Cystic fibrosis

What is it?

Cystic fibrosis (CF) is an inherited disease which affects certain glands in the body, especially in the lungs and the digestive system. In the lungs, it causes a thick, sticky mucus to be produced, which leads to frequent chest infections and can cause lung damage. In the digestive system, it affects the pancreas, so that the pancreas can’t produce the enzymes that are needed for food to be broken down and used by the body. Children with CF therefore often have a poor growth rate. It also affects the sweat glands, making the sweat saltier than usual.

There is no cure for CF. Most people who have CF have some degree of health problems though there are treatments available which go some way to counteracting the effects of it. These treatments have helped improve the life expectancy of people with CF (which is now on average around 30-40 years), but it is still sadly the case that some babies, children and young adults who have CF die from it.

CF occurs most commonly among people of white European origin.

It can be diagnosed antenatally by amniocentesis or chorionic villus sampling. And now there is Pre-implantation Genetic diagnosis which screens embryos for genetic defects and chromosonal abnormalities so that only healthy ones are implanted in the womb.

From October 2007, there will be a screening test for newborn babies that will help to diagnose CF babies early. Find out more here.

What causes it?

CF is an inherited genetic condition, which your baby will only be born with if he inherits the gene from both you and his other parent.

Looking after a child with cystic fibrosis

Children with CF can lead a relatively normal life, although they do need to follow a strict treatment regime and this inevitably affects their day-to-day activities. This regime will vary depending on how severe their symptoms are. It is likely to include daily chest physiotherapy, and taking various medicines and enzyme capsules. Also, because they don’t absorb their food very well, children with CF need a higher calorie intake than usual.

Most of this treatment can be carried out at home, although children with CF do need to be seen regularly by health professionals, which involves frequent visits to hospital.

Mother of three Clare Thomas writes about her experience of bringing up her son Jordan suffers from cystic fibrosis.

If you’d like more information about CF, go to the Cystic Fibrosis Trust site.