New advances in the treatment of sickle cell disease: focus on perioperative significance.

Sickle cell diseases comprise a group of inherited disorders that alter hemoglobin, ultimately causing hemolytic anemia and reoccurring instances of vascular occlusion that produce acute and chronic pain. Many patients with sickle cell disease require surgery for conditions associated with their disease. Painful vaso-occlusive episodes, which can be debilitating and require long hospital stays, are often precipitated by the stress of surgery. Poorly controlled postoperative pain also can worsen an impending painful crisis. Traditional therapy for patients with sickle cell disease undergoing surgery has included preoperative transfusion and postoperative opioid therapy. Recent studies have demonstrated that aggressive preoperative transfusion therapy is not beneficial over a more conservative approach. Postoperative pain control trends include nonsteroidal anti-inflammatory drugs such as ketorolac and opioid agonist-antagonist agents such as nalbuphine, as well as epidural analgesia to minimize respiratory depression. New preventive therapy for vaso-occlusive crisis includes hydroxyurea, a chemotherapeutic agent that stimulates the production of fetal hemoglobin. Inhaled nitric oxide is being used in clinical trials with success in slowing the sickling process and unsickling cells. Phase III clinical trials are in progress for 2 drugs that decrease sickling: poloxamer 188 and fructose 1-6 diphosphate. These new therapies should help improve the anesthetic course of the patient with sickle cell disease, reduce postoperative complications, and shorten hospital stays.