$Unique_ID{PAR00051} $Pretitle{} $Title{Medical Advice: Cystic Fibrosis} $Subtitle{} $Author{ Editors of Consumer Guide Chasnoff, Ira J} $Subject{Cystic Fibrosis frequent respiratory infections infection bronchitis pneumonia Chronic cough coughing no weight gain constipation diarrhea foul-smelling stools rectal Protrusion rectum fingertip Broadening fingertips toe Broad toes Salty skin sweat test lungs pancreas liver inherited gene inherit genes} $Log{ Secretions of Cystic Fibrosis*0005101.tif} Your Child: A Medical Guide Cystic Fibrosis Quick Reference SYMPTOMS - Frequent respiratory infections - Frequent bronchitis or pneumonia - Chronic cough - Failure to gain weight - Frequent constipation or diarrhea with foul-smelling stools - Protrusion of the rectum - Broadening of fingertips and toes - Salty taste to the skin when kissed HOME CARE - If you suspect cystic fibrosis, see your doctor. If cystic fibrosis is diagnosed, follow the doctor's instructions for home care. PRECAUTIONS - If your child shows any symptoms of cystic fibrosis, ask your doctor to perform a sweat test. - If there is any history of cystic fibrosis in your family, a sweat test should be considered for all children (even if they appear healthy). The sweat test is not reliable before the age of one month and is less reliable during adolescence. - The earlier cystic fibrosis is diagnosed and treated, the better the outcome. Cystic fibrosis is a chronic (lifelong), inherited disease. It affects the lungs, pancreas, sweat glands, and sometimes the liver and other organs. Cystic fibrosis is passed from parents to child through a particular gene. (The genes are the parts of the body's cells that determine such inherited characteristics as hair and eye color and blood type). To inherit cystic fibrosis, a child must receive that particular abnormal gene from both parents. A child who receives the abnormal gene for cystic fibrosis from only one parent will not have the disease. A person who carries the gene but does not have the disease is known as a "healthy carrier." When both parents are healthy carriers, there is a 25 percent risk that any one of their children will receive the gene from both parents and suffer from the disease. Five percent of white persons, 2 percent of American black persons, and less than 1 percent of Oriental and native African black persons are healthy carriers. One in 2,000 white children in the United States has cystic fibrosis. SIGNS AND SYMPTOMS Cystic fibrosis may appear at birth as an obstruction or blocking of the intestines. Symptoms may also appear during infancy or childhood or sometimes as late as adolescence. Some common symptoms of cystic fibrosis are frequent respiratory infections, including bronchitis and pneumonia; chronic cough; failure to gain adequate weight; constipation or diarrhea with foul-smelling stools; protrusion of the rectum; and clubbing (broadening) of the fingertips and toes. Almost all persons with cystic fibrosis have unusually salty sweat; the first sign of cystic fibrosis is often the very salty taste of the child's skin when kissed. HOME CARE If you have any reason to suspect that your child has cystic fibrosis, see your doctor. If cystic fibrosis is diagnosed, home treatment will be directed by your doctor. Treatment at home may include taking antibiotics, following a special diet, taking pancreatic enzymes by mouth, inhaling vapor and medications, and postural drainage (a technique for placing the child in specific positions and tapping over the chest to allow excess fluids to drain from the lungs). PRECAUTIONS - If your child shows any signs of cystic fibrosis, a sweat test should be done. A sweat test is a painless, harmless, inexpensive, and generally reliable test for cystic fibrosis. - If there is any history of cystic fibrosis in the family, a sweat test should be considered for all children, even if they appear to be healthy. - The sweat test is not reliable before the age of one month and is not as reliable during adolescence. MEDICAL TREATMENT The doctor will perform a sweat test and may also order a chest x-ray study. If cystic fibrosis is diagnosed, your doctor will most likely refer you to a medical center where there are specialists in treating cystic fibrosis. The outlook for a child with cystic fibrosis who is in relatively good health is better now than in the past, but a cure for cystic fibrosis is still being sought. The earlier the diagnosis is made and treatment is started, the better the outcome.