Setting a New Standard for Neurocognitive Sickle Cell Care

Patients with sickle cell disease can experience developmental, learning and cognitive challenges throughout their lives, but guidance for identifying and addressing those concerns hasn’t always been consistent. New national recommendations aim to change that.

Children’s Hospital Colorado pediatric psychologist Alyssa Schlenz, PhD, is leading the creation of new national standards and recommendations alongside a team of experts through the National Alliance of Sickle Cell Centers. The guidance provides a more detailed roadmap for identifying and addressing developmental, learning and cognitive challenges from infancy through adulthood.

The new guidelines, in part, grew from a need for greater consistency in sickle cell disease care. Current guidance often recommends further testing only after a specific concern is identified or following a major neurological event, such as a stroke. However, people with sickle cell disease can experience developmental and cognitive challenges without those complications. Further, previous guidance also neglected proper screening and comprehensive evaluation. The new standards better reflect current evidence and provide clearer guidance for hematologists, psychologists and other specialists who are developing neurodevelopmental programs and diagnosing patients.

“If we were going to take on this topic, we really wanted to take it on and have it be something that was much clearer in terms of our roadmap,” Dr. Schlenz says.

The recommendations build on previous guidance from the American Society of Hematology while expanding the focus across three levels of care: surveillance, screening and evaluation. They emphasize educating families about developmental risks, screening at key stages and clearly defining when a comprehensive neuropsychological evaluation may be needed.

The guidance also looks beyond childhood. For example, it recommends that patients have access to at least one evaluation before leaving pediatric care, helping them enter the next phase of life with information that can guide future support in an adult healthcare setting.

Building neurocognitive care into sickle cell treatment

At Children’s Colorado, this approach is already part of caring for the whole patient. Since 2019, the Hematology Program within the Center for Cancer and Blood Disorders has incorporated neurodevelopmental monitoring into routine sickle cell care through programming led by Dr. Schlenz and neuropsychologist Arianna Martin, PsyD.

"What makes our program unique is the continuity and depth of care we're able to provide from infancy through adulthood," says Christopher McKinney, MD, Director of the Pediatric Sickle Cell and Hemoglobinopathy Program. "We not only support patients medically but also prepare them to successfully transition out of the hospital and manage their care independently, no matter where life takes them."

Through this multidisciplinary model, hematologists work alongside psychologists and other specialists to identify concerns early and connect patients with assessments, educational support and additional resources. That experience not only helped inform the national recommendations but has also allowed Dr. Schlenz and her colleagues to consult with centers working to build similar programs.

“Our families deserve this level of care,” Dr. Schlenz says. “They deserve access to someone who specializes in neurodevelopmental disorders in the same way that other populations do who are at risk for neurologic complications.”

The guidance comes as research continues to expand treatment possibilities for sickle cell disease, including approved gene therapies and CRISPR gene-editing research. As treatment advances, Dr. Schlenz and her colleagues remain focused on ensuring patients receive the developmental and cognitive support they need to learn, build independence and thrive throughout life.