What is it?
Cystic fibrosis or CF, is an inherited disease of the secretory glands. Secretory glands include glands that make mucus and sweat. “Inherited,” means the disease is passed from parents to children through genes. People who have CF inherit two faulty genes for the disease—one from each parent. The parents likely don’t have the disease themselves. CF mainly affects the lungs, pancreas, liver, intestines, sinuses, and sex organs.
Risks and symptoms
Mucus is a substance made by tissues that line some organs and body cavities, such as the lungs and nose. Normally, mucus is a slippery, watery substance. It keeps the linings of certain organs moist and prevents them from drying out or getting infected. In someone with CF, the mucus becomes thick and sticky. It builds up in the lungs and blocks the airways. The buildup of mucus makes it easy for bacteria to grow. This leads to repeated, serious lung infections. Over time, these infections can severely damage the lungs. The thick, sticky mucus also can block tubes, or ducts, in the pancreas (an organ in the abdomen). As a result, the digestive enzymes that the pancreas makes can’t reach the small intestine. These enzymes help break down food. Without them, the intestines can’t fully absorb fats and proteins. This can cause vitamin deficiency and malnutrition because nutrients pass through the body without being used. CF patients may also have bulky stools, intestinal gas, a swollen belly from severe constipation, and pain or discomfort.
CF also causes a person’s sweat to become very salty. Thus, when one sweats, larger than normal amounts of salt are lost. This can upset the balance of minerals in the blood and cause many health problems. Examples of these problems include dehydration (a lack of fluid in the body), increased heart rate, fatigue (tiredness), weakness, decreased blood pressure, heat stroke, and, rarely, death.
People with CF, are also at higher risk for diabetes or a bone-thinning condition called osteoporosis. CF also causes infertility in men, and the disease can make it harder for women to get pregnant.
The symptoms and severity of CF vary. Someone with the disease may have serious lung and digestive problems. If the disease is mild, symptoms may not show up until the teen or adult years. The symptoms and severity of CF also vary over time. Sometimes one will have few symptoms. Other times, the symptoms may become more severe. As the disease gets worse, one may develop more severe symptoms more often. Lung function often starts to decline in early childhood in people who have CF. Over time; damage to the lungs can cause severe breathing problems. Respiratory failure is the most common cause of death in people who have CF.
Treatment
As treatments for CF continue to improve, so does life expectancy for those who have the disease. Today, some people who have CF are living into their forties or fifties, or longer. Early treatment for CF can improve quality of life and increase ones lifespan. Treatments may include nutritional and respiratory therapies, medicines, exercise, and other treatments.