Three  years ago, at the age of 27, I found myself hospitalized for a week in  the Progressive Care Unit in Wilmington, North Carolina. I was battling  the most severe bout of pneumonia I’d ever contracted with a total lung  function around 43% at the time. All of this happened during the height  of the COVID-19 pandemic and made for a very anxious and uncertain time.  Thankfully, in the weeks that followed, I was able to meet my new CF  team for the first time since my move to NC. During that first visit, I  was able to learn more about Trikafta and the incredible results that  were being reported from this miracle drug. It seemed too good to be  true; could I really get my health and life back fully? I was excited  and nervous when that first little box of pills arrived. I recall  thinking, what if this doesn’t work for me? Or perhaps the bigger  question, what if it does? What will I do then?

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After a  long journey of ups, downs, and building back my body and mind, I wanted  to push my limits and see what was possible with my newfound health.  Where is that prohibitive line at now for my body? After much training  and reaching several other lofty goals, I decided to attempt my first  ultramarathon run. An ultramarathon, for those who don’t know, is any  run longer than the standard 26.2-mile marathon. Currently only about  .03% of the US population has ever run one. My story isn’t written to  glorify or bring recognition to myself, but rather to bring awareness to  and highlight the endless possibilities for folks with CF when they  have access to the proper care and life-changing modulators. Hopefully,  the day comes soon when CF will indeed stand for Cure Found for all of  us.

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“We  can’t afford the time to sit here and wait for a chainsaw. We’ll have to  turn around and find a different route,” I stated as I hopped back into  the pickup truck, water dripping off my jacket as I sat down. It was  6:00 a.m. on a rainy Saturday morning in the mountains of North  Carolina, just a bit east of Asheville. My two lifelong and faithful  friends and comrades and I were navigating the winding mountain roads as  we headed to our first ultramarathon event. We had carefully planned  out our time for this morning to ensure early arrival at the starting  line that would allow ample time to stretch and mentally prepare  ourselves for the task at hand.

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As luck  would have it, though, here we were halfway to our destination and there  was a large tree sprawled across the entire roadway. Apparently, it had  been uprooted during the early morning hours by a quick, but fierce,  storm. My mind began to fill with negative thoughts and questions as we  drove back up the mountain, scouring Apple Maps for an alternate route.  What if I’d done all these months of hard training just to arrive late  and get disqualified before even leaving the starting line? Or perhaps, I  thought, what if this is a good thing and the universe is just saving  me from failing miserably and making a fool of myself? After all, the  longest race I’d run prior to this was only a half marathon and today’s  scheduled run was 35 miles.

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“Turn  right here,” I said, as our GPS guided us along this new route. “Time of  arrival is 7:30 am, so we’ll only be a half-hour late and fingers  crossed the director will be understanding and still allow us to run.” I  laid my head back against the headrest and dozed off for several  minutes. At exactly 7:30 a.m., we arrived at the now mostly deserted  race headquarters and sought out the director. After a brief explanation  of our events of the morning and expressing our eagerness to race, the  director graciously agreed. He laid down some quick ground rules and  directions and we hit the trail. Soon we were dodging puddles and  settling into our pace. What proceeded will forever be one of the most  memorable days of my life.

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The  first five miles of the run passed by quickly and easily. I was soaked  to the bone from the continued rainfall but I didn’t mind a bit. I  increased the volume to my music and got lost in the rhythm as I matched  my pace to the upbeat tempo vibrating in my ears. I smiled at the  beauty surrounding me and thought to myself, “I must be the luckiest  person alive to be able to be here in this moment and be healthy enough  to even attempt such a thing.” The enormous oak trees, moss-covered  boulders, and peaceful river grabbed my attention for a moment. I  reveled in the tranquility and then my mind wandered off again.

“This  view is incredible,” I said out loud, as I stopped for a moment to catch  my breath and stretch my calves, which were burning intensely by this  point. I had just ascended about 2,800 feet in the last three miles and  my body was letting me know. When I gazed out across the vast expanse  below me, I could see turkey vultures gliding in the wind. Fog rose out  of the valleys and created a giant curtain, mysteriously hiding the  peaks from view as if waiting for a stage to be perfectly set and  everyone in their places before revealing all its beauty. After some H2O  and energy gel, I continued the journey. The next 12 miles were filled  with more amazement at the endless beauty, a stop to shed some clothing  as the sun made an appearance and several stops at aid stations to fill  my water bladder and refuel on food (candy, pickles, and pancakes).

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“19.6  miles completed, now it gets real,” I huffed to my friend Mike, as I  checked the tracker on my phone. I’d finally caught up with him and we  were beginning another long ascent. We would gain roughly 3,000 vertical  feet over the next four miles. It was at this point that my body and  mind screamed out to quit and simply did not want to go on. But I pushed  forward—up and up. The sun kissed my skin and crystals of salt built up  on my face and arms.

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Around  mile 29, I left the last aid station refreshed after being baptized with  a bucket full of cold creek water and some Oreo cookies in my stomach.  The combination of sugar and adrenaline gave me a second burst of energy  and my body switched into cruise control for those last six miles. When  I approached the end of the run, a tsunami of mixed emotions washed  over me. On the one hand, I was completely ecstatic because I realized  I’d come this far and was actually going to complete this monumental  run. All the months of discipline and training had indeed paid off. The  overwhelming sense of accomplishment made me beam with pride and smile  like a kid as I did when I had just caught my first largemouth bass. My  whole life and journey with CF flashed before me like spools of footage  from an old projector: all the clinic visits, the hospital stays, and  the walk-a-thons. I then pictured each and everyone’s face—others with  CF—that I’d known throughout my life who had already passed. My eyes  welled up with tears causing my vision to blur. Why me? Why was I  allowed to live long enough to receive the new miracle drug? Why was I  lucky enough to have the most common CF mutation, meaning I was one of  the first to have access to these drugs? Why me instead of Eva, or  Jacob, or Danny—or the many other souls that also deserved a chance at a  good life? I dwelled on these heavy thoughts for that last hour, which  inevitably made that part of the trail zip by quickly, even though my  pace had slowed significantly from the first half of the day.

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The  beginning and end of the race was at a camp situated in a “holler,” as  they’d say here in the South. There were mountains on both the east and  west side, and the long narrow draw ran north and south. As I approached  the finish line, I saw the sun start slipping behind the ridge and I  could hear cheers and clapping echoing through the valley. I crossed  that line with an exhausted body and mind, but a heart that was  overflowing with gratefulness—for life, for health, for family and  friends. I was grateful for my two best friends who were by my side that  day, for the countless messages of encouragement that flashed on my  phone screen while I ran. I was grateful for my amazing CF team at Duke  University and for the miracle drug that had given me my life back along  with the ability to live a much more fulfilled physical life than I  ever could have imagined.

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***

I feel  all the gratitude in the world to be able to have lived this experience  and to write about it to all of you. However, I also feel so terribly  unworthy to share this, while there are still so many in the CF  community without access to modulators or who have debilitating effects  from the same drugs that are meant to improve our lives. For all of us  who have been fortunate enough to have success with modulators,  survivor’s guilt can be a truly complex web of emotion to untangle. For  me, though, the anguish that comes from knowing I have been given the  gift of health and not having done more than living in mere mediocrity,  becomes a much greater burden to bear.

So, as I  continue to challenge myself and push my body to new heights and  greater successes, I will do so with righteous vigor, intensity, and  purpose in part to prove what can be accomplished, yes, but also as a  tribute to honor those who never could so that in some small way their  lives and memory will live on and never be forgotten.

Marcus  Miller is 31 years old and has CF. He lives outside Wilmington, North  Carolina, about 30 miles from the Atlantic Coast. He has the best pup in  the world, a Siberian Husky, named Emma and she accompanies him on most  of his adventures. His true passions in life are hunting, archery,  running/fitness, hiking and camping, and basically anything that gets  him out in nature. If you’d like to follow his adventures or reach out  to him, you can find him on IG @marcusrmiller or send him an email at extrasaltyrunner@gmail.com.

In  the past three months, CF Roundtable readers have sent many questions  related to eligibility criteria for Social Security Disability or SSI  benefits.

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The  information provided in this article is only meant to be general  information. Nothing in this article is meant to be a guarantee that a  person will be eligible for SSDI, SSI, or any other government program.

Question 1:

Once Social Security approves my application for disability benefits, will I remain eligible for the rest of my life?

Answer:

No.  Social Security rules now require that a person be reviewed every three  to five years if the person receives Social Security Disability  Insurance (SSDI), SSI benefits, or Social Security benefits based on the  work record of a parent who is deceased, retired, or receiving SSDI  themselves. Social Security sends Continuing Disability Review (CDR)  paperwork in the mail. Also, if Social Security has evidence of work  activity that is more than the allowable amount, they may send a CDR.  CDR paperwork is very important. The paperwork may look like it is easy  to fill out, but answers given to the questions often lead to  termination of benefits. Often the person completing the forms does not  understand the questions and gives answers that are vague or unclear.

Many  people with CF are losing benefits because they do not think the CDR is  important and mistakenly think they will have benefits for life. A  person can contact the CF Legal Information Hotline if they have  questions about Social Security paperwork or a CDR.

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Question 2:

I have  had Social Security disability benefits since I was three years old.  Does Social Security review eligibility once I turn 18 years old?

Answer:

Yes.  Social Security must review anyone who has been receiving SSI benefits  once the person reaches 18 years of age even if a person has received  SSI benefits throughout their childhood. Social Security may stop  benefits after an 18-year-old review if the person no longer meets the  medical criteria or there is no evidence to support a finding that the  person cannot work more than 20 hours a week and make more than $1,470 a  month (before taxes are taken out of the work earnings) from work  activity or $1,050 a month from self-employment. The length of time a  person has been eligible for Social Security benefits in the past is not  a reason to have benefits continued.

Question 3:

Will Social Security consider me to be disabled as long as I have a diagnosis of cystic fibrosis?

Answer:

No.  “Diagnosis” and “disability” mean different things. Diagnosis is a  medical determination that a condition exists or is the cause of  symptoms. Disability for adults under Social Security rules means the  individual is incapable of substantial gainful employment as a result of  one or more medical conditions that limit the person’s ability to work.  For children the standard relates to the child’s ability to engage in  age-appropriate activities on a daily basis. Social Security considers a  person disabled if the signs and symptoms of the condition meet the  degree of severity listed in its regulations or is equivalent in  severity. The regulations set out specific symptoms and the degree of  severity that the medical evidence must show. This is called the Social  Security Listing and can be found in the Social Security Blue Book at www.ssa.gov.  In addition to showing a person meets one of the listed medical  criteria, a person must show daily limitations that prevent the person  from working more than the allowable amount. A person can also be  eligible for benefits if the person’s medical condition equals or is as  severe as the Social Security Medical Listing criteria.

