On World Sickle Cell Day, Firstpost interviewed Dr Praveen Gupta, Principal Director & Chief of Neurology at Fortis Hospital, to discuss the neurological challenges faced by individuals with sickle cell disease (SCD). Dr. Gupta provided valuable insights into the primary neurological complications, neurodevelopmental challenges in children, and the importance of early intervention and support systems.
Primary Neurological Complications
Dr. Gupta explained that the primary neurological complications in sickle cell disease include silent cerebral infarctions, ischemic or hemorrhagic stroke, moyamoya syndrome, posterior reversible encephalopathy syndrome, cerebral fat embolism, and cerebral venous sinus thrombosis. These complications can manifest as seizures, headaches, cognitive impairment, walking difficulty, and balance impairment.
Neurodevelopmental Challenges in Children
Children with sickle cell disease often face significant neurodevelopmental challenges. Dr. Gupta highlighted common issues such as cognitive impairment, difficulties with attention, memory, and learning, imbalance while walking, recurrent headaches, seizures, and decreased coordination between mind and body.
Early Intervention Strategies
To address cognitive impairment in SCD, Dr. Gupta emphasized the effectiveness of educational interventions, cognitive training, occupational therapy, and physical therapy. These interventions aim to overcome deficits caused by silent brain infarcts, supporting growth, learning, attention, and memory in children with SCD.
Effectiveness of Educational Interventions
Dr. Gupta explained that children with SCD may experience silent complications due to occlusive and arterioocclusive disease in the brain, leading to brain parenchyma damage and multiple deficits. He stressed the importance of recurrent neurological assessments and surveillance for early recognition of these deficits. Through cognitive therapy, occupational therapy, and multimodal rehabilitation services, the impact of brain parenchymal damage in these children can be significantly reduced.
Future Directions for Early Intervention
Dr. Gupta highlighted the need for early recognition through surveillance as a key future direction. He emphasized that if SCD can be diagnosed before the onset of neurological and cognitive complications and bone marrow transplants become more accessible, available, and affordable on a large scale, the impact of SCD in children could be mitigated, allowing them to lead reasonably healthy lives.
Relationship Between Stroke and SCD
Dr. Gupta explained that stroke in SCD is caused by the obstruction of blood supply to the brain. Due to the sickling of cells in the brain, these cells can clot together and occlude brain vessels, reducing blood supply to parts of the brain. This can result in manifest strokes, such as ischemic or hemorrhagic strokes, or lead to small silent cerebral infarctions that damage the brain.
Role of Family and Community Support
Dr. Gupta emphasized the crucial role of family and social support in the cognitive rehabilitation of children with SCD. He suggested organizing community services and schooling that incorporate multimodal rehabilitation programs, including cognitive therapy, physiotherapy, and occupational therapy, to significantly improve these children’s learning and skill-building, thereby enhancing their quality of life.
