I’ve  been back in the dating world for just a short time (since January of  this year) after a nearly nine-year hiatus. Before my double-lung and  kidney transplants in 2022, dating was just not possible. I was living  in the advanced lung disease stage—basically homebound, tethered to  devices and IVs all day, unable to walk even short distances on my own,  and my mental health was about as low as my lung function. To put it  simply, I lacked the physical ability and mental bandwidth to seek out  and maintain a romantic relationship. But around December of 2023, a  year and a half into my transplant recovery, the chaotic management of  my health began to settle down. With a quieter mind when it came to  managing cystic fibrosis, the sound of a “normal” experience like dating  began to rise.

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By  mid-January, a couple weeks after first downloading a dating app on my  phone, I was off on my first date. My personal online dating practice is  to keep the chatting through messages minimal before I meet someone. In  my experience, texting too much before meeting can lead to a false  sense of closeness and set a standard of communication that can become  hard to maintain when you have a chronic illness that drains your  energy. With messaging, I anticipated the subject of my health would  come up early. I’d inevitably be asked why I moved to San Francisco,  having listed my hometown as Anchorage, Alaska, and face the topic of  cystic fibrosis before really knowing someone. The reason I’d moved, to  be evaluated and eventually accepted for my transplants, was wholly  wrapped up in my health. How do you delicately and adequately present  that to a complete stranger?

Well,  that is a question I’m still learning how to answer. Navigating the  balance of how to open up but not overload has been my biggest area of  self-discovery in dating. With the knowledge that I’d likely approach  the subject of my transplants early on in an initial conversation, my  first approach was to bring it up before the other person led me there. I  felt getting the information out in the open would allow me to see if  that person could “handle” it before we made the effort to meet. In my  mind, saying it up front would, at the very least, weed out anyone who  may not want to proceed knowing I have a health condition. This was my  first lesson in dating, learned by trial and error.

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It turns  out, blurting out the fact that I received lung and kidney transplants  to a stranger in the middle of a casual conversation, especially through  a messaging app, just to get it over with, isn’t cute and flirty. More  importantly, it just didn’t feel right. This complex information that  I’d had severe health struggles felt worthy of a much higher level of  intimacy and trust than simply stating it in a message. Getting to know  someone isn’t a quick process. Sharing a big part of who I am shouldn’t  feel like dropping a bomb. The fact was that my transplants and what led  up to them were, and still are, one of the most personal parts of me.  It felt as though the approach I was taking of disclosing in a wave of  word vomit was a major overshare of a very raw reality that really  afforded much more privilege. My methods, I realized, needed  re-evaluating.

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Nine  months into my journey now, the way and when I share remains fluid. I  don’t believe there’s a “one size fits all” approach to opening up to  someone about my life with cystic fibrosis. There isn’t even a “one size  fits me” way, it seems. What I know for sure is that I always want to  hold true to who I am, whether I share every detail of myself with  someone or not. When and how I open up, especially about receiving organ  transplants, will depend on the person I’m sharing with, how the energy  feels between us, and the environment we’re in. If I’m on a dinner date  and I feel safe with the person, I might go into detail when they ask  what those capsules are as I pop my digestive enzymes into my mouth  before the meal. If I don’t know yet how I feel or the environment we’re  in doesn’t suit the conversation, I might just simply state their  purpose of digesting my food and change the subject. Dating is an act of  discovery about others, yes, but it’s also about discovering ourselves.  Honoring how I feel as I move through that act is as important as the  person with whom I choose to move through it.

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I was  recently seeing someone who knew what cystic fibrosis was before I had  to explain it. We had been on a few dates; I’d brought up that I’ve had  organ transplants and felt comfortable with his reaction. He didn’t make  it a big deal, didn’t apologize for the fact that I’d been through  that, but instead found it interesting and asked questions while  reassuring me he’d understand if I didn’t want to talk about it. To be  honest, I find it encouraging when people don’t shy away from asking  questions. Being curious is important. We were out one day when he  noticed some numbers I have in a tattoo on my forearm. He pointed at the  s F508 marking on my wrist and asked if that had to do with cystic  fibrosis. I’d not yet told him exactly what caused my need for  transplants and hadn’t said those two words to him. I eyed him a bit,  shocked, and asked, “wait, you know what that is?” and he replied that  of course he did. I wasn’t the first person he’d known with CF. Then, as  easily as he had asked about those numbers on my arm, we carried on.

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My point  is, when the feeling of safety with someone is there, things that seem  enormous will feel lighter. The topic of a complicated health history  (or whatever your personal hurdle may be) won’t feel like something that  needs to be kept hidden. Neither will it need to be forced out and  blurted into the ether to ease the anxiety that can come with being  vulnerable. The person you share with may or may not be in your life as  an everlasting partner but it will always be meaningful to share your  own story with a person who makes you feel comfortable, seen, and heard.  Allowing things to come up as they will, with purpose but without  forced vulnerability, is a lesson I’m happy I’ve learned.

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Ultimately,  dating has strengthened my boundaries while also opening me up in new  ways I wouldn’t have experienced had I not taken this leap. The reality  is, everyone in the dating world is weighing the balance of when and how  to share about their own life experiences. The one thing I’ve held in  myself is that I never want to lie or withhold the truth when it comes  to telling someone I have CF. It, and having had organ transplants,  affects so much of my life, that to omit them would be omitting  important parts of who I am. To truly allow someone to get to know me,  and in the act of getting to know someone myself, requires  vulnerability. It may not always be received the way I expect or come  out the way I intend, but being true to who I am and sharing honestly in  dating will always feel like the right choice for me.

Matison  is 32 years old and has CF. She was born and raised in Alaska, and  currently lives in San Francisco, where she received combined lung and  kidney transplants in 2022. She’s on the CF Foundation’s Rose Up  Committee; dedicates her time to advocating and spreading awareness for  CF, organ donation, and kidney disease on her social media pages; and,  in her spare time, she enjoys jigsaw puzzles. She can be contacted at mdeaton@usacfa.org and found on TikTok @onebreathatatime_ and on Instagram @matisondeaton.

For  some people, entering into a happy marriage means leaving behind the  complexities of dating in favor of a different landscape—one with its  own complexities that are often very different from those of other sorts  of intimate relationships. I’ve been married twice and just celebrated  eight years with my spouse at the end of June; we’ll mark 13 years  together this September. But like many couples, J and I have always had  an open relationship and marriage. This means that even though we’ve  shared our lives with one another for all that time, we’ve also dated  other people along the way.

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Sometimes  this practice of openness to intimacy with others has involved learning  hard lessons that can seem farther away from our daily reality with one  another. Lessons about what it means to care for someone and what it  means to support them. From the earliest days of our dating one another  after meeting through our Ph.D. program at FSU, J and I have maintained a  culture of genuine social support. I say “genuine” because as  sociologists, we understand that not every gesture intended as  supportive gets received as support by its recipient. True social  support involves interacting with people in ways that actually feel  supportive to them.

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Not  everyone in the adult CF community has experience with open  relationships. Indeed, some adults with CF have never dated at  all—whether for lack of opportunity or lack of interest. Yet from what  I’ve seen most of us do have experience with this broader phenomenon of  actions intended as supportive by others not always landing as support  for us. And in the context of intimate relationships, performative  “support” can take on some truly toxic dimensions that poison the whole  foundation of a partnership.

Sounds  terribly dire, doesn’t it? I wish I didn’t have direct lived experience  of this happening. That I didn’t still warily eye every cup of kindness  offered by another person for fear of deadly things lurking within it.  This doesn’t stop me from sharing of myself with other people. Entering  into another marriage even after several years of dating my spouse  required a substantial amount of “doing it scared” to begin with. I  don’t mind operating with a bit of fear in my heart. Doing so is simply  the wages of living a good life after trauma, in my experience. But that  fear sometimes looms very large. It has good reason to—because well  into my marriage I had another relationship that brought me face-to-face  with the damage “support” can do when focused more on the giver than on  the recipient.

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I’ve  hesitated to write this column, I should note. I don’t like speaking ill  of past partners, even if they abused me. That’s not what I’m going to  cover in this piece. Instead, I’m going to talk about a relationship  that showed me other depths of how bad something can go between two  people. How it can rot from the inside out after being built on poisoned  ground. I use situational language deliberately—talk of circumstances  rather than willful choices. I’ve always believed that people are  products of our own unaddressed trauma. The maxim “if you don’t heal  what hurt you, you’ll bleed on people who didn’t cut you” rings very  true for me. So as I share what I see as an essential message for fellow  adults with CF and the people who love us, I want to keep this  principle firmly in mind.

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Once  upon a time, I got bled on a lot. Eventually I had to leave that  relationship because there was nowhere else to go. At the time, it was  the great heartbreak of my adult life. That really is the crux of dating  when one is already happily married: No matter how warm and welcoming  one’s home life, there is always the potential for another heartbreak.  But how did Tennyson put it? ‘Tis better to have loved and lost than  never to have loved at all. I believe that absolutely. I value the time I  spent in the relationship that taught me these hard lessons. I also  still value the humanity of the person I once loved who gave me cause to  reflect on all this now. So I hope people reading this will as well.

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In this  past relationship of mine, I learned quite a few lessons about what I’ll  refer to as “toxic support” behavior. The situations that taught me  these lessons had certain things in common though—all of which I can  illustrate with one especially representative example. I think most of  us in the adult CF community are probably familiar with various  fundraising events that involve participants getting sponsored to do  some kind of physical activity together. The Great Strides walk  organized by the Cystic Fibrosis Foundation is probably the best known  of these, but there are several others.

