My  first book, Where the Watermelons Grow, was published in 2018. It was,  in many ways, inspired by my life—about a disabled mom, a resentful  daughter, and the ultimate recognition that families that look different  from the mainstream can still be wonderful.

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But it  wasn’t about cystic fibrosis. At that point, as a 30-year-old mother of a  toddler, coming off a long period of frequent hospitalizations and  health stress, I knew I wasn’t ready to look deeply enough at my own  baggage to write about a character with CF.

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For  years after writing Where the Watermelons Grow, I tossed around ideas  for a book about a character with CF. I made notes, brainstormed,  drafted a few chapters here or there. (In a painfully ironic twist,  halfway through this process, a movie and tie-in book, Five Feet Apart,  came out and both were eerily similar to one of my favorite ideas.)  Nothing I started ever quite felt right. In the beginning of 2020, I  decided it was time to write my CF novel, and I spent several days on a  book tour trip studiously outlining the book I finally felt ready to  write. And then, two weeks later, the world closed for the COVID-19  pandemic, and I wasn’t sure I’d ever be able to write again.

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When I  finally did sit down to squeeze out a few words that spring, they  weren’t anything like the outline I’d carefully crafted. They were  stream-of-consciousness poetry, and I followed where they took me.  Eventually those words coalesced into a novel in verse that combined all  of the things closest to my heart: poetry, dolphins, water, friendship,  family…and cystic fibrosis.

That  book ended up being called No Matter the Distance, and it releases from  HarperCollins Children’s Books on February 21. It’s the story of  11-year-old Penny Rooney, who has cystic fibrosis and bonds with a  dolphin she discovers lost in her backyard creek.

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Writing  and editing this book was like no other writing experience in my  career—I’ve joked that it was like 30+ years of therapy all rolled into  one writing experience. It was difficult, but also deeply cathartic and,  in many ways, joyful. Some of the things I learned through the writing  of this story were ones I expected, but many were surprising.

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I  discovered, for instance, that marine mammals are prone to pneumonia  caused by many of the same bacteria that colonize CF lungs, including Pseudomonas aeruginosa and Myco-bacterium abscessus. I  read articles in veterinary journals about researchers doing PFTs on  captive dolphins and administering nebulizers to manatees. I learned  that marine biologists do sputum cultures on sick whales and dolphins to  better treat their infections.

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I also  discovered just how deep ran my own learned impulse to put a happy face  on any situation, no matter how dire. One of my goals in writing No  Matter the Distance was to deconstruct and push back on the way so many  children with serious illness are raised to feel they must be upbeat,  positive, and “inspiring,” no matter how difficult their experiences may  be. When I was a kid, I felt surrounded by adults—many of them medical  professionals—who, during my most traumatic PICC placements or lonely  hospital nights, tried to reassure me by telling me stories of CFers  whose health was more critical than mine and pointing out that my  situation could be a lot worse.

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Instead  of cheering me up, this often invalidated my own feelings of grief or  pain and made me feel like I wasn’t “strong enough” to deal with routine  but traumatic CF experiences. In No Matter the Distance, I explored  this idea, trying to show through Penny’s story that hardship is  hardship, even if it “could be worse.” I let my main character be  frustrated when she had to go to the hospital and be afraid when she had  to get blood drawn or get a PICC placed, even though she and I both  understood that these were routine difficulties for a CFer.

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To my  surprise, when I got notes back from my editor, her biggest critique was  that I hadn’t let Penny struggle enough. “These are hard things she’s  going through!” she wrote. “Penny seems unrealistically optimistic to  me. I think you need to give her more room to be upset and angry about  what she’s experiencing.”

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It took  me several days to digest and understand my editor’s feedback. Hadn’t I  already done that?! Hadn’t that, explicitly, been what I was trying to  do with this book?

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As I  went back through and reread my draft, I not only saw all the places  where Penny was brushing off her own struggle—I saw how much I, as the  author, felt the need to brush off Penny’s struggle as well. I found  myself tempted to call my editor up and explain how really, a hospital  stay and a PICC line weren’t that big a deal, and Penny’s situation  could be so much worse.

But I  remembered what it felt like when I was 11 and experiencing my first  hospital stay since childhood and got my first PICC line. I remembered  the anxiety, the anger, the feeling of unfairness at what I was  experiencing compared to my friends without CF. I thought of all the  kids with CF and other serious illnesses who might read my book one day,  and, as I revised Penny’s story, I hoped that reading it would tell  them:

It’s okay to be angry.

It’s okay to be sad.

It’s okay to struggle.

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In the  process of revising No Matter the Distance, all of this inner turmoil  led to a light-bulb moment about something that has plagued me my whole  adult life. Throughout my whole 15-year relationship with my husband,  I’ve found myself irrationally angry when he gets sick. (And he’s a very  mild-mannered sick person—not a man-cold in sight!) As much as I try to  be a generous and supportive spouse, I’ve always struggled with feeling  impatient and unsympathetic whenever he or other “healthy” people were  sick.

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While  working on my revision, I finally realized why this was. After a  lifetime of being told that my own health challenges “could be so much  worse,” and rarely offered space to rage or grieve experiences that felt  difficult to me, I instinctively passed the same attitude on to others.  Oh, you’ve got a cold? People are dying out there. Buck up and deal  with it!

I found  myself needing to take a few quiet minutes to have a conversation with  my child self: I recognize that nobody honored your pain when you were  experiencing it, but they should have. You do not have to be strong.  It’s okay to fall apart.

And,  surprisingly enough—the next time my husband had a health issue, I was  able to remain neutral and loving and not be swallowed by my own  resentment.

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No Matter the Distance is not a universal CF story. There’s no way to cover in one novel the  myriad diverse experiences that different CF patients have. But I hope  that reading the book will help give other chronically ill patients a  little bit of the gift it gave me as I wrote it: the gift of validation  and an acknowledgment that growing up with a disease like CF can be  tough and traumatic, and that we all deserve space to struggle—no matter  how much worse it could be.

Cindy Baldwin is 34 years old and has CF. She is the author of several books with HarperCollins, including the upcoming No Matter the Distance (releases February, 2023), which features a protagonist with cystic  fibrosis. Cindy lives near Portland, OR, with her husband and daughter.

Many  people have submitted questions related to a COBRA extension of health  insurance benefits. Nothing in this article is meant to be legal advice  and is only information. Nothing in this article is meant to be a  guarantee a person will receive an extension of health insurance  benefits under COBRA.

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If you  have questions about laws related to health insurance, Social Security  benefits, Medicaid, Medicare, employment rights, or education rights,  you can contact the CF Legal Information Hotline (CFLIH) by emailing CFLegal@sufianpassamano.com or calling 1-800-622-0385 to schedule a time to speak with an attorney.  Emailing is the quickest way to set up a time to speak to the CFLIH.  All calls are confidential and there is no cost to the caller. The CFLIH  can only provide information to callers. The CFLIH cannot provide legal  advice and cannot provide legal representation to callers.

The CF Foundation provides funding for the CFLIH. CFLIH employees are not employed by the CF Foundation.

Question:  If an employee and dependent child are enrolled in an  employer-sponsored health benefit plan covered by COBRA and if both the  employee and dependent child elect COBRA extension coverage upon a  qualifying event, may the dependent child continue the COBRA extension  coverage for the entire COBRA extension period even when the parent ends  the COBRA extension coverage before the end of the entire COBRA  extension period?

Answer:  Independence of COBRA Election. The essence of this question is whether  the covered child’s COBRA rights are dependent on the parent’s COBRA  extension or is the covered child’s COBRA election independent of the  covered parent’s COBRA election. The Code of Federal Regulation explains  COBRA through a series of questions and answers. See 26 C.F.R. §  54.4980B-6. The regulation states that each qualified beneficiary  (including a covered child) make an independent election to receive  COBRA continuation coverage. Section 54.4980B-6 of the Regulation  explains it in this manner: Can each qualified beneficiary make an  independent election under COBRA? Yes. Each qualified beneficiary  (including a child...)...must be offered the opportunity to make an  independent election to receive COBRA continuation coverage.... An  election on behalf of a minor child can be made by the child’s parent or  legal guardian. 26 C.F.R. § 54.4980B-6.

Question:  If I am on COBRA, is there a way my health insurance coverage could end  before the set amount of time that I am supposed to receive an  extension of my health insurance benefits?

Answer: Specific Reasons COBRA Continuation of Coverage May End Before the Maximum Extension Period Expires.

Section  54.4980B-7 of the COBRA regulations specifies the events upon which a  health benefit plan may terminate COBRA continuation of coverage before  the end of the maximum extension period. A group health plan may  terminate coverage earlier than the end of the maximum period for any of  the following reasons:

1. Premiums are not paid in full on a timely basis;

2. The employer ceases to maintain any group health plan;

3. A qualified beneficiary begins coverage under another group health plan after electing continuation of coverage;

4. A qualified beneficiary becomes entitled to Medicare benefits after electing continuation coverage; or

5. A  qualified beneficiary engages in conduct that would justify the plan in  terminating coverage of a similarly situated participant or beneficiary  not receiving continuation coverage, such as fraud.

See 26 C.F.R. § 54.4980B-7.

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Significantly,  a parent ending their COBRA extension period early is not one of the  reasons that would permit a plan to end a dependent child’s COBRA  extension period before the end of the maximum period.

