Clinical Care Guidelines

Clinical Care Guidelines

To ensure that people with CF continue to experience steady gains in length and quality of life, the Foundation helps its accredited care centers provide a standard of CF care with guidelines. We base guidelines on the latest research, medical evidence, and consultation with experts on best practices.

Age-Specific Guidelines Age-Specific Guidelines

Cystic fibrosis is a chronic, lifelong disease, requiring treatment that changes with the needs of the person with CF as he or she ages in order to maintain health. The standard of care for CF from infant to adult care is laid out by the Foundation in its clinical practice guidelines.

CFTR Modulator Therapy CFTR Modulator Therapy
Diagnosis Care Guidelines Diagnosis Care Guidelines

Diagnosing cystic fibrosis is a multistep process. A complete diagnostic evaluation should include a sweat chloride test, a genetic or carrier test and a clinical evaluation at a CF Foundation-accredited care center.

Infection Prevention and Control Care Guidelines Infection Prevention and Control Care Guidelines

People with CF are at greater risk of getting lung infections because thick, sticky mucus builds up in their lungs, allowing germs to thrive and multiply. CF lung infections remain a serious and chronic problem for many living with the disease.

Nutrition and GI Care Guidelines Nutrition and GI Care Guidelines

Because nutrition is so important to a long and high-quality of life for people with CF, the CF Foundation has created several clinical care guidelines related to nutritional and gastrointestinal (GI) issues.

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Colorectal Cancer Screening Clinical Care Guidelines

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

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Enteral Tube Feeding Clinical Care Guidelines

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. 

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Nutrition in Pediatrics Clinical Care Guidelines

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.

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Nutrition in Children and Adults Clinical Care Guidelines

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. 

8 min read

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Pancreatic Enzymes Clinical Care Guidelines

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.

12 min read

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Vitamin D Deficiency Clinical Care Guidelines

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

11 min read
Other CF-Related Conditions Other CF-Related Conditions

Managing other health conditions related to cystic fibrosis is vital to maintaining the health and quality of life for a person living with the disease. Complications of CF can affect many different organ systems and can be caused directly by the malfunctioning CFTR protein or can be a result of treatments for CF.

Respiratory Care Guidelines Respiratory Care Guidelines

Healthy lungs and maintaining lung function is central to the health of people with CF. Below is information on CF clinical care guidelines developed to help people with CF improve and maintain respiratory health.

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Guidelines for the Care of Individuals With Advanced CF Lung Disease

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.

7 min read

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CF Airway Clearance Therapies Clinical Care Guidelines

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

11 min read

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Chronic Medications to Maintain Lung Health Clinical Care Guidelines

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.

9 min read

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Pulmonary Exacerbations Clinical Care Guidelines

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.

10 min read

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Lung Transplant Referral Guidelines

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.

8 min read

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Pneumothorax and Hemoptysis Clinical Care Guidelines

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.

13 min read
Screening and Treating Depression and Anxiety Screening and Treating Depression and Anxiety
Best Practices in the Management of the Gastrointestinal Aspects of CF