Preprint Article Version 1 Preserved in Portico This version is not peer-reviewed

Feasibility and Applicability of Standardized and Regular Follow-up of Sickle Cell Patients in a Remote Area of a Developing Country

Version 1 : Received: 18 May 2020 / Approved: 18 May 2020 / Online: 18 May 2020 (17:26:04 CEST)
Version 2 : Received: 20 September 2020 / Approved: 22 September 2020 / Online: 22 September 2020 (08:12:05 CEST)
Version 3 : Received: 10 October 2020 / Approved: 12 October 2020 / Online: 12 October 2020 (15:14:46 CEST)

A peer-reviewed article of this Preprint also exists.

Mbiya, B.M., Kalombo, D.K., Mukendi, Y.N. et al. Improvement of SCD morbimortality in children: experience in a remote area of an African country. BMC Health Serv Res 21, 294 (2021). https://doi.org/10.1186/s12913-021-06286-7 Mbiya, B.M., Kalombo, D.K., Mukendi, Y.N. et al. Improvement of SCD morbimortality in children: experience in a remote area of an African country. BMC Health Serv Res 21, 294 (2021). https://doi.org/10.1186/s12913-021-06286-7

Abstract

Background: Sickle cell disease (SCD) is the most common hemoglobinopathy in the Democratic Republic of Congo (DRC) and poses a public health problem. If, without clinical follow-up, more than 50% of children with SCD die before their 5th birthday. A regular medical follow-up is the first simple improvement that can be beneficial for SCD patients in a remote city of an African country. Method: A cohort of 143 children with SCD aged 10 years old (IQR [inter quartile range]: 6–15 years) (sex ratio male: female = 1.3) were clinically followed for 12 months without any specific intervention and then 12 months with a monthly medical visit, a biological follow-up and regular prophylaxis. A paired Student test and pairwise Wilcoxon test were used to compare study outcomes. Results: The median age of patients at the diagnosis of SCD was 2 years (IQR: 1–5). Anemia was noted in 100% of patients at inclusion, with severe and normocytic anemia in 80% of cases (n = 115). The implementation of standardized and regular follow-up has shown an increase in the annual mean hemoglobin level from the percentage of the low limit normal for the age from 54 to 77 % (p = 0.001), and a decrease of the lymphocytes count and spleen size (p < 0.001). The implementation of regular follow-up has also shown a significant decrease (p < 0.001) in the average annual number of hospitalizations and episodes with vaso-occlusive crises, blood transfusions, infections, and acute chest syndromes. Conclusions: This study showed that the regular follow-up of children with SCD and the application of SCD management recommendations are possible and applicable in the context of a remote city in a developing country. Simple and accessible measures included in conventional recommendations can reduce the morbimortality of these patients in remote areas if applied rigorously with regular follow-up.

Keywords

sickle cell disease; feasibility; regular follow-up; remote area; Democratic Republic of Congo

Subject

Medicine and Pharmacology, Pediatrics, Perinatology and Child Health

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