Assessing Major Organ Dysfunction in Children With Sickle Cell Disease: Protocol for a Cross-Sectional Study.

Background and Aims: Sickle cell disease (SCD) is the most common clinically significant genetic disorder affecting about 300,000 newborns globally each year. In Ghana, a prevalence of 1.8% exists among newborns. With increasing life expectancy, assessment of the effect of progressive organ damage on quality of life becomes essential. Organs involved could be the lungs, heart, brain, kidney, and bones. This study aims to describe major organ dysfunction and its associated factors in children aged 6-16 years with SCD in the Kumasi Centre for Sickle Cell Disease (KC-SCD) patient cohort in Kumasi, Ghana. Methods: It will be a cross-sectional study conducted over a 1-year 8-month period at the pediatric sickle cell clinic of the Komfo Anokye Teaching Hospital (KATH). All patients, except those coming for acute care or emergency visits, will be eligible for enrollment. Data on sociodemographic details, medical history, and previous complications, anthropometry, and pubertal stage will be collected. Full blood count, reticulocyte counts, lactate dehydrogenase counts, lung function tests, electrocardiogram, echocardiogram, mental health status assessment and pubertal staging will be conducted to assess end organ dysfunctions. Data will be entered into a REDCap database, cleaned, and exported to the STATA statistical software for analysis. The prevalence of specific end-organ dysfunction will be determined based on predefined criteria. Subsequently, the relationship between the various end-organ dysfunctions and clinical and laboratory covariates will be determined using inferential analysis. Conclusion: These results will help to know the burden of end organ damage in our pediatric population and subsequently feed into the recommendations and hospital policies for screening for end organ dysfunction.