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Finding Balance
In Dating

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.

Deaton

pearls of wisdom:

“But I’m Doing This For You!”—

The Darker Side Of
Dating With CF

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.

Pearls au24

Adventures Abound:

​Life’s A Dance

“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.

miller

Culinary Corner:
Swedish Meatballs In Gravy

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

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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

culinary

Ask the Attorney:
Readers’ Questions Answered

©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. 

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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.

attorney

Voices from the
Roundtable:
What the Gut?

By Andrea Eisenman

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.

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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. 

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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. 

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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. 

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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.

voices
tx talk

transplant Talk:
It Is Not Fair To Have CF
And Lung Cancer!

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.

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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.

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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. 

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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.

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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.

focus topic:
I Can’t Breathe And
I Cough A Lot,
But I Have A Great Smile

“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. 

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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. 

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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. 

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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.

Caise
LMK

Recipients Of The Higher Education (Formerly The Lauren Melissa Kelly) Scholarship Announced

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

research

Research Roundup

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

​

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.

https://tinyurl.com/bdhh8n7d s

 

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.

cfroundtable@usacfa.org

​

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*CF Roundtable® does not give medical advice. Any medical opinions represented in these articles are those of the writer and do not represent the views of USACFA, any of our community partners, or any other group or individual. We strongly suggest you consult your doctors regarding any medical references and before altering your medical regimen in any way. USACFA does not endorse any products or procedures. 

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