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A bit of  background on the person I was dating may be helpful. As often happens  when people are wrestling with deep trauma of their own, certain  repetitive behaviors with a self-harming element became commonplace for  them. Oftentimes this took the form of endlessly consuming liquor,  something thankfully beyond the scope of this article. It still hurts to  remember watching that play out and how my own life got tumbled in the  waves of a heavily moralized health issue for which no form of help ever  seemed to stick. Not the first time I’ve been there with a partner.  Hopefully the last. I don’t relish seeing people I love tortured by  addiction.

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Addiction  can take many forms though. Sometimes people develop addictions to  substances, others to experiences. Often both occur simultaneously—and  in absence of substance use, addictions to repetitive behaviors that  fill the brain with massive amounts of mood-altering chemicals often  come to the forefront. The idea of “addictive personality” has some  truth to it. At the end of the day, people seek a certain balance of  chemicals in our brains and will do almost anything to get it. That  balance isn’t necessarily the same for every person; more consistent is  the relentless pursuit of what feels like equilibrium to us. I do it.  You do it. Everyone does it. Some attempts are simply more stigmatized  than others.

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Some  addictions are also silent. That’s probably true for many addictive  patterns observed in our own community as adults with CF—and really  fodder for a whole series of articles on its own. We’ve often not talked  about the diverse and intense experiences of addiction within our own  community. But from what I’ve seen, our own familiarity with struggle  and with the crushing weight of others’ disappointment as they realize  we truly will never “get better” in any standard sense makes us highly  attuned to similar experiences in the lives of our peers without CF.

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Unfortunately,  I suspect a lot of us have also observed firsthand the collision  between another person’s addictions and our own attempts to live as well  as we can with our disease. I imagine many of us have known the horror  of realizing someone we loved was trying to use our life-threatening  illness in service of their own self-harm. If you are walking that path  presently, or have in the past, I hope you can feel the empathy in my  words. And I hope that empathy lands meaningfully as support as you  read. I cannot go back in time and give myself that understanding—but I  certainly want to share it with all of you now.

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To make a  long and meandering story very short, and to explain the title of this  article fully in the process: A former partner of mine whose addictions  included alcohol and running decided that they would sign up for a  charity distance race benefiting a CF organization. They did this  purportedly for my own sake—and balked at my distinctly unfavorable  response. “But I’m doing this for you,” they said. I don’t think what I  actually said in response would be smart to print in CF Roundtable. So  I’ll summarize the main idea instead. Essentially, I reacted with horror  and anger to someone willfully co-opting my disease as rationale for  doing something “noble” that would invariably send them down a fresh  spiral of literally running themselves into the ground.

This  angered me for a number of reasons. Leaving aside the fact that I had  witnessed prior instances of this person nearly killing themselves with  excessive running—to the point of giving their own body an impressive  list of damages to various organs that would rival my own from the  genetic disease none of us have any choice but to live with—I also  recoiled from the idea that I need people to do stuff like this in my  name. That I need “saving” or that I should live my life as an object of  misaimed pity. This has never sat well with me.

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Perhaps  this comes in part from being raised by parents who trusted me to do  things on my own and who never treated me as dependent for having a  serious illness. They nurtured my autonomy in every way they possibly  could. Indeed, my parents always encouraged me to seek first to help  others—a philosophy I have admittedly operationalized to a dangerous  degree at times. But I would rather live my life putting others first,  even as I work to ensure that my own humanity shows up at least  somewhere in that ecology of caring. Trauma is absolutely a potent drug  on its own when it comes to caring for oneself, or even believing  oneself worthy of that care.

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I think  this incident about the charity run rattled me so deeply in part because  I had made tremendous strides by that point in understanding my own  worth. So, to be treated like a passive recipient of others’ noblesse  oblige—a French-derived term that specifically invokes the idea of  altruism as both an incontrovertible requirement and a hierarchical act,  a cosmic duty of tremendous moral weight that flows in only one  direction from the haves to the have-nots in a superordinate  sense—frankly made me see red. I spent quite some time trying to claw my  way out from the trauma of another situation in which I very much was  seen as less for being sick. I certainly didn’t need the reminder then  of how the world will view me at the slightest opportunity.

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The  irony of it also sickened me on a deeper and more personal level. I’ve  never had a serious relationship in which I haven’t been the primary  earner by a good margin, or the primary person providing instrumental  support with the basic executive functions of daily living. I have  received quite a bit of physical care from my spouse over the years  because they have earned the opportunity to provide it and have me  actually accept it. A huge part of that earning comes from their eager  embrace of what actually feels like support to me. Which certainly is  not treating me like an invalid who “needs” the “kindness” of someone  opportunistically using my CF as an excuse to hurt themselves right in  front of me.

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I could  say a lot of other things about this. I could rehash old anger and old  pain for pages on end. Yet more than anything else, I feel sorrow  looking back on all this now. Sorrow for me being in that position, and  sorrow for my former partner seeing this as a viable path forward.  Eventually I accepted that nothing I could ever say or do, no amount of  love that I could ever give and no amount of support that I knew full  well landed as such on their end, would suffice to make them show  genuine social support to either themselves or me. I had to reckon with  my own limitations as a person—what I perceived at the time to be my own  failures—and step away. I was still operating from that mindset of  responsibility to train other people to treat me like a human being.  Abuse will do that; it casts a terribly long shadow.

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So if I  could give one piece of advice to other adults with CF navigating dating  and relationships, whether from within an existing partnership or as a  single individual, it would be never to lose sight of what support means  for you. Don’t let other people guilt you into letting them use your  illness in service of their own self-destructive behavior. Stand firm in  your right to be a full person who gives as well as takes, and who gets  to set the terms of how others interact with your CF. After all, you’re  the one who lives with it day in and day out. You know your body best  on the physical front; you also know your own needs best on the social  one. Honor that inner voice whenever you can. You’ll find the world is  full of people eager for the chance to help you do that. This too, I’m  happy to say, is a lesson I’ve learned from experience.

Dr. Alexandra “Xan” Nowakowski is 40 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.

“Life’s  a dance, you learn as you go…” are the lyrics that softly roll out of  the speaker as I play fetch with my Siberian husky, Emma. It’s a  pleasant day in early autumn and the sunshine filters through the canopy  of pecan leaves overhead. Off in the distance, the honk, honk noises of  Canada geese can be heard, and the familiar v shape of their formation  grabs my attention for a minute. They perform a sort of dance as the  current leader falls to the back of the v formation and a different one  takes the lead. I pick up the stuffed duck toy and throw it out across  the yard once more. Emma’s face is the epitome of joy as she dashes back  through the crunchy leaves and drops the toy at my feet once more. I’m  reminded of how simple the relationship between a dog and his/her human  is and the pureness of such a friendship. But I suppose today I won’t be  writing much about the friendship of dog and man, although that would  be much simpler to do. Rather, I will share some anecdotes and insight  into human relationships. While I am still very much in the “learn as  you go” portion of life and have many lessons yet to uncover, I do hope  these writings will give others some food for thought.

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In my  most recent article in the Summer 2024 issue of CF Roundtable, I wrote  about my arduous preparation for my second 50-mile race and the  discipline required to prepare for such a race. I’m pleased to write  that I accomplished the task once again and enjoyed the moments and the  beauty of that day immensely. The plethora of emotions that wash over me  all at once as I cross the finish line of such an endeavor never ceases  to amaze me. This time around was no different. In fact, it was one of  the most special moments I’ve had at any race. Not only were two of my  childhood best friends there waiting for me, but their wives and young  kids were there as well, greeting me with cheers, hugs, and high fives.  Oh, how I wished 17-year-old me could’ve been there to see it.

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As most  can attest, the transition from childhood to adulthood, also known as  being a teenager, is quite a journey. It’s the time when most of us  transition from reliance and inexperience to growth and responsibility.  Chaos seems to reign over every aspect of our lives as we start our  first job, learn how to drive, discover new hobbies, and not least of  all, deal with bodily changes that bring insecurity to even the most  confident of individuals. All of this happens while we’re also learning  about love, friendships, and our personal desires and passions. One of  the most difficult struggles is finding where we belong—to find that  group of people who accepts us for who we are. A group that we can call  our tribe. Now, pursuing anything new and worthwhile in life almost  always comes with its own set of risks and of course relationships are  no exception. Whether romantic in nature or friendships of any kind, I  suppose the greatest fear we have is that scary monster we call  rejection. But what is it about rejection that is so terrifying and such  a behemoth to overcome? Is it simply because we long for a personal  connection? Is it because we desire acceptance and inclusion? Is it  because we want affirmation from others? I think all of these are true  and innate natural desires that we long to have fulfilled. However, I  believe, deep down in our heart of hearts, the reason we are paralyzed  by rejection is because it forces us to confront the true monster. What  or who is this ghastly beast exactly? Well, for me, it turns out it’s  myself, Marcus. The man in the mirror. As the adage goes, we truly are  our own worst enemy. The beauty of life though is that it doesn’t have  to be this way. I’ve learned if we take the time to understand  ourselves, sort out our own complications, and make peace with our inner  demons that we can move forward and upward with confidence in our own  personage. Now, does this mean we will never face rejection again?  Absolutely not. In fact, the opposite will likely be true. When we  become self-assured in who we are then we no longer strive to impress  others for fear of losing those people. Rather, our time and energy is  prioritized and given to those deserving of it. More on all this later.  First let’s dive into some of my fears as related to CF and understand  the validity of those fears.

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When I  was a teenager, my greatest anxiety with regards to rejection was tied  to the fact that I had cystic fibrosis. I did everything in my power to  ensure nobody knew about it. I’d skip taking my enzymes at mealtime when  out with friends and blame my incessant cough on allergies or another  bug flying into my throat just to be sure CF was never brought up in  conversation. Laughably, the following day I’d blame my stomachache or  indigestion on ‘something in the food’ rather than admitting it was due  to not taking my pills. I was terrified to know what my friends would  think about me if they knew this about me. Would they think me to be  weak or exclude me from the group because I’m “weird” or “sickly?” Of  course, looking back now, that seems so ridiculous a thought, especially  given that many of my closest friends at the time are still my closest  friends now, some 15 years later. Out of curiosity though, I had to ask  some of my longtime comrades about my CF and what they thought of it  then and now.

