Karnataka has identified 192 cases of sickle cell disease and 2,004 individuals with sickle cell traits in the past year, after screening 56,000 tribal community members across seven districts. This effort is part of the National Sickle Cell Elimination Mission (NSCEM), launched on July 1, 2023, which aims to eradicate sickle cell disease in India by 2047. The program focuses on individuals aged 0 to 40 in Mysuru, Chamarajanagar, Kodagu, Udupi, Chikkamagaluru, Dakshina Kannada, and Uttara Kannada.
Through collaboration between the Centre, State government, and NGOs, Karnataka plans to screen 350,000 people in three years. The Department of Health has begun distributing Hydroxyurea to affected individuals. CTRITH, part of the Institute of Public Health in Bengaluru, supports this initiative. Deputy Director Shakila and Professor Deepa Bhat highlight the importance of multi-agency collaboration and advanced patient registry systems in managing the disease.
What is sickle cell disease?
Every year June 19 is celebrated as Sickle cell anemia awairness day. Sickle cell anemia is a form of the inherited blood disorder, sickle cell disease. Sickle cell anemia changes your red blood cells’ shape, turning round flexible discs into stiff and sticky sickle cells that block blood flow. Thanks to early detection and new treatments, about half of all people who have sickle cell anemia live into their 50s.
Neurological Complications in SCD
There are several primary neurological complications associated with SCD, including silent cerebral infarctions, ischemic or hemorrhagic stroke, moyamoya syndrome, posterior reversible encephalopathy syndrome, cerebral fat embolism, and cerebral venous sinus thrombosis. These conditions can result in seizures, headaches, cognitive impairment, walking difficulties, and balance issues.
Neurodevelopmental Challenges in Children
Children with SCD often face significant neurodevelopmental challenges such as cognitive impairment, attention deficits, memory and learning difficulties, walking imbalance, recurrent headaches, seizures, and decreased coordination.
Early Intervention Strategies
To support neurodevelopment in children with SCD, experts recommend early intervention strategies such as educational interventions, cognitive training, occupational therapy, and physical therapy. These approaches can help mitigate the deficits caused by silent brain infarcts, supporting children's growth, learning, attention, and memory.
Effectiveness of Educational Interventions
Experts say that the effectiveness of educational interventions in mitigating cognitive deficits in children with SCD. Regular neurological assessments and surveillance are essential for early recognition of these deficits. Cognitive therapy, occupational therapy, and multimodal rehabilitation services can significantly reduce the impact of brain parenchymal damage in these children.
Future Directions
Early recognition through surveillance and the widespread accessibility of bone marrow transplants. Early diagnosis before the onset of neurological and cognitive complications, coupled with accessible bone marrow transplants, could substantially improve the lives of children with SCD.
Relationship Between Stroke and SCD
The stroke in SCD results from the obstruction of blood supply to the brain due to sickled cells clumping together and occluding brain vessels. This can lead to ischemic or hemorrhagic strokes or silent cerebral infarctions, causing brain damage.
Role of Family and Community Support
There’s a crucial role of family and community support in mitigating the cognitive impact of SCD. Organizing community services and schools that incorporate multimodal rehabilitation programs, including cognitive therapy, physiotherapy, and occupational therapy, can significantly enhance the learning and skill-building of children with SCD, thereby improving their quality of life.