Cystic fibrosis (Paediatrics)

It is a disease that causes thick and sticky mucus to build up in the lungs, digestive tract, and other areas of the body.

It is a hereditary disease. It is one of the most common types of chronic lung disease in children and young adults.

Symptoms may be growth delay, inability to gain weight, salty taste on the skin, abdominal pain, constipation, nausea, faeces, cough or increased mucus in the sinuses or lungs, fatigue, nasal congestion caused by nasal polyps, recurrent episodes of pneumonia, etc.

A detailed medical history, complete physical examination, and additional tests such as general and specific blood tests, sweat tests, CT-scan or chest X-ray, faecal fat tests, lung function tests and oesophagogastroduodenal (OGD) transit tests are required for diagnosis.

Treatment is based on general control of symptoms. The treatment of lung damage caused by thick mucus and infections is essential in order to improve the quality of life of the patient. For the treatment of chronic and acute infections intravenous, inhaled and oral antibiotics are administered. Others are related to the treatment of diabetes with insulin and pancreatic disease with enzyme replacement. Additionally, the efficacy of different procedures, such as transplantation and gene therapy, is suggested in order to resolve some of the effects associated with this disease. A healthy diet, elevated exercise and aggressive treatments with antibiotics are increasing the life expectancy of those suffering from this disease.