New US guidelines target developmental, cognitive issues in SCD
Teams of experts stresses importance of proactive assessments
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A team of experts from across the U.S. has created a new set of national guidelines on how best to provide cognitive and developmental care to people with sickle cell disease (SCD).
The guidelines stress the importance of proactive assessments so that cognitive or developmental issues can be detected as soon as possible, allowing patients and their families to access needed support. The guidelines also stress the importance of clinical collaboration to deliver optimal care.
The guidelines were described in the study “Consensus Standards and Recommendations for Developmental and Cognitive Surveillance, Screening, and Evaluation in Sickle Cell Disease: Executive Summary From the National Alliance of Sickle Cell Centers Neurocognitive Workgroup,” which was published in Pediatric Blood & Cancer.
The work was led by the National Alliance of Sickle Cell Centers (NASCC), a group dedicated to improving outcomes for people with this disease.
“Our work is not only about establishing national standards for SCD care but also elevating the quality of that care across the board,” said Alyssa Schlenz, PhD, the study’s first author, a pediatric psychologist at Children’s Hospital Colorado, and chair of the NASCC neurocognitive workgroup, said in a press release from the hospital. “We have a responsibility to examine every aspect of this disease and ensure each is fully and thoughtfully addressed, regardless of where patients receive care.”
Guidelines feature a three-tiered structure
SCD is a genetic disorder in which red blood cells become deformed into a sickle shape, which can make them prone to getting stuck in blood vessels and disrupting blood flow. Problems with blood flow in the brain can lead to a range of problems, including severe health issues such as strokes.
However, it’s increasingly recognized that people with SCD may experience developmental and cognitive challenges even if they’ve never experienced major neurological health crises.
These new guidelines were developed to help standardize best practices for developmental and neurological care in SCD across the disease spectrum. The guidelines use a three-tiered structure to check for cognitive and developmental problems in people with SCD.
Surveillance. screening, in-depth evaluation
The first tier is called surveillance — essentially, this means that doctors should be proactively asking SCD patients and their families about potential cognitive and developmental challenges throughout the course of care. Ideally, doctors should also use these conversations to provide education to families about the potential issues that can arise in people with SCD.
The second tier is screening, where brief standardized tests are used to look for potential cognitive and developmental deficits. The guidelines suggest that screening should be done any time there are concerns raised during routine surveillance.
Researchers also recommend some screening for all children with SCD in early life — specifically, screening for developmental problems should be done at ages 9 months, 18 months, and 30 months, and screening for signs of autism should be done at 18 months and 24 months.
If surveillance and screening point to potential issues, the third tier, in-depth evaluation, comes into play. This includes a clinical interview and validated measures of developmental, cognitive, emotional, behavioral and/or adaptive functioning. The goal is to pinpoint specific challenges and then direct families toward support that can help them thrive.
Early identification of developmental and cognitive differences is critical. Without it, care teams risk the ability to connect patients with the right supports — from school accommodations to therapies and community resources — so they can succeed long-term and have the best possible quality of life.
The guidelines also note that comprehensive neuropsychological evaluations should be done on people with SCD who experience major neurological complications such as strokes or seizures.
“Early identification of developmental and cognitive differences is critical,” said Arianna Martin, one of the study’s authors, a pediatric neuropsychologist at Children’s Colorado, and member of the NASCC neurocognitive workgroup. “Without it, care teams risk the ability to connect patients with the right supports — from school accommodations to therapies and community resources — so they can succeed long-term and have the best possible quality of life.”
The guidelines stress that, in order to get the best results, it’s imperative that care for SCD patients should be an interdisciplinary, collaborative process. The researchers noted that some types of neurological evaluations and support interventions require considerable expertise to do correctly, so it’s crucial that doctors caring for people with SCD are willing to work with experts when appropriate.
“Effective implementation of these standards depends on strong partnerships between primary care providers and SCD specialty teams, as well as on coordinated involvement of psychology, neuropsychology, developmental pediatrics, occupational therapy, and other relevant disciplines across the lifespan,” the researchers concluded. “Multidisciplinary collaboration and role-specific expertise are essential for translating these consensus standards into meaningful, equitable, and sustainable clinical care for individuals living with SCD.”
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