CYSTIC FIBROSIS (CF) |
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DESCRIPTION
Cystic fibrosis is an inherited disease in which mucus-producing glands throughout the body--especially in the pancreas and lung -- fail to produce normal enzymes and overproduce mucus. The pancreas, lungs, sweat glands of the skin, and gastrointestinal tract are involved. Cystic fibrosis affects children of both sexes. It develops soon after birth.
Appropriate health care includes: home care after diagnosis; physician's monitoring of general condition and medications; psychotherapy or counseling to help parents and children adjust; surgery to relieve intestinal obstruction (sometimes); hospitalization to control serious infections, which occur frequently.
SIGNS & SYMPTOMS
Newborn period:
Thick, bulky stools, which may cause intestinal obstruction.
Later:
Poor weight gain despite good appetite.
Chronic cough.
Frequent, severe respiratory infections.
Thick, viscuous mucus secretions.
Salty sweat.
Enlarged liver and spleen, in some instances.
CAUSES
Genetic factors. Many people carry the genes for cystic fibrosis, and 1 in 2000 newborns is born with it. The genes cause abnormal mucus in the respiratory and gastrointestinal tracts and sweat glands. This causes lung obstruction and infection, poor digestion, and poor food absorption.
RISK FACTORS
Family history of cystic fibrosis. If both parents come from families with cystic fibrosis, the changes are 1 in 4 that each child will have the disease.
PREVENTING COMPLICATIONS OR RECURRENCEIf you have a family history of cystic fibrosis, seek genetic counseling before starting a family.
BASIC INFORMATION
MEDICAL TESTS
Your own observation of symptoms. Sometimes a parent notes a salty taste when kissing a child.
Medical history and physical exam by a doctor; laboratory studies to analyze sweat, stools, and digestive juices.
POSSIBLE COMPLICATIONS
Pneumonia; chronic bronchitis; bronchiectasis; fluid and electrolyte imbalance -- especially in hot weather; malnutrition; nasal polyps; rectal prolapse.
PROBABLE OUTCOME
Although this condition is currently considered incurable, careful long-term care by parents and professionals can help children lead reasonably comfortable lives. The current average life expectancy is 30.1 years, but many people live longer than this.
TREATMENT
HOME CARE
Learn as much as possible about this condition. Diet, medication, and early recognition of infection are very important.
You will need to perform daily postural drainage to drain mucus from the child's lungs, and chest percussion to shake loose sticky mucus plugs. Ask your doctor for instructions.
Keep your child's immunizations, including pneumonia and influenza vaccines, up to date. An immunization schedule appears in Appendix 1.
Join a support group for parents of children with cystic fibrosis.
Encourage your child to lead as normal and active a life as possible.
MEDICATION
Your doctor may prescribe:
Digestive enzymes.
Antibiotics when lung infections occur.
Aerosolized mucus-thinning agents and/or antibiotics by nebulizer to help loosen lung secretions and control infections.
ACTIVITY
As much as the condition permits.
DIET & FLUIDS
Your child should eat a low-fat diet with adequate protein. Consult a dietitian for specific instructions. Vitamin and mineral supplements may be necessary. Encourage intake of liquids, which helps thin mucus.
OK TO GO TO SCHOOL?Yes, activities should be limited only during periods when signs of infection have increased, appetite decreases, and alertness, strength, and feeling of well-being appear diminished.
CALL YOUR DOCTOR IF
You suspect your child has cystic fibrosis.
After diagnosis, your child develops fever, a worsening cough, change in level of tiredness, or decreased appetite.
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