1978).įortunately great strides have occurred over the past 40 years, and better management strategies have altered this previously bleak outlook. Without early identification or specific interventions, many patients with SCA have poor quality of life, and most die as young adults of SCA-related complications ( Diggs 1973 Rogers et al. Hemolytic anemia, acute vaso-occlusive events (VOEs), and chronic end-organ damage begin early in life, and complications accumulate throughout childhood. The natural history of untreated sickle cell anemia (SCA) is well described and documents serious morbidity and early mortality ( Powars 1975 Platt et al. Liberalized use of blood transfusions and early consideration of hydroxyurea treatment represent a new treatment paradigm for SCA management. Treatment approaches that previously highlighted acute vaso-occlusive events are now evolving to the concept of preventive therapy.
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Periodic evaluation by trained specialists helps provide comprehensive care, including transcranial Doppler examinations to identify children at risk for primary stroke, plus assessments for other parenchymal organ damage as patients become teens and adults. Ongoing education of families promotes the early recognition of disease-released complications, which allows prompt and appropriate medical evaluation and therapeutic intervention. The identification of affected infants by neonatal screening programs allows early initiation of prophylactic penicillin and pneumococcal immunizations, which help prevent overwhelming sepsis. Proper management of sickle cell anemia (SCA) begins with establishing the correct diagnosis early in life, ideally during the newborn period.
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