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 were developed by consensus based on expert opinion and a medical literature review to guide the monitoring and care of infants who have an abnormal cystic fibrosis newborn screening result, but do not meet the full criteria for a CF diagnosis.
Borowitz D, Robinson KA, Rosenfeld M, et al. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009 Dec; 155(6 Suppl):S73-S93.
Although many treatment issues are the same for individuals with cystic fibrosis regardless of when they are diagnosed, some are unique to the population of newborn infants who may not have overt symptoms of the disease before being referred to a CF care center.
In 2009, the Cystic Fibrosis Foundation convened a committee of experts to develop guidelines for care based on available evidence for this distinct population of newly diagnosed infants. These guidelines contain detailed recommendations regarding care of infants beginning at the time of diagnosis, and discuss topics such as feeding, nutrition, airway clearance, diagnostic testing, treatment of infection, and chronic pulmonary therapies.
The 2009 Evidence-Based Guidelines for Management of Infants With Cystic Fibrosis summarize recommendations from a multidisciplinary expert committee. These guidelines were created based on a systematic review of the evidence and expert opinion. The process for developing these guidelines included an expert committee, specific questions, systematic literature review, recommendations drafted, committee vote, and final recommendations.
The final recommendations were graded using the U.S. Preventative Task Force (USPSTF) Scheme. Find additional information about the USPSTF grading definitions.
Treatment for infants diagnosed with CF by newborn screening (NBS) should be done at an accredited CF care center, with the goal of initial visit within 24-72 hours of diagnosis (1-3 working days in absence of overt symptoms).
For infants with CF under 2 years of age, pancreatic functional status should be measured by fecal elastase or coefficient of fat absorption (CFA) in all individuals.
For infants with CF under 2 years of age, pancreatic enzyme replacement therapy (PERT) should be initiated at a dosage of 2,000-5,000 lipase units at each feeding. Adjustment should not exceed 2,500 lipase units/kg at each feeding with a maximum daily dose of 10,000 lipase units/kg.
Recommended in the CF Foundation Consensus Report on Nutrition for Pediatric Patients and the European Consensus on Nutrition in Patients with CF
For infants with CF under 2 years of age, PERT should be started:
For infants with CF under 2 years of age, PERT should not be started in infants with one or two CFTR mutations associated with pancreatic sufficiency, unless:
For all patients with CF, including those under 2 years of age, generic non-proprietary PERT should not be used.
Recommended in the CF Foundation Evidence-Based Practice Recommendations for Nutrition
For infants with CF under 2 years of age, human milk should be the initial feeding type.
For infants with CF under 2 years of age who are fed formula, standard infant formula (rather than hydrolyzed protein formulas) should be used.
For infants with CF under 2 years of age, calorie-dense feedings should be used if weight loss or inadequate weight gain is identified.
For infants with CF under 2 years of age, encourage positive feeding behaviors, such as by the provision of educational resources.
For infants with CF under 2 years of age, prescribe multivitamin supplements with recommended levels of vitamins A, D, E, and K for patients with CF shortly after diagnosis.
Recommended in the CF Foundation Consensus Report on Nutrition for Pediatric Patients
For infants with CF under 2 years of age, blood levels of fat-soluble vitamins should be measured approximately two months after supplement initiation and annually thereafter, with more frequent measurements if values are abnormal.
For infants with CF under 2 years of age, a trial of zinc supplementation (1 mg/kg/day of elemental zinc in divided doses for six months) may be given to some infants who are not adequately growing despite adequate caloric intake and PERT.
For infants with CF under 2 years of age, supplement with 1/8 teaspoon table salt per day starting at diagnosis, increasing to 1/4 teaspoon of table salt per day by 6 months of age.
Patients aged 6 months to 2 years whose community water supply contains less than 0.3 ppm should be supplemented with fluoride 0.25mg/dl.
Recommended in the Center for Disease Control and Prevention Guidelines
For children 1-12 years of age with growth deficits, intensive treatment with behavioral intervention in conjunction with nutritional counseling should be used to promote weight gain.
Recommended in the CF Foundation Evidence-based Practice Recommendations for Nutrition
For infants with CF under 2 years of age, a smoke-free environment should be provided and all caregivers be informed that cigarette exposure harms children with CF.
