complications and prognosis of people with cystic fibrosis

Complications of people with cystic fibrosis

3 different categories of complications:

Respiratory complications

Chronic respiratory infections:

Pneumonia, bronchitis, chronic sinusitis and bronchiectasis — an abnormal dilation of the walls of the bronchial tubes that makes it more difficult to clear your airways. Asthma can result from chronic inflammation of the bronchial lining.

Respiratory infections are common because thick mucus blocks the airways and provides an ideal breeding ground for bacteria. The most common infective agent in people with cystic fibrosis is Pseudomonas aeruginosa. It causes increased inflammation of the respiratory tract.

People with cystic fibrosis may also develop bleeding from the lungs causing them to cough up blood (hemoptysis), respiratory failure or collapsed lung (pneumothorax). Lung disease eventually may cause the lower right chamber (right ventricle) of the heart to fail. Ultimately, complications from lung problems may lead to fatality for people with cystic fibrosis.

Nutritional complications

People with cystic fibrosis are prone to chronic diarrhea and severe nutritional deficiencies. The thick secretions obstruct the ducts in pancreas, preventing enzymes that digest fats and proteins from reaching the intestines. These secretions also prevent the body from absorbing fat-soluble vitamins (A, D, E, K).

In addition, the bile duct, the duct that carries bile from liver and gallbladder to small intestine, may become blocked and inflamed, leading to liver problems, such as cirrhosis.

Reproductive complications

Cystic fibrosis also affects the reproductive system. The thick secretions often block the tube connecting the testis and prostate gland (vas deferens) causing men with cystic fibrosis to be infertile.

Prognosis of people with cystic fibrosis

Over the last three decades, the prognosis has improved dramatically, although there are still no options of cure for the disease. However, it is found that people with exocrine pancreatic sufficiency, male, absence of infective bacteria mentioned, balanced family function and coping as well as compliance with treatment produce a more favourable outcome of the disease compared to those who aren’t.

Screening is possible for parents who are pregnant to test for the presence of the disease in their newborn child. The development of new gene replacement therapy for cystic fibrosis transmembrane conductance regulator gene (CFTR – gene that causes cystic fibrosis to occur if defect) improve the prognosis of people susceptible to developing the disease.