cystic fibrosis

cystic fibrosis

What You Need to Know

What Is Cystic Fibrosis?

Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that:
clogs the lungs and leads to life-threatening lung infections; and
obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.

In the 1950s, few children with cystic fibrosis lived to attend elementary school. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond.

Symptoms of Cystic Fibrosis

People with CF can have a variety of symptoms, including:
very salty-tasting skin;
persistent coughing, at times with phlegm;
frequent lung infections;
wheezing or shortness of breath;
poor growth/weight gain in spite of a good appetite; and
frequent greasy, bulky stools or difficulty in bowel movements.

Statistics
About 1,000 new cases of cystic fibrosis are diagnosed each year.
More than 70% of patients are diagnosed by age two.
More than 45% of the CF patient population is age 18 or older.
The predicted median age of survival for a person with CF is more than 37 years.

The Cystic Fibrosis Foundation

Since 1955, the Cystic Fibrosis Foundation has been the driving force behind the pursuit of a cure. Thanks to the dedication and financial backing of our supporters--patients, families and friends, clinicians, researchers, volunteers, individual donors, corporations and staff, we are making a difference.

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.



Cystic Fibrosis
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What causes cystic fibrosis?
Who is at risk for cystic fibrosis?
What are the signs and symptoms of cystic fibrosis?
How is cystic fibrosis diagnosed?
How is cystic fibrosis treated?
Living with cystic fibrosis
Cystic Fibrosis At A Glance
Patient Discussions: Cystic Fibrosis - Describe Your Experience

What is cystic fibrosis?


Cystic fibrosis (CF) is an inherited disease of your mucus and sweat glands. It affects mostly your lungs, pancreas, liver, intestines, sinuses, and sex organs.

Normally, mucus is watery. It keeps the linings of certain organs moist and prevents them from drying out or getting infected. But in CF, an abnormal gene causes mucus to become thick and sticky.

The mucus builds up in your lungs and blocks the airways. This makes it easy for bacteria to grow and leads to repeated serious lung infections. Over time, these infections can cause serious damage to your lungs.

The thick, sticky mucus can also block tubes, or ducts, in your pancreas. As a result, digestive enzymes that are produced by your pancreas cannot reach your small intestine. These enzymes help break down the food that you eat. Without them, your intestines cannot absorb fats and proteins fully.

As a result:

Nutrients leave your body unused, and you can become malnourished.

Your stools become bulky.

You may not get enough vitamins A, D, E, and K .

You may have intestinal gas, a swollen belly, and pain or discomfort.

The abnormal gene also causes your sweat to become extremely salty. As a result, when you perspire, your body loses large amounts of salt. This can upset the balance of minerals in your blood. The imbalance may cause you to have a heat emergency.

CF can also cause infertility (mostly in men).

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

Respiratory failure is the most common cause of death in people with CF.

Until the 1980s, most deaths from CF occurred in children and teenagers. Today, with improved treatments, people with CF live, on average, to be more than 35 years old. Research continues to look for:

Better treatments