I discussed this with Joey and we were excited to  proceed. I scheduled the date, ordered my medicine, and, just as I was  about to pay for the procedure, the head doctor at the fertility office,  without even seeing or speaking to me, called me to say it was  cancelled because I was “too risky” a patient. I was crushed and didn’t  know where to go from here.

Right  before our wedding, I started getting short of breath just walking small  distances. When we got back from our honeymoon, after having many  different tests and ruling other causes out, I was told I was in chronic  rejection. I started treatment to try and stabilize my lung function  but nothing worked. My only option was a second double lung transplant.  My kidney function, however, prevented me from getting listed. After my  life support episode, my kidneys never recovered completely. I was told I  needed a kidney transplant before I was able to be relisted for a  second double lung transplant. Two of my sisters got tested and were  both a match. Christine was a slightly better match, so she donated one  of her kidneys to me in July 2014, and I was actively listed for lungs  again in August. In December, I got sick and was admitted to the  hospital and was told that I was too sick to continue to wait for lungs  at home. I knew that I was either going to be lucky and blessed with a  second double lung transplant or I was going to die waiting in the  hospital. After two dry runs, I was finally transplanted with my second  set of donor lungs in February 2015. I went home in March and started to  really dream about my future.

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In 2016,  I found a different fertility center apart from my reproductive  endocrinologist. They knew all about my complicated medical history and  had several other CF patients and were willing to let me try and freeze  my eggs. The hope was to freeze embryos and have my sister be our  surrogate. I did two rounds of hormone shots and retrievals, but the  embryos that were harvested never made it to the freezing stage. My  husband and I didn’t know what to do next, but I wasn’t ready to give up  on my dream of becoming a mom.

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In 2017,  two years after my second lung transplant, I was healthier than I had  been in a very long time, and I got the approval from my doctors to try  and carry a baby myself. My husband and I tried for six months naturally  and nothing happened. We moved on to IUIs. We did five and they all  failed. Each month, the anticipation of wondering if this could work,  followed by the devastation when it didn’t, was a lot. It was like a  rollercoaster of emotions, from hope and excitement to defeat. After the  failed IUIs, we decided we were going to take a quick break and start  IVF in 2018. Unfortunately, in 2018 I had some kidney complications and  had to restart a medication that was not safe to be taken while  pregnant.

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At this  point, we still had my sister willing to be a surrogate for us, but we  didn’t have any embryos. We decided to purchase a batch of donor eggs.  We went through a ton of egg donor profiles online and found one that  looked like me and had a similar background and characteristics. Six  eggs came in the batch. We fertilized them with my husband’s sperm, and  two of them made it to the freezing stage. We now had two embryos ready  to go. We were going through the process of drawing up legal contracts  and getting screened by the fertility center and ready to transfer an  embryo into my sister, when I found out I was unexpectedly pregnant. We  did not move ahead with the transfer into our surrogate. I couldn’t  believe that after all the time trying to get pregnant naturally, and  then the failed IUIs, that I just randomly conceived. I called Joey  right after I took a test and we were both freaking out; we couldn’t  believe it. I called my doctors right away and was taken off the  medicine that was not safe for pregnancy. I was in utter shock that I  was pregnant but also really excited and, if I’m being honest, a little  scared. Unfortunately I miscarried around nine weeks. I was completely  devastated, but I also had hope that one of the two donor egg embryos we  had frozen was going to work for my sister. We transferred one in  January 2019 and it took. At the eight-week scan, there was no growth or  heartbeat. We transferred the last embryo in July 2019 and it never  took; it was a failed transfer. We were back to square one.

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Deep  down I kept thinking and asking myself why I was able to get pregnant  after all of that time just to end in a miscarriage. I had this gut  feeling deep down that maybe even though I was three years older, that  it was a sign saying my eggs were healthier. I felt like it was someone  saying, try one more time. I decided to try and harvest my eggs one last  time at the fertility center where I was doing the IUIs. The doctor had  a different protocol of medications and, on New Year’s Eve, 2019, they  harvested 11 eggs. Six embryos fertilized successfully and two healthy  embryos made it to the freezing stage! We were ready to try one last  time with my sister as our gestational carrier and then COVID-19  happened. By the time procedures like an embryo transfer were allowed  again and the timing was right for my sister, we had a scheduled FET  (frozen embryo transfer) set for September 2, 2020. On September 11 we  got the phone call that said she was pregnant and her numbers were  looking amazing. We were cautiously optimistic at this point because we  had both been here before. I lost the baby at nine weeks, and she lost  one at eight weeks. We didn’t get to go to any doctor’s appointments  with her because of the rules they were following for COVID-19, but,  after every appointment, we were reassured that things were looking  great. We started to actually get excited that our dream of becoming  parents was finally happening. We did a gender reveal in November and  found out we were having a baby girl. On May 11, 2021, my sister  delivered our baby girl, Riley Kathryn Monte.

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Being a  mom and seeing Joey as a dad is the highlight of my life. Joey was ready  to give up at a certain point but now thanks me for being so stubborn  and never losing heart. I often catch myself driving and looking in the  rearview mirror in utter disbelief that she’s real and all mine! She’s  almost two years old and I can’t believe how fast the time goes. I am so  thankful for modern medicine, awesome doctors, and my sister for  helping our dreams come true. It took so many years for us to get here  from the time we just started thinking about freezing my eggs, to my  failed retrievals in 2016, and all that followed after. I hope one day  to share this all with Riley, so that she can see just how badly she was  wanted and how much she is loved.

Katy  Monte is 38 years old and has CF. She lives in Bayside, NY. Katy is a  registered nurse but currently a stay-at-home mom. She loves reading,  exercising, and spending quality time with her family.

On  Saturday March 5, 2016, I married the man of my dreams, Michael Kowal.  And from that point on, we tried for a baby. After months of nothing  happening, we looked into infertility treatments and other possibilities  to conceive our own little human. At the time, surrogacy was not  allowed in the state of New York and adoption was insanely expensive.  Some agencies I spoke with had a hard time adopting out to someone who  had a “terminal illness.” Michael got tested to see if he carried a  faulty CF gene. Luckily he does not, but, either way, we were going to  keep trying. On December 7, 2017, I had a hysteroscopy, dilation and  curettage (D and C), polyp removal, and endometrial ablation. The  doctors opined that if we were going to become pregnant on our own it  would be shortly after this surgery. Within three days after this  surgery, I was diagnosed with cystic fibrosis-related diabetes. During  all this, my sister and her daughter (our niece) lived with us. Gabby is  our niece whom we treated as our own; she is also our God Child. We did  everything with our Gabby—she was our child in any work function and  life event to which we were allowed to bring kids. We would pretend she  was ours at all times and snuggle her and love her more than she would  ever know.

