Samuel Ademola Adegoke

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.

Sickle cell disease clinical phenotypes in children from South-Western, Nigeria

BACKGROUND The clinical phenotypes of children with sickle cell disease (SCD) are poorly described in many sub-Saharan countries including Nigeria. OBJECTIVES The objective was to highlight various clinical phenotypes of SCD in children and investigate the influence of sociodemographic indices on the development of SCD complications. METHODS We carried out a cross-sectional study of 240 pediatric patients attending the sickle cell clinic and the emergency room in a teaching hospital in South-Western Nigeria over a 12-month period. The clinical phenotypes and severity of the disease were documented, and the influence of sociodemographic variables was investigated. RESULTS The five leading clinical phenotypes in our patients were significant pain episodes, that is, vaso-occlusive crisis in 159 (66.3%); anemic crisis in 62 (25.8%); severe bacterial infections, 57 (23.8%); acute chest syndrome (ACS), 27 (11.3%) and stroke, 7 (2.9%). Forty-two (33.1%) had a previous history of dactylitis (hand-foot syndrome). Other clinical phenotypes such as avascular necrosis of the femur, 4 (1.7%); nephropathy, 2 (0.8%); priapism, gallstone and chronic leg ulcer, one (0.4%) each, were not commonly seen. More children with a history of asthma had ACS. Furthermore, high steady-state white blood cell count was associated with severe disease. CONCLUSION The clinical phenotypes of SCD in children from South-Western Nigeria are highly variable with the disease manifesting very early and about 10% having significant complications. Sociodemographic characteristics appear to have little influence on the development of SCD complications among our patients, but age and low-socioeconomic class are associated with anemic crisis.

Psychosocial burden of sickle cell disease on the family, Nigeria.

Abstract Background Sickle Cell Disease (SCD), the most common genetic disorder amongst Black people, poses a significant psychosocial burden on the sufferers, the caregivers and their families. Objective and methods From 01 January 2011 to 30 September 2011 the caregivers of children with SCD treated at the Paediatric Haematology Clinic of the University Teaching Hospital in Ado-Ekiti, Nigeria, were included in a study, using a structured questionnaire and a validated, culture-relevant disease burden interview to assess the psychosocial burden of SCD on these caregivers. Three main objective psychosocial domains and some subjective domains, including the caregivers’ coping ability were assessed. Results A total of 225 caregivers, consisting of 202 mothers (89.8%), 15 grandmothers (6.7%) and 8 fathers (3.6%) were studied. In 53.3% of the families, the care of children with SCD adversely affected the provision of their basic needs, with 73.3% of the caregivers stating that time spent caring for the child made them lose income or financial benefits; 19.6% of the caregivers had to take out loans to meet the expenditure of the patients illness. Caring for children with SCD reportedly made 42.2% of the caregivers neglect other family members. In addition, 14.2% of the families experienced moderate to severe disruption in their day-to-day interactions within the family to the extent that 12.4% frequently quarrelled due to the childs illness. Conclusion Caregivers are faced with enormous financial, interpersonal and psychological problems. Social support should be available to alleviate caregivers’ and/or family members’ burdens.

Determinants of Oxygen Therapy in Childhood Pneumonia in a Resource-Constrained Region

Childhood pneumonia is a leading cause of morbidity and mortality among underfives particularly in the resource-constraint part of the world. A high proportion of these deaths are due to lack of oxygen, thereby making oxygen administration a life-saving adjunctive when indicated. However, many primary health centres that manage most of the cases often lack the adequate manpower and facilities to decide which patient should be on oxygen therapy. Therefore, this study aimed to determine factors that predict hypoxaemia at presentation in children with severe pneumonia. Four hundred and twenty children aged from 2 to 59 months (40% infants) with severe pneumonia admitted to a health centre in rural Gambia were assessed at presentation. Eighty-one of them (19.30%) had hypoxaemia (oxygen saturation < 90%). Children aged 2–11 months, with grunting respiration, cyanosis, and head nodding, and those with cardiomegaly on chest radiograph were at higher risk of hypoxaemia (P < 0.05). Grunting respiration (OR = 5.210, 95% CI 2.287–7.482) and cyanosis (OR = 83.200, 95% CI 5.248–355.111) were independent predictors of hypoxaemia in childhood pneumonia. We conclude that children that grunt and are centrally cyanosed should be preferentially commenced on oxygen therapy even when there is no facility to confirm hypoxaemia.

