Pain  is a part of life. Constant pain is a “pain!” It feels as if I have had  pain all of my life. My joints, especially my hips, hands, feet, neck,  and back give me trouble. When I was young, it usually wasn’t too bad,  but, as I age, it can be quite unpleasant. Sometimes it is almost  unbearable. Since I don’t use most pain medications, I have had to find  other ways to live with it.

My  favorite way to deal with pain is to find something that can take my  mind off it. I need to be distracted by doing something rather than  concentrating on the pain. Sometimes I read something that is very  interesting. That’s a good way to be lost. Another distraction is to  sew. I love sewing, so being at my sewing machine can help me pass  several hours. If what I am making takes a degree of concentration, I am  able to forget about my pain, at least for a while.

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I used  to do a lot of photography. I enjoyed doing portraits of people and  getting good shots of scenery. I could lose hours working on my  negatives and enlargements. If I was really into what I was working on,  six or eight hours could just fly by without my being aware of any pain.

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I  mentioned that I don’t use pain medication. The reasons for that include  having had bleeding ulcers, which make it problematic to take meds such  as aspirin. I can’t use ibuprofen because it causes me trouble with my  platelets. Since I have a factor 11 deficiency, it doesn’t make sense to  possibly compromise my platelet activity. I don’t use any opioids  because I don’t want anything to compromise my breathing and most of  them can do that. I avoid acetaminophen due to the potential liver  damage from it.

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I can  use one topical pain reliever gel—diclofenac. The brand name is  Voltaren. I use it sparingly, but it does give me some relief from my  worst back pain, and it can offer some relief for my hands and feet.

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I also  use heat to ease the pain in my back. I find that if I have a heating  pad on the low setting, I can sit with my back against it and get some  relief, at least for a while. I usually fall asleep when I do this, so I  believe that it must help the pain.

I  recently had bone density scans, spine and neck X-rays, and foot X-rays  done. I wasn’t surprised to learn that my bone density has decreased. My  spine is more twisted and curved than before. The discs in my neck have  degenerated even more than before and I have both osteoporosis and  osteopenia at various spots in my back. My spine still resembles a  pretzel. There is osteoporosis in the neck of the left femur, which  helps to explain some of the trouble I have had with that hip.

I had  the right foot X-rayed because there had been more pain in that foot  recently and I noticed the big toe was harder to flex. I know there is  arthritis in both feet, but the right one was giving me more trouble  than the left. Both of them feel as though I am walking on marbles,  which isn’t pleasant. The film showed that there is a prominent bunion  along with the arthritis. That explains the increased pain.

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There is  one thing that can give me some relief from that discomfort and it is a  warm paraffin bath. My husband got me a special paraffin bath unit. I  can warm it to the appropriate temperature and dip my hand or foot into  it repeatedly, until I get a good coat of the warm paraffin. I then put  the hand or foot into a special plastic bag and wrap it with a towel to  keep it warm. I am able to have the warmth on it for 15 or 20 minutes,  which is quite soothing.

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The pain  in my hands keeps me from doing some of my favorite things, such as  making bread.  No longer am I able to mix the dough by hand and I can’t  knead the bread by hand, either. So, since I still want homemade bread,  we got a bread machine. I can still use recipes that I like and let the  machine do all of the hard work. We love our homemade bread.

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I used  to experience severe gut pain. It could wake me from a sound sleep. That  pain and gastroesophageal reflux disease (GERD) caused me to lose a lot  of sleep. I couldn’t find any relief from it. That is, until I had half  of my colon removed and just like that the pain was gone. I did have to  have one more abdominal surgery after that, but it was unrelated to  either the pain or the partial colectomy. After the surgeries, I started  taking a generic form of Nexium and all of my GERD symptoms ceased.  Wahoo!

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I spent  the first 50+ years of my life suffering with severe sinus disease. That  pain was horrendous and it really had a negative impact on my life. I  had a succession of sinus surgeries, which made it better, but it wasn’t  until after endoscopic sinus surgery that the pain was gone. The relief  from pain was so marked that I didn’t even need any post-op pain  relievers. That earns another Wahoo! It has been 27 years since that  surgery, and I have had no recurrence of pain in my sinuses.

So, as I  creak on into old age, I keep hoping that there will be some kind of  relief for the aches and pains of arthritis and osteoporosis/osteopenia.  I am not holding my breath waiting for relief. Instead, I am dealing  with it and finding ways to live my life to the best of my ability.

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Please stay healthy and happy.

Kathy

Kathy  Russell is 78 and has CF. She is a former director and officer of  USACFA. She and her husband, Paul, have been living in the same house in  Gresham, Oregon, for more than 45 years. In March 2023, they will  celebrate 58 years of marriage.

I  have been through major transition in the last months. I learned that  my eye cancer has spread. I have ended my job of nearly nine years and  applied for disability. I have gone from a high-paced, stressed-out,  packed calendar to open and spacious days wondering what I was going to  do. I have gone from months without going to clinic to now going every  week for chemotherapy.

We all  go through transitions. Many of them are positive ones: graduating from  college or graduate school, marriage, becoming parents, getting a new  job, or transplant. Transitions are part of life: They mark a new stage  in our lives—a promotion to the next chapter. We have social rituals to  mark certain transitions: the cap and gown, the bridal shower or wedding  ceremony, the baby shower, or the retirement party. These are times to  gather with friends and family to acknowledge the changes in our lives.

Reaching  the end of life marks another transition to the unknown. We can believe  in an afterlife, but, in reality, we’ll only find out if one exists  when we die. The most common social ritual when someone dies is the  funeral. We gather to celebrate the life of the person who has  “transitioned” or mourn together with their community the transition to  their physical absence.

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We have  fewer rituals for undesirable non-death transitions. There are no  rituals for transitions like divorce, getting diagnosed with a serious  illness, or for going on disability. If we are lucky, a few friends may  take us out to lunch or do something to acknowledge the challenge.  Nowadays, it seems like setting up a Gofundme or CaringBridge.org site  is the newest ritual for a personal difficulty.

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We all  learn how to cope with life’s transitions in our own way. What helps me  spiritually and emotionally cope with transition is to see these  transitions as seasons in life. Many people are familiar with  Ecclesiastes 3:18 (or from The Byrds’ “Turn, Turn, Turn” song), which  says:

“There  is a time for everything, and a season for every activity under the  heavens: a time to be born and a time to die, a time to plant and a time  to uproot, a time to kill and a time to heal, a time to tear down and a  time to build, a time to weep and a time to laugh, a time to mourn and a  time to dance, a time to scatter stones and a time to gather them, a  time to embrace and a time to refrain from embracing, a time to search  and a time to give up, a time to keep and a time to throw away, a time  to tear and a time to mend, a time to be silent and a time to speak, a  time to love and a time to hate, a time for war and a time for peace.”

Regardless  of whether you follow the Bible, this age-old passage captures a  universal truth for all of us. We all have seasons in our lives. After  my transplant, I used to tell people, “I’m in the summertime of my  life!” I have been blessed for 18 years to live in my favorite season of  summertime. Though I had health issues arise here and there, for the  most part, I was strong and healthy and capable of working full time. I  was a bagpiper! I was a backpacker! I was a competitive swimmer! I  enjoyed every bit of it. I reveled in the miracle of easy breathing. And  then I remember the dark winter of my life with advanced CF, when the  future was bleak and breathing—and everything, really—was an effort.  Strangely, memories of hardship can fade and I hardly remember the  struggles I had. I had my turns for winter and summer and now this  season.

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I spent  most of my childhood dreading the fall. The autumn was my season of  sickness. I spent almost every Halloween of my adolescence in the  hospital. Most of my major health transitions happened in the fall.  Halloween or pretty sunsets or cooler weather did little to change my  opinion about the fall. No matter how much I dreaded the autumn, it was  guaranteed to return, year after year. I had no control over the  seasons. They were nature’s forward motion, the cycles of time as we  travel around the sun.

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And how I  loved springtime. Every time I traveled somewhere beautiful, I’d tell  my husband, “This place would be so amazing in the springtime!” I love  the greenery, the wildflowers, the moisture, the promise of rebirth and  the potential and possibility of life. Metaphorically, my life’s  springtime was between the age of six and ten, the longest stretch of my  pre-transplant life where I did not go into the hospital. For four long  years, my parents and I had hopes of a normal childhood, a healthy life  despite cystic fibrosis. Perhaps we (my sister and I) had won the  genetic lottery and our disease would be mild. We travelled to Japan and  Germany during those years, went camping in the mountains, and traveled  by boat with my uncle from Seattle to Canada. We soaked up the  normalcy. Unfortunately, starting in the autumn of my tenth year, the  chronic infections and hospitalizations started and would not stop.

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Perhaps  some of you are in your springtime of life. You may be starting college  or graduating with the world full of opportunities ahead, coupled with  your physical wellness. You are blooming and hope abounds! And then  there is love… when we find the right person to share our life with, and  our will to live gets rebooted and the future seems expansive. This  springtime of life is truly beautiful.

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There is  temptation to worry about what the next season will bring. That anxiety  is natural but can also diminish the present season’s joys. It takes  mental discipline to set aside worries of the future and be able to  absorb what is happening now. The sun is shining, the grass is green,  and let’s bask in the goodness of now.

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Now I’m  in a season of serious illness again. Just like nature’s seasons, I have  little power to prevent seasons from unfolding into the next. It just  is, and that surrender and acceptance saves me so much energy. I could  protest and wish it wasn’t so, but I have to live in reality. Having had  a little time to prepare for or expect this season has helped make the  blow less intense. I do what I can to seek out treatments, stay  compliant with my meds, do research for my situation, and collaborate  with doctors. I eat/sleep/rest/exercise well and the rest is not in my  control. I can appreciate the good that is in each season, even this  one. I feel great relief that I don’t have to push myself at work  anymore; I don’t have to juggle the demands of a job with the demands of  healthcare. I was at my breaking point. Thank God that season is over.