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Question 4:

Social  Security stopped my SSI benefits because it said my household income is  over the limit, but my monthly income has not changed. The only change  is that I recently got married.

Answer:

SSI has  eligibility criteria based on household income and resources. If a  person is over 18 years of age, the parents’ income and assets will no  longer be counted when determining SSI eligibility. However, if the  person who receives SSI benefits gets married, then the new spouse’s  work income and assets will be counted by Social Security.

If the  spouse’s assets put the person with the disability over the SSI asset  limit, then SSI benefits stop. If the spouse works, then the spouse’s  monthly income has to be below a certain amount; otherwise the SSI  stops.

SSDI eligibility standards do not consider a spouse’s assets or work income to determine eligibility for SSDI.

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Question 5:

Social  Security says my work income exceeds the limits, but my take-home pay is  less than the limit listed on the Social Security website. What amount  is counted to determine income?

Answer:

When  Social Security refers to income, it typically means gross income before  the subtracting of taxes, withholdings, or other deductions.

There  are many other questions about Social Security, both basic and advanced.  However, these few questions should help people with CF who review  Social Security befits understand some fundamental principles regarding  eligibility.

If you  have questions about laws related to Social Security benefits, Medicaid,  Medicare, health insurance, employment, or 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 generously funded by the CF Foundation,  but CFLIH employees are not employed by the CF Foundation. The CFLIH is  now in its 25th year.

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.

In  2009, I wrote an extra Spirit Medicine article during a flurry of  inspiration. I was 37 years old and wrote a list of the ways we leave a  legacy. It felt premature to publish, and alas, that was about five  laptops ago so I cannot find that document. Now, at 51, I’d like to  rehash this idea of legacy.

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A legacy  is often brought up at the end of life. But, of course, it is not  something we necessarily complete at the end of our lives. Every day, we  live out our legacies. It is the containment of our mark on the world.  Some are divinely ordained; callings that arise within us to pursue our  purpose. Some are actions and choices that add up to create a tapestry  of our lives. This is usually a tapestry of contentment, satisfaction,  and fulfillment—making us feel good about the life we’ve lived. Legacy  is how we make our imprint on the world. It is the ripple effect of our  deeds that is everlasting and outlives our short lives. Some can say our  legacies are promises to God to live the best life we can live in the  time we have.

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If we  look closely, there are legacies all around us. The library is filled  with stories, which are the legacies of peoples’ lives or imaginations.  The clay granaries hidden in the cliffs of the Southwest are the  legacies of the ancient people who survived in such harsh conditions.  The statues, plaques, museum exhibits, films, works of music, art, and  athletic prowess are also types of legacies.

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I thought I’d simply list a few types of legacies, so perhaps you can recognize the ways you are manifesting yours.

• A love  story of mythic proportions is one profound legacy. One that comes to  mind is the long marriage of Paul and Kathy Russell, leaders of the  USACFA organization for decades. I still remember them being glued at  the hip at CFRI conferences, laughing in synchronicity and telling  endless stories of life with CF back in the day. Theirs was a love story  that I believe helped Kathy live for as long as she did. Godspeed, dear  Kathy. Thank you for making a difference for our community. The legacy  of love always has a ripple effect.

•  Becoming a caregiver is another way to leave a legacy. Many of us make  life better for a four-legged animal or other vulnerable beings. For  some, this means impacting the life of a child. In my 20s my impact was  through being a mentor for my CF camp kid. Now I get to be an aunt to  three beautiful nieces. Many of my CF friends are raising their own  children and some are even grandparents. Parents get to pass on their  ethics, values, and teachings about what’s important to live a  meaningful life. Maybe becoming a parent is too much of a commitment  given health demands. You can still become a mentor to a child who needs  a guide in life. Boys and Girls clubs or the Scouts are eager for  teachers who show they care.

•  Becoming a specialist or expert in something you are passionate about  offers a rich legacy: an academic expertise, fixing motorcycles, running  marathons, being in a rock band, coaching your daughter’s basketball  game, or just following your bliss like playing the bagpipes. Isn’t this  why we are here? Just to take life and play with it? It sure balances  out all the messiness.

• A  professional legacy is the chance to contribute your creative energy to  something bigger than yourself—an entrepreneurial business, a health  care company, an environmental cause, a political or social cause, etc.  You can make a difference for good in this large world of ours. And we  have a choice: Our passion may have nothing to do with CF; just  something we love! And sometimes this legacy contributes to the security  of your financial legacy as well.

• People  with CF have a unique form of legacy by paying it forward for the next  generation through DEI awareness, clinical trials, or organ donation  awareness. Our voices raise awareness of these causes to directly impact  our community’s longevity. It can mean being bold and putting your CF  story out there; knowing that stories have the power to invite  connection, empathy, open-mindedness, self-discovery, and courage. Our  creative sharing has potential to be helpful to others, as evidenced by a  recent CFRI Writing to Heal group where writers told their stories with  such authenticity and insight. Just an aside, I have been writing  Spirit Medicine since 2007, following the footsteps of the late Lisa  McDonnell and Catherine Martinet. I remember Rich DeNagel making fun of  me, saying, “You know, whoever writes that column dies!” And I replied,  “Yes, whoever writes any column dies, Rich. And besides, this will be  part of my legacy.”

• A  spiritual legacy is another form of legacy. As a writer of this column  I’ve tried to write generally about spirituality to make the themes as  relevant and non-dogmatic as possible. Truthfully, my faith is defined  by God as love and my charge to be the loving being Jesus and God want  me to be. To me, a spiritual imprint is what you leave behind in terms  of your spiritual principles and beliefs for others to learn from.  Perhaps you’ve learned them from your family or cultural or ethnic  heritage. Have you incorporated spiritual habits and practices into your  life and your family’s? Do you have a lasting, trusting, relationship  with a higher power? Perhaps you have close friends or community who  have helped you develop your spiritual legacy as well. Are you at peace  with the path you’ve chosen for your spiritual maturity? Perhaps I will  be rewarded by my faith; perhaps not. Still, there can be comfort  knowing that a spiritual legacy is the treasure chest we invest in which  gives us security when we reach the end of this life and enter the  “next place.”

•  Finally, there are emotional and social legacies we leave behind. For  all my life in the CF community, I’ve been touched by observing the  emotional and social legacies of my peers during their adventures with  CF. When I think of social and emotional legacies, I think of these  questions: Over the course of our relationships with others, how have we  made people feel during our intersections with them? What did we bring  into our friends’ presence by walking into the room? Were we capable of  forgiveness, communicating conflicts, listening, and giving and  receiving support? Our energy can leave an imprint in the lives of those  we love. Our social circles can be enriched by knowing and loving us.  Just by living the best life we can live with a serious illness, we  convey virtues and principles about the sacredness and preciousness of  loving, and emotionally deep relationships with others. For some, it  takes a lifetime to learn of the sustaining force of connection.  Instead, CF has been the urgent teacher to get us to love each other!

Truthfully,  the end of my life is where legacy becomes most relevant. I’d like to  believe my spiritual legacy is a reflection from so many CF mentors: We  fought the good fight. We did our very best. We did not become  embittered. We chose gratitude. We cherished appreciation. We learned to  love ourselves. Our spirits did not break with the hardships of this  disease.

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A major  legacy in the CF community has been the capacity to face fear head on  and still maintain an optimistic attitude towards life. I’ve done my  best to parry the blows of the CF drama without being battered by them.  Through suffering and pain we can still hold on to values like kindness,  compassion, or non-harm. An ideal legacy is a life lived with no  regrets. I could ask for no better community, no more life-affirming and  evolved group of friends than those I’ve found among you. How lucky  I’ve been to be able to be one reflection on your legacy’s mirror. Thank  you for being part of my legacy.

Isa  Stenzel Byrnes is 51 years old and has CF. She lives in Redwood City,  California, with her husband, Andrew. She is 19 years post-lung  transplant.

Now  is an exciting time to be an adult with CF working in higher education.  In my work with our scholarships committee for USACFA over the past few  years, I’ve seen an explosion of fellow adult community members  pursuing new opportunities in education. Adult community members are  dreaming bigger and thinking bolder about what may be realistic for  them.

A  variety of therapeutic advancements in my lifetime have helped to make  these welcome changes possible. These include CFTR protein modulators,  which I’ve never been eligible for due to rare genetic mutations. They  also include off-label uses of therapies like inhaled corticosteroids,  which quite literally saved my life about a decade ago. Likewise, the  Americans with Disabilities Act and related legislation have made higher  education more accessible for people with CF across all of the spectrum  of disease presentation and progression.

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In this  transitional time, many adult community members also continue to  struggle with serious exacerbations and lasting consequences. So it’s  never been more important for adult learners to have mentors with CF who  can speak to “resume gaps” and other unique facets of education and  career journeys when writing recommendation letters or providing verbal  feedback about a prospective student or employee. Established  professionals with CF can also speak to the particular strengths people  with the disease bring to our learning programs and practice  applications. And working with mentors in our target fields can help us  envision and plan our own pathways.

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These  days our education and career timelines are often long and complex, with  plenty of twists and turns. I think about my own college dean, who  spoke of learning as a journey of constant “zigs and zags” that mentors  help us navigate thoughtfully. This general wisdom rings true now more  than ever for adults with CF and our highly specific experiences.  Regardless of whether we’re on modulators, many of us are living much  longer than we expected to and facing new horizons for our lives.

There’s a  personal angle here too, as always. I’ve worked full time—rather an  understatement, but true in essentials—for my entire adult life. At one  point, I had had three jobs. I’ve always been the primary source of  income for my households. I never stopped working to do my graduate  degrees. Being able to work in jobs that intersected with my education  and provided valuable learning opportunities of their own was certainly a  privilege and a boon, but it didn’t help my lung function or my  exocrine insufficiency in the slightest.

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I’m well  aware these days that I survived my journey through two master’s  degrees and a Ph.D. partly through sheer luck. I powered through a lot  of things that should have landed me in the hospital. Sometimes I  refused hospitalization outright because I knew well from my studies  that the “biographical disruption” of time away would hurt me mentally  and socially in ways I feared more than physical impairment or even  death.

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Absolutely  none of this is a flex. Frankly, it’s more of a horror story. These  choices had terrible costs that I’m still reckoning with today. Some of  these are fairly obvious: prosthetic teeth, rebuilt gums, destroyed  joints, painful breathing, muscle tremors, impaired circulation, kidney  damage, heart problems, and perpetual exhaustion. It hasn’t all been bad  news, certainly. I’ve never had a major organ transplant or been listed  for one. Because I live at sea level, I haven’t needed supplemental  oxygen for any sustained amount of time. But I could do several things  physically in my early 20s that I cannot do anymore. Like many adults  with CF born in the early 1980s, I have lost many things I’ll never get  back.