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A  dependent child of a covered employee is a COBRA qualified beneficiary.  The reasons specified in 26 C.F.R. § 54.4980B-7 apply to “qualified  beneficiaries”, which raises the question of whether a dependent child  of a covered employee is a COBRA-qualified beneficiary. The COBRA  regulations define “qualified beneficiary” as: any individual who, on  the day before a qualifying event, is covered under a group health plan  by virtue of being on that day either a covered employee, the spouse of a  covered employee, or a dependent child of the covered employee. 26  C.F.R. § 54.4980B-3(i).

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The  COBRA discussion will be continued in a blog post on the USACFA website  at the end of January 2023. Don’t miss out on blog posts! You can sign  up on our website to have blog posts delivered to your email address.

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I’m  wishing all of the CF Roundtable readers a healthy and happy 2023. The  year 2022 was not one of my favorite years. I am looking forward to  finding ways to make 2023 more peaceful, safer, and balanced. I am  grateful to have so many friends in the CF community who provide me with  love and support. I am so lucky to have so many friends around the  country who brighten my days and provide joy and encouragement. As we  enter our 25th year of the CF Legal Information Hotline, I look forward  to continuing our work helping those with CF, their families, and CF  care teams be informed of their legal rights.

Beth  Sufian is 57 years old and has CF. She is an attorney who focuses her  law practice on disability law and is the Vice President of USACFA. Her  contact information is on page 2. You may contact her with your legal  questions about CF-related issues at CFLegal@sufianpassamano.com.

So  much of our spiritual well-being is rooted in the energy in our heads  and hearts. Our thoughts create emotions such as anger, sadness,  resentment, or hatred, and those emotions, in turn, impact our actions.   Our actions impact our relationships and our community.

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Life  with cystic fibrosis can be hard. We spend so much energy managing our  bodies and keeping up with the demands of regular life. Since this is  the one life we have, I believe we all want to live our best life  possible. Cultivating inner peace and calm can parry the blow of life’s  challenges. And one way to find this peace and calm is to continually  access and tame our inner worlds: our automatic thoughts.

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We all  have around 300 thoughts per minute, every day of our lives. Automatic  thoughts are immediate, knee-jerk reactions to external stimuli. In  relationships, when someone gives us a look, we interpret that person or  their appraisal of us. If someone doesn’t call us back, we create a  story as to why. If we didn’t get the job, it’s because we are a  failure. If we are hurt, we blame someone. Automatic thoughts are a  rapid response to the world around us. They are usually learned as safe  patterns early in life but are not always helpful in the present moment.

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Usually,  automatic thoughts are negative. They alert us to a problem. We  catastrophize, judge, label, mind-read, blame, generalize, “should on  ourselves,” or personalize the issue at hand. We engage in unevolved  defenses or revert to old unhelpful patterns about our worth and value.  Automatic thoughts are also informed by learned biases and prejudices.  Think of automatic negative thoughts as the acronym “ANTs.” Your  thoughts literally are like a row of ants, marching onward, chaotically,  mechanically and mindlessly, sometimes multi-directionally toward  something. They sniff out trouble and head for the garbage. Like ants  coming indoors during the rain, ANTs become stronger during times of  stress. And automatic thoughts worsen our stress by releasing more  cortisol and adrenaline, hyping us up so we have the illusion of power  and strength.

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Often my  automatic thoughts are about my body—anticipating a health crisis or  doomsday. I recognize that many of my automatic thoughts are habits  risen from my long history where these reactions were helpful to get  treated when I was indeed in a crisis. When I was younger, many of my  ANTs were social. Walking into a room of strangers, my ANTs told me  people were thinking something negative about me based on what I looked  like. Eventually, I learned I cannot mind-read and cannot be bothered by  what others think and have since extinguished those ANTs.

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During  my recent travels, I had to walk many miles to go to sites I wanted to  see. My body was exhausted and weak, but I was traveling, and I wanted  to push myself. My regular automatic thoughts included: “I’m  diminishing,” “This could be my last trip,” “Something’s wrong with my  legs,” “Maybe the cancer is spreading,” or “I’m too slow for my family.”  The tangle of thoughts distracted me from what was in front of me. I  noticed them, tried to relax around them, and told myself, “This is just  a habit, and it’s OK.” Then I reminded myself compassionately that I am  on chemotherapy, that I am out of shape, but that I am capable of  walking, just at a slower speed with breaks. I felt gratitude for being  able to be well enough to travel. I looked forward to my next nap, or my  next chance to sit down. I thought of the thousands of disabled and  elderly who struggle to walk and yet keep going. I said a little prayer  to God to help me develop the stamina and patience to be OK with what  is. I communicated with my family about what their needs were, and  whether there was another strategy for them to keep going at a faster  pace. They told me they are fine with my pace, and I’m the one who is  always in a hurry, and eager to do more and see more! And then I made up  an excuse to stop for ice cream as a way to rest.

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So what  are some ways to cope with ANTs? True maturity is being able to pause,  notice an automatic thought, assess whether it is helpful, and find ways  to let it go or change it into more adaptive thinking. This pause turns  down the dial of stress that ANTs turn on. The pause offers  discernment; to wait for clarity on the thought at hand. This allows  time and space before the next move to become clear. And for our minds  to deliberately insert positive thoughts to refute the ANTs.

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I have  written in past articles that sometimes it can be helpful to find code  words or phrases that we can use during an impulsive reaction like ANTs.  These phrases serve as a yield sign to the brain and shift the focus to  something more adaptive. A code word or image might be a stop sign or  an untangling string or whatever helps hold the moment in space for a  second. I like to imagine a magnifying glass to remind me to look more  clearly at what’s going on with my thoughts. If an ANT is about my body,  I use the code word “iiwii” or “it is what it is.” I try to remind  myself what’s working and going well. That doesn’t mean dismissing the  thought, and carrying it through to assessment and action, but rather to  balance it with a reality-based thought about the situation at hand.

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There  are questions we can ask ourselves when an ANT pops into our minds. For  example: Is this thought true? What is this thought doing to my emotions  and is that justified? Is this thought serving me or is this unhelpful?  Is this a thought that is aligned with my values? What is my role or  responsibility in having this thought? What am I learning about myself  from this thought? Does this thought really matter? What does God want  for me now?

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ANTs are  often primitive animal instincts that serve to protect the ego from a  perceived threat. They can be seen as survival instincts. Like any  survival instinct there is a moment of decision making. Our conscious  selves can decide whether to believe in the thought and then whether to  act on it. Is there really a threat? What exactly is being threatened?  (Many times, the threat is created in one’s own mind.) If there really  is no threat, ignoring the thought is good enough. If we act on the  threat, the decision to take the high road or low road is in our hands.

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Noticing  automatic thoughts gives us access to our true, authentic, deeper Self.  The deeper Self is the observer who is watching the theater of thoughts  inside my head. The deeper Self is the healed Self who is in  equilibrium, peace, and harmony. My true mind wants to be relaxed. If  automatic thoughts are angry and resentful, then forgiveness is on the  pedestal of the deeper Self. As people with CF, we ought to live in a  way that supports the optimal functioning of our bodies, minds, and  spirits. Through struggles, CF gives us an opportunity some healthy  people don’t get until much later in life: the potential to become more  self-aware of our thoughts and control our well-being. To “know thyself”  is life’s greatest gift.

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If we  have cultivated a spiritual life, it can help to take automatic thoughts  and pass them along to a higher being like Jesus, Muhammad, Buddha,  God, Mother Earth, or our ancestors. We can accept our confused or  broken ways and invite divine grace as a force in our lives. We can call  on our values that guide us and help us to follow the loving path  toward ourselves and others. There is so much more power and strength in  love than in negativity! The spiritual paradox is both surrendering our  control to something beyond ourselves while also owning our control in  our lives. Taking responsibility for our own thoughts and feelings as  well as for our actions and reactions is the key to spiritual freedom.  Ultimately, controlling our inner world can help us be the person God  wants us to be.

Afternote:  If you have trouble accessing the parts of your mind that will allow  you to notice and cope with automatic negative thoughts, I recommend  seeking out a therapist to help you in this process. Therapy gives you  undistracted space and time to focus on you and your betterment.  Accessing our inner world first will help us become better advocates for  ourselves in the larger world. And make us better people to others.

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Accessibility  to mental healthcare is a whole other challenge, that hopefully others  have written about. Our best mental healthcare can be the safe and  trusting people in our lives. Trained therapists are available on a  sliding scale, remotely, and through organizations like CFRI. Spiritual  counselors (chaplains, spiritual direction coaches, or Stephen  Ministries) are also available. You can inquire at your local place of  worship or check out psychologytoday.com. Always ask for a sliding scale  if needed or call your insurance for a listing of covered therapists. I  think I am preaching to the choir, but just wanted to offer a few tips  on accessing mental and spiritual healthcare for this issue.

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  all seemed so innocent. I was living my life peacefully in Tennessee  when I landed in the hoosgow somehow. I guess it was a kill-shelter  because some nice people from NYC came from a rescue organization called  Social-Tees and whisked me up north to the Big Apple. They believe they  saved me from death row. Well, my tale (or tail), gets more involved  from there.

A bit  about me: I like a sedentary life—lying on the sofa, rolling on my back  and waiting for belly rubs. Who doesn’t?! And I like my food; just keep  it coming!

