Cystic fibrosis

What is it about ?

Cystic fibrosis (also called cystic fibrosis) is an inherited metabolic disease that affects the lungs and pancreas. People with this condition are particularly
susceptible to respiratory tract infections, leading to lung problems. Involvement of the pancreas disrupts the absorption of substances in the intestine, leading to fatty diarrhea. The disease begins in childhood, and complaints increase as the disease progresses. In men, the condition causes almost complete infertility and in women poorer fertility.

What is its frequency?

It is the most common inherited metabolic disease in Caucasians. In Belgium, it affects around 1 in 3,000 newborns.

How to recognize it?

There are several forms of manifestation.
Obstruction of the intestines occurs in 1 in 10 infants: the intestine closes because of a stool plug, which causes severe cramping and crying.
The main characteristics in babies and young children are the growth retardation and fatty stools.
Children mainly present persistent cough which gets worse with age and can lead to pneumonia. The other characteristics are the chronic sinusitis, diabetes and fertility problems.

How is the diagnosis made?

Since January 1, 2019, cystic fibrosis has been detected on the blood card when screening newborns. If this screening gives an abnormal result, the parents and their baby are referred to a cystic fibrosis referral center for a sweat test. This test measures the salt content in sweat. The diagnosis of cystic fibrosis will be made if the sweat test is abnormal. It will then be necessary to analyze the errors in the DNA.

What can your doctor do?

It is recommended to treat the disease in a hospital with a center specializing in cystic fibrosis. Affection of the pancreas requires a special diet. Drugs will also need to be given to replace substances that the pancreas can no longer produce. Antibiotics are often needed for respiratory tract infections. Excess mucus production is treated with aerosols, physical therapy, anti-inflammatory drugs, and physical activity. In more advanced stages, it may be necessary to consider a lung transplant.

Want to know more?

• Cystic fibrosis and employment, here, Where on this page of the AVIQ – Agency for a Quality Life
• Cystic Fibrosis – DocsAidants – Information for people who regularly help a loved one who is dependent on age, illness or disability

Are you looking for more specialized help?

• The LUSS – A federation of associations of patients and relatives
• Caregivers – An association that supports people who regularly help a loved one who is dependent on their age, illness or disability

Sources

• www.ebpnet.be
• ‘BCFR – Belgian Cystic Fibrosis Registry’ – Sciensano