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I was  feeling defeated after all these months and issues of not being able to  create life with the love of my life. We had a massive four-bedroom  house that we had this feeling we would never fill. As much as we didn’t  want to give up about expanding our family and carrying on my husband’s  family’s last name, it just did not seem to be happening. We listed the  massive house in search for a smaller home, and no longer cared about  the amount of bedrooms because we gave up on becoming parents. In August  2019, we bought our forever home in a very small community known for  its corn, cows, and tractor pulls. We love where we live and our new  one-bedroom home with its two and a half bathrooms (have to have enough  bathrooms with CF tummy).

Along  came Trikafta in November 2019—that medication made me feel absolutely  amazing. “The Purge” certainly happened and with EVERY SINGLE ORGAN of  mine. It was amazing how outstanding I felt, how much life became  better! But, here came COVID during March of 2020. And I was mandated to  stay home until further notice, but the story did not end there: It  became very interesting.

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On April  10, 2020, I knew something was off. I knew somewhere in the mass of old  bathroom items I had an expired pregnancy test. I took that test while  my husband was working out, and it came out with an answer I never  thought I would see: “PREGNANT.” I was shaking, excited, terrified, and  shocked. I walked into where my husband was working out and told him to  get up now, and then I showed him. We both didn’t believe it, and every  day for four days he brought home different pregnancy test brands for me  to try; all of them turned out to be positive. After I called CF  clinic, they suggested this would be happening as Trikafta cleans out  EVERYTHING, making people more prone to becoming pregnant. So here we  were in our newly purchased one-bedroom home: After mentally being okay  with never having children and just being amazing aunts and uncles, we  were now being upgraded to parents.

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All  because of Trikafta, our little miracle happened, when we least expected  it and weren’t even thinking of it. We were pregnant during a very  stressful time with COVID. My husband was not allowed to come to any of  my doctor appointments or FaceTime me while I was there. It was very  lonely at some times and at others so exciting. During my pregnancy I  had preeclampsia and my pregnant belly was always showing more than  what’s considered normal at each week during my pregnancy, probably due  to the diabetes. On Saturday November 14, 2020 my doctor advised that my  blood pressure was too high and to drive to the hospital, so I drove  myself and my husband to the hospital. We got there at about 11a.m. and I  began getting treated for stroke. Our miracle man Ernest (“Ernie”) was  born at 2:53 p.m. that very day, perfectly healthy and to the song  “Don’t Stop Believing” by Journey (our doctor asked what music we wanted  on during the C Section). We were able to both add a room to our home  and add this amazing gift with no change in my health. And Gabby was  under the impression Ernie was now her little brother.

Of  course, the thoughts of my CF interfering with being a good parent  crosses my mind all the time, but I also have Ernie “help” Mommy do  things to make sure I stay on top of my own health. He helps with  scanning my CGM and holds my nebulizer when I do it. I fully believe in  talking about Mommy’s cough, potty breaks with him, and how being “sick”  means we need to rest. Being open has helped me be the person I am  today and the parent I have become. Although right now things are  becoming different, as toddler years are a whole new ball game, I am  forever grateful for our miracle man and all of life’s precious moments.

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To  anyone out there looking to adventure down this road, be yourself and  own your issues. Let your doctors know your stance on life and your  hopeful adventures and desires. Tell your loved ones your plans and  goals. And truthfully just be prepared for whatever happens, because  life will most likely not go the way you expect but turn out much better  than you ever imagined. I wouldn’t change a thing that happened to us. I  love every aspect of our journey and I would love to talk to anyone  about what theirs could be.

Nicole Kowal is 35 years old and has CF. She lives in Buffalo, NY, with her husband. Her email is: abnormalnicole@gmail.com.

I’ve  been anxious most of my life. I’m anxious right now while writing this  piece. I had no idea that this anxiety would skyrocket as a result of   taking the drug I’d been waiting to have for 57 years.

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I  started Trikafta in 2020 in the hopes of seeking the miraculous  experience of clear sinuses. I was one and a half years post-transplant  at the time. If my pancreas or GI system also improved, that would be  gravy. My sinuses were a mess and sharing Pseudomonas with my new lungs.  To begin Trikafta I had to first switch from one anti-fungal to  another, which, in turn, required that I increase my daily dose of  tacrolimus, a nonnegotiable immunosuppressant, one of many  post-transplant drugs.

Within  days, my sinuses were wide open, my sense of smell increased, and I was  happily somewhat stunned. During the course of the first three months, I  also noticed a change in my GI system: Some of my symptoms were gone  but replaced with different ones. My need for insulin had begun to  decrease a bit.

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After  several weeks on Trikafta, my anxiety slowly intensified. Situational  anxiety became unbearable. I’d experience panic attacks—having that  end-of-the world feeling. I increased my exercise and mindfulness  practices in order to get through the day. It appeared that the  combination of Trikafta with my transplant meds was making my anxiety  escalate significantly. After three months it was obvious I had to stop  taking Trikafta. Clear sinuses and using a bit less insulin could not  outweigh living in a crazy wound-up mind.

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While my  clinic has left it up to me to decide to try a modified dose, I am  afraid to try it. I know others have had success with a modified dose  but I’m hesitant. I’m afraid I won’t get enough of the benefits and that  my existing anxiety will just increase. If anyone else has dealt with  anxiety issues and tried the modified dose, I’d like to connect with  you.

Suzanne  Joyce is 60 years old and has CF. She lives in Clearwater, FL. She has  been enjoying blues and rock music at outdoor venues and exploring  nature in Florida. You can email her at exesq1@gmail.com.

Editor’s  note: The story below is part two of Rachel’s two-part story depicting  her climb to Everest Base Camp four years ago. Part one was featured in  the Winter 2023 issue of CF Roundtable.