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This  woman who adopted me—I will call her the mom of me—is pretty chill  except when she decides we are going for walks. Not sure if they are for  me or for her, or Willie, who I will get to shortly. I could easily  spend my days lounging around. But no, we walk about one to two miles a  day. It started slow, though, to trick me. I used to just refuse to go  any farther than I absolutely needed to do my business—maybe two or  three blocks each walk. But I now realize it was a ruse to get me used  to walking farther. My mom’s husband, who thinks he is the boss, and  whom I will call the Boss, decided we should go hiking one day.

In this  family, I am the last to arrive. First was Willie, the oldest at 13, who  was owned by my mom’s mom. He’s pretty spry for an old guy. Then came  Roscoe, the big lug, who is three. And I am around seven, but look a bit  older. I will never tell my true age. A lady never tells. We make a  Motley crew. I like them both, my brudders. Outside, Roscoe and I tear  it up chasing after each other. Willie prefers a milder frolic so we  leave him alone ’cos he old!

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All of  these walks that Willie, me, and my mom were taking got longer and  longer so that we were out for almost an hour each time. I think it was  also possibly to help me lose my “pandemic pounds” I put on (or the ones  my mom did). It was disguised as trips to the dog run and then on from  there. And on and on.

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About my  mom. I like her. She is home a lot. She does these “treatments” in the  morning and at night. She cooks a lot and creates good smells in the  apartment. She also has “health” appointments frequently and assures me  and Willie she will be home soon to take us out again soon. Then we all  nap together almost every day under the covers. Which I enjoy. Roscoe  spends most of his day with the Boss.

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I guess  the hiking thing is what the Boss does. My mom takes me and Willie to  Long Island and we go walking at the beach or in the park or in the  yard—all level ground. So, I know nothing of hiking up a hill? No way.  Why does anyone want to do this?

One day  we all load into the Boss’s car and drive for five hours from New York  City, probably due North. And we end up in the Adirondack mountains. We  get there pretty late in the afternoon and, the next day, we hike up a  one-mile trek to a nice little lake. It was not horrible. We ate some,  walked around and then left to hike up an even bigger hill. Still about  one mile up, but waaaayyyy steeper. I even helped pull my mom up the  hilly parts. She was huffing and puffing and had to stop several times. I  acted like I could keep going but was happy to take a breather with  her, too.

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Eventually  we got to the top and I was not sure what the big deal was, but the two  leaders—Boss and mom—were elated. The Boss was amazed I made it up  there. I think my mom was even more amazed she did.

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After  this trek, I seriously considered packing up and heading back to  Tennessee, from whence I came. But then I see how much I am loved and  accepted and think I may stick around a bit more. No more pointless  hikes, PLEASE!

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All in  all, this whacky brood is fun. I think I will stay, as long as we don’t  have to climb too many more peaks. I am happy just walking a few miles a  day in Riverside Park or at the beach. I do see it is helpful for my  mom to have a willing companion for walks, to clear her lungs, and  someone to supervise her percussor after inhalations—also lung clearing.  As well as someone to nap with. She needs me. And I think I am  realizing, I need her, too, and even the Boss.

Andrea  Eisenman is 58 and has CF. She adopted Trixie in August 2022. She and  Steve Downey share Roscoe, Willie and Trixie, their travel companions on  the journey of life.

Please consider sharing your Pet’s Perspective in upcoming issues of CF Roundtable.

Financially  things are tough for a lot of people, including those in our CF  community. Groceries have gotten more expensive and those receiving  assistance from the Supplemental Nutrition Assistance Program (SNAP) are  finding it harder and harder to make tasty meals on a budget.

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The  great thing about a dish like hash is that it is so versatile you can  add almost anything in one pan. Hashes are, by nature, gluten free  because potatoes are the base, and hashes can be dairy free. They can be  vegetarian or vegan as well. I make a hash when I am running low on  ingredients or am trying to finish up any items that may go bad soon.

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My  Mexican hash is great because it utilizes some canned items that are  cheap and easy to come by.  The notes at the end of the recipe offer  suggestions of what to add or subtract, depending on what you have in  your pantry/fridge and based on your budget. I have written this recipe  for 4-6 servings, but if you are cooking for 1-2 people you can cut the  recipe in half, freeze the other portions, or eat them as leftovers for  the next couple of days.

Mexican Potato Hash

Yield: 4-6 servings

Prep time: 15 minutes

Total time: 45 minutes

Ingredients:

2 tbsp oil of your choosing

3-4 medium potatoes (any kind)

1 medium onion (white or red)

1 packet of taco spice mix or your own taco spices

1 15oz. can of black beans, drained

1 15 oz. can of corn, drained OR
1 cup of frozen corn kernels

2 cups shredded rotisserie chicken or canned chicken (optional)

Optional toppings:

Sour cream

Salsa

Green onions

Fresh cilantro

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Preparation:

Step 1:

Chop potatoes into bite sized pieces leaving skins on for ease and more nutrition. Slice onion into ¼ inch pieces.

Step 2:

Add oil  to sauté pan. Heat pan on medium to medium/low. Once oil is heated, add  potatoes. Let them start to sizzle and then stir. If potatoes seem to be  sticking, add a few tablespoons of water to help steam a bit. Once  potatoes start softening (after 20 minutes) add your sliced onion. Let  the onions cook for 2-3 minutes. When they start to become translucent  add the spice mixture and stir.

Step 3:

Add in  the black beans and corn. If your corn is frozen you can also add it  straight into the pan. Give everything a stir and test your potatoes  with a fork to see if they are done. When the fork pierces through  easily, they are cooked.

Step 4:

Finally,  add your chicken and cook for another 1-2 minutes until chicken is  heated through. Taste everything to make sure there is enough salt.  (Store-bought spice mixes usually have plenty of sodium.)

Step 5:

Plate the hash and add any toppings of your choice. Salsa, sour cream, and cilantro are my favorite toppings to add.

Notes:

This  dish is already a complete meal, but if you want to add some greens, add  spinach or kale at the end until they are wilted down. If you need to  get rid of any bell peppers, this is also a great addition. Just chop  into small pieces and add with the onion. Adding a fried egg on the top  gives it a more breakfast/brunch feel. You can also use any beans,  including a can of chili beans if you prefer them over black. Also, omit  the chicken if you want to keep it vegetarian or vegan. 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.

By  the time we reach adulthood, we CFers are well used to finding creative  solutions to get things done while fitting in treatments, doctor’s  visits, exercise routines, and hospital stays. But even for the savviest  of us, parenting can pose huge issues: Juggling parenthood and CF care  can often feel overwhelming, and it can be next to impossible to get  everything done. As with any other job, parenting with CF can require  creative accommodations.

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As a mom  to an almost-ten-year-old girl, I’ve had a lot of time to try, fail,  and try again to perfect the juggling act of caring for my daughter  while keeping myself as healthy as possible. Although I don’t always  strike the perfect balance, the following tips have helped me get as  close as I can.

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1. Make  it possible to do treatments on the go. While not everyone still does  multiple breathing treatments a day on highly effective modulators, lots  of us still do, and fitting those regular treatments in while raising a  small kid can be grueling. When my daughter was four or five,  battery-operated vests hit the market and helped revolutionize my  ability to get therapy done in all kinds of situations. I also have two  nebulizer compressors—one in my “normal therapy spot” in my family room,  and one in my car—so that I can do treatments while driving if needed.  These days, I often do 50% or more of my morning treatments in the car  while driving my daughter to various activities. (An inexpensive  compressor from Amazon can be a good car machine if your insurance won’t  cover one. I pair mine with a car inverter that plugs into my car’s  cigarette lighter and allows me to run my compressor while the car is  on.)

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2. Use  the tools you need to keep yourself healthy. Parenting a newborn is  overwhelming, especially when you tune into the many messages the world  sends about the right ways to raise a little one. But parenting with CF  sometimes requires compromises. When my daughter was little, I mostly  breastfed her, but my husband gave her one bottle of formula every night  so that I could get a few hours of uninterrupted sleep. Whether you  need to use formula, baby swings, or other tools to help care for your  child while you get what you need to stay healthy, almost nothing is  more important for your baby than having a healthy parent.

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3. Let  your kid join you in exercise! For several years when my daughter was  little, the only way I could get a workout in was if she was heavily  involved. We’d go for walks with her in the stroller, bike rides with  her in a child seat, and do exercise videos on YouTube geared for kids.  Cosmic Kids Yoga is a great YouTube channel with routines easy enough  for kids to follow, but rigorous enough to provide a light yoga workout  for busy parents. Did I get tired of Frozen-themed yoga? Definitely! But  it was worth it to get my exercise in without it cutting into my rare  moments of child-free time.

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4.  Minimize germ exposure where possible. Kids are serious germ factories,  and the sad fact of the matter is that, as a CF parent, you won’t be  able to escape all of their plague-ridden coughs and sniffles. But for  those of us who have immune system issues or for whom a cold is  generally a ticket to the hospital, it’s worth implementing  germ-mitigation strategies during times of high viral transmission. When  Vogmasks became popular while my daughter was a baby, I started making a  practice of wearing one in crowded public spaces when there were a lot  of viruses going around, and it dramatically reduced the number of colds  I got. These days, my husband, daughter, and I all mask up in high-risk  settings, and we’ve all stayed much healthier as a result. We’ve also  seen a reduction in the number of colds family members bring home when  the person who’s been in a high-risk setting, like school or work,  showers and changes clothes immediately after coming home. Finally,  communication is key: When planning play dates or get-togethers with  other parents and their kids, I’m up-front about my viral susceptibility  and honest in asking that they please let me know if their kids are  sick before we see them. I’ve found that, in many cases, parents simply  don’t realize how serious toddler sniffles can be for someone with a  condition like CF, and most people are happy to give a heads-up if a  child is sick.

