- 02 Jul, 2019
Management of sickle cell disease starts right from the neonatal period. Early diagnosis helps to prevent complications. If the diagnosis is missed and appropriate treatment is not given, death may occur due to sepsis and pneumonia. A small dried blood spot (DBS) for analysis, is collected from cord blood or the infant’s heel/toe to detect abnormal sickle hemoglobin (Hemoglobin S, HbS) by hemoglobin electrophoresis. The next step after diagnosis is the education of the parents and family. The disease is explained to them and the importance of antibiotic prophylaxis is stressed to prevent severe infections. Guidelines recommend penicillin prophylaxis and pneumococcal vaccine to prevent pneumococcal infection in children.
Possible treatment options like hydroxyurea and transfusions can be considered. Blood transfusion is needed to maintain hemoglobin (Hb) levels and to reduce HbS levels. Hydroxyurea decreases crises in patients with severe sickle cell crisis. Consultation for stem cell transplantation, if needed, may be given by experts.
Fever in these children should never be treated at home by family as it can mask severe infection. The parents should learn to recognize signs and symptoms of ASSC: pallor, fussiness or irritability, and tender splenomegaly. As the child grows older, parents must be vigilant and learn to identify symptoms of pain, respiratory distress, acute chest syndrome, and stroke. Mild pain may be managed at home with oral hydration, massage, self-relaxation techniques, and pain killers, as advised by the physician. Severe pain requires aggressive management in a hospital setting. Other severe complications are acute chest syndrome and stroke which need proper treatment.
Education must be continued in adolescents and adults to generate self-awareness and self-care. They must be taught the importance of preventing these complications and at the same time try to have a normal and productive life.
Patrick T. McGann et al, Current Management of Sickle Cell Anemia, Cold Spring Harb Perspect Med. 2013 Aug; 3(8): a011817.