Screening tool identifies developmental risks in young children with SCD: Study
Early detection can be helpful in mitigating academic difficulties, researchers say
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A neurodevelopmental screening tool may identify toddlers and preschool children with sickle cell disease (SCD) who are at risk for long-term academic difficulties, a study shows.
The tool, called the Ages and Stages Questionnaire (ASQ), is based on parent-reported acquisition of developmental milestones across fine and gross motor skills, communication, problem-solving, and personal-social skills. In preschool children, the tool may also predict later risk for stroke.
These findings highlight “the utility of developmental screening for the early detection of persistent developmental difficulties,” researchers wrote. “Early detection has important implications for accessing intervention before elementary school that can mitigate academic difficulties.”
The study, “Academic and cerebrovascular outcomes after neurodevelopmental screening in sickle cell disease: A longitudinal cohort study,” was published in Developmental Medicine & Child Neurology by a team of U.S. researchers.
Children with SCD may experience growth and developmental delays
SCD is caused by genetic mutations that result in the production of a faulty version of hemoglobin, the protein that carries oxygen in red blood cells, leading to cells acquiring a sickle-like shape. Faulty cells tend to get stuck in blood vessels, blocking blood flow and hastening their destruction, which can lead to a shortage of red blood cells.
Children with SCD may experience growth and developmental delays, caused by a shortage of oxygen and nutrient supply to tissues that can be the result of inflammation and events like stroke.
However, “long-term implications of developmental delay among young children with SCD are not well understood,” the researchers wrote.
In a previous developmental screening study, the team of researchers found that toddlers and early school-age children with SCD and developmental delays had lower parent-reported academic performance than those without developmental delays.
However, only the older children with developmental delay also had higher rates of academic attainment issues (such as failing a grade) and a greater need for developmental support services.
Now, the same team wanted to assess the long-term validity of ASQ, a widely used developmental screening tool, in predicting subsequent academic outcomes and cerebrovascular disease in children with SCD.
More preschool children had developmental issues in fine motor skills
A total of 111 toddlers (mean age 2.5 years) and 110 preschool children (mean age 4.4 years) with SCD were included in the study. Most were boys (59.5% of toddlers and 63.6% of children) and had the HbSS genetic profile (60% or more), which typically results in more severe disease.
None had known cardiovascular disease or an abnormal transcranial Doppler ultrasound (TCD) exam at the time of screening. TCD is a noninvasive ultrasound test that measures blood flow in the arteries of the brain and can help to identify children with SCD who have an increased risk for stroke.
All children underwent at least three TCD examinations over the 4- to 6-year follow-up period to assess stroke risk.
Developmental issues were defined as a positive ASQ screening, when one or more domain-level scores fall below the mean for the age group by a specific margin. Results showed that, for each of the five ASQ domains, about 10% of toddlers and 3% to 23% of preschool children had developmental issues.
A significantly higher proportion of preschool children had developmental issues in fine motor skills than toddlers (22.7% vs. 10.8%), while issues with personal social skills were significantly more frequent among toddlers (2.7% vs. 12.6%).
There were no significant differences between toddlers and children in rates of problems in communication (9.9% vs. 14.5%), gross motor skills (8.1% vs. 7.3%), and problem-solving (11.7% vs. 9.1%).
Toddlers with positive ASQ screenings were significantly more likely to have more academic problems than those with negative scores, while preschool children with positive screenings were significantly more likely to have both academic problems and increased stroke risk.
Daycare participation linked to fewer academic issues for toddlers
The study also collected data on social and environmental risk factors, including neighborhood-level socioeconomic status, attendance at a formal preschool or daycare program, and insurance status.
For toddlers, daycare program participation and a negative ASQ screening were significantly associated with fewer academic problems, and day care program participation was also linked to a negative ASQ score.
In preschool children, private health insurance, day care participation, negative ASQ screening, and fewer previous hospitalizations were significantly associated with fewer academic problems. Day care participation and prior hospitalization were also correlated with ASQ scores.
This study demonstrated that the ASQ is a developmental screening tool that can identify young children with SCD at risk for adverse long-term neurodevelopmental outcomes.
Further statistical analysis demonstrated that positive ASQ screening was the only independent predictor of long-term academic problems in both groups.
A later increased stroke risk was significantly associated with genotype severity and levels of hemoglobin and immature red blood cells in toddlers, and with levels of hemoglobin and previous hospitalization in preschool children. However, ASQ results were not associated with these variables.
“This study demonstrated that the ASQ is a developmental screening tool that can identify young children with SCD at risk for adverse long-term neurodevelopmental outcomes,” the researchers wrote. “Neurodevelopmental screening in SCD beyond the universally recommended 24- to 30-month age may provide more accurate risk stratification for school-age outcomes.”