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I asked  Michael, my running partner and fellow adventurer, whom I’ve known since  our late teen years, if his perception of me changed when he learned  that I had CF and here was his response: “In a way it has. I appreciated  you as a friend before but knowing your story truly helped me  understand you on a much deeper level and inspires me to keep pushing on  even when life gets tough.”

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From  Aaron, the friend I’ve known since we were only knee high to a  grasshopper, came the following response: “I’ve known you had CF for as  long as I can remember and while I didn’t really fear losing you when we  were younger, it was still in my mind as a possibility. You having CF  definitely didn’t change my perspective of you by any means  and I would  just like to commend you that you haven’t let this be a crutch in your  life, but rather you have embraced it and seemingly draw strength and  determination from it to achieve things that very few ‘normal’ people  achieve in their lives.”

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Bruce,  the astute thinker of the group and the friend that always has sage  advice to offer had this to say: “I’ve always felt like you were just  one of the boys. If anything, I looked up to you because of the shoes  you had to wear. I felt like you had a different perspective on life  that I could only get from reading some heavy books. It also made me  think, ‘man the future isn’t a guarantee and I just want to get the most  out the time we have together.’”

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How  could it be, the people that know me the best, that have seen me in my  weakest and most vulnerable state still choose to accept and support me  anyway? Talk about humbling. Considering all this reminded me of a quote  from renowned Russian author, Fyodor Dostoevsky: “To love someone means  to see him as God intended him.”

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So, what  changed between deliberately hiding my CF to talking openly about it  and even writing about it here? Well, when I finally started sharing  with my friends and being open about my CF, I found zero rejection  whatsoever. In fact, there was nothing but genuine care, interest and  encouragement from my friends. In time, I found that the more I share  and invite people into this part of my life, the more they show up and  support me. While I still use a great deal of discretion with whom I  choose to share the most vulnerable parts of my life with, I have  learned that if I am vulnerable to some degree, I can very quickly  ascertain who is genuine and who will enrich my life. What’s more, if I  share deep truths about myself, many times people will also share about  their lives in return and thus a reciprocal relationship can be formed,  and a state of vulnerable trust can be established.

Back now  to facing that monster in the mirror and why I feel it’s so important  to deal properly with him. As far as I can tell we have two options and  the results for each seem to have universally shared outcomes. On one  hand, we can simply push away reality and refuse to face the dragon that  stands before us. From my experience though, when we choose this path,  we welcome resentment, shallow relationships, and self-doubt in almost  all endeavors in life. On the other hand, when we face our lot in life  head on and slay that beast then we set ourselves up to successfully  master the other beasts that are sure to come along in life. If we do  the work to develop the proper relationship with ourselves, then  connecting with other humans is far more likely to be a genuine and  wholesome experience and the rich rewards from that are beyond compare.  While this requires a great deal of effort and continuous and honest  self-assessment that can be painful at times, it pales in comparison to  the diabolical experience of doing life alone. Finally, I believe  building deep, meaningful, and long-lasting relationships to be the most  incredible and fulfilling thing we can do here on earth. After all, the  experiences we have with other people, the things we accomplish  together, and the memories we made are the only things that remain when  all the stuff in life is stripped away.

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Marcus  Miller is 32 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 by email at mmiller@usacfa.org.

Fall  is here and as I type this up I am gearing up for a few days in the  hospital. Not exactly excited about the prospect of starting IVs, but it  has been 8 years since I needed any due to my double lung transplant  almost 10 years ago. I find as I get older and further away from needing  regular medical intervention, my anxiety gets worse. My brain starts to  spiral into panic thinking about IV access, allergies to meds,  communication with my medical team, and what to pack to keep me  occupied. I’m not sure if anyone else experiences this, but these days  my brain is always in overdrive.  For now, I’m channelling that nervous  energy into writing up comfort food recipes!

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I was  going through food pictures when deciding what to write for this issue  and I came across my Swedish meatball recipe. To me, this meal screams  comfort, cozy, and ready for the cooler temperatures. I like to make  mine with mashed potatoes and pair with a sweet jam. If you can find  lingonberry sauce, use that. Otherwise, cranberry sauce or any berry  jam/preserve with work as a great condiment. If you are making this just  for yourself or less than 4 people, I still like to make the entire  batch of meatballs, freeze half of them uncooked and freeze half of the  gravy. I always enjoy having some meal prep in my freezer for when I am  not as energized to cook. I hope you enjoy!

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Swedish Meatballs in Gravy

Serves 4

Meatball Ingredients:

• 1 small yellow onion, grated

• 1 lb ground beef

• 1 lb ground pork

• 2 cloves garlic, minced

• ¾ cup panko breadcrumbs

• 2 eggs, lightly whisked

• 2/3 cup milk

• ½ tsp oregano

• ½ tsp allspice

• ¼ tsp nutmeg

• 1 tsp salt

• ¼ tsp pepper

• 2 tbsp olive oil

Gravy Ingredients:

• 4 tbsp butter

• 4 tbsp flour

• 2 cups beef broth

• ½ cube chicken bouillon or ½ tsp Better Than Bouillon, chicken flavor

• 2 tsp Worcestershire sauce

• 1 tsp Dijon mustard

• ½ cup sour cream or heavy whipping cream

• 1 tbsp fresh parsley, chopped

• Mashed potatoes or egg noodles, for serving

• Whole-berry cranberry sauce, for serving

​

Preparation:

Step 1:

In a large bowl combine the grated onion, garlic, panko, egg, milk, and spices. Give that a little mix.

Step 2:

Add both meats to bowl and gently mix to combine. Do not overmix as the meatballs will be tough and not tender.

Step 3:

In a  large deep-sided skillet or braising pan, add the olive oil and turn the  heat up to medium. Working in batches and without crowding the pan,  brown the meatballs on all sides, about 2 to 3 minutes per side. Once  the meatballs are browned, set aside on a paper towel-lined plate for  later.

Step 4:

Add the  butter to the same skillet. Once the butter is melted, add the flour and  whisk constantly until everything is incorporated and there are no  lumps. Let that cook for 1-2 minutes to cook out any raw flour taste.  This is your roux for the sauce. The gravy should be light brown and  slightly thickened.

Step 5:

Add the  beef broth, mustard, Worcestershire sauce, and chicken bouillon to the  pan. Whisk to combine. Bring the sauce to a boil and then reduce the  heat to low and allow to simmer for 3-5 minutes until the liquid has  reduced.

Step 6:

Stir in  the sour cream or heavy whipping cream. Add the meatballs back into the  gravy and let cook for 10-15 minutes or until meatballs are cooked  through. Garnish with the parsley and serve with mashed potatoes or egg  noodles and whole-berry cranberry sauce. s

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

©2024 Beth Sufian. All Rights Reserved.

CF Roundtable readers have sent in many questions this past month. Remember, nothing  in this column is meant to be legal advice and is only meant to be legal  information. 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.

Question:

I  receive Supplemental Security Income (SSI) benefits. Before I turned 18,  my mother was my representative payee. The SSI checks were in my  mother’s name but the money was to be used for my benefit. I am now 19  years old but my SSI checks are still made out to my mother. How can I  get Social Security to change the name on the SSI check so that I am the  payee of the SSI check?

Answer:

When an  SSI recipient is under the age of 18 the parent or a legal guardian is  the SSI payee. Once the SSI recipient turns 18 the person can receive  their own SSI payment. Some SSA offices require a letter from a  physician stating the SSI recipient is capable of managing their own  money in order to switch the name on the SSI payment to the person with  the disability.

After  the age of 18 a person can receive their own SSI check unless they are  not capable of managing their own money. For example, a person who has  significant cognitive issues may continue to have a parent payee or  legal guardian payee who receives the SSI check. The SSI check is  supposed to be used to pay rent, pay for utilities and food, and buy  other things the SSI recipient needs.

Question:

I  thought having a diagnosis of CF made someone automatically eligible for  SSI or Social Security Disability Insurance (SSDI) benefits. Social  Security tells me there is no such thing as an automatic eligibility for  Social Security benefits. Are they correct?

Answer:

Social  Security is correct. There is no automatic eligibility for SSI or SSDI  benefits for a person with CF or for a person with any other medical  condition. A person must have evidence that the person meets or equals  the Social Security medical criteria and non-medical criteria in order  to be approved and receive SSI or SSDI benefits.

Recently,  people in the CF community have been seeing incorrect information  online posted by some law firms. Those law firms incorrectly post  information that says a person with CF is automatically eligible for SSI  or SSDI benefits. There is no automatic eligibility for SSI or SSDI  benefits for anyone. Unfortunately, when people with CF or CF Center  care team members think eligibility is automatic people with CF try to  obtain benefits on their own and think it will be easy. This  misconception has resulted in thousands of people with CF being denied  benefits because the person with CF or their parents were not aware that  there are complex medical and non-medical criteria set out in the  Social Security Act and Social Security regulations. When people with CF  think they only need to tell Social Security that they have CF in order  to be approved for benefits, the person will have their SSI or SSDI  benefit application denied and they will then wait for anywhere from one  to three years but will never be approved for benefits.

Question:

I  thought if I did not have health insurance a drug company would have to  give me free medication if I need the medication to treat CF. I have  been told by some drug companies that they do not have to provide free  drugs to any patient.

Answer:

If a  person does not have health insurance there is no legal duty for a drug  company to provide free drugs to any person. Some drug companies do have  free medication programs for people who do not have a way to access  either health insurance or Medicaid/Medicare benefits. However, there is  no federal law that requires a drug company to provide free  medications. Some drug companies do not provide free drugs even if the  patient will become extremely sick or even die without the medication.