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I have  witnessed so many of my post-transplant CF friends enter a difficult  season of illness, including my twin’s cancer 13 years after her  transplants. They have shown me the way—to face the challenge ahead with  hope, realism, grace, dignity, courage, determination, humor, and  feist. Some of my friends have died and some are still living. I accept  this season of cancer as inevitable after so many years of  immunosuppression and my summertime of wellness. I will keep living as  best as I can, for as long as I can, and I will appreciate each moment  of health. Like chapters in my book of life, I don’t know what the next  chapter will be about. Maybe I will die, maybe I will live. The only  thing that is guaranteed is that this season will change someday. The  next chapter will be written.

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I wonder  what season of life you are in. Sometimes the older we get, the harder  the season. But that may not be the case for us with CF. I believe  Trikafta has brought summertime to many friends with CF. But then the  next blockage, lung bleed, or major infection can bring all the hope  crashing down and we enter a colder season once again. We dance between  hope and despair, between wanting the season to never end and hoping the  season would change quickly. Such is the nature of these health  transitions. And those who are unable to use modulators may be  struggling with the wintertime of CF, desperate for new options. We all  ride the waves of hope for something better around the corner.

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Ralph  Waldo Emerson said, “Each moment of the year has its own beauty.” So I  proclaim to myself that this is also the season of mental and spiritual  wellness even if my physical body is not well. I wonder if you, too, can  find beauty in each season that you are in. Do you have affirmations  that help you get through the tough times?

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In  conclusion, here are some of my positive proclamations to boost my  mental and spiritual health as I endure this season of my life:

I  will not dread the future. I will stay in the present. I will surround  myself with supportive and loving people. I will absorb positive and  enriching books, podcasts, films, and lectures. I will engage in joy  every day. I will spend time in nature to remind me of the greater life  force and the seasons of all living things. I will believe in a greater  Spirit watching over me and showing me the way. I will remind myself of  how blessed I have been to have my turn to experience every season and  to learn and grow from them all.

May we all practice gratitude each day for all the rich and delightful seasons of our lives.

Isa  Stenzel Byrnes is 50 years old and has CF. She lives in Redwood City,  California, with her husband, Andrew. She is 18 years post-lung  transplant.

It  feels good to say that, as of late May, my face is no longer under  construction. If you followed my journey through months of  reconstructive work on my upper jaw, I appreciate it deeply. I also  regret to note that this whole saga stands as but one example of the  consequences of poor transitional care for adolescents with CF aging  into adulthood. So for our current focus topic, I want to explore some  of the other ways that inadequate transitional support for teenage and  young adult patients continues to harm our community.

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Getting  accustomed to losing things is part and parcel of aging with CF. We hear  early and often that, despite amazing advancements in treatment, CF  remains a progressive disease. No matter how much we manage to slow down  that progression in collaboration with our care teams, we still  experience damage to many different structures in our bodies.  Consequently, we often struggle to understand what is normal for our  bodies versus what requires focused intervention. And even for people  without CF, adolescence involves both volatility and change almost  constantly. How do we achieve a meaningful frame of reference for  normalcy in our journeys toward adulthood with CF?

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Losing  my teeth has been one of many things I have come to accept as usual  despite it hardly being normal. My adolescent and early adult years also  found me losing what remained of my exocrine pancreatic function;  sustaining permanent damage to my vaginal and cervical mucosa from  repeated infections; developing severe scarring and interstitial  inflammation in my bladder and kidneys from virulent bacteria; breathing  almost entirely through my mouth due to my sinuses becoming completely  obstructed with swelling and hardened mucus; and struggling through over  six years of antibiotic-resistant MAC pneumonia.

These  changes in physical health predictably exerted mental health tolls as  well. Medical trauma probably merits its own case file in this history.  Years of being gaslit and ignored by different types of clinicians  culminated in me having to dismiss a doctor from my room on an intensive  care ward lest I spent possibly the last day of my life fighting to be  heard by someone who would not listen. In a distinctly cruel twist of  irony, I later experienced suicidal ideation once I began making more  progress with my physical health in collaboration with providers who did  listen to and valued my input.

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The  “biographical disruption” in education and career that scholars in my  field describe as one of the hardest things for chronically ill people  to deal with hovered constantly in my mind. I learned the hard way that  the consequences of trying to avoid such disruptions by powering through  serious illness would prove equally devastating. Years later, I remain  exhausted physically and mentally. Since the start of the COVID-19  pandemic these feelings have only increased, accompanied by a general  sense of being left behind by the world. Constantly being shown how  little our lives matter to others can make caring for ourselves feel  insurmountably difficult.

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Indeed,  behavioral health also matters tremendously in transitional care as we  age into adulthood. Obvious areas like infection-control practices and  nutritional support strategies matter immensely. So, too, do less  obvious ones like sleep habits, substance use, intimate relationships,  and boundary setting. I have shared openly in prior columns about my  history of physical, emotional, and sexual abuse. I reflect now on the  irony of easily saying “no” to drugs that could harm my lungs or liver  while fearing the potentially deadly consequences of refusing sex or  anything else to an abusive man. The red flags that my healthcare  providers during those critical transitional years missed were as  numerous in the moment as they are devastating in retrospect.

It took a  hospitalization and me being completely alone in my room with a social  worker for someone finally to ask if I was being abused at home. Every  time I see similar questions in the social history questionnaires for my  CF clinic or other care providers, I feel my heart leap into my throat.  I cannot help but think in those moments about how different my life  might be now had someone asked those questions then. The social worker  who rounded on me in the hospital needed only seconds to understand what  questions they needed to ask. At the time, I could only avert my eyes  and cry silently while they sat with me and held space for my pain. But  that single supportive interaction changed the course of my life in ways  that would take years to appreciate fully.

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Barriers  to effective transitional support abound. We should not need to land in  intensive care to receive basic prevention and management services to  help us transition healthfully and safely into adulthood. Yet in the  fragmented patchwork that passes for a healthcare system in the U.S.,  many of us fall into the cracks between those disjointed pieces. And  some of us never come back out mentally even if we manage to survive  physically.

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Adult CF  care also remains a rare specialty relative to population need. This  disconnect between available providers and patients needing care will  only grow larger as survival increases with CFTR protein modulators and  other innovative therapies. The practice of separating adult and  pediatric patients that some clinics use—including my own—also creates  unfortunate consequences for transitional care. Having to start over  with a new team further undermines the continuity of care we already  struggle to achieve. It can also deepen our difficulty in building  meaningful trust with our providers over time. Those longstanding  trusting relationships prove critical in our adult years because nobody  really knows what healthy aging with CF looks like.

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Our  providers learn that information primarily by dialoguing with us as we  grow older. Although we often lack a defined sense of normalcy, we can  often narrate changes in our health in great detail, including observing  patterns that might easily escape notice by clinicians. So, even as it  poses substantial challenges, this time of increased longevity for many  adults with CF also brings unprecedented opportunities in transitional  care.

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As the  primary stewards of our own daily care, we are educating our providers  in uniquely valuable ways and actively charting what our care will look  like in the future. And as more of us enter health-related professions  ourselves, this process of collaborative education becomes easier and  more dynamic.

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Technology  also provides a vital safety net as we continue to navigate transitions  in our individual care amid uncertain global circumstances. Staying  connected with our different providers has never been easier or safer  thanks to widespread innovation in HIPAA-compliant communication  options. During the COVID-19 pandemic, many clinics have also switched  to more person-centered medical records platforms that give patients  greater control of both our own information and our correspondence with  care team members.

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My own  clinic changing to the Epic EMR system has proven transformational for  me and my providers. Having a medical record that highlights and affirms  both the complexity of my medical history and the intricacy of my  personal biography lets me spend more time on the content of my care and  less time trying to establish an appropriate context for it. These  changes have made my healthcare more accessible both physically and  socially. They have also made balancing care and career substantially  easier. I cherish any time I can spend sharing “pearls of wisdom” with  my clinical professions students rather than repeating myself in  educating my own providers.

Dr. Alexandra “Xan” Nowakowski is 38 years old and has CF. Xan is a director of CF Roundtable, in addition to being a medical sociologist and public health program  evaluator. They currently serve as an Associate Professor in the  Geriatrics and Behavioral Sciences and Social Medicine departments at  Florida State University College of Medicine. They also founded the  Write Where It Hurts project (www.writewhereithurts.net)  on scholarship engaging lessons from lived experience of illness and  trauma with their spouse, Dr. J Sumerau. You can find their contact  information on page 2.

Hi  everyone, my name is Maggie. I’m 34 years old with cystic fibrosis and  had a double lung transplant in November 2014 at Stanford in California.  Not even a year later, my British husband and I found ourselves  crossing oceans and moving to England to start the next chapter of our  lives. I have always enjoyed cooking, but my passion skyrocketed  post-transplant when everything started to taste better and I was  voraciously hungry all of the time. I started cooking up a storm for  anyone who would eat my food. I then went on to volunteer for a dementia  day center cooking their lunches once a week. I enjoyed the fact I  could make people happy with feeding them and began to realize this was  what I wanted to do. After a few odd jobs in cafes and looking after  people in their homes, I fell into being a private chef. I started  cooking out of my own home and making meals for individuals and families  based on their specific needs. I work very part time, but enjoy feeding  people when I can. My motto for my style of cooking is “I want the food  to feel like a hug.” I hope you make and enjoy the recipes I will be  writing about here in this new column.

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Cream of  broccoli soup and I go way back. I wasn’t a big eater as a kid and my  parents would do anything to get me to eat just a few mouthfuls of any  type of food. My mom knew she could get me to eat one thing  consistently, and that was Cream of Broccoli Soup at our local café,  Town Kitchen. It always had a sense of warmth to it, not just because it  was hot soup, but the cream and the hint of spiciness from the pepper  and the chunks of broccoli made me feel full and satisfied. Of course, I  make my own version now and every time I take that first bite, it  brings me right back to the plush, vinyl covered booths, with brown  panelling on the walls and an unlimited supply of saltine crackers in a  bowl on the table. It is that hug that I look for whenever I am eating.

