Learn about cystic fibrosis, a genetic disorder that affects the lungs, pancreas, and other organs, and how to treat and live with this chronic disease.
CF is a rare genetic disease found in about 30,000 people in the U.S. If you have CF or are considering testing for it, knowing about the role of genetics in CF can help you make informed decisions about your health care.
If you or your child has just been diagnosed with cystic fibrosis, or your doctor has recommended testing for CF, you may have many questions.
Diagnosing CF is a multistep process. A complete diagnostic evaluation should include a newborn screening, a sweat chloride test, a genetic or carrier test, and a clinical evaluation at a CF Foundation-accredited care center.
Raising a child with cystic fibrosis can bring up many questions because CF affects many aspects of your child’s life. Here you’ll find resources to help you manage your child’s daily needs and find the best possible CF care.
Living with cystic fibrosis comes with many challenges, including medical, social, and financial. By learning more about how you can manage your disease every day, you can ultimately help find a balance between your busy lifestyle and your CF care.
People with CF are living longer, healthier lives than ever before. As an adult with CF, you may reach key milestones you might not have considered. Planning for these life events requires careful thought as you make decisions that may impact your life.
People with cystic fibrosis are living longer and more fulfilling lives, thanks in part to specialized CF care and a range of treatment options.
Cystic Fibrosis Foundation-accredited care centers provide expert care and specialized disease management to people living with cystic fibrosis.
We provide funding for and accredit more than 120 care centers and 53 affiliate programs nationwide. The high quality of specialized care available throughout the care center network has led to the improved length and quality of life for people with CF.
The Cystic Fibrosis Foundation provides standard care guidelines based on the latest research, medical evidence, and consultation with experts on best practices.
As a clinician, you’re critical in helping people with CF maintain their quality of life. We’re committed to helping you partner with patients and their families by providing resources you can use to improve and continue to provide high-quality care.
As part of the Cystic Fibrosis Foundation's mission to help improve the lives of people living with cystic fibrosis, the PSDC initiative taps the CF community to inform key efforts to support the management of daily care.
Your cystic fibrosis care team includes a group of CF health care professionals who partner with you to provide specialized, comprehensive CF care.
Many people living with cystic fibrosis and their families face complicated issues related to getting the care they need. Cystic Fibrosis Foundation Compass makes sure that no one has to do it alone.
CF Foundation Compass is a service that helps people with CF and their families with navigating insurance options, connecting to legal information and experts, finding available financial resources, and tackling other life issues.
CF care team members are paramount in providing highly specialized care to people living with CF. CF Foundation Compass can help by serving as a strategic ally for care teams, so team members can focus on their patients’ care.
CF Foundation Compass can help you navigate insurance, financial, legal, and other issues you are facing. Use this online form to start your conversation with a Compass case manager today.
The Cystic Fibrosis Foundation is the world’s leader in the search for a cure for CF and supports a broad range of research initiatives to tackle the disease from all angles.
The CF Foundation offers a number of resources for learning about clinical trials and treatments that are being developed to improve the treatment of cystic fibrosis.
Our understanding of CF continues to evolve as scientists study what causes the disease and how it affects the body. These insights drive the development of new and better treatments and bring us one step closer to a cure.
Researchers, supported by the CF Foundation, have made tremendous advances to improve the health and quality of life of people with CF. We are committed to providing the tools and resources you need to continuously build upon this work.
The transition from my pediatric care team to my adult care team was a bit bumpy, but, as we got to know each other, our relationship has grown strong.
February 8, 2018
A Mountain for Avalyn
Participating in a Clinical Trial Is Empowering
The first memories I have of cystic fibrosis were of my mom telling me, “pack some books for tomorrow, we have to go to Charleston.” We would get up early in the morning, and I would sleep on the way, and would wake up as we were pulling into the parking garage, the only one I had ever seen. Slowly, I realized that we were at a hospital.
Those first pediatricians who saw me are long gone, and probably came with the bricks when they built the medical university. But, they were very passionate and caring physicians. Bedside manner seemed to be a top priority. True CF science was more science fiction at that point. Pancreatic enzymes were really all there was in terms of drugs specifically for CF patients. Everything else available was trying to treat symptoms and palliative care, for lack of a better term.
