Sickle cell disease. Primary Care management

Abstract Sickle cell disease is a genetic structural haemoglobinopathy characterized by the presence of haemoglobin S that is becoming more prevalent in our environment because of the present immigrating pressure. The abnormal haemoglobin is unstable, tends to polymerize occluding the microcirculation what produces acute and chronic multisystem manifestations and increases the susceptibility of infections. Genetic aspects, physiopathology, clinical and laboratory diagnosis, neonatal screening, appropriate care of the main acute manifestations as lifethreatening complications that may develop rapidly, a follow-up plan, immunizations and treatment are discussed. Due to the complexity of the disease a multidisciplinary care is necessary coordinating primary care with specialized clinical management that includes periodic comprehensive evaluations and patient and family education as this decreases morbidity and mortality and improves quality of life for these patients. Key words: Sickle cell disease, Primary Care, Children.