Relationship between serum 25-hydroxyvitamin D and inflammatory cytokines in paediatric sickle cell disease

Background Alteration in the concentration of inflammatory cytokines may contribute to pathogenesis in sickle cell anaemia (SCA). Vitamin D may suppress pro‐inflammatory cytokines and enhance anti‐inflammatory cytokines. Objective To compare steady state levels of pro‐and anti‐inflammatory cytokines of Nigerian SCA children with age‐ and sex‐matched healthy controls, and determine the relationship with 25‐hydroxyvitamin‐D (25‐OHD). Effects of three months of vitamin D supplementation on cytokines of SCA children with suboptimal 25‐OHD were also evaluated. Methods Serum 25‐OHD, IL‐1&bgr;, 2, 6, 8, 11, 12, 13, 17, 18 of 95 SCA children and 75 matched controls were determined using HPLC. The 12 SCA children with suboptimal 25‐OHD received 2000 IU of vitamin D daily for 3 months, and their post supplementation cytokines and 25‐OHD levels were compared with the baseline values. Results IL‐2, 6, 8, 12, 17 and 18 were higher in SCA children than the controls (p ≤ 0.001), but no significant variation in IL‐11 and 13 (p = 0.131 and 0.057 respectively). Patients with suboptimal serum 25‐OHD had higher IL‐6, 8 and 18 (p = 0.003, 0.010 and 0.002 respectively) and lower levels of IL‐11 (p = 0.005). Significant positive treatment effects were observed: post‐supplementation, serum 25‐OHD increased by 23.3 ng/mL, p < 0.001; proinflammatory cytokines IL‐2, 6, 8, 17 and 18 (p < 0.001) were reduced and anti‐inflammatory cytokine IL‐11 was increased, p < 0.001. Conclusions Suboptimal 25OHD is associated with enhanced levels of pro‐inflammatory markers in children with SCA. Three months of daily vitamin D supplementation reversed the trend. Hence; Vitamin D supplementation may reduce the inflammatory milieu and serve as an anti‐inflammatory agent in the management of SCA. HighlightsChronic inflammation results from imbalance in the activities of inflammatory cytokines in SCD.Vitamin D supplement improved anti‐inflammatory and lowered pro‐inflammatory cytokines in SCD.Vitamin D3 could be a new anti‐inflammatory agent for the future sickle cell disease management.

Influence of Lime Juice on the Severity of Sickle Cell Anemia

BACKGROUND AND OBJECTIVES The pain in sickle cell anemia (SCA) is often triggered by dehydration, acidosis, and fever that are usually due to malaria. Intake of lime juice was recently demonstrated to facilitate clearance of the malaria parasite. It was therefore sought to determine whether regular intake of lime juice will ameliorate crisis, especially recurrent bone pain. DESIGN In this preliminary, open-labeled, randomized study, the effects of lime juice on the clinical and some laboratory characteristics of children with SCA were tested. RESULTS Among the 113 children with SCA studied in two hospitals, the 58 receiving lime treatment had lower rates of significant painful episodes than the 55 without lime (37 versus 83 crises in 6 months, and 0.64±0.11 versus 1.51±0.34 average rates per child, p<0.001). Also, fewer subjects than the controls had significant painful episodes (50.0% versus 92.7%); febrile illness (46.6% versus 87.3%) and admission rate (3.4% versus 34.5%) (p<0.001). The mean hematocrit of the subjects (26.23±2.03%) at the end of the study was also higher, p<0.001. However, transfusion rate, presence of hepatomegaly, splenomegaly, and jaundice was similar. Treatment with lime did not cause any significant side-effect. CONCLUSIONS Regular intake of lime juice may be of great therapeutic and nutritional relevance in children with SCA.

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.

