What is Cystic Fibrosis and Bronchiectasis?
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Cystic Fibrosis is the most common inherited life shortening disease in the Caucasian population. The incidence is 1/2500 live births in North America. Cystic Fibrosis affects approximately 30,000 children and adults in the United States and approximately 80,000 individuals worldwide.
The defective gene and its protein causes the body to produce thick sticky mucous that clogs the lungs and leads to chronic life threatening lung infections with dilation and destruction of airways (bronchiectasis). It also obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food. Approximately 95% of patients with cystic fibrosis die from chronic respiratory failure. In the 1950s, few children with cystic fibrosis lived to attend elementary school.
Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond. Treatments require a multidisciplinary approach. Antibiotics, airway clearance techniques, airway hydration, mucous thinners and bronchodilators and new CFTR modulator therapies (treatment to correct the defective protein in CF) are the mainstay of treatment for CF lung disease.
Bronchiectasis, which is a part of cystic fibrosis lung disease, also can occur for other reasons. Bronchiectasis is permanent dilatation and obstruction of the airway wall. Potential causes include, but are not limited to: immunodeficiency, post infectious, and congenital abnormalities. Treatment of non-CF bronchiectasis is similar to that of cystic fibrosis-related bronchiectasis and often requires antibiotics and airway clearance techniques in addition to a comprehensive evaluation for underlying causes.