What is Cystic Fibrosis?
Cystic fibrosis (CF) is a disease of the secretory glands which produce mucus and sweat. Mucus is a substance made by the tissues that line certain organs and body cavities, such as the lungs and nose. Normally, mucus is a slippery and watery substance which keeps the linings of these organs moist and prevents them from drying out or getting infected. CF causes mucus to be thick and sticky. The mucus builds up in the lungs which clogs the airways causing breathing difficulties and increased bacteria production. The result is repeated and serious lung infections which lead to lung damage over time.
CF also affects the pancreas, liver, intestines, sinuses and sex organs. This thick and sticky mucus can block the ducts in the pancreas which prevents digestive enzymes from reaching the small intestine. These enzymes help breakdown food. Without them, fat and protein are not absorbed properly in the small intestine which leads to vitamin deficiency and malnutrition. Additionally patients experience intestinal gas, constipation and abdominal pain/swelling.
CF causes sweat to become very salty which causes patients to lose large amounts of salt. This can upset the balance of minerals in the blood which results in many health problems such as: dehydration, increased heart rate, fatigue, weakness, low blood pressure, heat stroke and in severe cases, death.
Children with CF are at higher risk of developing diabetes and bone-thinning diseases such as osteoporosis and osteopenia. CF can cause infertility (inability to have children) in men and difficulty becoming pregnant for women.