When  I was first diagnosed with CF back in 1991, my CF doctor at the time  reiterated the importance of going to Seton Medical Center and their  accompanying ER if/when I needed treatment after hours. Back then, we  didn’t have a CF care team per se and we definitely didn’t have an  accredited CF care center. Trips to see my CF doctor meant an  appointment first thing at his office (he was primarily a pediatrician),  bypassing the waiting room and nothing much different than a regular  doctor’s visit other than the addition of a lung function test. Until  recently, I’ve always gone to Seton for any ER visits and any CF  tune-ups. If you’ve read CF Roundtable or our blog for a few years now,  you might remember the one time I had to go to the ER in Dallas and the  subsequent chaos and financial burdens from that visit.

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This  past month I felt a pain in my sternum area which was similar to the  pain I felt when I unknowingly had gallstones back in 2008. Since I no  longer have a gallbladder and apparently have a short memory with pain  in that area, I chalked it up to the start of a blockage and referred  pain. This pain came and went for a few days and seemed consistent with a  blockage since I wasn’t really going to the bathroom much. On the third  day, that pain skyrocketed so I took a dose of magnesium citrate.  Within an hour I had severe cramping and excruciating pain that had me  in tears. I’ve never experienced such pain in my life and that’s saying  something since I had a frontal sinus obliteration in 2011. My husband  insisted on a trip to the ER as he had never heard me in such pain from a  blockage. Close to midnight on a Saturday, we make the trek to the ER  but we choose one that is still in network, but one that is much closer  to us and incidentally has better parking. If you live in Austin, I’m  sure you can relate to the parking woes everywhere.

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The ER  gave me some morphine and ordered a CT with contrast. The ER doctor said  the scan showed colitis, which causes a thickening of the intestinal  walls. He highly recommended I be admitted for a gastrointestinal (GI)  consult the following day. I went back and forth with the doctor as this  wasn’t really on my radar when we went in—it was just a blockage! I  would get some pain relief, get confirmation of the blockage, and be on  my merry way to suffer in misery at home. After the drama with the  hospital in Dallas, I knew transferring to Seton wasn’t in the cards as  my insurance would argue that I could get the same care at either place.  I begrudgingly agreed to be admitted. If you’ve ever been admitted,  whether through the ER or directly, you know that administrative wheels  move slowly. They moved me to a holding room (basically a different ER  bed that’s converted to a room temporarily until a bed opens up) in the  ER until around 10 a.m. the next morning when I was finally taken up to a  hospital room. Since I have a history of CF-related liver disease, I  was experiencing pain in the gallbladder area, and I’ve already had two  endoscopic retrograde cholangiopancreatography (ERCP) procedures to  clear out the sludge and stones in my bile duct, they ordered a magnetic  resonance cholangiopancreatography (MRCP) which is a specialized MRI  test used to look at the bile ducts, pancreas, and liver. They also  ordered an MRI with contrast of my abdomen. The results showed more  sludge and stones in my bile duct so I was put on a clear liquid diet  for the remainder of the day with NPO starting at midnight for another  ERCP on Monday. I had the procedure and felt much better.

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This is  the part of the story where I say why the hospital matters. During my  three-day stay, I was asked about my drug allergies multiple times but  they weren’t recorded completely and I have a rather extensive list of  drug allergies. The hospitalist (the hospital doctor who oversees  everything related to my admit at the hospital) ordered IV Zosyn to  treat my colitis while I was waiting for my procedure. I had to  reiterate that I was allergic to Zosyn after the second time it was  ordered. The hospitalist was frustrated with me and noted that I don’t  have many other options and I have to get something so I suggested  meropenem, which is what I’m typically given when admitted for a tune  up. Since they wanted to start IV antibiotics, I asked them to run them  through my port. I have terrible veins to begin with and peripheral IVs  usually only last a day before they need to start a new one. There’s a  reason why I have a port. Additionally, I came in with bilateral  bruising because we have a mouthy, nibbling puppy who leaves little love  bruises and bites all over my arms so my arms were already pretty  battered before they started doing labs and IVs. I asked and pleaded for  my port to be accessed many times from every nurse and lab tech that  came to my room. They refused. I got a variety of answers why: it’s not  clean, you’re allergic to chlorhexidine and hospital policy states that  anyone with a central line has to have a daily chlorhexidine-wipe bath  to keep the site clean and free of infection, etc. I’ve had my port for  almost a decade and I’ve never once had an infection or a problem  keeping it clean. Instead, they went through three peripheral lines and I  was poked several other times at 5 a.m. for lab draws. By the third  morning I growled at the lab tech that all of this could have been done  through my port and we could have saved all these blown veins! She asked  to draw blood from my hand and I emphatically denied her—hands are for  emergency access only and I’m clearly not about to die if this lab isn’t  drawn. Seton, on the other hand, lets me come into the hospital  accessed so long as my husband initials and dates the dressing so they  know when it’s time for a dressing and needle change. It wasn’t until I  was admitted elsewhere that I realized how much of a blessing that  policy is as I prefer my husband to access my port. There is a short  list of two people I allow to access my port thanks to my favorite home  health nurse who taught both of us to be protective of my port and who  accesses it.

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I was  also asked for my medication list multiple times but the pharmacy and  hospitalist collectively struggled getting most of them—many weren’t in  the formulary, many weren’t available in the correct dose, and others  took eight to ten hours to get to me. In the end, I had my husband bring  the necessary ones like Trikafta from home. Most of the vitamins I  skipped for since it was more of a hassle than anything. Additionally,  the entire time I was there, very few of the hospital staff wore a mask  and those that did wore them under the nose, which is pointless. None of  the staff I interacted with, either in the ER on the hospital floor,  gowned up. Zero contact precautions. I should have asked and insisted,  but at the time I just wanted to get the ERCP and get discharged. I was  apparently the only CF patient anyone had ever had so no one really knew  what CF was and what that meant in a hospital setting. Not  surprisingly, after my procedure (which has benefited from science since  the last time as it was all done through my throat without any  laparoscopic incisions) my throat was sore and my lungs felt junky. When  my cough came back with a vengeance (the anesthesia/surgery and a lack  of Trikafta from being NPO too long contributed to this) I asked for a  nebulizer treatment and a respiratory therapist (RT). This took way too  long to happen and they were only able to give me Xopenex. The 7%  hypertonic saline, pulmozyme, and cromolyn sodium that I usually  nebulize along with Xopenex when I do treatments at home weren’t an  option. The RT came to my room the following morning after visiting a  patient in the ICU. The RT was not gowned up and wasn’t wearing a mask.  While she was getting my meds together and charting, she asked if I  normally go to Seton. I nodded and she let me know she used to work as  an RT at Seton on the fourth floor, where the CF patients are admitted.  In fact, she remembers working with my first CF doctor. He was a force  to be reckoned with in the hospital—he got stuff done stat but he also  barked orders in the process. Despite all this and despite knowing CF  protocols and contact precautions, she still came to my room without a  mask and no gown. I was floored.

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In the  end, I did finally get the ok to go home with an oral antibiotic through  the end of the week with Benadryl pre-dosing to quell the allergic  reaction. Being insanely itchy gets old quickly; I’m grateful I didn’t  break out into hives and need an EpiPen.

I  learned a lot of lessons those three days. I’m glad I made the choice I  did if for nothing other than the knowledge down the road. I’m happy I  chose the closer ER as I did get stronger pain relief more quickly. I  came into the ER with an 11/10 on the pain scale and it took several  doses to bring it down to a 4/10. My pain hovered around 6/10 for most  of the time so I’m grateful that I didn’t have to argue for IV pain meds  when oral ibuprofen wouldn’t have worked at all. Seton and my CF clinic  across the board are both very strict with their pain medication  policies. I’m also happy I went to the closer hospital because my GI  doctor works next door to the hospital so I was able to see him  personally on Sunday and his team was able to do the procedure. They  don’t work with Seton so that would have been a different issue had I  gone to Seton in the first place. The hospital I went to is  unequivocally not set up for CF care. It’s just not something they’re  familiar with in any sense. Seton routinely has CF patients admitted so  the nurses on the fourth floor are well versed in CF protocols—the  antibiotics, the RT treatments, the contact precautions, etc. This  hospital had nicer rooms but I’ll take the familiarity with CF over the  amenities if given the choice. This whole endeavor was a glaring  reminder that I need to research and know ahead of time the best  hospital with experience in CF care when I travel just in case I need to  go to the ER and/or be admitted outside of my home hospital. The  hospital does matter. And CF is still a rare disease. It’s easy to think  otherwise when you’re plugged into the CF community most of the time  but outside that bubble, it’s not necessarily something people and the  medical community are particularly knowledgeable about, which is  worrisome when you’re relying on that medical community for care.

Sydna Marshall is 43 years old and has CF. She is the President and Managing Editor of USACFA and CF Roundtable. She  lives in Austin, TX with her husband, Adam, and her two furbabies,  Cutty (12) and Mickey (1). When she’s not corralling her toddler puppy  she’s reading, working on jigsaw puzzles, cooking up buttery goodness in  the kitchen, and recentering herself on her yoga mat. Her contact  information is on page 2.

Nothing  starts a new year off right like revisiting an old favorite story! In  thinking about what to write for my column for this Focus Topic on  Cancer, I once again found myself reminiscing about the experience of my  colonoscopy procedure in 2008. It certainly proved useful for the  findings; I don’t regret having the procedure. My gastroenterologist  back in New Jersey was able to give me a lot of insight about  inflammation in and damage to my intestinal mucosa from what was later  confirmed to be CF.

If  you’re new to the “Pearls of Wisdom” column, a quick refresher: I was  tentatively diagnosed with CF around age 5 but didn’t get a conclusive  diagnosis until just before turning 33. Having my colon explored with a  camera helped to document and make sense of some of the issues I’d been  experiencing for my entire life. It also introduced quite a few problems  of its own. Because of this, my care team here in Florida has taken a  creative approach to looking out for evidence of colon cancer to space  out routine colonoscopies as much as possible.

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Before I  go into any details on this, I should remind everyone that  gastrointestinal issues from CF exist on a spectrum and can vary widely  from one person to another. So what works well for my own care may be  completely inappropriate for another patient’s needs.

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Some  specifics: I’m fairly typical on the macro level in that I’ve had  exocrine pancreatic insufficiency for most of my life, although I had  better digestive enzyme output in childhood than a lot of my peers. And  like many other folks with CF who have a lot of gastrointestinal  involvement, I deal with a mixture of diarrhea and constipation. Even  after years of pancreatic enzyme replacement therapy, I still experience  steatorrhea (very loose stools full of oil) daily. Occasionally I also  get distal intestinal obstruction—blockages in a specific part of the  small bowel.

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Unlike  some people though, I usually only get partial obstructions and deal  with a lot more diarrhea than constipation most of the time. That last  bit matters tremendously because having generally rapid gastrointestinal  “transit” where nothing stays in a person’s body for long is often  associated with lower overall risk of colon cancer.

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Family  history also matters. I don’t know all of my immediate genetic relatives  and probably never will. As far as I know though, none of my direct  ancestors or siblings have experienced colon cancer. My maternal  grandmother did have a couple of large polyps removed from her colon  shortly before dying at age 91. The polyps weren’t malignant and no  further intervention was done. If I manage to survive to that age, I  suspect I won’t mind too much if I do eventually develop colon cancer.  In that situation, I doubt I’d elect to do anything other than have the  polyps removed. Maybe not even that, depending on how much they were  impacting my overall colon function.

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I’m  pretty frank about death; like many of us, I came very close in my early  20s. Indeed, my intensive care stay at 23 was part of the impetus for  getting that first colonoscopy done. In retrospect it all makes a lot of  sense; of course I was ravenously hungry all the time but unable to  keep anything down. Anything I did manage to hold down would just rot  inside me and come out as a foul sewage-like substance. I still remember  the time I vomited two quarts of stinking black slime in the middle of  the night from what was almost certainly a complete small bowel  blockage.

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Not  difficult to imagine why CF can elevate our collective risk for colon  cancer after seeing (and smelling) the contents of that sink.  Unfortunately I didn’t make it to the toilet. My mom heroically cleaned  up the mess after putting me in a fire carry and depositing me back in  bed.

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I  promised you a story, didn’t I? Well, here it is: When I had that first  colonoscopy, my mom took care of me afterwards while I recovered fully  from the anesthesia. Why she was the one doing the caregiving is beyond  the scope of this article. Let’s just say she wound up with a lot of  data behind the ominous warning she gave to my spouse when I had all  those gum surgeries many years later. Folks, this person married me  after I put them through an absolute gauntlet of horrors while heavily  sedated. I highly recommend getting a spouse who embraces the spirit of  “in sickness and in health” full weight.

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Anyway,  my mom is the original champion of wrangling me while anesthetized. This  might not sound like much of a challenge—I have weighed about 85 pounds  on a 5’4” frame for my entire adult life. However, my “skeleton  strength” becomes especially prominent when it is least useful. I also  have an unfortunate tendency to flail my proportionally long limbs  around like an octopus when heavily sedated. My spouse has observed  various other octopus-like behavior, such as latching different  appendages onto their shoulders and attempting to climb on them. Typical  post-surgical behavior for me is a combination of belligerent  aggression, attempts to escape, copious vomiting, and dubious bowel  control.

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This is  not a good combination. J initially suspected some hyperbole in my mom’s  warnings about what caring for me after surgery requiring sedation  would involve. You might say I made them a believer.

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Routine  colonoscopies are thus anything but for me. Both the preparation and the  procedures themselves basically throw me right back into the same  health experiences that sent me to intensive care at 23. I’m lucky to  have much better lung function than many of my age peers with the help  of inhaled corticosteroids and a lot of infection control precautions. I  got a much worse lot with my gastrointestinal function in exchange for  feeling like I can breathe reasonably well, a trade-off I’ll take any  day of the week. But although I’ve learned to live with constant  diarrhea that dramatically shapes my daily patterns of activity, I have  limited reserves for dealing with serious impediments to nutritional  health.

