Cystic fibrosis is a genetic disorder that is inherited in an autosomal recessive pattern and is characterized bya defective transport of chloride and sodium across the epithelium that causes thick, sticky mucus to build up in the lungs, digestive tract, and other areas of the body. It is one of the most common chronic lung diseases in children and young adults.
Some of the treatment options are discussed briefly here:
Daily chest percussion and physiotherapy with postural drainage reduces respiratory exacerbations
Early high dose , broad spectrum , long duration (minimum 2-3 weeks) antibiotics helps minimize lung damage in infective exacerbations. Oral or intravenous fluroquinolones combined with nebulized antibiotic is often the initial choice; resistance and chronic carriage needs intravenous combinations. Prophylactic antibiotics are also increasingly used.
These are helpful for symptomatic relief.
DNase- alpha mucolytics
These are administered as areosol spray. Mechanism of action is that it interferes with sputum neutrophil DNA, helping to liquefy sputum and encourage expectoration, thus helps in reducing cough.
Pancreatic enzyme replacement
Since patients with cystic fibrosis have pancreatic enzyme deficiency therefore enzyme replacement helps avoid malabsorption.
Routine pneumococcal and influenza vaccination are recommended for patients with cystic fibrosis.