Anaesthetic care for sickle cell disease

Abstract

Despite the high frequency of sickle cell disease in Europe, the disease is poorly managed. Critical periods are the hospital stays during which the anaesthesiologist plays an important role. Understanding the molecular basis of polymerization processes of haemoglobin S can help to avoid triggering a crisis. Differentiation of the various haemoglobin phenotypes helps to estimate the individual perioperative risk. Knowledge of the patient's history and the actual haemoglobin S level facilitates general anaesthesia, surgery and postoperative care. Damage to liver, spleen, eyes, bones, lung and central nervous system increases the perioperative risk. Preoperative preparation includes early admission, intravenous volume substitution, continuing pain therapy and prophylactic antibiotic medication. General anaesthesia seems to be better for patients with a high-risk profile rather than regional anaesthesia. Careful perioperative and postoperative monitoring should allow hypoxaemia, hypovolaemia, hypothermia, acidosis and overtransfusion to be avoided. Effective pain therapy includes a combination of opioids with peripherally acting analgesia.

Sickle Cell Anemia: Conclusions from a Forensic Case Report of a Young African Woman Who Died After Anesthesia

“…Furthermore, side effects of various anesthetics are not principally different in sickle cell patients and the healthy population [21]. It thus appears that the deleterious progression in the index patient could have been the consequence of minimal changes in several confounding parameters, such as reduced blood flow, hypothermia, acidosis, and-possibly of particular importance in the present case-reduced blood pressure and intravasal hypovolemia [4].…”

“…Laboratory examinations prior to a surgical intervention usually consist of hemoglobin concentration and of hematorit, even if patients appear to be healthy. In the literature there are numerous reports on the perioperative management of patients with sickle cell anemia [2,4,6,7], distinguishing between surgical treatments with low and high risk (such as thrombosis, embolia, infections, and blood loss). With respect to the as yet rather rare occurrence of sickle cell anemia and the predominating heterozygocity in Germany and other European countries, however, there are, for instance, no special demands of preoperative laboratory examinations in asymptomatic young patients in the guidelines of the German Society of Anesthetics and Intensive Medicine.…”

“…About 7% of death cases of patients suffering from sickle cell anemia are associated with surgical operations. Furthermore, 75% of surgical patients develop signs of postoperative morbidity, such as hand-and-foot syndrome (dactylitis), acute chest syndrome, central nervous systemic events, aplastic crises, and splenic sequestration crisis [4,5]. According to a U.S. study on 553 homozygous surgical patients [2], there were 10 lethal cases within a 30-day postoperative observation period.…”

“…As death causes, this study reported, among others, respiratory insufficiency syndrome, acute renal failure, sepsis, and disseminated intravasal coagulation. Independently of surgical intervention, hypoxia, acidosis, hypothermia, physical stress, infections, and shock can induce a sickle cell crisis, and these events are connected with episodic acute hemolysis and vascular occlusions [4]. On the other hand, orthopedic surgery in sickle cell patients and cardiac surgery in sickle cell trait have been successfully performed [6,7].…”

“…Though the overall risk of complications after surgery is distinctly increased [4], there is still a strong need to improve and maintain factual information about this disease even in regions where sickle cell disease is endemic [8]. Accordingly, there are only sporadic recommendations, but no guidelines concerning specific preoperative tests, especially for negroid patients (not only for those of actual African origin) to verify or falsify a possible state of sickle cell anemia and to provide a specific perioperative and anesthesiological management [6].…”