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.
Enteral tube feedings are an important treatment option in cystic fibrosis when oral intake is not adequate to meet nutritional goals. A multidisciplinary Cystic Fibrosis Foundation panel developed consensus evidence-based guidelines on all aspects of care regarding enteral feeding.
Enteral Tube Feeding for Individuals With Cystic Fibrosis: Cystic Fibrosis Foundation Evidence-Informed Guidelines
Schwarzenberg SJ, Hempstead SE, McDonald CM, Powers SW, Wooldridge J, Blair S, Freedman S, Harrington E, Murphy PJ, Palmer L, Schroeder AE, Shiel K, Sullivan J, Wallentine M, Marshall BC, Leonard AR. J Cyst Fibros. 2016. Nov;15(6):724-735.PMID: 27599607
Recommendations focus on care delivery by a multidisciplinary team including when to consider a tube and what to include in the pre-, peri- and post-operative assessment. Further research is needed in a variety of areas including the impact of enteral feeds on pulmonary status, optimal enzyme delivery, and formula composition.
Nutrition is an important component of CF care. Better nutrition is associated with improved pulmonary outcomes. Ensuring adequate nutrition requires a multidisciplinary team including, but not limited to, the patient and family, a registered dietitian/nutritionist, gastroenterologist, nurse, and primary CF physician. This multidisciplinary strategy allows for frequent and comprehensive assessments of barriers to nutrition and interventions as appropriate, including optimizing oral intake as well as bowel function. When strategies involving oral nutrition are insufficient to meet growth/weight goals, enteral tube feeding is the preferred route to optimize nutritional status. Evidence for this guideline is somewhat limited, and prospective, multicenter trials are needed to shape and strengthen further recommendations for nutritional assessment and intervention.
A multidisciplinary expert panel developed recommendations for enteral tube feeding. The panel included pediatric and adult dietitians, gastroenterologists, pulmonologists, a psychologist, a social worker, a nurse, the parent of a child with CF, and an individual with CF. Structured questions were developed utilizing the Population, Intervention, Control, Outcome (PICO) method. This was followed by a guided literature search and review. All consensus statements were discussed and voted on by the committee, which reached 100 percent agreement on each recommendation.
Enteral tube feeding as a means to improve age-dependent anthropometrics in individuals with CF that are unable to consume adequate calories and protein to meet growth/weight maintenance goals, despite appropriate evaluation and intervention by a multidisciplinary team.
Evaluation by a multidisciplinary CF team prior to enteral feeding tube placement in individuals with CF to identify and treat conditions that might be contributing to nutritional decline.
The use of supplemental enteral nutrition for pregnant or lactating women with CF who are unable to consume adequate calories and protein to meet nutritional goals despite appropriate evaluation and intervention by a multidisciplinary team.
Patient and family education about nutritional care including the role of enteral tube feeding be done throughout the lifetime of the individual with CF.
That the risks of certain conditions be considered and discussed with individuals with CF prior to the placement of an enteral feeding tube including but not limited to: coagulopathy, severe obstructive lung disease, ascites, portal hypertension, history of abdominal surgery, peritoneal dialysis, or alcohol and/or substance abuse.
Nasoenteral tube feeding in individuals with CF who require short-term (less than three months) nutritional repletion.
Discussion of third party/individual coverage of supplies and formula with individuals with CF prior to placement of an enteral feeding tube.
A comprehensive history and physical exam with specific attention to factors that represent potential complications be performed in advance of scheduling the placement of the percutaneous or surgical enteral feeding tube by the medical team performing the procedure in individuals with CF.
Clinical assessment of gastroesophageal reflux be performed prior to enteral feeding tube placement in individuals with CF.
To mitigate perioperative risk, the CF provider managing the pulmonary care of individuals with CF determine timing, based on pulmonary status, for percutaneous or surgical enteral feeding tube placement.
Platelet count and international normalized ratio (INR) be measured in individuals with CF prior to percutaneous enteral feeding tube placement.
Consultation with an anesthesiologist and the consideration of more intensive pulmonary therapy prior to placement of a percutaneous or surgical enteral feeding tube in individuals with CF and moderate to severe lung disease.
Enteral feeding tubes be placed by percutaneous endoscopic, laparoscopic, or radiologic technique when possible as opposed to open surgical techniques in individuals with CF.
That individuals with CF who are intolerant of gastric feeding receive jejunal feeding.
Airway clearance be re-initiated within 24 hours of percutaneous or surgical enteral feeding tube placement in children and adults with CF.
Optimal post-operative pain management to facilitate re-initiation of airway clearance in adults and children with CF who receive an enteral feeding tube.
Initiation of a bowel regimen to prevent post-operative constipation or distal intestinal obstruction syndrome in individuals with CF, especially those receiving narcotic pain management.
Adherence to the 2010 Clinical Care Guidelines for Cystic Fibrosis-Related Diabetes in individuals with CF who are using enteral feeding tubes.
The use of Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis to choose the best feeding type, breastmilk or formula, for enteral tube feeding in children with CF under 2 years of age.
Continuous nocturnal infusion for individuals with CF who are receiving supplemental enteral tube feeding.
A comprehensive planning approach with a multidisciplinary CF care team including the managing gastroenterologist, case manager, and home care agency prior to discharge.
That individuals with CF who have had enteral feeding tube placement be monitored at least annually by a gastroenterologist, preferably with enteral device experience, in addition to their quarterly CF care center visit.
Using FEV1 as an absolute contraindication to percutaneous or surgical enteral tube placement in individuals with CF.
Routine pH/impedance or radiographic procedures to assess gastroesophageal reflux in individuals with CF prior to percutaneous or surgical enteral feeding tube placement.
Placement of a percutaneous or surgical enteral feeding tube during acute illness.
Relevant manuscripts published after the original guidelines are listed below. These manuscripts have not been reviewed or endorsed by the guidelines committee.
No new publications
Adam C. Stein, M.D. (Northwestern University), and Amanda Leonard, MPH, RD, CDE (Johns Hopkins University)
The guidelines were published in November 2016, they were reviewed in April 2019 and it was determined that no update is needed at this time.
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