What Is Cystic Fibrosis?

by Aimee Amodio | More from this Blogger

25 Jul 2007 08:28 PM

Cystic fibrosis (CF) is a progressive disorder that affects many systems throughout your body, namely the respiratory system and the digestive system. This is an inherited disease of the mucus glands.

You know what mucus is, right? That slippery stuff that lubricates (and protects) your airways, digestive system, reproductive system, and other organs and tissues. For people with cystic fibrosis, the mucus the body produces is extra thick and sticky. It can obstruct airways, causing serious breathing problems and potential for bacterial infections in the lungs. The infections cause coughing, wheezing, and inflammation; the infections and the mucus buildup can cause permanent lung damage. This is where the disease gets its name: cysts and scar tissue (fibrosis) can form in the lungs over time.

Many people with cystic fibrosis also have digestive problems. The mucus interferes with pancreatic function by blocking ducts and keeping digestive enzymes from getting to the intestines. This can lead to diarrhea, malnutrition, weight loss, and poor growth. Some babies with cystic fibrosis experience a blockage of the intestines shortly after birth.

Men with cystic fibrosis are often infertile, thanks to mucus blocking the vas deferens. Women with CF may be infertile as well, but the problem is less common in women than in men. The disease is most often seen in the Caucasian population -- approximately one in 3200 children are born with the disease. The disease is much rarer in African Americans and Asian Americans.

CF is hereditary. The gene is recessive, so parents of an individual with cystic fibrosis may each carry the gene without showing any signs or symptoms of having CF themselves.

Early diagnosis and a thorough treatment plan can improve quality of life. Your doctor may prescribe antibiotics for respiratory infections, pancreatic enzymes to aid in digestion, vitamin supplements, and inhaled medications to help open airways. Other therapies are available to help thin the mucus (making it easier to cough up) or slow lung deterioration. Children ages five to thirteen have shown promising results when treated with ibuprofen. In some cases, a lung transplant is an option.

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