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.
These guidelines provide recommendations to the cystic fibrosis community regarding the management of advanced cystic fibrosis lung disease (ACFLD), including reducing practice variability, improving the quality of life and survival of those with ACFLD, and identifying future research directions.
S.G. Kapnadak, E. Dimango and D. Hadjiliadis et al. Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease. J Cyst Fibros. 2020. May;19(3):344-354 https://doi.org/10.1016/j.jcf.2020.02.015. Epub 2020 Feb 27.
While the quality of life and survival of individuals with cystic fibrosis are improving, advanced cystic fibrosis lung disease (ACFLD) remains common and the most frequent cause of death. ACFLD is associated with increased physical symptoms and emotional stress, pulmonary exacerbations, and health care utilization. Despite its importance, there is limited literature directed specifically at the unique challenges facing individuals with ACFLD. Furthermore, clinical experience suggests variability in practice patterns pertaining to ACFLD management with some aspects of recommended care shown to be inconsistently applied. The goal of these consensus guidelines is to provide guidance for management of ACFLD. Recognizing that care must be customized to each individual, these recommendations aim to reduce practice variability, improve the quality of life and survival of those with ACFLD, and identify gaps in clinical knowledge where future research is needed.
The Cystic Fibrosis Foundation assembled a multidisciplinary team that utilized PICO (Population, Intervention, Control, Outcome) methodology to guide systematic searches of the medical literature. Recommendations were developed, voted on, and accepted if 80 percent of the committee agreed, after which feedback from public comment was incorporated.
When individuals with CF meet criteria for ACFLD, the CF Foundation recommends routine advance care planning conversations with them and their caregiver(s), including communication about prognosis and goals of care, documentation of advance directives, and decision-making surrounding lung transplantation.
The CF Foundation recommends that individuals with ACFLD undergo screening for hypoxemia on exertion and sleep, hypercarbia, and pulmonary hypertension.
The CF Foundation recommends supplemental oxygen for individuals with ACFLD and exercise-induced or nocturnal hypoxemia.
The CF Foundation recommends consideration of nocturnal noninvasive ventilation for individuals with ACFLD and chronic hypercarbia.
The CF Foundation found insufficient evidence to make a recommendation regarding the use of pulmonary vasodilator therapy in individuals with ACFLD and pulmonary hypertension.
The CF Foundation recommends lung transplantation as a treatment option for individuals with ACFLD if congruent with goals of care.
The CF Foundation recommends that individuals with ACFLD and acute respiratory failure be considered eligible for intensive care unit management regardless of transplant status if congruent with goals of care.
The CF Foundation recommends that individuals with ACFLD and acute respiratory failure be considered for a trial of high flow nasal cannula oxygen and/or noninvasive ventilation.
For individuals with ACFLD and acute respiratory failure requiring invasive mechanical ventilation, the CF Foundation recommends consideration of early tracheostomy when anticipated need for mechanical ventilation is more than 5-7 days and support remains congruent with goals of care.
The CF Foundation recommends that individuals with ACFLD who develop refractory respiratory failure requiring invasive mechanical ventilation be considered for early transition to extracorporeal life support if congruent with goals of care.
For individuals with ACFLD, the CF Foundation recommends a trial of continuous alternating inhaled antibiotics as dictated by bacterial pathogens identified in respiratory culture.
The CF Foundation recommends that individuals with progressive ACFLD undergo screening for fungal pathogens in addition to standard microbiological screening.
The CF Foundation recommends that individuals with ACFLD participate in a pulmonary rehabilitation program.
The CF Foundation found insufficient evidence to make a recommendation regarding the use of systemic corticosteroids in individuals with ACFLD.
The CF Foundation found insufficient evidence to make a recommendation regarding routine screening for gastroesophageal reflux (GER) in individuals with ACFLD.
The CF Foundation recommends the use of enteral tube feeds for individuals with ACFLD and malnutrition after consideration of procedural risks versus benefits.
For individuals with ACFLD with frequent prior and continuing exposure to nephrotoxic and ototoxic agents, the CF Foundation recommends increased monitoring for accumulating toxicity.
The CF Foundation recommends that women with ACFLD contemplating pregnancy carefully consider the risks in consultation with high-risk obstetrics and CF providers.
For individuals with ACFLD with indications for opioids, the CF Foundation recommends treatment in accordance with established Center for Disease Control guidelines; this should include monitoring for adverse effects, and consultation with pain and/or palliative care specialists as appropriate.
For individuals with ACFLD and anxiety, the CF Foundation recommends management in accordance with the International Committee on Mental Health in Cystic Fibrosis: Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus statements for screening and treating depression and anxiety, reserving benzodiazepines for refractory symptoms or end of life symptom palliation.
When individuals with CF meet criteria for ACFLD, and with subsequent changes in clinical or social status, the CF Foundation recommends a formal care conference involving caregiver(s) and selected team members to develop a plan for ongoing psychosocial support.
In individuals with ACFLD, the CF Foundation recommends assessing the adequacy of financial resources at least biannually, and with changes in clinical or social status.
For pediatric patients with ACFLD nearing the age of transition to an adult CF care program, the CF Foundation recommends formally outlining a transition plan that provides flexibility in timing and coordination of transfer.
Kavalieratos D, Georgiopoulos AM, Dhingra L, et al. Models of Palliative Care Delivery for Individuals with Cystic Fibrosis: Cystic Fibrosis Foundation Evidence-Informed Consensus Guidelines. J Palliat Med. 2020 Sep 16. doi: 10.1089/jpm.2020.0311. Online ahead of print.
The guidelines were published in February 2020, and no updates are needed at this time.
Marc A. Sala, MD (Northwestern University Feinberg School of Medicine); Siddhartha G. Kapnadak, MD (University of Washington School of Medicine)
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