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.
Talking About a Lung Transplant
It is important to discuss a possible lung transplant long before you need to be listed. This will give you time to prepare for transplant as a future option and work with your cystic fibrosis care team to understand the implications and to create a plan.
The prospect of needing a lung transplant can be emotionally difficult. Some with cystic fibrosis may view needing a transplant as a “failure” to manage their disease. Others may see it as the “beginning of the end” or merely “trading one disease for another.” Given recent improvements in outcomes, lung transplantation can extend and improve the quality of life for many.
Although lung transplantation can be a difficult process, discussing the possibility of transplant early on -- even before you may actually need it -- will give you and your family more time to consider it as a treatment option in the future.
“It was drastic and seemed to come out of nowhere; my forced expiratory volume (FEV1) fell from 45 percent to 26 percent seemingly overnight. I found myself so out of breath just taking a shower that I'd have to sit down and recover for a few minutes before being able to get dressed.” -- Jen Bleecher, adult with CF, from the CF Community Blog
Cystic Fibrosis Foundation guidelines recommend that your CF care team start discussing lung transplant with you as a future treatment option long before the need for transplant arises. The guidelines define this as when your forced expiratory volume in one second (FEV1) is less than 50 percent of predicted. This does not necessarily mean that you need a transplant, only that transplant might be considered as an option at some point in the future.
Download this graphic to help you start a conversation about the lung transplant process with your family and care team.
It is important that all people with CF who need a lung transplant, or may need one in the future, understand what the transplant process entails and are given the information to make an informed decision. By having a conversation with your CF care team about your goals and what the transplant process is like, you can work through the emotions or feelings you may have about lung transplant.
Discussing transplant with your CF care team before you need one gives you time to learn about the type of support you'll need from your family and friends, and how best to partner with your CF care team to map out what the journey might look like for you.
An early referral to a transplant center gives you the chance to meet the multidisciplinary transplant team before a formal transplant evaluation. In addition, getting to know your transplant team prior to the need for listing may help identify and address any potential obstacles to you being listed for transplant in the future. You will work with the transplant team closely before and after the lung transplant surgery.
“Psychological services were available at my transplant center, and each patient is assigned a psychologist. I utilized my psychologist prior to transplant to cope with some anxiety surrounding intense pain after surgery. Additionally, while on the waitlist, [the transplant center] offered group psychoeducation sessions that focused on coping skills, anxiety reduction, and mindfulness.” -- Jenavese Armstrong, adult with CF, from the CF Community Blog
Lung transplantation is a process in which you and the family and friends who make up your social support team will need to make thoughtful decisions throughout many phases. These decisions will affect you and your social support team medically, logistically, financially, socially, and emotionally. Starting the conversation early will give you more time to plan ahead by:
Anyone living with CF knows it can be unpredictable. Although some may experience a slow decline in lung function, that decline can also happen very quickly, making it necessary to be referred for a lung transplant with little time to prepare.
The timing of the transplant procedure itself is a balancing act. People need to be sick enough to need a transplant, but also well enough to recover from the transplant surgery. This is known as the transplant window. By starting the referral process before you are in this window, you'll have more time to prepare.
Many people with CF want to delay a lung transplant as long as possible, but there is risk associated with this. Each year, approximately 10 percent of people with advanced cystic fibrosis die without a transplant, while only 6-8 percent undergo transplant as a life-sustaining treatment option.
The median survival for adults with CF who had lung transplant between 1999 and 2016 is 9.5 years. That means that half of the people with CF who have had lung transplants were alive 9.5 years after transplant.
In this clip, running from 24:32-25:00, 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
When considering a lung transplant as a future treatment option, you and your social support team of family and friends will want to learn as much as possible about the different phases of the transplant journey and the types of decisions you'll be asked to make during each phase.
Should you move forward with a lung transplant, it is important to form a strong relationship with the transplant team and ensure you feel respected and heard by them. As you move through the evaluation process, you will learn about the different roles on the transplant team and how the team members work together. You will develop a working relationship with the transplant team as you await donor lungs and the relationship will evolve after transplant.
As you go through the pre-transplant process, you are preparing for scenarios that will arise after transplant -- for example, having multiple appointments in a short period of time for your transplant evaluation prepares you for the many appointments you may have soon after transplant. Communication is vitally important as you learn about the new medications, common symptoms, and challenges after transplant.
Transplant teams select people they believe will have successful transplants, which begins with your physical health. By introducing a lung transplant referral before you need to be listed, you and your care team will have time to develop -- and act on -- a plan to ensure you are in the best physical condition possible. This can include creating an exercise plan that can help you maintain lung function, achieving a healthy weight and maintaining it, and getting your blood sugar under control if you have diabetes.
Many emotions are associated with the prospect of going through a transplant, including anxiety, depression, and even simply coming to terms with having a progressive disease like CF. Your care team can help you find the support you need to work through these and other emotions you may be experiencing during this process. Getting help to cope with emotional challenges should not affect transplant candidacy, but not getting help could hinder your access to medical care, including transplant.
Transplants are expensive. According to the Centers for Medicare & Medicaid Services (CMS), the average cost of a double-lung transplant was more than $1 million in 2017.1 Although transplant costs are often covered at least partly by health insurance, transplant recipients have to pay for some transplant-related expenses themselves (called out-of-pocket expenses).
Some costs, such as the costs of moving to a residence that is closer to the transplant center and living expenses, won't be covered by insurance. People undergoing a transplant evaluation need to show that their health insurance will cover transplant and that they have a plan in place for covering the out-of-pocket expenses and post-transplant care. These expenses may include co-pays for lab tests, medications, and physical rehabilitation, and relocation expenses (if necessary).
If you are considering purchasing or changing health insurance, this health insurance comparison tool can help you determine which plan is the best one for you. If you may consider a lung transplant in the future, it is important to understand if any health insurance coverage you have covers transplant.
Donated lungs cannot last for more than a few hours without blood flow and oxygen. In addition, you will need to attend many appointments with your transplant team before and after surgery. Therefore, many transplant centers will require that you live within a certain driving distance or arrange to have yourself flown to the transplant center quickly.
If you are added to the United Network for Organ Sharing (UNOS) transplant waiting list, from the moment you are listed and for a period of time after your transplant, you will need to live near your transplant center. You will visit the center often for medical tests, appointments, and rehabilitation. It is important that you learn what services your transplant center offers to help you meet these needs for a successful recovery.
Social support is vitally important before and after transplant. Most centers have strict requirements about the people who serve as part of your social support team and who can help care for you as a transplant recipient.
Many transplant centers require that two people commit to actively supporting the person with CF, and some centers require that caregivers commit to learning as much about transplant as the potential recipient before transplant. Some centers require that caregivers cannot have other obligations that would prevent them from providing care and support to the transplant recipient, including children or parents to care for. In addition, many centers do not permit hiring a professional caregiver or home care aid. For example, one center requires that your caregiver must be able to drive, be a nonsmoker, and may not be responsible for anybody else's care, such as children or parents.
The resources below can help you understand the transplant process and financing a transplant.
Cystic Fibrosis Foundation Compass is a personalized service that can help you understand insurance coverage and costs and find financial resources. You can contact Compass at any point to learn more about financial assistance programs to help you afford a transplant. You can contact Compass at:
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