by Kimberley Allen R.N.
Cystic Fibrosis (CF) is a genetic disorder that is inherited affecting the glands that secret mucous and produce sweat. there are over 30,000 children and young adults with CF in the US. Caucasians have the greatest risk of inheriting CF with incidence occurring in approximately 1 out of 3,600 children, where as only 1 out of every 17,000 African-Americans and 1 out of every 90,000 Asian-Americans are born with CF. CF occurs worldwide and in every ethnic group regardless of socioeconomic status.
It affects primarily the lungs and digestive system. The thin mucous that is needed to protect and help the lungs remove bacteria, as well as the different organs in the digestive system becomes very thick and sticky. Instead of trapping germs and clearing the lungs it remains trapped in the lungs which leads to frequent infections. When I was training as a nurse I did my pediatric rotation in a large teaching hospital. Every morning in the large playroom mothers and their young children with CF would gather with physical and respiratory therapists to learn ways to help their child move the thick mucous including chest percussion techniques.
The thick mucous also affects the digestive system blocking the vessels used to transport the digestive enzymes that our body’s need to breakdown food causing vitamin deficiencies and malnutrition. Children with CF are unable to gain weight even with a healthy diet, infants experience “failure to thrive” despite a good appetite and normal diet. Children with CF are also at greater risk for dehydration and electrolyte imbalances because they lose abnormaly large amounts of salt when they sweat.
The symptoms of CF frequently start with in the first year. Though they vary from child to child as well as in severity there are some general symptoms to watch for. These include; a persistant cough and repeated respiratory infections, as well as little to no weight gain despite a good appetite and nutritional diet.
There have great advances in medical science when it comes to treating CF, however there is still no cure at this time. The treatment of CF is specific to each child, however the goals are the same for all. First is to prevent and control respiratory infections, loosen and remove thick mucous from the lungs, prevent or treat blockages in the intestines, provide sufficient nutrition including vitamin supplements and prevent dehydration.
Research is continually being done to find a cure for CF, now it looks as though it could be close at hand. Last week Vertex Pharmaceuticals announced that a combination of it’s new drug Kalydeco, which was recently approved by the FDA, and an experimental drug known as VX-809 significantly improved the respiratory function of some CF patients. The new drug Kalydeco which was just approved 4 months ago is the first treatment that targets the cause of CF. The final results of this study will not be complete until later this year and there also needs to be a final set of studies done before Vertex can apply for approval, but the news is exciting and giving new hope to those who suffer with CF.
Kimberly Allen is a registered nurse with an AND in nursing. She has worked in ACF, LCF and psychiatric facilities, although she spent most of her career as a home health expert. She is now a regular contributor to HealthAndFitnessTalk.com, dispensing advice and knowledge about medical issues and questions. You can reach her with any comments or questions at firstname.lastname@example.org.