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There’s  also the broader angle of just feeling like garbage for very little  gain. Although I can readily find the humor in my mom’s recollections of  how I alternated between barraging her with lengthy streams of curse  words and going totally unresponsive for several hours after she brought  me back home from the outpatient surgical ward, it’s not so easy to see  the funny side of having my health sent quite literally down the toilet  for weeks on end just so someone can stick a camera up my rectum and  tell me things I already know.

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Teaching  medical school exposes me regularly to the concept of iatrogenic harm, a  fancy way of saying that sometimes getting a health care intervention  can hurt more than it helps. So my care team and I try to balance the  inherent harm of putting me under anesthesia to get my colon scoped with  the need for periodic monitoring for early warning signs of colorectal  cancer. Our approach for the past few years draws on our collective  knowledge of the clinical literature on gastrointestinal cancer  screening and our learned confidence in my willingness to collect  samples of my own poop for submission to labs.

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This  isn’t as easy as it sounds. I’ll spare everyone reading this a detailed  description of my feces and say instead that a lab tech whose nephew has  CF took one look at the sample I handed over and asked if I had the  same disease. So trying to corral a substance that is simultaneously a  liquid, an industrial-strength adhesive, and a textbook example of  non-Newtonian states of matter is par for the course in collecting a  stool sample for lab analysis. Not precisely my idea of fun—but the  alternative is much worse. And I absolutely find it a blessing to have  that option.

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Using  fecal analysis to screen for cancer indicators also lets us work smarter  rather than harder with other aspects of my care. Because my digestion  remains relatively poor even with the massive improvements I’ve seen on  enzyme therapy, we’d need to submit a stool sample periodically for  other tests like fecal fat and fecal pancreatic elastase. So when it’s  time to poop in a cup (or rather into a much larger collection vessel  before transferring some of the mess into a specimen jar) we can get a  lot of value out of a single sample by submitting it for multiple lab  orders at once.

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Combined  with routine bloodwork and observational data, screening my feces  periodically for occult blood (sadly less cool than it sounds) to gives  us regular information about my colorectal cancer status. I do  occasionally have to get a colonoscopy as well, of course. But being  able to go longer between procedures helps preserve my overall health.  And although my spouse is truly a champion caregiver just like my mom  was for so many years, I’d rather spare them the ridiculous antics  whenever possible!

Dr. Alexandra “Xan” Nowakowski is 40 years old and has CF. Xan is a director of CF Roundtable, and  is the Secretary, 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.

Ahh  spring is here. It’s my favorite time of year for so many reasons. I  love sleeping with the windows open at night, feeling the cool air and  falling asleep to the melodious chorus of spring peepers. I love  watching everything come back to life as the ground begins to warm the  curse of winter and give way to the sunshine. I love the way wisteria  and azaleas show off their dramatic color against a backdrop of  chartreuse-colored leaves that line every branch in the forest. I always  spend as many waking hours as possible in the woods, but in springtime  it’s just a little extra special. I can hear the gobble of wild turkeys,  watch baby fawns gallivanting around, and inhale the aroma of  honeysuckle and every other flowering plant around.

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I also  consider myself an amateur chef so of course with new life comes lots of  opportunities to gather wild things to eat. I love spending hours in  search of morel mushrooms with my dad just as he did with his father. I  also love gathering dandelion greens for cooking dandelion gravy. Now if  you’re having serious FOMO (Fear Of Missing Out) due to never having  tasted the delectable treat called dandelion gravy, I have good news for  you! The secret family recipe is included below.

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Spring  is also the season for Great Strides fundraiser events. Each year there  are around 300 walk-a-thons for cystic fibrosis held all over the United  States with hundreds of attendees at each one. Admittedly, it’s been  quite some time since I’ve been included in that count. Partially  because life is busy, I suppose, but there was certainly a time in my  life that I actively avoided going. I just wanted to live a normal life  and not be so different from everyone around me. That said, I do plan on  attending my local walk this year in Wilmington and I’m very much  looking forward to it. I have many great memories of Great Strides Day  when I was a youngster. My entire family and many family friends would  attend and volunteer to run different aspects of the event. I am  extremely humbled and eternally grateful now as I think back to the vast  support I received, both in time and monetary gifts to the foundation.

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One  not-so-pleasant memory has also stuck with me from a walk-a-thon one  year. While I don’t recall exactly how old I was, I suppose I was  between eight and ten years old. Early in the afternoon, prior to the  walk, I’d had a brief chat with the local radio station, FM 95.3,  talking about life with CF. After the interview while waiting for the  walk to begin I was roaming around reading the various signs around the  check-in tent. Some were details about what exactly CF is and how it  affects the human body. That was boring to me. I knew exactly how it  affected my body. Another sign made mention of the amount of money  raised over the years for research and the progress being made in that  regard. Of course, that was exciting to read. Then I found a sign laden  with numbers and statistics about CF: 30,000 people in the U.S. have it;  F508del is the most common mutation; 40 years is the average life  expectancy, and so forth. Wait, what. I had to reread that. 40 years is  the average life expectancy? Now, when you’re eight or ten years old, 40  seems awfully far away. But after doing some quick mathematical figures  in my head I came to the sobering realization, according to that sign,  I’ve already lived a quarter of my life. Whoa.

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The  remainder of that day was action packed and I was soon preoccupied with  conversations and other activities of the day. Nonetheless, from that  point on, that statistic floated around in the back of my mind haunting  me like a ghost. Life went on as it does and by the time I was 17 years  old I had watched three dear friends with CF all decline rather rapidly  and pass away: Eva, Jacob, and Danny all left this world far too soon.

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I first  met Eva when I was seven and she was a year younger than me. She and I  attended elementary school together which was awesome. Somehow, just  knowing someone else in that building was fighting the same battle made  it so much easier to walk up and down those halls. Our parents were also  close friends and organized many of the local CF fundraising events so  Eva and I were always present and helping where we could in an effort to  bring awareness and raise money for the cause. Everything from the  walk-a-thons, to banquets to an annual CF fundraiser at our school  called the Kiss-A-Pig contest. The thing that sticks out to me most  about Eva was her joyful spirit. While she had many complications with  CF including having a liver transplant and living with a feeding tube  for many years, I never once heard her complain and her sweet  disposition never faded. Eva passed on from this life when she was only  13 years old. I miss her dearly.

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Jacob  and Danny were brothers and I met them when I was in my early teens. We  were actually second cousins so our families would attend reunions and  have dinners together. Jacob loved to fish and one of my favorite  memories was fishing with him at the little pond on his family’s farm.  Danny loved to hunt and while I never got to hunt with him, I did  receive the 12-gauge Remington shotgun that used to be his. To this day I  still think of him every time I carry that gun into the field in search  of wild turkeys or other game birds. Jacob passed away when he was 19  years old and Danny passed away years later at the age of 35. I will  forever cherish the time I got to spend with both of them.

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Fast  forward to 2024. I’m starting my fourth decade of life, with forty being  the next big milestone on the horizon. Thankfully, today we see the  average life expectancy continue to increase each year and many folks  with CF are living into their 50s, 60s, and beyond. While I’ve made  peace with the fact that statistically my life may be shorter than  average, I also choose to let that fact be the impetus that pushes me to  live life fearlessly and adventurously. So, what does any of this have  to do with wild adventures as the name of my column suggests? Well, of  course life itself is the greatest adventure any of us could have but I  also find poetic correlations to the journey of life in many of my  quests.

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I was  having a conversation with my mother several days ago and she reminded  me of a discussion we had many years ago when I was quite young. She had  asked me if I ever wished to have been born without CF. I thought for a  moment and said, “No I really don’t wish that. If not for my CF, there  would be so many friends I never would have met and so many experiences I  never would have had.” That statement still rings true today.  Obviously, I don’t know what life would look like if I didn’t have CF.  What I do know however, is I’ve met and connected with some of the most  incredible people along the way that otherwise would have remained  strangers in this great big world. Life is tragic in many ways but with  family and friends by our side, I believe we can withstand any of the  storms that are sure to come.

Dandelion Gravy

This is  an old family recipe and a classic for our family and in the Amish  community. This recipe is a good guideline but feel free to add or  subtract ingredients according to your preferred taste. Try to pick  dandelion greens from plants that still have yellow flowers as these  will have the best flavor. This is best served over mashed potatoes but  baked or boiled potatoes will also do the trick. Enjoy!

• 1/2 lb of bacon (or more if you love bacon, as I do)

• 4 tbsp flour

• 3 cups milk at room temperature (add more if needed for preferred consistency)

• 2 tbsp white sugar

• Salt and pepper to taste

• 3 tbsp balsamic vinegar (adjust to personal preference)

• 5 hard-boiled eggs, sliced or diced

• Approximately 2 cups of fresh dandelion greens

Cut  bacon into small pieces and fry in a medium to large saucepan. Fry bacon  to your preference, leaving all of the grease in the pan. Whisk in the  flour, stirring constantly, and let cook for a few minutes. When the  flour slurry is brown, whisk in the milk. Let simmer briefly until gravy  thickens. Add the sugar, vinegar, and salt and pepper to taste. Add in  the eggs. Just before serving, add the star of the show, the dandelion  greens, and gently fold into the gravy.

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

As  you may remember, my name is Trixie (from Poughkeepsie, but I’m really  from Tennessee y’all). You may recall me from my last missive that I  wrote for this rag in Winter 2023. I wrote about being rescued and  placed with this whacky family. Things have been going decently. I  cannot complain much but do wish to lodge a complaint. To those who  care, see below.

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So, one  day in May last year, my mom slunk out with a backpack and just left me  for six days with my two brudders, Willie (so silly) and Roscoe (from  Moscow). I heard nothing from her until The Boss (aka Steve) dropped a  smelly shirt on my bed. I immediately didn’t want a stinky garment on my  snooze cushion until I realized it was full of scents of mom (who,  unbeknownst to me, wore it for three days straight). Inhaling those  first blasts of her essence hit my brain box and it immediately calmed  me. I slept on that shirt in my bed for three more days until her  return. Before that, I’d get up, look around and realize she was gone.  It was then I’d let out a big pitiful sigh/cry. How could she leave me?  I’d been a good girl, hadn’t I?!

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It  wasn’t until she returned that I understood she had been in the  hoosegow, a/k/a the hospital, a/k/a club med, a/k/a The Big House. I was  so excited to see and smell her in person; I lost my mind and did  donuts all around her jumping up to kiss her in the face. I had to  compete with the brudders but I know she missed me the mostest.

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She  tried to explain to me why she left and here is what I got—something  about getting rabbit cooties to keep her implanted lungs from being  battered by her moon system. Oh wait, I looked it up on the Google—she  said she got rATG antibodies, which are from bunnies and is a treatment  for people who are in rejection—who could reject my mom?! I would never.  With this treatment, she was even more immune-suppressed than before,  and this would last for three months. So, we didn’t see anyone outside  of our family unit unless it was outdoors or in a well-ventilated area. I  liked this because then I could be there with her. [Mom here, rATG or  Thymoglobulin is used to prevent or treat graft-versus-host disease or  rejection of transplanted solid organs and is only done in a hospital  setting.]

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Apparently,  her lung function was declining every time she went to transplant  clinic; not by leaps and bounds like classic rejection but  incrementally. I noticed on our walks that she was sometimes short of  breath and had to stop more frequently for breaks. Her transplant  doctors tried everything: steroids, IV antibiotics, time. Nothing  helped. So, she did what her doctors suggested and checked herself in  for just keeping what lung function she had from sliding away. It was  not going to give her much higher numbers on her PFTs but stabilize what  lung function she still had. There were potential side effects like  being allergic to the antibodies and having to be put in the ICU or  having a severe rash or flu-like symptoms. That is why massive amounts  of steroids are given while administering the rabbit antibodies. As a  result, her blood sugars were sky high. Thankfully, she said she only  had a headache once the treatment started and then after two days was  ok. Being the chef de cuisine at our doghouse, she said the food was  decent and she enjoyed the break of not having to figure out what to  cook each night. A mini vacation of sorts.

​

To  combat her high blood sugars and tedium, my mom requested a stationary  bike be brought into her room, but that was not possible so they gave  her a peddle bike that she could use while seated in a chair. She used  it while watching her stories on her iPad. It supposedly brought her  sugars down slightly but she had to increase her insulin use on hyper  drive, almost doubling the amount of insulin just to keep her sugars  around 200, which is supposedly high for a person. Sleep was quite  elusive (I cannot imagine that!) and she started taking melatonin to get  any sleep. She explained to me that in the wee hours of the morning,  she was awoken to be poked and prodded for blood work, blood pressure  checks, and oxygen saturation. My my, what I take for granted!

​

My mom  likes to get out and move and bikes at home. This I will never  understand. I am happiest when I’m just chilling with my peeps, with her  and The Boss, getting pets, eating my chow, and sleeping. Why does  anyone want to bike going nowhere?! Well, obviously she does. Even  though she says she doesn’t like it, it serves a purpose and it helps  her breathe better so now I encourage it. Since she got home from the  hospital, she is on that thing peddling away and huffing and puffing and  turning all shades of red. I am frequently close to dialing 911 on her  cell phone. But afterwards, she is calm and chill. Sometimes she gets on  the floor and that is when I pounce but she shoos me away so she can do  her core exercises or stretching. I know stretching is very  important—they don’t call it downward dog for no reason, I do that  stretch several times a day.

