Cystic fibrosis is a genetic disease in which sodium and chloride channels are abnormal, which causes thick mucus that fails drain well. Nearly all people with cystic fibrosis have nasal polyps. Many have severe chronic sinusitis. Sinusitis and pneumonia in cystic fibrosis develop because of this poor mucus clearance. People with cystic fibrosis can also develop diabetes (mellitus) from pancreatic involvement. Diabetes suppresses the immune system and increases the risk of infection. Treatment strategies for cystic fibrosis-related chronic sinusitis requires special dedication, often including a combination of medical and surgical therapies. Sinus surgery should reserved symptomatic patients refractory to aggressive medical therapy. Sinus surgery must be carefully performed by an experience sinus surgeon who is used to taking care of cystic fibrosis patients because scar tissue can develop easily in these individuals. A study I performed with my Johns Hopkins colleagues showed there are many novel therapies and approaches to cystic fibrosis chronic sinusitis that have been shown to significantly improve the quality of life of people with this disease (1). However, every patient is different and requires a tailored approach and more research is needed.
I encourage you to support cystic fibrosis research. Visit www.cff.org to learn how you can help.
Raise Awareness for Cystic Fibrosis
Consider these statistics:
- Thomas S. Higgins, MD, MSPH
(1) Liang J, Higgins TS, Ishman SL, Boss EF, Benke JR, Lin SY. Surgical management of chronic rhinosinusitis in cystic fibrosis: a systematic review. Int Forum Allergy Rhinol. 2013 Jul 9. doi: 10.1002/alr.21190. [Epub ahead of print]
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