What is cystic fibrosis?

The Cystic Fibrosis Disease and Who It Affects

Cystic Fibrosis, or CF as it is commonly called, is a disease caused by an inherited genetic defect. About 1 in 20 people in the United States carry at least one defective gene, which makes it the most common genetic defect of its severity in the United States.

 

People with CF suffer from chronic lung problems and digestive disorders. The lungs of people with CF become covered with sticky mucus, which is hard to remove and promotes infection by bacteria. Many people with CF require frequent hospitalizations and continuous use of antibiotics, enzyme supplements, and other medications.


The life expectancy of people with CF used to be very short; 30 years ago, the median life expectancy was about 8 years. Today, thanks to medical advances, the median life expectancy is in the mid-30s and increasing.

 

CF used to be known as a children’s disease, but as medical advances increase the life expectancy of people with cystic fibrosis, there are a new set of problems— going to college, getting a job, finding health insurance, building permanent relationships— all while keeping up the physical therapy and medications.

What is cystic fibrosis?

Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that:

  • clogs the lungs and leads to life-threatening lung infections
  • obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food

Symptoms of cystic fibrosis.

People with CF can have a variety of symptoms, including:

  • very salty-tasting skin
  • persistent coughing, sometimes with phlegm
  • frequent lung infections
  • wheezing or shortness of breath
  • poor growth/weight gain in spite of a good appetite and eating
  • frequent greasy, bulky stools or difficulty in bowel movements

Statistics

  • About 1,000 new cases of cystic fibrosis are diagnosed each year.
  • More than 70% of patients are diagnosed by age two.
  • More than 45% of the CF patient population is age 18 or older
  • The predicted median age of survival for a person with CF is in the late 30s