How to Treat Cystic Fibrosis


Cystic fibrosis is genetically an inherit­ed condition in which the mucus glands produce an abnormal­ly thick, sticky mucus in the lungs, pancreas, and bowel. A scientific breakthrough gave hope to thousands of families with children suffering this disease when researchers identified the exact gene responsible. However, a cure is still distant. Cystic fibrosis affects approximately one person in 1,500 to 2,000. It is one of the most common genetic diseases in the western world.

While many people carry the gene responsible for cystic fibrosis, they are completely unaffected. Should their partner also be a carrier—and there is a 1 in 400 chance that pairing will occur—then the couple has a 1 in 4 chance of having a child with cystic fibrosis; a 2 in 4 chance of having a child who is a carrier, but who doesn’t have the disease; and a 1 in 4 chance of having a child who is neither a carrier nor has the disease. The cystic fibrosis gene is not sex-linked, so boys and girls are equally affected.

Without treatment, cystic fibrosis can be a killer. The child’s bronchial tubes become clogged, leaving him prone to pneu­monia and other lung infections. Digestion is also affected, because the enzymes that normally flow from the pancreas into the small intestine are prevented from doing so. In the 1950s, when effective treatment wasn’t available, most children with cystic fibrosis died before reaching adulthood. Medical advances in the past forty years mean that approximately 70 percent of children with the disease survive into adult life, and sustain rel­atively little damage to their lungs.

Treatment for the condition has two essential components:

  • Physiotherapy. Treatment must be carried out regularly each day in order to keep the child’s lungs clear of mucus. Although initially treatment is provided by a qualified physiotherapist, eventually it can be carried out by the par­ents and child working together at home.
  • Enzyme substitutes to compensate for the pancreas problem. Often, the child takes a very large number of pills at meal­times, in conjunction with vitamin supplements and a high-protein diet.

Parents vary in their reaction to the diagnosis of cystic fibrosis in their child. Some become overprotective and isolate their child from peers as a precaution against infection. Others encourage their child to lead a normal life. A child with cystic fibrosis is usually able to attend a day-care center or nursery school; the only difficulty may be that the child will need phys­iotherapy around lunchtime, carried out at home. School staff should be aware that a child with cystic fibrosis is not fragile, although they should exercise caution if the child becomes breathless and starts to wheeze.

Filed Under: Health & Personal Care

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About the Author: Andrew Reinert is a health care professional who loves to share different tips on health and personal care. He is a regular contributor to MegaHowTo and lives in Canada.

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