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Perhaps  most of all though, I internalized a toxic culture of literally working  myself to death—something I’ve dedicated my career to fighting against  at a structural level. I’ve spent the last couple of years taking a hard  look at this and making intentional changes to ensure that what I model  for my students and junior colleagues reflects what I actually want  them to learn. As a sociologist, I’ve understood well for some time that  “do as I say, not as I do” scarcely proves effective as a teaching  strategy. We should be able to see diverse examples of healthy behavior  in our mentors to help us consider what thriving looks like for each of  us individually.

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Throughout  all this, I never had another adult with CF I could look to for  guidance or even just support. That’s made for a lonely journey, if one  that has taught me a tremendous amount about the importance of mentors  who can understand our lived experiences firsthand. It has also taught  me a lot about the importance of responsive mentorship in general,  something I strive to practice consistently in my own work. Lately this  includes not merely embracing but also actively centering how I model  healthy time management myself for my mentees to assimilate and emulate.

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Although  I never had a mentor with CF, I absolutely had great mentors who  understood how to work with someone with a serious chronic illness—and  to learn new things from me in that process. I don’t think I would have  been able to do a lot of things that I’ve done in my career without  their support. And I really have had great mentorship for my entire  life, starting with my parents who raised me in their neuroscience  research lab at Rutgers Robert Wood Johnson Medical School. They never  for a moment thought that I should temper my aspirations for the impact I  wanted to make in the world. My parents never exposed me to the idea  that being sick meant I shouldn’t set big goals or plan for the future.

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This may  have been easier because although I got a tentative clinical diagnosis  of CF at age 5, it would be another 27 years before I got the genetic  testing that confirmed I had the disease. By that point though, my lungs  were going downhill badly and I’d become much more disabled physically.  I’m honestly not sure how I continued doing as much as I did work wise  during that time. Fear of running out of time certainly factored in,  though. People have asked me sometimes how I completed a Ph.D. in two  years while working full time as a research project manager. “I was  dying” is the only answer that has ever seemed honest.

Here’s  the thing, though: I probably would have died had my doctoral advisor  not urged me to seek medical care and get extra rest when I was dealing  with an exacerbation. She didn’t have CF, but she understood well how to  tell me to slow down in ways I’d actually heed. I still hear her voice  in my head and replicate it for my own students. Moreover, I try my best  to tailor the messaging to the unique circumstances and needs of each  learner.

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It’s  gotten easier to do this as my work with USACFA has introduced me to  other adult professionals navigating career transitions and negotiating  balance between work and health. It’s also become more fun. We’re  stronger together than we are individually. I’ve seen this strength in  action by teaming up with fellow community members to offer mentorship  sessions hosted virtually by students with CF and their campus  organizations. I remember vividly how staying on the videoconference for  one of these sessions to talk with the student organizer led to a  candid and heartfelt conversation about how isolating it feels to have  no mentors who are like us—no one who understands the battles we fight  daily. Providing that support means the world to me and highlights the  power of listening and affirming.

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With  each new advancement in therapeutic options and access to them, adults  with CF are surviving and thriving more than ever. We are moving towards  a world where many in the adult community will not be going “back” to  school for their first experience in higher education because they will  never have left school for long periods of time. Mentorship needs for  adults with CF will certainly keep evolving as these transformations in  our survival and horizons continue.

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Organizations  that serve our adult community are innovating in all sorts of ways to  meet dynamic needs within and beyond education. This includes embracing  the breadth and diversity of higher education options. For example, some  organizations are leaning into support for trade school students and  learners seeking additional non-degree credentials. We’re also seeing  expanded support for college and university learning opportunities, from  disciplinary focus to type of enrollment.

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I feel  more fortunate than ever to work with our scholarships committee at  USACFA as our adult community lives longer and thrives more. This year  we’re working on some exciting new opportunities to better support all  of you in your educational goals, and grateful for the support of our  donors who are making that possible. In the process, we’re embracing  opportunities to champion adult community members navigating  intersecting forms of oppression in education and in life.

As we  grow our engagement in supporting adult learners, we’re also embracing  new ways of providing mentorship and support for everyone in our  community who’s pursuing additional education. Our Scholarships webinar  from December 2022 (https://www.youtube.com/watch?v=7LbbU0i2ARY) offers a brief overview of what we do to support adults with CF in  degree and credentialing programs. Our directors and contributing  writers are also eager to connect with you and help you connect with  impactful mentoring opportunities for your unique educational journey.  Reach out and let us know how we can help! 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  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.

We  all know I have a thing for broccoli. The first recipe I shared with  all of you was my Cream of Broccoli Soup. Well, here I am again with a  family favorite and a perfect dish for summer! Chopped Broccoli Salad  has been at every summer holiday get together since I was a kid. Summer  to me is about fresh, bright, and easy food to eat and share with  others. I love how crunchy this salad is and it looks great at any  summer picnic or potluck! This salad can easily be made vegetarian or  vegan by leaving out the bacon and using vegan mayo. It is also a super  easy side dish to scale up for a large party. Happy summer, everyone!

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Chopped Broccoli Salad

Yield: 6 servings

Ingredients:

1 bunch of broccoli

½ pound of bacon, cooked until crispy and then chopped up (optional)

1 red onion

2 tbsp sunflower seeds

3 tbsp dried cranberries or raisins

3 tbsp olive oil

3 tbsp mayo

1 ½ tbsp cider vinegar

½ tbsp maple syrup or honey

½ tbsp Dijon mustard

½ tsp salt

¼ tsp pepper

Preparation:

Step 1:

In a mixing bowl chop the broccoli into quarter-inch pieces, including the stalks.

Step 2:

Dice the red onion finely. Add the dried cranberries, sunflower seeds, and bacon (optional).

Step 3:

In a  separate bowl, add the olive oil, mayo, cider vinegar, maple syrup,  Dijon mustard, and salt and pepper. Whisk together. Add the dressing to  the broccoli mix and stir until fully coated.

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

Once  upon a time, my mom was very sad because her old dog crossed the  rainbow bridge and she was super lonely. Her friend made her go to the  shelter where they keep puppies and kitties.

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Just  over seven years ago, my mom picked me up from that shelter. She says it  was dirty, but I liked all the mud and playing with my six other  siblings.

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We were  found in the middle of the road, picked up by this lovely lady, and  brought to a shelter. These people who fed me named me Tuesday and Mom  says that’s cute because she picked me up on a Tuesday. Then she started  calling me Axil but rarely calls me that anymore. At first, it was Axil  but her friend’s son said I was like a jumping bean so he started  calling me Bean. I still don’t know what a jumping bean is, but it’s  probably something perfect and beautiful since that describes me. Also, I  have been the best dog ever and never do bad things.

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Mom  might disagree. I don’t mean to do bad things because I have the biggest  heart and love my mom. She calls me her treatment buddy, except I  haven’t seen her do many treatments lately, and it’s been a long time  since I stayed in the hospital. Our hospital here in Austin let me stay  with Mom while she was sick. It was great! All the nurses and doctors  loved coming to our room and I got lots of extra treats and bones!  Trikafta made Mom very healthy, which is nice. I still miss my friends  at the hospital some days. I hear rumors from my mom that her friend’s  dog, Husker, was the reason the hospital changed the dog visitation  policy. I don’t think I like this Husker character. I digress.

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We get  to go on more adventures than ever before. She takes me running and  swimming and hiking. We even go to Starbucks and get Pupcups! We have  the best times together!

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About  two years ago, Mom decided to get me a brother. He’s an orange cat and  does a lot of bad things. It made me nervous when we first got him  because he climbs on everything! He even jumps on the walls. I think he  needs to see a doctor because something is not right in his little  brain. He makes Mom very happy and I love having a little  brother…sometimes. Mom says I have to let him write about his story too.

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Hello,  my name is Orbit and I am very brave and strong! When I was little I  almost died from an unknown virus, but I sure as heck didn’t come from  the streets just to have a little virus take me down. I was also very  sick until I had my eyes completely removed because of something called  microphthalmia. It just means that my eyes never fully developed and  they were useless. I didn’t need those things, anyway. Mommy says I see  with my heart, which is weird because that’s not how hearts work.

Things I  like: beating up my brother, stealing his food, dressing up, getting  treats for being cute, jumping on the walls, climbing on everything,  going anywhere in the car with my mom and brother (like the brewery),  going for walks, and so much more.

I love  life! I sit outside on my patio and overlook my kingdom and remind any  other animals or people they are being watched. Mommy calls us her  “stinky boys” and that seems a bit unfair because Axil is much bigger so  he obviously stinks more.

I am a  really good helper to my mom. I help her stay happy and give her so many  kisses and snuggles when she is sad. I am so glad I chose her to be my  mom!

Ashley  Coleman is 36 years old and has CF. She is the Social Media Manager for  USACFA. Ashley lives in Austin, TX, with her dog, Axil, and her cat,  Orbit (@orbittheblindguy on IG). She enjoys going to concerts, running,  and exploring new things thanks to Trikafta.

Most  people in my life do not have Cystic Fibrosis. Inevitably, they are the  ones who ask what it feels like. My reply is always the same…

Imagine  breathing through a straw with your nostrils closed. Then, while  breathing only through that straw, run up and down a flight of  stairs—that feeling, that drowning, that breathlessness, that  desperation for air, and that panic when it doesn’t come—imagine that  all the time.

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I was  6'1'' and 150 pounds at 17 in high school. I wandered through hallways  and life with zero responsibilities and zero interference from cystic  fibrosis. My senior yearbook quote read: “High School was a three-ring  circus; I was the lion tamer.” As far as I was concerned, CF was a  disease in name only—it had no hold on my body or mind and, even though I  was well aware of it, the effects of CF felt about as destructive or  interfering as a landslide on a sand dune.

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My  freshman year of college was barely memorable for all the right reasons.  It was 1998 and I was young and healthy. My lung function was near  100%. I was on the crew team at St. Joseph’s University, doing two-a-day  workouts and raced in the winter regatta on the Schuylkill River in  November. There were few, if any, reasons to even think about CF. I had  it, I knew it, and I ignored it.

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By  September of 1999, that changed. The summer between my freshman and  sophomore years is when cystic fibrosis decided to demonstrate and  illuminate precisely how different I was and precisely how formidable it  was.

