Browsing by Author "Andemariam, B."
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Item Age of first pain crisis and associated complications in the CASiRe international sickle cell disease cohort(Blood Cells, Molecules and Diseases, 2021) Tartaglione, I.; Strunk, C.; Antwi-Boasiako, C.; Andemariam, B.; Colombatti, R.; Asare, E.V.; Piccone, C.M.; Manwani, D.; Boruchov, D.; Tavernier, F.; Farooq, F.; Akatue, S.; Oteng, B.; Urbonya, R.; Wilson, S.; Owda, A.; Bamfo, R.; Boatemaa, G.D.; Rao, Sudha; Zempsky, W.; Sey, F.; Inusa, B.P.D.; Perrotta, Silverio; Segbefia, C.; Campbell, A.D.Pain is a hallmark of Sickle Cell Disease (SCD) affecting patients throughout their life; the first pain crisis may occur at any age and is often the first presentation of the disease. Universal newborn screening identifies children with SCD at birth, significantly improving morbidity and mortality. Without early screening, diagnosis is generally made after disease manifestations appear. The Consortium for the Advancement of Sickle Cell Research (CASiRe) is an international collaborative group evaluating the clinical severity of subjects with SCD using a validated questionnaire and medical chart review, standardized across 4 countries (United States, United Kingdom, Italy and Ghana). We investigated the age of first pain crisis in 555 sickle cell subjects, 344 adults and 211 children. Median age of the first crisis in the whole group was 4 years old, 5 years old among adults and 2 years old among children. Patients from the United States generally reported the first crisis earlier than Ghanaians. Experiencing the first pain crisis early in life correlated with the genotype and disease severity. Early recognition of the first pain crisis could be useful to guide counseling and management of the disease.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 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.