Cystic Fibrosis Needs Attention in Iran

Abstract

Copyright © 2013, Kowsar Corp.; Published by Kowsar Corp. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The purpose of this editorial is to raise awareness of the care of cystic fibrosis (CF) patients in Iran. The outlook for patients diagnosed with cystic fibrosis internationally has improved substantially in the past 30 years, but the same does not hold true for a child born with cystic fibrosis in Iran. Numerous therapies such as antibiotics (1, 2), anti-inflammatory agents (3), mucolytics (4, 5), airway (6) and mucous (7) clearance, nutritional support (8), and cystic fibrosis transmembrane conductance regulator (CFTR) potentiators (9) have proven effective in select CF patients, and allowed the United States, Australia and Europe to make great strides in the treatment of CF. Other therapeutic approaches such as gene therapy have dealt with more difficulty than originally anticipated (10). The barriers to accessing quality health care for CF patients in Iran are multifaceted including access to medication, implementation of recognized CF standard adopted guidelines by providers, and lack of early diagnosis (11), management and treatments of such patients across the country. These difficulties were compounded by lack of pediatric Pulmonology subspecialty until 2010 that would have been an advocate for this disease. Throughout the world there are intensive efforts to find cure for patients with CF in the near future. CF patients in Iran can leap forward past decades of stagnation in their disease management and make the dream of cure into reality. This can be achieved through a systematic team approach with the goal of improving the life of CF patients and their families. This team effort can be initiated by education about the disease and training of team members to manage CF until a cure is found for all. Cystic fibrosis is a systemic autosomal recessive heritable disorder. A defective gene on chromosome 7 produces a defective protein (CFTR) that results in decreased chloride secretion and hyperabsorption of sodium. This produces dehydrated mucous within the airway and thick secretions in the pancreatic ducts leading to pathogenic colonization and chronic infection in the respiratory tract and destruction of pancreatic ducts. Thereby, producing the clinical symptoms of cough, chronic respiratory symptoms and infection, recurrent exacerbations, malnutrition and failure to thrive. Infection control measures (12) should be one of the early educational and quality improvement measures in the clinics and hospitals. Iran has improved substantially