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.
If your cystic fibrosis care team refers you to a lung transplant center, you and your transplant team will have the opportunity to get to know each other.
An early referral from your care team allows you to meet with the transplant team before moving forward with an evaluation for a lung transplant. Other times, the transplant team may recommend completing part or all of the transplant evaluation after your first visit. Referral is not the same as an evaluation, and an evaluation does not necessarily lead to listing for transplant. The goal is to identify any potential barriers to listing and attempt to address them prior to your need for listing for transplant.
Although declining lung function is important to help your cystic fibrosis care team determine if they should refer you, there are additional factors that your care team will consider before they recommend that you meet with a transplant team and possibly be evaluated for a lung transplant.
Although it's not the only factor, declining lung function is a key indicator for transplant referral. Cystic Fibrosis Foundation lung transplant referral guidelines recommend that your care team talk to you about lung transplant as a possibility when your forced expiratory volume in one second (FEV1) drops below 50 percent predicted. The guidelines recommend that care teams recommend referral for transplant when FEV1 is less than 50 percent and lung function has declined by more than 20 percent in 12 months.
Most people will not need to receive a transplant at this stage, but it is important to start the conversation early.
Talking about transplant early will give you more time to work with your CF care team to learn as much about the transplant process as possible, including the benefits and risks, and to identify any factors that may prevent you from being listed.
Overcoming barriers to being listed could take time, so the sooner they are identified, the more time you, your family, and your CF care team can have to work on them. Additionally, talking about transplant prior to your needing an urgent evaluation or listing gives you the opportunity to make a fully informed decision without the added pressure of being severely ill.
Although declining lung function is an important measure, other factors have been identified that may signal the need for referral -- even if your lung function is not low enough to qualify for immediate transplant. Care teams may consider these factors in addition to lung function, or independent of lung function, when deciding when to recommend transplant referral. These factors are referred to as “markers of increased disease severity,” and include:
Because these markers may indicate shortened survival, the guidelines recommend that people with severely reduced lung function (FEV1 less than 40 percent) and one or more of these markers be referred for transplant. In addition, when lung function decreases to 40 percent, care teams may begin to look for markers of increased disease severity.
In addition to lung function and markers of increased disease severity, your care team may also recommend lung transplant referral if:
The process of waiting for, receiving, and then recovering from a lung transplant can be long and stressful -- not just for you, but also for your loved ones. You or your family may want to talk to others who are going or have gone through it and understand what you're feeling -- both before and after the transplant.
“It is equally important for family caregivers to have ideas for keeping their loved ones' mental health intact. For example, my mother purchased a small fan for my room because my hot flashes made me feel miserable. She also asked the doctors if I could suck on hard candy since I was unable to eat or drink for weeks.” -- Jenavese Armstrong, an adult with CF, from the CF Community Blog
In this clip, from 36:01-36:57, Dr. Joseph Pilewski answers a question from Fanny Vlahos, an adult with CF and double-lung transplant recipient, about how the transplant evaluation process might differ between transplant centers.
“I think there is some uniformity in the outpatient evaluation process. It's typically a 3-4 day process and I think the testing is very similar at different sites. What differs somewhat among programs is how early they want to see patients go through the evaluation process. Some transplant centers look at records and think they can predict well what the disease course is going to be and make a conclusion that it's too early [for transplant referral] and ask patients to be re-referred when their disease progresses. I generally am not a big fan of that approach, frankly, because I think we don't predict the future very well.” -- Joseph Pilewski, MD
You will need similar support after your transplant. Keep in mind that people with CF who have undergone a lung transplant still can get and spread dangerous germs among others with CF because these germs can remain in their upper respiratory system. Because of the risk of cross-infection, people with CF should take the same precautions they took before lung transplantation.
Make sure your transplant center has support groups or can help you find one that's appropriate for you to minimize the risk of cross-infection after your transplant. It is equally important to have family and friends nearby to support you in both good times and bad. You'll experience both as you navigate the transplant process.
There are aspects of transplant that people don't understand unless they have been through it themselves. CF Peer Connect is a one-to-one peer support program for people with CF and their family members ages 16 and older. Connecting with a peer mentor provides an opportunity to talk with and learn from someone who is also affected by CF and has gone through similar experiences, such as lung transplant.
Another form of social support from the CF community can be found in virtual conferences and events. Designed by and for adults with cystic fibrosis and their families, virtual events -- including the annual BreatheCon, CF FamilyCon, and topic-specific CF MiniCons, including CF MiniCon: Transplant -- provide the opportunity to connect, share, and learn from peers through open and honest dialogue.
Your lung transplant center may offer you support throughout the transplant process, including psychological support, education, support groups, and Facebook groups. Some transplant centers have access to transplant psychiatrists, who are doctors trained in psychiatry with a special focus on individuals going through the transplant process.
The supply of donor lungs is limited and the transplant process requires a lot of work and commitment from you and those around you. That commitment, along with many other factors, can increase your chances of surviving the surgery and recovering afterward. The purpose of the evaluation is to help the transplant team determine if you would be likely to have a successful transplant.
A transplant team may determine that you are at too high a risk of doing poorly with the transplant surgery or after the surgery at their center. Factors that led to the decision to decline you for transplant at that center are referred to as barriers or contraindications to transplant. Depending on the specific barrier, there are some that you may be able to overcome.
Learning about these barriers as part of the early referral process for transplant can give you more time to work with your care team and to improve and become a stronger candidate for lung transplant.
“As I prepared to leave the hospital, I asked for a list of all the reasons I wasn't able to be listed for a transplant. Then, my team, my family, and I made the list of 'impossible' goals. I placed the list on the front of my refrigerator, my [do not resuscitate] form on the side of the fridge. Every day for that next year, I dedicated myself to checking items off that list until I was done.” -- Caleigh Haber, an adult with CF and two-time lung transplant recipient, from the CF Community Blog
Some of the barriers to transplant that are harder to change include:
Although you may not be able to change any of these factors, many transplant centers have different policies about what they consider barriers to transplant. Although some transplant centers may not accept people with certain health conditions, others may. For this reason, it is important that you understand the policies of a given transplant center and research more than one center.
The following organizations offer CF-specific information to help you learn more about lung transplant centers:
Barriers to transplant that you may have more control over include:
Starting the conversation about lung transplant before you need to be listed gives you more time to work with your CF care team to address these potential barriers. Working to correct the identified barriers to transplant will show the transplant team that you are engaged in your care and that you want to become a stronger candidate for transplant at their center.
“I also feel it is extremely important for the CF care center team to advocate for their patients. You will see in my video how lucky I have been to have such a wonderful CF care team that worked to find a transplant facility.” -- Mindy Ladd, adult with CF, from the CF Community Blog
You have the right to investigate being listed at more than one transplant center. Being listed in more than one center may give you primary access to more donors. This may or may not have an impact on your waiting time. When donor lungs become available, they are offered to the person who has the highest lung allocation score within a 250 nautical mile circle around the donor hospital. Because of insurance and/or geographical limitations, multiple listings can be logistically complicated.
More information on multiple listings is available from UNOS.
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