John Aneke

Current sickle cell disease management practices in Nigeria

BACKGROUND Although Nigeria has the highest burden of sickle cell disease (SCD) worldwide, there is still variable and poor utilisation of standard-of-care practices for SCD patients in the country. METHODS This was a questionnaire survey of doctors in some dedicated SCD clinics in Nigeria in order to document the facilities available and common management practices. RESULTS There were responses from 18 clinics based in 11 institutions. The number of patients being followed in each centre ranged from 15 to approximately 11 000. All clinics provided malaria prophylaxis and folic acid routinely to their patients. Only eight clinics prescribe penicillin prophylaxis. Eight prescribe hydroxyurea to patients who can afford it when indicated. All of the centres except three have electronic cell counters, but all had access to haemoglobin electrophoresis. Three had high-performance liquid chromatography machines installed but none was being routinely used. One institution had a functioning molecular biology laboratory. There is no official newborn screening programme in the country. All had access to microbiology and chemistry laboratories. Nine institutions had CT, six had MRI and three had transcranial Doppler facilities. CONCLUSION The care available for SCD in Nigeria is still suboptimal and there is an urgent need for concerted effort to tackle the problem, but to make a significant impact on the burden of the disease would require more focus at the primary care level. Some steps to achieving this are outlined.

Degrees of Kidney Disease in Nigerian Adults with Sickle-Cell Disease

Objective: To study degrees of chronic kidney disease (CKD) using creatinine clearance in adult Nigerian patients with sickle-cell disease (SCD). Methods: One hundred SCD patients, made up of 79 HbSS (homozygous haemoglobin S) patients and 21 HbSC (heterozygous haemoglobins S and C) patients, were investigated prospectively, along with 50 normal controls. Their sociodemographic data, weight and drug history were documented. Each participant underwent dipstick urinalysis, and creatinine clearance was calculated following a 24-hour urine collection and serum creatinine measurement. They were categorized into stages of CKD based on the creatinine clearance. Results: Of the 79 HbSS patients, 14 (18%), 28 (35%), 33 (42%) and 4 (5%) had stage 1, 2, 3 and 4 CKD, respectively. In the HbSC group, 3 (14%), 9 (43%) and 9 (43%) patients had stage 1, 2 and 3 CKD, respectively. Proteinuria was noted in 16 (20%) HbSS patients but not in any of the HbSC patients. Of the subjects aged ≤24 years (n = 49), 9 (18%), 18 (37%), 21 (43%) and 1 (2%) had stage 1, 2, 3 and 4 CKD, respectively. Of those aged >24 years (n = 51), 8 (16%), 19 (37%), 21 (41%) and 3 (6%) had stage 1, 2, 3 and 4 CKD, respectively. None of the subjects had stage 5 CKD. Conclusion: In this study, the adult subjects with SCD had various degrees of CKD. Adequate follow-up and active intervention are advocated to delay the onset of end-stage nephropathy.

Preliminary Evaluation of a Point-of-Care Testing Device (SickleSCAN™) in Screening for Sickle Cell Disease

Abstract Sickle cell disease affects about 150,000 births annually in Nigeria. Early diagnosis is hampered by factors such as centralized and urban localization of laboratories, high cost of diagnostic equipment and inadequate skilled manpower to operate them. The need for a low-cost, portable, easy-to-use diagnostic test for sickle cell disease is critical, especially in resource-poor countries. In this study, we evaluated the performance characteristics of a novel point-of-care testing device (SickleSCAN™), and its acceptability and feasibility, as a possible screening tool for sickle cell disease. In the first phase, we assessed the performance characteristics of SickleSCAN™ by evaluating 57 subjects comprising both children and adults attending a primary health center, for Hb SS (βS/βS; HBB: c.20A>T), Hb SC (βS/βC; HBB: c.19G>A) and Hb AS (βA/βS) using SickleSCAN™, cellulose acetate electrophoresis (CAE) and high performance liquid chromatography (HPLC). Performance characteristics such as diagnostic sensitivity and specificity were compared to HPLC as a standard method. We subsequently undertook a second phase wherein the acceptability and feasibility of the device for sickle cell disease screening, was evaluated using semi-structured and structured questionnaires among 197 healthcare personnel and 221 subjects, respectively. Sickle cell disease was carried by 3.4% of the subjects. The diagnostic sensitivity, specificity and test efficiency of SickleSCAN™ for sickle cell disease (Hb SS and Hb SC), were 100.0, 98.2 and 98.2%, respectively. Findings from this study showed SickleSCAN™ to be a viable screening tool that can easily be applied in community-based screening for early diagnosis of sickle cell disease with little expertise and low cost.