Rachel  worked in an ICU for two years and, in 2017, quit her job, sold her car,  put all her things in storage, and left to travel the world. She joined  a missions organization called Adventures in Missions and went on a  mission trip called “the world race.” This took her to 11 countries in  11 months (El Salvador, Guatemala, Honduras, Nicaragua, Côte d’Ivoire,  Ghana, Nepal, India, Thailand, Malaysia, and Indonesia). One of those  countries was Nepal—where this adventure took place—at the time she was  26. That year was life-altering for her. She got sick several times and  really questioned God’s goodness and plans. But she had no idea He would  take her to the heights He did. She continued traveling for another  nine months with a different organization called G42, with whom she  spent six months in Spain and three months in Iraq, working in a refugee  camp and underground church.

Day 6:

As soon  as I saw him, I knew. “Can you come in here please? He’s making this  weird, scary noise that I’ve never heard before.” Before I heard it, I  already knew. In critical care, it’s called, “the death rattle.” The  all-too-familiar sound pierced my heart. So many times before I had  heard this sound and not let it permeate me. This time, though—this time  I felt it. Deep. In my very being. This man was going to die.

​

***

I woke  up that morning after a hellish night. The enemy left me absolutely and  utterly defeated. I woke up anticipating having to announce to my  teammates that I could no longer go on and to go ahead without me. I was  preparing myself to spend three to four freezing nights here in this  small, abandoned tea lodge at 4,400 meters completely alone.

​

Today  was another acclimation day—a shorter hike than our first, but grueling,  nonetheless, or so I had heard. I stayed behind to rest. I lay in my  bed most of the morning, just staring at the wall and trying to use my  human comprehension to decipher just exactly what God’s plan was in  having me come on this trip, only to fail halfway. Perhaps I had made  the wrong choice coming in the first place. Maybe my expectations for  this adventure with God were too high. I was lost—lost in a sea of  confusion and condemnation, drifting farther and farther away from God.

​

I was  still lost in my head when my teammates came back, and we all sat down  together in the lodge for lunch that afternoon. I was trying to gather  my words to tell them I would not be able to continue with them, when  one of the trekkers we had met on the trail came into the small, sunlit  dining room and interrupted us. “Hey, Rachel, you’re a nurse, right?”  Oh, gosh, I’ve heard this question before. Usually it’s preceded by a  “Could you look at this weird spot on my (insert awkward body part  here)?” or “So I’ve had this weird rash.” Reluctantly I said, “Yes…?”  “Well, there’s this guy downstairs in the next lodge who isn’t doing so  well. I thought you might be able to take a look at him? He’s not really  responsive.” Any form of the word unresponsive is an immediate red flag  to a critical care nurse. I left the remains of my cup of hot chocolate  and moved quickly toward the door, leaving my jacket. As I followed him  down the stairs, out the front door, and toward the next lodge, I  immediately started praying.

​

February  is the very beginning of the season on Everest. Even though it is  “technically” in season, the villages are still ghost towns, the weather  is cold and unpredictable, and any form of medical treatment is  nonexistent.

​

Working  in the ICU in Oregon, I have never dealt with altitude sickness or been  exposed to it. The little research I did about it before I left, I knew  that, at its very worst end stage, it evolved into cerebral and/or  pulmonary edema, both of which I am very familiar with in the ICU.

​

I walked  toward the sick man’s open room door. There were several people  gathered outside. He was lying flat on his bed, in his many layers. I  smiled and met his gaze through his half-opened eyelids.

​

I  immediately snapped back into my nurse mode, which had been sitting  dusty on the shelf for the past seven months. I sat next to him and  grabbed his hands. They were cold. As I asked him, “How are you  feeling?” I reached for his pulse. It was fast and irregular. He  responded with a few simple words in English but it was clear that he  didn’t speak much English. A woman we had been trekking with spoke  English and knew Chinese as well, which just so happened to be what this  man spoke. Through translation and looking through this man’s bag and  wallet, we discovered that he was Chinese, travelling all by himself,  without a guide, porter, or insurance. He had arrived in Lukla a day  after us and had arrived here in Dingboche three days ago. A trek of  1,760 meters or almost 6,000 vertical feet that had taken us five days  he had done in just two. The only medicinal treatment for altitude  sickness is Diamox, which I was able to get him to swallow before he  became extremely lethargic. But the only real treatment for mountain  sickness is to descend in altitude and fast.

​

I walked  outside his room to talk with the people who had gathered, including  the owner of the lodge. They were discussing what to do. Most were under  the impression that because he didn’t have any insurance or money,  there was nothing that could be done. That’s when the woman came out of  the room and said, “Can you come in here, please? He’s making this  weird, scary noise that I’ve never heard before.” My heart began to beat  faster as I quickly went back into the room and got the man into a  sitting position, maintaining his airway. He had developed flash  pulmonary edema. He was drowning in his own lungs, gurgling evident with  every breath.

​

My  take-charge critical care nurse hat had to be put on and I knew it. I  walked out of the room back to the group arguing outside. I interrupted  them and, as politely as I could, let them know that this man was going  to die, and soon, if he didn’t get down in elevation. He didn’t have  time to be carried down the mountain; it would take hours to get to the  next village and, even then, there were still no medical facilities. He  needed a helicopter evac stat.

​

Thankfully,  while I was in the room, another man fought very passionately to get  the owner of the lodge to finally call for the helicopter. It would come  from Lukla and would be here in 30 minutes. There was a slight glimmer  of hope. Okay, I thought, 30 minutes, that might be just enough time. My  heart plummeted when, five minutes later, the lodge owner came in and  said there were no helicopters in Lukla and rescue had to come from  Kathmandu, a two-hour flight. There was only one option: prayer. A sense  of urgency rushed over me as I showed the woman who had been  translating for us how to keep the Chinese man in an upright position in  order to maintain his airway. I hurried out the door and started toward  our lodge. I was on a mission.

“The wonder and mystery of prayer is God waits until someone asks.”—Billy Graham

​

“Prayer moves the arm of God.”—Doris Johnson

​

It was  time to gather the troops. As my team filed into the tiny room, I was  anxious about what God might do. I simultaneously felt anxious, nervous,  excited, and scared As we laid our hands on his limp body and began to  pray, a feeling of peace superseded all of those emotions. The next two  hours were the longest of my life. I was counting the minutes, giving  oxygen via a spray can-looking device that someone had found buried away  in a room, trying to keep this man conscious and breathing, and  constantly had two fingers on his pulse, waiting. Just waiting for it to  stop.