The last pediatrician that followed me was middle-aged and had a truly warm disposition. He was more gastrointestinally oriented, which worked out well for me since he was the one who discovered my liver disease during my adolescence and got it under control. It is still managed with the medication he recommended more than 20 years ago. He was old school: long white coat, comfortable shoes, khaki pants, and a tie. Imagine Detective Columbo with a white coat. One of the purest people I have ever encountered in the medical field.
One day in the pediatric clinic during the early 90s, he told me that the new adult CF doctor was coming to meet some of the older pediatric patients who would be transitioning soon to adult care. In walks my new CF doctor, fresh on the scene. I immediately noticed the sharp contrast in appearance and projection to my pediatrician. He was a young, spiffy sort, with dark hair and a flair for really nice clothes and loafers. I already didn't like him. Sometimes you don't recognize greatness right away.
Before getting into this any further, I must explain that when it came to daily lung treatments, I was totally noncompliant. My lung function was close to 100 percent, I never had bronchitis or pneumonia, and I was staring down both barrels of a liver transplant, so to me it was a complete waste of time. That being said, there wasn't much available at that time for airway clearance. The Flutter™ had literally just come out, and it did nothing for me. Even the active cycle breathing technique, which was as cutting edge as Orkambi® is today, did not help me. Had my lungs been more involved at the time, I'm sure my parents would have been doing manual chest percussion on me and making me do the other lung treatments.
My new doctor sat down with me and asked how I was doing. I told him fine, to which he replied, “Are you telling the truth?” Then, after a conversation where he actually explained to me -- for the first time -- how the mucus in CF affects the lungs and covers the cilia, etc., he dropped the hammer on me.
“You're doing okay right now, but I am here to tell you that -- eventually -- you WILL end up in the hospital due to lung infection, and you WILL be on IV antibiotics.” This was not the bedside manner to which I was accustomed. It was also the first time I had ever been subjected to the harsh realities of CF.
He continued to visit me on the pediatric side for a quick couple of years, and time came for me to transition to the adult clinic when I was 18 years old. He emphasized that I was an adult now, and asked that my parents remain in the waiting room instead of going into the exam room with me. As harsh as this may sound, I think it is a good practice to draw a line and make patients take ownership of their care because -- past a certain age -- no one can make someone compliant. My mom was very reluctant about this, but she understood. What finally made me compliant was coughing up some mucus with traces of blood in it. Since then, I have been 100 percent compliant with every single treatment recommendation.
I don't know exactly when or how it happened, but after a couple of years, my doctor and I grew used to each other, and he and his nurse coordinator (who has now been with him more than 20 years) became the north star of my health. I have to see other specialists for other things like my liver, but it is all coordinated through my CF care center.
I think my doctor grew to appreciate my compliance with his suggestions, and saw that when he gave me an inch, I would not take a mile. I began to understand his vision and what he was building, and that he was not just a doctor, but an internationally known scientist who took an interest in the patient, not just the illness.
Through the years we have grown to know each other as people, and that has caused our doctor-patient relationship to get stronger. I know that when I email him, I will get a thorough and thoughtful response. I hear of patients who don't have that kind of relationship with their providers, or have to relocate and change clinics, and I feel for them. Worse yet, I know there are some CF patients who don't even go to a CF Foundation-accredited care center, even though they are able.
Change is hard. Growing up is harder. CF is hardest. Sometimes the combination of the three is overwhelming, and it isn't very smooth. But, don't lose hope. Sometimes what may start off as a shock -- or clash of personalities -- will eventually sync. The dunking of ice water that once shook us can also mean victory.
Adult with CF
Shay was diagnosed with cystic fibrosis at 5 months old in 1982. Originally from Sumter, SC, he currently lives in the Charleston area close to the Medical University of South Carolina (MUSC), the facility that has cared for his CF since diagnosis. He holds a master’s degree in Health Administration from MUSC and currently works for a national managed care organization. Writing has been a hobby since childhood, but Shay only recently began blogging about CF. He and his wife, Stacy, have 2 dachshunds and enjoy traveling. He also cofounded the surfing-related CF nonprofit, The 3rd Street Society, in 2017 with his best friend, who is an avid surfer. Follow Shay on Instagram @cf_icare.
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This site contains general information about cystic fibrosis, as well as personal insight from the CF community. Opinions and experiences shared by members of our community, including but not limited to people with CF and their families, belong solely to the blog post author and do not represent those of the Cystic Fibrosis Foundation, unless explicitly stated. In addition, the site is not intended as a substitute for treatment advice from a medical professional. Consult your doctor before making any changes to your treatment.
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