Cystic fibrosis is a life-threatening disorder that causes severe lung damage and nutritional deficiencies.
An inherited condition, cystic fibrosis affects the cells that produce mucus, sweat and digestive juices. Normally, these secretions are thin and slippery, but in cystic fibrosis, a defective gene causes the secretions to become thick and sticky. Instead of acting as a lubricant, the secretions plug up tubes, ducts and passageways, especially in the pancreas and lungs.
Cystic fibrosis happens most often in white people of northern European ancestry, occurring in about 1 out of 3,000 live births. In the past, most people with cystic fibrosis died in their teens. Improved screening and treatments now allow many people with cystic fibrosis to live into their 50s or even longer.
In cystic fibrosis, the lungs fill with thick mucus — a perfect breeding ground for bacteria and viruses. Most people with cystic fibrosis have almost constant infections in their lungs and ...
Cystic fibrosis signs and symptoms can vary from child to child, depending on the severity of the disease. Even in the same child, symptoms may worsen or improve as time passes. In some children, symptoms begin during infancy. Other people may not begin experiencing symptoms until adolescence or adulthood.
One of the first signs of cystic fibrosis is an excessively salty taste to the skin. People with cystic fibrosis tend to have higher than normal amounts of salt in their sweat. Parents often can taste the salt when they kiss their child.
Most of the other signs and symptoms of cystic fibrosis affect the respiratory system or the digestive system.
Respiratory signs and symptoms
Digestive signs and symptoms
When to see a doctor
Seek immediate medical care if your child has difficulty breathing.
In cystic fibrosis, a defective gene alters a protein that regulates the normal movement of salt (sodium chloride) in and out of cells. This results in thick, sticky secretions in the respiratory and digestive tracts, as well as in the reproductive system. It also causes increased salt in sweat.
The affected gene, which is inherited from a child's parents, is a recessive gene. With recessive genes, children need to inherit two copies of the gene, one from each parent, in order to have the disease. If children inherit only one copy, they won't develop cystic fibrosis, but will be carriers and possibly pass the gene to their own children.
Many different types of complications can occur with cystic fibrosis. The most common complications affect the respiratory, digestive and reproductive systems.
Respiratory system complications
Digestive system complications
Reproductive system complications
Although women with cystic fibrosis may be less fertile than other women, it's possible for them to conceive and to have successful pregnancies. Still, pregnancy can worsen the signs and symptoms of cystic fibrosis, so be sure to discuss the possible risks with your doctor.
Collapsed and normal lung
In a collapsed lung, air from the lung leaks into the chest cavity. A collapsed lung is a serious condition that can be life-threatening. The example shown is a complete left pneumothorax. Sometimes ...
Preparing for your appointment
While you may initially bring your concerns to the attention of your family doctor, he or she may refer you to a doctor who specializes in the treatment of cystic fibrosis.
What you can do
What to expect from your doctor
Tests and diagnosis
Lung function tests
Treatments and drugs
There is no cure for cystic fibrosis, but treatments can ease symptoms and reduce complications.
There also are mechanical devices that help loosen lung mucus. They include:
Surgical and other procedures
Lifestyle and home remedies
There are many things you can do at home to reduce the chances for developing cystic fibrosis complications.
Oral enzymes and better nutrition
Drink lots of fluids
Keep immunizations up to date
Encourage hand washing
Coping and support
If you or someone you love has cystic fibrosis, you may experience strong emotions such as anger or fear. These issues are especially common in teens. Talking openly about how you feel can help. It may also help to talk with others who are dealing with the same issues.
That might mean joining a support group for parents of children with cystic fibrosis. Older children with the disorder may want to join a cystic fibrosis group to meet and talk with others who have the disease. Psychologists are often an important part of the care team as the child approaches the teen years.
If you or your partner has close relatives who have cystic fibrosis, you both might want to undergo genetic testing before having children of your own. The test, which is performed in a lab on a sample of blood or saliva, can help determine your risk of having a child with cystic fibrosis.
If you're already pregnant and the test shows that your baby may be at risk of cystic fibrosis, your doctor can conduct additional tests on your developing child. The results of these tests can help you decide if you want to continue the pregnancy.
Genetic testing isn't for everyone. Before you decide to be tested, you should talk to a genetic counselor about the psychological impact the test results might carry.
Last Updated: 2010-03-02
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