Strategies to improve healthcare services for patients with sickle cell disease in Nigeria: The perspectives of stakeholders.

Background: Sickle cell disease (SCD) continues to pose physical and psychosocial burdens to patients, caregivers and health workers. Stakeholder engagement in the processes of policy making and implementation is increasingly becoming the cornerstone of best practices in healthcare. Aim and Objectives: To engage stakeholders with a view to assessing the knowledge of SCD; ascertain the challenges associated with accessibility and affordability of healthcare services; improve the quality of care, and thereby effect behavioral change through increasing attendance and follow-up of patients in the clinics. Methodology: A Stakeholders' Engagement meeting organized by the Sickle Pan Africa Research Consortium Nigeria Network (SPARC-NEt) was attended by patients, caregivers and members of patient support groups, healthcare providers and management/policymakers. The engagement was through PowerPoint presentations, structured questionnaires and an interactive session. The structured questionnaire assessed the knowledge of stakeholders about SCD; the quality of healthcare services; challenges with access and affordability; and SCD-related government policies. Results: Three hundred and twelve stakeholders attended the engagement meeting. Of the 133 that participated in the study, medical workers were the most represented. The majority had good knowledge of what causes SCD (96.2%) and the best place to get help during SCD crisis (98.5%). However, knowledge of the specific preventive measures of SCD and its crisis was not optimal. In terms of the role of community engagement and education, only about one-quarter of the study participants, 34 (25.6%) knew about their positive role in reducing the prevalence of SCD and alleviating SCD crises. Challenges identified include inadequate healthcare personnel and facilities, delay in obtaining laboratory results, long waiting time in the clinic, poor communication, absence of holistic consultation, uncoordinated healthcare services, high cost of care, ignorance, non-prioritization of SCD by government, lack of multisectoral collaboration and partnership with NGOs and international organizations. Strategies proffered to improve healthcare services include, community/stakeholder engagement and health education, sickle cell daycare services, access to a willing and dedicated multidisciplinary workforce, collaboration with support groups and government policies and programs. Conclusion: There is need for regular stakeholder engagement to improve access to healthcare services for SCD patients in Nigeria.

Maternal anaemia care in Kano state, Nigeria: an exploratory qualitative study of experiences of uptake and provision.

Background: Maternal anaemia (anaemia in pregnancy, childbirth, and the postpartum period) remains a persistent challenge, particularly in Kano State, Nigeria, which has the highest prevalence of maternal anaemia globally, at 72%. Methods: We conducted a qualitative study in Murtala Muhammad Specialist Hospital in Kano State, Nigeria. We aimed to identify factors constraining uptake and provision of maternal anaemia care, exploring perspectives across different stakeholders. We carried out 10 key informant interviews with policymakers and hospital managers, 28 in-depth interviews with healthcare providers and pregnant women using antenatal services and four focus group discussions with pregnant women's husbands and mothers-in-law. Data were analysed thematically. Results: Issues with provision include a lack of provider training and guidelines specific to maternal anaemia and blood transfusion, insufficient staff to meet increasing demand, and inadequate resources. Issues with uptake include the inability to afford informal user fees, distrust in health services and the blood transfusion process, and a lack of understanding of the causes, consequences, and treatment for anaemia, resulting in poor uptake of care and adherence to treatment. Conclusions: This study recommends the implementation of standardized guidelines and training sessions to better support healthcare providers in offering quality services and increasing funding allocated to supporting maternal anaemia care. Education initiatives for service users and the public are also recommended to build public trust in health services and to improve understanding of maternal anaemia.

Outcome of Hydroxyurea Use in SCD and Evaluation of Patients' Perception and Experience in Nigeria.