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Parenting  with CF requires a lot of creative accommodations—but being willing to  be flexible and think outside the box can go a long way in making  parenting accessible for us.

Cindy Baldwin is 34 years old and has CF. She is the author of several books with HarperCollins, including the upcoming No Matter the Distance (releases February, 2023), which features a protagonist with cystic  fibrosis. Cindy lives near Portland, OR, with her husband and daughter.

I  had a lung transplant 24 years ago and a kidney transplant 16 years  ago. Prior to my lung transplant, I experienced respiratory failure and  was on a ventilator for five months. I was in a rehabilitation facility  for three of those five months, where they were able to get me off the  ventilator. Prior to going home, we (me, my family, respiratory  therapists, and nurses) took a trip to my townhouse, which is three  stories (lots of stairs!) to determine what accommodations I needed to  live there in my weakened body. I almost cried when we got there; I  hadn’t been home in almost six months. One thing they determined I  needed was a chair placed on the landing of the stairs going up to the  bedroom. I couldn’t make it the whole way up without getting completely  exhausted and out of breath. I also needed a chair in the shower because  I was so weak I couldn’t stand very long. I had a wheelchair for the  same reason. Not that I was going out much, but I needed it for doctor  and lung transplant evaluation appointments. Since I was on oxygen, we  decided to replace our gas stove with an electric one just to be on the  safe side. My mother-in-law was the beneficiary of the gas stove; we  were glad she was able to use it! I had a big tank of oxygen in our  bedroom just in case the oxygen concentrator stopped working or we had a  power outage. The team advised us to call our electric company to be  put on a list of homes that need power in an emergency. We did lose  power and had to switch to the oxygen tank. This was very stressful  since we didn’t know when power would be restored, but luckily the  outage only lasted a few hours.

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Since I  was coughing a lot, we needed to prop up my side of the bed to a  45-degree angle. We used two big pillows and a bed rest pillow, which  helped immensely. I was also dealing with a lot of anxiety. I had to  take anti-anxiety medication every time I took a shower and used the  Vest. The Vest especially made me very claustrophobic, as you can  imagine. I could not bear to be alone, so my mom stayed in our bedroom  if my husband was out of town. We also had a walkie-talkie next to my  bed so I could communicate with my family downstairs. My family really  went all-in on trying to make my horrible situation bearable, always  thinking about ways to help me deal with my situation, both physically  and mentally. I am very lucky to have such a great family who are always  there when I need them.

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A few  years ago, I ended up with non-tuberculosis Mycobacterium (NTM) and  Aspergillus. That was pretty rough, but I got through it with a lot of  help from my CF, lung transplant, and infectious disease doctors. After  that happened, my doctors suggested I start doing hypertonic saline  nebulizers and using the Monarch Vest. I do these for maintenance  purposes, to keep my lungs clear, and I have not had a lung infection  since I started these therapies. I was not thrilled to be back on a  nebulizer treatment and postural drainage, but I try to adapt to keep  myself healthy. Additionally, I am also on Trikafta, which I also credit  with me not having lung infections. I tried taking the full dose, but  it caused my ankles to swell up. On a lower dose, I no longer have that  issue and I still get the benefit of Trikafta clearing out my sinuses.

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Skin  cancer is also something that I’ve been dealing with for a long time,  since people who are immunocompromised are more prone to getting skin  cancer. This time I have squamous cell carcinoma on my right thumb. I  started seeing a dermatology oncologist for this, since everything my  regular dermatologist tried didn’t work. She had me use several  medicated creams, but the cancer kept coming back. My doctor also tried  photodynamic therapy (PDT), which killed the cancer on my arms but not  my thumb. PDT is very painful since you are basically frying your skin  with a blue light for 15 minutes, but it was worth it since it got rid  of at least some of the cancer.

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After  exhausting all treatment options for my thumb, my doctor sent me back to  the radiation oncologist. He is the one who did my radiation on my head  for another squamous cell carcinoma in 2011. I had seen him about my  thumb to talk about my options, and he is the one who sent me to the  dermatology oncologist. He also (nicely) said he never wanted to see me  again. Ah, famous last words! I actually apologized to him for coming  back. He devised my plan of treatment and told me there was an 80%  chance it would kill the cancer. Good odds, so I asked, when do we  start? Within a couple of weeks, I started radiation therapy. I just  completed six weeks of radiation on my thumb, going to the hospital  every weekday. Not fun, but at least it was fairly close to my house,  and the radiation treatment itself only took about five minutes. In the  waiting room I saw people who had it much worse than me; I tried to keep  that in mind as well.

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My thumb  became blistered and very sore as the treatment went on, and my  thumbnail is half disintegrated and the rest is about to fall off. My  doctor said it probably would not grow back. Ewww! Now we wait for a  month for my thumb to heal and see if the cancer is still there. If the  cancer is still there, they may have to amputate my thumb. The only good  thing about this, besides getting rid of the cancer, is that I am left  handed. I did, however, start paying attention to how much I use/need my  right thumb. It does come in handy when opening jars or carrying things  or driving or typing. I also took note of things like gloves and oven  mitts which have a separate thumb section on them. Sigh. There will be  plenty of other accommodation and accessibility issues that I will have  to take into account and prepare for. I guess I will learn as I go, if  it comes to that.

Fingers (and thumbs) crossed that I will be cancer free and not thumb free!

Colleen Adamson is 53 and has CF. She is the Treasurer of USACFA, and lives in Alexandria, VA. Her contact info is on page 2.

In  our last interview, Jerry Cahill was losing his long-term disability at  age 66 and a half. It ended on January 2, 2023. As Jerry now embarks on  heading back to work full time, we sat down to go over what this might  entail at this point in his life: after a long career in retail,  volunteer work at Boomer Esiason Foundation (BEF), and coaching. Instead  of retiring, he is reinventing himself yet again. He talks about his  fears, his passions, and what propels him forward. He considers doing  all this a juggling act worthy of the center ring at Ringling Brothers  and Barnum & Bailey. Read on to learn more.

Explain what “reinventing yourself” means at this stage of your life at age 66.

Reinventing  has been something I look at in a positive way. I worked, I played  sports, I had to go off disability; I believe you are never done, that  you have to keep pushing forward. I try to live in the realm of  possibilities. Here I am again having to reinvent myself. I never  thought, at 66, that I’d be in this position of having to go back to  full-time work. I feel I am starting my career again, except at this  late stage in my life. It’s odd to me and I don’t quite understand it.  Even though I see people around me who are my age and I think they are  old, I just don’t think of myself as “old.” It’s an interesting time for  me. I feel I am walking into a dark room and finding my way.

What do you see as pluses or minuses as far as reentering the job market at this stage of your life?

The  minuses would be that growing up with CF, since I am ancient for someone  with CF, I never thought there was a future and never saved my earnings  to be able to fully retire. Even though I had great jobs with decent  salaries, I didn’t see the point of saving for retirement since I was  not guaranteed a long future. The pluses of getting back into the job  market include making money to support myself. Financially, it is not  easy for anyone to live while working, especially someone who has CF and  has lived through three transplants. Life is not easy. I feel as though  I’ve run this marathon in life and I wanted to finish and enjoy, but I  have to keep going [now back to work].

Due to  my age, I cannot do what I did when I was 30. This concerns me as I have  more fatigue and things take more of a toll on my body than they did 35  years ago. Whether it’s exercise or daily living or coaching, which is  my passion, it becomes harder to manage it all and stay on top of my  health.

Will you retire?

Within a  few years I will have to retire and live very frugally on a strict  budget. But I think I will always do some work. I do not think I will do  well being idle. I would definitely still coach, but that won’t pay my  bills. It is just something that is extremely gratifying to me.

What are your fears?

I may  have to give up certain things in my life that I like so that I can go  to work full time. I also have an A+ personality so I want to do the job  at 200%. I do not want to give up things solely to survive financially;  obviously, I want more life, more coaching, more of everything.  Honestly, I am unsure of how much I can do, so this all brings anxiety.

My  type-A personality helps propel me like a rocket flying through the air.  I am always moving on to see what’s next; what do I have to accomplish  now. I keep pressing on to move forward. I don’t give myself time to  think of what I just went through—it’s simply on to the next thing. It  can also hurt me as I do not take time to enjoy my achievements. I like  to keep busy and feel like I am helping others. I try to lead by example  so others with CF can see what is possible and possibly help them think  they can do it, too. It’s like we in the CF community are part of a  sports team and if we see others doing well, it gives others hope that  they can, too.

My fear  is that my time and commitments may have to be balanced while I work and  fit in the coaching, As it is now, my sleep suffers as does my personal  exercise routine. I have to manage my time so I can fit everything in.  Even now while I’m currently volunteering, I already feel as though I am  walking on egg shells and I worry I will get sick from being too burnt  out.

What do you do exactly when you coach?