Blood transfusion services for patients with sickle cell disease in Nigeria

BACKGROUND Safe, timely red blood cell transfusion saves lives and chronic transfusion therapy (CTT) prevents or limits morbidities such as stroke, therefore improving quality of life of patients with sickle cell disease (SCD). METHODS This questionnaire-based study assessed the ability of sickle cell centers in Nigeria to provide safe blood to patients with SCD between March and August 2014. RESULTS Out of the 73 hospitals contacted, responses were obtained from 31. Twenty four (78%) hospitals were unable to transfuse patients regularly due to blood scarcity. Packed red blood cells were available in 14 (45%), while only one provided leukocyte-depletion. Most centers assessed donor risk and screened for HIV in 30 (97%), hepatitis B in 31(100%) and hepatitis C in 27 (87%) hospitals. Extended phenotyping and alloantibody screening were not available in any center. A quarter of the hospitals could monitor iron overload, but only using serum ferritin. Access to iron chelators was limited and expensive. Seventeen (55%) tertiary hospitals offered CTT by top-up or manual exchange transfusion; previous stroke was the most common indication. CONCLUSION Current efforts of Nigerian public hospitals to provide safe blood and CTT fall short of best practice. Provision of apheresis machines, improvement of voluntary non-remunerated donor drive, screening for red cell antigens and antibodies, and availability of iron chelators would significantly improve SCD care in Nigeria.

Seroprevalence of transfusion transmissible viral markers in sickle cell disease patients and healthy controls in Ile-Ife, South-Western Nigeria: A case–control study

Background: The risk of acquiring transfusion transmissible viral infections is said to be higher in patients requiring regular blood transfusions such as those with sickle cell disease (SCD). Aim: We determined the seroprevalence of blood transfusion viral markers among our patients with SCD. Subjects and Methods: This a case–control analytical study consisting of 82 confirmed SCD patients on routine follow-up at our facility in steady state and 90 age-matched controls. Demographic and transfusion history were recorded while 5 ml of blood was drawn for hematocrit levels, and serum tested for transfusion transmissible viral markers for hepatitis B, (hepatitis B surface antigen [HBsAg], hepatitis B surface antibody [HBsAb], hepatitis B e antigen [HBeAg], hepatitis B e antibody [HBeAb], hepatitis B core antibody [HBcAb]), hepatitis C virus (HCV) and human immunodeficiency viruses (HIVs) using the rapid test kits. Ethical approval for the study was obtained from the Institutional Review Board and each participant gave informed consent. Data were analyzed using descriptive and inferential statistics. Results: The seroprevalence of HBsAg, HBsAb, and HBeAg in cases was 2 (2.4%), 7 (8.5%), and 0 (0.0%), respectively, whereas it was 7 (8.5%), 11 (13.4%), 6 (7.3%), and 2 (2.4%) for HBeAb, HBcAb, HCV, and HIV antibodies. Compared to the controls, cases had higher prevalence rate of HBeAb ( P = 0.005). No significant difference was observed in those with or without low hematocrit (≤18%) or those that received blood transfusion and those that did not ( P > 0.05). Conclusion: We conclude that blood transfusion did not significantly increase the seroprevalence of markers of transfusion transmissible viral infection in SCD patients.

Avascular necrosis significantly impairs quality of life in sickle cell disease

Introduction: Quality of life (QoL) assessment has become an integral component of the assessment of the holistic care of patients with chronic diseases, including sickle cell disease (SCD). Objective: To evaluate the quality of life in patients with SCD managed in our centre. Patients and Methods: Eighty consecutive patients with confirmed hemoglobin SS or SC were recruited. Age and sex-matched volunteers served as controls. Ethical approval was obtained from the Institutional Review Board and all participants gave informed consent. Information on socio-demographic, quality of life and clinical variables, including the presence of complications were recorded in a modified version of the WHO Quality of Life Brief version (WHOQOL-BREF) questionnaire. Data was analyzed using Microsoft Excel and SPSS 17 computer softwares. Descriptive statistics were used to represent socio-demographic variables while the Student t-test was used to explore relationship between the variables and the quality of life domains. Results: Significantly fewer participants with SCD are married compared to their age- and sex-matched controls (P = 0.01). Similarly, participants with SCD scored significantly lower in the physical and psychological domains as well as in overall QoL and general health domains compared to controls (P = 0.001). Avascular necrosis of the femur significantly affected the overall QoL and general health of participants with SCD, respectively while the means of the QoL assessment domains were not significantly different in participants with SCD with and without complications, except in the general health domain (P < 0.001). Conclusion: Avascular necrosis of the femoral head significantly affects overall QoL in participants with SCD.

