Characterization of Medical Conditions of Children with Sickle Cell Disease in the United States: Findings from the 2007-2018 National Health Interview Surveys (NHIS)
Authors: Gyamfi Joyce , Tampubolon Siphra , Lee Justin Tyler , Islam Farha , Ojo Temitope , Opeyemi Jumoke , Qiao Yuki , Mai Andi , Vieira Dorice , Peprah Emmanuel 
Published: November 2021

Abstract
Background: Sickle cell disease (SCD), one of the most common genetic disorders in the United States (US), affects 70,000 to 100,000 children. The burden is greatest among the African American population, where SCD impacts 1 in 365 live births. Children with SCD have been observed to have lower Health-Related Quality of life (HRQoL) due to the impact of severe disease-related complications and comorbidities, including infections, pain crises, acute chest syndrome, metabolic alterations and overt stroke. SCD and its related comorbidity and complications result in increased health care utilization, particularly emergency department use- which accounts for 80% of medical expenditure. Further, studies using US population-based data to characterize the health status and health care utilization for SCD children is lacking. With the exception of a study conducted by Boulet et al. in 2010, which examined the 1997-2005 National Health Interview Surveys (NHIS) data to describe health status and health services use among Black children aged 0-17 years with SCD. To fill this important gap, we used the 2007-2018 NHIS data to describe health status and health services use among SCD children aged 0-17 years and conducted a sub-analysis for Black children to better characterize SCD- related medical conditions affecting this population and health care utilization patterns.
Methods: The study included an unweighted sample of 133,542 children, with 68,745 male children and 64,797 female children. Questionnaires were answered by a parent/guardian on behalf of the child. We examined (1) the prevalence of medical comorbidities; (2) indicators of functional status and perceived health status; (3) use of healthcare services; (4) barriers to accessing healthcare services; and (5) association of the comorbidities with the utilization of care. We hypothesized that Black children with SCD would have higher levels of disease-related complications and comorbidities, as well as healthcare utilization than their counterparts without SCD. We used logistic regression to compare the associations between 'SCD' and 'No SCD' on various medical conditions for all races and for Black children only groups.
Results: Of the 133,542 children, 215 have SCD (.16%). Among the SCD children, 51.1% are male, Black: 170 (79.1%), White: 16 (7.44%), American Indian: 1(.47%), Asian 2 (.93%): other races: 21 (9.77%) and Multiple Races 5 (2.33%). Black children had a higher prevalence of SCD than other races and Black children with SCD were more likely to have a household income &lt; 1% of the federal poverty levels compared to those without. After applying the appropriate weights to each of the demographics, children of all races with SCD had higher adjusted odds (Adjusted Odds Ratio -AOR) of having anemia (AOR 37.6; CI: 22.3, 63.3: p=0.000), respiratory allergies (AOR 2.2; CI: 1.3, 3.8; p=0.005), food/digestive allergies (AOR 2.1; CI: 1.0, 4.1: p=0.042), eczema/skin allergies (AOR 2.1; CI: 1.3, 3.6: p=0.004), and frequent severe headaches/migraines (AOR 2.2; CI: 1.1, 4.3; p=0.024). Children with SCD are also more likely to experience frequent diarrhea/colitis, although statistically non-significant. Among Black children, those with SCD had higher odds of having the following medical conditions including anemia (AOR 18.7; CI: 10.7,32.3: p=0.000), and respiratory allergies (AOR 2.4; CI: 1.3, 4.4: p=0.004) and also more likely to experience other conditions including food/digestive allergies, eczema/skin allergies, and frequent severe headaches/migraines, although statistically non-significant (p&gt;0.5). Children of all races (26.0%) and Black children (28.8%) with SCD had at least 2 or more annual emergency room visits (p=0.000).
Conclusion: Our study findings indicate that children with SCD for all races experienced significant comorbid conditions associated with disease status. Moreover, among Black children, those with SCD have increased SCD related comorbidity, worse health status, and an increased number of visits to the emergency room compared to children without SCD. This creates the urgency to address the health burden for Black children with SCD, by identifying 'beneficial' health care utilization services (non-emergency services) that improves the quality of life for SCD children. Future research should address residual gaps in information about health burden and healthcare utilization among SCD population in the US.

Disclosures
No relevant conflicts of interest to declare.