When I  coach, I do it at two different schools twice a week. One is Iona Prep  and the other is Fordham Prep. I drive to each (I do not take public  transportation for health reasons) and it entails paying for parking,  tolls, and gas twice a week. I don’t get home until 10:30 p.m. and this  is all after a full day at work. It takes a lot of energy to rally these  high schoolers, but I love it. On weekends there are track meets that I  coach as well. Even though I am reimbursed for the travel expenses, I  am basically volunteering my time. I find that volunteering this way is  quite rewarding.

What I  do is cheerleading and positive attitude while jumping around and  encouraging them to be their best. It is exhausting, both mentally and  physically. After my practices, the kids on the teams probably go home  and sleep like babies. I get home at night and I am too amped up to  sleep.

When I  coach it is not just about teaching them a skill like pole vaulting; it  is life lessons. It is about being responsible. If they cannot make a  practice, they need to contact me. I also teach them respect for one  another and that we have ground rules to adhere to. I educate them about  being a better athlete and the importance of mutual respect and  understanding others. However, because I am immune suppressed, there is  no handshaking or group hugs. And if they have a cold or they are  getting sick, they have to tell me. That was a big learning process for  them and me. They are very good about it now. They are very aware of  their health status and share that with me. I like passing on what I  have learned in life.

Share a teachable moment with us.

Educating  them about having CF. I used to coach someone who is now a friend. I  assumed he knew I had CF, but it turned out he didn’t. He found out when  I invited him to a BEF relay event. He told me, “You are unbelievable!”  I was not sure what he meant. He told me he didn’t know I had CF and  yet I do all of this (volunteering at BEF plus running and biking). He  also said, “I guarantee that no one you coach knows you have CF!” So now  I tell my teams about CF. Once I had my lung transplant, I saw a  teachable moment—I described the importance of organ donation. These  kids had a lot of questions about issues we with CF take for  granted—where do they get these lungs, how long does this surgery take,  what is involved? Plus, I make sure they understand that they cannot  attend a practice if they are unwell or sick.

Are you getting your strength back?

I’ve  been building myself up post-liver and kidney transplants. I am seeing a  difference in my strength level. I do feel stronger but it’s a lot of  work. I feel like I fit more into a day than most normal people so I can  achieve all my goals. My friends are amazed at all that I do and I am  slightly embarrassed when someone asks me what I did.

You have mentioned Endure More®, what is this?

It is  similar to what I say about running a marathon and you’ve just got to  keep running. A friend of mine with CF who is involved with sports said  that we just have to endure more, like just keep going. And it is so  true even though I had transplants. I just have to keep moving forward.  You can give up or you keep on enduring and hopefully you get more life.  I think it’s a cool and true saying.

What keeps you going?

What  keeps me going is my will and passion to make a difference in my own  life and other people’s lives. This applies mostly to the coaching I do.  If I could afford to, I would coach all day long; it is that  gratifying. But it is not realistic because I cannot survive on that  salary. I am driven and, like an athlete who wants to break records, I  am going for the gold metaphorically.

“Pass it  on” is a line in the movie History Boys. A professor says to his  students, “Pass it on. It’s not for me, it’s not for you, but it’s for  someone out there”. And that is how I feel about coaching—I am passing  on lessons of what I learned about training but also life lessons. And I  continue to learn each day. I think it’s important in life to be able  to pass on what you have experienced. I have benefitted from others in  this respect as well by learning from others about what they have been  through. I continue to do this to support the communities I am part of.

This  issue we’re taking ‘Pearls of Wisdom’ to the front page to shine a  light on our Focus Topic of Accessibility! After three years of living  with COVID-19, this seems like a great time to reflect on the many  improvements the pandemic has fostered in virtual activity  participation. We’ve come a long way in the CF community and have much  to look forward to! Our future will only get better and brighter the  more we prioritize accessibility in the virtual spaces where we spend so  much of our time.

Let’s  zoom out a bit and think about what accessibility can mean—we’ve seen  many good examples in the past few years of how this term can refer to  many different experiences and needs. Many of us have seen things like  reserved parking spaces that meet the minimum criteria under the  Americans with Disabilities Act (ADA) for physical accessibility to  people who use devices such as wheelchairs and crutches. These are  certainly good examples of accessibility, but far from the full extent  of what this concept includes! Broadly speaking, accessibility is what  helps us live well with the functional limitations introduced by  something like CF, while participating fully in activities that matter  to us.

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Accessibility  is deeply individual; even within the CF community itself, people can  have very different needs and preferences for how we approach tasks.  Accessibility is also deeply contextual; what we need may vary quite a  bit from one situation to another. And of course, accessibility is  deeply intersectional; our needs are shaped by many different facets of  ourselves and our position in society. These can include—race and  ethnicity; culture and socialization; gender and sex; religion and  spirituality; and much more.

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Most  importantly, accessibility is realistic. The standard for determining  whether an activity is accessible is not participants being able to  engage with considerable struggle and effort, but rather participants  being able to engage with ease and comfort. This distinction has  thankfully become clearer to many people during the COVID-19 pandemic as  more neurodivergent people have “unmasked” ourselves in social spaces  that better meet our needs. The past three years have been a time of  tremendous learning about things like sensory accessibility, which makes  a huge difference for many of us neurodivergent CF patients. It feels  amazing not to spend the mental energy left over from the significant  physical and cognitive labor of managing CF itself on trying to “act  normal” when surrounded by sensory input that causes us terrible pain.

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Closed  captioning has also become much more commonplace during the pandemic.  Videoconference software platforms like Zoom, Google Meet, and Microsoft  Teams have greatly improved the functionality of their automatic  captioning utilities. These platforms have also introduced additional  ways to use third-party captioning tools and/or human live captioners  during events. Captioning not only improves accessibility during  synchronous activities for people with diverse hearing and auditory  processing characteristics, but also helps people work together  effectively in asynchronous contexts with less effort!

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“Accessibility  is for everyone” has become my mantra in encouraging new groups of  people to embrace captioning as standard practice. Often, abled people  become believers with astonishing speed when they realize how much  easier captioning and auto-transcription makes the process of taking  accurate meeting minutes. And of course, making space intentionally for  Deaf and Hard of Hearing colleagues in our virtual spaces matters  tremendously for the CF community, where many of our adult members have  lost significant hearing due to sensitivity to harsh antibiotics like  tobramycin.

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Here’s  the thing about accessibility: By its nature, it makes space for  tremendous diversity of both needs and preferences. Some folks find  captions distracting. That’s totally fine! In-meeting captions can  always be toggled on and off by individual users to optimize their own  experience. Accessibility embraces the uniqueness of each person as an  asset for justice in group settings. The standard of “reasonable  accommodation” included in the ADA reflects this spirit of always making  a good faith effort to fulfill as many different needs as possible,  which requires flexibility and creativity.

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Deliberately  imagining the world from the perspectives of our peers with different  lived experiences offers powerful support for accessibility in planning  group activities. I always think back fondly on the earnest question I  got from one of my students who had No Light Perception—a trait shared  by about 10% of the Blind community overall. They asked if I was Blind  myself because I always gave them directions that relied on non-visual  types of input—like things they would feel, hear, or smell as they  navigated a new physical space. This was just something I’d always done  because I grew up with a parent who has Very Low Vision. However, I had  never really thought about it until that moment.

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What  seemed so normal to me from growing up in my family and watching my  mother use assistive technology to complete highly precise visual tasks  in her neuroscience lab—and just being generally encouraged by my  parents and teachers to think about how my fellow people might  experience things differently than I did—often seems unusual to people  who grew up in ableist environments. So I try to embrace this same  energy in all of my advocacy for accessibility. Using our imagination to  anticipate the kinds of questions we’ll want to ask people about their  specific needs and preferences not only saves a lot of time, but also  communicates that we truly value the lived experiences of our peers.

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On the  visual accessibility front, many in the CF community deal with  significant visual changes from secondary conditions like diabetes and  vascular damage that our CF can cause. And of course, some folks with CF  have been Blind their entire lives for reasons completely unrelated to  this disease. A couple of basic strategies can make big changes for  visual accessibility in CF community events.

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Making  consistent use of ALT text is an easy and remarkably effective way to  support Blind community members as well as those with visual processing  challenges. This simple utility helps screen readers describe images  accurately for those who use them; people who don’t use screen readers  can also pull up the text directly and read it. When in doubt, right  click on images you’re adding to your presentations and communications.  Generally you’ll see an option to “edit ALT text” or provide an “image  description.” When you’re writing descriptive text, keep it simple! You  generally want a few words conveying the basics of the content in the  image—for example, “great blue heron resting on a pier overlooking a  lake at sunset.”

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Then  there’s the question of backgrounds and contrast. Here’s where it gets  tricky! What is optimally accessible for one person may be terrible for  another when it comes to presentation and styling. If you participated  in our Scholarships webinar on December 7, the slides we used for that  session went through some revisions to improve functionality for as many  folks as possible! Even with a Blind parent and some visual  accessibility needs of my own related to extreme photosensitivity, I  can’t always anticipate what type of content may work best for a mixed  group.

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But  getting things exactly right on the first try isn’t the standard for  justice. Rather, it’s the commitment to continuous and enthusiastic  improvement. I nailed the color contrast on my first version of those  webinar slides and incorporated ALT text consistently for images, but  the darker background on some of the more aesthetic slides might have  made it challenging for some folks to distinguish different content  elements. I restyled the slides to preserve the overall aesthetic on  those portions while offering a lighter background for the text items. I  had to put some thought into it, even though I’ve been thinking about  this stuff my whole life. And that’s okay—again, accessibility is about  making our best faith effort to provide the broadest and most  customizable functionality we can. It’s not about being perfect or  superhuman, just attentive and responsive.