Disease Severity Scores and Haemogram Parameters in Nigerian Sickle Cell Disease Patients

Background: Sickle cell disease (SCD) is associated with high mortality in Nigeria and the rest of sub-Saharan Africa; there is need to find easily available parameters that could predict disease severity and influence therapy. Objective: To evaluate the haemogram of a population of SCD patients and correlate these with objective scores of disease severity. Methods: Sixty (60) asymptomatic steady state (ASS) SCD patients in our clinic were randomly selected and interviewed with a questionnaire. Their haemogram was done using a 17 parameter, 3-part white cell differential, auto-analyser (KX 21N, Sysmex corporation, Chuo ku, Kobe, Japan) and objective severity scores calculated using a modification of the method proposed by Anyeagbu et al. Statistical analysis of data was done using Statistical Package for Social Sciences software, version 20 (SPSS Inc., IL, Chicago, USA), with significance assigned to p values less than 0.05. Result: Of the 60 subjects assessed, severity scores were calculated for 49: 11 (22.4%), 31 (63.3%) and 7 (14.3%) met the criteria for mild, moderate and severe disease respectively. The haemogram parameters that were significantly correlated with disease severity were mean corpuscular haemoglobin concentration (MCHC), and white blood cell count (WBC), p=0.014, and 0.001 respectively. Haemoglobin concentration (Hb) and packed cell volume (PCV) were negatively correlated with disease severity (p=0.001). Conclusion: In addition to already known haemogram parameters that affect SCD severity (such as WBC, Hb concentration, and PCV) MCHC also does same and can be manipulated by drugs and other kinds of therapy to ameliorate severity in patients.

A Multi-centre Survey of Acceptability of Newborn Screening for Sickle Cell Disease in Nigeria

Background Sickle cell disease (SCD) is a major genetic disease that manifests early in life and may lead to significant morbidities. One of the health care services that have been effective in reducing the burden of SCD in developed countries is newborn screening (NBS) followed by pneumococcal vaccines, penicillin prophylaxis, and hydroxyurea treatment. Yet, in sub-Saharan African countries, where about 75% of annual affected babies worldwide are born, NBS programmes are largely unavailable. It is not clear whether this is due to technical challenges associated with setting up such programmes, or significant cultural and social barriers to its acceptance in such settings. Objective Our aim was to ascertain the attitudes to and acceptability of NBS in Nigeria among various socio-demographic groups including health professionals, undergraduate students, parents of children with SCD and SCD patients. Methods Data on socio-demographic characteristics, knowledge of SCD and attitude towards NBS were collected using a semi-structured pre-tested questionnaire from April to July 2014 across 15 health institutions and university campuses in Nigeria. Data were collected from 1,301 respondents across Nigeria. Results There was good knowledge of SCD as an inherited blood disorder. Although 86% of respondents (n = 1,119) supported NBS, there was a statistically significant relationship between support for NBS and age (p = 003), educational status (p = 000) and religion (p = 000). Conclusion This study suggests that there is a good acceptability of NBS across Nigeria. The main barriers to its use are likely to be financial and practical, rather than social or cultural.

Occupational Exposure to Oxyacetylene Flame Could Predispose to Macrocytic Anaemia in Welders in Nnewi, South East Nigeria -

Background: Oxyacetylene flame is known to contain a number deleterious components which could be of potentially occupational health importance. Objective: To evaluate the effects of occupational exposure to oxyacetylene flame on haematological indices in a population of welders in Nnewi, Nigeria. Subjects and Methods: A total of 100 subjects were randomly selected, comprising of 50 apparently healthy welders and controls, respectively. Subjects were further stratified into those that consumed alcohol and/or tobacco products and those who did not. Three milliliter (3ml) of venous blood was collected from each participant and dispensed into potassium- ethylene diamine tetra acetic acid (K-EDTA) containers for full blood count determination. This was done using the Sysmex® automated haematology analyser, Model Number: PCE 210. Results were expressed as means ± SD while the students t-test and Analysis of Variance (ANOVA) were used for comparison of means; P was significant at