VCU Adult Cystic Fibrosis Program

Cystic Fibrosis Overview

Cystic Fibrosis (CF) is an inherited disease of your mucus and sweat glands. It affects mostly your lungs, pancreas, liver, intestines, sinuses, and sex organs.

 

 
Symptoms and Diagnosis
The most common symptoms of CF are frequent coughing with phlegm, frequent bouts of bronchitis and pneumonia, salty-tasting skin, dehydration, poor growth, and infertility, mostly in men. A sweat test is the most common diagnostic test for CF – it measures the amount of salt in your sweat. Other tests that can be used to help diagnose CF include a chest x ray, sinus x ray, CT-scan, lung function tests, analysis of sputum cultures and/or stool samples, and genetic testing of a blood sample. Prenatal genetic testing can help you find out if your baby is likely to have CF.

Associated Health Issues
Normally, mucus is watery. It keeps the linings of certain organs moist and prevents them from drying out or getting infected. But in CF, an abnormal gene causes mucus to become thick and sticky which can have negative affects on multiple areas of health. The symptoms and severity of CF vary from person to person. Some people with CF have serious lung and digestive problems. Other people have more mild disease that doesn't show up until they are adolescents or young adults.

In people with CF, mucus builds up in the lungs and blocks the airways, creating an environment that makes it easy for bacteria to grow and leads to repeated serious lung infections. Over time, these infections can cause serious damage to the lungs. Respiratory failure is the most common cause of death in people with CF.

The mucus can also block tubes, or ducts, in your pancreas. As a result, digestive enzymes that are produced by your pancreas cannot reach your small intestine. These enzymes help break down the food that you eat. Without them, your intestines cannot absorb fats and proteins fully. Nutrients leave your body unused, and you can become malnourished. Your stools become bulky. You may not get enough vitamins A, D, E, and K. You may have intestinal gas, a swollen belly, and pain or discomfort.

The abnormal gene also causes your sweat to become extremely salty. As a result, when you perspire, your body loses large amounts of salt. This can upset the balance of minerals in your blood. The imbalance may cause you to have a heat emergency. It is extremely important, especially in hot summer days, to always stay well hydrated.

CF can also cause infertility (mostly in men).

Treatment Overview
Antibiotics are the primary treatment for lung problems in CF. They treat airway infections. Other treatments include chest physical therapy, exercise, mucus-thinning drugs, and other medications to reduce inflammation in your airways and help open them up.

Lung transplantation is an option for some people with CF. The digestive problems in people with CF can be managed with nutritional therapy, enemas, mucus- thinning drugs, and medications to reduce stomach acid.

Ongoing medical care from a team of health care providers who specialize in CF is important. Good self-management includes eating a healthy diet, avoiding tobacco smoke, exercising frequently, doing chest physical therapy every day, drinking lots of fluids, and washing your hands often to reduce your chances of infection.

Progress
CF research continues to seek better treatments and a cure. However, much progress has been made in the past 50 years. Until the 1980s, most deaths from CF occurred in children and teenagers. Today, with improved treatments, people with CF live, on average, to be more than 38 years old.

 

 
For more informtion on Cystic Fibrosis, please continue to explore the VCU Adult Cystic Fibrosis Center's Information Center.