​

As a  nurse in general you must be a multitasker, but as a critical care  nurse, those skills must be even more fine-tuned. My nurse brain was on  overload. There was nothing left that I could do to help this man. All I  could do was keep him upright to prevent him from drowning in his own  lungs. I knew it would be horrendous if his heart stopped and I had to  perform CPR on this man, here, in the mountains all while having a crowd  of onlookers. I also knew that CPR would have no effect if I couldn’t  get an airway, which was impossible to do since his lungs were filled  with fluid.

​

After  what felt like an eternity, the lodge owner finally walked in and  announced that the helicopter would be here within minutes. Before I  even knew what was happening an old, tiny Sherpa had picked up the man  and put him on his back and began carrying him to the “landing pad,”  which is essentially a tiny pile of flat rocks on the edge of a cliff.

​

My  anxiety went into overdrive when I saw that the man was now lying flat  and face first on the man’s back. I knew that we had to get this man to  the helicopter, but I also knew that now all the fluid in his lungs  would obstruct his airway.

We  reached a large boulder next to the landing area and got the man into a  seated position again, with the help of several people, as he was now  dead weight. I quickly pushed away the blanket that was covering his  face and reached for his pulse again. Still fast and irregular. But I  could no longer hear his gurgling because he was barely breathing—yellow  fluid from his lungs was leaking from his mouth.

​

My mind  went back to the thought of having to perform CPR on this man. He was  barely holding on. I couldn’t believe he had made it this far, still  alive. As I gave him the rest of the oxygen that was left in the can, my  eyes were glued to his chest, watching it sporadically and faintly rise  and fall, waiting for it to stop at any second.

​

I have  never been happier to hear the sound of helicopter propellers in my  entire life. As we watched it fly toward us, my heart could start  beating again. It stopped for a brief second as the helicopter flew next  to us and then continued to fly on past us. We looked on in disbelief.  Someone quietly uttered the words we were all thinking: “Is that one not  for us?” In that instant, all my hope vanished. This man’s fate was  sealed. You know that saying that goes something like, “You wouldn’t be  able to fully appreciate joy if you didn’t know sorrow”? I felt the  utter despair of sorrow and the overwhelming joy of hope all in the same  10-second time period, as the helicopter began to make a wide right  turn, back toward us. Instant. Tears. That’s never happened to me  before. I didn’t know where they had come from or how they had gotten  there that fast, but I couldn’t stop them. This man was going to live.

​

The  helicopter was on the ground for no longer than two minutes. We picked  the man up and carried him the last 20 feet to the helicopter. I tried  not to trip over the huge, sharp rocks as I cradled his heavy,  dead-weight leg. The wind from the mountains combined with the  helicopter was overpowering as we approached the door. As soon as it  opened, my heart instantly sank. There was nothing inside, only a bench  seat. There was no medical equipment of any kind, no IV bags, no AED,  nothing. Not even a heart monitor. I doubt there was even a first aid  kit on this helicopter. In under 60 seconds he was up in the helicopter  and the doors were closed. The winds were even more fierce and we were  forced to turn our heads away from the force. I turned just in time to  see the helicopter disappear from my sight as it dove down in elevation  as quickly as it could, vanishing behind the cliff on which it had just  precariously landed.

​

I hadn’t  even taken two steps before the enemy attacked me again. You could have  done more. He won’t make it another two hours without medical  treatment; it was all in vain. Maybe if you had prayed a prayer with  more faith, God would have healed him.

​

I shared  my disappointment about the lack of medical equipment on the chopper  with the man with whom we had been trekking. And as matter-of-factly as  any medical professional should have known, he said, “Well, now he’s  going down in elevation and that’s the most important thing.”

​

I locked  myself in my tiny, cold lodge room. As I sat there on my single bed,  staring at the wall, trying to process all that had just taken place,  God stepped in, and I didn’t even have to ask Him to. A beautiful  assurance came over me as God let me know that the man was going to be  okay. He then allowed me to see the situation through His eyes, instead  of through the lens of the lies of the enemy. He saved this man’s life.  Just because it wasn’t through an instantaneous miracle, it didn’t make  it any less miraculous. All the pieces fell into perfect alignment as  every single person on that mountain that day played a role. An  English-speaking Chinese translator, an ICU nurse, and a team of prayer  warriors, all together in an almost abandoned village on the doorstep of  Mount Everest—God chose to use us to save this man’s life.

“Christ has no body now but yours. No hands, no feet on earth but yours.
Yours are the eyes through which he looks compassion on this world.
Yours are the feet with which he walks to do good.
Yours are the hands through which he blesses all the world.
Yours are the hands, yours are the feet, yours are the eyes, you are his body.
Christ has no body now on earth but yours.”—Teresa of Ávila

What a  grand and marvelous mystery that the God of the universe not only  allows, but deeply desires to include us in all His ways, in all His  adventures. His love truly is extravagant. I got an even bigger picture  of this grace-filled, awe-inspiring, just downright outrageous love two  days later when I finally stepped foot on Everest Base Camp.

​

We also  found out on our way down the mountain, that the Chinese man had made it  to a Kathmandu hospital alive and he was expected to make a full  recovery. s

Rachel  Johnston is 32 years old and has CF. She currently lives in Oregon with  her husband. She has traveled all over the world as part of various  mission trips. She came back to the United States in June 2019 and got  married in November 2021.

