Challenge of Managing Sickle Cell Disease in a Pediatric Population Living in Kinshasa, Democratic Republic of Congo: A Sickle Cell Center Experience

Hemoglobin ◽  
2014 ◽  
Vol 38 (3) ◽  
pp. 196-200 ◽  
Author(s):  
Michel Ntetani Aloni ◽  
Leonard Nkee
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Benoît Mukinayi Mbiya ◽  
Didier Kalenda Kalombo ◽  
Yannick Nkesu Mukendi ◽  
Valery Daubie ◽  
John Kalenda Mpoyi ◽  
...  

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.


2017 ◽  
Vol 1 (1) ◽  
pp. 3-9
Author(s):  
Salomon Agasa Batina ◽  
◽  
Paul Kombi Kambale ◽  
Marcel Poyo Sabiti ◽  
Charles Tshilumba Kayembe ◽  
...  

2020 ◽  
Vol 5 (3) ◽  
pp. 127
Author(s):  
Benoît Mukinayi Mbiya ◽  
Ghislain Tumba Disashi ◽  
Béatrice Gulbis

Background: Sickle cell disease is a major public health issue in the Democratic Republic of Congo (DRC), but it is still poorly understood by health professionals. The objective of this study was to assess the knowledge and practices of Congolese physicians treating sickle cell disease (SCD), in order to identify the areas for improvement in clinical care. Methods: This was a descriptive observational study conducted among Congolese physicians using a questionnaire. Participants were evaluated using a pre-established answer grid. Results: A total of 460 physicians participated, including 81 women (18%), with an average age of 35 years (range 25–60 years). Most physicians were general practitioners. Although self-assessment of their level of knowledge on SCD was estimated as average to good, less than half of the participants (n = 460; 46%) reported adequate management of vaso-occlusive crises, and only 1% of them had received specific training on SCD. Most physicians reported difficulties both in terms of diagnostic (65%) and management (79%) options of SCD patients. This study also showed that 85% of these physicians did not have access to the diagnostic tools for SCD. Conclusions: Insufficient knowledge on SCD and poor diagnostic and treatment options might contribute to increased morbidity and mortality of patients living in the DRC. Interventions aiming to improve physicians’ knowledge, patient follow-up, and treatment access are needed. Specific training alongside existing programs (HIV, malaria), early diagnosis of the disease, and the creation of patient advocacy groups should be implemented to improve SCD patient care.


2020 ◽  
Vol 3 (1) ◽  
pp. 57-62
Author(s):  
Mick Ya-Pongombo Shongo ◽  
◽  
Mimi Mujing Yav ◽  
Olivier Mukuku ◽  
Gaston Kankolongo ◽  
...  

Melioidosis and its germ are increasingly reported on the African continent and particularly in Central Africa, probably due to the increased awareness of clinicians and microbiologists and the growing recognition of the organism. It is called "Great Mimicker" because it produces a wide range of clinical characteristics such as would be found in patients living with sickle cell disease (SCD) in particular. However, to date, no publication presents this association between melioidosis and SCD. The authors describe here 3 clinical cases presenting this very rare association between melioidosis and SCD. These are 3 children with SCD (homozygous SS) residing in Lubumbashi in Haut-Katanga province in the Democratic Republic of Congo. One patient presented with sepsis as a clinical form of the disease. All 3 had presented a pulmonary form. Only one patient was treated specifically after the diagnosis of melioidosis; for the other two, this diagnosis was confirmed after their death. Thus the death rate is 66.67%. This article describes, through these 3 clinical cases, a very rare first association between melioidosis and SCD. This association requires research to establish whether, like Thalassemia, SCD can be considered a risk factor for melioidosis. A screening of cases of melioidosis in the general population should allow us to focus on this.


2018 ◽  
Vol 18 (1) ◽  
Author(s):  
Olivier Mukuku ◽  
Joseph K. Sungu ◽  
Augustin Mulangu Mutombo ◽  
Paul Makan Mawaw ◽  
Michel Ntetani Aloni ◽  
...  

Hematology ◽  
2020 ◽  
Vol 25 (1) ◽  
pp. 91-94 ◽  
Author(s):  
Paul Kambale-Kombi ◽  
Roland Marini Djang’eing’a ◽  
Jean-Pierre Alworong’a Opara ◽  
Serge Tonen-Wolyec ◽  
Charles Kayembe Tshilumba ◽  
...  

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