Hydroxyurea with Dose-Escalation to Reduce Primary Stroke Risk in Children with Sickle Cell Anemia in Tanzania: Primary Results of the Sphere Trial

Ambrose, Emmanuela E.; Latham, Teresa; Songoro, Primrose; Charles, Mwesige; Lane, Adam; Stuber, Susan E.; Makubi, Abel; Ware, Russell E.; Smart, Luke R.

doi:10.1182/blood-2022-164787

Cited by 2 publications

(6 citation statements)

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“…This study showed an SCD prevalence of 33.3% in children between 4 to 5 years which is high when compared to similar studies at Amana Referral Hospital and Mnazi Mmoja Regional Hospital located in Ilala District, Dar es Salaam where children between the age group of 3 to 5 years tested positive with SCD recorded 14.9% (24/161 children) and 9.6% (11/115 children), respectively [11]. Some of the reasons for the high prevalence of SCD in Buhigwe district as compared to Ilala District might be due to poor delivery of health services since Ilala District is served by many private and public hospitals including Amana referral hospital while Buhigwe district has only Heri Mission Hospital [17].…”

Section: Discussionsupporting

confidence: 42%

“…Various surveys have been carried out in Tanzania concerning the prevalence of SCD in major cities like Dar es Salaam, Dodoma, Morogoro, and Mwanza [11,15,16], but none has been done in rural towns, thus this was the first survey carried at a hospital located in the remote town of Buhigwe district. In this study, 364 patients in age 0 -5 years were enrolled as the study sample size.…”

Section: Discussionmentioning

confidence: 99%

“…The World Health Organization estimates that 70% of SCD deaths in Africa are preventable with simple, cost-effective interventions such as early identification of SCD patients by newborn screening (NBS) and the subsequent provision of comprehensive care [10]. As of the year 2022, Tanzania has been ranked among the 20 countries that have achieved a reduction of 57% in mortality of children under the age of five years old [11]. Despite this observation, still there is no sufficient research data that has been documented to disclose the prevalence of sickle cell disease in various provinces and their districts in Tanzania, therefore calling for studies on the prevalence of sickle cell disease.…”

Section: Introductionmentioning

confidence: 99%

“…World Health Organization suggests that countries will need to develop policies to directly reduce the mortality of children due to noncommunicable diseases (NCDs) especially SCD so as to fall below 50/500 live births [12]. A recent study in a tertiary hospital in Tanzania reported a median survival of 33 years in the SCD population [11,13]. This suggests that in some areas of Tanzania, there is an increased survival in SCD, which will result in a high prevalence of SCD among the population.…”

Section: Introductionmentioning

confidence: 99%

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A Cross-Sectional Study of the Prevalence of Sickle Cell Disease Among Children of Under the Age of Five Years at Heri Mission Hospital in Buhigwe District – Kigoma, Tanzania

Ally¹,

Mauti²

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Background: Sickle cell disease has become a common inherited hemoglobin disorder. Various reports have shown that a high mortality rate of children under five years of age was reported in the year 2022 in Tanzania. This research aimed to determine the prevalence of sickle cell disease (SCD) amongst children under five years of age and their family history at Heri Mission Hospital in Buhigwe District, Kigoma. Methods: A convenient cross-sectional study was carried out to enroll 204 children under the age of five years at Heri Mission Hospital. The collected data were subjected to analysis of variances using the statistical package for Social Sciences (SPSS) version 28.0.1.0 software (SPSS Inc., USA). The Chi-square test was utilized in comparing proportions and frequency of occurrence of Haemoglobin (Hb) in the variables at P < 0.05 as the significance of variance. Results: The prevalence of SCD among children under five years was 28.3% (103/364). Among the positively tested with SCD, the majority were female at 71.7% (65/91), whereas males were 28.3% (26/91). Family records of the children with SCD showed that the proportional of SCD was significantly higher among family members with SCD 73.1% (38/52) than none SCD (14/52) 26.9% (X2 = 7.513, P < 0.05). Conclusion: The findings in this research showed a high rate of SCD amongst children under five years, and the family history of the victims showed it’s inheritable. Recommendation: Health awareness and proper mitigation strategies need to be addressed on how to curb the SCD in Buhigwe District and Tanzania as a country.