Jacob Greene is a 24-year-old medical student with cystic fibrosis and  is our newest member of the USACFA Board! Born in Tigard, Oregon, Jacob  was diagnosed with cystic fibrosis at birth when he was treated for  meconium ileus. He grew up in Seattle, Washington, Seattle Children’s  Hospital served as his home CF center throughout his childhood. In  junior high and high school, Jacob was involved with robotics, Boy  Scouts, his school’s track and field and wrestling teams, and did  medical research at Fred Hutchinson Cancer Research Center. These  experiences, primarily his lived experience with CF and work at Fred  Hutch, piqued Jacob’s interest in pursuing a career in medicine. In  2017, Jacob moved to California to start college at Stanford University.  At Stanford, Jacob did neurology research in the Monje and Gibson Labs.  This work led to Jacob being published in the journals Cell and Neuron.  Jacob also volunteered with Stanford Health Care’s Cardinal Free  Clinics and was a Residential Assistant in Roble Hall. In 2021, Jacob  graduated from Stanford with a Bachelor of Science with Honors in  Biology. Following graduation, Jacob worked at Genentech in the Research  and Early Development group working to develop drugs for neurological  diseases like Alzheimer’s and multiple sclerosis. This work led to a  publication in Glia. In July of 2022, Jacob left Genentech to begin  medical school at the University of California San Francisco (UCSF)  School of Medicine. At UCSF, Jacob is a co-coordinator of HealthLink—a  program for high school students interested in medical careers. He also  serves as the UCSF School of Medicine delegate to the Medical Student  Section of the American Medical Association, sits on the Student Medical  Education Council, and does research in the Jonathan Pan Lab. Jacob  joins the USACFA Board of Directors after having written for the CF  Roundtable publication and participating as a panelist in our  scholarship application webinar in December. We’re thrilled to have  Jacob on our board!

The  application deadline for the Higher Education (formerly the Lauren  Melissa Kelly) Scholarship is June 30, 2023. Any student seeking a  degree in higher education, from Associate to Ph.D., is welcome to  apply. We look for students who demonstrate tremendous academic  achievement, community involvement, and a powerful understanding of how  their CF—matched with these achievements—places them in a unique  situation to gain leadership roles within the community. We believe that  any higher education is a strong foundation for advocacy and  involvement in the CF community.

​

Nancy  Wech established this scholarship in honor of her daughter, Lauren  Melissa Kelly. This semester’s winners demonstrated outstanding  potential, just like Lauren years ago. Lauren was an inspiration to all  who knew her. An incredible leader and scholar, her drive and success  are the foundation of her memory. She was transformative in every aspect  of her life. She had distinguished herself as a member of the Golden  Key Honor Society, Mortar Board, Phi Upsilon Omicron, Gamma Beta Phi,  Delta Gamma sorority, and was chosen as one of ten Senior Leads at the  University of Georgia. She acted as one of the re-founding members of  the Phi Kappa Literary Society and was significant in the metamorphosis  of the Z Club into the William Tate Society. Although Lauren lost her  battle with cystic fibrosis late in her senior year, her hard work and  memory continue to live on through her inspiring involvement.

​

Two  scholarships are awarded each application cycle. More information,  including the application and relevant deadlines, can be found on our  website. For questions about future scholarships or anything related to  the application process, please contact us at scholarships@usacfa.org.

Cystic Fibrosis Clinical Trials | A Drug Pipeline Analysis Report 2023

Over 80+  Cystic Fibrosis pipeline therapies are in various stages of  development. Leading Cystic Fibrosis companies developing novel drug  candidates to improve the Cystic Fibrosis treatment landscape include  Algi Pharma, Verona Pharma, Translate Bio, Calithera Biosciences,  Krystal Biotech, SpliSense, and others. Promising Cystic Fibrosis  pipeline therapies in various stages of development include OligoG,  Ensifentrine, MRT5005, CB280, KB407, SPL84231, and others. Discover more  about the emerging Cystic Fibrosis drugs @ Cystic Fibrosis Treatment  Drugs. Find out more about the Cystic Fibrosis treatment options in  development @ Cystic Fibrosis Clinical Trials.

https://tinyurl.com/mw34dxhc

AND

https://tinyurl.com/yc2ae9e8

Cystic Fibrosis Life Expectancy: What To Know

The life  expectancy for people with cystic fibrosis (CF) has greatly improved  over time. Early diagnosis and treatment can improve both life  expectancy and quality of life for people with the condition. According  to the Cystic Fibrosis Foundation’s 2021 annual report, the median  predicted survival rate for people born in 2017–2021 is around 53 years.  This means that 50% of people born in those years are expected to reach  more than 53 years of age. It is important to remember that these  figures are estimates and are based on previous medical studies. Even  with improved treatments, there are factors that can affect the life  expectancy of people with CF. New medications are opening doors to  better long-term lung function. Effective treatments include:

Bronchodilators: These are medications that help open airways by relaxing the lungs’ muscles.

Airway  clearance techniques (ACTs): ACTs are techniques that include clapping  or huffing to help clear mucus buildup. In addition, nebulizers, which  are devices that turn liquid medications into an inhalable mist, can be  beneficial. Exercise machinery and vests that vibrate the chest may be  used as well.

Antibiotics: These medications can treat recurrent lung infections.

CFTR modulators: These are medications that can help prevent mucus buildup tied to the genetic mutations of CF.

Additional  treatment avenues that can improve life expectancy for people with CF  are lung and liver transplants in the event of severe organ damage.

https://tinyurl.com/55fncevh

Cystic  Fibrosis Chronicle: Why Has The Often-Deadly CF Gene Not Passed Out Of  The Human Genome? And What New Treatments Are Being Developed?

The  mutation that causes cystic fibrosis arose in the early Bronze Age and  spread across Europe during ancient migrations. Cystic fibrosis is the  most common fatal genetic disease in the US, and yet has genetic  characteristics that should hinder it’s spread or remove the mutation  from the gene pool altogether. We would expect natural selection to  eliminate alleles with negative effects from a population, and yet many  populations include individuals carrying such alleles. So why are these  deleterious alleles still around? What might keep natural selection from  getting rid of them? First the longer projected life expectancy means  that survivors have more of an opportunity to pass along these killer  mutations. Second, CF is a recessive genetic disorder; this means both  parents must carry one copy of the gene for a child to have a chance of  being born with the disorder and, even then, the risk is one in 4. In  most cases, these two characteristics would have limited the spread of  the deadly gene. However, in the case of CF, they created an  evolutionary niche that has allowed the gene to grow in the population  to the point where approximately 10 million Americans carry it. This is  partly due to the gene’s recessive nature: you can carry one copy  without exhibiting any symptoms of the disease. Only a genetic screening  will alert you to the presence of the GF gene in your DNA. This has  allowed the gene to ‘silently’ propagate though the population. Lastly,  it’s been proposed that carrying one copy of the gene for CF actually  conferred an evolutionary benefit to prevent people from dying of  tuberculosis and cholera. In other words, the negative effects of the  genes involved were counterbalanced by their positive evolutionary  contributions. This hypothesis is now regarded by many as the best  explanation for why such a lethal genetic disease became so common.

