Browsing by Author "Rao, S."
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Item An Analysis of Racial and Ethnic Backgrounds Within the CASiRe International Cohort of Sickle Cell Disease Patients: Implications for Disease Phenotype and Clinical Research(Journal of Racial and Ethnic Health Disparities, 2020-04-16) Boatemaa, G.D.; Campbell, A.D.; Colombatti, R.; Andemariam, B.; Strunk, C.; Tartaglione, I.; Piccone, C.M.; Manwani, D.; Asare, E.V.; Boruchov, D.; Farooq, F.; Urbonya, R.; Perrotta, S.; Sainati, L.; Rivers, A.; Rao, S.; Zempsky, W.; Sey, F.; Segbefia, C.; Inusa, B.; Antwi-Boasiako, C.Millions are affected by sickle cell disease (SCD) worldwide with the greatest burden in sub-Saharan Africa. While its origin lies historically within the malaria belt, ongoing changes in migration patterns have shifted the burden of disease resulting in a global public health concern. We created the Consortium for the Advancement of Sickle Cell Research (CASiRe) to understand the different phenotypes of SCD across 4 countries (USA, UK, Italy, and Ghana). Here, we report the multi-generational ethnic and racial background of 877 SCD patients recruited in Ghana (n = 365, 41.6%), the USA (n = 254, 29%), Italy (n = 81, 9.2%), and the UK (n = 177, 20.2%). West Africa (including Benin Gulf) (N = 556, 63.4%) was the most common geographic region of origin, followed by North America (N = 184, 21%), Caribbean (N = 51, 5.8%), Europe (N = 27, 3.1%), Central Africa (N = 24, 2.7%), and West Africa (excluding Benin Gulf) (N = 21, 2.4%). SCD patients in Europe were primarily West African (73%), European (10%), Caribbean (8%), and Central African (8%). In the USA, patients were largely African American (71%), Caribbean (13%), or West African (10%). Most subjects identified themselves as Black or African American; the European cohort had the largest group of Caucasian SCD patients (8%), including 21% of the Italian patients. This is the first report of a comprehensive analysis of ethnicity within an international, transcontinental group of SCD patients. The diverse ethnic backgrounds observed in our cohort raises the possibility that genetic and environmental heterogeneity within each SCD population subgroup can affect the clinical phenotype and research outcomes.Item Global geographic differences in healthcare utilization for sickle cell disease pain crises in the CASiRe cohort(Blood Cells, Molecules and Diseases, 2021) Strunk, C.; Tartaglione, I.; Piccone, C.M.; Colombatti, R.; Andemariam, B.; Manwani, D.; Smith, A.; Haile, H.; Kim, E.; Wilson, S.; Asare, E.V.; Rivers, A.; Farooq, F.; Urbonya, R.; Boruchov, D.; Boatemaa, G.D.; Perrotta, S.; Ekem, I.; Sainati, L.; Rao, S.; Zempsky, W.; Sey, F.; Antwi-Boasiako, C.; Segbefia, C.; Inusa, B.; Campbell, A.D.Background: Sickle cell disease (SCD) is characterized by frequent, unpredictable pain episodes and other vasoocclusive crises (VOCs) leading to significant healthcare utilization. VOC frequency is often an endpoint in clinical trials investigating novel therapies for this devastating disease. Procedure: The Consortium for the Advancement of Sickle Cell Research (CASiRe) is an international collaboration investigating clinical severity in SCD using a validated questionnaire and medical chart review standardized across four countries (United States, United Kingdom, Italy and Ghana). Results: This study, focused on pain crisis incidence and healthcare utilization, included 868 patients, equally represented according to age and gender. HgbSS was the most common genotype. Patients from Ghana used the Emergency Room/Day Hospital for pain more frequently (annualized mean 2.01) than patients from other regions (annualized mean 1.56 U.S.; 1.09 U.K.; 0.02 Italy), while U.K. patients were hospitalized for pain more often (annualized mean: U.K. 2.98) than patients in other regions (annualized mean 1.98 U.S.; 1.18 Ghana; Italy 0.54). Italy's hospitalization rate for pain (annualized mean: 0.57) was