​

She had  to go back to her vet, I mean doctor, many times that summer. We were  hoping for an increase in her PFTs. At first, it was not moving up or  down from her last one prior to antibodies. And then, it started to go  up slightly—huzzahs all around. And then, it started to go down and she  was getting short of breath again. What was happening??? Her doctor did  another bronchoscopy and a fungus was among us! She was put on an  antifungal medication and since then her PFTs have risen, including her  volume, whatever that is. But she has been super busy on that stationary  bike, still breathing hard but now I understand, she is doing it so she  can spend more time with me and more time out of the hospital.

It has  been nine months since her treatment. It’s been a whirlwind. At her last  clinic visit, her PFTs were the highest they’ve been in two years. I  know she is trying hard to do whatever she can to never leave my side.  She belongs at home. After the hospital, I know my mom was elated to see  me and be home. But probably not as relieved and happy as me.

Andrea  Eisenman is 59 and has CF. She recently realized that her initials are  AGED: Andrea Gail Eisenman Downey (her husband’s surname)! She lives in  New York, NY, with her husband Steve and dogs, Willie, Roscoe, and  Trixie. Andrea is the Executive Editor for USACFA. She enjoys cooking  new recipes, playing pickle ball, biking, tennis when possible, and  staying active as her health allows. Her contact information is on page  2.

Hi  again everyone! When I wrote my “Transplant Talk” article for the  Winter 2024 issue of CF Roundtable, I talked about the skin cancer  surgery I had on my thumb. I was going to the wound care center once a  week to make sure I was progressing well. At one of these appointments,  they suggested I would benefit from Hyperbaric Oxygen Therapy (HBOT),  because I have a hard time healing due to my immunosuppression and my  thumb had been radiated. HBOT is also used to treat bone and skin grafts  that are infected, decompression sickness, arterial gas embolisms,  brain abscesses, gangrene, sudden deafness and vision loss, burns,  carbon monoxide poisoning, and severe anemia. Talk about a game  changer!

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I will  admit right now that I am claustrophobic. When I saw the HBOT chambers, I  started to freak out just a bit. I knew nothing about HBOT. I thought  just my thumb would be in a tube of 100% oxygen, but that’s not how it  works, unfortunately for us claustrophobics. HBOT involves putting your  entire body into an acrylic/clear tube where you are lying down, but  propped up a bit at the head, and you breathe in 100% oxygen (normal air  only has 21% oxygen). This oxygen circulates throughout the body,  including to your wound(s), thereby helping with the healing process.  The extra oxygen helps fight bacteria and triggers the release of growth  factors and stem cells, which promote healing. Also, in a HBOT chamber,  the pressure is increased two to three times higher than normal air  pressure. Because of this, your lungs can gather much more oxygen than  they could just breathing pure oxygen at normal air pressure.

​

The tube  is pressurized in the first few minutes you are in the tube (my ears  popped, like on an airplane), and depressurized in the last few minutes  of therapy. Note that after therapy you are required to get your ears  checked immediately for fluid or ear drum damage by the HBOT doctor. For  me, pressurization and depressurization took about 10 minutes each and I  never felt any pressure during pressurization. I never had any ear  issues after my therapy. I was in the tube for a total of two hours per  session, and I had sessions Monday through Friday for a month. I had to  shower prior to every treatment, and I could not put on lotion,  deodorant, makeup, my wig, and my glucose monitor. One nice thing about  this is that once I showered I just got dressed and left which, as you  can imagine, saves some precious time. I also had to change the type of  dressings and tape I was using on my thumb. Basically, anything that can  cause a fire cannot be in the tube with you. Other items you can’t have  in there include newspaper, Kindles, books, dentures, hearing aids,  cell phones, hand warmers, cigarettes of any kind, nail polish, lip  balm, and velcro. You have to take off all of your clothes and change  into a gown approved to be used in the chamber.

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I am  sure that my fellow claustrophobics out there are cringing right now. I  have good news for you though. You get used to this very quickly. I was  offered Xanax but I would have to stay the extra four to six hours after  the therapy for it to wear off before I was allowed to drive home. No  thank you! I just bit the bullet and did it. I was very surprised at my  lack of panic when they put me into the chamber for the first time.  Since you can see through the chamber, you can convince yourself you are  not really in there. Plus, you have your own TV right in front of you  outside the chamber with any and all channels/shows/streaming services  you could want. I watched The Crown, which was excellent and kept my  mind off of the fact that I was stuck in a tube for two hours. Other  patients slept or meditated. One of the other patients asked to watch  the news, which I personally thought was not a healthy choice, but to  each his own. I was very surprised that I actually came to enjoy HBOT,  and the HBOT staff told me that a lot of people have said the same  thing. It was kind of nice to be away from the world for a while. You  literally can do nothing in the chamber; no work, no catching up on  emails, nothing like that, so you might as well enjoy it. The HBOT staff  were terrific also, that really helped. They were super nice and  understanding of people’s fears and/or confusion. They sat right outside  of the chambers so if you needed them you just had to knock. There were  four HBOT chambers in the room, and they all had names; the ones I used  were “Nautilus” and “Jacques Cousteau.” Cute!

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The  wound management people declared me healed after my four weeks of HBOT. I  was amazed at how good my thumb looked, and how quickly it healed. I  could not even tell that there had been a (fake) skin graft put on it.  Thumbs up to that!

Colleen  Adamson is 55 and has CF. She is the Treasurer of USACFA, lives in  Alexandria, VA, and is back to washing dishes. Her contact info is on  page 2.

This  recipe is near and dear to my heart as I have been eating it since I  can remember. Sicilian Supper was a recipe my grandmother found in one  of those “share a recipe” cookbooks that was put together by mothers to  raise funds for their kids’ school. My grandma made it for her family,  my mom made it for us, and I continue to make it for me and my husband. I  like to describe it as a deconstructed lasagne and it has no relation  to Sicily whatsoever. Italians, please don’t come for me! This is  American through and through! It is a comforting baked pasta dish that  can be frozen in portions and reheated easily.

​

Sicilian Supper

Yield: Serves 4

Ingredients:

• 2 tbsp olive oil

• 1 onion

• 1 lb ground beef

• 1 small can or 4 tbsp tomato paste

• ¾ cup water

• 2 cups wide egg noodles

• 1 8-oz package of cream cheese

• ¾ cup milk (preferably whole milk)

• ½ cup parmesan cheese, grated (plus more for topping)

• 1-2 tsp garlic salt

• Salt and pepper to taste

Preparation:

Step
1:

Pre-heat  oven to 350 degrees. Add water to a medium-sized pot and bring it to a  boil for the egg noodles. Dice the onion finely. Add two tbsp of olive  oil to a skillet on medium heat. Add the onion to the skillet, cooking  for five minutes or until the onion starts to turn translucent. Add the  ground beef and cook until no longer pink, about 10 minutes.

Step
2:

Drain  the fat from the ground beef. Add the beef and onion mixture back into  the skillet along with the tomato paste and water. Stir until it is well  combined. Add salt and pepper to taste and keep on low heat.

Step
3:

Add the  egg noodles to the boiling pot of water with one tbsp of salt. Cook the  egg noodles per package directions for al dente.

Step
4:

Add the  cream cheese and milk to a microwaveable medium-sized mixing bowl. Put  in microwave for 30 seconds to a minute until cream cheese is soft and  you can easily whisk to combine milk and cream cheese into a sauce. Add  the parmesan cheese and garlic salt.

Step
5:

Once the pasta is cooked and drained, add the egg noodles to the cheese and milk mixture. Mix to combine.

Step
6:

Take an  8”x8” baking dish, glass or metal, and start layering your beef and  pasta mixture. Layer half the noodle mixture on the bottom of the baking  dish. Add half the meat mixture and spread evenly over the noodles.  Repeat the layer one more time for a total of two layers. Sprinkle some  extra parmesan cheese on top and bake in the oven for 25 minutes.

Step
7:

Once  baked, take out of the oven and let it sit for five minutes before  dishing up. Serve with a salad or any vegetables you like. Enjoy!

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

In  December of 2023, one year and four months after receiving both  double-lung and kidney transplants, I was diagnosed with squamous cell  carcinoma in situ. Squamous cell carcinoma is a type of skin cancer that  commonly develops post-transplant due to the immune suppression  medications transplant patients must take to protect against possible  organ rejection. In my lung transplant education process, from the  preparation phase through the recovery phase, it was impressed upon me  that skin cancer was such a common experience that it was most likely  not a question of if it would happen, with my pale skin and tendency to  burn with even minor sun exposure, but when. So, I wasn’t entirely  surprised to hear, at the end of November, as I sat in a beige exam room  of my dermatologist’s office, the phrase, “I think it’s best to do a  biopsy.”

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The spot  that needed a biopsy was a scaly, red dot on my lower left shin, about  four inches above the top of my foot. It had been there for years, even  before my transplants, and I’d never thought much of it other than  occasionally wondering where it came from as I rubbed lotion over it or  dried my leg after a shower. It never itched; it never changed shape nor  grew in size and so I gave it no more attention. What I did not know  then was that any unexplained new mark on my skin should be considered  suspicious. At my second skin check with the dermatologist, I pointed at  it with seriousness and stated that the spot was most certainly getting  bigger. And with her little single-eye magnifying glass hovering over  my shin, my doctor agreed.

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Part of  my post-transplant care includes these regular dermatology visits, more  specifically an exam known as a full body skin check. At my transplant  center, if you have no history of prior skin cancer occurrences, these  checkups are recommended annually. In a skin check, the doctor examines  every inch of your skin for any suspicious marks, moles, or lesions:  each limb is lifted, the hair on your head is parted, even the bottoms  of your feet are inspected while you sit fully undressed in a paper-thin  gown. If something appears suspicious, as in my case, a biopsy is  performed and the small sample of tissue they remove is sent for  pathology. After my five-minute skin scrape biopsy, they placed a  bandage over the area and I went home to await the results.

​

About  two weeks later, as I prepared dinner, my phone rang. I clicked the  green accept button, and my heart sank a little hearing the voice of my  doctor herself. If you have experience with awaiting test results, you  might know that if a doctor personally delivers news, it may not be  good. I took a deep breath, confirmed my date of birth, and tried to  steel myself for more bad news. After she revealed my diagnosis, she  quickly shifted into treatment and prognosis. As she spoke, the anxiety  of accepting yet another new and potentially serious diagnosis began  creeping to the forefront of my mind. I attempted to quell its presence,  reminding myself I need to listen to what she’s telling me instead of  listening to these thoughts spiraling into worst-case-scenarios, my  brain’s go-to response when getting bad news.

​

It’s  very easy for me, someone who for most of my life has only received bad  news, to jump to the worst conclusions. Living through end stage  respiratory disease, kidney failure, the continued denial for the  life-saving transplants I needed for six years, and everything that  happened in between those events has made anxiety, worry, and fear, as  strange as it may sound, my comfort zone. Even with the healing and  quality of life improvement my transplants brought, my old habit of  expecting, fearing, and preparing for the worst remains, to this day, a  hard one to break. What has become easier since gaining my health back  in the last year and a half, is talking—or, more accurately, thinking—my  way through it. And in that moment on the phone, despite the spike in  anxiety, I knew I needed to pause and just listen. Thankfully, she’d  just gotten to the good news: in situ, a term unfamiliar to me, meant  this squamous cell carcinoma hadn’t grown deep into my tissue or spread  from the visible area. In other words, it was most likely treatable.

​

My two  treatment options were a bit concerning to hear as well: topical  chemotherapy or surgery. Chemo-therapy doesn’t really imply an easy or  comfortable option, even if just in the topical form. And the thought of  another surgery on the heels of the most challenging surgeries of my  life was, to put it mildly, undesirable. Luckily, though, the surgery  did sound comparatively mild, and my dermatologist thought topical  chemotherapy wasn’t the best option for me. Given that the cancerous  tissue was well-contained in one spot, I decided to go the surgery  route. I was to see a surgeon who specializes in Mohs surgery, a  procedure in which layers of cancerous tissue are removed and tested  under a microscope one at a time until no more cancer is seen.

​

After  the phone call with my dermatologist, I had to update my transplant  team. I figured they had a regular protocol since they were so familiar  with transplant patients developing skin cancer. At this point, my nurse  coordinator agreed that Mohs surgery was the best option. This  diagnosis, for now, wouldn’t mean any changes to my immune suppression  medications, but if I were to face a second occurrence of skin cancer,  that’s when my dosages would be lowered to allow my body a little bit of  extra protection. The immune system is the first line of defense in  preventing the development of cancer cells; however, mine was suppressed  from the transplant drugs and I lacked that defense. In the meantime,  I’d need to continue protecting my skin from the sun, follow up with my  skin checks every six months going forward, switch to a high-risk  dermatologist—an easy change as it would be in the same office—and  continue monitoring my skin for any new concerning spots.

​

The last  point she mentioned had me thinking: why had this spot, that appeared  before my transplants and in an area hidden from sun, turned cancerous  now? I wondered out loud if it could be that this spot had been  pre-cancerous all along and my immune system pre-transplant had been  doing its job the best it could to keep it from developing further.  However, since my transplants, with my immune system greatly suppressed,  did this give those cells the chance to mutate fully into carcinoma?  Her answer was simple: likely, yes. With a suppressed immune system,  this red mark on my skin probably became cancerous only recently.

​

Finally,  on January 5, 2024, armed with all this information and a newfound  confidence, it was time for Mohs surgery. The outpatient procedure  lasted about five hours, and most of that time was spent waiting for  test results. The lesion on my left shin was measured and then numbed  with a lidocaine injection. Once I could no longer feel the prick of a  needle, the doctor began to slice a layer of tissue off the affected  area. I tried to watch—I’ve always been fascinated by the surgical  procedures I endure—but as the doctor began to remove the skin with  tweezers I had to look away. The wound was then cauterized lightly to  prevent bleeding and that layer was taken to be tested.