https://tinyurl.com/2kc7no84

CFTR Mutations Impair SARS-CoV-2 Virus, Study Finds

CFTR  gene mutations significantly reduce the cell entry and replication of  SARS-CoV-2, the virus responsible for COVID-19. The research team noted  that several studies have suggested that incidence of severe COVID-19 in  CF patients is lower than what was expected. Because of this, the team  set out to uncover the cellular mechanisms that could explain this  situation. The results suggest that CF may hamper the cytokine release  syndrome triggered by SARS-CoV-2 S protein stimulation in airway  epithelia, thus strengthening the hypothesis that CF may constitute a  biological advantage by decreasing the risk of developing unfavorable  COVID-19 outcomes.

https://tinyurl.com/4j2x36ys

AND

https://tinyurl.com/2ehwxt9t

AI Model Can Help Detect Collapsed Lung Using Chest X-rays

A new  study shows that an artificial intelligence (AI) model can accurately  detect simple and tension pneumothorax on chest radiographs. A  pneumothorax is a collapsed lung that occurs when air leaks into the  space between the lung and chest wall. This air pushes on the outside of  the lung and makes it collapse either wholly or partially. The study  noted that early detection of pneumothorax is crucial, as the severity  of the condition will determine the need for emergency intervention. A  pneumothorax is typically detected and diagnosed using a chest X-ray and  radiologist interpretation, but the authors hypothesized that AI could  help improve this process. The researchers explained that first, AI  could assist in triaging chest radiographs for sooner interpretation by a  radiologist based on the suspected presence of a pneumothorax. Second,  it could provide a second ‘set of eyes’ to support identification of a  pneumothorax. The findings suggest that the ability to accurately detect  and rapidly triage pneumothorax with an AI model could assist with  earlier identification and improve patient care if integrated into the  clinical workflow.

https://tinyurl.com/4x42cx66

High-Dose Vitamin D Therapy Successful For Patients With Cystic Fibrosis

Vitamin D  levels in patients with a deficiency and cystic fibrosis increased with  a single high dose, or stoss therapy. Vitamin D deficiency can lead to  poor bone health (osteomalacia) and has been associated with worsening  lung disease in persons with cystic fibrosis (CF). Despite this  knowledge and prescription of daily vitamin D supplementation for  persons with CF, vitamin D deficiency has remained common in adult and  pediatric cystic fibrosis clinics. Investigators found that stoss  vitamin D dosing can be successfully implemented in routine cystic  fibrosis care and increases vitamin D levels in and vitamin D  deficiency.

https://tinyurl.com/3ekwrt9b

Bisphosphonates For Osteoporosis In People With Cystic Fibrosis

Around  23.5% of people with CF experience reduced bone mineral density (BMD),  commonly known as osteoporosis, which increases the likelihood of bone  fractures. The short-term and long-term effects of fractures (e.g. ribs  and in the spine) may make lung disease worse, and hospitalization more  frequent. Bisphosphonates are drugs that increase BMD by slowing down  how fast bone is resorbed. This current review of the literature found  that bisphosphonates consistently increased BMD in adults at the lumbar  spine and hip regions. Treatment with bisphosphonates did not appear to  reduce the rates of fractures (either in the spine or elsewhere) or  deaths in adults. Severe bone pain and flu-like symptoms were commonly  linked to intravenous bisphosphonates, especially in people not using  corticosteroids. The reviewers concluded that additional trials are  needed to determine if bone pain is more common or severe (or both) with  the stronger drug zoledronate and if corticosteroids lessen or prevent  these adverse events. Additional trials should also assess  gastrointestinal adverse effects in the stomach and digestive tract  which are linked to oral bisphosphonates. Trials with larger numbers of  participants with longer follow-up are needed to show how  bisphosphonates affect fracture rate and survival.

https://tinyurl.com/ybbwh8pc

Position Paper: Models Of Post-Transplant Care For Individuals With Cystic Fibrosis

There is  no consensus on the best model of care for individuals with CF to  manage the non-pulmonary complications that persist after lung  transplant. The CF Foundation virtually convened a group of  international experts in CF and lung-transplant care. The committee  reviewed literature and shared the post-lung transplant model of care  practiced by their programs. The committee then developed a survey that  was distributed internationally to both the clinical and individual with  CF/family audiences to determine the strengths, weaknesses, and  preferences for various models of transplant care. Discussion generated  two models to accomplish optimal CF care after transplant. The first  model incorporates the CF team into care and proposes delineation of  responsibilities for the CF and transplant teams. This model is reliant  on outstanding communication between the teams, while leveraging the  expertise of the CF team for management of the non-pulmonary  manifestations of CF. The transplant team manages all aspects of the  transplant, including pulmonary concerns and management of  immunosuppression. The second model consolidates care in one center and  may be more practical for transplant programs that have expertise  managing CF and have access to CF multidisciplinary care team members  (e.g., located in the same institution). The best model for each program  is influenced by several factors and model selection needs to be  decided between the transplant and the CF center and may vary from  center to center. In either model, CF lung transplant recipients require  a clear delineation of the roles and responsibilities of their  providers and mechanisms for effective communication.

https://tinyurl.com/ywk4cad8

Living Near Composting Sites May Increase Risk Of Worse CF For Adults

Living  closer to composting sites, where solid urban waste is collected and  stored, may increase the risk of more severe disease for adults with  cystic fibrosis (CF). Close proximity to such sites was linked with  worse lung function and more fungal infections among adults. The study  also noted a higher risk of pulmonary exacerbations, or periods of  sudden symptom worsening, in this patient population. Notably, no impact  on lung function was seen for children living within or beyond about  2.5 miles of composting sites. If further research confirms the present  study’s results, the associations identified could have important  implications on i) the living environments of pwCF, ii) clinical advice  to pwCF; iii) public health advice provided to vulnerable populations  living near permitted composting sites (PCS); and iv) how PCS are  regulated and permitted.