Cream of Broccoli Soup

Yield: 4–6 servings

Prep time: 15 minutes

Total time: 1 hour

Ingredients:

• 2 pounds of broccoli; cut both florets and stems into small, bite-size pieces

• 1 medium-to-large onion, finely chopped

• 6 tablespoons unsalted butter

• ¼ cup all-purpose flour

• 3 cups of vegetable or chicken stock

• 2 cups of heavy whipping cream

• Salt and pepper to taste

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Preparation

Step 1:

Melt 3  tablespoons of butter in a large, heavy-bottomed pot over medium heat.  Add onion and a pinch of salt. Cook, stirring occasionally, until onion  is softened and translucent, about 5–8 minutes.

Step 2:

Add  broccoli stems and florets and season with salt and pepper. Cook,  stirring occasionally, until broccoli is bright green and slightly  tender, about 8–10 minutes. Using a large spoon, transfer broccoli  mixture to a medium bowl and set aside.

Step 3:

Heat  remaining 3 tablespoons of butter in the same pot over medium heat. Add  flour and whisk consistently, cooking until mixture has turned a pale  golden brown, about 2–3 minutes.

Step 4:

Gradually  whisk in the stock until no lumps remain (it will thicken considerably  at first), followed by heavy cream, and bring to a simmer. Add the  broccoli mixture back to the pot.

Step 5:

Reduce  heat to low and simmer until liquid has thickened and reduced by about a  quarter and the broccoli is completely tender. Season soup with salt  and pepper and divide among bowls. Top with some grated cheese or your  favorite crackers. You can serve with a salad, sandwich, grilled cheese,  or just a piece of bread for dipping. s

Maggie  Williamson is 34 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.

What are your thoughts post-transplants (liver and kidney)?

Getting  two organs at once is pretty rare and doing so brings with it many  complications, like blood loss, rejection, and possibly death. I am  amazed and humbled at what it takes to go through this experience.

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I just  celebrated my one-year anniversary of receiving the two organ  transplants (July 22, 2021). It has been a long, slow process to heal  and recover. But I am thankful and grateful for more life. There are a  lot of realizations after receiving two more transplants. I thought  after getting a bilateral lung transplant, I am done and I can get on  with my life. So, it was jarring to have to be listed as needing two  more organs. I can’t even imagine what it must be like to receive a  second lung transplant. This experience has made me realize that life is  short and precious.

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What is exercise like for you now?

Getting  stronger is a lot more difficult than with my lung transplant. I am  older, and, at 66, I can no longer do things I did post-lung transplant,  like running a marathon or biking centuries. Life is still good,  though. I still volunteer some at Boomer Esiason Foundation and coach  track and pole vaulting at Iona Prep. To remain strong and fit I now  walk and hike. I don’t jog or run any longer, but I decided to start  back to biking outside. Instead of road bikes and 100-mile bike rides, I  own a bike that is more upright and I don’t go so far. I went from  biking on high-end road bikes to a lower-end hybrid bike. Luckily, the  bike store guy sold my road bikes for me and suggested I buy a more  comfortable hybrid. I keep having to reinvent myself to keep moving  forward.

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My  transplant pulmonologist, Dr. Arcasoy, and I have different ways to  measure my success with the new transplants. We have this back and forth  on most of my visits because I am frustrated with my level of fitness  and he is elated I am still alive. He laughs when I lament my strength  level. In his opinion, I am doing better than 99% of all transplant  patients he knows, and he feels I am fortunate I got the transplants  when I did. That sets me off and I say, “Well if they weren’t going to  do it here (at Columbia), I would have gone elsewhere!” He smiles and  says, “Nobody would have taken you. I had to argue with liver and kidney  teams for you to get it.” He says he doesn’t want to hear how strong or  weak I was. In the end, though, he was my fiercest ally in pushing for  me to get these two transplants. Speaking honestly with me he said, “No  one expected you to get through this surgery.” Obviously I was sicker  than I realized.

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What are you up to aside from exercise?

With  three transplanted organs, my biggest job is staying on top of my  health: being aware of rejection if it starts; constantly going to  doctor appointments; and getting lab work weekly to make sure all is  right. Initially, right after the liver and kidney transplants, I had to  be seen once a week with each of those two transplant teams on  different days. Now I see each of them once a month and, as I am farther  out from the surgeries, it will probably become every three months or  so. I continue to see the lung transplant folks every two months, but,  if there are issues, I have to see doctors more frequently.

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It is a  balancing act, as you well know. For anyone with CF or specifically  transplant, a small infection can bloom into a major problem quickly due  to being immunosuppressed and not having the ability to fight things  off on my own.

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Do you get biopsies to check for rejection for these two new organs like you did for your lung transplant?

They  have not done biopsies yet. Blood work apparently is the be-all and  end-all. So they can see a lot of information about adjusting my  immunosuppressants through blood work. I do deal with high potassium  levels in my blood, which can be dangerous for my new kidney. So, to  keep it in check, I am prescribed a powder that I ingest to reduce  potassium in my system. I am no longer allowed to eat bananas and  tomatoes, which are high in potassium.

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Maintaining  my health also means seeing my skin doctor every few months (to remove  skin cancers, which have become prevalent post-transplant); checking in  with my sinus doctor every few months; and seeing the GI doctor for an  endoscopy and colonoscopy. It’s a juggling act to fit it all in. The  medical stuff is not my life but becomes a major part of it. I enjoy my  volunteer work, but, at times, it is hard to do it all. I have started  napping in the afternoons after the liver/kidney transplants because I  am more fatigued. Sometimes it is overwhelming, but I do try to do what I  can and remain positive.

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How do you view transitioning from pediatric to adult care and then to transplant?

In  regards to transitioning, I look at it as a promotion because most  people with CF do go on to adult care. I compare it to sports. If you  are a freshman or sophomore playing a sport, your goal is to be in  varsity or the Olympic team. Similar to when you are in pediatric care,  going to adult care is the ideal goal. If you do well, you move onward.  It’s part of maturing. In high school I was going to the CF doctor on my  own, but, if there were issues with my lungs, they contacted my  parents. I feel like most people with CF grow up sooner than their  healthy peers because we are forced to deal with life-and-death issues  that must be dealt with—it is unavoidable. It forced me to grow up  faster and be more responsible for my own care.

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My  experience with transitioning was difficult. I was in the dark ages of  CF treatment, and I saw only a pediatrician into my 30s. It seemed like  all of a sudden it became mandatory to see an adult CF pulmonologist.  But there really were no adult pulmonologists who specialized in CF care  like I was used to at my clinic. Once my pediatric pulmonary physician,  Dr. Gorvoy, retired, I was forced to see a few other adult  pulmonologists. At that time I was 30-something. That didn’t work out  well. One was mostly asking me about CF and for me to explain to him  what this or that meant as if he didn’t know anything about CF or how to  treat it. I then saw a few others and none worked out, so I continued  seeing Dr. Gorvoy until he finally retired. He insisted I see an adult  pulmonologist and finally sent me to another one at Columbia. He was  also a transplant pulmonologist as well as the director of the  transplant program at that time. Because I was told I had to be seen at a  CF center, I started seeing an adult CF pulmonologist at Columbia named  Dr. DiMango, whom I liked a lot. She knew CF well and I felt I was  getting good care and even taking part in clinical trials for drug  studies. I finally ended up with proper care by an adult pulmonologist,  but it was a lot of stops and starts to get there.

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What were your thoughts about lung transplant?

Eventually,  with my declining PFT scores, it was recommended to me by the CF center  that I get listed for transplant. That, too, took a circuitous route,  but I ended up back at Columbia after being seen at UNC Chapel Hill.

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In the  end, I didn’t list with UNC because I would have had to move there with a  family member to wait for a transplant. I am very independent and I  said I would not be comfortable doing that. I don’t like to have to rely  on others, even if it is my own family. Thankfully, around the same  time, one of the nurses at the transplant center at Columbia called me  and said they have a transplant team with a new surgeon. She told me  about Dr. Sonett and the transplant pulmonologist, Dr. Arcasoy. I met  them both and liked their approach. I then decided to list solely at  Columbia Presbyterian in New York City.

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Dr.  Sonett is still the surgeon I see if I need any surgical corrections,  but, thankfully, I have not. I am happy I was able to remain close to  home in New York and not have to move to get my lung transplant.

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Are you taking Trikafta again?

Prior to  receiving my new liver and kidney transplants, I could not be on the  prescribed dosing because it was damaging my liver. I was only on it  twice a week so it was not ideal, I was only getting partial results. I  am now on a full dose because my liver is fully functioning. Now that I  am on a full dose of Trikafta, I see a huge difference in my sinuses.  The incidence of infection of my lungs due to my sinuses so far has  reduced to zero. Even my ENT is amazed at how great my sinuses look.

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What side effects, if any, do you have from Trikafta?

So far, I  have had no side effects from Trikafta. I don’t know that I am really  human or alive! Sometimes I think I am a robot. I have had so many  COVID-19 vaccine injections and Evusheld shots and have had zero  reactions. There are a lot of medications and vaccinations coursing  through me and yet I don’t have any side effects. But I actually did get  COVID-19 in May.

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What was your experience having COVID-19 as a transplant patient?

One  Saturday in May I started feeling run-down and thought I was overdoing  it with volunteering. I felt achy and had a sore throat. By the next day  I felt slightly worse and someone suggested I test for COVID-19. I  tested positive on two home tests. Because I have a great relationship  with my transplant pulmonologist, Dr. Arcasoy, I texted him immediately.  Within a few hours, I had arranged for monoclonal antibodies through  Maggie Carroll, the transplant coordinator. After filling out the  prerequisite paperwork online, a nurse administered the antibodies that  evening via IV push over a five-minute period. The nurse said I would be  fine the next day. I was dubious because I woke up in the early morning  with a fever of 101 degrees. But by late morning the next day I felt  completely fine. I did quarantine at home for ten days and was told I  might remain positive for COVID-19 for up to two months.

