Abstract
Background: Sickle cell disease (SCD) is a heterogenous group of inherited haemoglobin disorder characterized by inheritance of β s gene and another β s gene (homozygous sickle cell anaemia -SCA) or haemoglobin S gene (HbSC), haemoglobin D or haemoglobin E gene.. SCA is the commonest type accounting for about 70% of SCD worldwide. Nigeria has the highest burden worldwide with an estimated 150,000 SCD births annually out of the estimated 275,000 annual global births. SCD patients presents with acute painful, haemolytic and sequestration crises, acute chest syndrome, and stroke.
Aims/Objectives: To describe the pattern of presentation and treatment modalities offered SCD patients in the setting of a University health centre.
Materials and method: A descriptive cross sectional study was conducted at the University of Ibadan Health Centre among SCD patients who presented over a five-year period. Data was extracted from the case folders and analyzed using SPSS version 23.
Results: Seventy-eight SCD patients were documented to have utilized the health centre in the period under review. The median age was 24 years (IQR: 16, 52). The male to female ratio was 1.3: 1. Homozygous HbSS constituted 64(82%) while 14(18%) were HbSC. All the patients had presented to the clinic on account of bone pain crisis and a few 12(15%) presented with symptoms of haemolytic crises. The commonest used analgesics were NSAIDS 54(74%), dihydrocodeine 43(54%)) and pentacozine 43 (54%).
Conclusion: Sickle cell disease is a life- long disease where cure is not readily attained hence the recommendation of early intervention of expert to ensure proper management of acute complications of the disease. There is a need to continue to train General practitioners on current management principles and guidelines in care of SCD patients.
Keywords: Sickle cell disease, presentation pattern, analgesic usage, University Health Centre, Ibadan.
Résumé
Contexte: La drépanocytose (SCD) est un groupe hétérogène de trouble héréditaire de l’hémoglobine caractérisé par l’hérédité du gène âs et d’un autre gène âs (drépanocytose homozygote -SCA) ou du gène de l’hémoglobine C (HbSC), de l’hémoglobine D ou du gène de l’hémoglobine E. Le SCA est le type le plus courant représentant environ 70% des SCD dans le monde. Le Nigéria a le fardeau le plus élevé au monde avec environ 150 000 naissances de drépanocytose par an sur les 275 000 naissances annuelles dans le monde. Les patients SCD présentent des crises douloureuses aiguës, hémolytiques et de séquestration, un syndrome thoracique aigu et un accident vasculaire cérébral.
Buts / Objectifs: Décrire le modèle de présentations et les modalités de traitement offertes aux patients atteints de drépanocytose dans le cadre d’un centre de santé universitaire.
Matériel et méthode: Une étude transversale descriptive a été menée au centre de santé de l’Université d’Ibadan parmi des patients atteints de drépanocytose qui se sont présentés sur une période de cinq ans. Les données ont été extraites des dossiers de cas et analysées à l’aide de la version 23 de SPSS.
Résultats: Soixante-dix-huit patients atteints de drépanocytose ont été documentés comme ayant utilisé la clinique du centre de santé au cours de la période considérée. L’âge médian était de 24 ans (IQR: 16, 52). Le ratio homme / femme était de 1,3: 1. L’HbSS homozygote constituait 64 (82%) tandis que 14 (18%) étaient l’HbSC. Tous les patients s’étaient présentés à la clinique pour crise de douleur osseuse et quelques 12 (15%) présentaient des symptômes de crise hémolytique. Les analgésiques les plus couramment utilisés étaient les AINS 54 (74%), la dihydrocodéine 43 (54%)) et la pentacozine 43 (54%).
Conclusion: La drépanocytose est une maladie à vie où la guérison n’est pas facilement obtenue, d’où la recommandation d’une intervention précoce d’un expert pour assurer une prise en charge adéquate des complications aiguës de la maladie. Il est nécessaire de continuer à former les médecins généralistes sur les principes de prise en charge actuels et les lignes directrices en matière de soins des patients atteints de drépanocytose.
Mots clés: drépanocytose, schéma de présentation, usage analgésique, centre de santé universitaire, Ibadan.
Corespondence: Dr S. P. Ogundeji, Department of Haematology, College of Medicine, University of Ibadan, Ibadan Nigeria. E-mail: peterogundeji22@gmail.com
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