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I was  living in Galway, Ireland, for those few months and, when I returned to  Philadelphia, my lung function had dropped by 50% capacity.  Back-to-back-to-back cases of pneumonia took half of my lung function in  less than four months.

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Despite  that, going back to college that second year seemed imperative. I was  desperate to continue the life I had known. Still, every two weeks, I  would drive to St. Christopher’s Hospital in Philadelphia, where I would  watch my FEV1 dip with every visit: three percent drop, five percent  drop, eight percent drop. The PFT machine was my nightmare, slowly  parading an objective truth that all CF patients know—as we lose our  lungs, as we lose our breath, we lose ourselves, both body and mind.

There  are different kinds of anxiety in life. There’s the anxiety of running  into traffic on your way to a meeting, the anxiety of misplacing your  car keys or cell phone, or the anxiety of being late on a bill payment,  for example. There’s another altogether separate layer and level of  anxiety—something otherworldly, supernatural, impossible to explain or  understand unless you, yourself, have experienced it. That is the  anxiety of watching your body being overtaken by cystic fibrosis, the  anxiety of knowing your lungs and body are failing you, abandoning you,  discarding your will and desire—your frenzied, frenetic, and frantic  attempts to survive.

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I  finally left college my junior year. It was Thanksgiving and I drove  myself to St. Christopher’s Hospital. I remember crawling across the  street from the parking lot to the automatic doors of the hospital. I  remember dragging my feet over the asphalt and breathing so fiercely  that I nearly lost consciousness. I remember feeling the cool air on my  face when I stumbled through the hospital doors and collapsed into the  arms of my pulmonologist.

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As I lay  in that hospital bed as the holiday came and went, a switch turned on  in my mind. A simple switch with immediate effects and terrible  consequences. I would no longer talk to people, I would no longer  express love or joy, I would no longer smile or laugh or try and enjoy  this life I was living. Instead, I would survive. I would not die. And,  if a miracle did happen, if a call came for a transplant, then, and only  then, would I allow myself to feel again.

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I was  surrounded by mountains of love, but my mind told me, express nothing,  love no one, cut everyone and everything off—then, when you die, you  won’t be as missed, it won’t be as tragic.

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Two and a half years. Thirty months. 913 days of waiting. Then, on July 21, 2002, the phone rang.

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The  gratitude for my transplant was all-consuming. It was immeasurable and  still is. It is difficult to express this level of relief, difficult to  find the words to speak or thoughts to think, though with this new life  came new complications—survivor’s guilt, PTSD, confusion, anger, and  overwhelming joy.

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Rather  than taking the necessary time to heal emotionally, I tried returning to  my old life. At 24, I returned to college. I was a “college kid” again,  at least in name.

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Sitting  in those classrooms, I found myself wondering and whispering to no one  and nothing in particular how meaningless it all seemed. I was no longer  a kid, partying on the weekends, skipping class with a hangover from  last night’s bender. I was no longer living in some cramped dorm room  with eight other boys pretending to be men. My life had altogether taken  on a new meaning and was now defined by terms that my peers knew  nothing about. I couldn’t relate to anyone. On top of that, I felt like  time had passed me by. Every person my age was already working and  starting careers, getting engaged, moving onto the next phase of their  lives. How could I catch up? What shortcuts could I take? Before my  transplant, my life had become years of racing towards a red light and  now, I was seeing green lights for miles, but with no direction in mind,  so I just kept driving.

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In 2005,  I graduated from St. Joseph’s University with a degree in literature. I  worked for a few years in the television and film industry. The  transplant allowed me to breathe. My physical health flourished, but the  emotional distress that I had ignored had finally became a shadow that  was impossible to escape. And so, one day, without much warning or  consideration, I did the only rational thing I could imagine: I left.

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I left  my life behind. Everything. All of it. Family and friends, my  possessions, my job and money, security and relationships, even my  doctors. I packed a bag, bought a one-way ticket for Europe, boarded a  flight, and began a new life. At 26, I was just beginning to understand  what my life was going to be and who I would be in it. Cystic fibrosis  was and will always be a part of my story, but only one chapter. The  rest are for me.

Andrew  Corcoran is 43 years old, has CF and received a lung transplant in  2002. He now lives in South Jersey with his family and friends. He is a  writer. He can be reached at AndrewBCorcoran@gmail.com.

I  couldn’t believe it—I  was a practicing doctor again after 17 years of  being away! I had already tried twice in the last 10 years to return to  direct patient care and, when those attempts didn’t pan out, I started  to give up altogether. Fortunately, I had forgotten to unsubscribe from  an allergy and asthma physician job board, even after coming to terms  that I would absolutely never return to work again. One week after  making this final decision, a dream job popped up and fell into my lap  for employment as an allergy physician in a private practice. I chose to  work part-time hours three days per week. I guess the third time’s the  charm! Having worked there for one year already, I’m thrilled to say  that it’s been a fabulous ride so far.

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Prior to  starting the new job, I was fairly nervous—I wondered if I’d fall ill  the moment I started as I had the first time I tried to go back to work  10 years prior. At that time, I spent one day treating allergy patients,  went home and suffered a heavy episode of hemoptysis. That was it for  that! The biggest difference between then and now was that in the  interim I was able to start the CF protein modulator Kalydeco, which  changed the course of my life. I distinctly remember after one week on  Kalydeco wondering if the changes I was experiencing were real and  sustainable. I thought, “Someone pinch me!” My health continued to  improve year after year. The regular hospitalizations, productive cough,  wheezing, GI issues pre-Kalydeco transformed into days of minimal  respiratory and GI dysfunction, improved sleep, and more energy. These  changes again motivated me to consider going back to work and I  subscribed to an allergy doctor job board to peruse potential  opportunities.

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In  December 2019, Kalydeco was replaced with the latest modulator Trikafta,  which seemed for me to keep back-to-back colds from progressing to full  blown CF flares. This additional benefit prompted me to work in January  2020 helping a pediatrician friend with her patients. I’d barely gotten  my feet wet when two months later the pandemic befell the world and I  didn’t want to risk my health treating patients in-person.

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As I  considered my third attempt to return to work, there were a multitude of  other worries leading up to the first day on the job. So much had  changed about medicine during the 17 years that I was on disability. I  had only worked in a hospital environment with students, residents,  fellows, and other allergy specialists supporting the clinic, which was a  totally different structure than a private practice where you’re it and  you do just about everything for the patient. I thought my head was  going to explode trying to remember all I had relearned and newly  learned in the months leading up to the first day. I had maintained my  allergy and asthma board certification all these years, but I wanted to  have all the latest information at my fingertips as much as possible.  All my knowledge was tied up into what felt like a tight ball of yarn,  which I was worried wouldn’t effectively unravel during a patient visit.

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There  was also Practice Fusion, the electronic medical record system that I  had never used before. The whole system was like learning a foreign  language. I also met a new set of coworkers—nurses, assistants, billing,  and other staff—all of whose names I was trying to remember, along with  figuring out who did what.

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Nothing  could prepare me for the first day, though. I was fairly slow and ended  up spending 45 minutes on the first “follow-up” patient. The appointment  length was slotted for 15 minutes. The patient requested a test that is  only done in allergy research. It was a bizarre request and I tried to  explain that only medical research environments conducted this test. It  was very frustrating, but eventually the visit ended with what felt like  half my hair pulled out. I thought, “Oh, my god, is this how patients  are now?” Fortunately, the remainder of the day went smoothly as have  the great majority of clinic days since. The patient population has  overall been delightful this past year, and I’ve never come across  anyone quite like that first patient so far. Whew!

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Three  months into practicing, I thought my biggest fear had come true. I  contracted the flu, thank you to a visitor from Las Vegas. Evidently  what happens in Vegas does not stay in Vegas. My health went south very  quickly and I ended up in the hospital on oxygen. Fortunately, the  allergist whom I work with was very supportive and, with the help of a  physician assistant, covered for me for three weeks.

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During  those three weeks, I started to feel strongly that returning to work  would be too difficult and that with each passing day, the probability  was slipping away. After two weeks I still needed oxygen and was fairly  breathless with any activity, even showering. I could’ve used two or  three more weeks off, but my colleagues had planned travel soon and  wouldn’t be able to cover. By the third week, I was off oxygen and,  despite my fatigue and weakness, I returned to work (again!) sensing  that this would be a day where I’d make it or break it and decide to  stop working. I was seriously uncertain if I’d survive the day. As I  entered the office, I almost broke down crying but kept it together  holding back tears in my eyes. I couldn’t believe I made it back. The  day ended up being a welcome distraction from my health and showed me  that I’d be okay; I was stronger than I thought. Fortunately, slowly but  surely, over the next four to five weeks, my baseline lung function and  physical and emotional strength returned.

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The big  chunk of 17 years on disability were tumultuous health years and it took  every single one of those years to enable me to return to direct  patient care. I knew others with CF who had tried to return to work full  time and ended up leaving the job due to severe exacerbations primarily  from not having enough time to take care of themselves. Their  experiences pushed me to consider part-time work only, which has been  essential to success this past year. Part time allows me to fully take  care of myself, get plenty of rest, exercise, keep up my daily breathing  treatments, and appreciate time off! Also, I had a feeling that  setbacks would occur but hoped they wouldn’t be so severe as to stop me  from working altogether. I believe anyone going back to work can  realistically expect that tweaking of your routines will be needed and  yes, setbacks will occur. In a supportive work environment, setbacks  don’t signal the end of employment but can be overcome.

Jeanie  is 61 years old and has CF. She is a physician who lives in Los  Angeles, CA, with her husband John. Jeanie has three amazing grown  children and two beautiful granddaughters. Her allergy practice is in  Glendale, CA. She welcomes your comments and asks that you send them to cfroundtable@usacfa.org.

Practicing  law was my second career. I went to law school at age 33. I worked full  time after graduation for two and a half years then changed firms and  switched to part-time work. Four years later, I stopped working because I  was in that “seesaw” place—if I kept up with my work, my health  suffered; and if I was fully compliant with my daily health demands,  then I couldn’t keep up with work. At 43 I wasn’t bouncing back after  exacerbations like I used to. I hated not being able to fully perform my  work responsibilities, so I had to let work go. At age 59, three years  post-transplant, I went back to work part time as a lawyer at my old  firm for one year. I worked remotely from Florida with my firm in New  York City. One benefit of COVID-19 was that it proved to employers that  people can work from home successfully. I wanted to return to work as I  felt so much better physically and I was desperately seeking  intellectual and social stimulation.

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I  thoroughly enjoyed using my brain again. I did legal research, drafted  litigation papers, and discussed cases with partners and associates. I  also enjoyed the sense of purpose and accomplishment I regained. I had a  job to do. It felt good to make my own money again.