​

After  about 45 minutes of waiting for the first layer’s test results, the  surgeon returned to remove a second layer, one that was a little wider  this time to capture the margins of the lesion as cancer was seen at the  very edges. This time, with my eyes firmly on my book, the second layer  was removed and tested with the result finally showing all cancerous  tissue had been removed. I was officially cancer free. The last step was  a nurse adding a compression bandage and explaining my care  instructions. I was to leave the compression bandage on for 48 hours,  and from there I’d wash it twice a day, slather it in petroleum jelly,  and keep it covered, all of this until it was fully healed.

​

Today,  three months later, it’s still healing. As I write this, my wound, while  no longer the size of a quarter, is covered with Aquaphor and a  non-stick pad. The Aquaphor keeps it moist and prevents it from scabbing  over and healing like a “crater” in my leg, as my doctor put it. I wear  a compression sock over it, a recommendation given at my two-week wound  checkup, to keep at bay any swelling that may prolong healing. Skin  cancer is a common experience among post-transplant patients, and I’m  grateful mine was as mild as it was. As time goes on, I’ll likely face  this hurdle again, and other than hoping for a similar prognosis, what I  can do to prevent it is what we all can do: use a high Sun Protection  Factor (SPF) sunscreen religiously and sun-protective clothing daily.  It’s the “Mohs” vital step to prevent skin cancer (pun definitely  intended).

Matison  is 31 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 Instagram @matisondeaton.

My  sister finally had her transplant. I had my own transplant in 2002. For  me, having CF is like swimming in the ocean. Oftentimes, the waves are  small and gentle, pushing you along in the current. However, there are  those times when, seemingly out of nowhere, a hurricane blows in and  destroys everything in its path. The waves are 30 feet high and any  chance of a leisurely, comfortable existence is thrown into chaos. This  is what happened in 2013.

​

After  Maura’s transplant, I moved out to Southern California. My girlfriend  was living out there with her family and San Diego seemed like a nice  place to start over. I was tired of traveling, tired of being in  different countries and cities, and tired of the hassle of not knowing  the language of the land I was living in and the customs that it  followed. I have been in hospitals all over this world and there is  nothing like the security of a hospital with a proper CF center and  transplant team. However, since my transplant, I had been so healthy for  over a decade that I nearly forgot the harsh realities of CF and its  potential for utter destruction. I began to believe I was a “normal”  person, with a “normal” body.

​

After  only a month of being in San Diego, I got sick. I was short of breath  for the first time in ten years and could barely walk to the bathroom.  Eating and drinking, taking a shower, even sleeping were as difficult as  they were before my transplant. In hindsight, perhaps I was not able to  let myself believe that I still could get that sick after having new  lungs.

​

On a  Friday afternoon, I dragged myself into the University of California,  San Diego (UCSD) emergency department. Within minutes I was seeing their  transplant doctor. To this day, he was one of the brightest and caring  physicians I had ever met. He was not much older than me but had that  scary level of intelligence and I was immediately relieved to be there.  Of course, nothing would prepare me for the events that transpired over  the coming week. He wanted my entire medical history and was quickly on  the phone with my team at Penn. However, they hadn’t seen me in quite  some time and the situation was becoming more and more dire by the  hour.

​

In my  life, there have been certain times where I was in trouble and, in those  moments, for reasons entirely unknown to me, the exact right person or  exact right remedy has taken place in the exact right way I needed it  to. It’s almost as if some unsaid, unheard signal was thrown out into  the universe – and the universe answered. This was one of those  moments.

​

Just as  the transplant doctor was asking me questions about my CF in early  childhood, my phone rang. On the line was my childhood physician and CF  doctor from St. Christopher’s Hospital for Children, Dr. Daniel  Schidlow. I hadn’t seen or spoken to him since our chance encounter five  years earlier in Madrid, Spain. Yet here he was, calling in the most  opportune moment. It was nothing short of miraculous.

​

For the  first time in 11 years I was now gravely sick and diagnosed with  pneumonia; but, it didn’t end there. After a week in the hospital, I was  only getting worse. Even with antibiotics, my body was refusing to  heal. My girlfriend at the time was living in Palm Springs and  immediately drove to San Diego with her sister when I called to tell her  it was more serious than I thought. According to her, my mind wandered  in and out of madness for the next week, a week of which I have no  memory. I was hypoxic for most of that week and, at some point, my  parents showed up in my hospital room. I was terribly confused by how  quickly the sickness took over me. By the end of that week, things were  critical. After a decade of being amazingly healthy, having a lung  function of close to 100%, I was once again back in survival mode.

​

The  thing that struck me the most was how quickly my mind and body  remembered what “survival mode” meant. It was like a switch flipped on  my brain, telling myself that, once again, it’s game time. No time for  tears, no time for fear. No time for sadness or pain. I was fighting in  another death match where there was no room for anything other than  living through the day and night. “Do not die today” and “do not die in  your sleep” were the only thoughts in my mind and only action in my  body.

Eventually  a decision was made to put me in a coma. I was lying in bed with my  parents, girlfriend, and the doctor’s assistant in my room when, within  moments, I couldn’t take another breath. Panic took over and I  absolutely felt myself slipping away. I begged for relief. I begged to  be put into the coma right then and there. The team scrambled as I was  gasping for air. Within what felt like seconds, an endotracheal tube was  inserted at my bedside and I was placed on a ventilator—a mechanical  breathing machine would now do my breathing for me. Just like that, I  was out of this world entirely. I was now unconscious, but the situation  would become far worse before it got better.

​

I later  learned that the first night a blood infection, sepsis, ravaged my body.  By the second day, I developed acute respiratory distress syndrome  (ARDS). On the outside, I only know what I was told afterwards. On the  inside, something entirely different was happening. Something I never  knew existed. Something I wish I still didn’t know existed.

​

A coma  is a strange thing. I certainly do not recommend it. That said, what  happened to me while I was in that coma was life changing. It is very  difficult to describe these things. A near-death experience is  simultaneously unique to everyone, and yet, certain aspects seem to be  universal. What happened in that month is something I have ever only  told a handful of people. Suffice it to say, I felt as if I was given a  choice, a decision had to be made. How and why this happened is still  unclear and probably always will be, but what happened is not.

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At some  point, I was in a line of people. I could see all of their faces as  clearly as I see this computer screen in front of me. In dreams, if you  look at your hand, you don’t see the lines, the veins, the  idiosyncrasies. This wasn’t that. I saw every detail of every face in  front of me and behind me. The face of an old man in front of me will  forever be an image in my mind. We were boarding some kind of vessel,  the closest I can explain it would be similar to an airplane, but it  wasn’t that. We sat in circles. Everyone was looking at each other, but  no one was speaking. Not a word was said. There were looks of comfort,  sadness, despair, and relief. A voice came over me, like a loudspeaker,  like some final instructions—don’t look down. This was repeated over and  over and over until everyone’s head seemed to understand and nod  backwards, eyes closed. The walls of this chamber were like liquid  metal; melding and moving and changing everywhere and always.

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And then  there was a moment within that in-between world and it was clear to me  that if I chose to leave, I could and if I chose to stay, I could as  well. I stood up and walked out. I left that in-between place and a  month later, I returned to this world. I woke up. I had no sense of time  when I awoke. I knew who I was, but how long it had been and what  happened on the outside was unknown to me. My muscles were so atrophied  that I could not use my thumb to change the channel on the remote  control. I was still intubated for the next week, which was extremely  frustrating. There were questions of whether I would ever regain my  previous strength, whether I would be able to walk again. I had to learn  how to do each everyday things all over—eat, speak, walk, shower, use  the bathroom—it was all challenging because it all felt new.

​

Ultimately,  it took a year and a half to fully return to where my body was prior to  that hospitalization. However, my lungs never fully recovered. I had  lost roughly 40% of my lung function. I now had chronic pain, chronic  shortness of breath, and perhaps worst of all, chronic anxiety of what  would happen next. I was diagnosed with post-ICU post-traumatic stress  disorder (PTSD), which in many ways, was far worse than the physical  pain associated with recovery. This was the second time PTSD entered my  life. First, immediately after my transplant and now, after this coma.

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Before  getting sick in 2013, my anxiety had been virtually nonexistent. From  2006 until 2013, I had no reason for it. The current of life was calmly  taking me along with it, peacefully and gently. Now, everything had  changed. I was hyperaware and worried about when and where the next shoe  would drop.

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That  anxiety has stayed with me ever since. For me, this is one of the more  sinister qualities of CF – that ability to not let myself put my guard  down. The need to be on top of everything all the time. It is one thing  to be diligent and aware but another thing entirely to be wired and  worried of things yet to come. That struggle is one that many people  with CF face—the battle to be cautious while still maintaining a healthy  degree of ease and comfort.

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We have a  saying in my family, “Don’t worry until you have to worry.” This has  been a mantra of mine for many years. It has helped me during the trying  times and allowed me to enjoy and thrive during the calmer days. Still,  there is a part of me that always wonders when the next big infection,  the next serious hospitalization, or the next “survival mode” will come.  Since 2013, there have been two or three times when I’ve needed to flip  that switch in my mind again and I’m certain that there will be  additional times in the future. This is where the strength of the CF  community is fundamental and essential. My family and friends, doctors,  nurses, social workers, and everyone in the cystic fibrosis orbit plays a  part in the physical and emotional well-being of all of us patients. It  is a small, but fierce community; a joyous group of dedicated people  who go into battle on a regular basis.

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As a  child with CF, I never gave serious consideration to what it would  actually mean to have a disease my whole life. I knew it was somehow  important but I never imagined that it would be a defining  characteristic. Of course, having a disease is just one part of who we  are, but a substantial one nonetheless. And though it is often filled  with uncertainty and pain, it also provides an enormous amount of  strength and resilience. Like water on concrete, my CF has been involved  in every aspect of my life, whether it be relationships, career, money,  love, and even death. Having cystic fibrosis has given me a perspective  on life that sometimes makes it difficult to relate to everyone else.  For the longest time, I didn’t know if this was a good thing. One  certainty that I have come to not only accept, but cherish, is the idea  that nothing in this world is trivial. Nothing is trivial. Every smile  and handshake, every I love you, every ounce of pain and every moment of  joy, every delicious meal, every laugh, every great movie, and every  hug and kiss. It all means something, even if we don’t realize it in the  moment. One day, when we are facing the end, we can look back and smile  at those precious moments and be grateful. This is what CF has given  me. And, for that, I have come to understand its true potential and be  grateful that I was born with this disease.

Andrew  Corcoran is 43 years old and has CF. He received a lung transplant in  2002. He now lives in South Jersey with his family and friends. He is a  writer. Andrew’s email is acorcoran@usacfa.org.

Age: 30

Home:
Boston, MA

Readers,  I’m excited to offer a deeper look into the life and work of one of our  former board members, my dear friend Ela Castillo! Ela and I first  connected because of our shared passion for disability justice and our  experiences as ethnic minority patients in “the final 10%” with rare  CFTR mutations. Since joining USACFA as a Director, Ela has led content  translation and community outreach efforts for Spanish speakers and  contributed consistently to accessibility improvements with our website  and materials. We are tremendously lucky to have had Ela on the board  and are looking forward more collaboration in the future!

What  would you most like our readers to know about who you are and what makes  you feel connected to others with CF? How has connecting with other  disabled people with CF impacted you?

The  thing that most connects me to other disabled people with CF is the  sense of understanding. Whether we have similar or different  experiences, we support each other first and foremost, through highs and  lows and all the rest—and we’re still here. Our community understands  those dynamics uniquely.

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Can you  tell us about your work in disability advocacy and accessibility  education? How have your interactions with other disabled folks with CF  shaped your work in these areas?

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After  working for a few years post-university graduation, I noticed a bunch of  trends in my work life that some might frame as negative  experiences—though they could also be learning experiences. These kept  making me want to learn how to better navigate those situations and  specifically things like how we disclose our CF at work and how we  generally navigate disability related to it. I read things that were  more like general advice about disability rather than something specific  to CF. I had been involved in various disability organizations outside  of the CF community and was finding the available resources were not  applicable enough in a practical sense. So I started asking people with  CF about their experiences. I found I wasn’t alone at all in my  experiences with disclosing my CF and trying to maintain my health while  working. I thought “there’s something here that’s not being fulfilled  within the dialogue of being an adult with CF or being a disabled adult  in general.” So I decided I needed to make some resources, then I  decided to sell them to people and companies. Some companies were doing  employee research networks—but these days they actually budget for it  and take it seriously.

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What do  you feel proudest of in your work lately? Can you share some anecdotes  about the impact you have been able to make with your consulting?

I’ve  been in a bit of a transition. Utopia was not really the goal of a lot  of these companies. I had to communicate pro-disability ideas in a way  that was very capitalistic. I didn’t feel great about selling justice  that way. Other disabled friends agreed outcomes mattered most, but it  was still draining to work that way. Now I’m moving into broader  projects. Instead of playing whack-a-mole with how individual companies  can become more accessible, I’m trying to impact accessibility design on  a larger scale. It would be easier to make things like Zoom more  accessible across the board, for example. Deaf advocates have focused on  improving captioning because everyone should have access to the  information. It’s very bottom-line—and of course we know people in the  CF community want this. Whether we’re vesting during our treatments or  have hearing loss from Tobi or unrelated factors, everybody should have  access to materials. Same thing for visual accessibility, neurodiverse  accessibility, and everything else.

Tell us  more about your own journey with CF. Besides lung issues, what are your  biggest challenges? What is your unique presentation of the disease  like?

My  physician would say I mostly fall under the category of “mild” CF  symptoms. As a baby I had respiratory problems and low weight; that’s  how I got diagnosed. I had a PICC line when I was 15 but one time it  fell out. Then I wasn’t believed about it falling out—I told a nurse  something felt cold and then hot, and then it burned and felt painful.  She said “no way”—but we did an X-ray, which showed the line was 9  centimeters out. I just said “Hop off my team. This bus is only for  people who believe me.” Having milder symptoms is also really tied up in  ableism and the idea of being a “good” disabled person in terms of what  you can do, like athletics. That’s always been a challenge in CF care.  Sometimes people don’t realize folks with rare mutations or mild  symptoms can still have unmet needs and be disabled.

