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Cystic Fibrosis Center

Cystic Fibrosis Center
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Sweat Test

The sweat test is a simple, diagnostic procedure, which measures the amount of chloride (salt) in the sweat. It has been the gold standard for diagnosing cystic fibrosis (CF). CF is a genetic disease which affects the lungs and digestive system. Its symptoms may include chronic cough, repeated pneumonia, chronic diarrhea, and difficulty gaining weight. For more info, click here.

Treatments

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

  • First an inhaled medication to open up the lung passages
  • Then an airway clearance technique to mobilize the thick mucus from the lungs
  • Finally medications, if prescribed, to treat infection or help thin mucous

Common Inhaled Medications

The Cystic Fibrosis Foundation recommends the following sequence for inhaled medications:

  • Bronchodilators (Albuterol®, Xopenex®) to open the airways
  • Hypertonic Saline (7%) to mobilize mucus and improve airway clearance
  • Pulmozyme® (DNAse) to thin mucus
  • *Airway Clearance Technique: Vest, Acapella®, Aerobika®, Chest PT, etc.
  • Antibiotics (tobramycin, TOBI®  podhaler, Colistin®, Cayston®). The previous therapies open and clear the airways of mucus, allowing these antibiotics to work on remaining bacteria.

* When using the Vest for airway clearance, make sure there is aerosol delivery during the entire vest session.

Common Airway Clearance Techniques

  • Chest Physical Therapy: Using cupped hands to clap on the back and chest
  • Percussor: A hand-held device that assists in the mobilization of bronchial secretions by manually/mechanically “clapping” the chest wall.
  • Acapella® or Aerobika®:  A pocket device that 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 to mobilize secretions.
  • ThAIRapy® Vest: Known as high frequency chest compression (HFCC), this device is worn like a vest. It works in two 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 the long-term health, quality of life, and survival. Therefore, prompt and aggressive treatment with a multidisciplinary approach is recommended.

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 with exercise
  • Feeling tired or “just not feeling well”
  • Not eating well or not feeling hungry
  • Losing weight or not gaining weight (for children) in between clinic visits
  • Fevers, even low grade fevers
  • Small decreases in lung function over 3-6 months
  • New findings on chest X-rays

Treatment for a mild pulmonary exacerbation includes an increase in breathing and airway clearance treatments (CPT/Vest) and antibiotics (oral, sometimes intravenous).  If an exacerbation is severe, then an admission to the hospital may be needed.

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