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.
Pancreatic insufficiency (PI) remains a significant issue for the majority of individuals with cystic fibrosis. Recommendations include target doses of pancreatic enzyme replacement therapy (PERT) in infants, children, and adolescents.
Borowitz DS, Grant RJ Durie PR, the Consensus Committee. Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy. J Pediatr. 1995; 127:681-84.
Pancreatic insufficiency (PI) remains a significant issue for the majority of individuals with cystic fibrosis. Left untreated, it could lead to growth failure, weight loss, abdominal bloating, foul-smelling stools, or diarrhea. The use of pancreatic enzyme replacement therapy (PERT) has helped to alleviate these symptoms.
However, the Cystic Fibrosis Foundation received reports of 35 cases of colonic stricture between January 1990 and December 1994, which were considered to be related to high doses of PERT. Guidelines for dosing of PERT and avoidance of fibrosing colonopathy were published in 1995 based on a consensus conference organized by the CF Foundation in conjunction with the U.S. Food and Drug Administration (FDA). Recommendations included target doses of PERT in infants, children, and adolescents, and a warning to use caution when PERT doses exceeded 2,500 lipase units/kg/meal. Since the publication of the original guideline in 1995, there have been a number of new publications relevant to the use of PERT in CF, some of which are mentioned below.
The majority of individuals with CF are pancreatic insufficient. Thus, provision of safe and effective pancreatic enzyme replacement is a key therapy in CF.
Pancreatic insufficient patients should consume a high-calorie diet with unrestricted fat that is appropriate to age and clinical status. Such diets have been shown superior to low-fat diets in promoting growth and lung function.
A nutritional assessment should be performed regularly as a component of routine care in patients with CF. Additional assessment should occur when dosing of PERT is altered.
Enzyme dosing may be done either by grams of fat ingested or by weight. Dosing by grams of fat is more likely to mimic the normal pancreatic response to a meal, although weight-based dosing may be simpler and more convenient, particularly in older children and adults.
Infants generally require 450-900 lipase units/g of fat, OR 2,000-4,000 lipase units per 120 ml of formula or when breastfeeding. Infants generally ingest a higher amount of fat/kg of body weight than do adults.
Older children and adults generally require 500-4,000 lipase units per gram of fat ingested (mean, 1,800 lipase units/g of fat), OR 500-2,500 lipase units/kg/meal, 250-1,250 lipase units/kg/snack, with three meals and two to three snacks per day. It is suggested that initial dosing be in the lower range and titrated up as needed to treat malabsorption.
Doses of enzyme exceeding 2,500 lipase units/kg/meal, or 4,000 lipase units/g of fat warrant further investigation. Doses of enzyme >6,000 lipase units/kg/meal have been associated with fibrosing colonopathy. It is not clear if doses >2,500 lipase units/kg/meal or >4,000 lipase units/g of fat are safe. It is also unlikely that higher enzyme doses will improve clinical condition in patients with CF and poor growth or gastrointestinal symptoms, thus it is recommended not to exceed these doses and that patients on higher doses be titrated down to a lower dosing range.
Patients should receive only the product brands prescribed by their CF care center. Enteric-coated microencapsulated enzymes are the most effective treatment for PI in CF. Patients should not use health food store enzymes or enzymes without an enteric coating unless directed to do so by their CF physician.
Enzyme capsules may be opened and the contents mixed with a small quantity of applesauce or another non-alkaline food, but they should not be crushed or allowed to sit in food. Enzymes may be inactivated by exposure to alkaline environments or prolonged contact with a moist environment.
Sarah Jane Schwarzenberg, M.D. (University of Minnesota Health) and Jill Dorsey, M.D., M.S. (Nemours Children's Specialty Care)
The guidelines were published in November 1995, they were reviewed in April 2019 and it was determined that no update is needed at this time.
Reference to any specific product, process, or service does not necessarily constitute or imply its endorsement, recommendation, or favoring by the Cystic Fibrosis Foundation. Further, the appearance of external hyperlinks does not constitute endorsement by the Cystic Fibrosis Foundation of the linked websites, or the information, products or services contained therein.
Information contained on this site does not cover all possible uses, actions, precautions, side effects, or interactions. This site is not intended as a substitute for treatment advice from a medical professional. Consult your doctor before making any changes to your treatment.
Approved drug information is available at www.dailymed.nlm.nih.gov/dailymed.
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