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.
There are many things that you can do while waiting for donor lungs to become available. Preparing for a lung transplant includes maintaining your health, performing your routine cystic fibrosis care, and being ready to respond when donor lungs are available.
Deciding when to get listed for a lung transplant can be overwhelming. You have to be physically and emotionally ready for the commitment before you decide to list. There are several factors that determine when you get new lungs. First, of course, is how urgently you need lungs, which is represented by your lung allocation score (LAS). Next, you have to be physically compatible with the donor lungs, which includes matching your blood and tissue types. In addition, the donor lungs need to be the right size to fit into your chest. Once you're listed, there is no way to predict when the transplant call will come. It could be as soon as the next day or you could wait years.
In addition, many people with cystic fibrosis adapt to their physical decline. It becomes a “new normal.” Thus, they feel that they aren't ready to be listed because they “don't feel that bad.”
Your CF care team can guide you to a transplant center with expertise in cases like yours so that you can be fully informed prior to making your decision.
It is critical to discuss your life goals with your care team and learn about what you can expect throughout the lung transplantation process. Doing so will enable the team to recommend the best plan for you. The more they know about what you want, the better they'll be able to help you weigh the benefits and risks that matter most to you.
Jeremy Weyandt, an adult with cystic fibrosis, describes his journey to getting a transplant.
Lung transplantation can extend and improve your life, but it is not without risks. It is possible that none of these complications will affect you; it is also possible that you will experience more than one complication.
It is essential to the success of your transplant that the lungs you receive are closely matched to your body. Finding lungs that are a good match for you is not as simple as matching your blood type. The tissue that your donor's lungs are made of must also be compatible with the antibodies you have circulating in your blood. Additionally, the donor's lungs must be the right size to fit into your chest cavity.
There are four blood types: A, B, AB, and O. Every person has one type of blood. Not all blood types are compatible with another. If you get donor lungs from someone whose blood type is not compatible with yours, your immune system will recognize the lungs as foreign and try to destroy them.
Your body uses human leukocyte antigens (HLAs) as one way of identifying foreign proteins. HLAs are proteins that are located on the surfaces of your white blood cells and other tissues in your body. People who have been exposed to someone's HLA (for example, through pregnancy, blood transfusion, or prior transplant) can develop antibodies against those HLAs. Blood tests are done during your evaluation to determine your HLA type and whether you have HLA antibodies. If you have HLA antibodies, your donor's HLA will have to be screened before lungs can be accepted for you.
A lung transplant is not without risks, but it offers you a chance you may not otherwise have to improve your health and extend your life. If the transplant is successful, you will have a healthy set of lungs that are not affected by cystic fibrosis. It is important to remember, however, that a lung transplant is not a cure and you still will have CF in other parts of your body. You still will need to continue your CF care routine for the affected areas.
Doctors and researchers are always working to improve surgical techniques, immunosuppressive drugs, post-transplant care procedures, and the methods used to preserve donor organs until they can be transplanted. Advances in these areas have helped to increase the number of successful transplants done every year, as well as the quality of life of transplant recipients.
In this clip, from 18:12-18:30, Joseph Pilewski, MD, discusses that among adults with CF, more than 80 percent of lung transplant recipients are still alive after one year, and more than 50 percent are alive after nine years.
“So you see this drop in survival over the first 5-10 years and a little bit of a flattening out of the curve as you go further out. But it clearly demonstrates that the survival isn't perfect, and we want to be careful not to have patients get transplanted too soon because this clearly has some significant risk of death in the first several years after transplant.” -- Joseph Pilewski, MD
Many people who have had successful transplants report being able to breathe more easily and resume doing things they enjoyed before their lung function declined. Some people have been able to participate in challenging physical activities after transplant.
“The past year has been rough, but exciting and full of huge changes. I used to be on an oxygen leash 24/7, nebulized breathing treatments every four hours, and IV antibiotics every three weeks. Now, I am IV-less, rarely cough, and no longer use supplemental oxygen.” -- Tiffany Rich, adult with CF, from the CF Community Blog
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