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What is your life view?

Living  with transplants is different. Living on disability is  challenging—nothing is easy. And transitioning to retirement is not  going to be possible for me now.

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Currently  I still have physical struggles as I don’t feel as strong or as in  shape as I would like. There are struggles financially because the Long  Term Disability I was blessed with will be ending December 31, 2022. I  will be starting on Social Security and I can’t live solely on that.  Since I grew up so long ago when CF was only a childhood disease, I was  never taught to save for a “rainy day.” Most people with CF that I grew  up with are now no longer alive. I was never taught to save for my  future as I was never assured I would have one. It is scary to think I  might have to get a job at this age. But I am ready for whatever I have  to do and to keep moving my life forward.

Andrea  and Jerry will continue their conversations about life after  transplant. If you have questions for Jerry Cahill, email us at: cfroundtable@usacfa.org.

The  time has come to transition to adult care—new doctor, new location, new  CF team. Is this scary, exciting, overwhelming, nerve-racking, freeing,  or all of the above? For me, it’s all of the above.

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You  essentially have the same CF care team your entire pediatric life. These  people have known you since you were a baby, and now they are sending  you off to a whole new team with whom you will start from scratch. It’s  bittersweet but also very humbling because there was a point in my  childhood where my CF doctor didn’t even think I would live to see my  teens, much less adulthood, and yet here I am transitioning to adult  care. For that, I give myself a huge pat on the back. I would tell my  younger self, the one who was sitting in that hospital room with IVs  hooked up to her, oxygen in her nose, a feeding tube supplying her  nutrients, and staring at the ceiling during that lonely night by  herself: “Don’t give up. You may not see the light now, but trust me  it’s there and you will see it very soon.”

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My  transition to adult care started when I was still in pediatric care. My  mother passed away when I was 17 years old, and she was my biggest voice  when it came to advocating for my care. She was a pharmacist, so she  knew the meds and what they could do to my body as well as the mental  stress it could take on me. I didn’t transition to adult care until six  years after my mother’s death, but I should have done so much sooner. I  blamed my doctor for the lack of time I had with my mother in her last  days on this earth. My mother had cancer—she was sick and in the  hospital. I had a doctor appointment that same week, and my lung  function test didn’t go very well, so my doctor decided to throw me in  the hospital for a tune-up, even though I explained that my mother would  die soon. He didn’t seem to care. While I was in the hospital, my  father showed up to tell me that my mother’s oncologist said she had  less than 24 hours to live. I did not see my mother that last week she  was alive. Once she passed away, my father and I took on the mantle of  advocacy. I began speaking up on my behalf and my father would always  back me up. He looked at the facts and numbers, but he also knew how his  daughter felt. Pediatric doctors want patients to have the highest lung  function possible despite other factors or side effects. I fell into a  state of depression and lost 75% of my hearing from medicine being  pumped in me as my pediatric doctors obsessed only about optimal lung  function.

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At some  point, I got fed up and hated the fact that I was never listened to—I  wanted to make my own choices regarding my CF care. So I disappeared. I  stopped going to doctor appointments and stopped taking doctor calls or  emails until I was in the hospital every other month and was forced to  see them. They would only see me when I was sick and this was unhealthy.  So, at the age of 23, I had a conversation to move to adult care.  Pediatric care wasn’t working for me. I had a toxic relationship with my  doctor and I couldn’t forgive him. I was ready for a fresh start and a  healthier/more positive experience with my doctors.

During  one hospital visit, I was able to meet the entire adult CF care team. I  asked if they could stop by so I could formally meet them and ask them  questions. I was here in the room, anyway, and couldn’t go anywhere, so  they might as well come by while making their rounds. The main physician  came in, followed by the pulmonologist, and the nurse practitioner. I  liked that they asked, “Who is Mariah?” and I answered: “Mariah is a  sassy, God-loving girl who was diagnosed with cystic fibrosis at four  months old. She graduated with her master’s degree in accounting from  University of Michigan and currently works as an accountant. She likes  crocheting, kickboxing, writing poetry and traveling in her free time.  She is also 75% deaf so, if she doesn’t hear you the first time, she’s  not ignoring you—she just didn’t hear you. She received a cochlear  implant back in 2020, so she’s taking her hearing back one decibel at a  time. She has one motto she lives by and that is ‘God saved my life, so I  better live it.’ She continues to strive for the best and won’t let  cystic fibrosis get in her way.”

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The  adult doctor also came to one of my clinic visits and introduced herself  to me along with my current team. It was a casual visit, which I  thought was cool because it wasn’t an official “okay, bye, see you  later” moment. I actually got to have an informal meeting with her and  just talk about who I am. I shared my background, how sick I was, and  she shared how adult life was different than pediatric life. Pediatric  care is more of a forced process where you basically do what your doctor  says. You go to the doctor appointments and they remind you all the  time and are constantly on top of you as far as taking care of your  health. Adult care, as I have learned, is the complete opposite. It’s  all on the patient now and there is more autonomy in managing your own  health. There are pros and cons to both. I am stubborn and don’t really  like to accept help even if it’s necessary. I like to be in control and  be my own boss. But the pediatric nature of doctoring made me angry  because I would be admitted to the hospital when I wouldn’t want to go  and forced to go to doctor appointments. Adult care is weird—if you  don’t want to go, they won’t force you, they won’t check up on you, and  they leave it to you. During one visit I straight up said I didn’t want  to do PFTs and, to my utter shock, they said, “Okay we won’t do them.  You’re an adult, and you don’t want to know your lung functions, so we  won’t force you.” And that humbled me a lot. This whole time I was  complaining about my pediatric doctor basically forcing me to do things  but then, when the adult doctor listened to me, I was like, “Wait, no  fight back? No hard time? No explaining to me why it’s important and why  I should do it?” Just an “Okay, we won’t do it.” I ended up doing PFTs  because I was just so taken aback. Call it reverse psychology or call it  the reality of adulting.

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Transitioning  to adult care came with hurdles to overcome as well as a new sense of  freedom. I had a full-time job as an accountant and, while transitioning  to adult care, I was also transitioning to adult life in general. I had  recently graduated with my master’s degree in accounting, so I was  transitioning from being in school to having a professional job, and  having my first car (which I bought for myself), all while managing  doctor appointments, visits, and my own health. My father is still a  huge help and supporter while I’m negotiating adulthood, but my friends,  who would always come to visit, are also all adults and have moved to  different states. When I’m sick or hospitalized, they can’t come to  visit like they used to when we were adolescents in high school.  However, going from pediatric to adult care, I will say that the focus  is not just solely on my lungs but on other health goals as well. They  explain what the numbers on my chart and lab results mean, and I found  out that I had asthma. I didn’t know I had asthma! Certain things that  were ignored in pediatrics weren’t ignored in adult care; in adult care  they want to be as transparent as possible, whereas in pediatric care, I  realized they will hold back certain information so that the  family/parents will agree with certain treatment or go with the  treatment that the doctor wants. Adult doctors want to have a  relationship with you and your input matters. They care about my mental  health, my life goals, my aspirations, and all sorts of concerns  unrelated to CF. They are not just focused on a number on a paper;  rather how the person is feeling is just as important. I’m not saying my  pediatric experience was terrible, but I’m also not saying it was  great. In the end, however, I made it to adulthood because of my care  team, for which I’m forever grateful.

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If there  was one thing, I would want to change about my transition it would be  the scare tactic. I was fully transitioned to the adult care team by age  23, but that was later than I intended because doctors and nurses would  scare me into not going into adult care. They would say they are not as  patient as us, you can’t pull any crap with them, they don’t care  whether you show up or not, and they won’t baby you. I wish I didn’t  have that ingrained in my head before going in because it made me scared  my care would be worse and that these people had cold hearts, which  wasn’t the case at all. Don’t fill the patient’s head with scary stories  or scare tactics just so you can manipulate them.

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New  doctors equate to new perspectives. Change is good; change is scary;  change is inevitable; but it’s how you adapt to change that makes the  whole experience worthwhile. Transitioning can be smooth or bumpy, but  so can cystic fibrosis. It’s truly not about the destination, but about  the journey. Each journey has different paths, detours, hiccups, and end  results, but that’s what makes it special along the way. So here I  am—fully into adult CF care—still learning as I go, but I feel prepared  and ready for the long haul.

Mariah  Caise is 26 years old and has CF. She was born and raised in East  Brunswick, NJ, where she currently resides. In her free time she likes  to sleep! On the rare occasion when she’s not sleeping, working, or  doing treatments, she likes to crochet, write poetry, go bowling, hang  out with her friends, and watch her reality tv shows about housewives.

As  a kid born in the 80s, and one who is part of the late-diagnosis cohort  of people with CF, the transition from pediatric to adult care was less  than ideal. Back then we didn’t have organized pediatric clinics,  organized adult clinics, a national registry of clinics, nor any real  method to the madness when it came to coordinated care.

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I  started out seeing a pediatrician when I was diagnosed in 1991. This  pediatrician specialized in CF but wasn’t a certified pulmonologist, to  my knowledge. He was also the only option in my town. He was demanding  of his patients and his bedside demeanor wasn’t warm and fuzzy, to say  the least. However, he had a long-standing policy that all CF patients  were seen immediately, regardless of his schedule. I was always able to  get in to see him within the hour. It wasn’t until the late 90s or early  00s that they divided the waiting room lobby into “well patient” and  “sick patient” sides, with CF patients always sitting on the  well-patient side to avoid further exposure from other sick kids. Beyond  that, we didn’t really have infection protocols in place. We also had  maybe two inhaled drugs for CF back when I was diagnosed—the  proliferation of nebulized medication didn’t come until I was graduating  high school and starting college. Nebulizers could be rented from local  pharmacies for use at camp, medications were pulled from the big amber  bottles with syringes and put in the nebulizer cup, and we rinsed cups  with water. Long before Xopenex (and other medications) came in plastic  vials, it came in glass vials that had demarcations on the end to easily  snap off the ends to pour into the nebulizer cups. However, sharps  containers weren’t part of that process. Things were so different in the  early days of CF!

