Cystic Fibrosis

Health Management

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Sweat Test

The sweat test is a simple diagnostic procedure that measures the amount of chloride (salt) in the sweat. It is the gold standard for diagnosing CF.


The basic daily care program varies to suit individual needs. The following are some common pulmonary therapy treatments:

  • An inhaled medication to open the lung passages
  • An airway clearance technique to mobilize the thick mucus from the lungs
  • Medications to treat infection or help thin mucus
  • CFTR Modulators

Common Inhaled Medications

The Cystic Fibrosis Foundation recommends the following sequence for inhaled medications:
1. Bronchodilators to open the airways
2. Hypertonic saline (7%) to mobilize mucus and improve airway clearance
3. Pulmozyme (DNase) to thin mucus
4. Airway clearance technique: Vest, Acapella, Aerobika, chest physical therapy, etc.
5. Antibiotics (tobramycin, TOBI Podhaler, Colistin, Cayston); the previous therapies open and clear the airways of mucus, allowing these antibiotics to work on remaining bacteria

Common Airway Clearance Techniques

  • Chest physical therapy uses cupped hands to clap on the back and chest.
  • Percussor: This handheld device helps mobilize bronchial secretions by manually or mechanically “clapping” the chest wall.
  • TheraPEP.
  • Acapella or Aerobika: This pocket device provides positive expiratory pressure (PEP) therapy. Exhaling through your mouth into the PEP device with a moderate force produces oscillation. Oscillation is transmitted throughout the airways, loosening secretions. The force of exhalation helps mobilize secretions.
  • Vest: Known as high-frequency chest compression, this device is worn like a vest. It works in 2 ways: The chest wall is vibrated to break up sputum, then chest wall oscillation causes outward airflow, like a miniature cough.

Pulmonary Exacerbations

Patients with CF can have an increase in their respiratory symptoms leading to a pulmonary exacerbation. Pulmonary exacerbations have very important consequences in long-term health, quality of life, and survival. Therefore, we recommend prompt and aggressive treatment with a multidisciplinary approach.

Signs and symptoms of pulmonary exacerbation may include:

  • Any change in frequency or severity of cough
  • Any change in mucus production
  • Coughing up blood, even a small amount in streaks or flecks in the mucus
  • New noises made by the lungs (wheezes, crackles, “chest congestion”)
  • Shortness of breath while sitting, walking, climbing stairs, or exercising
  • Feeling tired or “just not feeling well”
  • Not eating well or not feeling hungry
  • Losing weight or not gaining weight (for children) between clinic visits
  • Fevers, even low-grade fevers
  • Small decreases in lung function over 3 to 6 months
  • New findings on chest X-rays

Treatment for a mild pulmonary exacerbation includes an increase in breathing and airway clearance treatments (chest physical therapy or vest) and antibiotics (oral, sometimes intravenous). If an exacerbation is severe, then an admission to the hospital or home IV antibiotics may be needed.

Timely recognition and appropriate treatment are vital to preserving lung function and optimal health for long-term survival.