What is Cystic Fibrosis?

According to the Cystic Fibrosis Foundation, 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; and
• obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.

In the 1950s, few children with cystic fibrosis lived to attend elementary school. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond.

Symptoms of Cystic Fibrosis

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

• very salty-tasting skin;
• persistent coughing, at times with phlegm;
• frequent lung infections;
• wheezing or shortness of breath;
• poor growth/weight gain in spite of a good appetite; and
• 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.

The Most Common Problems in CF

The two most common problems in CF, partly caused by abnormal mucus, involve the lungs (respiratory system) and the digestive system. The body makes mucus as a way to keep itself lubricated and clean (rather like motor oil in a car engine). In healthy people, the lungs and the digestive tract both make mucus. In people with CF, the body makes mucus, but the mucus is different; it is much thicker and stickier. Abnormal mucus causes several problems. The thick and sticky mucus in the lungs of people with CF is hard to cough up. When mucus stays in the lungs, it makes an ideal place for bacteria to grow. This is why most people with CF eventually have lung infections.

Most people with CF also have problems digesting food, and this is a big reason they may have problems with growing. The thick, sticky mucus blocks the ducts from the pancreas to the small intestine, so that the digestive enzymes from the pancreas cannot get to the small intestine to help digest food. This is called malabsorption or pancreatic insufficiency.

Download a copy of An Introduction to Cystic Fibrosis: For Patients and Their Families,

a comprehensive book (202 pages) that covers everything from how CF is diagnosed to living with the disease as an adult.