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Shortly  after I graduated college in 2001, I was admitted to the hospital for  IVs and a pulmonary tune-up for the first time. Because my hospital  didn’t have a children’s wing and the existing children’s hospital  wasn’t even in the cards back then, I was admitted to the main hospital  on the respiratory floor. At the time, and still to this day, that  hospital and floor are the only floors people with CF can be admitted to  as they have the resources and protocols to take care of us better.  That first hospitalization was scary! I sobbed. Now, I’d give anything  for the easy, breezy admits back then. I was given a four-hour pass to  leave the hospital every day! My mom would come visit and we’d sit  downstairs in the gift shop and have coffee; she’d take me to lunch; we  would go get pedicures, etc. She brought me snacks and stuffed animals  and called me every day, multiple times a day. There were no contact  precautions back then. The PICC lines at that time were stitched into  the arm—the stat lock to hold the catheter in place wasn’t a thing in  the beginning.

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Over  time, these things slowly changed. More awareness around infection  control meant keeping the door to your room closed. Masks were worn  outside the room. Eventually, I cultured Pseudomonas and I was put on  contact precautions so everyone had to gown up to enter my room. This  was a hard habit for family members to break—they live with me, they  share the same germs, why did they need gowns?! After a few years, the  four-hour passes were revoked. Insurance, and likely doctors, decided  those of us admitted for 14 days who felt well enough to leave weren’t  that sick and didn’t need to be admitted and therefore insurance didn’t  want to pay for the tune-ups in the hospital.

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Over  time, I developed drug resistance and drug allergies so we had to try  newer, stronger drugs that had to infuse over longer periods of time  more frequently so hospital stays weren’t “spa” stays anymore. They were  full days of rigorous nebulizer treatments four times a day, IV  antibiotics every eight hours, plus labs and PFTs and little to no  sleep. The respiratory floor can be loud at night.

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In my  late 20s, we started to get more coordinated care and specialized  clinics. The pediatric clinic was the first to officially exist—after  all, CF was still a pediatric disease then. At clinic we were checked in  to the hospital, essentially, but we saw the pulmonologist, dietician,  respiratory therapist, and eventually a social worker. When the  pediatric clinic was organized, talks of an adult clinic began  circulating. And, with that, I would eventually have to switch clinics  and pulmonologists. I had been with my pediatric doctor this entire  time. He was the one-man-show! He alone visited every single patient he  had who was admitted to the hospital every single day. He missed one  visit one time in all those years. He referred me to some of my absolute  favorite specialists in that time. He saw me in his office every single  day for months before my first admit because I was that sick and he was  that persistent in trying to get me healthy. He was always available to  see me, even last minute, and he took the time to call me back  personally with any results from cultures. It took us a long time to  reach an understanding, but I respected his no-nonsense attitude and he  heard my concerns. He also delivered quite a few devastating one-liner  comments in those years, but, ultimately, I needed the harsh truths.  Mostly, he kept me alive.

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The  adult clinic was also structured, even more than the pediatric  clinic—more specialists at each appointment, more forms to fill out  before appointments, and more processes and protocols to follow, etc.  Some of these changes could have been general changes across all  clinics, not just the adult. Regardless, switching to the adult  pulmonologist also meant I would have a team of doctors who have an  on-call rotation for visiting patients admitted to the hospital so it  wasn’t guaranteed that I’d see my CF doctor during any admits. I was  assured that records were shared and this would be an easy and seamless  transition. I would also lose my pediatric nurse coordinator, who was an  absolute joy and made the frustrations of CF and clinic easier. I  waffled on what was the best approach—wait out the change until my  doctor retired or jump ship and learn to swim.

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On my  30th birthday I switched to the adult clinic. It’s still one of the most  poignant moments of my entire life of CF care. I wrote my doctor a long  note thanking him for helping me make it to 30! At that time, I had  outlived my life expectancy twice.

The  switch to the adult clinic was a huge adjustment for me. The day-to-day  clinic experience was essentially the same. The hospital admits,  however, were another story. Contrary to what I was assured, even though  records were supposedly shared, the on-call doctor rarely wanted to  make “the call” for any change in treatment or protocols or plans as I  wasn’t their patient when outside of the hospital setting. This was  infuriating, especially when you have a complex medical history and  putting all the pieces together like a jigsaw puzzle is a challenge.  During one of my admits, I had an on-call doctor threaten to pull and  discontinue every single medication on my chart if I wouldn’t produce a  urine sample for a pregnancy test, despite my continued arguments that  I’d had an Essure and an ablation and pregnancy was impossible. I’m  still irate over that!

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Now that  we’re a decade plus into this transition, I’m happy with the change. As  the CF population changes and ages with the advent of these new  therapies, new modulators, and new medications, so does the care. The  infection control, the communication between specialists, the online  patient portals, and the push for a primary care physician to handle  regular aging has all been a tremendous step forward. Do I miss seeing  the same doctor each day in the hospital? Yes. Does he visit me just to  say hi when he’s in the hospital for another patient? YES! In the end, I  benefit from the specialty care that comes with a pulmonologist and a  clinic that stays up on all the latest research and trends in the  community.

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We’re on  the precipice of a huge learning curve across the community. CF is no  longer just a pediatric disease. People with CF are living longer,  fuller lives and the adult care will be critical in this next chapter.  And the transition from pediatric to adult, in theory, should be much  smoother for the younger patients as we, the doctors, specialists, and  patients, know more now than we did when I was first diagnosed about  managing CF.

When  I was an infant, my parents visited many different doctors in an effort  to figure out what was going on and why I wasn’t healthy. It wasn’t  until I was 16 months old that my gastroenterologist diagnosed me with  CF. For as long as I can remember, I have regularly seen various  specialists and doctors as a routine part of my CF care.

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At least  one of my parents has always accompanied me to my doctor appointments  and, whenever possible, both will come. They are always very involved  and have lots of good questions for the doctors. I think they will  continue to be involved as I move into adult care.

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In the  summer of 2021, at 16 years old, I was scheduled to have a feeding tube  (a G-tube) placed. However, the surgeon was unable to proceed, because a  scope revealed varices (swollen blood vessels) around the stomach and  esophagus. I would have been at risk of internal bleeding if they had  placed the feeding tube. I was then referred for a liver transplant.

Later  that summer, I went to Texas Children’s Hospital for a transplant  evaluation. Over the course of several days, I met with a team of  doctors and had various tests. They recommended that we proceed with the  transplant. However, I was starting my junior year of high school, with  multiple advanced classes. I asked if we could postpone until the end  of the school year. They agreed. I went on the organ donor list on June  3. Just 12 days later, we got the call that there was a match. I was in  the hospital for two weeks.

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After my  discharge from the hospital, I had to stay in Houston for another month  for close monitoring, while I recovered from the surgery. My parents  and I stayed in an apartment near the hospital district. It was nice and  I had my own room, but it wasn’t what I am used to. There was less  space for medicine. I couldn’t do my breathing treatments in my room.  And I didn’t have a very good bed. However, I was close to my transplant  team, and I think that helped me recover faster than if I had gone  straight back home to Fort Worth. Being back home now is nice because I  have my own room and plenty of space for all my medical equipment. I can  do my treatments in my own room and I get better sleep without all the  interruptions. I used to share a room with my little brother, which made  everything a bit more difficult, but he never got in my way too much. I  definitely think it is better to have my own room now, though.

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The  liver transplant has given me a lot of new medicines to take for about  one year. One medicine has to be taken at both 9:00 a.m. and 9:00 p.m.  every day for the rest of my life. Transplant has also impacted my  diabetes—I now have to take fast-acting insulin before I eat, and I  closely watch my blood sugar with a continuous glucose monitor. This has  been very complicated due to one of the medicines I have been taking. I  wasn’t able to get back into physical activity straight away.

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The  first three months after transplant are probably the most challenging. I  just hit that milestone. Now, I am going to start going back to school  on campus and I can start playing tennis again. This is my senior year.  Fortunately, I have completed most of my high school credits already and  will be taking a light course load and won’t have to be at school until  about 10:00 a.m. This will give me more time for treatments and to  ensure my health continues to improve before starting college.

With my  liver disease, I had to stop playing contact sports. Both my liver and  spleen were enlarged and the doctors were concerned about the risk of  impact. This was very disappointing. I had played soccer since first  grade, until switching to tennis last year. Recently, three different  doctors have noticed that my spleen has significantly reduced in size as  a benefit of the transplant. I don’t think anyone expected this to  happen so quickly or as much. With a new liver and a smaller spleen, I  might get to play soccer again one day.

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I have  been seen by multiple doctors for many years. Throughout all of my  childhood, I have had five ENTs, four gastroenterologists, three  endocrinologists, two pediatricians, and only one pulmonologist. One of  the biggest changes was when we switched hospitals for some new  providers at Children’s Health in Dallas when I was 14 years old. When  my liver disease began to worsen, my pulmonologist (the lead doctor on  my CF clinic team) referred us to a new gastroenterologist in Dallas,  who had more expertise in CF and liver disease. Over time, we changed to  an endocrinologist and an ENT in Dallas as well. All of these doctors  work closely with the CF clinic there. The Dallas doctors helped me by  monitoring my liver disease more closely and getting my diabetes under  control with less medication. We are able to see the gastroenterologist  and endocrinologist on the same day in the same clinic. My  gastroenterologist in Dallas referred me to the transplant team at Texas  Children’s in Houston because of their experience with liver  transplants for CF patients. My pediatric doctors have taken a genuine  interest in my health and we have developed a team that works well  together. This has been reassuring through all of these challenges.

As I  mentioned, I am currently a senior in high school and will be going to  college in a year, so I will be at school while I transition to adult  care. I am considering some colleges that are out of town; however, my  current top choice is a local university, which would allow me to live  at home. I think being at home would be good because I could still have  parents to help me with my medical needs. For a while longer, I will be  able to work with my current team of doctors.

