Sunday, April 30, 2017

Treatment For Cystic Fibrosis

Cystic fibrosis
is a genetic disorder that is inherited in an autosomal recessive pattern and is characterized by a 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:

1. Physiotherapy
Daily chest percussion and physiotherapy with postural drainage reduces respiratory exacerbations

3. Antibiotics
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.

3. Bronchodilators
These are helpful for symptomatic relief.

4. 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.

5. Pancreatic enzyme replacement
Since patients with cystic fibrosis have pancreatic enzyme deficiency therefore enzyme replacement helps avoid malabsorption.

6. Immunization Routine pneumococcal and influenza vaccination are recommended for patients with cystic fibrosis.

7. Lung transplantation
Considered if pulmonary function is < 30% with chronically infected, purulent bronchiectasis. Side effects of immunosuppresion are significant with only 50% of patients surviving after 5 yrears.

8. Palliative care
It is needed in advanced cases of Cystic fibrosis.

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