including the CF Responsibility, Independence, Self-Care, and Education( R. I. S. E) questionnaire. Introduced to nationwide CF centers by experts to aid transition, this questionnaire assesses patients’ and caregivers’ knowledge in a variety of CF related topics, ranging from proper lung health and airway clearance to work-life complications for those living with CF. 21 Similar to other studies throughout the country, this questionnaire has been positively viewed and proved beneficial addressing deficits of the transition process. After completion of the questionnaire, the pediatric and adult providers at the Mountain State CF Center cohesively evaluate patients to determine readiness for transition. In the year of transition, adult providers will introduce themselves to patients during their first two pediatric visits, and once they are transitioned to the adult side, pediatric providers will check in with patients to see how their transition is going. Advancements in pharmacological therapy have increased the prevalence of CF in adulthood as demonstrated by Burgel et al( Figure 2). 22 For the past decade, there have been more adults than children living with CF. 22 With advancements of care for those with CF in rural areas, we can continue to improve this prevalence of CF into adulthood.
TREATMENT CHALLENGES IN RURAL MEDICINE
Rural geography poses a unique challenge to patients and providers and is further exacerbated when caring for those with a complex illness. Economic barriers have led to rural hospital closures throughout the state, such as Williamstown Memorial Hospital in 2020. 23 These closures further complicate healthcare access, as 14.6 % of West Virginians are unable to afford healthcare and 20 % are without a primary care physician. 24 Described as the“ rural penalty,” studies have highlighted care inequalities for those with chronic respiratory diseases stratified by urbanization. 25 Singh et al first explored an age-standardized mortality rate( ASMR) in 2023 of those diagnosed with CF living in rural environments, finding a higher ASMR in these areas, including a decrease in life expectancy by 13 years. 25 Currently, all 55 counties in WV are categorized as a health professional shortage area, further highlighting access difficulties due to lack of transportation and resources. 23 Jessup et al explored the impact of rural geography on those diag- nosed with CF. 26 Although performed in Australia, geographical isolation can be related to those in rural WV. 26 Geographical isolation experienced by those in WV further clarifies the importance of family collaboration in those diagnosed with CF. Hempstead et al found that family collaboration in CF care improves patient-centered care. 27 In a questionnaire, 91 % of responders found inclusion of family members improved overall care and outcomes. 27 This family-centered approach should be adopted when caring for those with CF in rural areas, such as WV.
Telemedicine plays an essential role in healthcare delivery, highlighted during the COVID-19 pandemic, proving a possible solution to healthcare disparities faced by those in a rural environment isolated by geographical constraints. 28 Despite the surge of COVID-19 throughout the country during the pandemic, cases among the CF population were few. 29 The Mountain State CF Center utilized video visits, telemedicine, at-home spirometry, and mail-in sputum samples, ultimately reducing the number of in-person visits and proving an effective solution to improve outcomes. 29 Further, a retrospective cohort study of 110,999 patient
FIGURE 2
Number of Patients
7500 7000 6500 6000 5500 5000 4500 4000 3500 3000 2500 2000 1500 1000 500 0
58.6 %
17.8 %
41.4 % 82.2 %
1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013 2014 2015 2016 2017 2018 2019
Children
Adults
Demonstrating the progression of CF prevalence from a pediatric to adult condition, which has more than doubled from 1992 to 2019. 22 Figure obtained from“ The Changing Epidemiology of Cystic Fibrosis: The Implications of Adult Care”. From [ CHEST, Burgel, P. R., Burnet, E., Regard, L. & Martin, C, The Changing Epidemiology of Cystic Fibrosis: The Implications for Adult Care, Volume No. 163, Page 89-99. Copyright 2023 Elsevier. Figure reprinted with permission from Elsevier.
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