Risk factors for mortality in childhood pneumonia in a rural West African region

Pneumonia is a major killer of children worldwide. It is responsible for 19% of under-five-year-old mortality, of which 70% occurs in sub-Saharan Africa and South East Asia. A substantial proportion of deaths attributed to pneumonia is caused by failure to recognise factors at presentation that affect prognosis. The present study was aimed to assess for factors at presentation that determine mortality among children with WHO ARI defined severe pneumonia. This was a prospective observational study of consecutive children aged 2 to 59 months admitted with severe pneumonia at a major health centre in rural Gambia to de- termine the risk factors for mortality using logistic regression analysis. Four hundred and twenty (27.6%) out of the 1517 under- five admissions during the study period fulfilled the criteria of severe pneumonia using the WHO ARI criteria. Fifteen of the 420 cases died giving case fatality of 36 per 1000 admissions, with pneumonia accounting for 21.4% of all 70 deaths during the period. Although age ranges 12-23 months and 36-47 months, overcrowding, hypothermia at presentation, oedematous PEM, severe wasting, grunting respiration, convulsion, somnolence and hypoxaemia were significantly associated with mortality (p < 0.05); only convulsions ( OR = 16.64, 95% CI 1.028-1.033) and severe wasting (OR = 5.05, 95% CI 1.459-20.484,) were independent determinants of mortality. We conclude that children with severe pneumonia who in addition have severe wasting and convulsion are at increased risks of dying and should be managed in better equipped secondary or tertiary health facilities.

Intraventricular hemorrhage in newborns weighing <1500 g: Epidemiology and short-term clinical outcome in a resource-poor setting

Background: Intraventricular hemorrhage (IVH) is a major cause of death and the most important predictor of neurodevelopmetal disabilities in very low birth weight (VLBW) infants. Materials and Methods: Maternal, perinatal and infant data of 87 babies weighing <1500 g who had transfontanelle ultrasonography (TFU) scan were obtained to determine the prevalence, risk factors, features and outcome of IVH by multivariate analyses. Results: The prevalence of IVH was 24.1%. Gestational age <28 weeks; early onset sepsis (EOS) and severe birth asphyxia (SBA) at 5-min were associated with a greater risk of development of IVH. The use of antenatal steroids however reduced the risk. Respiratory distress, clinically identifiable seizures, temperature instability and acidosis, were more common in babies with IVH. Mortality rate was also significantly higher among them (P = 0.005). Babies with severe IVH were 23 times more likely to die than those without IVH (relative risk = 23.3, 95% confidence interval = 1.58-343.42, P = 0.010). Conclusion: The high-rate, morbidity and mortality of IVH emphasize the need for routine TFU scans among VLBW infants. Deliveries before 28 weeks, EOS, failure of antenatal steroids treatment and SBA contributed to the development of IVH. Aggressive prevention/management of these factors are necessary to reduce the burden of IVH in these high-risk neonates.

Predictors of paediatric injury mortality

Background. Childhood injury is a major public health issue in many parts of the world, contributing significantly to paediatric morbidity and mortality. World Health Organization recently projected that with the current trends, trauma and infectious diseases will account for equal numbers of year of potential life lost worldwide by the year 2020. Aim. This study aimed to investigate factors predictive of childhood injury mortality. Design. A prospective hospital-based cross-sectional study. Methods. All attendances and admissions to the Children’s Emergency Room at the Wesley Guild Hospital, Ilesa, Osun State, Nigeria, over a period of 2 years (1 June 2007 - 30 May 2009) were serially documented and all patients with injury were recruited into the study. The socio-demographic variables, injury characteristics, Pediatric Trauma Score (PTS) and Glasgow Coma Score (GCS) were tested against outcome by binary logistic regression analysis. Results. Five hundred and seventy-six children presented with injury during the study period with 22 deaths, giving an injury mortality rate of 3.8%. Logistic regression modelling found infancy (odds ratio (OR) 1.14, 95% confidence interval (CI) 0.04 - 0.37), head injury (OR 2.51, 95% CI 0.10 - 0.61), low PTS (≤8) (OR 8.95, 95% CI 0.86 - 0.94) and low GCS (<9) (OR 5.22, 95% CI 0.40 - 0.69) to be significant independent predictors of childhood injury mortality. Conclusion. Prompt identification of the above factors in children with injury may prevent many deaths.