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.
A lung transplant may be a treatment option when your diseased lungs can no longer support your body's needs.
Lung transplantation is a surgical option for people with cystic fibrosis who have advanced lung disease. During the operation, diseased lungs are replaced with healthy ones gifted from a deceased organ donor.
Median survival for people with CF who have had a transplant has improved. According to the 2018 International Society for Heart and Lung Transplantation Registry report, the median survival for adults has improved from 5.5 years for those transplanted
between 1990 and 1998 to 9.5 years for those transplanted between 1999 and 2016. This means that half of individuals transplanted between 1999 and 2016 were alive 9.5 years after transplant. Different factors potentially affect survival in children
with CF for whom the median survival was 5.4 years for those who received transplants between 1999 and 20161.
Lung transplantation can extend and improve your quality of life, but it involves an extensive evaluation and dedication to living the lifestyle required to keep your new lungs healthy.
The decision to pursue a lung transplant also requires great commitment from your family and friends who are part of your social support system. This is why knowing
what to expect will help you and your loved ones plan ahead to determine if transplant is a treatment option for you.
Download this graphic to help you start a discussion about the lung transplant process with your family and care team.
Lung transplant a multiphase process:
Talking about transplant early -- discussing transplant before you need to be referred so you can learn, plan, and work on improving your chances for a successful transplant
Being referred -- visiting with the transplant team so they get to know you as a person, you learn about them as partners in your care, and what to expect during an evaluation, surgery, and recovery at their center
Getting evaluated -- consulting with the transplant team and having tests done to assess your ability to successfully undergo transplant surgery
Having your case reviewed -- the transplant committee will recommend that you either be listed, have additional tests, live as usual until your lungs get worse, work on your health or social support, or not pursue a
transplant at their center, at which point your care team can help you get opinions from other transplant centers
Ready to be listed -- if the team recommends that you be listed for a transplant, they will help you decide when to get on the UNOS waiting list and manage your health so you have the best chance for a good recovery from
Receiving new lungs -- you may need to move closer to the transplant center so that once donor lungs are available, you’ll be able to come quickly to the hospital for surgery to replace your diseased lungs with healthy
Living with new lungs – keeping your new lungs healthy by meeting with your care teams, exercising, and managing a new care plan that includes medications to prevent rejection and infections, and reducing the risk of
germs, including germs from others with CF
There are more people who need new lungs than there are available donor lungs. So, when donor lungs are available, those people who are at the highest risk of dying while they are on the lung transplant waiting list are considered first.
Waitlist urgency and transplant benefit are represented by a number called the Lung Allocation Score (LAS). The LAS is a number, ranging from 0 to 100. It is the result of a calculation that takes into account a person's age, body mass index (BMI), and certain medical test results. A higher LAS represents a more urgent need for those on a waiting list for a transplant. More information on how the LAS is calculated is available from the Organ Procurement and Transplantation Network (OPTN).
Everyone on the waiting list who is 12 years of age or older has an LAS to determine his or her place on the list. The LAS is not used for anyone younger than age 12. Instead, children in this age range are assigned a priority level based on their medical condition.
Those with an urgent need for new lungs are categorized as Priority 1; all others are categorized as Priority 2.
According to the Cystic Fibrosis Foundation's Patient Registry for 2017, there were 1,548 people with CF who had received a transplant, including 250 people who reported to have received a
lung transplant in 2017. The majority of lung transplant recipients were age 30 and older.
Healthy lungs become available when someone dies and has expressed their desire to gift their organs to those in need. If a person dies without letting their families know that they want to donate their organs, their family is not obligated to
give doctors permission to donate their lungs.2
This will be a difficult and emotional time. The donor's family will have lost a loved one, and you will be receiving another chance at life. Many transplant recipients want to express their gratitude with the donor's family. However, you will not be
allowed to contact the donor's family immediately but may be able to do so after some time has passed and the donor's family agrees to be contacted. Organ procurement organizations (OPOs) will help facilitate the process of connecting recipients to donor families, so you can contact your local OPO to learn more.
Hear Craig Giddens, whose spouse received a double-lung transplant, read a letter expressing his gratitude to the donor's family.
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