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Cystic fibrosis: Treatment, symptoms, advice and help

About cystic fibrosis

Cystic fibrosis is a severe inherited disease which involves the secretory glands of the respiratory and digestive system mainly. These secretory glands also sometimes called mucous glands are responsible for secreting mucus which lines the membranes of body tissues. In cystic fibrosis, increased production of mucus and its consistency is noted which result in serious and potentially fatal manifestations.

Cystic fibrosis: Incidence, age and sex

Cystic fibrosis is a rare, genetic disease which usually presents at birth. However the disease may also remain undiagnosed till young adulthood.

Signs and symptoms of cystic fibrosis: Diagnosis

The clinical features result from excess mucus production in the lungs and the digestive system. An individual with cystic fibrosis may present with frequent lung infections manifesting with cough, breathlessness and fever. Organs like the liver, the gall bladder, the intestines and the pancreas of the digestive system are affected leading to persistent diarrhoea with bulky and foul smelling stool. Some individuals experience sense of abdominal fullness and gas in the stomach. Other features include stunted growth in children, nutritional deficiencies, salty sweat and exhaustion. The affected individuals have increased chances of being afflicted with diabetes and osteoporosis. Genetic testing may be done to establish the diagnosis. Immunoreactive trypsinogen test is done in newborns whereas electrolyte sweat test may be done in children and young adults for confirmation.

Causes and prevention of cystic fibrosis

Cystic fibrosis is an inherited disorder which occurs due to a defective gene F508. This results in thickening of the mucus which clogs the air passages which predisposes such individuals to bacterial infections of the lung. Moreover the mucus may also clog the ducts of the pancreas or the gall bladder and the liver. This results in failure of movement of digestive enzymes to appropriate locations which hampers the normal digestive process.

Cystic fibrosis: Complications

The complications of cystic fibrosis range from mild to life-threatening. Disorders like sinusitis, nasal polyps and infertility may be seen. There are increased chances of fractures due to thinning of the bones. Moreover, increased loss of salts in sweat may cause a significant dip in blood pressure. Serious health concerns like respiratory failure and right heart failure may occur which can result in death. The usual life span of an individual with cystic fibrosis is 25 to 30 years.

Cystic fibrosis: Treatment

Unfortunately there is no cure for this inherited disease which is usually life threatening. However, symptomatic relief may be provided which depends on the organ affected. Medications like antibiotics and anti inflammatory drugs may be prescribed to treat bacterial lung infections and inflammation, respectively. Mucolytic drugs may help in breaking down the thickened mucous and ease the airways. Chest physiotherapy may also be tried to relieve the patient of increased chest secretions. Nutritional supplements and pancreatic enzymes are needed to prevent malnutrition and nutritional deficiencies. Surgical intervention may be needed in some individuals to remove the gall bladder.