nearly 20 times greater than its emergency room/day hospital only visits for pain (annualized mean: 0.03). When categorized by genotype and age, similar results were seen.Item Implementing newborn screening for sickle cell disease in Korle Bu Teaching Hospital, Accra: Results and lessons learned(Wiley, 2021) Segbefia, C.I.; Goka, B.; Welbeck, J.; Amegan-Aho, K.; Dwuma-Badu, D.; Rao, S.; Salifu, N.; Salifu, N.; Oppong, S.A.; Odei, E.; Ohene-Frempong, K.; Odame, I.Early diagnosis of sickle cell disease (SCD) through newborn screening (NBS) is a cost-effective intervention, which reduces morbidity and mortality. In sub-Saharan Africa (SSA) where disease burden is greatest, there are no universal NBS programs and few institutions have the capacity to conduct NBS. We determined the feasibility and challenges of implementing NBS for SCD in Ghana's largest public hospital. The SCD NBS program at Korle Bu Teaching Hospital (KBTH) is a multiyear partnership between the hospital and the SickKids Center for Global Child Health, Toronto, being implemented in phases. The 13-month demonstration phase (June 2017–July 2018) and phase one (November 2018–December 2019) focused on staff training and the feasibility of universal screening of babies born in KBTH. During the demonstration phase, 115 public health nurses and midwives acquired competency in heel stick for dried blood spot sampling. Out of 9990 newborns, 4427 babies (44.3%) were screened, of which 79 (1.8%) were identified with presumptive SCD (P-SCD). Major challenges identified included inadequate nursing staff to perform screening, shortage of screening supplies, and delays in receiving screening results. Strategies to overcome some of the challenges were incorporated into phase one, resulting in increased screening coverage to 83.7%. Implementing NBS for SCD in KBTH presented challenges with implications on achieving and sustaining universal NBS in KBTH and other settings in SSA. Specific steps addressing these challenges comprehensively will help build on the modest initial gains, moving closer toward a sustainable national NBS program.Item A study of the geographic distribution and associated risk factors of leg ulcers within an international cohort of sickle cell disease patients: the CASiRe group analysis(Annals of Hematology, 2020-05-06) Antwi-Boasiako, C.; Andemariam, B.; Colombatti, R.; Asare, E.V.; Strunk, C.; Piccone, C.M.; Manwani, D.; Boruchov, D.; Farooq, F.; Urbonya, R.; Wilson, S.; Boatemaa, G.D.; Perrotta, S.; Sainati, L.; Rivers, A.; Rao, S.; Zempsky, W.; Ekem, I.; Sey, F.; Segbefia, C.; Inusa, B.; Tartaglione, I.; Campbell, A.D.Vasculopathy is a hallmark of sickle cell disease ultimately resulting in chronic end organ damage. Leg ulcer is one of its sequelae, occurring in ~ 5–10% of adult sickle cell patients. The majority of leg ulcer publications to date have emanated from single center cohort studies. As such, there are limited studies on the geographic distribution of leg ulcers and associated risk factors worldwide. The Consortium for the Advancement of Sickle Cell Research (CASiRe) was formed to improve the understanding of the different phenotypes of sickle cell disease patients living in different geographic locations around the world (USA, UK, Italy, Ghana). This cross-sectional cohort sub-study of 659 sickle cell patients aimed to determine the geographic distribution and risk factors associated with leg ulcers. The prevalence of leg ulcers was 10.3% and was associated with older age, SS genotype, male gender, and Ghanaian origin. In fact, the highest prevalence (18.6%) was observed in Ghana. Albuminuria, proteinuria, increased markers of hemolysis (lower hemoglobin, higher total bilirubin), lower oxygen saturation, and lower body mass index were also associated with leg ulceration. Overall, our study identified a predominance of leg ulcers within male hemoglobin SS patients living in sub-Saharan Africa with renal dysfunction and increased hemolysis.