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.
The purpose of these guidelines is to provide recommendations to the CF community regarding appropriate identification and timely referral of individuals with advanced CF lung disease to transplant centers.
Ramos KJ, Smith PJ, McKone EF, Pilewski JM, et al. Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines. J Cyst Fibros. 2019 May;18(3):321-333. doi: 10.1016/j.jcf.2019.03.002. Epub 2019 Mar 27.
Despite improvements in life expectancy, pulmonary disease remains the major cause of morbidity and mortality for individuals with cystic fibrosis. Lung transplantation (lung transplant) therefore will continue to remain an important therapeutic option for individuals with advanced CF lung disease. However, a significant number of individuals with CF die each year without lung transplant referral.
Early referral for lung transplant increases the likelihood that an individual with CF will be deemed an appropriate candidate for lung transplant and ultimately undergo successful transplant. Early referral also allows modifiable barriers to transplant to be addressed proactively, thereby optimizing candidacy.
Conversely, late referral may result in missing the ideal “window” for transplantation, increasing the risks of waiting list mortality and severe post-transplant complications. The purpose of these guidelines is to provide recommendations to the CF community regarding appropriate identification and timely referral of individuals with advanced CF lung disease to transplant centers.
The Cystic Fibrosis Foundation assembled a multi-disciplinary committee that utilized PICO (population, intervention, control, outcome) methodology to guide systematic searches of the medical literature. Additional information was gathered from a focus group of transplant recipients with CF and spouses of recipients. Draft recommendations were developed, voted on by the committee, and accepted if 80 percent of the committee agreed. Public comments were then solicited, leading to another round of revisions and voting by the committee. Twenty-one recommendations were ultimately included in the final guidelines, each with 100 percent agreement among committee members.
The CF Foundation recommends routine clinician-led efforts to discuss disease trajectory and treatment options, including a discussion of lung transplantation.
The CF Foundation recommends CF care team-initiated discussion regarding lung transplantation with all individuals with CF and a forced expiratory volume in one second (FEV1) < 50% predicted.
The CF Foundation recommends the use of up-to-date CF-specific transplant resources to promote understanding of the transplant journey and to minimize misconceptions regarding outcomes.
The CF Foundation recommends that CF clinicians develop relationships with peers at partnering transplant centers.
The CF Foundation recommends that the individual's CF care team elicit and address CF-specific psychosocial and physical concerns about lung transplantation to facilitate transition to transplant.
The CF Foundation recommends that modifiable barriers to lung transplantation be addressed preemptively to optimize transplant candidacy; however, unresolved barriers should not preclude referral.
The CF Foundation recommends the CF and lung transplant care teams acknowledge and provide support for mental health concerns regarding the referral and evaluation process for transplant that are unique to individuals with CF.
The CF Foundation recommends lung transplant evaluation, regardless of FEV1, when there are markers of shortened survival including: 6-minute walk test (6MWT) distance < 400 meters, hypoxemia (at rest or exertion), hypercarbia, or pulmonary hypertension.
For individuals with CF who are 18 years of age and older, the CF Foundation recommends lung transplant referral no later than when:
For individuals with CF who are under the age of 18 years, the CF Foundation recommends lung transplant referral no later than when:
For individuals with CF and an FEV1 <40% predicted, the CF Foundation recommends an annual 6MWT, assessment of need for supplemental oxygen, and venous blood gas to screen for markers of severe disease that may warrant transplant referral.
For individuals with CF who are 18 years and older with FEV1 < 40% predicted, the CF Foundation recommends a baseline echocardiogram to screen for pulmonary hypertension.
The CF Foundation recommends lung transplant referral for adults with CF with a body mass index (BMI) < 18 and FEV1 < 40% predicted while concurrently working to improve nutritional status.
The CF Foundation recommends lung transplant referral for individuals with FEV1 < 40% predicted and> 2 exacerbations per year requiring intravenous (IV) antibiotics, or 1 exacerbation requiring positive pressure ventilation, regardless of FEV1.
The CF Foundation recommends lung transplant referral for individuals with FEV1 < 40% predicted and massive hemoptysis (>240 mL) requiring intensive care unit (ICU) admission or bronchial artery embolization.
The CF Foundation recommends lung transplant referral for individuals with FEV1 < 40% predicted and pneumothorax.
For females with CF, especially those who are younger, the CF Foundation recommends special consideration for lung transplant referral even when other thresholds have not been met.
For individuals with short stature (height <162 cm), the CF Foundation recommends special consideration for lung transplant referral even when other thresholds have not been met.
The CF Foundation recommends CF clinician consultation with local and geographically distant lung transplant centers for individuals with microorganisms that may pose a risk for lung transplantation.
Before determining that an individual is not a transplant candidate, the CF Foundation recommends consultation with at least two transplant centers, one of which should have experience addressing the individual's potential contraindications to transplantation.
For transplant candidates, the CF Foundation recommends communication between the CF and lung transplant care teams at least every 6 months and with major clinical changes.
Relevant manuscripts published after the original guidelines are listed below. These manuscripts have not been reviewed or endorsed by the guidelines committee.
Identifies predictors of mortality that are not included in the lung allocation score for individuals with CF listed for lung transplant. A 30 percent relative decline in FEV1 during the year prior to listing is associated with increased risk of death on the waiting list.
Describes a validated predictive risk model for mortality in the CF population.
Kathleen J. Ramos, MD, (University of Washington Medical Center), David M. Sayah, MD, PhD (David Geffen School of Medicine at UCLA)
The guidelines were published in May 2019, they were reviewed in February 2020 and it was determined that no update is needed at this time.
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