Cystic Fibrosis

Overview

Cystic fibrosis (CF) is an inherited disease of the mucus and sweat glands. It affects mostly your lungs, pancreas, liver, intestines, sinuses, and sex organs. CF causes your mucus to be thick and sticky. The mucus clogs the lungs, causing breathing problems and making it easy for bacteria to grow. This can lead to repeated lung infections and lung damage.

The symptoms and severity of CF can vary. Some people have serious problems from birth. Others have a milder version of the disease. CF is diagnosed through various tests, such as gene, blood, and sweat tests.

There is no cure for CF, but treatments have improved greatly in recent years. In the past, most deaths from CF were in children and teenagers. Today, with improved treatments, some people who have CF are living into their forties, fifties, or older.1

In the United States:2

  • 30,000 children and adults have cystic fibrosis
  • 1,000 new cases are diagnosed each year
  • More than 75% of people with CF are diagnosed by age 2
  • More than half of the CF population is age 18 or older

Treatment

The goals of CF treatment include:3

  • Preventing and controlling lung infections
  • Loosening and removing thick, sticky mucus from the lungs
  • Preventing or treating blockages in the intestines
  • Providing enough nutrition
  • Preventing dehydration (a lack of fluid in the body)

Treatment may include:2,4

  • Airway clearance — to help loosen and get rid of the thick mucus that can build up in the lungs
  • Inhaled medicines — to open the airways or thin the mucus
  • Pancreatic enzyme supplement — capsules to improve the absorption of vital nutrients
  • CFTR Modulators — correct the function of the defective protein made by the CF gene

References

1.https://medlineplus.gov/cysticfibrosis.html#summary Accessed 8/29/17

2.https://www.cff.org/What-is-CF/About-Cystic-Fibrosis/ Accessed 8/29/17

3.https://www.nhlbi.nih.gov/health/health-topics/topics/cf/treatment Accessed 8/29/17

4.https://www.cff.org/Life-With-CF/Treatments-and-Therapies/CFTR-Modulator-Therapies/ Accessed 8/29/17