Introduction: Hydroxyurea (HU) has been shown to be beneficial in the management of sickle cell disease (SCD) as it improves treatment outcomes. However, despite the benefits of HU, its uptake among SCD patients in Nigeria remains low. Objective: This study aimed to assess the perception and experience of patients with SCD in Nigeria who are using or had used HU, thereby informing and promoting its use. Methodology: A multi-centre, cross-sectional study was conducted among 378 SCD patients aged 1-53 years who have enrolled on Sickle Pan African Research Consortium (SPARCO) registry as HU users. The SPARCO project was funded by the National Institutes of Health (NIH) to establish a sickle cell disease (SCD) registry, strengthen skills and plan research in three African countries. The Nigerian SPARCO registry had 6453 SCD patients at the time of this report with <15% of this population on HU. Data on sociodemographics, perception and experience about HU use were obtained and analysed using descriptive statistics. Findings: Out of the 378 participants, 339 (89.7%) were using HU while 39 (10.3%) had stopped using HU at the time of the study. 281 (74.3%) found HU expensive, while 194 (51.3%) reported none to minimal side effects while using HU. Among patients that stopped HU, cost (59%) and availability (51.3%) were the commonest reasons for discontinuing the drug. Furthermore, 347 (92.5%) had fewer pain crises, 173 (84.8%) had a fewer need for blood transfusion, 145 (86.3%) had improved PCV and 318 (84.6%) had fewer hospital admissions. Finally, the study also showed that 322 (85.2%) respondents would recommend the drug to other patients, whereas 14 respondents (3.7%) would not. Mean corpuscular volume (MCV) and fetal hemoglobin (HbF) levels were not collected in this study and may have improved findings. Conclusion: This study showed that the majority of the SCD patients had good perception and experience with the use of HU while a few had to stop the medication mostly on account of cost and availability. Patients' based advocacy could be leveraged to improve HU uptake while more efforts are needed to ensure that it is readily available and affordable.

High Systolic Blood Pressure, Anterior Segment Changes and Visual Impairment Independently Predict Sickle Cell Retinopathy.

Sickle cell disease is often complicated by retinopathy, which can be proliferative or non proliferative. Proliferative sickle cell retinopathy potentially leads to blindness. There is a paucity of data on sickle cell disease-related retinopathy from Africa, where the disease is most prevalent. We aimed to determine the clinical, ophthalmic, and laboratory predictors of sickle cell retinopathy in an African population. We conducted a cross-sectional study of 262 participants, aged 13 years and above, with sickle cell disease. Demographic and clinical data were collected using a structured questionnaire and standard physical examinations. Vitreo-retinal specialists performed eye examinations on all the participants. Hematological and biochemical assessments were conducted using standard methods. A multivariate stepwise forward logistic regression was performed to determine the predictors of retinopathy. The median age of the participants was 20 years (interquartile range: 17-25 years). Most of the participants had a homozygous Hb S (HBB: c.20A>T) genotype (96.9%), with 3.1% who carried a Hb S/Hb C (HBB: c.19G>A) genotype. The prevalence of non proliferative sickle cell retinopathy was 24.4%. Only 1.9% had proliferative sickle cell retinopathy (PSCR). Elevated systolic blood pressure (BP) [odds ratio (OR): 6.85, 95% confidence interval (95% CI): 1.05-44.45, p = 0.059], moderate visual impairment (OR: 5.2, 95% CI: 1.39-19.63, p = 0.015), and anterior segment changes (OR: 2.21, 95% CI: 1.19-4.13, p = 0.012) were independently predictive of retinopathy. This study provides new insight into predictors of retinopathy in sickle cell disease, with implications on early screening and prevention.

Sickle cell disease clinical phenotypes in Nigeria: A preliminary analysis of the Sickle Pan Africa Research Consortium Nigeria database.

