Cystic Fibrosis

Cystic fibrosis is a medical condition that starts in early childhood, sometimes as early as from the time of birth. A defective gene causes the problem and there is no known cure, though there are some promising experiments going on towards gene therapy. The medical profession understands the symptomatic treatment well and the average lifespan of persons with cystic fibrosis is increasing.

Nature’s mechanism for filtering out the dust and microorganisms people breathe in is to secrete thin mucus in the airways and the lungs, and clear it through the nose or the digestive tract. For the person with cystic fibrosis, this mucus, which normally is thin and slippery, becomes thick and sticky. Other affected and thickened secretions are sweat, digestive juices and the reproductive system.

Since the lungs are congested, the pulmonary capacity drops. The blocked airways cause severe breathing difficulties and asthma-like wheezing. The digestive juices from the pancreas and liver do not reach the intestine, as the ducts get blocked. The fats and proteins are not digested. Though the patient eats normally, maybe even in excess, he is undernourished and is under weight. Fat-soluble vitamins become deficient.

There are approximately ten million symptomless carriers of the defective cystic fibrosis gene in America. A person needs to inherit two defective cystic fibrosis genes to be afflicted with cystic fibrosis. Every time two carriers produce a child, the chances are 25% that the child may be affected with cystic fibrosis, 50% that the child may be a carrier and 25% that the child may be a non-carrier.

To diagnose cystic fibrosis, the laboratory carries out a sweat test. The treatment is only symptomatic. We cannot at this stage make the secretions thin, but can neutralize the effects of thickened secretions by regular treatment and management. A high-fat diet with enzyme and vitamin supplements helps the patient with nutrition.

A strict lifestyle regime to prevent complications is very important. Drinking plenty of liquid loosens the mucus. Regular exercise, as much as possible, keeps the respiratory system clear and improves the cardiovascular system. The cystic fibrosis patient must avoid smoky or dusty places. Hand cleaning is a simple but very effective step to prevent infection.

Few cystic fibrosis patients lived beyond their teens in the past, but thanks to improved management, 40% of the 30,000 Americans with cystic fibrosis are over 18 and many are into their 30s and 40s.