What is Cystic Fibrosis? 3 Drug Treatment Options Safe for Children

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Jan 27, 2016 05:30 AM EST
Tags Ivacaftor

The Mayo Clinic describes cystic fibrosis or CF as a life-threatening disease that affects the lungs and digestive system. It has no cure but several treatments are available, even for young children.

According to Kids Health, the disease affects how the epithelial cells work. These cells are found in the skin and lines areas within the lungs, liver, pancreas and inside the reproductive and digestive systems.

In 30,000 cases in the United States, individuals inherit the CF gene. This gene causes the body to produce a flawed protein known as the cystic fibrosis transmembrane conductance regulator or CFTR.

The protein causes the body produces mucus that is difficult to remove and can get easily infected. This mucus prevents the body from absorbing nutrients properly.

Although there is no cure for the condition, medical advances have made it more possible for people to live productive lives.

According to Boston Children's Hospital, back in the 1950s, only a handful of kids with CF would live long enough to attend school. Now, life expectancy has improved significantly, with individuals living past their 40s.

The National Institutes of Health says doctors often prescribe medicines such as antibiotics, bronchodilators, and anti-inflammatory medicines to help clear the mucus from the lungs.

A recent clinical trial conducted by Jane Davies and her colleagues reveals that Ivacaftor may be given to kids between the ages of 2 and 5 years. Unlike other medications, Cystic Fibrosis News Today says the drug targets the cause of CF rather than its symptoms.

Another experimental treatment option for kids involves gene therapy. In a report by Kid's Health, the treatment involves using an inhalant which contains healthy CF genes.

Approximately nine medical centers in the United States are conducting clinical trials to see the effectiveness of the treatment.

Some doctors may also prescribe nutritional therapy. For kids with CF, the diet involves eating foods that are rich in fat, calories and proteins.

Kids may be advised to take fat-soluble supplements containing vitamins A, D, E, and K to make up for the nutrients that cannot be absorbed by the body through the intestines.

Besides vitamins, the Cystic Fibrosis Foundation says supplements containing calcium, sodium chloride and iron may also be prescribed to young patients with CF. These supplements can help keep the bones, nerves and muscles healthy and functioning normally.

Kids with CF may also need to take pancreatic enzymes. These oral supplements are needed to help the body digest food properly and absorb the needed nutrients.

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