https://tinyurl.com/2q5bwbhs

The Clinical Association Between Aspergillus Fumigatus And Respiratory Outcomes In Adolescents And Adults With Cystic Fibrosis

Positive  Aspergillus fumigatus (Af) culture was not associated with lower  patient-reported respiratory-related quality of life (QOL). Yet,  positive Af culture was associated with both lower FEV1 percent  predicted and increased frequency of severe pulmonary exacerbations  (PEx) warranting intravenous antibiotics in adolescents and adults with  CF. Future studies are required to better understand the direct role of  Af in lung disease progression in CF.

https://tinyurl.com/2eyj3knd

Airway Bacterial Community Composition In Persons With Advanced Cystic Fibrosis Lung Disease

The  progression of lung disease in people with cystic fibrosis (pwCF) has  been associated with a decrease in the diversity of airway bacterial  communities. The absence of a dominant genus, presence of  methicillin-susceptible Staphylococcus aureus, and greater bacterial  richness positively correlated with lung function. Higher relative  abundance of the dominant genus and greater antimicrobial use negatively  correlated with lung function. PwCF with a low diversity community and  dominant genus had reduced lung transplant-free survival compared to  those without. In summary, a considerable proportion of pwCF with  advanced lung disease do not have airway bacterial communities  characterized by low diversity and a dominant genus and these  individuals had better survival. An understanding of the antecedents of  low diversity airway communities– and the impact these may have on lung  disease trajectory - may provide avenues for improved management  strategies.

https://tinyurl.com/yn5kkmts

AND

https://tinyurl.com/2ol9wfwt

Diabetes Is Associated With Increased Burden Of Gastrointestinal Symptoms In Adults With Cystic Fibrosis

Individuals  with CFRD overall, have a higher gastrointestinal (GI) symptom burden,  according to CF-specific GI symptom questionnaire CFAbd-Scores. The  CFAbd-Score total score (0–100pts), its 5 domains, alongside nine  specific GI symptoms associated with diabetes mellitus (DM), were  compared between CFRD and non-CFRD groups. Total CFAbd-Score and the two  domains: gastroesophageal reflux disease and disorders of appetite were  significantly higher in the CFRD group compared to the non-CFRD group.  Among the nine GI symptoms commonly reported as elevated in DM, bloating  and nausea were significantly more common in individuals with CFRD  compared to those without.

https://tinyurl.com/34upuzer

AND

https://tinyurl.com/2va6j58h

AND

https://tinyurl.com/2kkkta8n

Giving CGM Access To All People With Type 2 And Other Diabetes

People  with cystic fibrosis-related diabetes (CFRD) require careful management  of blood sugar levels to avoid complications such as heart disease,  kidney disease, and nerve damage. There is evidence that lack of glucose  control worsens lung function and that fast blood glucose tests are not  effective in identifying glucose variability. One of the greatest  challenges when addressing CFRD is initially screening for diabetes. The  CF Foundation currently recommends an oral glucose tolerance test  (OGTT) for people with cystic fibrosis over 10 years old. However, rates  of annual OGTT testing in adults are very low – only 30% of adults with  CF were screened for diabetes. Continuous glucose monitors (CGM) may  potentially be an effective tool for expanded screening. CGM may also be  helpful in the ongoing management of CFRD, both with and without AID  (automated insulin delivery) technology. The limited data that exists  suggest that use of a CGM can make the diagnosis of diabetes earlier,  improve outcomes and motivate people to make healthier choices in diet  and lifestyle, no matter if they are taking insulin or not.

https://tinyurl.com/mry8veb6

Drug-Drug Interactions With CFTR Modulator Therapy In Cystic Fibrosis: Focus On Trikafta®/Kaftrio®

The  combination of CFTR modulators ivacaftor, tezacaftor and elexacaftor  (Trikafta®, Kaftrio®) significantly improve outcomes, including survival  in a broad range of cystic fibrosis patients. These drugs have  complicated metabolic profiles that make the potential for drug  interactions an important consideration for prescribers, care providers  and patients. Prolonged survival also increases risk of age-related  disease and their associated pharmacotherapy, further increasing the  risk of drug interactions and the need for increased vigilance amongst  care providers. The authors systematically searched the literature for  studies identifying and evaluating pharmacokinetic and pharmacodynamic  drug interactions involving the components of Trikafta®/Kaftrio®. They   also searched electronic databases of drugs for possible drug  interactions based on metabolic profiles. 86 potential drug interactions  were identified, of which 13 were supported by 14 studies. There is a  significant need for research to describe the likelihood, magnitude and  clinical impact of drug interactions.

https://tinyurl.com/4hszfb7p

Effect Of Elexacaftor/Tezacaftor/Ivacaftor On Annual Rate Of Lung Function Decline In People With Cystic Fibrosis

Elexacaftor/tezacaftor/ivacaftor  (ELX/TEZ/IVA) was shown to be safe and efficacious in people with  cystic fibrosis (CF) with ≥ 1 F508del-CFTR allele in Phase 3 clinical  trials. ELX/TEZ/IVA treatment led to improved lung function, with  increases in percent predicted forced expiratory volume in 1 second  (ppFEV1) and Cystic Fibrosis Questionnaire-Revised respiratory domain  score. Here, the impact of ELX/TEZ/IVA on the rate of lung function  decline over time  was evaluated by comparing changes in ppFEV1 in  participants from the Phase 3 trials with a matched group of people with  CF from the US Cystic Fibrosis Foundation Patient Registry not eligible  for cystic fibrosis transmembrane conductance regulator (CFTR)  modulator therapy. Participants treated with ELX/TEZ/IVA had on average  no loss of pulmonary function over a 2-year period. ELX/TEZ/IVA is the  first CFTR modulator therapy shown to halt lung function decline over an  extended time period.

https://tinyurl.com/27rszwuf

Lived  Experiences Of People With Cystic Fibrosis That Were Not Eligible For  Elexacaftor-Tezacaftor-Ivacaftor (ETI): A Qualitative Study

