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Cystic Fibrosis

TRANSCRIPT

Dr. Green
Cystic fibrosis is an inherited disease of the secretory glands, which include glands that make mucus and sweat. Dr. Mansfield, can you tell us more about cystic fibrosis?

Dr. Mansfield
Absolutely, Dr. Green. People who have cystic fibrosis inherit two faulty genes, or a genetic mutation, for the disease -- one from each parent. -- However, it often happens that the parents don't have the disease themselves.

Normally, mucus is a slippery, watery substance that keeps the linings of certain organs moist and prevents them from drying out or getting infected. But in cystic fibrosis, the mucus becomes thick and sticky. As a result, it builds up in the lungs and blocks the airways.

In addition, the buildup of mucus makes it easy for bacteria to grow, which can lead to repeated, serious lung infections. Over time, these infections can severely damage the lungs.

Cystic fibrosis can also lead to problems in other parts of the body, including:

  • The pancreas
  • Liver
  • Intestines
  • Sinuses, and
  • Sex organs

Although there is no cure for cystic fibrosis, treatment can effectively prevent and control complications. Treatments may include:

  • Chest physical therapy, which focuses on techniques to help loosen mucus
  • Aerobic exercise
  • Medications, which may include antibiotics, anti-inflammatory medicines, bronchodilators, or medicines to help clear mucus
  • Pulmonary rehabilitation
  • Oxygen therapy for advanced disease, or
  • A lung transplant for severe disease