114. Hemoglobinopathies, Excluding Thalassemia—Clinical: Hydroxyurea for Sickle Cell Disease: Treatment Benefits and Potential Reproductive Risks for Women

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Shehu Umar Abdullahi, Binta W. Jibir, Halima Bello-Manga, et al.

Authors Conclusion from the Abstract: For primary stroke prevention in children with SCA, fixed low-dose when compared to fixed moderate-dose hydroxyurea therapy, demonstrated no difference in the incidence rate of strokes. Both fixed low- and moderate -dose hydroxyurea doses are superior to no treatment for primary stroke prevention with abnormal TCD values. In partnership with Katsina, Kano, and Kaduna health department’s leaders in Nigeria, 9 distinct SCA and primary stroke prevention clinics have been established, with the provision of free fixed low-dose hydroxyurea therapy (Bond Chemical, Nigeria; $0.15 per 500 mg) for abnormal TCD values, and biannual CBCs as standard care, for over 40,000 children with SCA.

Shehu Umar Abdullahi,  Surayya M. Sunusi, Mohammed Sani Abba, et al. 

Authors Conclusion from the Abstract: For secondary stroke prevention, in a randomized controlled trial in children with SCA and new onset of ischemic strokes, fixed low-dose, when compared to fixed moderate-dose hydroxyurea therapy, demonstrated no difference in the incidence rate of stroke recurrence. Fixed low-dose hydroxyurea for secondary prevention of strokes in Nigeria provided a similar stroke recurrence rate, when compared to the SWiTCH Trial (Blood. 2012;119(17):3925-3932) with the maximum tolerated dose of hydroxyurea of 7.0 and 5.7 events per 100 person-years, respectively. For secondary stroke prevention, in low-income settings without access to indefinite regular blood transfusion therapy, fixed low-dose hydroxyurea of at least 10 mg/kg/day with biannual CBC is a new evidence-based strategy to prevent strokes and minimize unnecessary laboratory testing.

Lydia H. Pecker, Sarah Hussain, Jaanvi Mahesh, et al.

Authors Conclusion from the Abstract: In this pilot study of ovarian reserve in young adult women with SCA, AMH is significantly lower in age- and race-matched controls, and levels are similar to a historic SCA cohort. AMH and AFC were both lower in older subjects, suggesting that AMH is a reasonable ovarian reserve marker in SCA. No women had POI. DOR was associated with lower AFC and higher MCV. In young adult women of reproductive age with SCA, taking HU may be a risk for DOR. This data forms the basis for ongoing research on the pathobiological mechanisms and clinical significance of DOR in women with SCA.

Lana Mucalo, Amanda M. Brandow, Sadie F. Mason, et al.

Authors Conclusion from the Abstract: Our findings show that individuals with SCD who have COVID-19 infection have higher rates of death due to COVID-19 than the general Black population. Also, a large proportion of COVID hospitalization for the SCD population occurs among the younger age group. Further analysis is planned to examine the effects of underlying comorbidities and prior SCD-associated complications on the severity of COVID-19 in individuals with SCD.

Fuad A El Rassi, John James, Biree Andemariam, et al. 

Authors Conclusion from the Abstract: Patients reporting HU use at the time of SWAY had a lower VOC burden than patients not reporting HU use. QoL indicators were similar for the 2 groups, but the overall symptom burden was higher for patients who reported HU use compared with those who did not. However, the 2 groups were not matched for pre-treatment disease burden and no information was collected regarding adherence or duration of previous HU use. Overall, although many patients reported treatment satisfaction, many wanted alternative pain management therapies. A wider range of treatment options is needed to reduce SCD symptoms and improve QoL, ultimately helping patients achieve their treatment goals.