Prevalence and factors associated with non-screening of siblings of children attending the Sickle Cell Clinic of Mulago National Referral Hospital

Abstract

Background: Sickle cell disease (SCD) is the most common inherited hemoglobinopathy caused by an abnormal Haemoglobin S and is characterized by recurrent pain episodes and chronic anemia, progressive organ damage, and poor quality of life. Early diagnosis with early institution of treatment is key in mitigating SCD-associated morbidity and mortality. In Uganda, neonatal screening for SCD was rolled out in 2014, in the high burden districts; however, no universal newborn screening program exists largely due to cost implications. As such, most children are diagnosed later in life, usually after complications have already manifested. Screening of siblings of known sickle cell patients has the potential to bridge the gap in the diagnosis of SCD in countries where newborn screening is not universal. Study objective: The objective of this study was to determine the prevalence and factors associated with the non-screening of siblings of sickle cell disease patients attending SCC of Mulago NRH. Methods: A total of 380 caretakers of children with SCD attending the clinic, were interviewed in a descriptive cross-sectional study at the Mulago Sickle Cell clinic. Enrollment was done by systematic sampling and a structured interviewer-administered questionnaire was used to collect data from the caretakers. Kobo collect was used for data collection and entry and was exported to strata 17.0 for analysis. The proportion of siblings of SCD patients attending the Mulago Sickle cell clinic, who are tested for SCD status was reported. Multivariate Poisson regression was used to identify the factors associated with non-screening of siblings of SCD patients. Factors with a p-value <0.05 were regarded as significant. Results: The prevalence of non-screening of siblings of SCD patients was 72.1%. The primary sickle cell disease patient factors like being the firstborn, age above 5 years at the time of diagnosis, the parent having initiated that patient’s screening process at diagnosis, the patient having displayed SCD symptoms by the time of diagnosis, and the caretaker being aware of their own SCD status were associated with non-screening of the siblings. Conclusion: Sibling non-screening prevalence is high; there is a need for more proactiveness by clinicians to initiate SCD screening, Health education should be given to all people, sibling screening should be thought about as an alternative SCD screening measure, and a qualitative study is recommended to better understand the associated factors.