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At 17  years old, I do not have to transition to adult care just yet. But,  before long, I will. So far, my medical needs have always been met and I  anticipate that this will continue to be the case. Because I already  have experienced going to three different hospitals to find the care  that I needed, I understand that a beneficial transition is possible,  even if I have to make changes or go some extra distance.

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I hope  that my future doctors will be able to make my transition into adult  care as smooth as possible, but that will not be easy. I have two rare  mutations (so I’m not eligible for any of the modulators available right  now) and I have complications that come with these mutations. I  regularly see specialists at three different hospitals. My pulmonologist  is the only one who has been part of my CF care team from the  beginning. I will continue to see a lot of doctors frequently into the  future. It is going to be a lot to change all of these doctors. I just  hope it goes smoothly and all the new doctors collaborate, communicate,  and really listen to my concerns and understand my situation.

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When I  transition to adult care, I hope my new doctors will see things  similarly to my current doctors. I hope I don’t have to change doctors  as much or as often as I have at times. I hope they understand  everything that is going on and can give the same help and care I have  received in the past.

Turner  DeMott is 17 years old and has CF. He was born and raised in Fort  Worth, Texas. He is a senior in high school. As a former soccer player  and current tennis player, his primary hobby is playing and watching  sports. His favorite team is Manchester United and he once saw them win  at Wembley Stadium.

Waking  up in a hospital bed to sounds of gurgling from a tracheotomy being  suctioned in the next bed was a bit shocking. But now, as I look back in  disgust at that memory, I am even more horrified that there were no  infection protocols in place during my old hospital stays. I was placed  in rooms that had three other adult patients in them, on an adult ward.  This was in the late 1980s, and it boggles my mind as to what I was  unknowingly exposed to back then.

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This was  all striking because, whenever I was admitted to the hospital in my  late teens to mid-twenties, I was in what was termed the “juvenile wing”  of Mount Sinai. I had my own room and the nurses were well versed in  treating CF. I had a chest physiotherapist come to my room twice a day  after my inhalations. We had art therapy sessions, group therapy chats,  or cooking classes a few times a week. My doctor and CF care team were  pediatric pulmonary folks. I felt they knew all about me and CF and felt  confident in their care from the dietician to the PFT lab tech to my  favorite nurse who wore many hats.

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My first  hospitalization was when I was about 17, before I went to college. My  doctor felt I could benefit from a tune-up. I went in and it was my  first bout of getting IV antibiotics for two weeks. This was when a  person with CF had to remain in the hospital for a full 14 days to do  IVs—home IVs weren’t offered or part of the protocol back then. Of  course, while there, I suffered through boredom, but many people visited  me and I was very close to the age of the nurses so we had a lot of fun  together, too. We played Pictionary with other patients when they had  down time. There were sad times, too, because some patients would die  and all of the nurses were down. They got to know their patients,  especially those with CF—we were the “repeat offenders.”

I was  admitted yearly after that until my senior year and then it began to be  every six months. I was always admitted to the same floor. At some  point, my pediatric pulmonology team told me it was mandated that adults  with CF be seen by an adult pulmonologist. I then met with a very nice  one who was aligned with my CF doctor. She was more of a consulting  doctor—my primary care was handled by my original CF doctor, where I  continued to go to a CF clinic for PFTs, to meet with the dietician, to  see the social worker, etc.

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I didn’t  mind seeing the adult pulmonologist outside of the hospital setting,  but I didn’t know that I was no longer allowed to go to the juvenile  ward when admitted—I had to “graduate” to the adult hospital floor. This  was a big shock. Not only did I have three other women in a room with  me, I had to share a bathroom with them, those that were ambulatory at  any rate. Also upsetting, every time the nurse came into the room to  administer my IVs or inhaled aerosols, the other three patients wanted  this or that so the nurse hated coming in the room. The adult floor had a  shortage of nurses working. Nurses were charged with taking care of  triple the number of patients compared to those on the pediatric floor.  They were overworked, and I almost got the wrong IV medication a few  times, which is terrifying. I was lucky to get my medications on time,  but forget about getting my enzymes before meals, so I brought my own  stash. Also, there was no chest physiotherapy, the food was barely  edible, and there was no art therapy or cooking classes. But I would say  the worst thing was the wild rantings and screaming coming from the  other three beds after 10 p.m. I barely slept.

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So why  was I still seeing an adult pulmonologist if I continued at my pediatric  pulmonary CF center? Good question. The adult pulmonologist had the  adult hospital rights for when I was admitted. My pediatric team,  thankfully, still came to see me but could not write any medical orders  on my chart when I was hospitalized—the adult pulmonologist was the only  one who could. This was so badly managed that I am sometimes surprised I  am still alive. I was exposed to people cleaning the radiators by  expressing their exhaust, which were filthy, next to my bed. Right after  that, I blew my nose and black gunk came out. I don’t even want to  think about what went into my lungs! With those traches being suctioned,  other patients coughing, and, at that time, no thoughts of  cross-infection precautions, I shudder to think of what could have been  with infectious effluvia flying about the room. This nightmare scenario  must have helped keep me out of the hospital and staying well. I somehow  managed to keep hospitalizations to a bare minimum. And then, suddenly,  I heard about people getting home IV antibiotics. I was elated,  although my doctor was less so. I had to sell him on it. He was used to  dealing with pediatric patients, not a 20-something woman who would no  longer do in-hospital IVs if her life depended on it, which soon it  would. I told him I would be open to doing IVs more often without the  hassle of being admitted. This was in the nascent period of the health  industry allowing patients to care for themselves at home. Eventually, I  was persuasive and he agreed to have me start IVs in the hospital for  five days and then finish the rest of the 14-day treatment at home. I  then was allowed to do the whole treatment at home.

While I  still saw the adult pulmonologist, my care eventually went back fully to  my pediatric CF clinic. I was no longer hospitalized until I became  deathly ill. Then I was back in the adult unit. But. because I was so  sick, I was not that upset because I was barely aware.

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After a  major lung bleed that entailed an ER visit, I was told to go back to see  the adult pulmonologist because it was time to contemplate a lung  transplant. She was in charge of Mount Sinai’s lung transplant center.  Through her, I did all of the pretesting that was needed at that time to  get into their transplant program. But because they had performed very  few lung transplants, my family and I decided to look elsewhere that had  more experience and for a center than had a proven success rate for  those with CF. With my testing done at Mount Sinai, my family and I went  to Columbia Presbyterian where they had done many transplants and I  liked the pulmonologist who was to be my doctor there. So, in a sense, I  still saw an adult pulmonary doctor at Columbia and remained under the  care of my pediatric pulmonary team until I received my transplant at  age 35.

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I am  fully aware that adult CF centers are now the norm and I can see the  great benefit that they offer. But when I was transitioning, it was a  haphazard piecemeal approach. There was no such thing as an adult CF  center, only adult pulmonologists. Maybe that is why things run so  smoothly now and kids are transitioned from a pediatric care team to an  adult care team in their early to late teens. It takes a village because  CF is not just a lung disease, but intestinal and endocrine as well. A  team approach is required.

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While my  doctors were all great, no one knew better back then because adult care  was still a new concept and going from pediatric to adult care was all  uncharted territory. So much has improved since my early 20s with  hospital care and obviously new treatments for those with CF. Most  likely many with CF are not hospitalized so much. I guess I was part of  the learning curve. I look back in amazement that I am still alive and,  frankly, I think my CF pediatric pulmonologist does, too.

Andrea  Eisenman is 57 and has CF. She lives in New York, NY, with her husband  Steve and dogs, Willie, Roscoe and new girl, Trixie. Andrea is the  Executive Editor for USACFA. She enjoys cooking new recipes, playing  pickle ball, biking, tennis when possible, and staying active as her  health allows. Her contact information is on page 2.

Arshaad:  a four-year-old boy in Bangladesh whose parents were broken when they  found out their son carried a fatal disease they had never heard of  before. Yasmin: a seven-year-old girl in the Netherlands whose uncle  died at the age of thirteen from the same lung disease she has. Natalia:  a sixteen-year-old girl in the Ukrainian city of Mariupol, which was  bombed and occupied by Russian troops, who fled the blockade of her city  after three weeks of Russian occupation, hungry and cold. All three of  these children have cystic fibrosis and each one lives in a medical  environment where access to a therapy vest, a basic health essential in  the United States, is painfully difficult. These children are trapped in  the geography where the possibility of obtaining a $20,000 piece of  equipment without the assistance of robust health insurance coverage is  grossly unattainable. Rod Spadinger, President and Founder of CF Vests  Worldwide (CFVWW), established this charity in June 2020 with a mission  of providing vests to CF patients around the world. As of October 2022,  CFVWW has donated 155 vests to CF patients in 40 countries, ranging from  Ecuador to Thailand, with dozens of countries in between.

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Rod,  born and raised in Honolulu, Hawaii, has navigated his way through the  many bumps and hurdles CF brings since his diagnosis at three months of  age. At four years old, Rod became very sick—it was 1976 and both CF  knowledge and care in that period were limited. Desperate for an answer,  Rod’s parents contacted the CF Foundation seeking the best cystic  fibrosis doctor in the United States. The Foundation recommended seeing  Dr. Robert Kramer in Texas. Dr. Kramer’s sage advice for Rod to run five  miles a day, each day, is the reason Rod remained alive and relatively  healthy—relatively healthy in the world of cystic fibrosis, that is.

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In  August 2017, a week before his 45th birthday, with 17% lung function,  Rod was graced with a double lung and liver transplant from his angel  donor, Joni Marie. As a way to express his heartfelt appreciation to  her, Rod published his memoir titled A Collection of 50 Stories Inspired  By My Angel Donor, Joni Marie exactly two years after his transplant.  This was a means of giving back to his donor’s family by honoring their  angel daughter. A year later, Rod discovered another method of giving  back.

