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.
Having already been underwhelmed by Orkambi® and Symdeko®, I was skeptical about whether the triple-combination therapy would live up to the hype. However, after a month of being on Trikafta®, I feel improvements in my lung function, weight, and overall energy.
January 23, 2020
Learning That CF Isn’t My Fault
My Rocky Road to Lung Transplant
When Trikafta was approved in late October 2019, I was overcome with a variety of emotions: anxiety, excitement, and skepticism. I spent many days pondering numerous questions in my already overloaded brain: “Is this drug for real? What exactly does it do? Is it as good as some people claim it to be? Will my insurance cover the cost? Will I see positive results? What does this mean for my future?” The very next morning after its approval, I was in contact with my CF clinic to receive Trikafta.
On a daily basis, I was going on an internet and social media search frenzy: I was seeking out CF patients from the clinic trials to ask them about it; I was reading news articles; and I was browsing YouTube videos. I found myself devouring anything with Trikafta or “triple combo” mentioned. I even found myself putting my multi-lingual skills to the test by reading articles relating to the medical breakthrough in Italian, French, and Spanish. Although all these articles and clinical trial data touted this as a great medical breakthrough for cystic fibrosis, there was still a part of me doubting its achievements.
When Kalydeco® was approved in 2012, I was overjoyed for those who could benefit from it. Although my genetic mutation wasn't approved for Kalydeco, I knew this was a major stepping stone -- not only for cystic fibrosis research but also for modern medicine. I did have moments of jealousy that I couldn't benefit from Kalydeco, but I knew my day would come.
A few years later during the summer of 2015, I received a phone call from my CF clinic instructing me to come in immediately to fill out the paperwork for the newly approved, Orkambi®. I quickly researched the new medication and was excited to begin this journey. Was Orkambi the drug I had been waiting for? Was this a cure? Within a week, Orkambi was in my hands.
When I started Orkambi, I experienced two weeks of exhaustion and cold-like symptoms. My breathing seemed to improve, but I noted no changes in my appetite. I was disappointed when I went to my first clinic visit after starting Orkambi and noted a minimal increase in my pulmonary function and weight. Was my body rejecting Orkambi? Did I trick myself into thinking Orkambi was more than it was?
A few short years later, Symdeko® was newly approved, and I promptly switched. This time, I had fewer initial side effects but noted no change in my body's reaction. If anything, Symdeko (like Orkambi) was keeping me at my baseline.
Over the years, I've heard rumblings of this “triple-combination” therapy. Going off my prior experiences with Orkambi and Symdeko, I was doubtful it was truly different.
Around Halloween, I received my approval for Trikafta and figured it was time to unleash my inner superhero powers and vanquish my doubts. Despite my mix of emotions, I couldn't help but get excited to start this new journey.
Fast forward, I am overjoyed to announce that Trikafta has proven me wrong, shattering both my pessimistic attitude and expectations.
I received Trikafta almost four weeks after its FDA approval. It was a week before Thanksgiving when I returned home from work to see it waiting for me on my kitchen counter. The next morning at 6 a.m., I began another chapter of my life.
Within hours, I wasn't sure if it was my mind playing tricks on me or if what was happening to me was real. I was coughing more, bringing stuff up that looked like it came from a sci-fi movie. I felt like I had the beginning stages of a flu, but I pushed through and finished the last three workdays of the week.
That weekend I found myself getting air into parts of my lungs that I haven't felt since I was a child.
There are no words to describe the feeling of taking deep breaths and having it travel through your entire body. My cough was quickly subsiding as I began coughing up white, clear, saliva-looking mucus.
After consulting family, my CF clinic, and other Trikafta users, I was shocked to learn this is what normal mucus resembles. It wasn't my mind playing tricks on me; the medication was apparently doing its job.
Suddenly, my cough vanished, and I was met with an overpowering appetite. I was devouring any food in sight, having four meals a day and gaining weight. The leftovers from Thanksgiving didn't stand a chance with me always on the prowl. It was enjoyable to have a family holiday and not feel nauseous from hacking; for once, I didn't have to remove myself from the table to have a coughing fit and catch my breath.
As I progressed through the month of December, I honestly felt like a normal human being. I had energy; I was eating and gaining weight; I never coughed; my mental health was improving; and for once, I was looking forward to my next CF clinic visit. The changes my body was going through were noticeable to my family and friends -- for once, I felt like I was really living my life.
A little over a month since starting Trikafta, it was the real moment of truth … my first CF clinic visit of the new year. Although I was feeling amazing, I knew my mind wouldn't rest until my initial weigh-in and pulmonary function exam. During my visit I was informed that I had gained more than 15 pounds and my pulmonary function was up more than 15 points. My FEV1 was at a level I have not seen since my teenage years. Never in my wildest dreams would I expect to see a number in the mid-80s, but there it was. This was not a dream; this was reality.
While I was excited to share the great news about Trikafta's impact on my life over social media, I suddenly found myself feeling melancholy. On various online platforms, I was coming across CF patients who couldn't receive Trikafta whether it was due to their genetic mutation, their insurance coverage, or they were not in the United States. I also began thinking about all the CF patients who aren't here to partake in the joy of this new drug. One could call what I was feeling a variation of “survivor's guilt.” I was blessed with this life-changing medication, while others are struggling.
Hopefully soon, everyone who is eligible for Trikafta will receive it. For the CF patients who are not eligible for Trikafta, you are not forgotten! We all will not stop until there are options for the entire CF population; we are all in this together.
I'd like to take the opportunity to thank Vertex Pharmaceuticals, the Cystic Fibrosis Foundation, my CF clinic, and the fundraising volunteers and donors who all had a hand in making this a reality. I'd also like to thank my parents, especially my mother, who has had a hand in helping me maintain my health throughout my life!
Adult with CF
Vincent is a New York native who was first diagnosed with CF when he was 6 months old. In addition to speaking over five languages, Vincent earned his bachelor's degree in Marketing from Hofstra University and a master's degree in Accounting from Molloy College. When he isn't working as an accountant, Vincent enjoys spending time with family and friends, working out, binging on his favorite TV shows, and cheering for the New York Rangers. Follow @vincenz99 on Instagram and @vincenzo99 on Twitter.
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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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