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After so  many years living without a schedule, I struggled to establish a  schedule for working, eating, staying hydrated, and exercising. I had a  tendency before to get tunneled in to my work and ignore a lot of these  fundamental things. I was surprised how quickly I fell back into that  pattern. Additionally, litigation is demanding and has deadlines so  there really is no way to just work four to five hours per day. Some  days the job demands that you keep going until the papers are done.  Sometimes I would hit a wall mentally. It took a while for my brain to  build up endurance and hone the ability to focus for longer periods of  time. I didn’t see that as a bad thing; it seemed normal that I would  experience this after not working for so long.

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I  expected that I would have more social interaction with others in the  firm. That was something I missed after I stopped working the first  time. This did not happen and it was mostly because I was working solely  from home—I never went into the office nor did I participate in social  gatherings.

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Surprisingly  to me, I found that it was still a physical challenge at times to even  work part time after my transplant. I developed GI problems beyond my  normal malabsorption/constipation issues. Randomly, yet not  infrequently, I experienced weakness and had to lie down for an hour or  so. This of course was very disruptive to my work and frustrated me. I  had more specialist doctor appointments, tests, and procedures than  before transplant. It took me at least an hour each way to get to these  appointments.

My  contract ended this past February and was not renewed because of a new  nepotism policy established at the firm (my husband works there). I  chose not to look for another firm because what I had at my old firm  were people who understood my situation and it was highly unlikely that I  would find a new law firm that would permit me to have such a flexible  work schedule. The legal field is by and large not supportive of people  with health issues. At 60 years old, I did not feel like being put in  the position of explaining my health situation, having the anxiety of  interviewing, the additional anxiety over a potential “new job,” or of  having the feeling of disappointing someone because I can’t always work  the hours an employer needs.

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Overall,  I am glad I did this work for a year. At first, I was very angry and  disappointed in the firm for not keeping me on. But now I am ok with not  working and the absence of work pressure is actually something I  appreciate.

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.

Six  years ago, in March 2017, I packed all of my medications, medical  supplies, and devices into three suitcases, put my mask on for a 12-hour  day of flying (even pre-pandemic I always wore a mask when I flew), and  headed off to the first stop on my journey to transplant: Duke  University Hospital. I would spend six long days there, in North  Carolina, going through evaluation for a double lung transplant. It was  grueling and stressful and yet I had hope for the future. I was  cautiously optimistic that this could be my time for a second chance at  life. I would no longer need oxygen 24/7. I would soon not need the  daily intravenous and oral antibiotics I had been taking continuously  for eight years at that point. I would be able to start living life  again instead of existing in a state of slowly dying. And then, after  those six days of invasive testing and countless appointments with  doctors, and just one day after returning home to Anchorage, Alaska, the  phone call came that changed the course of my life.

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I was not a candidate.

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Now, let  me take you back to 2009. I was 17 and in my junior year of high  school. I should’ve been spending time with my friends before  graduating, figuring out which colleges I was going to apply to, and  prepping for my last year at Dimond High. Instead, I was on a mandated  quarantine at my mom’s house where I was living half the time, isolating  from the people around me, and waiting to find out the results from a  sputum sample I had given two weeks prior. I had recently been  discharged back home after a week of debilitating fevers and body aches.  My breathing had started to decline so badly that I had to quit my  school’s soccer team because I could no longer run. It was only the  beginning of my decline with cystic fibrosis. In the hospital, after  being stumped by the origin of my symptoms, my pediatrician brought in  an infectious disease specialist—one who would later become like family  to me. She, too, was completely stumped after running blood tests,  collecting numerous sputum samples, and checking every organ in my body  for the culprit.

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After a  slew of inconclusive results, her best guess was that I may have some  kind of mycobacterial infection. At that time in Alaska, the most common  of these was Mycobacterium tuberculosis (TB). After getting my fevers  under control and starting me on a treatment regimen, I was discharged  and sent home under a strict quarantine due to the extremely infectious  nature of TB. That infectious disease doctor sent my sputum samples out  to independent labs to further identify what bacteria was growing. She  told me that there were many different kinds of mycobacteria and that  her gut was telling her it wasn’t TB. She predicted that it would come  back as either Mycobacterium avium complex (MAC) or Mycobacterium  abscessus (M. abscessus). For some reason, whether by intuition or just  curiosity, I asked her, in her opinion, which outcome would be worse for  me. Her reply came easily, “My hope is that it’s not M. abscessus.”

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Flash  forward two weeks, and the results were back. It was, to both her dismay  and mine, Mycobacterium abscessus. I was instantly anxious. What did  this mean? I had never heard of this infection before. I had never even  heard of non-tuberculous mycobacteria in general. I had no idea what  would happen, what this would cause, and just how devastating this  outcome would turn out to be.

This  infection became the biggest hurdle in my life, especially when it came  to transplant. In fact, years passed between my Duke rejection, the  first attempt of many at a lung transplant evaluation, and the next  attempt I made because I was so distraught and disappointed. For my next  few referrals to transplant centers, my pulmonologist’s office sent my  records out only to receive rejections in the form of one-page letters,  sometimes even just one paragraph in length. Each one was quick and to  the point: “Mycobacterium abscessus infection is a contraindication for  lung transplantation.” There would be no further look into my history or  who I was. I was, to put it simply, too high risk.

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This  infection in particular made surgery very dangerous for me. For one, M.  abscessus can colonize not just your lungs, but also your trachea,  sinuses, and mouth. This means there is potential for it to eventually  infiltrate transplanted lungs. Secondly, there is obviously a lot of  cutting that happens during a lung transplant. If they were to cut into a  pocket of infection as they removed my original lungs, the bacteria  could spill into my chest and infect the new lungs or even the incision  site, as M. abscessus is also a commonly known skin infection. And the  worst-case scenario: If it spilled into my blood stream, it would travel  throughout my whole body, eventually infecting my brain. One doctor  described this as a “slow, miserable death,” your body basically wasting  away as it tries to fight off the infection, to no avail.

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So, for a  while, after nine total rejections in one year, I put the thought of  transplant away. It was too hard to hear that my life was not worth the  risk. At least, that’s how it felt. Trust me, I understood those valid  reasons why I was an “undesirable candidate.” I would also not want  those things to happen to me. Additionally, I understand the concept  behind “do no harm,” and that they don’t want to put a transplanted  organ in someone who may not survive because organs are in high demand.  But how could I not take this kind of rejection personally? To me, it  was my life. It was nothing but personal.

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And  then, two years after the last rejection letter I received, after enough  time went by to heal my emotional wounds, something shifted in me. I  was ready to try again. I had grieved those devastating blows, those  losses, and regained clarity through them. My life meant something to  me. No matter how many times people told me no, I could not give up.  This is part of what I learned from this process: It’s not over until  it’s over. I know it’s a cliché and we hear it all the time from people  trying to give us encouragement, but I came to see that it was true: If  my heart is still beating, if I am still breathing, it’s not over.

So I tried again.

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I  rallied my home team, my pulmonologist, his nurse, and got to work. This  time, rather than just sending a referral to the biggest transplant  centers, I started googling centers that actually had a history of  transplanting patients with M. abscessus. I put together a list of the  top 10 I felt most likely to accept me, and my records were sent to the  first one. My pulmonologist had decided we would go down the list,  waiting for one to say no before moving on to the next. Within a week, I  heard back. First, I would travel to Baltimore, Maryland, to be  evaluated at The Johns Hopkins Hospital. Unfortunately, but not  surprisingly, it ended with another rejection. But I kept going. Next on  my list was the University of California San Diego. Here I received a  brief phone call letting me down, which was at least a step up from a  generic letter in the mail. Third on the list was the University of  California San Francisco. Here, I received a glimmer of hope.

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I began  my relationship with UCSF at the end of 2019. They were upfront with me  that they did perform lung transplants on patients with M. abscessus;  however, because I had never been able to reduce the burden of the  infection in my decade-long battle against it, they weren’t sure yet  whether I was a potential candidate. But, they had hope and again I  found myself cautiously optimistic. It was the first time I had heard a  transplant doctor tell me in no uncertain terms that he thought they  could do it. Yes, they needed to discuss as a team and review my history  further and they would have to meet with me in person before making  their final decision, but it was the foot in the door that I needed.

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At  first, the process with UCSF was slow. It started with virtual  appointments and testing done locally, as I was still living in  Anchorage. When the pandemic hit, my chance to fly to San Francisco to  be evaluated in person came, but I was no longer comfortable getting on  an airplane. My thought was it wouldn’t be worth the journey if I caught  COVID-19 on the way there and could no longer go through with the  evaluation. At this point, the vaccines had just barely come out. I  decided to wait, hoping my health would at least stay stable until the  time felt right.

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Finally,  in 2022, that time came. UCSF came up with a plan to get me there, and  it began to move quickly. In April, I was brought into my local hospital  to be transferred via Medevac to UCSF Medical Center. My oxygen  settings at the time were too high to take a commercial flight, so this  was my only option. When I arrived, I was inpatient for five days, going  through the formal evaluation and meeting their whole team. I was then  discharged but had to stay in the area. If they were to accept me to  their program, I would need to permanently remain in San Francisco, at  least until two years post-transplant. On May 18, they called me with  their decision.

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This time, I was accepted. My heart leapt. I could not believe it.

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I was  placed on the waiting list for a double lung transplant and a kidney  transplant as, by this time, due to my continuous antibiotic treatment  for M. abscessus, I had injured my kidneys irreparably and developed  renal failure. The goal was that I would receive both organs from one  donor.

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Because  this opportunity had eluded my grip for so long, I naturally doubted it  would come to fruition. Due to my previous experiences, it became  instinctual to protect myself from further rejection this way. However,  despite my disbelief, on August 20, 2022, after spending three months on  the waitlist, I received my life-saving transplants form a deceased  donor. It was the hardest, most emotional thing I have ever been  through. Words cannot sufficiently describe the feeling of waking up  that first day; of taking your first breaths after extubation; of being  told you no longer need that oxygen cannula; of seeing your family’s  reactions as you begin to change before their eyes. It was so much to  process; my brain could not keep up. Even to this day, I have a hard  time wrapping my mind around the experience. Recovery was difficult,  frustrating, and slow. And then, suddenly, it was fast, rewarding, and I  began to see it for the blessing it was. I am forever grateful to UCSF,  the incredible team there, and, most importantly, my donor and their  family, for making the most courageous, selfless decision.