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What  were your experiences with transitional care like during your teen  years? What did you struggle with most making the switch to adult care?  What went well during that time?

I wasn’t  very compliant with treatments, especially in my teen years. I hated  doing my vest every day—I still hate doing it. I think there was a lot  of identity wrapped up in those things, too. If your PFTs are good,  people think you are good. If they dip, that makes people think badly of  you. I always used to have an FEV1 above 100%. When it started going  lower, it was hard to deal with—because I was always “the good one” to  my providers. I wasn’t a gifted child in any other respect, like  academically, but I had that. I was rewarded for being a “good” CF  patient. There was so much anxiety and pressure around that number and  what it meant. I’m not blaming anyone specifically for that; it’s just  how things were back then. I didn’t have any model for how it could be  reframed at all.

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How does  being from Latin and Jewish cultural backgrounds shape your experiences  in the CF community as a whole? What makes community activities  welcoming and affirming for you?

Sometimes  being mixed can create internal conflict but there’s beauty as well,  and things that are heightened because of those influences—whether it’s  food or music or anything else. I feel enriched in my experience of the  world. That includes feeling mixed in terms of hearing and Deaf identity  too. Currently I’m identifying as Hard of Hearing. I’ve been closer to  Deaf when I was younger; I’ve been more hearing before as well. It’s  similar to my experience being mixed ethnically—like the best of both  worlds. In the CF community, I do feel isolated still because of my rare  mutations. Sometimes I feel left out or forgotten but adults with CF  are passionate about supporting each other and continually remind the  community of the importance of inclusion. Especially as people talk  about returning to “normal” we’re here to advocate for hybrid events and  meaningful virtual participation options that offer a comparable  experience.

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What  helps you cope with your CF? Are there any community resources you've  found helpful in living with CF—like websites, newsletters, apps, social  media groups?

Learning  American Sign Language has been like breathing air. I walked into it  hardly knowing anything save some alphabet stuff and words I’d picked up  here and there. I didn’t know much about grammatical systems or other  nuances. I also didn’t know any of the pillars of Deaf culture—the  mythologies, the best practices, the shared understanding. I didn’t know  any of it—and walking into it just felt like coming home.

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What  accessibility resources are you most excited about right now? What are  the most helpful technologies you have been exploring since the start of  the COVID-19 pandemic and how have they impacted your work?

I love  to be the person who talks about how much they love captioning and  translation. The Cystic Fibrosis Research Institute does amazing  captions with Worldly AI, for example. And software like CQ Fluency  opens up content for people who speak different languages without  putting unrealistic expectations on people. That’s what disability  utopia looks like to me. It’s not always easy to get access to all the  resources we need as people with CF—or even know where to look. A huge  problem in the CF community is that a lot of people assume that you  already know about things. But I know so many things now because I’ve  had to go searching for them; I’ve had to become the expert. I initially  started following CF research simply for self-preservation—to know what  clinical trials and innovative therapies are available to me, which is  especially important as someone with rare mutations and other coexisting  conditions. I wish I could have “accessibility bingo” where my multiple  conditions and disabilities can all be accommodated. Some things  overlap and go really well together—for example, epilepsy and CF both  require high-fat diets oftentimes. And being a CF patient and Hard of  Hearing, having captions is a huge win—a lot of us have hearing loss  from things like harsh antibiotics or sinus infections. However, I’m  struggling more to find that equilibrium with my epilepsy; a lot of  resources for Deaf and Hard of Hearing folks are incompatible.  Everything flashes! When things flash I don’t have bad photosensitivity,  but I do lose my balance. How do you find that happy medium when not  all of your disabilities get along with each other? That’s something I’m  still navigating.

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What are your hopes for the future—in your work and otherwise? What do you look forward to most these days?

I hope  that the work I do will help everybody become better and do better—that  everybody will rise up together—whether it’s disability specific or  CF-community specific. By creating a more accessible world, and then a  more inclusive world—an intersectionally inclusive world—everybody wins.  I don’t know if my criticism comes across too harshly sometimes. I’m  not neurotypical enough to know if that’s happening! But I do give a lot  of feedback and continue to show up uninvited to get feedback, because I  love the community. I don’t do it out of malice or anything like that.  If I’m making a criticism or a complaint on something that is not  working so well, I hope to give glowing feedback once the issue is  fixed.

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Do you have a funny CF story you'd feel comfortable sharing? Is there an experience you look back on now that makes you laugh?

This is  so not a “top five” CF issue, but I have these two toes that swell up  and turn purple. It’s probably related in some way—a lot of us have  vascular problems—but nobody can figure out how! And I’ve had to explain  all this to several different types of providers with limited  accommodations to help me communicate. When I go to clinic people want  my oxygen levels of my PFTs and I’m trying desperately to talk about  this toe thing. When it gets bad I can’t fit my foot in shoes  anymore—and this is Boston, and it’s winter! I can’t wear boots, so I  can’t walk anywhere; I can barely walk inside my house on it. I went to  urgent care and they were trying to run all these totally useless tests.  I’m amazed they didn’t try to give me a pregnancy test! Meanwhile, I  could hardly understand anything folks were saying because there was  just no accessibility, and nobody was making the effort to communicate  with me. Then this one guy did but he kept yelling at me so my  captioning device couldn’t process what he was saying. I kept telling  him “please just talk normally” while this other doctor was explaining  why it was okay for people to treat me like crap. Finally, I just went  home and followed up with my primary care doctor. Eventually I had an  MRI on the toes—but we still don’t know why they’re turning purple,  swelling up, and losing feeling.

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Living  with CF constantly requires us to change our plans and accept difficult  things. What would a perfect day look like for you? Where would you go  and what would you do?

It’s not  about waiting to be cured. It’s not about waiting for anything like  hearing aids. I know better devices are out there somewhere—and at the  beginning of the pandemic, I was hung up on hearing aids. I really  thought that was like my cure. I thought I’d save up enough money—or  maybe somebody would help me pay—and I’d be ready to go. If there’s ever  something appropriate out there, I might still go for it. But the  question for me now is “What do I want to get out of the hearing aids?  How am I moving towards my own personal utopia?” Lately I’ve tried to  ask all the households that I’m living in or connected to the same  question. How do we move towards our own utopia? How do we reduce  friction in getting there? Our journey doesn’t have to be  monumental—we’re not like Rocky climbing the steps. But it does require  being intentional—for us to seek our own utopia individually and  collaboratively.

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

When I was born, I laid in my mothers warm embrace

until a doctor noticed something wasn’t quite right

A curse, became folklore

“Woe to the child who tastes salty from a kiss on the brow, for he is cursed and soon will die.”

My parents told I had Cystic Fibrosis

Wave after wave of doctors

Fear crashed over them

Inhale

Exhale

Normal Breathing

Deep breath

Faster Faster

Exhaling my version of a whale spray

Hours of treatments.

Too many pills to swallow

enzymes sprinkled like sand atop my food

Yet

Pushed toward learning and growing

So much joy and laughter

-

I’d wake as if pulled from the ocean

Coughing sea spray

Flipped around to different positions like a small boat in the middle of the sea

Waves beating the sides

Loosening mucus from my lungs

-

In a household rooted in purpose

With fundraising leaflets

And events where I glimpsed sunlight shimmering through the saltwater

I was at risk of sharing germs

“Not too close”, my mother warned. “Flowers need space between them to help them grow.”

But I choose connection over caution

and stole a hug or two

I leaned into the light

I spoke at those splashy events

Shared my hopeful truth

Until those waves crashed over me

Seeing facts I hadn’t known

Hope was not my truth

Seeing the darkness in the ocean

I stopped planning for the sun

And the ocean’s rip current pulled me under

Salt and sand heavy in my lungs

Friends gone out to sea and not returned

I am next, I am sure

And when I visit the doctor

I’m still in that boat, spinning on the sea

Inhale

Exhale

Normal Breathing

Deep breath

Faster Faster

Exhaling my version of a whale spray

-

It was a delicate balance to stay rooted

Surrounded by rocky seas

Where I knew others had been lost

That fear was always there

Yet

My body ages to match my soul

Living in the moment, yet planning for an uncertain future

The ocean receded and my roots grew, stabilizing me

When our eyes met, I knew

Yet I held my breath as I told him

Waiting for the tide to rush back in and knock me over

He just held out a hand and offered me the sun-

Jumping into the ocean

Where the waves toss us

The salt stinging our eyes

Yet, we swim together

Holding each other up through any storm

Our love and hope evergreen

Katherine  Lockwood is 35 years old and has CF. She lives on Cape Cod with her  husband Arden and her girls, Rose and Magnolia. She is a therapist for  Verge Therapy and focuses on supporting individuals and couples  experiencing disability in the family. She is the author of Why Me, Mama?, an  award-winning children’s book about the disability experience. She is  currently working on two picture book projects: Salt & Roses—  supporting families when a parent has cystic fibrosis—and OUCH! OOPS! & OH NO!, a set of three books to support pre-k to third graders in reducing  bullying. You can follow Katie’s picture book projects at  acorncottagepress.com and on Instagram @acorn_cottage_press.

I  was 33 years old when I was diagnosed with small bowel cancer. I had  gone through the ups and downs of cystic fibrosis my whole life but I  never imagined I would be diagnosed with cancer. I didn’t know many  people with both CF and cancer (post-transplant cancer was a different  story) and to me it was one of those things that just didn’t happen or  was the “ultimate crappy card” on top of already having cystic  fibrosis.

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Getting  this diagnosis was hard—I was pregnant at the time the pain started so I  just thought maybe it was an issue related to that but after the pain  persisted post pregnancy, I knew it was something else. I told my CF  team about my symptoms: constipation, diarrhea, pain that ranged from an  8 to a 10 on the pain scale, and my bowel movements were different as  well. My dietitian and I just chalked it up to CF—I already had DIOS  (Distal Intestinal Obstruction Syndrome) and a lot of my symptoms were  so similar except for the pain. I had never experienced such severe pain  so I went to the CF clinic’s GI doctor and from there I had a CT scan,  endoscopy, and colonoscopy. Boy was I glad we went that route! Having  cystic fibrosis made it hard to get answers because just about  everything that was happening was “normal CF” in my case. I had  constipation, diarrhea, pain from time to time, but the truly telling  signs didn’t happen until a few months before I received the official  diagnosis.

Months  after seeking answers we found out via my colonoscopy results (I had two  separate colonoscopies) that I had small bowel cancer.

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I was  scared, upset, numb, and overall shocked. I thought I was already up  against a tough battle as it is; how could I possibly handle any more?

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I was  sent to a GI oncologist where we discussed surgery and chemotherapy and  ultimately decided on a small bowel resection first followed by  chemotherapy. I was diagnosed with stage IIIA small bowel cancer, which  had possibly been growing for close to ten years based on the size of my  adenocarcinoma.

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Having  cystic fibrosis along with small bowel cancer was hard emotionally;  however, it was only after I was nearly finished with chemo that I  realized how strong I was and how I just stuffed down my emotions. I  went through cancer with “grace,” with a tough face, and with a “this is  just another thing to deal with” attitude. I truly believe my life with  cystic fibrosis was the reason why I fought hard and didn’t let it get  to me too often. When I did let it get to me, my emotions overwhelmed  me: I cried hard, I begged for others to truly understand how hard this  was to manage on top of CF and how unbelievably complicated everything  in my life is with both diagnoses. I don’t understand the ‘why me’  mentality but I do understand that I am thankful I had already grappled  with my mortality before Trikafta was even an option for people with CF.  I feel like CF prepared me for this in ways that (and I cannot believe I  am saying this) made me thankful for it. The hardest part of having  cancer on top of cystic fibrosis was the recurrent infections and their  toll on my body. I got sick frequently and when I did it was far worse  than any sickness I had experienced in the last few years. I got  COVID-19 for the first time (not just once, but twice) during  chemotherapy, along with recurrent lung infections, colds and a bout of  pneumonia. Surprisingly, my weight remained semi stable and only  fluctuated up and down five pounds during the two-week course leading up  to my next chemo treatment. I truly believe Trikafta and CF allowed me  to tolerate the harsh chemicals going into my body. I ached, I couldn’t  sleep, I had no appetite one day and then was ravenous the next. I  couldn’t tolerate cold drinks or touching anything cold for a couple  days at first and then for the first ten days the further along I got in  the 12 rounds of chemo I completed. I started to realize how similar  the side effects of CF were to those of cancer: the longer I was on  chemo (just as with antibiotic use) the worse the side effects were and  the harder it was to tolerate them. I knew that I had to endure this  though.

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Fast  forward to December 2023 and my diagnosis changed from stage IIIA to  stage IV because it metastasized to my ovary. As of January 2024, I had  surgery to remove the metastases to my ovaries with the recommendation  of another round of chemo afterwards. My oncologist called me a couple  weeks after the surgery and said we no longer needed to do chemotherapy  as my Signatera blood tests were negative (meaning no signs of cancer). I  know there is a chance for recurrence or metastasis again but I am  hopeful just as I am now with CF because of drug advancement. For other  CF patients who may have to go through cancer, regardless of whether it  is similar to mine, know that you are not alone. You will have good and  bad days as we have had all our lives, and there is a massive support  system within the cancer community, and some within the CF community. I  am with you on this and with you through this because we all need  someone who is like us to help make it easier. I want you to also know  you are allowed to feel confused, upset, questioning and so many more  emotions. Because of cystic fibrosis, I am and will always be a fighter.

