Monday, September 13, 2010
Special Needs Focus-- Cystic Fibrosis
People with CF have a shorter-than-normal life expectancy. The good news is that as treatments for CF improve, the life expectancy for people with the disease is rising. Fifty years ago, children with CF often died before attending elementary school. Today many people with the disease live into their 30s, 40s and beyond. Getting early treatment for CF can improve your quality of life and your lifespan.
In some people with CF, the disease begins in childhood. Others have a milder form of the disease and don't start getting sick until they are teenagers or adults. Lung function often starts declining in childhood, eventually leading to severe breathing problems. The most common cause of death in people with CF is respiratory failure.
What Causes Cystic Fibrosis?
A person must inherit two defective CF genes—one from each parent—to have CF. Each time two carriers of the defective gene conceive, there is a:
25 percent chance that their child will have cystic fibrosis
50 percent chance that the child will inherit one defective gene and be a carrier but not have the disease
25 percent chance that the child will not have the gene at all
How is Cystic Fibrosis Treated?
Treatment for CF depends on the stage of the disease and the organs involved. It may include airway clearance techniques to clear mucus from the lungs. One technique is called postural drainage and percussion. The person with CF sits, stands or lies in a position that helps free up mucus. The chest and back are pounded and clapped to loosen the mucus. A person with CF may use a mechanical vest or blow into a device that shakes the mucus loose. Inhaled medicines are also commonly used for CF treatment. These may be taken with a nebulizer, a machine that changes liquid medicine into a fine mist which makes it easier to inhale deep into the lungs. Some medicines can also be breathed in through a metered dose inhaler (MDI). Other types of medicines used for CF treatment include Azithromycin, an antibiotic that fights bacteria in the lungs and anti-inflammatory medicines such as ibuprofen to help reduce swelling in the airways. Lung transplantation may be an option in some severe cases of CF.
Today, almost 45 percent of the cystic fibrosis population is aged 18 years or older. As people with CF live longer, they are more likely to develop middle-aged health issues, such as CF-related diabetes, osteoporosis and infertility in men. A person with CF is always at risk of lung infections. Ways to lessen the risk include:
Frequent hand washing with soap and water or hand gel
Avoid unnecessary contact with people who have a cold or other contagious illness
Get a flu shot every year
Don't smoke and stay away from secondhand smoke
Children and adults with CF may need a high-calorie, high-fat diet to make up for the improper absorption of nutrients caused by the disease. You also may need pancreatic enzyme supplements, and vitamin and mineral supplements. For more in depth information about living with a child with CF you can go to the following website: http://www.cff.org/