About Cystic Fibrosis

Cystic fibrosis is a chronic inherited disease that affects the lungs and digestive system. The Cystic Fibrosis Foundations states there are 30,000 people with CF in the United States, and 70,000 throughout the world. Few children with cystic fibrosis lived to attend high school in the 1950's, but now advances in medicine have prolonged the life span of those with the condition, allowing some to live into their 40's or longer.

Cause:

A person with cystic fibrosis inherits a defective gene, one from each parent. The parents usually do not have the condition themselves. These defective genes cause the secretory glands to work improperly. Instead of watery, slippery mucus that keeps organ linings moist, a person with CF will have thick and sticky mucus that allows for blockages, infection and mineral imbalances in the body.

Complications:

Lungs- Mucus buildup in the lungs makes great breeding ground for bacterial infections, which leads to serious lung damage over time.

Pancreas- Sticky mucus also causes blockages in ducts leading from the pancreas. Much needed enzymes the pancreas makes are then not able to reach the small intestine.

Intestines- Since the pancreatic enzymes do not reach the small intestine, fat and protein absorption is inadequate, which leads to vitamin deficiency and malnutrition, along with gastrointestinal problems.

Minerals- The sweat of cystic fibrosis patients is very salty. This leads to lack of minerals the body needs and can cause dehydration, heat stroke, tiredness, low blood pressure, and sometimes death.

Considerations:

Cystic fibrosis puts people at greater risk of developing diabetes and osteoporosis. It also affects fertility in box sexes.

Symptoms:

Symptoms associated with cystic fibrosis include salty tasting skin, a persistent cough, shortness of breath, wheezing, infections in the lungs, poor weight gain and growth in children, and bowel problems such as gassy, bulky stools.

Treatment:

There is no cure for cystic fibrosis but treatment options are available to improve quality of life for those with the condition. Bronchodilators to help with breathing and antibiotics to fight lung infections, along with breathing exercises to dislodge mucus are used for some with the condition. Physical therapy using percussion techniques and other devices to help with breathing and mucus breakup are also employed. For patients in whom the disease affects the digestive system, special diet, vitamins and enzyme replacement are called for. Heart, lung, or heart and lung transplants have been used to treat some patients with cystic fibrosis. Whatever treatment plan, people with the condition must keep regular appointments with their medical care team and stay on top of the disease for optimal care.

http://www.cff.org/AboutCF/

http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_what.html

http://www.yourlunghealth.org/lung_disease/cystic_fibrosis/treatment/index.cfm

http://www.nlm.nih.gov/medlineplus/cysticfibrosis.html#cat3