Improvement of SCD morbimortality in children: experience in a remote area of an African country

Abstract

Background Sickle cell disease (SCD) is a public health problem in the Democratic Republic of Congo. While reference sickle cell centers have been implemented in capital cities of African countries and have proven to be beneficial for SCD patients. In the Democratic Republic of Congo, they have never been set up in remote areas for families with low or very low sources of income. Method A cohort of 143 children with SCD aged 10 years old (IQR (interquartile range): 6–15 years) (sex ratio male/female = 1.3) were clinically followed for 12 months without any specific intervention aside from the management of acute events, and then for 12 months with a monthly medical visit, biological follow-up, and chemoprophylaxis (folic acid/penicillin), adequate fluids and malaria prevention. Results The median age of patients at the diagnosis of SCD was 2 years (IQR: 1–5). The implementation of standardized and regular follow-ups in a new sickle cell reference center in a remote city showed an increase in the annual mean hemoglobin level from 50 to 70 g/L (p = 0.001), and a decrease in the lymphocyte count and spleen size (p < 0.001). A significant decrease (p < 0.001) in the average annual number of hospitalizations and episodes of vaso-occlusive crises, blood transfusions, infections, and acute chest syndromes were also observed. Conclusions The creation of a sickle cell reference center and the regular follow-up of children with sickle cell disease are possible and applicable in the context of a remote city of an African country and represent simple and accessible measures that can reduce the morbimortality of children with sickle cell disease.

Evidence-based interventions for reducing sickle cell disease-associated morbidity and mortality in sub-Saharan Africa: A scoping review

“…We excluded 129 articles for the following reasons: no empirical evaluation of the effectiveness of the intervention ( n = 42), non-SSA countries ( n = 40), non-English language studies ( n = 12), non-evidenced-based studies ( n = 10), study protocols ( n = 5), abstracts ( n = 5) and adults population only ( n = 15). Finally, 36 articles were included in this study 7 , 27 – 60 – Figure 1 .…”

Acute Complications of Sickle Cell Disease in Children Under 5 Years at a Level II Hospital

“…This highlights the disparity of resources between develop and underdeveloped countries, as can be seen in a study recently published by a sickle cell center in the Democratic Republic of Congo, where the median diagnosis age was 2 years (IQR: 1-5), with only 37% being diagnosed under 12 months, with no neonatal screening. 10 …”

“…This highlights the disparity of resources between develop and underdeveloped countries, as can be seen in a study recently published by a sickle cell center in the Democratic Republic of Congo, where the median diagnosis age was 2 years (IQR: 1-5), with only 37% being diagnosed under 12 months, with no neonatal screening. 10 It was not possible to correlate the age of diagnosis and the number of hospitalizations, although it would make sense that an earlier diagnosis would be associated with fewer hospitalizations. This was not possible probably due to the small sample and the criteria established.…”

Co-occurrence of sickle cell disease and oculocutaneous albinism in a Congolese patient: a case report

“…Its origin is linked to a point mutation in the globin gene located on chromosome 11 (11p 11-5) giving rise to abnormal hemoglobin, called hemoglobin S (HbS) [ 5 ]. The disease affects 20–25 million people worldwide, among which 50–80% of those born in sub-Saharan Africa die before the age of five if they do not benefit from optimal medical monitoring, including prevention of infectious disease [ 6 , 7 ]. In the Democratic Republic of Congo (DRC), as highlighted by recent epidemiological data, 2% of newborns are homozygous for hemoglobin S [ 8 , 9 ].…”