It is  important for people with CF to make sure they have health insurance  coverage. If a person must meet certain eligibility criteria in order to  be insured the person should make sure they understand what the  eligibility criteria is and make sure they meet the criteria. For a  person to be eligible for Medicaid coverage a person must meet certain  low-income and low asset criteria. If a person is married then Medicaid  looks at household assets as well as monthly income and the assets and  monthly income combined must be below a certain amount at any time. A  person with CF should make sure they know what the monthly asset and  income limit is in order to maintain Medicaid coverage. If a person  lives in a state that does not have Medicaid expansion for low-income  adults the person should ask their CF Center for other ways to access  health insurance coverage.

​

Question:

I have  had medical coverage since I was a baby. I just turned 19 and my state  Medicaid has stopped. I thought I had Medicaid coverage for life because  I have CF.

Answer:

No one  has guaranteed Medicaid coverage for life. A person must meet all income  and asset criteria at any given time. If the person does not meet the  asset or income criteria the person will lose Medicaid benefits.

There  are ten states that have not expanded Medicaid coverage to low-income  adults: Alabama, Florida, Georgia, Kansas, Mississippi, South Carolina,  Tennessee, Texas, Wisconsin, and Wyoming. This means that once a person  reaches the age of 18 or 19 (depending on the state), then Medicaid  (based on being a child who lives in a low-income household) will stop  at 18 or 19 years of age. In those ten states the ability to obtain  Medicaid, which would cover most CF medical needs, will require applying  for and becoming eligible for SSI benefits. If a person has SSI  benefits, a person becomes eligible for Medicaid coverage even if their  state has not expanded Medicaid to low-income adults. If a person is in  need of coverage and is not eligible for SSI and Medicaid is the only  option, but the state does not offer Medicaid expansion, a person could  move to a state that offers Medicaid to low-income adults.

In the  other 40 states, a person can obtain Medicaid benefits if they meet the  low-income and low-asset criteria for benefits. The person must be a  resident of the state that offers Medicaid to low-income residents. s

Beth  Sufian is 59 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.

One  of my good friends has been suffering with gastroparesis (GP) for  around eight years. When I write suffering, I mean when she is having a  flare-up—her gut is so painful that she can barely eat and even then,  only certain things. She gets to a point where she will start to vomit  and then when nothing is left, she dry heaves, which she tells me hurts  her ribs. This all leaves her incapacitated and extremely fatigued.

​

I have  watched her go through these flare-ups many times. I have seen what she  does: smaller meals, more often and no fiber. She doesn’t have CF or  diabetes. She spoke of GP, when it was bad, as something that made her  want to stop living.

​

Unfortunately,  I am now living my good friend’s life! I was diagnosed with GP two  months ago, in July, after undergoing a slew of GI tests. First, I had  to do a barium swallow test, which is an x-ray test used to examine the  esophagus for things like reflux. I had to swallow a barium solution so  the radiologist could follow the movements of the liquid in my esophagus  and see how the different structures reacted to swallowing. I also had  to do a gastric emptying test, which measures the amount of time it  takes for food to empty from my stomach. The doctor then ordered a Bravo  pH monitoring study (via an endoscopy) to measure the acidity in my  esophagus for 96 hours. All of this GI testing came about because I was  treated in the hospital with thymoglobulin for rejection in May 2023 and  acid reflux can be a cause of rejection. Even though I had already had a  gastric emptying test in 2015 (with normal results), this recent one  was abnormal and showed very delayed emptying. Plus, both my swallow  test and the Bravo study were positive for reflux.

​

Why is  this so important? When a person has had a lung transplant, the  pulmonologists and surgeons worry that if that person also has acid  reflux, the acid could travel up from the stomach through their  esophagus to their transplanted lung(s), thus causing infection or  rejection. The most secure way to fix acid reflux for lung recipients is  a surgery called Nisssen fundoplication. Surgeons create a sphincter,  which essentially is a tightening muscle. In other words, it’s a stomach  wrap. That newly-created muscle at the bottom of the esophagus prevents  acid reflux by restricting the flow of acid upwards from the stomach  through the esophagus. I was told that most transplant centers perform  this surgery soon after transplant to prevent reflux from happening,  even if the patient is already on antacid medication.

​

After an  endoscopy, a colonoscopy, and the Bravo study, I met with my GI doctor  who told me that while the results of the Bravo study was still  unavailable to her, she could tell that my esophagus was not that  scarred and she was subsequently not so worried about acid reflux. Also,  I never felt the discomfort from it. She noted that if my level of acid  reflux was above 6% I would be a candidate for a Nissen fundoplication  surgery to stop the acid from leaving my stomach. The possibility of  having to undergo that procedure didn’t excite me. I have talked to  others who had this surgery—a few people told me it helped them  tremendously while others said it was a slow and painful recovery.

​

During  this same appointment, I mentioned to my GI that in the past I got these  horrible, dull pains in my stomach, ones that lead to sharper pains  that crept in from the sides of my ribs. I also mentioned that if I did  not take an antispasmodic right away, I inevitably wound up in the ER  because I couldn’t stop vomiting for several days. From this, I got  dehydrated and my liver enzymes skyrocketed. During one of those  episodes, the doctors thought I had a stone in my biliary duct; however,  nothing was wrong after a few days of IV fluids. Still, I had to remain  in the hospital. Basically, mayhem ensues and I am unable to get out of  bed, take my immunosuppressants, or drink anything without puking. She  told me, “You have gastroparesis, maybe even more severe than I thought.  While your gastric emptying test showed signs of slow emptying, since  you didn’t complain, I did not think it was so bad.”

​

Then,  things got worse. After she received the Bravo study download, it turns  out that I have over 8% reflux which made me a prime candidate for the  stomach wrap. Thankfully, she wanted to present my case to the  transplant team, which is comprised of transplant pulmonologists and  cardiothoracic surgeons, to find out what course of action they thought  was best. She didn’t like the idea of doing this surgery on someone who  is 24-years-post-lung transplant. It turned out, neither did they. I  heartily concurred.

​

Instead  of the surgery, she suggested I take a proton pump inhibitor (PPI) for  acid reflux. I already take one every day anyway—except during the Bravo  study because it was forbidden. If I felt full all the time, and I did,  maybe giving me a motility drug would be beneficial. When one feels  full, and motility is slow, acid can only go upwards—which is bad for a  lung-transplant patient. If my stomach could empty faster, I’d have less  acid accumulation. But then, as I indicated, things got worse because I  suddenly got constipated. I had no idea what was going on as I had  never realized I was full of stool, either currently or on prior  occasions. However, my GI told me that it has been seen and noted in  previous scans and X-rays.

​

She  suggested I start taking Miralax twice a day. This only helped so much. I  know most people with CF already know to use Miralax, but I just really  hated feeling like I had diarrhea daily. During this time, I had severe  pain and cramping and ultimately lost my appetite. She suggested I try  milk of magnesia and that, in conjunction with Miralax, definitely  helped. I was afraid to eat things I normally ate because I wasn’t sure  what was causing me so much pain. I also learned from reading various  online threads that fiber, which I used to eat a lot of in leafy greens  and beans, cannot be digested in patients with GP. It can slow gut  motility even further. What was happening? To combat my rather rapid  weight loss, I bought a bunch of protein shakes just to get calories in  me.

​

I felt  the worst while my GI doctor was away on vacation. Each day my gut was  cramping and I slept a lot because of the constant pain. My good friend  was there for me to tell me what worked for her: “My best friend is my  heating pad.” “Be kind to yourself and rest if you need to.” Both are  easier said than done but I had no choice as everything was an effort. I  rested and used a heating pad plus rubbed my stomach counterclockwise,  breathed deeply into my diaphragm, and practiced reiki. Everything  helped minimally. I was frustrated.

​

When my  GI came back from vacation, she started me on something called Linzess.  She also told me to stop taking Miralax while taking this new drug  because Linzess is commonly prescribed to help with constipation;  however, it can cause cramping. And yes, it did! I had even more extreme  stomach cramping than before. Supposedly the cramping will improve  within a month. In order for the GI to give me one of the new motility  drugs, I had to try Linzess first, otherwise insurance would not cover  the other motility drug.

​

Right  now, I am waiting to get a pH-impedance test to see if I now have acid  reflux while on pantoprazole, one of many PPIs on the market. This is a  24-hour test that determines when and if I have reflux. A probe, which  extends down to my stomach entrance, is inserted in my nose and remains  there for 24 hours. This catheter records data during that time period. I  will wear a recording box and note on a piece of paper all my symptoms,  even when I am laying down. I will also have an esophageal manometry  test for swallowing. This test measures the muscle contractions of my  esophagus as water moves through to my stomach. If I show acid during  the pH test, I will be scheduling a meeting with a cardiothoracic  surgeon to talk about the Nissen fundoplication surgery. I want to avoid  this surgery if possible. Recovery is difficult and some have told me,  eating can be a challenge afterwards. One of my joys in life has been my  food and cooking. I do not want to stop enjoying my gustatorial  delights.

​

I am  waiting to start the motility drug, ideally before I get the two tests  described above. If I can start that before the pH test, and have less  acid in my gut, hurrah! I may avoid the surgery and get things “moving  in the right direction.”

Another  wrinkle stemming from all of this was my history of post-transplant  lymphoproliferative disorder (PTLD), which I’ve had twice. PTLD can  return in the abdominal area so I had to have a PET scan to rule out  cancer. This caused me to worry. It was possible that I had cancer again  because, to me, these GI symptoms came on so suddenly, as symptoms of  cancer can do. However, being a seasoned CFer, I tried to worry only  when necessary to conserve energy. So, I compartmentalized it as “to be  continued.” Unpack it if need be. It turns out, my scan was negative, or  at least that was how it read to me. Of course, it would be lovely to  get confirmation from someone with an M.D. after their name.

