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Dx Cystic Fibrosis Treatments:

Cystic Fibrosis

Merck Manual Home Edition
"Cystic fibrosis is the most common inherited disease leading to a shortened life span among white people. In the United States, it occurs in about 1 of 3,300 white infants and in 1 of 15,300 black infants. It is rare in Asians. Because improvements in treatment have extended life expectancy for people with cystic fibrosis, 48% of people in the United States with this disease are adults. Cystic fibrosis is equally common among boys and girls.

* Cystic fibrosis is caused by inherited genetic mutations that cause thick, sticky secretions to clog the lungs and other organs. Typical symptoms include vomiting and abdominal bloating, loose stools, and poor weight gain in newborns, as well as coughing, wheezing, and frequent respiratory tract infections throughout life. The diagnosis is based on sweat test results and/or genetic testing. Half of the people with this disease live to their late 30s. Treatments include antibiotics, bronchodilators, drugs to thin lung secretions, airway clearance treatments for respiratory problems, supplements of pancreatic enzymes and vitamins for digestive problems, and a drug to improve cystic fibrosis protein activity in people with a certain mutation. Some people benefit from lung transplantation."

Medications Used in Treatment:
1. Aminoglycoside Antibiotics: gentamycin
2. Recombinant Human DNAses: Pulmozyme®
3. Mucolytics: acetylcysteine
4. FTR Potentiators: Kalydeco®
5. Monobactam Antibiotics: Azactam

Suggested Links
*N.H.S. Choice


Cystic Fibrosis Association

*[Editor] The use of compounded nebulized glutathione improved pulmonary function in confirmed patients with cystic fibrosis in this 2005 study.

*[Editor] A Cochrane Database review of the use of human growth hormone in children and young adults with cystic fibrosis found "Recombinant growth hormone therapy is effective in improving the intermediate outcomes in height, weight and lean tissue mass when compared with no treatment. One measure of pulmonary function test showed moderate improvement."

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