BACKGROUND/OBJECTIVE: Sickle cell disease (SCD) is a monogenic disease with multiple phenotypic expressions. Previous studies describing SCD clinical phenotypes in Nigeria were localized, with limited data, hence the need to understand how SCD varies across Nigeria. METHOD: The Sickle Pan African Research Consortium (SPARCO) with a hub in Tanzania and collaborative sites in Tanzania, Ghana and Nigeria, is establishing a single patient-consented electronic database with a target of 13,000 SCD patients. In collaboration with the Sickle Cell Support Society of Nigeria, 20 hospitals, with paediatric and adult SCD clinics, are participating in patient recruitment. Demographic and clinical information, collected with uniform case report forms, were entered into Excel spreadsheets and uploaded into Research Electronic Data Capture software by trained data clerks and frequency tables generated. RESULT: Data were available on 3622 patients enrolled in the database, comprising 1889 (52.9%) females and 1434 (39.6%) children ≤15 years. The frequencies of Hb SS, Hb SC and Hb Sß thalassemia in this data set were 97.5%, 2.5% and 0% respectively. Sixty percent, 23.8%, 5.9%, 4.8% and 2.5% have had bone pain crisis, dactylitis, acute chest syndrome, priapism and stroke respectively. The most frequent chronic complications were: leg ulcers (6.5%), avascular necrosis of bone (6.0%), renal (6.3%) and pulmonary hypertension (1.1%). Only 13.2% had been hospitalized while 67.5% had received blood transfusion. CONCLUSION: These data on the spectrum of clinical phenotypes of SCD are useful for planning, improving the management of SCD across Nigeria and provide a foundation for genomic research on SCD.

Red cell exchange for patients with sickle cell disease: an international survey of current practices.

INTRODUCTION: Red cell exchange (RCE) therapy is increasingly used to treat patients with acute or chronic manifestations of sickle cell disease (SCD). However, little is known regarding the most safe and effective practice parameters associated with this particular therapy. METHODS: A SCD subcommittee of members of the American Society for Apheresis (ASFA) developed a 122-question survey and administered it via email to other ASFA members. The survey inquired about clinical indications for treatment, practice patterns, and transfusion policies for RCE when used for patients with SCD. RESULTS: Ninety-nine distinct institutions completed the survey. Twenty-one (21%) were from outside of the US. Twenty-two (22%) provided chronic transfusion therapy to >10 patients, and both adult (25%) and pediatric-focused services (20%) were represented. Common acute indications for RCE included acute chest syndrome, acute ischemic stroke, and pre-surgical prophylaxis. Common chronic indications included primary stroke prophylaxis, secondary stroke prophylaxis, and recurrent acute chest syndrome. Respondents most commonly set a post-RCE treatment target of 30% for the hematocrit and hemoglobin S levels, regardless of the therapeutic indication. Units for RCE were phenotypically matched in 95% of cases. About 40% of respondents reported using isovolemic hemodilution. CONCLUSIONS: This survey solicited the current practice variations in RCE from a diverse range of practice sites. Many sites reported similar practice patterns and challenges but some variations emerged. To our knowledge, this survey represents the largest and most in-depth investigation of the use of RCE for patients with SCD, and could inform future studies in the field.

Maternal Instruction About Jaundice and the Incidence of Acute Bilirubin Encephalopathy in Nigeria.

OBJECTIVE: To evaluate whether teaching mothers about neonatal jaundice will decrease the incidence of acute bilirubin encephalopathy among infants admitted for jaundice. STUDY DESIGN: This was a multicenter, before-after and cross-sectional study. Baseline incidences of encephalopathy were obtained at 4 collaborating medical centers between January 2014 and May 2015 (Phase 1). Structured jaundice instruction was then offered (May to November 2015; Phase 2) in antenatal clinics and postpartum. Descriptive statistics and logistic regression models compared 3 groups: 843 Phase 1 controls, 338 Phase 2 infants whose mothers received both antenatal and postnatal instruction (group A), and 215 Phase 2 infants whose mothers received no instruction (group B) either because the program was not offered to them or by choice. RESULTS: Acute bilirubin encephalopathy occurred in 147 of 843 (17%) Phase 1 and 85 of 659 (13%) Phase 2 admissions, which included 63 of 215 (29%) group B and 5 of 338 (1.5%) group A infants. OR for having acute bilirubin encephalopathy, comparing group A and group B infants adjusted for confounding risk factors, was 0.12 (95% CI 0.03-0.60). Delayed care-seeking (defined as an admission total bilirubin ≥18 mg/dL at age ≥48 hours) was the strongest single predictor of acute bilirubin encephalopathy (OR 11.4; 6.6-19.5). Instruction decreased delay from 49% to 17%. Other major risk factors were home births (OR 2.67; 1.69-4.22) and hemolytic disease (hematocrit ≤35% plus bilirubin ≥20 mg/dL) (OR 3.03; 1.77-5.18). The greater rate of acute bilirubin encephalopathy with home vs hospital birth disappeared if mothers received jaundice instruction. CONCLUSIONS: Providing information about jaundice to mothers was associated with a reduction in the incidence of bilirubin encephalopathy per hospital admission.