​

I know  many people with CF have motility issues; it was never my thing, or at  least I thought. My GI told me that most people with CF and transplant  are prone to GP because the vagal nerve can be cut or severed during the  lung transplant surgery. I was blissfully unaware. I am constantly  reminded of how cruel CF can be. I can look at it in a glass half full  sort of way—it took around 24 years after my transplant for GP to  develop to this point. I will try to be grateful that I now know about  all these new GI symptoms and try to still enjoy the food I can, albeit  mindfully.

Andrea  Eisenman is 59 years old and has CF. She is the Executive Editor of  USACFA. Andrea just celebrated her 24th lung transplant anniversary. She  and her husband, Steve Downey, live in NYC with their three  dogs—Roscoe, Trixie, and Willie. Her contact information is on page 2.

I  have had these nodules in my lower right lobe for about 10 years now. I  get a CT scan every year and they have never changed, until this year.  One nodule was denser and one was a bit bigger. To be on the safe side,  my lung transplant clinic wanted me to get biopsies of the nodules.  After the biopsy procedure, I thought I heard the surgeon say that he  looked at the biopsies under the microscope and one (from the denser  nodule) looked cancerous. On the way home, when I was a little more with  it, I asked my husband Scott, “Did the doctor just say I have lung  cancer?” Unfortunately, yes.

​

Luckily,  things moved quickly after that. I met with the lung surgeon, the lung  oncologist, and the lung radiation oncologist all at the same time. They  described my options and agreed that my best option was the removal of  my lower right lobe. I was on board with that too because radiation  would only target the cancerous nodule and the other spot would be  monitored. There were too many risks and time associated with radiation  for that to be a good option for me. Surgery was scheduled for  07/24/2024, two weeks after my meeting with the doctors.

​

Immediately  following my consultation, I had a brain MRI which showed a potential  clot on one side of my head. The MRI had to be assessed by multiple  doctors, including a neuroradiologist. Additional tests were done and  the doctors determined that it was a small, old clot and nothing to  worry about in terms of my upcoming surgery. I did not have the head  space to deal with this (no one wants to think about having a clot in  their brain) as I was just focused on getting through the surgery. I  also had a PET scan to make sure I didn’t have cancer lurking anywhere  else.

​

The  surgery was uneventful except it took three hours instead of two because  of all the scar tissue they had to go through. I was in the hospital  for three days and went home on Dilaudid for my pain. The pain wasn’t  too bad but I did have to add in Tylenol (as suggested by the oncology  nurse) for better pain relief. On a Friday two weeks after my surgery I  asked the nurse for a refill of the Dilaudid. That did not go over well;  she explained that “most people” do not need more pain medicine “this  far out from surgery.” I told her I didn’t appreciate being treated like  a drug addict, that I was still in pain and needed the medicine. She  prescribed exactly nine more pills for me, even though I was supposed to  take them every four hours (I was taking them more like every eight  hours to begin with.) She also scheduled a virtual appointment with the  surgeon for the following Monday. There was no apology for treating me  like a drug addict.

​

I didn’t  realize how much this bothered me at first because I was really mad.  Then, I was so bothered by it I stopped taking the Dilaudid altogether.  I’m not sure why I stopped taking it; maybe because I was made to feel  like a drug addict! Regardless, I should have continued taking it  because I felt like crap the next day. Concurrently with this situation,  I was told I had Aspergillus in my biopsied lobe so I started taking  Cresemba at the same time I stopped the Dilaudid. I’m not sure what  exactly made me feel so ill—stopping the Dilaudid or starting the  Cresemba—but I lost my appetite and was exhausted.

​

At my  virtual appointment with the surgeon on the following Monday, I cried  through the entire appointment. I am not a big cryer; this surprised  even me. But that’s how much this implication of being a drug addict  affected me. I told the surgeon all about what happened and he agreed  with me that yes, I should still be in pain because it was an unusually  complicated surgery. I told him he needs to explain this to his nurse,  that instead of treating us like drug-seeking patients she needs to know  that we are not all the same when it comes to pain. He agreed and  suggested that my pain management should be handled by the lung  transplant clinic since they know me and know I’m not a drug addict. I  happily agreed.

​

Little did I know this would be the least of my problems. More to come in the next issue!

Colleen  Adamson is 55 and has CF. She is the Treasurer of USACFA, and lives in  Alexandria, VA with her husband Scott. Her contact info is on page 2.

“I  can’t breathe and I cough a lot, but I have a great smile,” is my go-to  pick up line. It’s a great segue into the reality of dealing with  someone who has cystic fibrosis.

​

I think  the biggest decision when starting to talk to someone seriously is when  to tell them about a chronic illness that won’t go away. Do you tell  them at the beginning and get it over with, do you wait until you’re  somewhat serious to tell them, or do you just wait until you get sick to  then explain to them that you have an illness? All of these are neither  right nor wrong, it’s just a matter of when the chronically ill person  feels it is the right time without the other person thinking that they  weren’t being honest with them or hiding something so critical from  them. I personally tell people straight away—because if they don’t want  to deal with it they can go about their merry way; however, if they are  accepting of and eager to learn about it, then let’s get the party  started! One time when I told someone about my CF they said, “oh I can’t  be around that; I can’t handle all that; I wish you the best.” I was  shocked by the response because it was the first time my CF actually was  the reason that someone didn’t want to be with me (well, at least the  first time someone said so to my face.) I thought to myself “how he can  he say he can’t deal with it when, I’m the one living with it!” It made  me mad, sad, and confused, but in the end, I was able to get over it  because there are so many people in this world and he obviously wasn’t  meant for me. Telling men right away will weed out the non-serious and  immature ones. There were probably several other men who didn’t want to  be with me because being with someone who has CF comes with a lot of  sacrifices and you start thinking to yourself “why would someone choose  the sick girl or the girl with the illness when there are perfectly  healthy people they can date?” It’s a sad thought, but it crosses my  mind a lot when dating. It makes it even worse when it happens to you.

​

Cystic  fibrosis is a job within itself—unless you stick to the necessary  treatments and meds, your health won’t get any better or any more  stable. Dating is also a job—unless you stick to it and continue putting  yourself out there, you won’t get anywhere. Dating is very similar to  CF in that regard. Dating sometimes felt annoying and honestly required  more energy than I had to give. Having CF, we are already fatigued as it  is and it takes more energy than the average person to do all  activities (including breathing) so it can make it hard to want to date  when you think of the extra energy needed for it. However, when doing  the job well, such as when you’re having great lung function and  successfully maintaining your health, it seems as though you get more  attention from guys versus when you’re not doing so well on the job  (you’re sick, you’re in the hospital, your lung function is lower,  you’re coughing more), the men tend to retreat and not approach you. It  can make it hard to see who is genuine and who will be there for the  long journey, not just when things are going well. I have been pretty  stable for the past few years and it’s crazy the attention I get from  men. I never had this in prior years when I was in and out of the  hospital multiple times. Who doesn’t love the attention? But let’s face  it—the attention is based on physical appearance. I may have CF but CF  doesn’t have me. That’s the crazy part about an invisible illness—you  can look completely fine on the outside, but really you could be dying  on the inside. The attention can draw someone in, but will it stay and,  do I want to find out? Sometimes the answer is yes and sometimes it’s  no. As far as dating goes, I feel like I go through waves—trying and  then choosing not to try. Sometimes I must focus on keeping my health  stable and adding in a relationship would only complicate it and cause  me to lose focus. At the end of the day, it’s all about balance and I  have been doing that all my life when it comes to treatments, managing  my CF, completing college, having a full-time job, and now throwing  dating into the mix. It can be an adjustment and be overwhelming, but  I’m used to that part. Cystic fibrosis is overwhelming but I do find  that having someone who likes you for you and will be there to support  you in sickness and in health is really important (and also rare).

​

Dating  is already hard enough as a person who doesn’t have cystic fibrosis, but  for people with CF it’s even harder. It’s harder for several reasons.  Primarily for me, CF is not always pretty, which sucks because I like to  be cute all the time. Let’s face it, cystic fibrosis is not cute a lot  of the time. Coughing up green sticky mucus, for example. Real cute,  right? And the scars that I have on my body from multiple procedures and  multiple surgeries. Let’s not forget the bruising from IVs I’ve had  too. It can make you feel ugly; yes, but they are battle wounds and I am  so proud of them, even if they are not pretty to look at. They are part  of my story, one that I am forever blessed to have made it through but  it can make me self-conscious at times. Sometimes I do try to cover them  up, but then I also think I need to expose them because people need to  see that it’s okay to have them and that you are still fabulous and  attractive to them. It’s not about what’s on the outside, but what’s on  the inside that should truly matter; however, I can admit it makes it  difficult sometimes in the beginning because a lot of dating starts out  with whether you are physically attracted to the person and scars can be  a deterrent. I know they shouldn’t be but that is the reality and it  can actually weed out the non-serious men.

​

Another  reason dating when you have CF can be harder is the vulnerability that  comes with it. It’s already hard for the average human to be vulnerable  with another human as trust is hard to come by these days. But with CF,  there is no choice to be or not be vulnerable. I don’t have a choice to  skip meds, skip treatments, or skip doctor appointments so someone I  would potentially date would see me taking pills and doing treatments or  using an inhaler. It’s a part of my everyday routine so I must be  vulnerable because hiding it would be doing myself a disservice as not  taking my meds or skipping treatments can be detrimental to my life. I  have found that doing all of these in front of someone I’m dating really  humbles them most of the time and they even want to learn more and help  me out in any way they can. Most people don’t see these things, so I  can teach them a lot and they can have eye-opening experiences. It draws  me closer to people I’m talking to or dating because of the  vulnerability.

