1. Cystic Fibrosis Disease Process
Lungs are protected by a thin film of fluid that surrounds them. People with cystic fibrosis (CF) have a gene mutation that causes sticky mucus. This mucus surrounds lung and other internal organs. CF patients also have an issue with salt absorption whereby the sodium and chloride losses are increased. This affects the protective thin fluid film which surrounds the lung. These two combinations allow thick mucus to sit around the lungs and increase chances of infections.
2. Genetic Testing
How would you find out if you have CF? In order to understand the testing, let's look at how we sweat and what our sweat contains. Human sweat contains sodium and chloride, the two major constituents of salt. Sweat is generally produced at the base of the sweat glands. When the sweat is coming up, the sodium and chloride ions escape into the skin layer called the epithelium, leaving the liquid behind; what comes up on the surface is only slightly salty. In CF patients, this mechanism of sodium and chloride absorption is faulty and therefore what comes up as sweat has large amounts of sodium and chloride.
The testing to get the diagnosis of CF actually looks at the amount of chloride in the sweat and if it is unusually high, the diagnosis of CF is made.
3. Dehydration
When it is very hot and we are physically very active, we sweat a lot and loose a lot of fluids. If we do not replace this fluid, we get truly dehydrated. When CF patients get dehydrated, they encounter several problems. They lose a lot of salt along with the fluids through their sweat. This leaves more sticky mucus on the lung surfaces and without drinking plenty of fluids, CF patients are not able to loosen this mucus and remove it. Plenty of fluid intake is essential for CF patients to keep up their hydration. Also they may require 500 to 1000mg of additional salt to replenish their loss.
4. Good Nutrition
It is very important to keep up good nutritional status for CF patients since they have so many underlying issues that directly affect their nutritional health and their chances of getting infections. A good prescription for eating well would be an intake of a well-balanced diet, plenty of fluids, prescribed pancreatic enzymes with meals and snacks, and water soluble vitamins in the proper amounts.
5. Future Directions
Researchers have learned the ways of faulty mechanisms of sodium and chloride metabolism in CF patients. Now they are trying to come up with medications that will help replenish the salt and fluids on the surface of the lungs of the CF patients. This will protect their lungs and will reduce lung infections.
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