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.
Nutritional support is a vital component of care for patients with cystic fibrosis. These recommendations cover caloric intake, dosing for pancreatic enzyme replacement therapy, and monitoring growth in childhood and weight status in patients of all ages.
Stallings VA, Stark LJ, Robinson KA, Feranchak AP, Quinton H, Clinical Practice Guidelines on Growth and Nutrition Subcommittee, Ad Hoc Working Group. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008 May;108(5):832-9.PMID: 18442507
This set of recommendations resulted from the review of evidence in three areas: dietary energy (caloric) intake, dosing for pancreatic enzyme replacement therapy (PERT), and monitoring growth in childhood and weight status in patients of all ages. It was a partial update of the 2002 consensus report recommendations for nutrition for pediatric patients with cystic fibrosis. Increased resting energy expenditure (patients require more dietary calories than healthy peers) is common in patients with cystic fibrosis and often is combined with poor absorption of dietary fat secondary to pancreatic exocrine insufficiency (PEI). This results in increased risk for both macro- and micronutrient deficiencies. Malnutrition in CF has been shown to affect important clinical outcomes including pulmonary function, rate of pulmonary decline, frequency of hospital admission, and quality of life.
Due to a paucity of high-quality, prospective studies and clinical trials, most of the 2008 recommendations were based on the results of retrospective studies of the Cystic Fibrosis Foundation Patient Registry, census-based practice guidelines, and historical practice patterns. Since the 2008 guidelines, new evidence shows that optimization of patient nutritional status is a key component of clinical care and should be addressed in the care of all patients. Future work will likely focus on identifying more informative indicators of nutritional status, such as body composition (fat mass, fat distribution, fat-free or lean mass), in addition to weight and stature. Identification of early nutritional biomarkers that predict disease progression or support of individualized nutrition regimes may be required to optimize the use of newer therapies.
Since its initial publication, the 2008 evidence-based nutritional guidelines have served as an invaluable resource to CF dietitians, pulmonologists, gastroenterologists, endocrinologists, and primary care providers. Expanded and successful implementation of nutritional therapy, combined with advances in respiratory care, has led to increased life expectancy and improved quality of life. At its core, these guidelines have provided a framework for non-nutrition experts to provide optimal nutritional care for all patients with CF.
The CF Foundation worked with investigators at Johns Hopkins University to conduct a systematic review. This review assisted the subcommittee in making recommendations. They looked at articles published from January 1988 to February 2005 to inform the development of clinical care guidelines and encourage evidence-based practice. The Subcommittee on Growth and Nutrition reviewed the evidence in two areas: energy intake and dosing for PERT. Evidence-based recommendations are presented here. Also, an ad hoc working group conducted a review of the literature and performed new analyses using the CF Patient Registry to update the recommendations for growth and weight-status monitoring. The evidence base for each recommendation was graded using the system developed by the U.S. Preventive Services Task Force. Find additional information about the USPSTF grading definitions.
Several areas had insufficient evidence to make a recommendation and additional research is needed to:
Relevant manuscripts published after the original guidelines are listed below. These manuscripts have not been reviewed or endorsed by the guidelines committee.
A. Jay Freeman, M.D., MSc (Children's Healthcare of Atlanta/Emory University School of Medicine) and Virginia A. Stallings, M.D., M.S. (Children's Hospital of Philadelphia/Perelman School of Medicine)
The guidelines were published in May 2008, they were reviewed in April 2019 and it was determined that no update is needed at this time.
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