​

Overall,  dating is an adventure that you must mentally prepare for when you have  cystic fibrosis. It’s not as easy as someone without CF, and it takes  confidence and strength to put yourself out there. I think about it this  way—if I have the courage to have tubes go into my nose then down into  my stomach, get poked and prodded all day long, and have my skull get  cut into, then dating is another hurdle and that doesn’t seem so bad  compared to what I’ve been through already. Cystic fibrosis has taken a  lot from me but it has also given me so much—it has taught me to take  risks and not let it dictate my life, especially when it comes to  dating. I’m wiser because of my CF, I’m more vulnerable, and I’m not  scared to put myself out there.

Mariah  is 27 years old and has CF. She currently resides in New Jersey. Mariah  is a full-time accountant who graduated from the University of  Michigan. She is always crunching numbers and always saying Go Blue.  When she’s not crunching numbers and eagerly watching her Wolverines  play, you will most likely find her at the bowling alley. Mariah is in  three bowling leagues and has participated in tournaments. She also  enjoys roller skating as it’s a thrill skating down the rink. You can  contact her at mariahcaise1996@gmail.com; she is always eager for a chat.

The U.S. Adult CF Association (USACFA) is pleased to announce the recipients of the Higher Education  Scholarship.

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In our  evaluation, 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. The scholarship  is open to all pursuing any degree, from associates to Ph.Ds. We  believe that any higher education is a strong foundation for advocacy  and involvement in the CF community.

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

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We are  pleased to announce Monserrat Tejeda-Munoz and Grace Lidgett as the two  winners of the scholarship for the 2024-2025 academic cycle. They were  each awarded $2,500. Congratulations to both!

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Monserrat  is a freshman at the University of Georgia in Athens, Georgia with  plans of majoring in biology and becoming an anesthesiologist. She hopes  to use both her bilingual and leadership skills to open a clinic in the  underserved rural city of Fort Valley, Georgia. Living in a city with  limited access to healthcare facilities and with a high number of  Hispanics who do not speak the English language has inspired Monserrat  to pursue a career that will help foster change in her community.

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Grace  Lidgett is a sophomore at Grace College and Seminary in Winona Lake,  Indiana. She dreams of becoming a graphic designer and creating art from  her varied life experiences. In high school, she was active in both  track and cross country, ultimately becoming a 12-time state qualifier  and a five-time state medalist between the two. Grace is still a  long-distance runner and loves how her reasons for running have evolved  with time. She has coordinated with the Iowa Chapter of the Cystic  Fibrosis Foundation on various projects including a website graphic  banner, a fundraiser tote bag design, and a large-scale art piece  describing “What does it mean to breathe?” Grace has also partnered with  AbbVie on a digital media promotion. Grace is committed to  participating in multiple research trials, even while enrolled in  school.

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Both  scholarship winners demonstrated the leadership, intelligence, and drive  of Lauren Melissa Kelly. All of us at USACFA look forward to seeing  them further develop their leadership and advocacy in the cystic  fibrosis community.

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

Allergic Bronchopulmonary Aspergillosis In A Lung Transplant Recipient Treated With Mepolizumab

Allergic  bronchopulmonary aspergillosis (ABPA) is a complex hypersensitivity  reaction to Aspergillus spp. ABPA diagnosis may be challenging due to  its non-specific presentation. Standard ABPA treatment consists of  systemic corticosteroids and antifungal agents. Mepolizumab, a  monoclonal antibody against interleukin-5 seems to be a promising  treatment for ABPA. Data about ABPA following lung transplantation  (LuTx) are scarce. LuTx recipients are at higher risk for adverse  effects of ABPA treatment compared to the general population. Presented  here is a case of a LuTx recipient who was successfully treated with  mepolizumab for ABPA following LuTx. Prolonged administration of high  dose prednisone was thus avoided. To these researchers knowledge, this  is the first case describing mepolizumab administration following LuTx.  Mepolizumab seems particularly attractive as a corticosteroid-sparing  agent or as an alternative option to antifungal treatments, because of  its excellent safety profile and low risk of drug interactions.

https://tinyurl.com/bder4x9y

Extent Of Fetal Exposure To Maternal Elexacaftor/Tezacaftor/Ivacaftor During Pregnancy

The risk  of ETI (Elexacaftor/Tezacaftor/Ivacaftor) to fetuses remains unknown.  Thus the effect of maternally administered ETI on fetal genetic and  structural development was investigated. Pregnant Sprague Dawley rats  were orally treated with ETI for 7 days. Tissue samples collected at the  end were analyzed using histology and RNA sequencing. Histological  changes and differentially expressed genes (DEG) were assessed. No overt  structural abnormalities were found in fetal pancreas, liver, lung and  small intestine after 7-day ETI exposure. Very few non-functionally  associated DEG in fetal liver, lung and small intestine were identified  using RNA-seq. 29 DEG were identified in thymus and most were  functionally linked to each other. Gene ontology enrichment analysis  revealed that multiple muscle-related terms were significantly enriched.  Many more DEG were identified in the cortex and a group of these were  involved in central nervous system and brain development. In conclusion,  sub-chronic ETI treatment in late pregnancy does not appear to pose a  significant risk to the genetic and structural development of many fetal  tissues. However, significant gene changes in fetal thymic myoid cells  and cortical neuronal development requires future follow-up studies to  assess the risk to these organs.

https://tinyurl.com/bdeww8zj

Widespread  Alterations In Systemic Immune Profile Are Linked To Lung Function  Heterogeneity And Airway Microbes In Cystic Fibrosis

Excessive  inflammation and recurrent airway infections characterize people with  CF (pwCF). How the overall immune response is affected in pwCF, its  relationships with the lung microbiome, and the source of clinical  heterogeneity have not been fully elucidated. In pwCF, researchers found  a specific systemic immune profile characterized by widespread  hyperactivation and altered frequencies of several subsets.  Concentrations and frequencies of key immune cells and marker expression  correlated with the relative abundance of commensal and pathogenic  bacteria in the lungs. In conclusion, the CF-specific immune signature  is a potential source of lung function heterogeneity. The activity of  specific microbes contributes to disrupting the balance of the immune  response. This data provides a unique foundation for identifying novel  markers and immunomodulatory targets to develop the future of cystic  fibrosis treatment and management.

https://tinyurl.com/24yzmjrn

Impact Of Extended Elexacaftor/Tezacaftor/Ivacaftor Therapy On The Gut Microbiome In Cystic Fibrosis

There is  very little knowledge on the longer-term effects of CF transmembrane  conductance regulator (CFTR) modulator therapies upon the gut microbiome  and associated outcomes. In a pilot study, these researchers  investigated longitudinal Elexacaftor/Tezacaftor/Ivacaftor (ETI) therapy  on the gut microbiota, metabolomic functioning, and clinical outcomes  in people with CF (pwCF). Results showed extended ETI therapy increased  core microbiota diversity and composition, which translated to gradual  shifts in whole microbiota composition towards that observed in healthy  controls. Despite becoming more similar over time, CF microbiota and  functional metabolite compositions remained significantly different to  healthy controls. Antibiotic treatment for pulmonary infection  significantly explained a relatively large degree of variation within  the whole microbiota and rarer satellite taxa. Clinical outcomes were  not significantly different following ETI. In conclusion, while  differences persisted, a positive trajectory towards the microbiota  observed in healthy controls was found. Researchers recommend future  studies use integrated omics approaches within a combination of  long-term longitudinal patient studies and model experimental systems.

https://tinyurl.com/5n8m8xpe

Maternal And Fetal Outcomes In Multiparous Women With Cystic Fibrosis

Maternal  and perinatal outcomes in women with CF (wwCF) are similar to those  seen in the general population. However, the effect of undergoing  multiple pregnancies is unknown. A greater decline in ppFEV1 was seen in  multiparous women, primarily in pancreatic insufficient wwCF and those  with two severe mutations. Multigravid pregnancies were shorter,  especially in wwCF over 30 years old, who had high rates of prematurity  and newborn complications. There was no effect on pulmonary  exacerbations or disease-related complications. In conclusion, multiple  pregnancies in wwCF are associated with accelerated respiratory  deterioration and higher rates of preterm births. Therefore, strict  follow-up by a multidisciplinary CF and obstetric team is needed in  women who desire to carry multiple pregnancies.

https://tinyurl.com/5y8nfcst

Changes  In Vitamins And Trace Elements After Initiation Of Highly Effective CFTR  Modulator Therapy In Children And Adults With Cystic Fibrosis – A  Real-Life Insight

Highly-effective  CFTR-modulator therapy with elexa-/teza-/ivacaftor (ETI) has led to  improvements in pulmonary outcomes, sweat chloride, BMI and quality of  life in people with CF. Improved uptake of fat-soluble vitamins and  micronutrients has been reported for CFTR-modulators but data regarding  ETI therapy is lacking. This single-center retrospective study evaluated  FEV-1, sweat chloride, BMI, transaminases (AST, ALT), bilirubin,  vitamins A, D, E, zinc and selenium in children and adults eligible for  ETI. Parameters were assessed before and up to one year after initiation  of ETI. FEV-1 and sweat chloride improved significantly after ETI.  There were no changes in BMI or AST. ALT was increased significantly  after 4 weeks of ETI but returned to normal levels in further course.  Bilirubin levels remained elevated after ETI. Vitamin A was  significantly higher 12 months after ETI. No changes were found for  vitamins D, E, zinc and selenium. This study adds to the evidence that  improvements of some fat-soluble vitamin levels can be found after ETI.  No changes regarding micronutrients were noted. Individualized follow-up  and supplementation are recommended

https://tinyurl.com/54ebpers

Subclinical  Vascular, Hemodynamic And Arterial Stiffness Changes In Adults With  Cystic Fibrosis: Cross-Sectional Observational Study