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We’ve  seen quite a few improvements these past three years on the social  accessibility front that reflect this spirit of knowledge as power. It’s  now becoming commonplace to attend CF community events whose platforms  intentionally make space for sharing things like our pronouns, prefixes,  and name pronunciations. These types of information help folks know how  to address us properly without having to ask. Feeling seen is  absolutely an important part of being accommodated—and an excellent  illustration of how accessibility really is for everyone.

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Likewise,  event platforms these days often give folks the opportunity to share  about our sexualities, sex and gender identities, religious and  spiritual traditions, racial and ethnic identities, cultural  backgrounds, and much more. The COVID-19 pandemic and related global  advocacy efforts in social justice have demonstrated the destructive  power of exclusion. In the CF community, we have seen the devastating  consequences of delayed diagnosis—or more often, preventable death from  no diagnosis ever being made—in patients of color. And those of us from  ethnic minority backgrounds still struggle for equitable access to  highly effective drug therapies.

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I was  fortunate to get tested for CF as a preschooler and receive some  appropriate treatment in childhood, even though my conclusive diagnosis  came from genetic testing at age 32. As far as I know, I’m still the  only diagnosed case of CF in the U.S. with my specific genetic  profile—not an unusual situation for patients with substantial  Indigenous lineage. Like others in “the final 10 percent,” I can’t take  Trikafta or any other CFTR protein modulator. I feel lucky to thrive as  much as I do on my existing care regimen. And, for the moment, I’m alive  to say that; many of my peers no longer are.

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I don’t  doubt for a moment that I would have gone untested and probably died if I  hadn’t been racially white. Our community is missing the voices and  faces of many Black and Brown people with CF whose families were gaslit  by medical professionals who believed the disease only impacted white  people. And now many of our spaces remain socially inaccessible to  racial and ethnic minority patients who rarely encounter meaningful  representation of our own lived experiences. This has begun to  change—and it will keep changing as we continue to advocate.

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Importantly,  many event platforms now ask registrants if we have specific  accessibility needs for our participation! Even just asking this broad  question and allowing people to respond freely in a comment box does  wonders for inclusion and affirmation. Certainly it gives the event  organizers vital information to use in crafting the virtual environment.  Moreover though, it communicates to participants that our experiences,  needs, and wishes matter. Starting from that baseline of intentionally  valuing one another makes all the difference. It gives us room to work  on the finer details of accommodating each other as best we can.

As  you’re thinking about how to improve accessibility in your own CF  community activities, remember above all never to let the perfect become  the enemy of the good. Make your best faith effort; embrace  opportunities to do better; and share what you learn in the process. It  really is that easy.

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Dr. Alexandra “Xan” Nowakowski is 39 years old and has CF. Xan is a director of CF Roundtable, in addition to being a medical sociologist and public health program  evaluator. They currently serve as an Associate Professor in the  Geriatrics and Behavioral Sciences and Social Medicine departments at  Florida State University College of Medicine. They also founded the  Write Where It Hurts project (www.writewhereithurts.net)  on scholarship engaging lessons from lived experience of illness and  trauma with their spouse, Dr. J Sumerau. You can find their contact  information on page 2.

Editor’s  note: The story below is part one of Rachel’s two-part story depicting  her climb to Everest Base Camp four years ago. Part two will be featured  in the Spring 2023 issue of CF Roundtable.

Rachel  worked in an ICU for two years and, in 2017, quit her job, sold her car,  put all her things in storage, and left to travel the world. She joined  a missions organization called Adventures in Missions and went on a  mission trip called “the world race.” This took her to 11 countries in  11 months (El Salvador, Guatemala, Honduras, Nicaragua, Côte d’Ivoire,  Ghana, Nepal, India, Thailand, Malaysia, and Indonesia). One of those  countries was Nepal—where this adventure took place- at the time she was  26. That year was life-altering for for. She got sick several times and  really questioned God’s goodness and plans. But she had no idea He  would take her to the heights He did. She continued traveling for  another nine months with a different organization called G42, with whom  she spent six months in Spain and three months in Iraq, working in a  refugee camp and underground church.

On  Sunday, February 11, 2018, after eight neverending days, 8,500 feet in  altitude gain, frigid temperatures, and 40 miles trekking through the  tallest mountain range in the world, I finally reached Everest Base  Camp. Did I just write that? A small-town girl from Oregon hiked Everest  Base Camp? A girl who was told at nine years of age that a disease I  could not control would steal my breath from me and that I should make  every effort to protect it. A girl who is the perfect poster child for  how not to live with cystic fibrosis—pursuing a career as a nurse in the  ICU who is continually surrounded by deadly, contagious bacteria every  day, one who is always forgetting to take her medication, one who only  does nebulizer treatments as a last resort, one who is always letting  physical activity take a backseat to her very stressful job, and one who  quit her job to travel to some of the world’s poorest countries for  months on end, filtering her drinking water, living in less than ideal  conditions for even a healthy person—this girl? This girl just hiked to  Everest Base Camp? Living a relatively normal life with CF without  oxygen is rare, but hiking Everest Base Camp with cystic fibrosis and  without oxygen? That. That is a miracle. I am a miracle.

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Ever  since the first week of 2018, when I began to ask the Lord for healing  from cystic fibrosis, I have been waiting expectantly—I really felt like  the Lord was going to do something miraculous in January. So, when  January passed and we traded Africa for Nepal, I began to feel doubt  creep in. The enemy attacked me with condemnation. You could have been  praying today during the time you were playing cards with your team. You  could have read your Bible more this morning. You could have worshipped  with more intentionality tonight. You are asking the Lord for this big  thing of healing, but you are so unworthy to receive it. You won’t ever  be able to walk that closely with God to receive a gift like that. Wow.  How quickly I believed and succumbed to the lies of the enemy without  even realizing it. It wasn’t until I received a message from my dad one  morning that snapped me back into the reality of grace:

“I  was praying this morning and you came to my mind. You talked about your  reluctance to think you could ask God to heal you. We know that healing  is something we can’t earn. It’s a part of God’s grace that He  dispenses according to His will. Keep asking, seeking, and knocking.”

“He rescued me because He delighted in me.”

Psalm 18:19

Believe  it or not, I did not put a lot of thought into what hiking to Everest  Base Camp (EBC) would be like. While still in Africa, our squad received  an email inviting us to hike EBC with our squad leader. We had two days  to make a decision. Five of us said yes. And then we had 17 days to  wrap our minds around what we had signed up to do and buy all our gear  in Kathmandu. All of our traveling for the past seven months had been  in  countries with average temperatures of about 85 degrees, so we  needed to buy a lot more clothes to stay warm.

Day 1:

Flying  into Lukla alone is already one of the most dangerous places to fly into  in the world, and that was just day one! The first day and a half of  the hike was actually very pleasant. I talked with God and prayed and  thanked Him for this incredible adventure. My mind went wild with  thoughts of what crazy things He was going to do over the next 11 days. I  was freshly showered, the sky was clear, the sun was warm, and the  views were breathtaking. We traversed several beautiful suspension  bridges over crystal blue water and the climb was initially a gradual  downhill terrain until we came to this double suspension bridge. At this  point, I now had to climb up to the very top bridge, cross it, and then  proceed to climb 800 vertical meters (or about 2,500 feet) to get to  Namche Bazaar. After eight hours of brutal switchbacks, with the sun  sinking, we finally made it.

Day 2:

On only  day two, I was forced to take a good, long hard look at what I had said  yes to. How quickly my thoughts changed from what awesome things God  might do on this adventure to survival mode.

Day 3:

The next  day was a “rest” or acclimatation day. I was so relieved, but this  feeling did not last long. Acclimatation day is not restful in the  least. You climb up a few hundred meters and then come back down and  stay the night at the same elevation, to make sure your body can adjust  to the altitude and to prevent deadly altitude sickness or, as the  Sherpas call it, “the mountain sickness.” This was the hardest day of  the whole trek for me. It was endless. It was cold, windy, and snowing.  It was vertical. It was so vertical, they couldn’t even make  switchbacks; it was just straight up. The only thing that kept me from  turning back was the promise of an incredible Everest view from the  top—our first chance of the trek to see this legendary mountain. As I  climbed, I prayed with every step. My prayer for the Lord to sustain me  crept back in. It wasn’t a prayer to thrive; it was a prayer just to  survive. When we finally reached the top, my lungs were heavier than I  had ever felt them and they felt like they were on fire. As we looked  toward the north, our guide regretfully told us that we were unable to  see Everest today because there were too many clouds. Out of  desperation, I prayed, “God can I at least just see it?” At this point I  couldn’t imagine any possible way that I was going to make it another  five days and thousands of vertical feet with thinner and thinner air.  Tears came to my eyes as He quickly replied. “No. Because I’m going to  take you to it.”

Day 4:

I woke  up the next morning with an unreal sense of renewed energy. The climb  was a hard one, but, after the day I had before, this one seemed doable.  It was a huge mental challenge, though, as we had to hike down 400  meters before lunch and then go right back up, almost 800 meters to get  to Deboche, where we would spend the night. That night I struggled with  sleep. My chest felt like it was a hundred pounds—it felt crushing. I  assumed it was from working out my diaphragm the past four days, hiking  for six to eight hours each day and struggling to breathe deeper as the  air thinned.

