Key takeaways:

• Sickle cell disease is associated with deficits in processing speed, executive function and reasoning.
• Previous standards lacked consistency.
• Serial assessments can help catch cognitive concerns earlier.

Serial assessments for neurodevelopmental disorders should take place earlier and more often for people with sickle cell disease, according to new standards and recommendations released by the National Alliance of Sickle Cell Centers.

The new guidelines are needed in part because current national guidelines do not offer enough clarity for how SCD centers should best identify and navigate cognitive risks, said Alyssa Schlenz, PhD, associate professor of pediatrics with Children’s Hospital Colorado.

Data derived from Schlenz A, et al. Pediatr Blood Cancer. 2026:doi:10.1002/1545-5017.70569.

“If you’re a patient with sickle cell disease and you receive care in one state, that care can look very different from the care you might receive if you move to another state,” Schlenz told Healio. “We’re trying to solve that problem, and this is a problem that definitely exists specific to neurodevelopmental, neuropsychological care.”

SCD is a blood disorder that is also associated with neurocognitive deficits in processing speed, executive function and reasoning. The new, data-based standards seek to build on standards released in 2020 by the American Society of Hematology that focused on managing cerebrovascular risk and common comorbidities in SCD.

Standards specifically address the process of surveilling, screening and evaluating for development and cognition across a person’s lifespan.

“One of the main harms that we’re trying to avoid is late identification and support of individuals who have neuronal cognitive impairment,” Schlenz said. “We have really good data suggesting that these difficulties can emerge as early as infancy and that risk increases with age.”

The big picture

The NASCC workgroup included 10 psychologists, two physicians and one occupational therapist. Team members collectively represented the fields of pediatric and lifespan neuropsychology, pediatric psychology, developmental pediatrics/neurodevelopmental disabilities, hematology and occupational therapy.

Although neurological risk is more strongly associated with HbSS and HbS-beta zero genotypes, the new guidelines apply to people with all forms of SCD, the authors wrote.

Schlenz and colleagues said their standards and recommendations were based around overarching principles, which included:

• promoting education;
• embedding surveillance,
• screening and evaluation into all SCD specialty care;
• clinicians only engaging in standardized assessments within their scope of training;
• looking beyond test results to also incorporate family member interviews and medical history into the decision-making process; and
• identifying development and cognition concerns as early as possible.

Identifying concerns early can be critical when it comes to understanding support needs and adjusting self-management expectations, Schlenz said. For SCD, consistent surveillance and screening in early childhood will identify about one in three children who have an associated neurodevelopmental disorder by the age of 5, she said.

For standards related to surveillance of those in early childhood, authors called for simplified signaling questions about developmental delays. When a child reaches school age, standards also begin to entail asking about academic or behavioral problems or symptoms of inattention, hyperactivity or impulsivity.

Surveillance recommendations build on standards by also calling for the signaling questions to be asked annually starting at 9 months of age. Recommendations also include having resources available both inside and outside the clinic for addressing any concerns that come up.

Annual assessments serve several purposes, according to Arianna Martin, PsyD, a pediatric neuropsychologist with Children’s Hospital Colorado. They can help catch changing needs and new medical complications that can develop and affect thinking, learning and attention, she said.

“If we were only to look at one time point, we’re only getting a snapshot of the patient’s functioning, and we may miss some of these factors,” Martin told Healio. “By having these serial assessments, we can track development over time. We can detect subtle changes that may have otherwise gone unnoticed, and then we can urge concerns as early as possible.”

Screening standards include screenings for children aged 9, 18 and 30 months, along with an autism screening at 18 and 24 months or when development concerns are raised. At school age and into adulthood, standards include cognitive screenings when warranted by concerns identified during surveillance, authors wrote.

Recommendations at the screening level include social-emotional screening for children aged 9, 18, 24 or 30 months and annually at ages 3 and 4 years, which should take place within a sickle cell clinic. Cognitive screening for school-age youth is also recommended approximately every 5 years beginning at the start of elementary school.

The process should include interpreting results for family members and proving them with resources for intervention, authors wrote.

“Right now, our goal is to give patients the best quality of life possible and the most options for that life possible, and the best way we can do that is through this timely intervention support,” Schlenz told Healio.

For evaluation, the authors wrote that standards should include developmental or neuropsychological evaluation in cases where suspicion for neurodevelopmental disorder is high. They recommended a neuropsychological evaluation at least once for each person prior to transitioning to adult care.

Schlenz and colleagues emphasized the importance of catering services to the individual level based on needs and access to resources. The guidelines needed to be flexible to account for the reality that some communities may not have resources to meet all needs, she said.

“It’s really important to understand their cognitive and developmental functioning within the context of their everyday lives,” Schlenz said.

The workgroup also stressed the importance of collaboration across disciplines, including primary care providers, SCD specialty teams and other experts that may be needed, such as psychologists and occupational therapists. Although collaboration is important for the treatment of many diseases, SCD can affect nearly every system in the body, Martin said.

Psychologists play a particularly important role when it comes to evaluation, Martin said.

“We also provide an immense amount of support, and we can have the time to really address any cultural factors that may be affecting patient and family, and address any barriers to medical appointments or screening,” Martin told Healio. “I think that having us as part of the team, we can really help support overall medical adherence and just overall quality of life.”

For more information:

Alyssa Schlenz, PhD, and Arianna Martin, PsyD, can be reached at neurology@healio.com.