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As I  said before, I learned so much through my tumultuous journey to  transplant. It was far from easy, and absolutely nothing happened the  way I expected it to. But I learned so much about myself and gained so  much knowledge about what’s important to me. And that timing really is  everything. In hindsight, I am thankful it happened when it did and not a  moment sooner. I needed to go through, and grow through, those trials  to become the person I was at the time of my transplants. I gained  perspective in my life that I wouldn’t have otherwise and became the  person I am now.

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My years  of fighting and advocating for myself taught me that I am capable. I  have power within me, even at my weakest. At one point during my  recovery in the hospital, after I was brought to the step-down unit, I  had a conversation with my lung transplant team. I felt the need to  thank them for taking a risk on me, for giving me this second chance. As  I finished my heartfelt statement of gratitude, the response from one  of the three doctors sitting in front of me caught me off guard. His  opinion was a little different than mine: I had myself to thank for this  gift of life—“you got yourself here.” He felt I had made them take that  chance on me. He credited my grit, my perseverance, and my  unwillingness to give up as the true reasons for my getting to that  moment. I beat down their door and they let me stay.

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I also  learned that life is made of the small moments, the ones you might look  over had you not been paying attention. It is not the big moments of  excitement and adventure that I longed for all those years I spent too  sick to fully live, thinking that my future was just my death. It was  the tiny, miniscule ones. I wanted nothing more than to sit on the couch  on a Friday night, watching a movie cuddled up with my cats, and have  absolutely nothing else on my mind. I wished for the day I could play a  board game with my family again, in the comfort of my own home, on my  own time, instead of at my bedside in the hospital, when a nurse wasn’t  connecting me to an IV line. I always thought of the day I would be able  to walk out the door again at a moment’s notice, not having to think  about how many oxygen batteries I needed to carry with me or when my  next antibiotic was due, counting the minutes until I could get back  home the whole time because just the action of getting dressed was so  exhausting.

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Before  my transplants, every moment of my existence seemed to hang in the  balance. To be able to exist quietly, softly, presently, within the  simple, mundane moments of my day, is all I ever wanted. Transplant, my  experience up to it, made me see what matters to me. It taught me to  slow down and take in the view. And it showed me that even in my weakest  moments, I am strong.

Matison  Deaton is 31 years old and originally from Anchorage, Alaska. She now  lives in San Francisco. Matison has two black cat furbabies, loves  puzzling, and walking in and connecting with nature. She also finds joy  in being a part of the CF community. Matison is on the Rose Up Committee  and has dedicated time to spreading awareness for CF and now organ  transplantation.

USACFA  proudly offers three different scholarships! Both the Scholarship for  the Arts and the Higher Education Scholarship were set up in memory of a  loved one. Our newest scholarship kicks off this fall with a one-time  deadline of 10/30/2023. You may apply for more than one scholarship each  year, but you may only be awarded one per academic year. If you do not  win, your application can be moved to the pool of applicants for another  relevant scholarship in the same cycle. For questions about future  scholarships or anything related to the application process, please  contact us at scholarships@usacfa.org.

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Scholarship for the Arts
(05/30/24):

This  scholarship will award two deserving students $5,000 each toward their  tuition in their respective field of the arts: fine arts, computer  graphics, design, music, choral, photography, filmmaking, creative  writing, poetry, dance, and theater arts, to name a few. It is open to  anyone seeking a creative arts degree, whether it be an associate’s or a  doctorate.

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The  Scholarship for the Arts was established by Andrea Eisenman to honor her  mother, Helen Eisenman. Helen was a single mother devoted to her  daughter, Andrea, who has cystic fibrosis.

https://www.cfroundtable.com/arts-scholarship

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Higher Education Scholarship
(06/30/24):

The  Higher Education Scholarship was set up by Nancy Wech, in memory of her  daughter, Lauren Melissa Kelly. The academic scholarships of up to  $2,500 are awarded to two adults with cystic fibrosis who are pursuing  career certifications, associate’s, bachelor’s, and graduate degrees.

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Any  student seeking a degree in higher education, from associate’s to  doctorate, 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.

https://www.cfroundtable.com/highereducationscholarship

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William Coon, Jr. Scholarship
(10/30/23):

Any  student seeking a degree in any of the following is welcome to apply:  business, economics, communications, political science, information,  project management, finance, accounting, public administration, or  marketing. We believe that any higher education is a strong foundation  for advocacy and involvement in the CF community.

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William  J. Coon, Jr. established $20,000.00 in scholarship funds to be awarded  in $2,500.00 scholarships for four students each year over a period of  five years, totaling 20 scholarships. Mr. Coon was both a cystic  fibrosis patient and a businessman who valued the importance of  education and “paying it forward.”

https://www.cfroundtable.com/williamcoonjrscholarship

Are  you interested in establishing a memorial scholarship honoring a loved  one from the CF community who has passed away? Please reach out to us at  scholarships@usacfa.org to learn more. A member of our Scholarships Committee will follow up with you promptly!

It  is with great sadness that we share the passing of Kathy Russell at age  79. Kathy was so many things, aside from being a founder of USACFA and  CF Roundtable. She was a loyal friend, a licensed practical nurse,  exceptional cook, canner of her own grown tomatoes and vegetables. She  had a clever sense of humor and could make a statue chuckle. She imbued  her empathy and caring to share her stories of her own CF experience on  the pages of CF Roundtable, but also edited other people’s submissions  so that their voices shone through. Kathy was also part of a strong  bonded team with her husband Paul. Wherever Kathy was, Paul was not far  away. They were married on Marsh 27, 1965 (58 years!), and had a very  happy and loving life together throughout life’s curves and bumps. They  weathered it together.

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Kathy  Russell and a few other people with CF heard a call for assistance from  Lisa McDonagh to help her publish what was then known simply as The  Roundtable. She was publishing it all on her own and, after each issue,  she would be hospitalized with pneumonia, so she knew she needed help.  Along with Kathy, there were: Melinda Timmermans, from Florida; Joe  Kowalski, from Connecticut; Connie Knoles, from Oregon; Ken O’Brien,  from Illinois; and Larry Culp, from Pennsylvania. These five people  became the founders of the United States Adult Cystic Fibrosis  Association, Inc. (USACFA). They registered it to be a nonprofit in  1990. While Kathy was a founder, she was designated as the treasurer in  1990 and later elected to the board of directors in 1993. She held many  positions at the organization by being elected to every officer position  from treasurer, secretary, editor, and director. She was president from  1997 to 2005 and managing editor of this publication from autumn 2011  to autumn 2019. She started her column called Speeding Past 50 in winter  2006 when she was 62. In this column, she shared her own life lessons  living with CF: Topics ranged from senior aches and pains to to sinus  disease and what she did to combat it to wearing comfortable shoes  because they just felt better than the coveted polka-dotted high-heals  she loved. She adapted to life as it came her way with a smile and a  good sense of humor.

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She was  much looked up to in the CF community as she was also very involved in  planning conferences and almost always attending them all over the  country (before stricter infection protocols did away with most of  them). She was also one of the oldest living people with CF most people  knew. Oddly, her parents were told when she was diagnosed in 1956 at age  12, she would only live another 6 months longer. That they should take  her home and cherish the time they had left. She showed those doctors!  Which is why she was a great problem solver. She didn’t take no for an  answer if she felt she could do it. If she wanted something, she worked  hard to get it. Her favorite job was working as a nurse in the newborn  unit, and actually, probably the safest for someone with CF because it  was free from infectious disease. She was a smart cookie.

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Her  favorite accomplishment that made her proudest, according to Paul, was  being part of CF Roundtable and the CF community itself. She loved  nothing better than talking to people who reached out to her and who  might have wanted guidance, support or just an available ear to discuss  CF. She was a kind and interested listener. In later years as she needed  more time for herself, she mainly emailed with people.

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As the  years advanced, Kathy loved sitting in her comfy recliner doing  1000-plus-piece puzzles, taking day trips with Paul, cooking extravagant  dinners and deserts for just the two of them, visiting friends and  family when she could. She will clearly be missed by all who knew her.  The CF community has lost a loving and fierce advocate. She leaves  behind a beautiful legacy that generations will benefit from even if  they did not have any connection to Kathy. She paved the way in many  ways to living a long and fruitful life that enriched so many. It was an  honor to know her.

It is Kathy’s wish that if anyone is so moved to make donations to CF Roundtable (https://www.cfroundtable.com/donorbox), CFRI (https://41224.thankyou4caring.org/donate-now) and CFF (https://www.cff.org/donate).

I’m  proudly enclosing a check in honor of my daughter, Debra Radler, who  achieved the miracle of celebrating her 60th birthday. When she was  born, the life expectancy was 18 years. None of this would have been  possible without the dedication of USACFA. I am beyond thankful to  everyone who has worked so hard to have made this miracle happen! God  bless!

Loretta Mulatz

Kathy  was like a sister to me and my sister, Paige. We have spent the last few  days sharing stories and our sadness at her death. Paul called us and  other cousins shortly after Kathy had died. I write as I receive your   newsletter that Kathy and others started in 1990. What a journey it has  been. I’ve learned so much over the years…I’m sure there will be an  article about Kathy and though it will be sad to read about her  passing/it’s amazing the legacy she leaves behind.

Kathy was an amazing individual who lived and loved her life—especially with her best friend, Paul.

In tears,

Laurie Worth

Would you like to be featured in our publication?
Do you want to be in the spotlight?
Let us shine a spotlight on you and your life!

We would love to showcase you and your life in our publication. If you  are interested in being interviewed for an upcoming issue of CF  Roundtable, please email us at CFRoundtable@usacfa.org.  We will pair you with an interviewer who will arrange a time to talk  and then write questions based on your answers for you to fill out at  your leisure. Each interview is crafted to bring out what each person  wants featured about themselves. To go along with your interview, we ask  for two photos that will go into the publication: one headshot and one  photo of you with your peeps, family, pet, on vacation, graduation, etc.  We want you to shine so that others can benefit from your experiences  living with this shared disease.

