Anyssa Young

Sickle cell disease (SCD) and iron deficiency (ID) cause anemia and cognitive deficits. The frequency of ID among youth with SCD (Y‐SCD) remains unclear because screening has not been standard. Using clinically obtained ferritin results, we identified that ≥9% of Y‐SCD at our center may have ID. Those with ID were often >5 years old, premenarche, and male and had a mean corpuscular volume that was, on average, 7.3 fL lower than iron‐replete Y‐SCD ( p < 0.001). These findings suggest ID screening is indicated among Y‐SCD. Studies are needed to determine mechanisms and whether normalizing iron stores improves outcomes.

Respiratory syncytial virus (RSV) imposes substantial risk to all infants, but its impact on youth with SCD (Y‐SCD) is underexplored. With newer, highly effective RSV monoclonal antibodies (RSV‐mAb), research is needed to inform their use in Y‐SCD. This single‐center study of Y‐SCD aged 0–21 years identified 667 hospitalizations that included RSV testing. Of these, 5% were positive for RSV, and 68% occurred in Y‐SCD beyond their first RSV season. Intensive care, positive pressure ventilation, and transfusion were required in 21%, 32%, and 21% of hospitalizations, respectively. Findings indicate that multicenter studies are warranted to determine if RSV‐mAbs improve outcomes in Y‐SCD.

Prescription and claims data indicate that hydroxyurea (HU) adherence is low among youth with sickle cell disease (SCD). Electronic monitors (EM) are a gold standard measure, yet existing models have significant drawbacks that limit their use in youth with SCD. We developed a small, cellular-connected EM that is compatible with both liquid and capsule HU formulation bottles. In testing this EM among a sample of adolescents and young adults and caregivers of young children with SCD, we found that the EM is feasible and acceptable among youth with SCD and caregivers of young children with SCD in the short-term. We also found that the EM are useful for determining HU adherence and gained user insights that may improve their use longer-term. Future studies should examine the EM ability to determine which youth with SCD could most benefit from HU adherence intervention and treatment escalation in those with optimized adherence.

Suicidal ideation (SI) and suicidal attempt (SA) rates are rising among youth in the United States; however, research on SI/SA in youth with sickle cell disease (Y‐SCD) remains limited. Using the Pediatric Health Information System, we examined the prevalence and demographic factors associated with SI/SA among Y‐SCD (10–18 years) seeking acute care between 2015 and 2024. Overall, 358 (3.08%) Y‐SCD had ≥ 1 SI/SA encounter and 0.76% of all acute encounters among Y‐SCD included a code for SI/SA. Female sex ( p < 0.0001) and public insurance ( p = 0.0009) were significantly associated with SI/SA. Findings highlight the need for suicide screening in acute care settings for Y‐SCD.

Summary: Sickle cell disease (SCD) and its treatments may lead to gonadal dysfunction. Limited research has examined how these effects translate into actual fertility outcomes and family‐building perspectives. We aimed to determine the frequency of infertility and to examine family‐building goals, knowledge and concerns among 91 adults with SCD from The Ohio State University Wexner Medical Center SCD clinic. Participants completed surveys capturing their demographic and medical information. Fertility status and family‐building perspectives were measured using modified versions of surveys used in fertility‐related literature in other populations. Descriptive statistics summarized demographics, frequency of infertility and family‐building perspectives. Most participants expressed a desire for children. Approximately half met the clinical definition of infertility at some point, but only a few who met this definition had knowledge of it. Lastly, most reported low to moderate fertility and reproductive concerns potentially due to lack of awareness about their infertility status. These findings underscore the need to increase infertility education and counselling for individuals with SCD.

It is unclear if adolescents with sickle cell disease (SCD) are screened for depression, since primary care provider (PCP) visits decline with age and socioeconomic disadvantage may impact receipt of care. This 1‐year study identified 97% of adolescents with SCD at Nationwide Children's Hospital (NCH) had a PCP. Among those with an NCH PCP (n = 55), 40% saw their PCP and 33% were screened for depression. Socioeconomic disadvantage was not associated with receipt of depression screening (p = 0.55) or PCP care (p = 0.22). While adolescents with SCD often have PCPs, interventions to increase PCP engagement may improve depression identification in this high‐risk population.