About Cystic Fibrosis

Cystic Fibrosis and Salt Levels

Cystic fibrosis is an inherited disease that affects multiple bodily systems and nearly all exocrine glands or glands that secrete fluids into a duct. The secretions become abnormal and can affect gland functions in different ways. This condition results from an inherited mutation of a CFTR gene that controls the production of a protein regulating chloride and sodium (salt) transport across the cell membrane. People with CF have disrupted transport of chloride and sodium, therefore, dehydration and increased viscosity of secretions occur. Excessive salt in cystic fibrosis sufferers is also released by the sweat glands. As a result, sweat glands and parotid glands secrete fluids that have higher salt levels.

Salt Loss in Cystic Fibrosis

CF affects the body’s ability to move salt and water on the cellular level. The protein created by the mutated CFTR gene is attached to outer cell membranes of exocrine glands and organs. This protein acts as a channel that connects the inner cell material or cytoplasm with the surrounding fluid environment. In sweat ducts, these proteins facilitate the flow of chloride from the sweat to the cytoplasm.

Defective CFTR proteins result to chloride ions being trapped inside the cells of respiratory airways and outside the skin. Negatively charged chloride ions tend to attract positively charged ions outside the cell and this includes sodium, the most common ion. Chloride and sodium combined produces salt, which is lost in high amounts in CF patients. Salt levels in cystic fibrosis also upset the blood’s mineral balance and often leads to fever and dehydration especially in hot weather.

Diagnosing CF with Sweat Test

The electrolyte sweat test is the most commonly-used testing method in diagnosing cystic fibrosis and salt levels of patients. It determines the level of chloride in the sweat and pain free.

Sweat chloride concentrations less than and within 40-60 mmol/L are considered normal. Values greater than 60 mmol/L confirms the diagnosis for CF. The positive result for the test is permanent throughout life and is not affected by other ailments.

Cystic Fibrosis and Salt Level Management

Managing symptoms and salt levels in patients with cystic fibrosis include medications, nutritional supplements and pancreatic enzyme replacements. CF patients need additional salt and fluid intake to make up for excessive loss of it. Any food that tastes salty like chips, fast food, frozen dinners are good sources of the mineral for CF patients.

Salt is essential for patients with cystic fibrosis for muscle function, nerve cell health and to improve the flow of fluid in cells. Since patients are losing higher than normal levels of it when they sweat, replacing this loss is very vital. Infants with cystic fibrosis must have salt added to their formula or baby food. In children, teens and adults, sodium chloride levels can be maintained by adding liberal amounts of the mineral to their diet, especially during hot weather. The doctor or health care provider can help patients determine the right amount of salt for them.

Disclaimer: This website provides general information about cystic fibrosis and in no means should be taken as a medical or health advice. Please consult your doctor before acting on any of its information.