Tamara  Jimison is 35 years old and has cystic fibrosis and Stage IV small  bowel cancer. She lives in Washington State with her husband John and  two children, Julian (8) and Audrey (2). She loves reading, writing,  crafting, music, drawing and creating yummy meals for her family. Tamara  loves being a mom more than anything. You can contact her at tamarakayroth@yahoo.com.

Did  you know people living with cystic fibrosis (CF) are five to ten times  more likely to get colon cancer and if you have had a transplant, you  are 20 times more likely? Also, CF carriers also have an elevated risk  of colon cancer. Yeah, neither did I until I was diagnosed with stage IV  colon cancer at age 34. That’s one of the reasons I have been trying to  raise awareness for earlier screening. I want to make sure people have  the knowledge about their elevated risk.

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I was  diagnosed with stage IV colon kancer (I spell it with a “k” to take away  some of its power) in July of 2021. I had just started experiencing the  positive impact of Trikafta and I was feeling better than ever. My  journey to a stage IV diagnosis doesn’t even involve a diagnostic  colonoscopy.

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I had  developed a dime size mass in my groin in the summer of 2021. I had  shown it to a few doctors and no one could really figure out what it  was. At the same time, no one seemed very alarmed either. Around the  same time I also developed some strange digestive symptoms that mimicked  CF digestive symptoms. I alerted my CF team and they ordered some  x-rays and a gallon or two of Golytely. In the meantime, the mass had  become the size of a golf ball and I knew I couldn’t wait the two weeks  to get the scheduled CAT scan. I needed help and I needed it now. My CF  doctor sent me to the ER to get the CAT scan and I’m so grateful he did.  I spent four days inpatient waiting for a diagnosis and/or a biopsy of  the mass. Before the biopsy was performed a team of doctors came in and  told me they believed that the mass was malignant, and that my life was  about to get harder.

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The  biopsy came back a week or so later, which was the longest week of my  life and it confirmed all of my worst fears—it was colon kancer, it was  likely stage IV, and it was aggressive. My CF doctor had already called  the oncologist about my case when he delivered the news to me. This  helped me get an appointment as soon as possible. I remember crying—ugly  crying—for almost two hours straight while yelling this was not fair. I  was supposed to get a break; TriKafta was supposed to give me a break.

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Currently,  the standard recommendation for people with CF is to undergo their  first colonoscopy at age 40 to screen for colon cancer. I couldn’t help  but feel like this outcome could have easily been avoided with earlier  colon cancer screening. I was only 34 when I was diagnosed with colon  cancer (I’m 37 now). I would have never made it to 40 despite having had  no family history or genetic markers to point to colon cancer.

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It’s  frustrating that colon cancer is one of the most preventable cancers if  caught early enough. If you catch it too late it transforms into the  second most deadly cancer. It doesn’t help that colon cancer hides in  the shadows. It typically manifests as a silent cancer with few or no  symptoms. If a person with CF does have symptoms, they often mimic the  symptoms of CF. The unfortunate truth is that it is nearly impossible to  detect colon cancer in someone with CF without screening. How many  people are walking around with colon cancer right now and don’t know it?  That’s my concern; that’s what drives me to share my story in the hope  that maybe one less person has to hear the words “you have stage IV  colon kancer.”

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My story  shows that too many people are going to fall through the cracks. I wish  I could claim to be a rare exception, but both data and evidence tell  me I’m far from alone. A recent publication from the American Cancer  Society stated that colon cancer is the number one cancer killer for men  ages 20-49 and the number two cancer killer for women. By 2030, colon  cancer will be the number one cancer killer for people under 50. As CF  patients we are at an elevated risk: five to ten times higher than the  standard risk for those of us who haven’t had transplants and 20 times  higher if you have had a transplant.

​

I had to  re-learn how to live my life with kancer and still manage my CF. I  still work full time and I still undergo chemotherapy every two weeks.  I’m not complaining; this is just how it is and will be until science  and medicine find something better.

I  couldn’t save myself but I’m hoping I can save you—a colonoscopy is  annoying but I can assure you it’s worth it. If I had a DeLorean to go  back in time I would in a heartbeat. I would have asked to get a  colonoscopy in my early 20’s just to confirm that my digestive issues  were due to CF, not cancer. I can’t do that but maybe you can. The colon  kancer lived inside me silently for years without me knowing it. A  polyp can take ten years to develop into cancer. I unknowingly got  married with kancer, got divorced with kancer, and ran for office with  kancer.

​

Next  time you go to the doctor, I’m urging you to ask for a colon cancer  screening. If something feels off don’t write it off or ignore it. I  know, I know, it’s not particularly fair to put that additional burden  on CF patients, but it’s the only option we have. We have to be our own  fierce advocates until the screening age is lowered. Your doctor might  push back, but don’t take no for an answer! CF doctors and care centers  use the guidance set by medical experts and regulatory agencies to  determine when to screen CF patients for colon cancer. And, even with  these guidelines, not all CF doctors are aware of the well-known  elevated risk of gastrointestinal cancers among their patients. Early  screening could mean early detection of an otherwise hidden cancer.  Early detection means potentially finding cancer in earlier stages,  which dramatically increases the possibility of effective treatments or  even cures. Treatments and cures mean saving lives, lives that matter.  Lives like mine.

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I’m not  advocating for myself. I’m hoping to save you. Please help me honor  those who are no longer with us by educating those who are.

Anna  is 37 years young and has CF. She resides in Langhorne, PA. She enjoys  time with her beloved dog Roman and works full time for Bucks County  Commissioner, Diane Marseglia, while also being an elected Middletown  Township Supervisor. She is also the Vice Chair for the PA Rare Disease  Advisory Council and helped co-found the Bucks County Cystic Fibrosis  Alliance. You can follow her on Twitter @AnnaPayne4PA or on instagram  @apayne226. You can also email her directly at apayne226@gmail.com.

It  used to be, back in the 1950s, the word cancer was whispered or not  even said at all, but rather called the “C-word” or the “Big C.” Most  likely it was not spoken of because by the time a person was diagnosed  with it, it had progressed too far and they had mere months to live. It  was just too scary to say out loud.

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Treatments  and screenings were not what they are today. Nowadays, having a  diagnosis of cancer can be something a person can live with, like a  chronic condition, or at least choose a plethora of treatment options  that are not as full-body decimating as classic chemotherapy. With newer  personalized treatments available people are able to take drugs that  target just the cancer with less collateral damage to other cells and/or  organs.

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Before I  received my lung transplant in April 2000, I read other people’s  stories about their lives after transplant. One thing stood out in all  those stories—for many, a few years after transplant the prevalence of  cancer and sadly, death. At the time, I didn’t understand the connection  between the lack of a strong immune system to fight off unwanted cells  or invading viruses and cancer cells having an open invitation to invade  the recipient. Some cancers are more opportunistic in CF and  transplanted people—colon, skin, and surprisingly, lymphoma.

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A person  can live the healthiest of lifestyles—at least I thought I did—and  still get cancer. When I was diagnosed with post-transplant  lymphoproliferative disorder (PTLD) in October 2017, I was extremely  angry. I spent so much time doing what I thought were the right  things—eating right, exercising, sleeping enough, etc. However, when a  stressful situation presents itself and knocks one flat on their back,  an opportunistic virus can run amok in an immune-suppressed body. For me  this was the Epstein Barr virus (EBV), which my donor most likely had  and I did not. Learning about this was a several month-long journey when  I was just starting to get my bearings after my mom passed away.

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Most  people with CF, at least those of my age, who grew up relying on their  parents for many things such as emotional support and help with  treatments, had an incredibly close relationship and bond to one, if not  both parents. I was fortunate to have this with my mom, who not only  helped me when I was sick as a child, she also took care of me while I  waited for a lung transplant when I was unable to cook, clean, or do  much of anything for myself. She did this selflessly as she always did  when I needed help. So, as she was aging and needed my help, I stayed  with her and took care of her. I dreaded the day she might not be  around. We had a symbiotic relationship that may not have been the  healthiest but we knew how to care for one another.

When my  mom’s health took a turn for the worse and when she subsequently died at  the end of May 2017, I was devastated by this loss. During the five  weeks she was in and out of the hospital and then finally in hospice, I  was stressed far beyond anything I can remember. I cried a lot and felt  helpless in getting my mom the best care. Trying to be at the hospital,  do my treatments before and after, get to the gym, and take care of my  dogs all took a toll on my health.

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Immediately  after her memorial, I was treated for a sinus infection for which I did  IV antibiotics for about six weeks in July and August. During that  time, I started noticing bumps protruding from my body. First was in my  groin, then on my upper left chest and then one on my back. The three  separate bumps were looked at by different specialists but no one  connected the dots (bumps) until I was on the IV antibiotics that summer  via a peripherally inserted central catheter (PICC) line and I felt I  had a blood clot. My transplant nurse told me to go to an ER and it was  there that I had an upper chest sonogram that confirmed a clot around  the PICC line. The ER also ordered a chest CT with contrast. It wasn’t  until several months after that, in October, that the radiologist  reviewing the CT scan from the ER called my transplant doctor and told  her that they saw lesions all throughout my trunk—the bumps were  protruding lymph nodes. Aha! I was sent for another CT with contrast  that confirmed the lesions were not only still there but they had also  grown in size. During this time, I was blissfully ignorant about what  all this testing was for—I was just relieved my sinus infection was over  and I no longer had the PICC line in. I remained incredibly sad about  my mom and had a hard time remembering the fun times we shared. The last  five weeks of her life were tortuous and those memories were crowding  out the good times and laughs we had.

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Once my  transplant doctor knew these lesions were present, I was ordered to do  more labs. This was all before MyChart or EPIC so I had no idea what my  results were or why they were testing my EBV levels. The levels must  have been high because I was told to get a surgical biopsy of my lymph  nodes as they were enlarged and a needle biopsy would not be sufficient  for testing for cancer. It was then that I found out what they were  looking for—lymphoma. At first, it was difficult for me to find a  surgeon who could do the biopsy but once I did, the results confirmed my  transplant doctor’s fear of PTLD, where the immune-suppressed  transplant recipient can be more susceptible to the donor’s EBV. This  usually happens immediately after transplant, not 17 years later, as I  was at the time. I had also experienced extreme fatigue and was not sure  if it was from depression surrounding my mom’s death or that I just  needed to sleep and nap a lot every day.

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Finding  out I had this cancer upset me tremendously. I was in shock and,  initially, lost my drive to fight it. But my transplant doctor said that  we did have to treat it. I wanted none of what I knew about getting  cancer treatments: the long days in a hospital getting infusions, losing  one’s hair, vomiting, injections for white blood cells or red blood  cells, and so on.

Thankfully,  my transplant team sent me to a world-renowned oncologist who was  brought to NY Presbyterian Hospital specifically for this type of  cancer. Even though it is rare, this cancer requires an experienced  physician who is also familiar with transplant patients and their unique  needs. By November 2017, it was decided I would get a port in my chest  for an IV medication called Rituximab, which is used for B-cell lymphoma  and other diseases. With this drug, there was no hair loss, like with  some chemotherapy treatments, but shortness of breath was an issue when  it was administered weekly.

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As  treatments grew farther apart, my shortness of breath reduced and I felt  less fatigue. I was back to a somewhat normal life and after two and a  half years, I was considered to be in remission. I received my last  Rituximab treatment in December 2019. I was in remission for two years  until April 2021. I had a feeling it had returned because I felt that  incredible fatigue again. I was not surprised that my EBV level was  rising and my PET scan confirmed it was baaa-ack! Since I could not be  treated with Rituximab again, I had to meet with a new oncology team  doing clinical trials on treating EBV in post-transplanted people. When  they decided to take me on as a patient, they congratulated me on having  several rare diseases all at the same time—CF, lung transplant, and  PTLD. Thankfully this oncology team existed! And they had a sense of  humor.

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Under  their care, I was to be given donor cells from people who had EBV and  their own body had successfully fought off the virus. Ostensibly, these  donor cells would kill off the EBV in my body. The treatment length  would depend on how my body responded. I went through six rounds,  lasting about eight months in total, with a PICC line, because they  could not use my port for the cells. For my sixth treatment, I was told  that even though the treatment had worked on the cancer, some of it was  still present in a lymph node in my right axilla and I would have to  stop receiving the donor cells. Since the remaining cancer was  localized, it was suggested I start radiation for eradicating it  completely. Yet another oncologist, three in total—a regular one, a  clinical trial, and then a radiation oncologist. Thankfully, they were  all lovely and eager to see me get well.

Radiation  was hard in that it was every day for a month and even though I joked  that NY Presbyterian is my second home I started to feel as though it  was my first! I did get the “sunburn” affect after a week and had to  apply petroleum jelly twice daily to keep from scarring. I was also  warned by the radiation oncologist that the radiation could scar my  lungs (since it was in my arm pit) and, in possibly 10-15 years, could  cause cancer. Let’s just deal with today folks, I do not expect to be  kicking around that long!

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When I  finished with radiation I exuberantly rang the bell in the waiting room  for those who are done being treated in a way to exclaim, I made it! And  I am sure it gives others hope that they will ring that bell one day  too. However, I felt I was not done; maybe for now, but when will it  come back?

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As of  March 2024, I am in remission. For how long, I am uncertain. I still see  my regular oncology team every four months. I get lab work for EBV  levels every two months and hope that even if the levels are high one  month, they reduce the next. Any treatments I receive are weighed with  what they will do to my EBV levels. Like being on steroids or having an  infection, both will send the level much higher. As my dog Trixie shares  on page 10, when I was treated with Thymoglobulin (antibodies) for  rejection, my levels were stratospheric but expected to be such. They  were almost so high, my doctor and I laughed about it after the fact.  But the fear remains and the EBV is most likely here to stay in my body.  I just hope that it will remain dormant. I am still tired a lot and  that worries me as the extreme fatigue can be a sign that PTLD has  returned. I remain grateful to be alive, but with cancer, I feel the  specter of its possibility always lurking. I am careful not to get too  stressed out, keep exercising, practice mindfulness, eat a healthy diet,  and be grateful for what I have today. And I am.