For infants with CF under 2 years of age, airway clearance therapy should be initiated in the first few months of life.
For infants with CF under 2 years of age, use albuterol before percussion and postural drainage.
For infants with CF under 2 years of age, do not use the head-down position for percussion and postural drainage. (Percussion and postural drainage is recommended as the form of airway clearance for most infants in the Airway Clearance Guidelines.)
For infants with CF under 2 years of age, newly diagnosed patients should be separated from other patients cared for in CF clinics until adequate infection control education has been provided to, and is understood by, the caregivers.
For infants with CF under 2 years of age, infection control measures should be implemented in compliance with CF Foundation recommendations to minimize transmission of bacterial infection to infants.
Recommended in the CF Foundation Consensus Conference on Infection Control
Annual influenza vaccination is recommended for infants with CF ≥6 months of age, as well as all household members and health care providers caring for these infants. Household contacts and out-of-home caregivers for children with CF <6 months of age should receive annual influenza vaccination.
Recommended in American Academy of Pediatrics Guidelines and the CF Foundation Consensus Conference on Infection Control
For infants with CF under 2 years of age, the use of palivizumab should be considered for prophylaxis of respiratory syncytial virus.
For infants with CF under 2 years of age, oropharyngeal cultures should be performed at least quarterly.
For infants with CF under 2 years of age, bronchoscopy and bronchoalveolar lavage should be considered in infants with signs or symptoms of lung disease, particularly with failed response to appropriate intervention.
For infants with CF under 2 years of age, the CF Foundation recommends against the prophylactic use of oral anti-staphylococcal antibiotics in asymptomatic infants.
Recommended in the CF Foundation Pulmonary Guidelines on Chronic Medications
For infants with CF under 2 years of age, the CF Foundation recommends against the use of chronic antibiotics for prophylaxis to prevent Pseudomonas aeruginosa.
For infants with CF under 2 years of age, new acquisition of Pseudomonas aeruginosa, defined as initial acquisition or new acquisition after “successful” eradication therapy, should be treated with anti-pseudomonal antibiotics and increased airway clearance in the presence or absence of symptoms.
Infants with CF under 2 years of age who remain persistently colonized with Pseudomonas aeruginosa after two attempts at eradication should be treated chronically with alternate month tobramycin solution for inhalation.
For infants with CF under 2 years of age, there is insufficient evidence to recommend for or against active attempts to eradicate Staphylococcus aureus in asymptomatic infants.
For infants with CF under 2 years of age, there is insufficient evidence to recommend for or against active attempts to eradicate methicillin-resistant Staphylococcus aureus (MRSA) in asymptomatic infants.
For infants with CF under 2 years of age, pulse oximetry measurements should be obtained with acute respiratory symptoms.
For infants with CF under 2 years of age, a baseline chest X-ray should be obtained within the first 3-6 months and again within the first two years of life.
For infants with CF under 2 years of age, the CF Foundation recommends against the use of chest computed tomography (CT) scans for routine surveillance.
For infants with CF under 2 years of age, chest CT scans should be considered in infants with signs or symptoms of lung disease who fail to respond to appropriate interventions.
For infants with CF under 2 years of age, infant pulmonary function tests (PFTs) should be considered as an adjunctive tool to monitor respiratory status.
For infants with CF under 2 years of age, there is insufficient evidence to recommend for or against pulse oximetry routinely as an adjunctive tool to detect lung disease.
For infants with CF under 2 years of age, dornase alfa (recombinant human DNase) may be used in symptomatic infants.
For infants with CF under 2 years of age, 7 percent hypertonic saline may be used in symptomatic infants.
For infants with CF under 2 years of age without airway reactivity or asthma, the CF Foundation does not recommend the use of inhaled corticosteroids to improve lung function or reduce exacerbations.
For infants with CF under 2 years of age, there is insufficient evidence to recommend for or against chronic azithromycin in patients colonized with Pseudomonas aeruginosa.
Relevant manuscripts published after the original guidelines are listed below. These manuscripts have not been reviewed or endorsed by the guidelines committee.
Sylvia Szentpetery, M.D., (Children's Hospital of Pittsburgh) and Margaret Rosenfeld, M.D., MPH, (Seattle Children's Hospital)
The guidelines were published in December 2009, they were reviewed in April 2019 and it was determined that no update is needed at this time.
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