Cystic Fibrosis Disease

Cystic fibrosis is a serious genetic disorder with reduced life expectancy. A common genetic disorder among Caucasian people, this disease occurs in one of every 3,200 Caucasian births. The mutation in the gene varies according to the geographical background. Cystic fibrosis occurs in one out of every 15,000 African-American births. The life expectancy of cystic fibrosis patients has been increasing over the past 40 years. In the 1980s life expectancy of people with cystic fibrosis was 14 years.
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10 years back, the life expectancy of a person with cystic fibrosis was around 18 years. Today it is 35 years. The median life expectancy of a newborn with cystic fibrosis has increased from 4 years to 32 years. It has been estimated that there has been more than 10 year increase in the average life expectancy of affected people in North America. Over 90% of the affected infants now survive beyond one year. Studies show that life expectancy of children will exceed 40 years. Life expectancy of individuals with adequate pancreatic function can be more than 50 years.

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.

Cystic Fibrosis Symptoms

Diagnosis of cystic fibrosis is confirmed by a sweat test or DNA testing. Cystic fibrosis causes the sweat glands to secrete excessive salt. The most common symptoms of cystic fibrosis are coughing and chronic breathing difficulties. This causes repeated lung infections. The lungs and the digestive system are more affected by cystic fibrosis. The symptoms of cystic fibrosis include chronic cough, chest infection, small growth in nose, diabetes, weight loss, pancreatitis, and diarrhea. The treatment of cystic fibrosis includes oral or inhaled antibiotics, corticosteroid tablets, dietary vitamins, anti-asthma therapy and medication.

Supplements Important to Cystic Fibrosis Patients

Malabsorption of fat soluble vitamins is likely in most patients with cystic fibrosis, especially for those who are pancreatic insufficient. Vitamins A, D and E have been found to be deficient in early diagnosed infants as well as older patients. Different levels of these vitamins are prescribed depending on the age of the patient, whether the patient is pancreatic insufficient or sufficient and other health factors that can be affected by cystic fibrosis. Vitamin K may also be prescribed under certain conditions.

The recommended daily supplements which usually achieve normal plasma levels in infancy are vitamin A 4000 iu (120 mcg), vitamin D 400 iu (10 mcg) and vitamin E 37 - 75 iu (25 - 50 mg). The recommended doses for children over 1 year of age are vitamin A 8000 iu, vitamin D 800 iu and vitamin E 100 - 200 mg.

Cystic fibrosis (CF) is one of the most common genetic diseases.