Andrea  Eisenman is 59 and has CF. She recently realized that her initials are  AGED: Andrea Gail Eisenman Downey (her husband’s surname)! She lives in  New York, NY, with her husband Steve and dogs, Willie, Roscoe, and  Trixie. Andrea is the Executive Editor for USACFA. She enjoys cooking  new recipes, playing pickle ball, biking, tennis when possible, and  staying active as her health allows. Her contact information is on page  2.

USACFA  proudly offers four different scholarships! You may apply for more than  one scholarship each year, but you may only be awarded one per academic  year. If you do not win, your application can be moved to the pool of  applicants for another relevant scholarship in the same cycle. For  questions about future scholarships or anything related to the  application process, please contact us at scholarships@usacfa.org.

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

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

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

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Higher Education Scholarship (Deadline: 06/30/24):

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

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Any  student seeking a degree in higher education, from associate’s to  doctorate, is welcome to apply. We look for students who demonstrate  tremendous academic achievement, community involvement, and a powerful  understanding of how their CF—matched with these achievements—places  them in a unique situation to gain leadership roles within the  community.

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The Stenzel Scholarship (Deadline: 03/30/25):

The Ana  and Isa Stenzel Scholarship may be awarded once annually to a person  with cystic fibrosis who is, during the period for which the scholarship  award is paid, enrolled in a course of higher education leading to a  degree granted by an institution in the United States in either health  science, social work, mental health science, genetic counseling, or  environmental science.

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The  Stenzel Scholarship was established in 2023 in memory of two amazing  women with CF, Isa Stenzel-Byrnes and Ana Stenzel. Isa was a licensed  social worker and had a Master’s of Public Health. She imparted her  wisdom to CF Roundtable readers for 17 years in her CF Roundtable  column, Spirit Medicine. Ana Stenzel was Isa’s twin sister. Ana was a  genetic counselor at Stanford hospital for 16 years. Isa and Ana  dedicated their lives to helping others. They provided education, hope  and comfort to thousands of people throughout their lives. They showed  the world that people with CF could find meaning in their lives by  making a difference in the lives of others.

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William Coon Jr. Scholarship (Deadline: 04/30/25):

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

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

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

Identification Of Prognostic Biomarkers For Antibiotic Associated Nephrotoxicity In Cystic Fibrosis.

The  objective of this study was to discover novel urinary biomarkers of  antibiotic-associated nephrotoxicity using an ex-vivo human  microphysiological system (MPS) and to translate these findings to a  prospectively enrolled cystic fibrosis (CF) population receiving  aminoglycosides and/or polymyxin E (colistin) for a pulmonary  exacerbation. Polymyxin E treatment resulted in a statistically  significant increase in the pro-apoptotic Fas gene relative to control  in RNAseq of MPS. Effluent analysis demonstrated an acute rise of  soluble Fas (sFas) concentrations that correlated with cellular injury.  In 16 patients with CF, urinary sFas concentrations were significantly  elevated during antibiotic treatment, regardless of development of AKI.  Over a median of three years of follow up, we identified seven cases of  incident chronic kidney disease (CKD). Urinary sFas concentrations  during antibiotic treatment were significantly associated with  subsequent development of incident CKD. Using an ex-vivo MPS, a novel  biomarker of proximal tubule epithelial cell injury was identified, and  translated these findings to a clinical cohort of patients with CF.

https://tinyurl.com/mr3kh5c3

The Lived Experience Of African American Persons With Cystic Fibrosis.

Cystic  fibrosis (CF) is a rare genetic disease affecting approximately 30,000  people in the United States (US). African American persons with CF are  even rarer, comprising approximately 5% of this population. The purpose  of this study was to explore the lived experiences of African American  persons with CF to identify potential disparities in health care.  Descriptive phenomenology was used to explore lived experiences of  African American persons with CF over age 18 recruited from CF  Foundation-accredited Centers in the US, CF-specific social media, and  via snowball sampling. Study data was obtained through telephone  interviews that were audio-recorded, transcribed verbatim, and analyzed  using Colaizzi’s method of thematic analysis. Results: Six men and six  women (ages 23–45) completed the study. Interviews revealed three  themes: (1) Accepting a Diagnosis of CF; (2) Desiring a Normal Life  while Living with an Invisible Disease; and 3) A Slippery Slope of  Subtle Racism. Each theme had 2–3 subthemes. In conclusion it is  critical to explore the unique challenges faced by African American  persons with CF in order to develop interventions that improve their  daily lives and create better futures.

https://tinyurl.com/4pnjdsvr

Home-Spirometry Exacerbation Profiles In Children With Cystic Fibrosis.

Pulmonary  exacerbations (PEx) are strong predictors of respiratory disease  progression in children with cystic fibrosis (CwCF) and may be  associated with persistent decreased lung function after acute  management. Telemonitoring devices can be used for early detection and  monitoring of PEx, but its utility is debated. Researchers asked, “Which  symptoms and telemonitoring spirometry characterics are related to  outcome dynamics following initial PEx management?” This retrospective  study included CwCF followed at Bordeaux University Hospital, France.  All severe PEx episodes treated with intravenous (IV) antibiotics (ATB)  between 1 January 2017 and 31 December 2021 in CwCF using home  telemonitoring were analyzed. Symptoms and home spirometry data were  collected 45 days before and up to 60 days after each IV ATB course. We  defined three response profiles based on terciles of baseline forced  expiratory volume in 1 s (FEV1) recovery. A total of 346 IV ATB courses  for PEx were administered to 65 CwCF during the study period. The drop  in FEV1 became significant 8 days before IV ATB initiation. Forty-one  percent of IV ATB courses failed to restore baseline FEV1. The magnitude  of FEV1 drop and a greater delay in the initiation of treatment  correlated with a low response level. On the 14th day of the IV  treatment, a FEV1 recovery less than 94% of baseline was associated with  a nonresponder profile.Home spirometry may facilitate the early  recognition of PEx to implement earlier interventions. This study also  provides an outcome lung function threshold which identifies low  responders to IV ATB.

https://tinyurl.com/4ernvp5x

Computational Analysis Of Long-Range Allosteric Communications In CFTR.

CFTR  functions as an anion channel, the gating of which is controlled by  long-range allosteric communications. Allostery also has direct bearings  on CF treatment: the most effective CFTR drugs modulate its activity  allosterically. Researchers integrated Gaussian network model, transfer  entropy, and anisotropic normal mode-Langevin dynamics and investigated  the allosteric communications network of CFTR. The results are in  remarkable agreement with experimental observations and mutational  analysis and provide extensive novel insight. They identified residues  that serve as pivotal allosteric sources and transducers, many of which  correspond to disease-causing mutations. They found that in the ATP-free  form, dynamic fluctuations of the residues that comprise the  ATP-binding sites facilitate the initial binding of the nucleotide.  Subsequent binding of ATP then brings to the fore and focuses on dynamic  fluctuations that were present in a latent and diffuse form in the  absence of ATP. They demonstrated that drugs that potentiate CFTR’s  conductance do so not by directly acting on the gating residues, but  rather by mimicking the allosteric signal sent by the ATP-binding sites.  They also uncovered a previously undiscovered allosteric ‘hotspot’  located proximal to the docking site of the phosphorylated regulatory  (R) domain, thereby establishing a molecular foundation for its  phosphorylation-dependent excitatory role. This study unveils the  molecular underpinnings of allosteric connectivity within CFTR and  highlights a novel allosteric ‘hotspot’ that could serve as a promising  target for the development of novel therapeutic interventions.

https://tinyurl.com/5n7r3pmf

Long-Term Evaluation Of Fecal Calprotectin Levels In A European Cohort Of Children With Cystic Fibrosis.

Intestinal  inflammation with contradictory data on fecal calprotectin (fCP) levels  is documented in patients with cystic fibrosis (CF). The aim of this  study was to longitudinally evaluate fCP in a cohort of children with CF  and their relationship with clinical variables. Prospective  observational study to assess evolution of fCP levels, primarily aimed  at improving fat absorption. Along 1.5 years of follow-up (November  2016–May 2018) with four study visits pertaining to a pilot study (two  of four) and to a clinical trial (two of four), the study outcomes were  measured. This study included children with CF and pancreatic  insufficiency (2–18 years old). Main outcome measurements are fCP  levels, pulmonary function and coefficient of fat absorption (CFA).  Additionally, in the last two visits, gastrointestinal (GI) symptoms  were evaluated through the PedsQL-GI Questionnaire. Linear mixed  regression models were applied to assess association between fCP and  FEV1, CFA and GI symptoms. Twenty-nine children with CF and pancreatic  insufficiency were included. fCP levels were inversely associated with  total modified specific PedsQL-GI score and positively associated with  diarrhea, but not with CFA. Along the four study visits, fCP  significantly increased and pulmonary function decreased, with a  significant inverse association between the two study outcomes. In  conclusion, in children with CF, fCP levels are inversely associated  with pulmonary function and thus the specificity of fCP as a marker of  intestinal inflammation in pediatric patients with CF warrants further  investigation.

https://tinyurl.com/2b285kdf

Plasma Levels Of Chemokines Decrease During Elexacaftor/Tezacaftor/Ivacaftor Therapy In Adults With Cystic Fibrosis.

CF is  associated with dysregulated immune responses, exaggerated inflammation  and chronic infection. CF transmembrane conductance regulator (CFTR)  modulator therapies directly target the underlying protein defects and  resulted in significant clinical benefits for people with CF (pwCF).  This study analyzed the effects of triple CFTR modulator therapy  elexacaftor/tezacaftor/ivacaftor (ETI) on CF-associated inflammation,  especially systemic chemokines. A bead-based immunoassay was used to  quantify proinflammatory chemokines in plasma samples from pwCF  collected before, at three, and at six months after starting ETI  therapy. Fifty-one pwCF were included. At baseline, 67 % were already  receiving CFTR modulator therapy with tezacaftor/ivacaftor or  lumacaftor/ivacaftor. After initiation of ETI therapy there was a  significant improvement in percent predicted forced expiratory volume in  1 s and a significant decrease in sweat chloride levels. After 6  months’ treatment with ETI therapy there were significant decreases in  plasma levels of MIP-3α, GROα, ENA-78 and I-TAC. IL-8 exhibited a  reduction that did not reach statistical significance; levels of other  assessed cytokines did not change significantly from baseline. In  conclusion, ETI appears to affect a distinct group of chemokines that  are predominantly associated with neutrophilic inflammation,  demonstrating the anti-inflammatory properties of ETI therapy.

https://tinyurl.com/yxc2hs53

Dual  Species Sphingosine-1-Phosphate Lyase Inhibitors To Combine Antifungal  And Anti-Inflammatory Activities In Cystic Fibrosis: A Feasibility  Study.

CF is an  autosomal recessive disorder characterized by respiratory failure due  to a vicious cycle of defective CFTR function, chronic inflammation and  recurrent bacterial and fungal infections. Although the recent  introduction of CFTR correctors/potentiators has revolutionized the  clinical management of CF patients, resurgence of inflammation and  persistence of pathogens still pose a major concern and should be  targeted contextually. On the background of a network-based selectivity  that allows to target the same enzyme in the host and microbes with  different outcomes, researchers focused on sphingosine-1-phosphate (S1P)  lyase (SPL) of the sphingolipid metabolism as a potential candidate to  uniquely induce anti-inflammatory and antifungal activities in CF. As a  feasibility study, they show that interfering with S1P metabolism  improved the immune response in a murine model of CF with aspergillosis  while preventing germination of Aspergillus fumigatus conidia. In  addition, in an early drug discovery process, we purified human and A.  fumigatus SPL, characterized their biochemical and structural  properties, and performed an in silico screening to identify potential  dual species SPL inhibitors. They identified two hits behaving as  competitive inhibitors of pathogen and host SPL, thus paving the way for  hit-to-lead and translational studies for the development of drug  candidates capable of restraining fungal growth and increasing  antifungal resistance.

https://tinyurl.com/436xkbnn

Inhaled Aztreonam Lysine In The Management Of Pseudomonas Aeruginosa In Patients With Cystic Fibrosis: Real-Life Effectiveness.

Inhaled  antibiotics have achieved or stabilized the clinical condition of  patients with CF and chronic Pseudomonas aeruginosa infection. In this  study, researchers aimed to determine the effectiveness of aztreonam  lysine inhaled solution (AZLI) in patients with CF and chronic P.  aeruginosa infection. A retrospective observational study was conducted  on patients with CF and chronic P. aeruginosa infection who received  AZLI between July 2012 and September 2018 inclusive in three Spanish  hospitals in a routine clinical practice setting. The primary endpoint  was the absolute change in the percentage of predicted forced expiratory  volume in 1 second (FEV1) compared with the previous 12 months, at the  start of AZLI treatment and 12 months after starting the drug. Other  variables analyzed were exacerbations, hospitalisations, type and route  of antibiotics prescribed, weight and BMI and adverse drug reactions. In  a cohort of 52 patients, AZLI treatment led to stabilization of FEV1,  changing from a mean value of 55.60 (21.3)% at the start of treatment to  56.8 (20.4)% after 12 months of treatment in patients who had not  previously received the drug. In addition, it significantly reduced  exacerbations from a median of 2.0 in the 12 months prior to AZLI to 1.0  in the 12 months after treatment initiation. AZLI also reduced the need  for other antibiotics and prevented a decrease in BMI, with an adequate  safety profile. In conclusion, AZLI achieved stabilization of lung  function measured by FEV1 in patients with CF and chronic P. aeruginosa  infection, along with an adequate safety profile.

https://tinyurl.com/2s3hz7vb

Social-Environmental  Phenotypes Of Rapid Cystic Fibrosis Lung Disease Progression In  Adolescents And Young Adults Living In The United States.