Elexacaftor-tezacaftor-ivacaftor  (ETI) represents a significant step forward in cystic fibrosis (CF)  care and could change the course of CF lung disease and quality of life  for many people with CF (PwCF). However, several PwCF cannot benefit  from these modulators because their rare mutations are not eligible for  treatment. This study aimed to investigate the lived experiences of PwCF  who are not eligible for ETI. Data were collected through  semi-structured interviews. The investigators found that PwCF who are  not eligible for ETI experience intense disappointment and conflicting  emotions that can influence their decision-making linked to  diminishing/renewal hope. Integrated care, including mental health  monitoring programs, should be provided to these patients to aid them in  overcoming their disappointment and to improve their coping.

https://tinyurl.com/2jfdadjs

Elexacaftor/Tezacaftor/Ivacaftor  Projected Survival And Long-Term Health Outcomes In People With Cystic  Fibrosis Homozygous For F508del

A series  of phase 3 clinical trials have demonstrated that elexacaftor plus  tezacaftor plus ivacaftor (ELX/TEZ/IVA) is safe and efficacious in  people with cystic fibrosis (pwCF). The impact of this treatment on  lifetime clinical outcomes and survival, however, has yet to be  assessed. The researchers used a person-level microsimulation model to  estimate the survival and lifetime clinical benefits of ELX/TEZ/IVA  treatment versus other CFTR modulator combinations (tezacaftor plus  ivacaftor [TEZ/IVA] or lumacaftor plus ivacaftor [LUM/IVA]) or best  supportive care (BSC) alone in pwCF. The median projected survival for  pwCF homozygous for F508del-CFTR treated with ELX/TEZ/IVA was 71.6  years. This was an increase of 23.2 years versus TEZ/IVA, 26.2 years  versus LUM/IVA, and 33.5 years versus BSC alone. Treatment with  ELX/TEZ/IVA also reduced disease severity as well as the number of  pulmonary exacerbations and lung transplants.

https://tinyurl.com/ye2x4nf3

Long-Term  Tezacaftor/Ivacaftor Safety And Efficacy In People With Cystic Fibrosis  And An F508del-CFTR Mutation: 96-Week, Open-Label Extension Of The  EXTEND Trial

Study  661-110 (EXTEND) is a phase 3, open-label, three-part rollover study  designed to assess the long-term safety and efficacy of  tezacaftor/ivacaftor (TEZ/IVA) in participants aged ≥12 years homozygous  for F508del (F/F) or heterozygous for F508del and a residual function  mutation (F/RF). TEZ/IVA was shown to be safe and efficacious for up to  120 weeks in Part A. Results from Part B, which evaluated safety and  efficacy for an additional 96 weeks found that TEZ/IVA was generally  safe and well tolerated over a further 96 weeks; safety data were  consistent with Part A. Improvements in ppFEV1 and pulmonary  exacerbation rates were maintained for an additional 96 weeks in Part B.  The most common adverse events, which were generally consistent with  common manifestations of CF, included infective pulmonary exacerbation  of CF, cough, nasopharyngitis, hemoptysis, and headache. Lung function  was maintained over 96 weeks in both genotype groups.

https://tinyurl.com/bdznxdbw

CFTR Modulators Safe While Pregnant, Breastfeeding: Case Series

Data on  the safety profile of CFTR modulators to guide patients during pregnancy  is still scarce. The Phase 3 trials that supported their approval have  excluded pregnant women with CF. Also, recent studies suggest the  therapies can cross the placenta and reach the fetus’ blood circulation.  However, treatment with CFTR modulators was safe during pregnancy and  breastfeeding in two women with cystic fibrosis (CF). Despite being  advised to stop treatment, both women continued on their CFTR modulator  during pregnancy and breastfeeding without any safety concerns.  Continuation of CFTR modulators during pregnancy and lactation requires  careful multidisciplinary considerations and patient discussion  regarding risk and benefit.

https://tinyurl.com/3rxk55bj

CFTR Modulators Found To Improve Antibiotic Efficacy In CF: Study

Despite  their established clinical benefits, response to CFTR modulators can  vary from patient to patient, suggesting other factors may influence the  effectiveness of modulators. Because bacterial infections commonly  occur in CF lungs, the impact of CFTR modulators on lung bacteria also  may impact their efficacy. To find out, a team of scientists tested the  effects of CFTR modulators, either alone or combined with antibiotics,  on bacteria samples isolated from CF lungs. The CFTR modulators tested  included ivacaftor (sold as Kalydeco), lumacaftor, approved in  combination with ivacaftor as Orkambi, and tezacaftor, sold in  combination with ivacaftor as Symdeko. Elexacaftor, combined with  tezacaftor and ivacaftor as the triple combination therapy Trikafta, and  Trikafta itself, also were assessed. Samples of two bacteria commonly  found in CF airways — Staphylococcus aureus and Pseudomonas aeruginosa —  were collected from CF patients at the early stages of colonization,  and again at later stages when the bacteria had adapted to the CF lung  environment. The bacteria were then exposed to increasing levels of CFTR  modulators. Antibacterial activity was measured by the lowest  concentration of medicine that effectively suppressed bacterial growth,  called the minimum inhibitory concentration, or MIC. Lower MIC values  indicated more potent antibacterial activity. Results revealed that  ivacaftor had the most potent antibacterial activity for all the S.  aureus samples. This was followed by elexacaftor. Lumacaftor and  tezacaftor showed little or no signs of antibacterial activity. Triple  combination therapy elexacaftor/tezacaftor/ivacaftor (ETI) also showed  strong antibacterial activity. By contrast, all CFTR modulators showed  little or no activity against P. aeruginosa samples. Next, the  researchers mixed CFTR modulators with antibiotics to measure potential  additive or synergistic effects. Ivacaftor enhanced the activity of the  antibiotic linezolid in most S. aureus samples while enhancing  amoxicillin, vancomycin, and teicoplanin on a more limited scope.  Lumacaftor and ivacaftor both enhanced vancomycin and teicoplanin  against S. aureus. Even though CFTR modulators showed no activity  against P. aeruginosa on their own, ivacaftor strongly enhanced the  activities of colistin and polymyxin B, against more than 95% of the P.  aeruginosa samples. Triple combination ETI showed an additive effect on  the activities of colistin and polymyxin B in several P. aeruginosa  samples.

https://tinyurl.com/yc4xp3v9 s

Laura  Tillman is 75 years old and has CF. She is a former director and  President of USACFA. She and her husband, Lew, live in Northville, MI.