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Section: Discussionsupporting

confidence: 42%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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A Cross-Sectional Study of the Prevalence of Sickle Cell Disease Among Children of Under the Age of Five Years at Heri Mission Hospital in Buhigwe District – Kigoma, Tanzania

Ally¹,

Mauti²

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“…As a direct outcome of these studies, three states in Nigeria (Kano, Kaduna, Katsina) have acted to ensure government purchase of hydroxyurea to support local stroke prevention programming 54 . Finally, the Stroke Prevention with Hydroxyurea Enabled through Research and Education (SPHERE), was a Phase 2, open‐label primary stroke prevention study in Mwanza, Tanzania, that trained local staff to perform screening TCD studies for 202 children with SCA (age 6.8 ± 3.5 years of age), and provided hydroxyurea 20 mg/kg/day treatment with hydroxyurea dose escalated to MTD for those with conditional or abnormal TCD velocities ( n = 45) 55 . The study demonstrated successful reduction in TCD velocities and stroke risk category for most participants.…”

Section: Hydroxyurea In Sub‐saharan Africa: Evidence From Recent Studiesmentioning

confidence: 99%

Hydroxyurea for children with sickle cell disease in sub‐Saharan Africa: A summary of the evidence, opportunities, and challenges

Dexter

McGann

2023

Pharmacotherapy

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Sickle cell disease (SCD) is a common and life‐threatening inherited blood disorder that affects more than 300,000 newborns per year. Because of the origins of the sickle gene mutation as a protective mechanism against malaria for those with sickle cell trait, more than 90% of annual SCD births are in sub‐Saharan Africa (sSA). Over the past several decades, there have been many important advances in the care of individuals with SCD, including early diagnosis through newborn screening programs (NBS), prophylactic penicillin, the development of vaccines to prevent invasive bacterial infections, and the emergence of hydroxyurea as the primary disease‐modifying pharmacologic therapy. These relatively simple and inexpensive interventions have significantly reduced the morbidity and mortality of sickle cell anemia (SCA) such that individuals with SCD can live longer and more complete lives. Unfortunately, although these interventions are relatively inexpensive and evidence‐based, they are only readily available for affected individuals living in high‐income settings, representing <5% of the global SCD population. In sSA, where >90% of the global SCD burden exists, SCD still results in early mortality with 50–90% of infants likely dying before reaching 5 years of age. Recently, there are an increasing number of efforts in many African countries to prioritize SCA with pilot NBS programs, improved diagnostics, and expanded SCD education for health care professionals and the general public. It is essential to include access to hydroxyurea as a core component of any SCD care program, but there are still many barriers that prevent the widespread global use of hydroxyurea. Here, we summarize what we know about SCD and hydroxyurea use in Africa and discuss a strategy to respond to what we consider to be a public health imperative to maximize access to and appropriate use of hydroxyurea for all individuals with SCD using innovative dosing and monitoring strategies.

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Hydroxyurea with Dose-Escalation to Reduce Primary Stroke Risk in Children with Sickle Cell Anemia in Tanzania: Primary Results of the Sphere Trial

Cited by 2 publications

References 0 publications

A Cross-Sectional Study of the Prevalence of Sickle Cell Disease Among Children of Under the Age of Five Years at Heri Mission Hospital in Buhigwe District – Kigoma, Tanzania

A Cross-Sectional Study of the Prevalence of Sickle Cell Disease Among Children of Under the Age of Five Years at Heri Mission Hospital in Buhigwe District – Kigoma, Tanzania

Hydroxyurea for children with sickle cell disease in sub‐Saharan Africa: A summary of the evidence, opportunities, and challenges

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