Sickle cell hemoglobinopathies: a protocol for management.

Two cases have been presented: one shows complications when a latent form of sickle cell disease was manifested after orthognathic surgery; the other illustrates a protocol for management of patients with abnormal hemoglobins who are candidates for orthognathic surgery. The protocol includes an initial screening test for all black patients who are hospitalized for elective orthognathic surgery. When the screening test is positive, afollow-up hemoglobin electrophoresis is obtained to identify the specific hemoglobinopathy. Transfusions, when indicated, are given to alter the percentages so that the patient has no greater than 40% abnormal hemoglobin. Precautions are taken during anesthesia and after surgery to ensure that the patient is well oxygenated and is not in a hypothermic state. Serial electrophoreses are performed and transfusions, if indicated, are given during the postoperative and healing phases according to the postoperative status.