Cystic Fibrosis and Lung Disease

Cystic fibrosis (CF) is a severe, genetically-determined disease involving the lungs, the gastrointestinal tract, and other organs. 

Key info

  • Cystic Fibrosis is a genetic disease causing the build up of thick, sticky mucus in membranes of the body’s organs.
  • Cystic Fibrosis can be treated, but not cured medically
  • Lung transplant is recommended in severe cases among those who are eligible

With cystic fibrosis, the body produces abnormally thick, sticky mucus. This mucus clogs the lungs, causing life-threatening lung infections, and obstructs the pancreas, impairing the ability of the intestines to break down food.

Cystic fibrosis occurs in about 1 in every 3,400 US births. Boys and girls get cystic fibrosis equally. At present, the predicted median age for survival for a person with CF is 37 years old. However, that age is rising as an increasing number of people are only mildly affected and survive longer.

Children with cystic fibrosis typically have frequent pulmonary infections. Since cystic fibrosis affects the glands that secrete sweat, they may also suffer from salt depletion while sweating during hot weather.

Typical features of acute cystic fibrosis include:

  • Fever
  • Cough
  • Difficulty in breathing
  • Fast respiration
  • Flaring of the nostrils
  • Poor appetite
  • Reduced activity 

Cystic fibrosis is typically diagnosed in newborns through screening blood tests and genetic testing for mutations in the CFTR genes. A sweat test, which measures the amount of salt in perspiration, is another tool for diagnosing cystic fibrosis. If that sweat test is positive, it should be confirmed with a second test.

Medical Treatment for Cystic Fibrosis

Cystic fibrosis is often treated with antibiotics, as well as through special exercises for draining mucus, including physical therapy for the chest, physical exercise, and aerosols. Diet therapy, which emphasizes the replacement of deficient digestive enzymes, is also critically important.

Some of the tenacity of the mucus and secretions results from increased amounts of DNA. An inhaled medication, Dornase alfa (brand name Pulmozyme) contains an enzyme that breaks down the DNA, thereby liquefying secretions and making them easier to clear.

Surgical Treatment for Cystic Fibrosis

Although current medical management strategies can control the disease into adulthood, lung transplantation offers extended survival in to those with advancing pulmonary disease.

Next Steps

If you need help for a lung or chest issue, we’re here for you. Call (212) 305-7771 or request an appointment online to get started today.

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