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.
A panel of pediatric and adult gastroenterologists created this document to summarize and disseminate the existing medical literature regarding cystic fibrosis-specific gastrointestinal disease.
Freedman SD, Wilschanski M (eds.) Best Practices in the Management of the Gastrointestinal Aspects of Cystic Fibrosis. J Cyst Fibros. 2017 Nov;16 Suppl
A 3-year clinical training program for pediatric and adult gastroenterologists named DIGEST (Developing Innovative Gastrointestinal Specialty Training) was created to address gastrointestinal (GI) care for people with cystic fibrosis. The group developed an initiative to compile and review existing medical literature related to specific GI disease in CF, summarize the information, and disseminate this knowledge through the publication of a GI-related supplement to the Journal of Cystic Fibrosis. The work was carried out by pairing pediatric and adult gastroenterologists (DIGEST trainees and mentors/senior authors) in an international, collaborative writing effort.
The journal supplement was developed to address 10 key CF-specific GI topic areas, providing reviews aiming to educate non-gastroenterologist CF care providers. Each article included:
Where there was sufficient evidence and agreement across the literature, the authors proposed algorithms on how to approach the evaluation and management of these disorders and diseases.
Most of these articles reviewed current understanding of genotype-phenotype relationships.
Physiology and pathophysiology specific to CF
Unique aspects of reflux and CF: complications, emerging concepts, and controversies
Pathophysiology specific to CF, including:
Routine evaluation methodologies, emerging modalities for assessment and management
Pathophysiology specific to CF: differentiation of types of pain, red flags for pain
Associated conditions and observations
Differentiation of constipation in CF from that in non-affected individuals and from DIOS
Pathophysiology and risk factors in CF, including:
Colorectal cancer in patients with CF, and the importance of adequate screening and surveillance in this at increased risk population
Pathophysiology and emerging concepts with respect to mechanisms of risk and description of the spectrum of hepatobiliary involvement in CF and patterns observed
Evaluation (including the examination) and management specific to CF
Use of ursodeoxycholic acid including concepts and controversies and dosing recommendations
Specific risk factors including: environmental factors, luminal factors, and effect of transplantation
Concepts and controversies regarding use of ursodeoxycholic acid
Current status of indirect and direct testing for exocrine pancreatic insufficiency
Pancreatic enzyme replacement therapy and novel emerging therapies
Intrinsic and extrinsic factors, cystic fibrosis transmembrane conductance regulator (CFTR) modulators, and a model for risk for pancreatitis related to CFTR function and acinar reserve
Mechanisms and risk factors for development of cystosis and management algorithm
Nutritional status and interventions on current and future outcomes with respect to growth, pulmonary function, and other outcomes of clinical interest
Timing and targets/goals of nutritional therapy and multidisciplinary approach to evaluation and management
Several themes that run across disorders reviewed in this publication include:
Steven D Freedman MD (Beth Israel Deaconess Medical Center), Asim Maqbool, MD (The University of Pennsylvania Perelman School of Medicine)
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