Characteristics and growth and pubertal patterns among patients with sickle cell disease at Kenyatta national hospital, Nairobi

Abstract

Background: Sickle cell disease (SCD) is an autosomal recessive disorder characterized by production of abnormal (sickle) hemoglobin, resulting in anemia, susceptibility to pneumococcal and other infections and multiple organ failure. The aim of this study was to describe the characteristics as well as growth and pubertal patterns among children with sickle cell disease in the Kenyan setting.
Methods: This was a cross-sectional descriptive study involving children with confirmed sickle cell disease seen at the Kenyatta National Hospital. Demographic information was obtained using a standard questionnaire and information on prescribed drugs was extracted from medical records. Anthropometric measurements were obtained by measuring weight and height and puberty status was assessed in boys and girls
Results: A total of 142 children with sickle cell disease were recruited into the study of whom 86 (60.6%) were males and 56 (39.4%) were females. Their median age was 7.7 years (IQR 5.5, 9.5). Two thirds of the study population (65%) was from the Luo ethnic community With regards to treatment, 86% of the children were on Hydroxyurea, 70% were on Folic acid and only a small proportion were on Paludrine (3.5%) and Penicillin V (2.9%). Among children aged below 5 years 17.1% were underweight (WAZ score <-2SD). Only 9 (27.2%) boys aged > 9 years had testicular size ≥3cc while 4 (17.4%) girls aged >8 years had breast tanner 2.
Conclusion: There was low uptake of the recommended antibiotic and antimalarial prophylaxis in our study population. Growth failure and delayed puberty was also observed.
Key Words: Sickle cell disease, Hydroxyurea, Folic acid, Penicillin V, antimalarial, Growth and Puberty