LONG TERM MANAGEMENT OF CYSTIC FIBROSIS

Introduction: Cystic fibrosis (CF) is a lung disease that involves more than 80,000 people globally. It is a life-threatening genetic disorder characterized by the buildup of thick, viscous mucus secretions in various organ systems, most commonly the pulmonary, gastrointestinal, and genitourinary systems. This article reviews the long-term management of cystic fibrosis

Aim of the study: The review aims to understand clinical manifestations of cystic fibrosis, its diagnosis, and monitoring of patients, as well as guidelines for management and emerging pharmacologic treatments.

Methodology: The review is a comprehensive research of PUBMED from year to 1990 to 2008.

Conclusion: There is an immense improvement in the management of cystic fibrosis, especially after approval of CFTR modulators, but it is still in question since management of cystic fibrosis not only the protein rectifiers but also symptomatic treatment and intensive physiotherapy, which require concomitant therapies. The genotype myriad also poses a challenge, and most correcting drugs are for children older than 12 years of age. Most of these drugs have serious hepatic toxicity and other side effects. The psychological and social burden of disease should also need to be concerned.

 Keywords: Cystic fibrosis, antimicrobials, nutritional therapy, respiratory status