Paediatric sickle cell disease at a tertiary hospital in Malawi: a retrospective cross-sectional study

Abstract

IntroductionSickle cell disease (SCD) remains a major cause of childhood mortality and morbidity in Malawi. However, literature to comprehensively describe the disease in the paediatric population is lacking.MethodsA retrospective review of clinical files of children with SCD was conducted. Descriptive statistics were performed to summarise the data. χ2 or Fisher’s exact test was used to look for significant associations between predictor variables and outcome variables (case fatality and length of hospital stay). Predictor variables that were significantly associated with outcome variables (p≤0.05) in a χ2 or Fisher’s exact test were carried forward for analysis in a binary logistic regression. A multivariable binary logistic regression was used to identify covariates that independently predicted length of hospital stay.ResultsThere were 16 333 paediatric hospitalisations during the study period. Of these, 512 were patients with SCD representing 3.1% (95% CI: 2.9%- 3.4%). Sixty-eight of the 512 children (13.3%; 95% CI: 10.5% - 16.5%) were newly diagnosed cases. Of these, only 13.2% (95% CI: 6.2% - 23.6%) were diagnosed in infancy. Anaemia (94.1%), sepsis (79.5%) and painful crisis (54.3%) were the most recorded clinical features. The mean values of haematological parameters were as follows: haemoglobin (g/dL) 6.4 (SD=1.9), platelets (×109/L) 358.8 (SD=200.9) while median value for white cell count (×109/L) was 23.5 (IQR: 18.0–31.2). Case fatality was 1.4% (95% CI: 0.6% - 2.8%)and 15.2% (95% CI: 12.2% -18.6%) of the children had a prolonged hospital stay (>5 days). Patients with painful crisis were 1.7 (95% CI: 1.02 - 2.86) times more likely to have prolonged hospital stay than those without the complication.ConclusionAnaemia, sepsis and painful crisis were the most common clinical features paediatric patients with SCD presented with. Patients with painful crisis were more likely to have prolonged hospital stay. Delayed diagnosis of SCD is a problem that needs immediate attention in this setting. Although somewhat encouraging, the relatively low in-hospital mortality among SCD children may under-report the true mortality from the disease considering community deaths and deaths occurring before SCD diagnosis is made.

Prevalence, Pattern of Disease and Outcome of Children with Sickle Cell Disease Admitted in a Private Health Facility in Southern Nigeria

“…It is pertinent to note that priapism and acute sequestration crisis were one of the least observed morbidities in children admitted with SCD in the present study. This was consistent with other studies [14,17] which observed priapism as the least common morbidity. In contrast, priapism was not documented in some other studies [3,10,15,18,21].…”

“…Males predominated in the present study as similarly documented in other studies in Nigeria [10,[15][16][17]19,20], Malawi, [14] India [3,21] Iraq [18] and Barbados [22]. Although SCD is an autosomal recessive disorder and as such not related to gender, Gladwin et al [23] and Ilesanmi [24] in their studies showed that nitric oxide was found more in females than males during both steady state and crisis which thus indicates that males could be more prone to crisis and attendant complications.…”

“…Sickle cell disease in the present study accounted for 3.7% of the total admission. This is similar to the 3.9% reported in Enugu [13] eastern Nigeria but higher than the 3.1%, 2.9% and 2.2% reported in Malawi [14], Port Harcourt [15] southern and another study in Enugu [16], eastern Nigeria respectively. It was however lower than the 4.1%, 5.75% and 10.1% documented in Uyo [17] southern Nigeria, Iraq [18] and Zaria [19] northern Nigeria respectively.…”

“…It is worthy of note that it is a regular practice to commence all children diagnosed with SCD in the present study centre on prophylactic penicillin. Acute painful crisis/VOC which was 2 nd commonest in the present study was reported as the commonest morbidity in other parts of Nigeria [10,15,19,20], Saudi Arabia [1], Iraq [18] and Barbados [22] whereas severe anaemia was the commonest in Malawi [14] and India [21] while malaria was the commonest in Ibadan [17] and a previous study in Port Harcourt [15] Nigeria. Malaria being the 3 rd commonest cause of hospitalization in the present study, a private health facility could be attributed to the possibility of less exposure to the mosquitoes, better housing and better compliance to malaria chemoprophylaxis.…”

“…Ikefuna and Emodi [16] in Enugu, Nigeria reported age group 1-5 years as the commonest age group admitted whereas Jain et al [3] in India reported 1-< 3 years as the commonest age group implicated. In contrast, older children above 5 years were reported as the commonest age groups documented by Abd El-Ghany et al [1], Chimbatata et al [14], Salman and Hassan [18], Brown et al [10] and Etuk & Akpan [17] in Saudi Arabia, Malawi, Iraq, and Nigeria respectively. These variations could be due to differences in the pattern of diseases and their varying geographic locations.…”