Cardiovascular  diseases can be an emerging complication in cystic fibrosis (CF), as  the median life expectancy has improved considerably. The objective of  this study was to compare vascular, hemodynamic parameters and arterial  stiffness in adult CF patients with healthy participants paired by sex  and age, and to assess the factors associated with arterial stiffness in  the CF group. This is a cross-sectional observational study. The  evaluation of cardiovascular parameters was performed non-invasively  using Mobil-O-Graph. 36 individuals with CF and 35 controls were  evaluated. The mean arterial pressure, cardiac output, and systolic  volume ml, were significantly lower in the CF group. The heart rate was  higher in the CF when compared to the control. The augmentation index  was higher in the CF than control. Individuals with CF presented lower  arterial blood pressures and changes in cardiac function with lower  stroke volume and cardiac output. The AIx@75, an indirect index of  arterial stiffness and direct index of left ventricular overload, is  increased in this population. The subclinical findings suggest the need  for earlier cardiovascular assessment in this population due to  increased risks of cardiovascular disease.

https://tinyurl.com/357mmdhy

Microbial  Community Organization Designates Distinct Pulmonary Exacerbation Types  And Predicts Treatment Outcome In Cystic Fibrosis

Polymicrobial  infection of the airways is a hallmark of obstructive lung diseases  such as CF, non-CF bronchiectasis, and chronic obstructive pulmonary  disease. Pulmonary exacerbations (PEx) in these conditions are  associated with accelerated lung function decline and higher mortality  rates. Understanding PEx ecology is challenged by high inter-patient  variability in airway microbial community profiles. Researchers analyzed  bacterial communities in 880 CF sputum samples collected during an  observational prospective cohort study and developed microbiome  descriptors to model community reorganization prior to and during 18  PEx. They identified two microbial dysbiosis regimes with opposing  ecology and dynamics. Pathogen-governed PEx show hierarchical community  reorganization and reduced diversity, whereas anaerobic bloom PEx  displays stochasticity and increased diversity. A simulation of  antimicrobial treatment predicts better efficacy for hierarchically  organized communities. This link between PEx, microbiome organization,  and treatment success advances the development of personalized clinical  management in CF and, potentially, other obstructive lung diseases.

https://tinyurl.com/45h6yhcp

In Vivo Editing Of Lung Stem Cells For Durable Gene Correction In Mice

In vivo  genome correction holds promise for generating durable disease cures;  yet, effective stem cell editing remains challenging. Here, researchers  demonstrate that optimized lung-targeting lipid nanoparticles (LNPs)  enable high levels of genome editing in stem cells, yielding durable  responses. Intravenously administered gene-editing LNPs in activatable  tdTomato mice achieved >70% lung stem cell editing, sustaining  tdTomato expression in >80% of lung epithelial cells for 660 days.  Addressing cystic fibrosis (CF), NG-ABE8e messenger RNA (mRNA)–sgR553X  LNPs mediated >95% cystic fibrosis transmembrane conductance  regulator (CFTR) DNA correction, restored CFTR function in primary  patient-derived bronchial epithelial cells equivalent to Trikafta for  F508del, corrected intestinal organoids and corrected R553X nonsense  mutations in 50% of lung stem cells in CF mice. These findings introduce  LNP-enabled tissue stem cell editing for disease-modifying genome  correction.

https://tinyurl.com/3msryz8k

The  Solute Carrier Family 26 Member 9 Modifies Rapidly Progressing Cystic  Fibrosis Associated With Homozygous F508del CFTR Mutation

CF is an  autosomal recessive disease caused by mutations to the CF transmembrane  conductance regulator (CFTR). Symptoms and severity of the disease can  be quite variable suggesting modifier genes play an important role.  Exome sequencing was performed on six individuals carrying homozygous  deltaF508 for CFTR genotype but present with rapidly progressing CF  (RPCF). Data was analyzed using an unbiased genome-wide genetic burden  test against 3076 controls. Single cell RNA sequencing data from LungMAP  was utilized to evaluate unique and co-expression of candidate genes,  and structural modeling to evaluate the deleterious effects of  identified candidate variants. These researchers have identified solute  carrier family 26 member 9 (SLC26A9) as a modifier gene to be associated  with RPCF. Two rare missense SLC26A9 variants were discovered in three  of six individuals deemed to have RPCF: c.229G > A; p.G77S (present  in two patients), and c.1885C > T; p.P629S. Co-expression of SLC26A9  and CFTR mRNA is limited across different lung cell types, with the  highest level of co-expression seen in human and mouse alveolar type 2  cells. Structural modeling suggests deleterious effects of these  mutations as they are in critical protein domains which might affect the  anion transport capability of SLC26A9. The enrichment of rare and  potentially deleterious SLC26A9 mutations in patients with RPCF suggests  SLC26A9 may act as an alternative anion transporter in CF and is a  modifier gene associated with this lung phenotype.

https://tinyurl.com/4b4pzazv

Long-Term Therapy With CFTR Modulators Consistently Improves Glucose Metabolism In Adolescents And Adults With Cystic Fibrosis

Impaired  glycemic control and the subsequent development of Cystic fibrosis  Related Diabetes (CFRD) are prevalent complications, affecting up to 50 %  of adults with CF. Initial findings indicate that CFTRm (CFTR modulator  therapies) may have a positive impact on short-term glycemic control;  however, long-term effects remain uncertain at present. In this  retrospective study, data were collected and analyzed on 15 pwCF, ages  13–37 years, started on CFTRm therapy. Oral Glucose Tolerance Test  (OGTT) results were compared pre- and post-CFTRm therapy. In conclusion,  CFTRm therapy may decelerate the glycemic control deterioration in pwCF  over an extended period. These findings indicate the need for periodic  OGTTs following the initiation of CFTRm therapy to appropriately adjust  insulin requirements and prevent hypoglycemia. Further larger cohorts  are required to authenticate and substantiate these findings.

https://tinyurl.com/n4dnsy5d

Comparative Microbiome Analysis In Cystic Fibrosis And Non-Cystic Fibrosis Bronchiectasis.

Bronchiectasis  is a condition characterized by abnormal and irreversible bronchial  dilation resulting from lung tissue damage and can be categorized into  two main groups: cystic fibrosis (CF) and non-CF bronchiectasis (NCFB).  Both diseases are marked by recurrent infections, inflammatory  exacerbations, and lung damage. Given that infections are the primary  drivers of disease progression, characterization of the respiratory  microbiome can shed light on compositional alterations and  susceptibility to antimicrobial drugs in these cases compared to healthy  individuals. To assess the microbiota in the two studied diseases, 35  subjects were recruited, comprising 10 NCFB and 13 CF patients and 12  healthy individuals. Researchers observed reduced species diversity in  both disease cohorts, along with distinct microbial compositions and  profiles of antimicrobial resistance genes, compared to healthy  individuals. The nasopharynx exhibited a consistent microbiota  composition across all cohorts. Enrichment of members of the  Burkholderiaceae family and an increased Firmicutes/Bacteroidetes ratio  in the CF cohort emerged as key distinguishing factors compared to NCFB  group. Staphylococcus aureus and Prevotella shahii also presented  differential abundance in the CF and NCFB cohorts, respectively, in the  lower respiratory tract. Considering antimicrobial resistance, a high  number of genes related to antibiotic efflux were detected in both  disease groups, which correlated with the patient’s clinical data.  Bronchiectasis is associated with reduced microbial diversity and a  shift in microbial and resistome composition compared to healthy  subjects. Despite some similarities, CF and NCFB present significant  differences in microbiome composition and antimicrobial resistance  profiles, suggesting the need for customized management strategies for  each disease.

https://tinyurl.com/3ds7z7zd

The Impact Of Cost Of Living On The Quality Of Life Of Cystic Fibrosis Patients: A Study In Greece

The  objective of this study was to analyze the financial consequences of  having CF on patients, evaluate their general state of health, and  specifically investigate the impact of living expenses on their quality  of life. The study obtained a response rate of 93.2%, with 105  participants consenting to and effectively finishing the questionnaire.  The mean age of the patients was 32.1 years, with 46.7% being female and  53.3% being male. Medication was being administered to 46.7% of the  patients. The condition incurred an average cost of 767€ in the  preceding semester. The maximum cost was 1007€. Patients with a higher  monthly family income and those who were taking medication exhibited  superior physical performance and functional capacity. The research  emphasizes that implementing causative treatment and minimizing  hospitalizations can potentially enhance life satisfaction. The findings  suggest possible approaches to enhance the quality of life in people  with cystic fibrosis, in conjunction with the implementation of novel or  enhanced treatment modalities.

https://tinyurl.com/exhhn5bw

The  Criteria For Chronic Rhinosinusitis In Children With Cystic Fibrosis Are  Rarely Fulfilled After Initiation Of CFTR Modulator Treatment

The vast  majority of pwCF have untreated secondary chronic rhinosinusitis (CRS).  Whereas the introduction of the cystic fibrosis transmembrane  conductance regulator modulator (CFTRm) treatment regime has improved  the lung function of pwCF, few studies have been published examining the  effect on sinonasal symptoms in children. The aim of these researchers  was to explore the effect of double CFTRm treatment on CRS and olfaction  in children with CF. pwCF were included in this non-randomized  cross-sectional study, where an otolaryngologist performed a complete  ENT examination before initiating treatment with  elaxacaftor/tezacaftor/ivacaftor (ETI). Twenty-three pwCF aged  6–12 years were included. Eighteen of 23 patients were on a double CFTRm  treatment, and 5 patients were CFTRm naive, respectively. Altogether,  19 had normal olfaction, 20 had none or mild CRS symptoms according to  SNOT-22, and 14 had a normal endoscopy. None of the patients had  symptoms of chronic rhinosinusitis lasting for more than 12 weeks, thus  none of the patients fulfilled the criteria for CRS. Children with CF  treated with double CFTRm have few to no symptoms of CRS and normal  olfaction, which is an improvement compared with children following  treatment modalities prior to CFTRm.