Correlating transcranial arterial Doppler velocities with haematologic parameters and haemolytic indices of Nigerian children with sickle cell anaemia.

INTRODUCTION: Cerebral vasculopathy, elevated transcranial Doppler velocities and stroke are linked to excessive intravascular haemolysis in sickle cell anaemia. This study determined the prevalence and pattern of abnormal blood flow velocities in children with sickle cell anaemia from Northern Nigeria using transcranial Doppler and to correlate transcranial Doppler velocities with haematological and biochemical markers of haemolysis. METHODS: Full blood count, reticulocyte count, fetal haemoglobin and some selected biochemical markers of haemolysis of 100 children with sickle cell anaemia were determined at steady state. The time-averaged mean of maximal velocities in four major intracranial blood vessels was measured using non-imaging transcranial Doppler, which were then classified according to the stroke prevention in sickle cell disease trial protocol. A simple linear correlation between time-averaged mean of maximal velocities in the four major intracranial vessels as the dependent variable and haematological and biochemical markers of haemolysis as independent variables was also determined at the significance level of 0.05. RESULTS: Abnormal transcranial Doppler velocities, classified as high risk, were found in 3% of the studied patients; 11% had intermediate risk while 84% had standard risk. Most high velocities were detected in the middle cerebral artery. The time-averaged mean of maximal velocities of middle cerebral artery positively correlated with total white blood cell count, absolute neutrophil count, platelet count, reticulocyte count, serum lactate dehydrogenase and total bilirubin, while it was negatively correlated with haematocrit and fetal haemoglobin levels. CONCLUSION: Our study showed low prevalence of abnormal transcranial Doppler velocities and low prevalence is unrelated to low markers of haemolysis in our study population.

Evaluating laboratory request forms submitted to haematology and blood transfusion departments at a hospital in Northwest Nigeria.

BACKGROUND: The laboratory request form (LRF) is a communication link between laboratories, requesting physicians and users of laboratory services. Inadequate information or errors arising from the process of filling out LRFs can significantly impact the quality of laboratory results and, ultimately, patient outcomes. OBJECTIVE: We assessed routinely-submitted LRFs to determine the degree of correctness, completeness and consistency. METHODS: LRFs submitted to the Department of Haematology (DH) and Blood Transfusion Services (BTS) of Aminu Kano Teaching Hospital in Kano, Nigeria, between October 2014 and December 2014, were evaluated for completion of all items on the forms. Performance in four quality indicator domains, including patient identifiers, test request details, laboratory details and physician details, was derived as a composite percentage. RESULTS: Of the 2084 LRFs evaluated, 999 were from DH and 1085 from BTS. Overall, LRF completeness was 89.5% for DH and 81.2% for BTS. Information on patient name, patient location and laboratory number were 100% complete for DH, whereas only patient name was 100% complete for BTS. Incomplete information was mostly encountered on BTS forms for physician's signature (60.8%) and signature of laboratory receiver (63.5%). None of the DH and only 9.4% of BTS LRFs met all quality indicator indices. CONCLUSION: The level of completion of LRFs from these two departments was suboptimal. This underscores the need to review and redesign the LRF, improve on training and communication between laboratory and clinical staff and review specimen rejection practices.