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24

May

2018

Cystic Fibrosis

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What Is Cystic Fibrosis?

Cystic fibrosis (SIS-tik fi-BRO-sis), or CF, is an inherited disease of your secretory glands, including the glands that make mucus and sweat.

“Inherited” means that the disease is passed through the genes from parents to children. People who have CF inherit two faulty CF genes—one from each parent. The parents likely don’t have the disease themselves.

CF mostly affects the lungs, pancreas, liver, intestines, sinuses, and sex organs.

Overview

Mucus is a substance made by the lining of some body tissues. Normally, mucus is a slippery, watery substance. It keeps the linings of certain organs moist and prevents them from drying out or getting infected. However, if you have CF, your mucus becomes thick and sticky.

The mucus builds up in your lungs and blocks your airways—the tubes that carry air in and out of your lungs. 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 your lungs.

The thick, sticky mucus also can block tubes, or ducts, in your pancreas. As a result, the digestive enzymes that your pancreas makes can’t reach your small intestine.

These enzymes help break down the food that you eat. Without them, your intestines can’t fully absorb fats and proteins. This can cause vitamin deficiency and malnutrition because nutrients leave your body unused. It also can cause bulky stools, intestinal gas, a swollen belly from severe constipation, and pain or discomfort.

CF also causes your sweat to become very salty. As a result, your body loses large amounts of salt when you sweat. This can upset the balance of minerals in your blood and cause a number of health problems. Examples include dehydration (a condition in which your body doesn’t have enough fluids), increased heart rate, tiredness, weakness, decreased blood pressure, heat stroke, and, rarely, death.

If you or your child has CF, you’re also at increased risk for diabetes or a bone-thinning condition called osteoporosis. CF also causes infertility in men, and it can make it harder for women to get pregnant.

Outlook

The symptoms and severity of CF vary from person to person. Some people who have CF have serious lung and digestive problems. Other people have more mild disease that doesn’t show up until they’re adolescents or adults.

The symptoms and severity of CF also vary over time. Sometimes, you will have few symptoms. Other times, your symptoms may become more severe. As the disease gets worse, you will have more severe symptoms more often.

Lung function often starts to decline in early childhood in people who have CF. Over time, permanent damage to the lungs can cause severe breathing problems. Respiratory failure is the most common cause of death in people who have CF.

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, fifties, or older.

Early treatment for CF can improve both your quality of life and lifespan. Such early treatment includes nutritional and respiratory therapies, medicines, exercise, and other treatments.

Source: National Heart, Lung and Blood Institute