Treatment of Mucoviscidosis (cystic Fibrosis)

About the disease

Cystic fibrosis, also known as mucoviscidosis, is an inherited disorder that affects the lungs and digestive system by attacking the cells responsible for the production of bodily fluids. Mucus and saliva are thicker than normal, causing breathing passageways to become congested and the functioning of the lungs and pancreas to be disrupted. A child will only inherit cystic fibrosis if both parents have the defective gene that causes this disorder.

In the past, children with cystic fibrosis would often not live past their teens. Today, although it cannot be completely cured, this condition can be controlled and life expectancy has increased. People with cystic fibrosis can still go to school, attend university and go to work. Advancements in medicine mean that in the future, people with cystic fibrosis are expected to live a lot longer.

Babies are usually diagnosed with cystic fibrosis within the first few months after they are born. The diagnosis can be made even before any symptoms arise. In some cases, symptoms do not appear until adolescence or adulthood, but it is still important to attend regular check-ups at the doctor. Some people only experience symptoms of cystic fibrosis occasionally, while others suffer inflammation of the lungs or pancreas on a regular basis.

Symptoms

• Cough with very thick mucus
• Recurrent inflammation of lungs or pancreas
• Shortness of breath
• Wheezing
• Frequent infections of respiratory and digestive systems
• Constipation
• Excessively salty sweat
• Babies are slow to gain weight

Diagnosis

• During a general examination, the doctor will use a stethoscope to listen to the child’s lungs, to check for wheezing or other breathing problems.
• The doctor will also ask the child’s parents if they or anyone in the family have cystic fibrosis.
• A blood test can be used to analyze the chemicals released by the child’s pancreas. High levels of one specific chemical can indicate the presence of cystic fibrosis.
• Genetic testing can establish whether a child has a gene that causes cystic fibrosis.

Treatment

• Conservative treatment can alleviate the symptoms of cystic fibrosis and prolong life expectancy.
• Various antibiotics and anti-inflammatory drugs are prescribed, to prevent the reappearance of infections and reduce inflammation.
• Special drugs that thin the mucus are also prescribed, to improve the functioning of the lungs.

Authors: Dr. Vadim Zhiliuk, Dr. Sergey Pashchenko