1 - Serviço Neurologia Pediátrica, Hospital Dona Estefânia, Centro Hospitalar Lisboa Central EPE, Lisboa;
2 - Unidade Neurossonologia, Hospital S José, Centro Hospitalar Lisboa Central EPE, Lisboa;
3 - Unidade Hematologia, Hospital Dona Estefânia, Centro Hospitalar Lisboa Central EPE, Lisboa
- 13th International Child Neurology Congress, Iguazu Brasil, 4-9 Maio 2014 (poster)
Sickle cell disease (SCD) is a group of inherited hemoglobin disorders, of which the most common and severe is Sickle Cell anemia. It affects mainly children of African descent in whom it is the major cause of stroke, contributing to significant mortality and morbidity. The STOP Study (published in 2008) changed the standard practice Guidelines introducing the recommendation of systematic transcranial doppler (TCD) monitoring in order to stratify stroke risk and implement regular blood transfusions to prevent stroke. In a recent study, 590 patients were identified in Portugal, 62% are less than 25 years old and 530 (90%) live in the Lisbon area.About 100 children and adolescents with SCD are currently followed in our Institution. In order to improve the neurological care of these patients, a Pediatric Neurovascular Consultation was created in articulation with the Hematology Unit (where these patients are regularly followed) and the Neurosonology Unit. This organizational effort improved the communication between different specialties, increased the number of patients that systematically perform TCD and reduced the number of stroke events. Between 2009-2011, in the Neurosonology Unit, 97 children with SCD performed regular TCD and among them, six had increased stroke risk and only one suffered an ischemic stroke, after sudden interruption of regular blood transfusions when she immigrated to a different country.
After permission, we translated and adapted the Sickle Cell and Stroke Leaflet (from The Stroke Association) that is given to parents and teachers and is a useful resource in educating caregivers regarding Stroke prevention.
Palavras Chave: Doença Células Falciformes, prevenção, doppler