Day 5:

This  morning I woke up just not feeling well at all. I felt like I was sick. I  didn’t have an appetite, which, if you know me at all, is unheard of. I  struggled hiking all day. It was miserable. I had to force my body to  move. When we finally made it to Dingboche, doubt began to creep in  again. Worry and anxiety gripped me as I sat in the tiny tea lodge with a  few other trekkers and teammates that night. One of them had brought a  pulse oximeter with them, which was such a brilliant idea and something I  appreciated as a nurse! I felt a lump in my throat as I put it on my  finger and watched the numbers pop up. Heart rate: 125. SpO2 (blood  oxygen level) 80%. Now, a normal heart rate is anywhere between 60-100  beats per minute. However, a resting heart rate should be closer to  60-80 beats per minute. These vitals were taken after I had been sitting  and resting for several hours. Blood oxygen level should be higher than  94%. A Sherpa informed me that normal levels at 4,400 meters were 85%,  which blew my mind as nurse. This slightly eased my anxiety, but not for  long. The rest of my teammates’ vitals were heart rates ranging between  70-100 and oxygen levels of 88-95%. That night, as I lay in my triple  layers of clothing, hat, gloves, sleeping bag liner, -10  degree-thermal-rating down sleeping bag, and two giant comforters piled  on top, I broke down in fear. I knew now why I had struggled to sleep  the night before and why my chest was so heavy—it wasn’t from my  diaphragm: It was heart pain. My heart rate was way too high and I had  no idea how to slow it down. I was lying in bed for crying out loud and  my heart was ticking away like I had just gotten done running sprints.

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The  enemy shrouded me in fear. I began thinking about how many days I could  stay in this cold tea hut alone and wait for my teammates to come back  for me after getting to base camp. I knew if my heart rate was this high  just lying here, there was no way I could possibly hike. The enemy  sneered at me. How could I have ever thought I could do something like  this, with a lung disease? How foolish I was. I peeled myself out of all  my layers and desperately walked toward my other teammate’s rooms, to  ask for prayer. I lightly tapped on their doors. Nothing. They went to  bed over an hour ago and are warm in their beds. How could you possibly  think or ask them to get out in the freezing cold just to pray for you?  They need their rest. This is foolish. This is not their fight. This is  yours. You are alone. For as long as I could stand, I walked outside and  stared up into the black starry sky through the frost of my own breath  as I cried and pleaded for God to help me. He doesn’t hear you. What do  you want? A miracle? He already gave you Everest Base Camp. You chose  this, knowing your physical limitations. You want more now? You’ve made  it this far, that is already far more than you deserve. I went to bed  defeated. Under a crushing darkness of the enemy’s lies.

But nothing could have prepared me for what Day 6 would hold.

Rachel’s story will be continued in the upcoming Spring 2023 issue.

Rachel  Johnston is 31 years old and has CF. She currently lives in Oregon with  her husband. She has traveled all over the world as part of various  mission trips. She came back to the United States in June 2019 and got  married in November 2021.

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 adults in the CF community who  are interested in pursuing a degree in the arts.

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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.

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 

By 2040, Over 70% Of CF Patients In US Will Be Adults

According  to a study, with continued advances in treatment, more people with  cystic fibrosis (CF) are surviving well into adulthood and by 2040,  nearly three-quarters of these patients in the U.S. will be adults and  more than one-third of these adults will have minimal problems with lung  function — specifically a score of 90% or higher on percent predicted  forced expiratory volume in one second (FEV1pp). In the early 1990s, the  expected lifespan for someone born with CF was around 30 years. Now,  it’s closer to 50. Today, more than half (57%) of people living with CF  are 18 or older. It is likely that this growing, aging adult CF  population will be more medically complex because of non-lung and  age-related comorbidities, so coordination of care between CF care  teams, subspecialists, and transplant centers will remain critical.

https://tinyurl.com/492tz8kd

Contemporary Cystic Fibrosis Incidence Rates In Canada And The United States

The  availability of new diagnostic algorithms for cystic fibrosis (CF),  changing population demographics and programs that impact family  planning decisions can influence incidence rates. Thus, previously  reported incidence rates in Canada and the United States (US) may be  outdated. The objectives of this study were to estimate contemporary CF  incidence rates in Canada and the US and to determine if the incidence  rate has changed over time. Contemporary CF incidence rates suggest CF  incidence is lower than previously reported and varies widely within  North America. This information is important for resource planning and  for tracking how programs (e.g., genetic counseling, modulator  availability etc.) may impact the incidence of CF moving forward.

https://tinyurl.com/4c5n3swu

AND

https://tinyurl.com/mr4as9nx

Current Prices Versus Minimum Costs Of Production For CFTR Modulators

This  study aims to estimate the minimum costs of production of CFTR  modulators, assuming robust generic competition, and to compare them  with current list prices to evaluate the feasibility of increased global  access to treatment. Minimum costs of production for CFTR modulators  were estimated via an algorithm validated in previous literature and  identification of cost-limiting key starting materials from published  routes of chemical synthesis. Costs of production for  elexacaftor/tezacaftor/ivacaftor are estimated at $5,676 [$4,628-6,723]  per year, over 90% lower than the US list price. Total cost of triple  therapy for all eligible diagnosed CF patients worldwide would be $489  million per year. Comparatively, the annual cost at US list price would  be $31.2 billion. Thus, elexacaftor/tezacaftor/ivacaftor could be  produced via generic companies for a fraction of the list price. The  current pricing model restricts access to the best available therapy,  thereby exacerbating existing inequalities in CF care.

https://tinyurl.com/2fkwxzv2

The Impact Of Elexacaftor/Ivacaftor/Tezacaftor On Cystic Fibrosis Patients Who Acquire COVID-19 Infection

The  combination of medication containing elexacaftor, ivacaftor, and  tezacaftor (EIT) has dramatically impacted the treatment and prognosis  for patients with cystic fibrosis (CF). Lung function, weight, and  self-reported quality of life have improved for many of these patients,  but little is known about whether this treatment will have a beneficial  effect in preventing morbidity and/or mortality from respiratory  infections such as COVID-19. This study shows that cystic fibrosis  patients who were already receiving treatment with the combination of  elexacaftor/ivacaftor/tezacaftor had a significantly decreased risk of  developing acute respiratory failure after becoming infected with  COVID-19. This study also showed a 50% increased likelihood of patients  who were not on treatment with EIT developing ventilator dependence, but  the low numbers of patients likely contributed to that difference not  achieving statistical significance. By improving chloride ion flow  through the CFTR channel, EIT substantially improves lung function and  decreases respiratory morbidity in CF patients. This improved lung  function results in improved tolerance to the pulmonary insult caused by  COVID-19 infection. Overall, this data makes a compelling argument to  consider this combination of medications for any CF patient who is a  candidate.

https://tinyurl.com/yckj48pd

VX-522, For CF Patients Who Can’t Use Modulators, To Enter Trial

Vertex  Pharmaceuticals is launching a clinical trial to test VX-522, its  inhaled messenger RNA (mRNA) therapy for lung disease in cystic fibrosis  (CF) patients who are not eligible for treatment with an existing CFTR  modulator. The study will assess VX-522’s safety and tolerability at  single ascending doses in adults with CF, ages 18 and older. As an  inhaled treatment, VX-522 is intended to deliver full-length CFTR mRNA  directly to cells in the lungs. The mRNA is enclosed in lipid (fat)  nanoparticles and, once inside the cells, is expected to give rise to  the production of a working CFTR protein.

https://tinyurl.com/4aettex3

Dose Adjustments Of Elexacaftor/Tezacaftor/Ivacaftor In Response To Mental Health Side Effects In Adults With Cystic Fibrosis

In this  case series, the authors report the use of sweat chloride as an indirect  biomarker to guide dose adjustments of elexacafor/tezacaftor/ivacaftor  (ELX/TEZ/IVA) in people experiencing significant mental health problems  (depression, anxiety, brain fog and insomnia) after starting treatment.  The researchers hypothesised that these adverse events (AEs) are a  result of a combination of predisposition to anxiety and/or depression,  the presence of a premorbid mental health condition, individual  variation in elexacaftor metabolism and increased systemic CFTR  expression. They found that dose adjustment of ELX/TEZ/IVA was  associated with improvement in mental health adverse events without  significant clinical deterioration.

https://tinyurl.com/4wdpmwv7

Elexacaftor-Tezacaftor-Ivacaftor Improve Gastro-Oesophageal Reflux And Sinonasal Symptoms In Advanced Cystic Fibrosis

Upper  gastrointestinal and upper airway disease are common in cystic fibrosis  (CF) and may contribute to lower airway infection and inflammation. In a  longitudinal cohort study of 32 patients with advanced CF lung disease  starting elexacaftor-tezacaftor-ivacaftor, the reflux symptom index  score fell from a pre-treatment median (IQR) of 15 to 5 (2.8-7.3), the  Hull airway reflux score fell from a median of 26.5 to 7.5, and the  sinonasal outcome score from a median of 36.5 to 20 at 6 months on  treatment. Mean FEV1% predicted rose by 9.2 points, the median  respiratory domain score of the CF Questionnaire-Revised rose by 27.8  points and mean body mass index rose by 2.6 kg/m. In addition to  improving lung function and weight, CFTR modulators improve upper airway  and gastro-oesophageal reflux symptoms in advanced CF.

