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CF Care Guidelines - Respiratory

Because healthy lungs are important for a long life for people with CF, the Foundation has developed in-depth guidelines covering several topics. Below is information on the CF care guidelines for maintaining respiratory health.


Chronic Medications for Lung Health Maintenance

People with CF now have a number of drugs available to keep their lungs healthy. The guidelines describe the science behind the recommendations for use of:

  • Inhaled antibiotics
  • Dornase alfa
  • Azithromycin
  • Hypertonic saline
  • High-dose ibuprofen
  • Ivacaftor

Care Guidelines

Information for People with CF and their Families

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CF Airway Clearance Therapies

Clearing the airways of mucus and germs is key to keeping CF lungs healthy. The guidelines cover topics such as the:

  • Importance of daily airway clearance
  • Variations of airway clearance techniques
  • Impact of aerobic exercise on airway clearance

Care Guidelines

Information for People with CF and their Families

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Pulmonary Exacerbations

People with CF often have pulmonary exacerbations — a period when lung disease worsens. This can include an infection, an increase in cough and sputum, a drop in lung function and weight loss. To improve lung function and treat exacerbations, care guidelines for the treatment of pulmonary exacerbations describe best practices for:

  • Treatment of an exacerbation — antibiotics and airway clearance
  • Chronic medications (i.e., inhaled antibiotics) during an exacerbation

Care Guidelines

Information for People with CF and their Families

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Pulmonary Complications

As CF progresses, it is possible for people with the disease to experience pulmonary complications such as a partial collapse of the lung (pneumothorax) or coughing up blood from the lungs (hemoptysis). The CF care guidelines present expert opinion from CF care specialists and interventional radiologists on the treatment of these conditions.

Pneumothorax – best practices for:

  • Hospital admission
  • Chest tube placement
  • Preventing recurrence
  • Airway clearance
  • Inhaled medications
  • Antibiotics
  • Restricting activities

Hemoptysis – best practices for:

  • Hospital admission
  • Preventing recurrence
  • Airway clearance
  • Inhaled medications
  • Antibiotics

Care Guidelines

Information for People with CF and their Families

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Allergic Bronchopulmonary Aspergillosis (ABPA)

Some people with CF have ABPA — an allergy to Aspergillus. This is a fungus commonly found in dust and dirt. ABPA can cause coughing and wheezing. The care guidelines describe:

  • How to diagnose ABPA in CF
  • How and when to treat ABPA

Care Guidelines

Information for People with CF and their Families

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Lung Transplants

People with severe CF lung disease may consider a lung transplant. The CF Foundation brought together a panel of experts in 1996 to examine important issues surrounding lung transplantation and create recommendations for pre-transplant evaluation of infections and post-transplant care. However, lung transplantation has changed a lot in recent years and new care guidelines are being planned.

Care Guidelines

Information for People with CF and their Families

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Updated 11/05/13

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