Children’s Cystic Fibrosis (CF)

© Václav Hroch -

Cystic fibrosis (CF) is a genetic disorder that makes a child more susceptible to lung infections. CF is also known to affect the digestive system.

Thanks to advancement in genetics and drug therapy, kids suffering from this condition can now live more comfortably. Extensive research on the subject has enabled physicians to develop a better understanding of this condition and identify new therapies for treating it.

In the United States alone, more than 30,000 children suffer from CF. The epithelial cells, which form the passageways in lungs, pancreas and liver, become disrupted in a patient of cystic fibrosis. The CF genes discharge a defective protein known as cystic fibrosis transmembrane conductance regulator (CFTR) in the epithelial cells. The defected epithelial cells are unable to regulate the passage of chloride across the cell membrane, disrupting the balance of water and salt to yield mucus and other fluids in the lungs and other passageways.

In normal conditions, mucus prevents germs from entering the lungs. Through the body’s natural mechanism, mucus is excreted from the air pipe. However, in case of CF patients, germs and mucus are trapped in the lungs, making them infected.

CF also secretes thick mucus in the pancreas, preventing important enzymes from reaching the food in the intestines. The food is not digested properly, causing kids to gain weight even when they consume a normal diet.

Identifying CF in kids requires parents to perform genetic tests during pregnancy. The tests will reveal if the unborn infant will suffer from CF or not. However, the intensity of the condition cannot be predicted.

The standard test performed on children to diagnose CF is known as sweat test. It is a painless and accurate way of identifying CF in kids. A small electric current travels into the skin of the forearm, triggering the body to produce sweat. The sweat is collected and used to test the level of chloride.

Cystic Fibrosis Foundation-accredited labs perform two sweat tests for diagnosing CF. On the two separate sweat tests, a child with sweat result greater than 60 has CF.

Kids diagnosed with this disorder may be hospitalized for some time depending on their condition. They will have to undergo nutritional assessment and breathing measurements. The doctor also ensures that the patient’s lungs are clear. Kids are also required to start a diet rich in vitamins and digestive enzymes to help with digestion of food. Follow-up visits are to be made once every 2 or 3 months.

A daily care program helps kids in coping with the condition. A nutritional therapy consisting of diet rich in calorie and fat along with vitamin supplements is recommended. Pulmonary therapy for treating the lungs is also included in the program. Occasional antibiotics may be prescribed to treat lung infections.