Menstrual characteristics of sickle cell disease patients seen at a tertiary institution in North Western Nigeria.

Introduction: The burden of sickle cell disease (SCD) is high in Sub-Saharan Africa, including Nigeria, and with improved care and survival, reproductive health issues, including menstruation, have become increasingly important and may impact on long-term health and social wellbeing. Objectives: This study was carried out to characterize the menstrual characteristics of SCD patients. Methodology: Using a cross-sectional study design, a semi-structured questionnaire was administered and information collected on bio-demographic data, medical history of SCD, reproductive, and menstrual history. Results: One hundred and sixty female patients participated in the study. The mean age was 24.9 years (standard deviation [SD] ± 8.8). The mean age at menarche was 15.6 years (SD ± 2.7) for those menstruating. Most respondents had regular menstrual cycles 120 (82%); normal menstrual cycle lengths 120 (81%) significantly associated with respondent's age (P < 0.05); normal duration of flow 140 (97%); light menstrual flow 104 (71%) significantly associated with body mass index BMI (P < 0.05). Overall, however, 114 (78%) had abnormal menses and 32 (22%) had normal menses. Eighty-one respondents (56%) experienced menstrual pain, which was severe in only 28 cases (35%). The presence of menstrual pain did not significantly affect the annual frequency of crisis (P > 0.05). Conclusion: The mean age at menarche was high at 15.6 years. Most respondents had light menstrual flow and overall abnormal menstrual patterns. Menstrual pain was common but was not significantly associated with the frequency of crisis.

RésuméIntroduction: Le taux d'infection de la drépanocytose est élevé en Afrique Sub-Saharien, inclus le Nigéria. Avec les avancements en soin et survie, les problèmes de santé de reproductions, ci inclus le cycle menstruel, sont devenus très préoccupants et peuvent avoir un impact à longue terme sur la santé et le bien-être du patient. Objectifs: Cette étude a pour but de décrire les caractéristiques du cycle menstruel des drépanocytaires. Méthodologie: Utilisant la conception de l'étude transversale, un questionnaire semi-structuré a été administré pour collectionner les informations sur les données biodémographiques, les antécédents médicaux de la drépanocytose ainsi que l'histoire de reproduction et de menstruation des drépanocytaires. Résultats: Cent soixante (160) drépanocytaires de sexe féminin ont pris part à l'étude comme échantillons. La moyenne d'âge était de 24,9 ans (Écart-Type de ±8,8). La moyenne d'âge à ménarche était de 15,6 ans (Écart-Type de ±2,7) pour celles qui sont en cycle menstruel. La plupart des participantes avaient un cycle menstruel régulier (120 sur 160) donc un taux de 82% ; 120 avaient un cycle menstruel de durée normale (81%) qui correspondait significativement très bien à l'âge des participantes (P- Probabilité <0,05) ; 140 avaient une durée d'écoulement normal donc (97%) ; 104 avaient un écoulement léger c'est- à-dire 71% qui correspondait significativement à l'indice de masse corporelle (P<0,05). En totalité, 114 dont 78% ont eu des cycles menstruels anormaux et 32 (22%) ont eu des cycles menstruels douloureux avec 28 cas sévères donc 35%. La présence du cycle menstruel douloureux n'a pas eu d'effet significatif sur la fréquence de crises annuelles (P>0,05). Conclusion: La moyenne d'âge à ménarche était élevée à 15,6 ans. La plupart des participantes ont eu des écoulements légers et globalement, des cycles anormaux. Les cycles douloureux étaient fréquents, mais n'étaient significativement pas liés à la fréquence de crise. Mots-clés: Caractéristiques menstruelles, Menstruation (règles), Nord-Ouest du Nigéria, Drépanocytose.

Low-dose hydroxycarbamide therapy may offer similar benefit as maximum tolerated dose for children and young adults with sickle cell disease in low-middle-income settings.

The multiple clinical benefits of hydroxycarbamide in sickle cell disease are supported by a large body of evidence. The maximum tolerated dose (MTD) is the regimen recommended by guidelines from a panel of National Heart, Lung, and Blood Institute (NHLBI) experts, but other dosage regimens have been used in babies (BABY-HUG) 9 to 18 months old (20 mg/kg per day) and developing countries such as India (10 mg/kg per day); however, there has been no direct comparison of the efficacy, effectiveness, or cost-effectiveness of these different regimens. The purpose of this review was to investigate the current situation with various hydroxycarbamide regimens with particular relevance to low-middle-income countries. In regard to methodology, a literature review was undertaken by using multiple databases in PubMed and Google and the search terms included sickle cell disease, hydroxyurea, hydroxycarbamide, sickle cell anaemia, low-middle-income countries, Sub-Saharan Africa, and India. Although MTD regimens have been widely used in research, especially within North America, clinical trials elsewhere tend to use fixed-dose regimens. In a survey of haematologists across Europe and Africa, 60% (75% response rate) did not use the MTD regimen for hydroxycarbamide treatment of sickle cell disease. The recommendations are (1) for practical purposes to commence using fixed-dose hydroxycarbamide in line with BABY-HUG recommendations and then (2) to consider or propose a trial comparing MTD escalation with various fixed doses and to include as end points health-related quality of life, haemoglobin F levels, adherence, and cost-effectiveness.

Utilising the 'Getting to Outcomes®' Framework in Community Engagement for Development and Implementation of Sickle Cell Disease Newborn Screening in Kaduna State, Nigeria.

BACKGROUND: Sickle Cell Disease (SCD) has been designated by WHO as a public health problem in sub-Saharan Africa, and the development of newborn screening (NBS) is crucial to the reduction of high SCD morbidity and mortality. Strategies from the field of implementation science can be useful for supporting the translation of NBS evidence from high income countries to the unique cultural context of sub-Saharan Africa. One such strategy is community engagement at all levels of the healthcare system, and a widely-used implementation science framework, "Getting to Outcomes®" (GTO), which incorporates continuous multilevel evaluation by stakeholders about the quality of the implementation. OBJECTIVES: (1) to obtain critical information on potential barriers to NBS in the disparate ethnic groups and settings (rural and urban) in the healthcare system of Kaduna State in Nigeria; and, (2) to assist in the readiness assessment of Kaduna in the implementation of a sustainable NBS programme for SCD. METHODS: Needs assessment was conducted with stakeholder focus groups for two days in Kaduna state, Nigeria, in November 2017. RESULTS: The two-day focus group workshop had a total of 52 participants. Asking and answering the 10 GTO accountability questions provided a structured format to understand strengths and weaknesses in implementation. For example, we found a major communication gap between policy-makers and user groups. CONCLUSION: In a two-day community engagement workshop, stakeholders worked successfully together to address SCD issues, to engage with each other, to share knowledge, and to prepare to build NBS for SCD in the existing healthcare system.