CF Patients Using Kaftrio/Trikafta May Enjoy ‘Near Normal’ Lifespans

Use of  Kaftrio (elexacaftor/tezacaftor/ivacaftor - Trikafta) may help people  with cystic fibrosis (CF) and the F508del mutation in both CFTR gene  copies live to a median age of 71.6 and possibly longer if treatment is  started in adolescence. In a scenario where treatment with Kaftrio is  initiated between 12 and 17 years old, a patient’s median estimated  lifespan is 82.5 years. That’s 45.4 years longer than estimates for CF  patients on best supportive care alone. To determine estimates of such  benefits with Kaftrio as opposed to other treatment options, scientists  used computers to run a microsimulation, a model that mimics the effects  of treatment on individual members of the CF population. They drew on  data of disease progression from articles published in scientific  journals. Data on how well each treatment worked came from Phase 3  clinical trials and extrapolations of clinical information. In addition,  it was estimated that people on Kaftrio would experience better lung  function across greater spans of life than those on best supportive care  and, subsequently, less time with severe lung disease. Life-years were  defined as the estimated years of life assigned with a given treatment  in these simulations. While living longer, people on Kaftrio also were  projected to have 13 fewer pulmonary exacerbations over a lifetime than  those on best supportive care. The greatest survival benefit relative to  those on best supportive care was observed when patients started on  Kaftrio at a mean age of 14.9, the study reported. Relative to best  supported care alone, the median projected survival was 82.5 years for  those who started treatment at ages 12 to 17. Among simulation patients  started with Kaftrio between 18 and 24 years old, their anticipated  lifespan was  77.9 years; it was  62.2 years for those who beginning  treatment at age 25 or older.

https://tinyurl.com/2n32c7fz

AND

https://tinyurl.com/2jmspbwe

Cost Burden Among The CF Population In The United States: A Focus On Debt, Food Insecurity, Housing And Health Services

Advancements  in the cystic fibrosis (CF) field have resulted in longer lifespans for  individuals with CF. This has led to more responsibility for complex  care regimens, frequent health care, and prescription medication  utilization that are costly and may not be fully covered by health  insurance. There are outstanding questions about unmet medical needs  among the U.S. population with CF and how the financial burden of CF is  associated with debt, housing instability, and food insecurity.  Researchers developed the CF Health Insurance Survey (CF HIS) to survey a  sample of people living with CF in the U.S.  A total of 1,856 CF  patients in the U.S. were included in the study. Of these, 64% faced a  financial burden: 55% of respondents faced debt issues, 26% housing  issues, and 33% food insecurity issues. A third reported at least one  unmet medical need: 24% faced unmet prescription needs, 12% delayed or  shortened a hospitalization, and 10% delayed or skipped a care center  visit as a result of the cost of care. Income is the biggest risk factor  for financial burden for people with CF, with people dually covered by  Medicare and Medicaid particularly at risk.

https://tinyurl.com/3997v8v4

The Risk  Of Colorectal Cancer In Individuals With Mutations Of The Cystic  Fibrosis Transmembrane Cond-uctance Regulator (CFTR) Gene: An English  Population-Based Study

Studies  have demonstrated a higher risk of developing colorectal cancer (CRC) in  individuals with Cystic Fibrosis (CF). Life expectancy for those with  CF is rising, increasing the number at risk of developing CRC. When  compared to other population studies the overall prevalence of CFTR  mutations in the CRC population was significantly higher than expected.  This study demonstrates an increased risk of CRC among individuals with  CF and a higher than expected incidence of certain CFTR mutations among  those with CRC. When age-standardized the incidence of CRC was five  times higher in individuals with CF. This increase in predisposition is  likely to be multifactorial due to a combination of factors such as CFTR  dysfunction, increased local and systemic inflammation, altered gut  microbiota and the use of high fat and low fibre diets. The study also  suggests a higher predominance of right sided tumors and an increase  incidence in females, compared to the population without CF. This is a  significant finding as right sided tumors of the colon have been shown  to have worse survival and are frequently diagnosed at a later stage due  to their symptom profile.

https://tinyurl.com/bdf2ed5y

Human Papillomavirus Prevalence, Persistence And Cervical Dysplasia In Females With Cystic Fibrosis

A higher  risk of human papillomavirus (HPV)-related cervical intra-epithelial  neoplasia (CIN) is suspected among females with cystic fibrosis (CF).  The researchers found that transplanted females had high-risk (HR-HPV),  abnormal cervical cytology and CIN prevalence rates compared to the  general non-CF population. Although HR-HPV prevalence and persistence  were globally not significantly different in non-transplanted females  compared to the general population, high frequencies of abnormal  cytology and CIN were reported. Cervical cancer screening and prevention  should be promoted among females with CF.

https://tinyurl.com/2hm2pvuu

Disease Severity Of People With Cystic Fibrosis Carrying Residual Function Mutations: Data From The ECFS Patient Registry

People  with cystic fibrosis carrying residual function (RF) mutations are  considered to have a mild disease course. This may influence caregivers  and patients on how intensive the treatments should be. Demographic,  clinical characteristics, lung function and death probability of  patients carrying at least one RF mutation were analyzed and compared to  patients homozygous to minimal function mutations (MF). Patients  carrying RF mutations were older, diagnosed at a later age, had lower  sweat chloride at diagnosis and better FEV1pp at each age group.  However, their FEV1pp declined with age and rates of chronic Pseudomonas  aeruginosa increased with age. 4.5% of RF patients were treated with  oxygen and 2.61% had a lung transplant. With increasing age, 26.6% of RF  patients were treated with pancreatic enzymes associated with a more  severe lung disease. RF patients had shortened life spans, with  mortality starting around the age of 20 years. Thus, patients carrying  an RF mutation experience a decline of pulmonary function with age,  leading to life-shortening. Standard of care therapies and augmenting  CFTR function may improve their survival and quality of life.

https://tinyurl.com/46dzfrct

Lung Infection May Be Less Transmissible Than Thought

Recent  research found that various strains of the bacterium Mycobacterium  abscessus were genetically similar, stoking fears that it was spreading  from person to person. But a new study by researchers calls those  findings into question, offering an alternative explanation behind the  genetic similarity of clinical clusters. This suggests that the pathogen  may not be that prone to person-to-person transmission after all. The  results argue against direct person-to-person transmission in clinical  settings and instead point to M. abscessus infections being acquired  from the home or other environmental exposures.

https://tinyurl.com/2hpdrf8s

Breakthrough Drug Combination Remains Safe And Effective Long-Term In Patients With Cystic Fibrosis

Patients  12 years and older who received the combination regimen of tezacaftor  (TEZ) and ivacaftor (IVA) for four years did not experience any serious  safety concerns and tolerated it well, suggesting its appropriateness  for long-term use. Not only did patients tolerate the combination  regimen over the course of the 96-week study, but they also maintained  the improvements they had achieved in an earlier trial. The trial  assessed the long-term efficacy and safety of treating patients with a  combination of two CF modulators, TEZ and IVA (TEZ/IVA). While this  trial was still being conducted, a three-part regimen composed of  TEZ/IVA plus elexacaftor (Trikafta) was approved. With TEZ/IVA, there is  about a 2% to 3% increase in lung function, whereas with the triple,  there is a 15% improvement in the lung function. Patients are also less  likely to require hospitalization. Although clinicians recommend taking  the three-drug combination regimen because of its increased  effectiveness in improving lung function compared to TEZ/IVA, some  patients cannot tolerate it and so must remain on the dual therapy. This  study’s findings that TEZ/IVA is safe and well-tolerated over the long  term also builds confidence that two of the three drugs in the  standard-of-care therapy can be used safely over time. Again, this is  important reassurance for patients who will likely take the drugs for  the rest of their lives. Should safety issues arise, these data will be  useful in pinpointing the problematic agent in the combination regimen.

https://tinyurl.com/2lnsjd5l

AND

https://tinyurl.com/yrskmtyt

AND

https://tinyurl.com/3m6uaejd

AND

https://tinyurl.com/36bp9k85

Orkambi Eased Airway Inflammation, Boosted Bacterial Diversity

Treatment  with Orkambi (lumacaftor/ivacaftor) for six months lessened airway  inflammation and enhanced airway bacterial diversity in people with  cystic fibrosis (CF), but only when administered before patients were  chronically infected with the bacterium Pseudomonas aeruginosa. The  findings suggest that CFTR modulators should be started as soon as  possible, ideally before the patient is chronically colonized with P.  Aeruginosa. Since bacterial infections commonly occur in the lungs of  those with CF, the benefits of Orkambi also may be linked with changes  in the airways’ microbiota, the community of microorganisms living in  the lungs, and a reduction in inflammation. Import-antly, a correlation  between diversity of the microbiota and lung function was found, with  patients who had worse lung function showing significant increases in  the relative abundances of the bacteria Pseudomonas and Lautropia. Those  with a ppFEV1 of 80% or higher, showed an abundance of the bacterium  Streptococcus, Porphyromonas, Actinomyces, TM7x, and Peptostreptococcus.  In terms of fungi (mycobiota) diversity, no differences were seen  regarding age or lung function. Calprotectin — a measure of airway  inflammation — levels significantly increased with age and were  negatively correlated with lung function and bacterial diversity,  meaning higher levels of calprotectin associated with lower lung  function and less bacterial diversity. Calprotectin levels were  significantly higher — indicative of enhanced inflammation — in patients  with chronic P. aeruginosa infection when compared to those without it.  After six months of Orkambi treatment, patients had a significant  increase in weight when compared to baseline, as measured by the body  mass index (BMI). In patients not chronically infected with P.  aeruginosa at baseline, the levels of calprotectin were lower and the  bacterial diversity significantly increased after Orkambi treatment. No  differences were seen in fungi diversity.

https://tinyurl.com/munvhdn7

Orkambi’s Anti-Inflammatory Benefits Seen In A Real-World Study

Beyond  improving lung function, Orkambi (ivacaftor/lumacaftor) has potent  anti-inflammatory benefits that may limit immune-related lung damage in  people with cystic fibrosis (CF). Researchers found that one year of  treatment significantly reduced the levels of pro-inflammatory signaling  molecules in the bloodstream and airways of CF patients. Moreover,  measuring these molecules in blood and sputum is a relatively  non-invasive way to monitor disease-related inflammatory activity and  the impact of therapeutics. In the lungs, mucus build-up can trigger an  inflammatory response, resulting in lung damage. These responses are  characterized by elevated levels of pro-inflammatory immune signaling  proteins called cytokines in the lungs and bloodstream, likely  contributing to tissue damage. After three and 12 months of therapy,  there was an early and sustained improvement in mean lung function, and a  significantly reduced sweat chloride concentration, an indicator of  improved CFTR function. The need for intravenous antibiotics dropped by  68% in the first year of treatment compared with the year before, as did  the mean number of pulmonary exacerbations, and hospitalizations for  exacerbations, per patient. Blood tests showed Orkambi significantly  reduced the levels of three pro-inflammatory cytokines — TNF-alpha,  interleukin-1-beta (IL-1-beta), as well as interleukin-8 (IL-8), a  cytokine well known to attract large numbers of inflammatory immune  cells to the lungs in CF patients. No change in pro-inflammatory  interleukin-6 (IL-6) or anti-inflammatory interleukin-10 (IL-10) was  seen. Consistent with blood test results, Orkambi treatment  significantly reduced sputum levels of TNF-alpha, IL-1-beta, and IL-8,  but also of IL-6. No changes in IL-10 levels were detected. A comparison  of bloodstream cytokines to clinical features showed patients with  elevated IL-1-beta had worse lung function before and after one year of  treatment. Likewise, those with high levels of IL-6 had a lower body  mass index, a measure of body fat. Elevated TNF-alpha, IL-1-beta, and  IL-8 in sputum samples significantly correlated with more intravenous  antibiotic courses, as did high IL-6 and IL-1-beta in blood samples,  “suggesting subjects with high cytokine levels tend to exacerbate more  compared with subjects with lower cytokine levels. The researchers  concluded that in addition to ensuring significant improvements in  parameters of lung function, Orkambi therapy produced sustained  improvements in both circulatory and airway inflammation.