https://tinyurl.com/2mkbnwu3

Extracorpeal  Membrane Oxygenation In A Cystic Fibrosis Patient With Septic Shock Due  To Methicillin-Resistant Staphylococcus Aureus

This  report presents the case of an 18-year-old male with CF who developed  septic shock due to methicillin-resistant Staphylococcus aureus (MRSA)  bacteremia. He had a history of poor nutritional status and uncontrolled  CF-related diabetes, both contributing to his rapidly declining  condition. Despite aggressive treatment, including extracorporeal  membrane oxygenation, his hospital course continued to deteriorate,  including worsening respiratory failure and the need for lower extremity  amputation secondary to ischemia. Ultimately, the decision to withdraw  life support was made after it was determined the patient had  unrecoverable respiratory failure. Our goal in presenting this case is  to demonstrate the serious consequences of MRSA infection in patients  with CF, who are often severely immunocompromised, and to emphasize the  need for early detection and aggressive intervention among patients of  this group.

https://tinyurl.com/2rv3k5du

Improved Early Growth In Danish Children With Cystic Fibrosis From 2000-2022

Improved  growth in children with CF may have resulted from advances in treatment  for CF over the past two decades, including the implementation of  newborn screening in Denmark in 2016. This observational cohort study  focuses on changes in early growth in Danish children with CF born  between 2000 and January 2022. Researchers included 255 children in the  analyses. Cubic spline mixed effects models show that catch-up growth  improved in birth cohorts over time, with the 2016–2022 birth cohort  achieving growth reference curve values in WAZ, LAZ/HAZ and BMZ the  earliest. The proportion of underweight and stunting observations among  children born 2000–2004 decreased by the 2016–2022 birth cohort, while  the proportion of overweight, low BMZ and high BMZ observations  increased. Advances in care for young children with CF have led to  improvements in growth – with the 2016–2022 birth cohort approaching  potential for overweight. Nonetheless, low BMZ remains. Immediate,  individualized nutrition care throughout early childhood remains crucial  in mitigating malnutrition.

https://tinyurl.com/47h9rbvh

Behavioral And Sleep Issues After Initiation Of Elexacaftor–Tezacaftor–Ivacaftor In Preschool-Age Children With Cystic Fibrosis

The  introduction of triple combination therapy with  elexacaftor–tezacaftor–ivacaftor (ETI), has revolutionized the prognosis  for people with CF carrying at least one F508del allele. A  placebo-controlled study showed that ETI improves lung clearance,  bodyweight, and sweat chloride concentration after 6 months of treatment  in children with cystic fibrosis aged 2–6 years, with an overall  acceptable safety profile. This study aimed to describe key CF disease  outcomes and adverse events over the course of 5 years. All children  started ETI at baseline. Sleep difficulties and behavioral issues were  observed from the first week of treatment and persisted at 3 months for  58 of 93 children. Immediate recovery in symptoms was seen for four (4%)  of 93 children, all aged 4 years, who underwent a dose reduction of  approximately 50%, and two (2%) children, aged 3 years and 4 years,  respectively, who stopped treatment. The children who were reported to  have behavioral issues or sleep difficulties at 1 month of follow-up  were similar to those not experiencing behavioral issues in terms of  age, bodyweight, and sweat test at baseline and at 1 month.

https://tinyurl.com/mtbjyb6n

Tezacaftor Is A Direct Inhibitor Of Sphingolipid Delta-4 Desaturase Enzyme (DEGS)

These  researchers recently demonstrated that 48 h exposure of primary human  bronchial epithelial (hBE) cells, obtained from both CF (F508del  homozygous) and non-CF subjects, to the triple drug combination  Elexacaftor/Tezacaftor/Ivacaftor (ETI) results in a CFTR  genotype-independent modulation of the de novo synthetic pathway of  sphingolipids, with an accumulation of dihydroceramides (dHCer). Since  dHCer are converted into ceramides (Cer) by the action of a delta-4  sphingolipid desaturase (DEGS) enzyme, they aimed to better understand  this off-target effect of ETI. Researchers demonstrated that 1) dHCer  accumulates in hBE with time following prolonged ETI exposure, that 2)  similar inhibition occurs in wild-type primary human hepatocytes and  that 3) this does not result in an alteration of DEGS expression. They  then proved that 4) ETI is a direct inhibitor of DEGS, that 5)  Tezacaftor is the molecule responsible for this effect, that 6) the  inhibition is concentration dependent. Finally, after repeated oral  administration of ETI to naïve, non-CF, mice, they observed a slight  accumulation of dHCer in the brain. These researchers believe that  further investigations on Tezacaftor should be envisaged, particularly  for the use of ETI during pregnancy, breastfeeding and in the early  stages of development. DEGS dysfunction and dHCer accumulation causes  impairment in the development of the nervous system, due to a  derangement in myelin formation and maintenance.

https://tinyurl.com/yc8m7x5e

Anti-Inflammatory Effects Of Elexacaftor/Tezacaftor/Ivacaftor In Adults With Cystic Fibrosis Heterozygous For F508del

Inflammation  is a key driver in the pathogenesis CF. Researchers assessed the  effectiveness of elexacaftor/tezacaftor/ivacaftor (ETI) therapy on down  regulating systemic and immune cell-derived inflammatory cytokines. They  also monitored the impact of ETI therapy on clinical outcome. Adults  with CF, heterozygous for F508del, were assessed at baseline, one month  and three months following ETI therapy, and clinical outcomes were  measured, including sweat chloride, lung function, weight, neutrophil  count and C-reactive protein (CRP). ETI therapy resulted in decreased  sweat chloride concentrations, CRP and neutrophil count and increased  percent predicted forced expiratory volume from baseline to three  months, alongside a trend increase in weight. While ETI therapy is  highly effective at reducing sweat chloride and improving lung function,  it also displays potent anti-inflammatory properties, which are likely  to contribute to improved long-term clinical outcomes.

https://tinyurl.com/mr42497f

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Evolution And Host-Specific Adaptation Of Pseudomonas Aeruginosa

The  major human bacterial pathogen Pseudomonas aeruginosa causes  multidrug-resistant infections, particularly in people with underlying  immunodeficiencies or inflammatory lung diseases such as CF. However, it  remains unclear how P. aeruginosa has evolved into a highly adapted,  globally disseminated pathogen. This study sought to understand the  pathogenic evolution of P. aeruginosa by combining population-level  genomic exploration with transcriptomic and phenotypic studies. They  found that epidemic clones appeared to have intrinsic preferences for CF  or non-CF individuals, and discovered a clear expression signature of  genes positively and negatively associated with CF affinity. They found  that high-CF-affinity clones were better able to survive within CF  macrophages, in part mediated by expression of the stringent response  modulator DksA1, suggesting that enhanced host innate immune evasion  might explain the intrinsic success at infecting CF patients of certain  epidemic clones. It was found that the products of these patho adaptive  genes were tightly interconnected, indicating their likely coordinated  functional roles. Many genes were more frequently mutated in either CF  or non-CF isolates, suggesting that distinct functional programs were  being modified as part of host-specific adaptation. These findings  describe the key sequential steps involved in the evolution of P.  aeruginosa from an environmental organism to a major human pathogen.

https://tinyurl.com/2bwtkpnx

Dietary Intake And Quality Among Adults With Cystic Fibrosis: A systematic Review

This  systematic review aims to describe the dietary quality, dietary intake  and related behaviors of adults diagnosed with cystic fibrosis. Nineteen  observational studies were included and considered high to moderate  quality. Most studies reported that individuals with cystic fibrosis  were consuming high-energy diets; where studies reported energy intake  as a proportion of requirements met, energy intake was high, even when  using individualized or cystic fibrosis-specific referents. In addition,  fat intakes as a proportion of energy appeared high (29%–39% of total  energy), particularly as current guidelines recommend a macronutrient  profile similar to the general population (<30% of total energy).  There was considerable variation in the reporting of fatty acid profiles  and other nutrients. Five studies reported on concerns regarding diet  and eating in this population. Findings from the current review suggest  dietary intakes of adults with cystic fibrosis appear to be less than  optimal and concerns about diet, weight and food may be emerging in this  population. Future research utilizing consistent measures of dietary  assessment and reporting, reporting of medical therapies, and exploring  potential concerns about diet and eating is warranted.

https://tinyurl.com/4a5yp8x3

Sex Differences Persist After Treatment With Ivacaftor In People With Cystic Fibrosis

Historically,  studies show that female patients with CF have worse pulmonary outcomes  than male patients, including decreased life expectancy. It is unknown  whether this disparity persists in the new era of highly effective  modulator therapies. Ivacaftor has been available in the United States  for > 10 years, allowing for the opportunity to understand the impact  this therapy may have on sex disparities in CF. These researchers  hypothesized that female patients will continue to show worse outcomes  because they suspect that the disparity is not driven solely by ion  channel dysfunction. They conducted a retrospective cohort study using  the CF Foundation Patient Registry comparing changes in pulmonary  exacerbation rate, lung function (FEV1 % predicted), and presence of  Pseudomonas aeruginosa among male patients vs female patients before and  after initiation of treatment with the highly effective modulator  ivacaftor. Male patients showed a significant decrease in pulmonary  exacerbations after ivacaftor treatment , whereas female patients did  not. FEV1 % predicted similarly decreased in both male and female  patients before vs after ivacaftor treatment. P aeruginosa prevalence  decreased to a similar extent in both male and female patients after  ivacaftor treatment. These  findings demonstrate that sex disparities in  CF persist in those treated with ivacaftor because of differences in  pulmonary exacerbations. More research is needed to determine the  specific pathophysiologic drivers of this disparity.

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Aimee  Lecointre is 38 and has CF. She lives in Salt Lake City, UT. She loves  reading, cooking, writing, and spending time with her husband.