Question 1

What is cystic fibrosis?

Accepted Answer

Cystic fibrosis is an inherited disorder that affects many organs in the body, including the lungs, liver, pancreas, urinary tract, reproductive tract and sweat glands. Specific cells in these organs normally produce mucus and other watery secretions. In cystic fibrosis, these cells produce secretions that are thicker than normal, causing disruptions in the body's water balance and ability to handle salt. This creates other problems. For example, in the lungs, thickened secretions trap germs, causing repeated lung infections.

Question 2

What are the symptoms of cystic fibrosis?

Accepted Answer

The symptoms of cystic fibrosis usually appear within the first year of life, but may not appear until late childhood or adolescence. The severity of symptoms varies from person to person. Some people have severe symptoms involving various parts of the body; others have much milder disease involving only one area of the body. At birth, a child with cystic fibrosis can develop a swollen belly and vomiting due to a condition called meconium ileus, in which the bowel is plugged up with meconium, a thick, dark, sticky substance produced by the baby's intestine while still in the uterus.

Question 3

How is cystic fibrosis diagnosed?

Accepted Answer

Your doctor may suspect cystic fibrosis based on your child's symptoms and medical history, a family history of cystic fibrosis, and the results of a physical examination. To confirm his or her suspicions, your doctor will order a sweat test, a painless procedure that can diagnose cystic fibrosis by measuring the amount of salt in skin sweat. In newborns, who are too tiny to produce enough sweat for a sweat test, a blood test can be done to confirm the diagnosis. Many states now routinely screen newborns for cystic fibrosis.

Question 4

Can cystic fibrosis be prevented?

Accepted Answer

There is no way to prevent cystic fibrosis. People with a family history of cystic fibrosis can seek genetic counseling to determine their chance of passing this disease on to their children. A blood test can detect most, but not all, of the genetic mutations that cause cystic fibrosis.

Question 5

How is cystic fibrosis treated?

Accepted Answer

Treatment for cystic fibrosis depends on the severity of the disease and which parts of the body are affected. Regular monitoring is essential. Lung disease can be treated with antibiotics, mucus-thinning drugs and chest physical therapy, a form of physical therapy in which another person drums the patient's chest and back with cupped hands to help clear mucus from the lungs. Older patients sometimes can do this therapy themselves, and machines are now available that can vibrate the chest to help loosen mucus. In severe cases, a lung transplant may be an option. Digestive problems are treated with high-calorie diets, pancreatic enzymes and vitamin supplements.

Question 6

When should you see a doctor about cystic fibrosis?

Accepted Answer

Call your doctor if your child has symptoms suggestive of cystic fibrosis, including frequent respiratory infections, persistent abdominal discomfort and gas, repeated episodes of diarrhea, greasy foul-smelling stools or poor weight gain in spite of a good appetite.

Question 7

What is the outlook for cystic fibrosis?

Accepted Answer

The life expectancy of people with cystic fibrosis depends on the severity of disease and the parts of the body involved. Lung disease has the strongest impact on the severity of the illness, and death usually is caused by lung complications. Between 1 percent and 2 percent of people with cystic fibrosis are affected only mildly, with minimal lung disease or no evidence of pancreas dysfunction. With regular monitoring and improved treatment, the average life span of a person with cystic fibrosis has increased to 32 years, although some people with mild symptoms live much longer.

Cystic Fibrosis

Key points

What Is It?

Symptoms

Explore health topics

More in this topic

Polycystic kidney disease

Cystic Fibrosis

Key points

What Is It?

Symptoms

Explore health topics

More in this topic

Polycystic kidney disease

Diagnosis

Expected Duration

Prevention

Treatment

When To Call A Professional

Prognosis

Cysticercosis

Cystic acne