https://tinyurl.com/52hhaztt

Trikafta Rapidly Improves Lung Function In Real-World Study

Trikafta  significantly improved the lung function of cystic fibrosis patients  after only two weeks of treatment, with the benefits sustained after  nearly four months. Early LCI [lung clearance index] measurements can  help to assess the patient’s response to this high-cost therapy. LCI is a  test that involves measuring how long it takes for a tracer gas to be  cleared from the lungs. No significant improvements were seen with  Orkambi, however. Trikafta led to significant improvements in lung  function within the first two weeks of treatment, as measured by both  LCI and ppFEV1. These improvements persisted by weeks four and 16.  Patients with more severe disease at baseline, as shown by a higher LCI,  had the largest improvements after 16 weeks. LCI values after 16 weeks  significantly correlated with baseline LCI, but no such relation was  found for ppFEV1. Changes in LCI showed no association with baseline  ppFEV1.

https://tinyurl.com/5n8vhr6y

Elexacaftor  – Tezacaftor – Ivacaftor Treatment Improves Systemic Infection  Parameters And Pseudomonas Aer-uginosa Colonization Rate In Patients  With Cystic Fibrosis A Monocentric Observational Study

The aim  of this study was to methodically evaluate the effects of Elexaftor –  Tezacaftor - Ivacaftor (ETI) treatment on clinical, biochemical data and  Pseudomonas colonization in order to demonstrate its efficacy. Marked  improvements  were observed in biochemical markers of systemic  inflammation as white blood cell count, levels of immunoglobulins A, G  and M and albumin within 24 weeks of therapy. ETI treatment proved to be  effective as seen by amelioration of lung function and sweat chloride  concentration. Assessment of PsA colonization status revealed a  conversion from a positive to negative detection in 36% of the cases  after one year of therapy. Thus, ETI treatment effectively improves  systemic inflammation parameters and shows promising results in PsA  status conversion.

https://tinyurl.com/mrypjzr7

AND

https://tinyurl.com/kdc8v9ay

Eradication Of Mycobacterium Abscessus Infection In Cystic Fibrosis With Initiation Of Elexacaftor/Tezacaftor/Ivacaftor

Mycobacterium  abscessus is a nontuberculous mycobacterium that is often multi-drug  resistant, difficult to eradicate and associated with a rapid decline in  lung function in cystic fibrosis (CF). Elexacaftor/Tezacaftor/Ivacaftor  (ETI) is a combination CFTR modulator that improves lung function and  decreases exacerbations, but limited data exists about its impact on  respiratory infections. A 23-year-old male with CF (F508del, unknown)  was diagnosed with Mycobacterium abscessus subspecies  abscessusinfection. He completed 12-weeks of intensive therapy, followed  by oral continuation therapy. Antimicrobials were later discontinued  for optic neuritis secondary to linezolid. He remained off  antimicrobials with persistently positive sputum cultures. He then  initiated ETI, and bronchoscopy eight months later suggested eradication  of M. abscessus. By modulating CFTR protein function, ETI may improve  innate airway defense mechanisms, facilitating the clearance of  infections such as M. abscessus. This case highlights the potential  positive implications of ETI on the challenging treatment of M.  abscessus infections in CF.

https://tinyurl.com/zw44z834

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

Elexacaftor/tezacaftor/ivacaftor  (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. The  impact of ELX/TEZ/IVA was evaluated on the rate of lung function decline  over time by comparing changes in ppFEV1 in participants from the Phase  3 trials with a matched group of people with CF 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 compared with a 1.92 percentage  point annual decline in ppFEV1 in untreated controls. ELX/TEZ/IVA is the  first CFTR modulator therapy shown to halt lung function decline over  an extended time period.

https://tinyurl.com/2p8bp2j3

Real-World  Safety And Effectiveness Of Elexacaftor/Tezacaftor/Ivacaftor In People  With Cystic Fibrosis: Interim Results Of A Long-Term Registry-Based  Study

To  assess long-term effects of ELX/TEZ/IVA under real-world conditions of  use, a 5-year observational registry-based study is being conducted.  Interim results from the first 2 years of follow-up show that  ELX/TEZ/IVA treatment was associated with sustained improvements in lung  function, reduced frequency of pulmonary exacerbations and all-cause  hospitalization, increased BMI, and lower prevalence of positive  bacterial cultures. Additionally, there was a 72% lower rate of death  and 85% lower rate of lung transplantation relative to the year before  ELX/TEZ/IVA availability.

https://tinyurl.com/2x2hwsy3

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

Several  people with CF (PwCF) cannot benefit from  Elexacaftor-tezacaftor-ivacaftor (ETI) 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. Two main themes and  six subthemes were identified. The first main theme (being deemed  ineligible for ETI) had four subthemes (disappointment, information,  happiness, and concerns). The second main theme (coping with a life  without ETI) had two subthemes (lack of hope and continued hope). Thus,  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/2un8cdwk

Sex Differences In Outcomes Of People With Cystic Fibrosis Treated With Elexacaftor/Tezacaftor/Ivacaftor

The  effects of elexacaftor–tezacaftor–ivacaftor (ETI) use by sex prior to,  versus after initiation, of ETI by pulmonary exacerbations (PEx),  percent predicted forced expiratory volume in one second (ppFEV1),  presence of Pseudomonas aeruginosa in sputum cultures, and body mass  index (BMI) were evaluated. After treatment with ETI, there was a  greater decline in PEx in males versus females. No statistical  difference by sex for ppFEV1, presence of Pseudomonas aeruginosa or BMI  pre- to post-ETI by sex was found.

https://tinyurl.com/yccd57tb

Personality Traits May Influence Quality Of Life With CF: Study

The  personality traits of people with  cystic fibrosis (CF) might influence  their health-related quality of life (HRQoL). Scientists found that  patients could be clustered into two distinct personality groups and  that self-reported quality of life differed significantly between them.  Personality — each person’s unique pattern of characteristics and  behaviors — may have a substantial impact on how they respond to stress  and can influence life quality for people with chronic disease. But the  potential role personality plays in patients’ health and life quality  hasn’t been fully explored. Patients were split into two groups of five  distinct traits: negativistic (pessimism/resistance to others’  suggestions), schizoid (disinterest in social relationships), borderline  (difficulty regulating emotion), depressed, and paranoid (being on  guard). No specific personality trait was associated with clinical  factors, including lung function, life quality, body mass index, or  adherence to therapy. The patients in whom the traits were less  accentuated were found to have higher quality of life than those in whom  the traits were accentuated. Other health outcomes didn’t differ  between the groups. The researchers interpreted the findings as meaning  that patients belonging to the cluster with lower scores for those five  traits might be more “psychologically adjusted,” whereas those in the  other group are “vulnerable” to life quality declines. While the reasons  underlying the relationship aren’t clear, the scientists suggested it  could be related to resilience, or the ability to “bounce back” from  adversity and to cope well with difficult situations.

https://tinyurl.com/59x7ubct

Impact  Of Chronic Medication De-Escalation In Patients With Cystic Fibrosis  Taking Elexacaftor, Tezacaftor, Ivacaftor: A Retrospective Review

This  study evaluated the effects of de-escalating cystic fibrosis (CF)  supportive therapies in patients on elexacaftor/tezacaftor/ivacaftor  (ETI). For many with CF, the clinical benefit of ETI exceeds that of  supportive therapies. The primary objective was to assess  non-inferiority of supportive therapies de-escalation by comparing the  absolute change in percent predicted (ppFEV1) from baseline to month 1  versus the absolute change from baseline to month 12 after initiating  ETI with patients. The investigators found that de-escalating supportive  therapies for those on ETI was non-inferior to remaining on all  supportive therapies. This suggests that medications may be able to be  discontinued under the context of a de-escalation process, which may  decrease medication burden and cost and increase quality of life.

https://tinyurl.com/mr2484d5

AND

https://tinyurl.com/4u9z88en

AND

https://tinyurl.com/bd3pzr6h

Medication Use In People With Cystic Fibrosis Before And After Modulator Therapy.

Changes  in medication dispensings among people with Cystic Fibrosis (PwCF)  following modulator initiation were investigated, using national  prescription claims data in Australia. Immediately after modulator  initiation, the mean number of dispensings was 0.9 higher in the  modulator group, but then decreased to the level of controls after  approximately 5 years. Modulator initiation in PwCF was associated with  decreased dispensings of opioids and psychotropics, and increased  dispensings of women’s health medications, suggesting improved patient  outcomes across multiple clinical domains.

https://tinyurl.com/yawf7bvu

AND

https://tinyurl.com/5brtt56t

Cystic Fibrosis: Management And Monitoring Of Respiratory Manifestations

This  comprehensive article provides an overview of the pharmacological  management of respiratory symptoms, highlighting the support that should  be given to people with CF to enable them to obtain maximum benefit  from their medication regime.

https://tinyurl.com/4r7zvsxy

Cystic Fibrosis: Management Of Non-Respiratory Manifestations

This  article outlines the effects of CFTR dysfunction on the pancreas, liver  and gastrointestinal tract. It also discusses the pharmacological  management of these symptoms and describes the monitoring required for  treatment optimization.

https://tinyurl.com/34akuuts

Acute Pancreatitis In Pancreatic-Insufficient Cystic Fibrosis Patients Treated With CFTR Modulators

The  researchers present two cases of pancreatic-insufficient CF patients who  presented with acute pancreatitis shortly after commencing  elexacaftor/tezacaftor/ivacaftor modulator therapy. They propose that  highly effective modulator combination therapy may restore additional  pancreatic acinar activity, resulting in the development of acute  pancreatitis in the interim until ductal flow is improved. This report  adds to the growing evidence for possible restoration of pancreatic  function in patients receiving modulator therapy, and highlights that  treatment with elexacaftor/tezacaftor/ivacaftor may be associated with  acute pancreatitis until ductal flow is restored, even in  pancreatic-insufficient CF patients.

https://tinyurl.com/2zqc4jq9 

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.