CF is a  genetic disease but is greatly impacted by non-genetic  (social/environmental and stochastic) influences. Some people with CF  experience rapid decline, a precipitous drop in lung function relative  to patient- and/or center-level norms. Those who experience rapid  decline in early adulthood, compared to adolescence, typically exhibit  less severe clinical disease but greater loss of lung function. The  extent to which timing and degree of rapid decline are informed by  social and environmental determinants of health (geomarkers) is unknown.  A longitudinal cohort study was performed (24,228 patients, aged 6–21  years) using the U.S. CF Foundation Patient Registry. Geomarkers at the  ZIP Code Tabulation Area level measured air pollution/respiratory  hazards, greenspace, crime, and socioeconomic deprivation. A composite  score quantifying social-environmental adversity was created and used in  covariate-adjusted functional principal component analysis, which was  applied to cluster longitudinal lung function trajectories.  Social-environmental phenotyping yielded three primary phenotypes that  corresponded to early, middle, and late timing of peak decline in lung  function over age. Geographic differences were related to distinct  cultural and socioeconomic regions. Extent of peak decline, estimated as  forced expiratory volume in 1 s of % predicted/year, ranged from 2.8 to  4.1 % predicted/year depending on social-environmental adversity.  Middle decliners with increased social-environmental adversity  experienced rapid decline 14.2 months earlier than their counterparts  with lower social-environmental adversity, while timing was similar  within other phenotypes. Early and middle decliners experienced  mortality peaks during early adolescence and adulthood, respectively.  While early decliners had the most severe CF lung disease, middle and  late decliners lost more lung function. Higher social-environmental  adversity associated with increased risk of rapid decline and mortality  during young adulthood among middle decliners. This sub-phenotype may  benefit from enhanced lung-function monitoring and personalized  secondary environmental health interventions to mitigate chemical and  non-chemical stressors.

https://tinyurl.com/k7rdw5h8

Diminished Airway Host Innate Response In People With Cystic Fibrosis Who Experience Frequent Pulmonary Exacerbations.

Pulmonary  exacerbations (PEx) are clinically impactful events that accelerate CF  lung disease progression. The pathophysiological mechanisms underlying  an increased frequency of PEx have not been explored. The objectives  were to compare host immune response during intravenous antibiotic  treatment of PEx in people with CF who have a history of frequent versus  infrequent exacerbations. Adults with CF were recruited at onset of  antibiotic treatment of a PEx and were categorized as infrequent or  frequent exacerbators based on their PEx frequency in the previous  12 months. Clinical parameters, sputum bacterial load and sputum  inflammatory markers were measured on day 0, day 5 and at the end of  treatment. Shotgun proteomic analysis was performed on sputum using  liquid chromatography-mass spectrometry. Many sputum proteins were  differentially enriched between infrequent and frequent exacerbators.  The majority of these proteins had a higher abundance in infrequent  exacerbators and were secreted innate host defense proteins with  antimicrobial, antiprotease and immunomodulatory functions. Several  differentially enriched proteins were validated by ELISA and Western  blot including SLPI, lipocalin-1 and cystatin SA. Sputum from frequent  exacerbators demonstrated potent ability to cleave exogenous recombinant  SLPI in an NE-dependent manner. Frequent exacerbators had increased  sputum inflammatory markers and total bacterial load compared to  infrequent exacerbators.In conclusion diminished innate host protein  defense may play a role in the pathophysiological mechanisms of frequent  CF PEx. Frequent exacerbators may benefit from therapies targeting this  dysregulated host immune response.

https://tinyurl.com/mr2e9h8

Frequency  Of Obstructive Sleep Apnea In Patients With Cystic Fibrosis And  Non-Cystic Fibrosis Bronchiectasis And Its Association With Clinical  Findings.

This  study was designed to assess obstructive sleep apnea (OSA) in adult  patients with cystic fibrosis (CF) and non-CF bronchiectasis (BE) and to  relate it with clinical characteristics.

In this  study thirty-five CF and 35 non-CF BE patients were included.  Demographic characteristics, medications, comorbidities, BMI, dyspnea  scales, pulmonary functions, sputum cultures, exacerbations, and  hospitalizations were recorded. The Epworth Sleepiness Scale (ESS)  questionnaire was filled and polysomnography was performed for each  patient. ESS scores did not show any significant difference between CF  and non-CF BE patients. Thirty-seven (53%) of all patients had OSA.  There was no significant difference in OSA risk between CF and non-CF BE  patients (54% vs 51%, respectively). Male gender was found to be a risk  factor for OSA (68% of males vs 41% of females, respectively). Total  sleep time, sleep efficiency, sleep latency, time spent awake after  falling asleep, oxygen desaturation index, apnea-hypopnea-index (AHI),  AHI in the supine position, and rapid eye movement phase did not show  any significant difference between CF and non-CF patients. CF patients  had significantly lower mean oxygen saturation and lowest oxygen  saturation levels and higher heart rate compared to non-CF BE patients.  Multiple logistic regression analysis of all patients revealed male  gender and disease duration as risk factors for OSA. In conclusion, it  is remarkable that more than half of the patients in both CF and non-CF  bronchiectasis groups had OSA. Male gender and disease duration were  found as risk factors for OSA.

https://tinyurl.com/bhcdc6fb

Association Between Sputum Culture Results And Pulmonary Changes In Children With Cystic Fibrosis.

Despite  the significant improvement in the prognosis of CF, it is still regarded  as the most common life-shortening genetic disease in Caucasian  populations. This disease is the most important cause of chronic lung  disease and exocrine pancreatic insufficiency in infancy and childhood.  The aim of this study was to assess the potential association between  bacterial colonization detected by sputum cultures and pulmonary  structural and functional changes in Iranian children with CF. In this  cross-sectional study, 76 CF children ≥6 years old registered in the CF  Foundation of Children’s Medical Center Hospital, Tehran, Iran, who  underwent high resolution CT scan (HRCT), pulmonary function test, and  sputum cultures within a month of each other during the study period  were included. For each patient, demographic characteristics (age and  sex), results of sputum cultures, FEV1, and chest HRCT findings based on  the Bhalla scoring system were recorded in a check list. Sixty seven  percent of the patients had positive sputum cultures. Based on  categorization of Bhalla scores, none of the patients had severe  pulmonary involvement. FEV1 was mainly >70%. There was a  statistically significant correlation between colonization with mucoid  and lower Bhalla scores in children aged 14-16 years. Colonization with  mucoid was also significantly associated with the patient’s age and  FEV-1. Severity of lung involvement in CF children is clearly dependent  on mucoid colonization in airways and this notorious bacterium is the  most prevalent one in Iranian CF children. Prompt identification and  eradication by proper nebulized and systemic antibiotics can have  valueless effects on patients’ quality of life and prevent lifelong  destructive complications such as bronchiectasis. Timely lung CT scan  wisely advised by expert CF treatment team can meticulously detect  injuries and it seems to act more efficacious than -still  helpful-clinical scores and pulmonary function tests.

https://tinyurl.com/ycy5nsay

Longitudinal Microbial And Molecular Dynamics In The Cystic Fibrosis Lung After Elexacaftor–Tezacaftor–Ivacaftor Therapy.

CF is a  genetic disorder causing poor mucociliary clearance in the airways and  subsequent respiratory infection. The recently approved triple therapy  Elexacaftor–Tezacaftor–Ivacaftor (ETI) has significantly improved lung  function and decreased airway infection in persons with CF. This  improvement has been shown to occur rapidly, within the first few weeks  of treatment. The effects of longer term ETI therapy on lung infection  dynamics, however, remain mostly unknown. Here, these researchers  applied 16S rRNA gene amplicon sequencing, untargeted metabolomics, and  neutral models to high-resolution, longitudinally collected sputum  samples from pwCF on ETI therapy (162 samples, 7 patients) and compared  to similarly collected data set from pwCF not taking ETI (630 samples, 9  patients). Because ETI reduces sputum production, samples were  collected in freezers provided in the subject’s homes at least 3 months  after first taking ETI, with those on ETI collecting a sample  approximately weekly. The lung function of those in our longitudinal  cohort significantly improved after ETI, indicating our study cohort was  responsive to ETI. The daily variation of alpha- and beta-diversity of  both the microbiome and metabolome was higher for those on ETI,  reflecting a more dynamic microbial community and chemical environment  during treatment. Four of the seven subjects on ETI were persistently  infected with Pseudomonas or Burkholderia in their sputum throughout the  sampling period while the total bacterial load significantly decreased  with time in only one subject. The microbiome and metabolome dynamics on  ETI were personalized, where some subjects had a progressive change  with time on therapy, whereas others had no association with time on  treatment. To further classify the augmented variance of the CF  microbiome under therapy, researchers fit the microbiome data to a  Hubbell neutral dynamics model in a patient-stratified manner and found  that the subjects on ETI had better fit to a neutral model. This study  shows that the longitudinal microbiology and chemistry in airway  secretions from subjects on ETI has become more dynamic and neutral and  that after the initial improvement in lung function, many are still  persistently infected with CF pathogens.

https://tinyurl.com/256bf9pm

Reprogramming The Cells Secretory Machinery: A Cystic Fibrosis Rescue.

CF is  attributed to mutation in the CF Transmembrane Conductance Regulator  (CFTR) gene that codes for a chloride transporting channel at the cell  plasma membrane. More than 2,000 mutations in the CF gene have been  identified; however, the ∆F508 CFTR is the most common, accounting for  approximately 70% of all CFTR mutations. Earlier studies demonstrate the  CFTR protein to be among the nearly 30 proteins constituting the  porosome secretory machinery at the plasma membrane in human airway  epithelial mucous-secreting cells. Additionally, recent studies show  that stimulated human airway epithelial cells pre-exposed to CFTR  inhibitors result in loss of mucus secretion, suggesting the involvement  of CFTR in porosome-mediated mucus secretion. To further test the  hypothesis that CFTR is involved in porosome-mediated mucus secretion in  the human airways, and to develop a therapeutic approach to overcome  this defect in ∆F508 CFTR human bronchial epithelial cells, the current  study was undertaken. Mass spectrometry and Western Blot analysis of  porosomes isolated from WT-CFTR Human Bronchial Epithelial (HBE) Cells  and ∆F508-CFTR CF HBE cells, demonstrate a varying loss or gain of  several porosome proteins, including a decrease in the t-SNARE protein  SNAP-23 and undetectable levels of the Ras GTPase activating  like-protein IQGAP1 in the ∆F508-CFTR CF cells. This suggested that  mutation in porosome-associated CFTR protein additionally affects other  proteins within the porosome secretory machinery, negatively impacting  mucus secretion. To ameliorate defects in mucus secretion in CF, the  reconstitution of functional porosomes obtained from WT-CFTR HBE cells  into the plasma membrane of ∆F508-CFTR mutant cells was performed.  Results from the study demonstrate that porosome reconstitution rescues  mucus secretion approximately four-fold more effectively than the  currently available CF drugs Tezacaftor and Ivacaftor.

https://tinyurl.com/2ht5cdaj

A Cross-Sectional Study Of Pediatric Feeding Disorder In Children With Cystic Fibrosis.

The  exact prevalence of feeding problems in children with CF is unknown.  Pediatric feeding disorder (PFD) encompasses poor oral intake with  associated medical, nutrition, psychosocial, or feeding skill  dysfunction. In this study, researchers hypothesized that PFD is common  in CF and aimed to categorize feeding dysfunction across various domains  in children with CF. An observational cross-sectional study was  conducted in children with CF. Data collected included anthropometrics,  nutrition data (including need for tube feeding/enteral nutrition [EN]  or high-energy beverages, dietary diversity), feeding skills (Pediatric  version of the Eating Assessment tool [pEAT]), and psychosocial function  (About Your Child’s Eating questionnaire [AYCE] in children 2–17 years  of age/Behavioral Pediatric Feeding Assessment Scale [BPFAS] in children  12–23 months of age). PFD was defined as poor oral intake with: (a)  pEAT score > 5; and/or (b) AYCE or BPFAS score > 2 standard  deviation of normative controls; and/or (c) nutrition dysfunction (body  mass index/weight-for-length z score < −1 and/or preference of oral  high energy beverages or dependence on EN and/or decreased dietary  diversity). Results showed that of 103 children in the study, 62 had  PFD, 7 children were malnourished, 10 needed EN, and 30 needed oral  high-energy beverages. Dietary diversity was decreased in 42 children, 1  child had feeding skill dysfunction, and 11 met criteria for  psychosocial dysfunction. In conclusion almost 2/3rd of children with CF  have PFD and many have poor dietary diversity. A significant percentage  of children rely on EN and oral supplements, but psychosocial  dysfunction is less prevalent.

https://tinyurl.com/53jjry2n

Treatment With Macrolide Antibiotics (Including Azithromycin) For People With Cystic Fibrosis.

People with CF are more prone to chest infections caused by bacteria that can be hard to treat.

Macrolide  antibiotics (such as azithromycin and clarithromycin) may lessen the  effects of bacteria. One macrolide antibiotic, azithromycin, can improve  lung function at six months compared to a placebo (treatment with no  active ingredient) and may reduce the risk of getting a flare-up of lung  infection. It is not sure whether a higher dose of azithromycin is any  better than a lower dose, or if azithromycin when inhaled is any better  than azithromycin when swallowed (oral). Oral azithromycin once a week  compared to every day probably leads to less of an improvement in lung  function, but there is probably a longer time to a flare-up in the  weekly group. Current evidence does not support longer-term use of  azithromycin for all people with CF.

https://tinyurl.com/3j9tp5yf

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.