In  January 2020, Rod received a message on social media from Vale, a young  lady from Chile whom he did not know. Vale asked Rod if he could donate a  vest to her. He thought at first this person was likely a fraud. “Who  is this person, how did she get my contact information, and why in the  world would anyone ever give someone they don’t know a vest worth tens  of thousands of dollars?” were his immediate thoughts.

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When Rod  realized he no longer needed the Hill-Rom enclosed in the dark blue  duffle bag that had been sitting for two and a half years unused under a  table, six feet away from his laptop, on which he received Vale’s  message, he decided to FedEx his vest to her. That turned out to be the  very first vest the soon-to-be-created CFVWW would donate. The second  vest request came two months later from Mohamad, a CF patient and a  Palestinian refugee living in Lebanon. This was another message to Rod  by someone he did not know. Mohamad, like Vale, was a stranger. Upon  Mohamad’s vest request, Rod reached out to the online CF Community  asking if anyone could donate a vest to Mohamad, expecting that no one  would ever respond. “Who just gives vests away to strangers? No one,”  Rod mused. However, within a week, Danie from Ohio heard his call. In a  matter of days, Rod was able to facilitate Danie’s vest to Mohammad in  Lebanon. Danie became the first CFVWW vest donor, with Mohamad being the  second CFVWW vest recipient.

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Two  strangers living 8,500 miles apart contacted, within months of each  other, a CF patient in Texas whom neither knew. These two strangers  unwittingly spurred the creation of CFVWW. Thirty-four months after the  message from Vale to Rod, CFVWW is gaining momentum as it drives to the  next milestone of 200 vests to CF patients around the world. CFVWW will  not stop, as the need is too great.

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CFVWW’s  mission to continue donating life-saving vests to those living in  medical deserts accelerates forward as there are thousands of CF  patients across the globe who are without reliable care and no means for  proper airway clearance. Want to become a member of CFVWW’s worldwide  mission? If you have a vest machine and/or vest garment sitting unused  in a closet or resting under a table, please contact Rod at rod@cfvww.org or visit www.cfvww.org.  Your donation of a vest will make an unimaginable difference in  extending the life of a CF patient unknown to you in a far-off country.  What’s more, your donation will elevate you to hero-status for that CF  patient and their family, as your vest will truly be a gift to them  worth more than gold.

Rod  Spadinger is 50 years old and has CF. He was born and raised in  Honolulu, HI, but now lives in Dallas, TX. He enjoys following the  financial markets, catching football games on the weekends during the  season, and reading when he can find the time.

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

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In our  evaluation, we look for students who demonstrate tremendous academic  achievement, community involvement, and a powerful understanding of how  their CF—matched with these achievements—places them in a unique  situation to gain leadership roles within the community. Our scholarship  is open to all pursuing any degree, from associates to Ph.Ds. We  believe that any higher education is a strong foundation for advocacy  and involvement in the CF community.

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

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We are  pleased to announce Jacob Greene, Samantha Johnson, and Else Mayo as the  recipients of the scholarships for this calendar year. They will each  be awarded $2,500. Congratulations to all three!

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Jacob  Greene is a determined and accomplished individual. He earned his  Bachelor of Science with Honors from Stanford University in 2021. He has  presented at multiple conferences in the last few years, in addition to  his published works. He has been a research intern and assistant at  various university labs and pharmaceutical companies. Jacob started his  first semester at University of California, San Francisco School of  Medicine this fall.

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Samantha  Johnson plans to graduate with a Bachelor of Arts in Global Studies and  Women and Gender Studies with a concentration in Global Health from the  University of South Carolina in May 2023. Samantha has authored  multiple articles in various magazines in the last few years. She has  also served as one of the South Carolina Maternal and Child Health (MCH)  Leadership, Education, and Advancement in Undergraduate Pathways (LEAP)  mentees at her university. The MCH LEAP program bridges trainees’  transition to MCH-related graduate education or public healthcare jobs  upon graduation. Additionally, Samantha has served as both the regional  director and writing intern of the Borgen Project in Seattle,  Washington.

Else  Mayo is earning her Bachelor of Science in Biology at University of  Puget Sound in Alaska. Else is very active in the University of Puget  Sound Symphony Orchestra as well as chamber music ensembles. She is an  active volunteer and participant in various modulator research studies.  Else is passionate about access to care and strives to bring both  awareness and better access to care for everyone with cystic fibrosis.

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

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

USACFA  is proud to announce that the application period for their newest  scholarship, the Scholarship for the Arts, established by Andrea  Eisenman to honor her mother, Helen Eisenman, is now open. Helen valued  education and had a great appreciation for the arts; she found immense  joy in music, opera, photography, and fine arts. She would be delighted  knowing this scholarship would benefit other adults in the CF community  who are interested in pursuing a degree in the arts.

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, and poetry, to name a few. It is open to anyone seeking a  creative arts degree, whether it be an associate’s or a doctoral.

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Helen  was a single mother devoted to her daughter, Andrea, who has cystic  fibrosis. She made many sacrifices in order to help Andrea live a long  and healthy life. Helen also fit in her passion for the arts. She was a  talented photographer, writer, and editor (she used to proofread CF Roundtable, too).

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Helen  was born in 1928, near Vienna, Austria, and was a Holocaust survivor.  She and her parents were admirers of the arts in Vienna and later in New  York City, where they resettled after 1940. Helen was proficient in  several languages—German, French, Portuguese, and English—which would  later serve her well in her career writing subtitles for foreign films.  She majored in English at Queens College and landed her first job  writing for radio. Helen later joined the film industry as an editor  and, eventually, was known as the “Doyenne of Subtitles”—the go-to  person for subtitling foreign feature films.

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In her  50s, Helen combined her love of learning and photography by pursuing a  master’s degree at the New School of Social Research in NYC, where she  studied new media studies. This led to a few photo exhibits in Long  Island, NY. For her thesis, she taught kindergarten children this  medium, creating a photography program and building a darkroom in a  local public school. It was a way for young children to communicate  through images about their lives and the world around them.

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Helen  eventually added Spanish to her language proficiency repertoire in order  to broaden her freelance career as a subtitle translator. Having her  own company allowed her to care for her daughter, Andrea, who received a  double-lung transplant in 2000. Helen never let CF get her down and  never stopped fighting for her daughter. She instilled in Andrea the  appreciation of life and the arts. Andrea went to college and became a  graphic designer with a minor in silkscreen printing. Currently she  volunteers her time to USACFA and CF Roundtable.

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To apply  for this scholarship, please specify what type of creative degree you  are pursuing (e.g., fine arts, graphics, music, singing, photography,  filmmaking, writing, poetry, dance, theatre, and other performance  arts). Candidates should have a minimum GPA of 3.0. Please submit essay  answers, electronic samples of your work for consideration, transcripts,  and a letter from your physician confirming CF diagnosis. The  application deadline is April 30, 2023.

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

CF Foundation Estimates Increase In CF Population

The CF  Foundation announced that the population of people with cystic fibrosis  has increased over the past decade, according to a new estimate. Close  to 40,000 children and adults are living with cystic fibrosis in the  United States and a total estimated 105,000 people have been diagnosed  with CF across 94 countries. The CF population was last estimated in  2012 to be more than 30,000 people in the U.S. and 70,000 globally. in  the past few decades, evolutions in the CF care model have helped change  the face of cystic fibrosis. Advancements in multidisciplinary care,  treatments, nutrition, and universal newborn screening have extended  lives. For a child born between 2017 and 2021, the median predicted age  of survival is 53 years old — up from 38 years a decade prior 34,  resulting in the expanded population. There are now more adults living  with CF than children. People with CF are achieving milestones never  before thought possible — attending college, building careers, getting  married, and starting families of their own. As people with CF get  older, they often experience more complications and a more complex  course of this progressive disease.

https://tinyurl.com/3frc822y

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Lupin Gets Tentative Approval For Cystic Fibrosis Drug

The drug  manufacturer announced that it has received tentative approval from the  US Food & Drug Administration (USFDA) for its Abbreviated New Drug  Application (ANDA) for Ivacaftor tablets, 150 mg. Ivacaftor tablet is a  generic equivalent of Kalydeco tablets manufactured by Vertex  Pharmaceuticals. Ivacaftor tablets are used to treat certain types of  cystic fibrosis in adults and children 4 months of age and older.

https://tinyurl.com/556w2ysm

New Research Could Prevent Hearing Loss For 50% Of People With Cystic Fibrosis

People  with cystic fibrosis are prone to recurring lung infections which need  to be treated with aminoglycoside antibiotics. Aminoglycoside  antibiotics are very effective against life threatening infections and  are associated with low rates of antibiotic resistance, however they  enter and kill the sensory hair cells in the inner ear that are vital  for hearing. Researchers estimate it may be as high as 50% of adults  with hearing loss. Researchers are attempting to develop new  aminoglycosides that are less toxic to hearing. By the end of three  years the researchers hope to have at least three new aminoglycosides  that can be moved towards clinical testing.

