Cystic fibrosis (CF) is a chronic life-threatening disease resulting from a genetic birth defect. Between 70,000 and 100,000 people suffer with CF globally; this is considered an underestimation because many children die without being diagnosed, or there are no registries or data collection, or symptoms may be treated without a diagnosis. The prevalence of CF is highest among those of Central and Northern European ancestry, with prevalence lowest in Asia and the Middle East. Although the assumption was made that CF prevalence was low among East Indians, initiatives are demonstrating that lack of appropriate assessment and diagnosis contributed to this conclusion when indeed prevalence was higher than previously thought. Life expectancy in those with CF varies widely, from 35 to 40 years in the United States and the United Kingdom to below 15 years in low-resource countries including El Salvador, India, and Bulgaria (World Wide Cystic Fibrosis Day, 2014).
Globally, efforts are being made in an attempt to reduce morbidity and mortality associated with CF and increase public awareness of this birth defect. Cystic Fibrosis Worldwide (CFW) is a global nonprofit organization in 67 countries that was organized in 2002, with headquarters in Massachusetts and Turkey. This organization is working to establish CF centers throughout the world and to foster the global networking of information about CF and diagnosis and management of CF. They focus on countries in which there is a paucity of information about CF and those born with CF live without access to appropriate care. For example, CFW is reaching out to those living in Oman, Palestine, India, Armenia, Macedonia, and Brazil.
CF was first identified in the Hashemite Kingdom of Jordan in 1984, with an increasing identification of this disorder reflecting the high level of consanguinity in the country. Healthcare providers at Queen Rania al Abdulla Hospital for Children in Amman treat the majority of those with CF. Concerns include high levels of recurrent hypotonic dehydration and infants with pseudomonas colonization associated with lack of neonatal screening programs and lack of physician knowledge about the disease. Their association with CFW is helping to meet the challenges identified by healthcare providers working with those with CF.
Support is offered for persons with CF and their families through http://CysticLife.org, which is the social network for the CF community. It was launched in 2010 through the grassroots initiative of Ronnie Sharpe, a 30-year old with CF who started a blog about his experiences with the disease. In a few short years, there are now over 1,400 members from all 50 states and 30 countries. One user noted, "For all that are dealing with CF for the first time, CysticLife is such a blessing. I wish CysticLife had been around when I first heard those words 'cystic fibrosis.' Dealing with CF alone is a scary place to be and now no one has to experience the CF life alone" (CysticLife, 2014).
Research initiatives for management of CF are funded by CFW through the generosity of donors. The Worldwide CF Day was held in September 2014 in order to raise awareness about this disorder. Interprofessional collaboration is the hallmark of this organization, as promoted by the Partnership for Maternal, Newborn, and Child Health. This partnership includes governments, global agencies, nongovernmental organizations, healthcare and professional organizations, and the public. The International Council of Nurses is also part of this collaboration addressing quality, equity, and access to care (Shamian, 2014).
Focusing on the hallmarks of quality, equity, and access to care in the global health agenda has been described in a recent work (Scott & Jha, 2014), which states that "doing more isn't better. Doing better is better" (p. 5). "Doing better" includes preconception healthcare (medical genetic services where feasible and culturally appropriate), prenatal carrier screening and intrauterine diagnosis of CF, addition neonatal screening for CF, and evidence-based care for those suffering from CF, particularly in low-resource areas of the world.
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