https://tinyurl.com/casmjape

Elexacaftor/tezacaftor/ivacaftor And Gastrointestinal Outcomes In Cystic Fibrosis: Report Of Promise-GI

Elexacaftor/tezacaftor/ivacaftor  (ETI) improves pulmonary disease in people with cystic fibrosis (PwCF),  but its effect on gastrointestinal symptoms is not clear.  Gastrointestinal symptoms, evaluated by validated questionnaires:  Patient Assessment of Upper Gastrointestinal Disorders-Symptom  (PAGI-SYM), Patient Assessment of Constipation-Symptom (PAC-SYM),  Patient Assessment of Constipation-Quality of Life (PAC-QOL)), fecal  calprotectin, steatocrit and elastase-1 were measured before and 6  months after ETI initiation.  After 6 months of ETI, fecal markers of  inflammation decreased. Gastrointestinal symptoms improved, but the  effect size was small. Pancreatic insufficiency did not improve.

https://tinyurl.com/ufdxxuar

Trikafta Helps To Resolve Inflammation In CF: Study

Treatment  with the triple-combination therapy Trikafta  (elexacaftor/tezacaftor/ivacaftor) reduces inflammation and promotes  lung tissue repair in cystic fibrosis (CF). Results showed that levels  of interleukin-6 (IL-6), a molecule implicated in inflammation,  decreased significantly within three months of starting on Symdeko  (tezacaftor/ivacaftor). Levels of IL-6 and C-reactive protein (CRP), an  inflammation marker, were significantly reduced at one month with  Trikafta, which was sustained through one year of treatment. Results at  one year of Trikafta therapy showed significant reductions in the levels  of IL-20 and MMP-10, both implicated in wound healing. Levels of  calprotectin — a risk marker of pulmonary exacerbations decreased  significantly within one year of starting treatment with Trikafta. A  trend toward calprotectin decrease was also seen with Symdeko within one  year. Radiological improvements were visible in all those who underwent  CT scanning following a year of Trikafta.

https://tinyurl.com/3p7fz3uh

Magnetic  Resonance Imaging Detects Improvements Of Pulmonary And Paranasal Sinus  Abnormalities In Response To Elexacaftor/Tezacaftor/Ivacaftor Therapy  In Adults With Cystic Fibrosis

Effects  of Elexacaftor/Tezacaftor/Ivacaftor (ETI) on structural and functional  lung abnormalities and chronic rhinosinusitis have not been studied by  imaging. Researchers found that MRI detects improvements of chest MRI  and chronic rhinosinusitis (CRS-MRI) scores in adult CF patients who  first received ETI, demonstrating reversibility of structural lung and  paranasal sinus abnormalities in patients with established disease.

https://tinyurl.com/bdffe9h3

Trikafta Aids Quality Of Life With CF In Multitude Of Ways: Study

Researchers  analyzed the 11 non-respiratory domains (non-RDs) of the CF  Questionnaire–Revised (CFQ-R), which assess general health-related  quality of life (i.e., Physical Functioning, Role Functioning, Vitality,  Health Perceptions, Emotional Functioning, and Social Functioning) and  quality of life impacted by CF (i.e., Body Image, Eating Problems,  Treatment Burden, Weight, and Digestive Symptoms), for participants in  two Phase 3 trials. ELX/TEZ/IVA treatment led to higher scores in all  CFQ-R non-RDs, with improvements in most domains compared with control  treatments. These findings demonstrate that Trikafta improves a range of  CF-specific symptoms and general functioning and well-being.

Orkambi’s Benefits In The Real World Go Beyond Lung Function: Study

For a  small group of people with cystic fibrosis (CF), one year of treatment  with Orkambi (ivacaftor/lumacaftor) improved bone health and stabilized  pancreatic function, nutritional status, reproductive hormone levels,  and lung function.

https://tinyurl.com/26bfunuy

Orkambi Found To Improve Exercise Endurance In 3 CF Adults In Study

Treatment  with Orkambi (lumacaftor/ivacaftor) for six months was found to improve  exercise endurance in three adults with cystic fibrosis (CF). Moreover,  by the end of the study, all three men experienced less leg discomfort  and less dyspnea, or shortness of breath. After six months of treatment,  exercise endurance time increased for all three patients — by 87%, 52%,  and 23% among the men. Inspiratory capacity also improved for all  three, and oxygen uptake increased in two patients. Additionally, all  patients reported less breathing discomfort and leg fatigue.

https://tinyurl.com/4jxuz2y6

Discontinuation  Versus Continuation Of Hypertonic Saline Or Dornase Alfa In Modulator  Treated People With Cystic Fibrosis (SIMPLIFY): Results From Two  Parallel, Multicentre, Open-Label, Randomised, Controlled,  Non-Inferiority Trials

The  SIMPLIFY study aimed to assess the effects of discontinuing nebulised  hypertonic saline or dornase alfa in individuals using the CFTR  modulator elexacaftor plus tezacaftor plus ivacaftor (ETI). Participants  on both hypertonic saline and dornase alfa were randomly assigned to  one of the two trials, and those on a single therapy were assigned to  the applicable trial. All participants were then randomly assigned 1:1  to continue or discontinue therapy for 6 weeks. For participants  randomly assigned to continue their therapy during a given trial, this  therapy was instructed to be taken at least once daily according to each  participant’s pre-existing, clinically prescribed regimen. The primary  objective for each trial was to determine whether discontinuing was  non-inferior to continuing, measured by the 6-week change in ppFEV1.  Participants across both trials had an average ppFEV1 of 96.9%.  Discontinuing treatment was non-inferior to continuing treatment with  respect to the absolute 6-week change in ppFEV1. The researchers  established that in individuals with cystic fibrosis on ETI with  relatively well preserved pulmonary function, discontinuing daily  hypertonic saline or dornase alfa for 6 weeks did not result in  clinically meaningful differences in pulmonary function when compared  with continuing treatment.

https://tinyurl.com/2n63sl4y

Onset Of  Systemic Arterial Hypertension After Initiation Of  Elexacaftor/Tezacaftor/Ivacaftor In Adults With Cystic Fibrosis: A Case  Series

Initiation  with elexacaftor/tezacaftor/ivacaftor treatment might be associated  with acute onset of systemic arterial hypertension. Careful monitoring  of cardiovascular paramenters is recommended in patients starting  elexacaftor/tezacaftor/ivacaftor treatment. Referrals should be made to  cardiologists when patients present elevated blood pressure after the  start with elexacaftor/tezacaftor/ivacaftor.

https://tinyurl.com/2fvm5xw5

Heart Failure Common In Cystic Fibrosis Patients

Roughly  one in 10 adults with cystic fibrosis also had a diagnosis of heart  failure. Among the roughly 15,000 cystic fibrosis patients, acute  myocardial infarction (MI) and atrial fibrillation (Afib) were the other  most commonly identified cardiac disorders, each present in about one  in 20 patients. As patients have grown older, cardiac sequelae,  including pulmonary hypertension, right heart dysfunction, and  cardiomyopathies have increasingly been linked to cystic fibrosis.  Cystic fibrosis might affect the cardiovascular system directly, through  CFTR gene dysfunction in the heart and coronary arteries, or indirectly  through hypoxia, chronic inflammation, diabetes, and pulmonary  hypertension.

https://tinyurl.com/mryev4ea

Acne May Be Side Effect Of Treating CF With Trikafta: Case Study

Researchers  describe 19 patients with acne appearing or getting worse after  starting Trikafta. All were eligible for Trikafta treatment. Nine had  acne before starting treatment and 18 reported acne within eight months  of initiating therapy, including nine who reported it within the first  three months and four who reported it at six months or later. The face  was the most commonly affected part of the body, with some patients  referring also to involvement of the chest and back. No patient  discontinued Trikafta due to acne as the therapy’s benefits in lung  function outweighed the risk. How Trikafta results in acne is unknown.  The researchers hypothesized that it might be related to alterations in  the salt content of sweat, due to changes in the skin microbiome. This  would be similar to changes in the lung microbiome observed in patients  treated with Trikafta. There is little research in CF patients on the  skin microbiome, the collection of microbes, such as fungi, bacteria,  and viruses, that naturally live in the body. An alternate hypothesis  could be that the rapid decrease in sweat chloride itself precipitates  an inflammatory response. The researchers emphasized that further  research is needed to obtain a better understanding of various  dermatologic changes that may occur with initiation of Trikafta.

https://tinyurl.com/yckmw7cx

Oral Antibiotics Found To Be More Effective In Treating Cystic Fibrosis

A recent  study analyzed the effectiveness of traditional antibiotic therapy as  compared to intravenous antibiotic therapy in treating Pseudomonas  aeruginosa infection among cystic fibrosis patients. This study was a  10-year trial. During the study, 137 patients were treated using  intravenous antibiotics while 149 patients were given oral antibiotics.  After medicine administration, 44% of patients given intravenous  antibiotics recovered while a 52% recovery rate was reported in the case  of traditional antibiotics. After the eradication treatment, fewer  patients got hospitalized in case of intravenous treatment as compared  to oral treatment. Not only were traditional antibiotics more effective  than intravenous ones but they also cost less.

https://tinyurl.com/2p8hwkcx 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.