Kids and Health Care: Cystic Fibrosis
While leukemia is the most-common acute cancer that strikes children, cystic fibrosis (CF) is perhaps the best-known chronic disease. CF is an inherited condition that affects tissues that produce mucus secretions; these include the tissues that line the airways in the lungs, the gastrointestinal tract, the ducts of the pancreas and the ducts of the liver.
In short, CF changes the chemical properties of mucus, causing thicker mucus that can lead to obstructions in the respiratory and digestive systems. In the respiratory system, this means obstructed airways and conditions that lead to repeated infections in the lungs; in the digestive system, it means obstructed ducts in organs involved in digestion.
CF can interfere with the normal processes of the liver, the pancreas (the organ that secretes the hormone insulin, which the body uses to break down sugar and that produces digestive enzymes) as well as other organs that are part of the digestive process. This makes it difficult for persons with cystic fibrosis to digest food and absorb nutrients.
Frequent and prolonged inflammation in the lungs eventually results in cardio-respiratory failure, the primary cause of death in people with CF. This often comes when the person is in his or her late 20s or early 30s.
Cystic fibrosis is an inherited genetic disorder. The gene that causes cystic fibrosis is recessive, which means in order to get the disease a child must inherit the gene from both of his or her parents.
If you only inherit the gene from one parent, you become a carrier (you will not get the disease, but you can pass the gene on to your children). If you are a carrier and you and another person who is a carrier have a baby together, each child will have a 25 percent chance of having the disease and a 50 percent chance of being a carrier.
There is no single therapy that cures CF. If the disease is identified early enough, a combination of treatments can reduce its effects and lengthen -- and improve the quality of -- a child's life. These treatments include the regular use of:
- antibiotics for lung infections;
- decongestants, bronchodilators (drugs that open airways congested with mucus) and anti-inflammatory drugs;
- chest or back clapping (to loosen mucus from lungs) and postural drainage (to drain mucus from lungs);
- pancreatic enzymes (to aid digestion);
- a diet rich in proteins and calories; and
- vitamins and other dietary supplements (to add more nutrients to the diet).
A lot of genetic research is focused on finding chemical "gene therapies" that could eliminate CF and its effects. But these treatments truly are experimental and may be decades away from reliable use.
In the meantime, from the financial perspective, CF means a steady, regular use of moderately expensive prescription drugs. Most managed care programs and insurance policies cover these drugs -- though the effect of out-of-pocket copayments and deductibles could take a major toll after several years. If your child is going to be on a steady schedule of prescription drugs, it will be important to find out about the maximums on your coverage -- both the maximum that you have to pay in any given period and the maximum (if any) that the plan will pay over the course of your child's life.
