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 cystic fibrosis patients and families face complicated issues related to getting the care they need. But CF Foundation Compass makes sure that no one has to do it alone.
For many people with cystic fibrosis, dealing with insurance is as much a part of living with the disease as nebulizers and vests. Many people with CF and their families face issues related to getting the care they need, but no one has to do it alone.
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.
Our goal is to educate policy makers about the needs of people with cystic fibrosis so that they make smart decisions about CF-related research, treatment, and access to care.
We recognize the value of tapping into the expertise that only people with CF and their families have. We invite you to share insights to help improve and develop programs and services that support the daily lives of people with CF.
Our mission is to find a cure for cystic fibrosis and improve the quality of life for those living with the disease. We can't do it alone. Help us add tomorrows by giving today.
In addition to working for a cure, the CF Foundation supports programs and policies to improve the lives of people with CF. Help us by raising awareness of CF, participating in a fundraising event, or volunteering with your local chapter.
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.
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. It 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 and determine if transplant is a treatment option for you.
Lung transplant is a multiphase process, and the transplant surgery is only one step in this process. The lung transplant process is divided into seven phases:
The amount of time people with cystic fibrosis have lived after transplant has improved in recent years. According to the 2018 International Society for Heart and Lung Transplantation Registry report, for adults with CF, median survival has improved from 5.5 years for those transplanted between 1990 and 1998 to 9.2 years for those transplanted between 1999 and 2016. This means that half of individuals transplanted between 1999 and 2016 were alive 9.2 years after transplant. This is higher than the median survival after lung transplant for other diseases such as chronic obstructive pulmonary disease (COPD), which is 5.9 years.
The median survival for children with CF was 5.4 years for those who received transplants between 1999 and 2016, although several different potential factors affect survival in children after lung transplant.
Joseph Pilewski, MD, explains that because CF progression can be unpredictable, experts now recommend people with CF be referred for transplant evaluation before transplant is needed.
“About 10 percent of the patients who did not get a transplant die each year. So, the risk in the first year, second year, it's about 10 percent for individuals with FEV1 of about 30 percent of predicted. That gives us some idea of what the population "at risk" looks like. Based on these considerations, we've come to a consensus that we want to recommend early referral.” -- Joseph Pilewski, MD
There are more people who need new lungs than there are available donor lungs. So, when donor lungs are available, they are offered first to people who are at the highest risk of dying while they are on the lung transplant waiting list.
Waitlist urgency and transplant benefit is 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 old 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.
Donors may have previously agreed to give their healthy lungs to people like you after they die. However, it is vital that a person who wants to donate their organs when they die lets their families know this is what they want to do. If a person dies unexpectedly without stating they want their organs donated to those in need, their family may not give doctors permission to donate their lungs.1
This will be a difficult and emotional time for you and the donor's family. The donor's family will have lost a loved one, and you will be receiving the gift of a chance at life. You will not be allowed to contact the donor's family immediately, but you 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 about these services.
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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