Abstract
Improvements in pediatric care since the 1970s as a result of Comprehensive Sickle Cell Centers, newborn screening, and prophylactic penicillin has led to an increase in life expectancy for patients with sickle cell disease (SCD) and has resulted in an increase in the number of adults with progressive end-organ damage/dysfunction. The inability of the U.S. Health Care system to adequately address the needs of this increasing patient population, along with stereotyping of SCD patients, has inevitably led to disparities in care, with an ever increasing disease burden and cost of care. Recognition of these issues has led U.S. Federal Health Care and Biomedical Research agencies (CDC, NIH, HRSA) to develop and implement programs to tackle this growing problem. Recently, NHLBI and NIMHD issued an RFA (HL-16-010) to address through implementation science the unmet health care needs of adolescents and adults (ages ≥15 years) with SCD. This program seeks to improve the health care and outcomes of this population through rigorous implementation of evidence-based guidelines. This initiative prompted us to analyze the demographic characteristics of the adult SCD population served by the GRU Sickle Cell Center, in an effort to better understand the opportunities and challenges posed by this initiative. The GRU Sickle Cell Center has been in existence since 1972, and serves ~1500 pediatric and adult SCD patients through its clinical program. Although based at the GRU campus in Augusta, GA (the second largest metropolitan area in the state with a population of >540,000), the Center has operated extensive outreach activities in rural south Georgia for the last 30 years, covering both pediatric and adult patients. The adult program holds monthly or every other month clinics in 5 sites in central, eastern, and southern Georgia. As of 2015, the Center has 580 active adult patients (>18 years). Fifty six percent are female and 44% male. Over half (54%) are followed at the Augusta clinic, and the remaining 46% in primarily rural outreach sites. The distribution of different genotypes is as follows: SS 392 (69%), SC 114 (20%), S-β+-thal 37 (6%), S-β0-thal 16 (3%) and others 11 (2%). The median age of the male patients is 31 (17-82), whereas for females is 34 (18-68). The median age for SS patients is 32 (17-65), and for SC is 34 (19-71). Overall, 62% of the population is in the 18-40 age group. Only 10% of the patients are >50. There is an age dependent increase in the proportion of female patients (70.6% > 61). Similarly, the proportion of SC patients increases to 56.3%, while SS decreases to 31.3% among subjects >61 years of age. Fifty-one percent of all patients (mostly SS) were prescribed hydroxyurea (HU). However, as reported earlier (Chand et al, ASH poster, 2014), only 59.9% had an adequate response; 26.3% were non-adherent, and 13.9% were on suboptimal doses. These data show that the adult SCD population in Georgia is young, with median age in the lower 30s. It also confirms the well-known observations that SC genotype and female gender are overrepresented in the older age groups. The opportunities to improve the health of this patient population in the next 5-10 years include: the existing outreach infrastructure, the partnership forged between the GRU Sickle Cell Center and some primary care practices (Family Medicine) and Hematology/Oncology practices in various outreach sites, and the implementation of an emergency department fast track pathway to treat vaso-occlusive crises in two of these outreach sites. The challenges, on the other hand, are persisting barriers to adequate/appropriate use of HU, partnering with community providers in the provision of appropriate pain management, implementation of evidence based transfusion practices in outlying hospitals and implementation of long-term evidence based health maintenance and primary care.
Disclosures
No relevant conflicts of interest to declare.
Identifying barriers to evidence-based care for sickle cell disease: results from the Sickle Cell Disease Implementation Consortium cross-sectional survey of healthcare providers in the USA
