About Cystic Fibrosis

Cystic Fibrosis in Children

Living with cystic fibrosis (CF) can be tough for young children. Their lives will be characteristically limited compared to those of other kids because of their condition. They cannot do all the things that normal, healthy children can do. Most of all, because CF does not has a cure as yet, these children will have to deal with their illness for the rest of their lives.

Defining Cystic Fibrosis

Cystic fibrosis is a hereditary condition, passed from parents to offspring. One out of 3,200 children is born with this disease worldwide. The condition is most common among children of European ancestry and among children born to the Ashkenazi Jews, the largest Jewish population in the world.

Because cystic fibrosis is a hereditary disease, its cause lies in the genes. The condition is due to a mutation of the cystic fibrosis trans-membrane conductance regulator gene, or CFTR. The CFTR gene is responsible for the body’s ability to produce sweat, mucus and digestive juices.

Diagnosing Cystic Fibrosis in Children

CF can be detected in children even before they are born. If their parents suspect a possibility of their unborn infant having the condition, the mother can subject herself to a series of genetic testing. Newborn screening tests also make it possible to diagnose cystic fibrosis in babies shortly after they were born. In addition, the sweat test is also used, since people who have the disease tend to have a higher salt content in their sweat.

Altough CF is hereditary, only 10% to 15% of babies have symptoms at birth. Children with cystic fibrosis have exocrine glands that produce an excess of mucus in their lungs, liver, intestines and pancreas. Because their mucus is so thick, it can be a thriving breeding ground for bacteria in their lungs, which can lead to infections as well as a weakened immune system.

Since their pancreas is also affected, children with this condition experience poor growth rates, even malnutrition. Their pancreas is incapable of providing enough enzymes to help the body absorb nutrients, particularly the fat-soluble vitamins. This results nutrient deficiencies, and thus, poor growth.

Living with Cystic Fibrosis

Incessant coughing is the constant companion of a child living with CF. Coughing is necessary to clear the lungs of the thick mucus building up in there. The patient also needs to take good care of him/herself because of his or her heightened susceptibility to infection.

Because a child living with cystic fibrosis gets easily tired, he or she may not be able to participate for long in sports or any rough games that children often play. He or she also needs to eat well all the time because CF makes it harder to digest nutrients and the body uses up more calories than usual for daily functions such as breathing.

Children with cystic fibrosis tend to have short life expectancies, typically living up to 20 to 30 years. Recent developments in medicine, however, have lengthened the life expectancy to 40 or even 50 years.

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.