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

Background: In the United States , sickle cell disease (SCD), affects about 100,000 individuals, with roughly 2,000 babies born annually with SCD. African Americans are disproportionately affected by SCD. In addition to medical conditions, psychosocial and affective comorbidities are also observed in relation to the frequency and intensity of pain crises. Evidence also suggests robust academic challenges that begin in elementary school and persist until high school. It is estimated that about 25% to 33% of children with SCD experience neurodevelopmental complications. Neurological complications lead to cognitive deficits and educational challenges. African Americans and those with low socioeconomic status are affected the most by neurological complications. Among Children with SCD, data suggest that specialized school service utilization rates are about 37%, and grade retention ranges from 28% to 40%. We used the 2007-2018 NHIS to describe additional neurocognitive impairments and health services used among Black children aged 0-17 years with SCD and place findings into context.

Methods: Data was acquired from the NHIS database. 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 neurological conditions; (2) indicators of functional status and perceived health status; (3) use of healthcare and special educational services; (4) barriers to accessing healthcare services for a national sample of children with SCD; (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 neurological 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, 170 (79.1%) are Black, 16 (7.44%) are White, 1(.47%) are American Indian, 2 (.93%) are Asian, 21 (9.77%) are other races, and 5 (2.33%) are of Multiple Races. Among all races, Black children had a higher prevalence of SCD than other races. Black children with SCD had a higher predominance of girls than did those without SCD. They were also more likely than their counterparts without SCD to have a household income &lt; 1% of the federal poverty levels. After applying the appropriate weights to each of the demographics, Children with SCD had higher odds of having a developmental condition such as: learning disability (Adjusted odds ratio -AOR 1.5; CI: .7, 3.5: p=0.311), intellectual disability (AOR 4.2; CI: 1.3, 13.3: p=0.014), trouble hearing (AOR 2.6; CI: 1.1, 6.1: p=0.026), trouble seeing (AOR 2.3; CI: 1.0, 5.3: p=0.061) and another developmental delay (AOR .8; CI: .1, 6.2: p=0.838). We see similar results in the Black children only sample where Black children with SCD were more likely to have a learning disability (AOR 1.3; CI: .5, 3.2; p=0.590), intellectual disability (AOR 2.4; CI: .6, 10.2: p=0.236), trouble hearing (AOR 1.6; CI: .6, 4.1: p=0.310) or trouble seeing (AOR 2.3; CI: 1.0, 5.4; p=0.048). They also exhibit other developmental delays (AOR 1.9; CI: .2, 22.4: p=0.625), although not statistically significant.

Conclusion: In this study sample, it is clear that children with SCD had higher odds of having a developmental condition and in addition Black children with SCD also have a comparable odd of developing neurological complications compared to children with SCD of other races. A similar pattern was identified that Black children with SCD reported increased utilization of healthcare and special education services in the past 12 months. This creates the urgency to address the health burden for Black children with SCD that has been exaggerated by barriers to accessing comprehensive health care. Studies that further assess Black children's health burden with SCD, its neurocognitive impairment and healthcare utilization is crucial.

Disclosures

No relevant conflicts of interest to declare.