https://tinyurl.com/2xvap35k

Exposure To Air Pollution Particles May Add To CF Clinical Variability

Exposure  to carbon nanoparticles — tiny air pollution particles that account for  a substantial part of air pollution in urban areas — significantly  reduces the levels of CFTR, the faulty protein in cystic fibrosis (CF).  Being exposed to these air pollution particles also increased signs of  cellular stress, DNA damage, and cell death. These findings suggest  exposure to air pollution such as carbon nanoparticles may explain the  clinical variability among CF patients, including those carrying the  same disease-causing mutation. Airway cells from CF patients have been  shown to be more susceptible to nanoparticle uptake and accumulation  than those from people without the disease. Previous research showed  that the damaging effects of carbon nanoparticles are linked to  inflammation and oxidative stress, a type of cellular damage implicated  in CF. A team of researchers evaluated the effects of pure carbon  nanoparticles on human bronchial epithelial cells grown at an air-liquid  interface. Bronchial epithelial cells, those lining the main  passageways into the lungs, form the interface between the external and  internal airway environment. Results showed a four-hour exposure to  aerosol-delivered carbon nanoparticles impaired the integrity of the  cells’ natural barrier when compared with before exposure. Carbon  nanoparticle exposure also significantly reduced both the activity of  the CFTR gene and CFTR protein levels by about 40% relative to exposure  to clean air. A significant reduction in the activity levels of two CF  genetic modifiers — TMEM16A by 60% and TNFAIP3 by 70% — also was  observed. Genetic modifiers are genes or genetic variants that can  increase or reduce the severity of a condition without necessarily  causing the disease themselves. TMEM16A encodes a protein shown to be  critical to CFTR’s presence in the cell membrane. Meanwhile, lower  TNFAIP3 activity has been associated with worse lung function in CF  patients. These findings highlight that exposure to aerosol-delivered  carbon nanoparticles reduces barrier integrity and CFTR protein levels,  while promoting oxidative stress, DNA damage, and cell death in  lab-grown human airway cells. Further studies are needed to confirm this  association and understand the mechanisms behind carbon  nanoparticle-induced reduced CFTR levels and increased cellular stress,  damage, and death, the researchers noted.

https://tinyurl.com/2y76vhnm

AND

https://tinyurl.com/mrcspmzk

The Negative Impact Of Chronic Rhinosinusitis On The Health-Related Quality Of Life Among Adult Patients With Cystic Fibrosis

With  improved survival in cystic fibrosis (CF) patients, it is crucial to  evaluate the impact of chronic co-morbidities such as chronic  rhinosinusitis (CRS). The objectives were 1) To determine the prevalence  of CRS with a large series of CF patients 2) To evaluate the impact of  CRS on the Health-Related Quality of Life (HRQoL) of CF patients and 3)  To compare CRS-specific, CF-specific and general HRQoL instruments. CF  patients were recruited and completed the 22-Item Nasal Outcome Test  (SNOT-22), Cystic Fibrosis Questionnaire-Revised for adolescents and  adults over 14 years of age (CFQ-R), Cystic Fibrosis Quality of Life  Evaluative Self-administered Test (CF-QUEST) and the 36-Item Short Form  Survey (SF-36). Out of 195 patients eligible for analysis, the  prevalence of CRS with positive endoscopic findings was 42.6%. CRS  patients reported significantly lower HRQoL with higher SNOT-22 scores  and lower scores in the respiratory domain of CFQ-R and physical health  domains of CF-QUEST and SF-36. The physical and mental domains of SF-36  and CF-QUEST had a strong correlation with SNOT-22. Higher scores of  SNOT-22 nasal subdomains correlated with lower scores of SF-36, CFQ-R  and CF-QUEST. Thus,CRS is a prevalent co-morbidity of CF patients, which  significantly reduces HRQoL. SNOT-22, CFQ-R, CF-QUEST and SF-36 were  strongly correlated. Severity of sinonasal symptoms have a strong  correlation with HRQoL in CF patients.

https://tinyurl.com/2ctbputf

Impact Of Antibiotic Eradication Therapy Of Pseudomonas Aeruginosa On Long Term Lung Function In Cystic Fibrosis

While  antibiotic eradication therapy (AET) of early Pseudomonas aeruginosa  infection is considered standard of care, its long-term effect on the  subsequent course of cystic fibrosis lung disease remains unclear. CF  patients who were P. aeruginosa-free for at least a year and had a  minimum of 10 years of pulmonary function measurements were included.  Subjects were categorized as Never if they never had P. aeruginosa  isolated from a respiratory tract sample. Subjects changed to the  Eradicated group if they had a P. aeruginosa infection, were treated  with AET, and subsequently cleared their infection. Subjects changed to  the Chronic group if AET did not clear their P. aeruginosa infection.  The primary outcome was absolute FEV1 decline over time, with age as the  time variable. The researchers concluded that AET against P. aeruginosa  improves lung function trajectory in CF patients.

https://tinyurl.com/3urwrdaf

Regular Antibiotic Use In CF Linked To Hypersensitivity Reactions

Regular  use of antibiotics to treat persistent infections is linked with  hypersensitivity reactions in individuals with cystic fibrosis.  Specifically, the risk is highly associated with the number of  cumulative exposures to antibiotics and increasing age. And intravenous  administration of antibiotic treatments was tied to a much higher  percentage of hypersensitivity cases. However, most cases involved mild  to moderate skin reactions. Severe reactions, such as anaphylaxis, were  rare. People with CF are prone to infections with Pseudomonas aeruginosa  and often need 10- to 14-day intravenous treatment courses with  antibiotics. Among antibiotics of choice are beta-lactams such as  ceftazidime and piperacillin, aminoglycoside antibiotics including  tobramycin, or polymyxin antibiotics such as colistimethate sodium.  However, CF patients are known to be susceptible to antibiotic  hypersensitivity — though it is unclear how frequently such reactions  occur and what are the factors increasing the risk. A team of scientists  led a retrospective study that addressed this knowledge gap. The rate  of antibiotic hypersensitivity reactions was 31%. A vast majority of  cases (98%) were skin reactions — 30% itching, 23% skin eruptions or  rashes, and 18% hives. Some patients experienced tingling sensations,  and rare cases of nausea, vomiting, diarrhea, shortness of breath,  coughing, dizziness, and low blood pressure also occurred. Three  reactions were potentially life-threatening and classified as  anaphylaxis. In 48% of cases, hypersensitivity reactions occurred  between two and 10 days after the antibiotics were given, but in 28% of  cases, the reactions happened occurred within the first hour of  antibiotic injection. Other reactions occurred after one hour but before  24 hours of the first antibiotic dose. Beta-lactam antibiotics were  responsible for 71% of all reactions and 81% of non-oral courses.  Piperacillin was the most common cause of intravenous reactions,  followed by ceftazidime, colistimethate, and then meropenem. Regarding  oral antibiotic treatments, cotrimoxazole was the most common cause of  hypersensitivity reactions, followed by amoxicillin and ciprofloxacin.  Skin eruptions or rashes, angioedema (swelling under the skin), hives,  itching, and flushing were the most common reactions to oral  antibiotics. The researchers noted that no patients developed  anaphylaxis after oral treatment. Intravenous antibiotics were almost  six times more likely to cause hypersensitivity reactions than oral  antibiotics. The number of antibiotic courses significantly predicted  having these reactions, with patients taking the most courses displaying  the greatest risk.

https://tinyurl.com/f36kjt5u

Antibiotic Switch With CF Pulmonary Exacerbation Not Likely To Help

Pulmonary  exacerbations in CF patients are often caused by bacterial infections.  Usually, after a test of which microbes are responsible, patients are  treated with antibiotics to kill off the pathogen. In individuals who  don’t respond to a first antibiotic, healthcare providers may switch  treatments. Switches included antibiotic additions, subtractions,  substitutions, or regimen changes. Common reasons for such switches  included a change in antibiotic administration for discharge patients,  drug reactions, and targeting additional microbes. In other cases, a  switch was made because the exacerbation failed to respond to initial  treatment, as evidenced by a lack of symptom improvement or by FEV1  values not returning to at least 90% of what they were prior to the  flare. Researchers then assessed whether the switch benefitted lung  function in these non-responders by comparing them with non-responders  who did not change their antibiotic treatment. Most clinical features  did not differ between the switch and no-switch groups. Baseline lung  function, or that prior to a pulmonary exacerbation, was poorer among  those given an antibiotic switch than those who were not. But lung  function was similar among patients in both groups at the time a first  treatment was initiated. Overall, those in the switch group saw  significantly smaller absolute improvements in FEV1 by the end of  treatment, even after switching antibiotics, than those in the no-switch  group. In a final analysis, the researchers determined that an  antibiotic switch did not have a significant positive or negative  influence on lung function at the end of treatment or at a follow-up  about three months later. The proportion of patients whose FEV1 values  returned to at least 90% of baseline values also was not significantly  different between those who switched antibiotics and those who did not.  However, given the lower baseline FEV1 values among patients who  switched antibiotics, findings imply that these individuals may have  more substantial lung damage which may affect their treatment response.  Gains in lung health noted with an initial treatment in some patients  may be slower to arrive for others, which in turn influences the  response of healthcare providers. It’s also possible that in some cases,  the wrong bacteria is being targeted for treatment, or that the cause  of the pulmonary exacerbation was not infectious in origin.

https://tinyurl.com/bd36bpa6

Duration Of Intravenous Antibiotic Treatment For Acute Exacerbations Of Cystic Fibrosis: A Systematic Review

Acute  exacerbations of Cystic Fibrosis (AECF) are associated with significant  morbidity. Recommendations are to treat for 2-3 weeks despite limited  data. Spirometry is a measure of clinical response yet appears to  plateau at 7-10 days. While durations <9 days have been associated  with poorer outcomes, a duration of 10 days may be as effective as 14  days, potentially conferring advantages in terms of cost and adverse  events. A 2019 Cochrane review did not identify any randomised  controlled trials (RCT) comparing durations of treatment. Utilising data  from non-randomised studies (NRS), researchers reported a systematic  review of intravenous antibiotic treatment, exploring changes in FEV1  (Forced Expiratory Volume in 1 second), CRP (C-reactive protein) and  peripheral WBC (white blood cell) count in studies with different  treatment durations. Studies were categorized according to their  duration of treatment, of 10-12 days and 13-15 days. No significant  differences in change in FEV1 were observed based on duration of  treatment. They also determined that treatment setting may affect  outcome. When the entire treatment course was as an inpatient, no  significant difference in change in FEV1 was observed. However, where  some of the total treatment duration was administered as an inpatient,  studies with longer treatment durations had a significantly greater  change in FEV1 compared with shorter durations. This systematic review  provides evidence that shorter durations of treatment may be associated  with similar changes in FEV1, CRP and WBC compared with longer  durations.

https://tinyurl.com/t2d3xe7w s

Laura  Tillman is 74 years old and has CF. She is a former director and  President of USACFA. She and her husband, Lew, live in Northville, MI.