Clinical Care Guidelines

The Cystic Fibrosis Foundation organized a multidisciplinary committee to reevaluate key aspects of the current care model and considered potential modifications based on a widening range of health needs among people with CF.

The Cystic Fibrosis Foundation assembled a panel to summarize available evidence, synthesize expert opinion, and provide clinical guidance for the composition of the care team at an accredited CF center in the United States.

Palliative care guidelines provide recommendations to help reduce physical and emotional symptoms and improve quality of life for people with cystic fibrosis throughout their lives.

There is no consensus on the best model of care for individuals with CF to manage the non-pulmonary complications that persist after lung transplant. This position paper outlines two models of post-transplant care to accomplish optimal CF care after transplant.

Most newborns in the United States are screened for CF at birth through newborn screening. All 50 states and the District of Columbia now screen for CF.

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.

These preschool guidelines provide clinical care guidelines for people with cystic fibrosis between 2 and 5 years. It includes 53 recommendations intended to help make informed decisions in the care of preschool-aged children with CF.

These clinical care guidelines from the Cystic Fibrosis Foundation provide recommendations on how best to care for adults with CF.

These evidence-based guidelines were created by a multidisciplinary committee to help cystic fibrosis specialists and primary care providers who care for people with CFTR-related metabolic disorder and their families. It should supplement the standard care provided in primary care. 

To help standardize the diagnosis of both infants with positive newborn screening results and older patients with what may be cystic fibrosis symptoms, the Cystic Fibrosis Foundation worked with CF medical experts to develop guidelines on the steps leading to a confirmed diagnosis.

A well-performed and well-interpreted sweat test is the gold standard for accurately diagnosing cystic fibrosis. These guidelines were developed by consensus based on expert opinion and a review of the medical literature.

Updated from 2003, these guidelines on infection prevention and control for cystic fibrosis provide recommendations for people with CF, their families, and health care providers to help reduce the spread of germs in the clinic and hospital setting, as well as in everyday life.

Allergic bronchopulmonary aspergillosis is a hypersensitivity lung disease that results from exposure to Aspergillus fumigatus and occurs primarily in people with asthma or with cystic fibrosis. These guidelines were developed via a consensus conference of experts in 2003. 

Chronic infection with P. aeruginosa is associated with increased morbidity and mortality. Effective regimens include antibiotic therapy for newly acquired infection and routine surveillance with oropharyngeal cultures.

Nontuberculous mycobacteria can cause chronic pulmonary infection or can reside in the lungs without causing progressive disease. Challenges include making decisions on when and how to initiate treatment. 

These clinical care guidelines from the Cystic Fibrosis Foundation provide recommendations for colorectal cancer screening for adults with CF. 

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. 

Normal growth is associated with good lung function in children with CF. The CF Foundation gathered a team of experts to determine the best ways to ensure good nutrition for healthy growth.

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. 

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.

These guidelines present recommendations on vitamin D screening, diagnosis, supplementation and treatment in children and adults with cystic fibrosis.

A panel of pediatric and adult gastroenterologists created this document to summarize and disseminate the existing medical literature regarding cystic fibrosis-specific gastrointestinal disease.

The Cystic Fibrosis Foundation organized a multidisciplinary committee to develop a nutrition position paper to provide interim advice and considerations to the CF community around CF nutrition based on the rapidly changing nutrition landscape in CF.

Despite its prevalence and impact, there is no CF-specific guidance for the assessment and management of pain. The Cystic Fibrosis Foundation assembled an expert panel of clinicians, researchers, people with CF, and caregivers to develop consensus recommendations for pain management in CF.

As early cystic fibrosis-related diabetes (CFRD) may be clinically silent, these guidelines highlight the importance of regular screening. Management recommendations focus on insulin therapy and ongoing care by a team with knowledge of CF and diabetes.

Prevention, early recognition, and treatment of cystic fibrosis-related bone disease are imperative to sustain bone health. This guideline provides recommendations regarding screening and treatment to optimize bone health in people with CF.

The Cystic Fibrosis Foundation assembled a committee to evaluate research advances and revise guidelines for CF-associated liver disease.

These guidelines were created to help care center teams to integrate screening and treating depression and anxiety into comprehensive cystic fibrosis care.

This executive summary highlights key content from articles reviewing the pathogenesis, diagnosis, and management of CF endocrine disorders.

These consensus statement recommendations were developed to help guide optimal care for children and adults with CF with otolaryngologic (ear, nose, and throat) disease. 

To aid clinicians, patients, and families in the best use of modulators, the Cystic Fibrosis Foundation sponsored the creation of guidelines to inform discussions and support decision-making.

These guidelines provide recommendations to the cystic fibrosis community regarding the management of advanced cystic fibrosis lung disease (ACFLD), including reducing practice variability, improving the quality of life and survival of those with ACFLD, and identifying future research directions.

Airway clearance therapies improve the efficacy of the mucociliary escalator and elicit a cough. These guidelines reviewed and compared the various techniques and devices.

These guidelines were developed by consensus based on expert opinion and a medical literature review to provide evidence-based recommendations for chronic medication use for lung health maintenance.

Pulmonary exacerbations are common among people with cystic fibrosis, yet little is known about best treatment practices. These guidelines were developed by consensus based on expert opinion and a review of the medical literature.

The purpose of these guidelines is to provide recommendations to the CF community regarding appropriate identification and timely referral of individuals with advanced CF lung disease to transplant centers.

Hemoptysis and pneumothorax are complications reported in people with cystic fibrosis. This document reflects the Cystic Fibrosis Foundation's Pulmonary Therapies Committee recommendations for the treatment of hemoptysis and pneumothorax.