Whilst most patients will be aware that they have SCD, it is prudent to ensure testing has been done on all non-Northern Europeans prior to surgery. For routine surgery this can be done in the preoperative assessment clinic, by requesting full blood count, film and haemoglobinopathy screen (by HPLC or alternative laboratory method). Patients who are identified to have SCD should be discussed with the hematology and anesthetic team and a management plan developed as outlined below. In the emergency setting, where the sickle cell status is not known at the time of surgery, an urgent sickle solubility test, full blood count and haemoglobinopathy screen should be requested, but often the result of the haemoglobinopathy screen will be not be available immediately. A negative sickle solubility test will exclude a sickling disorder. If positive it will not discriminate between carrier or disease status. In this situation the full blood count result and blood film should be reviewed. If the patient has a normal hemoglobin concentration and no history of painful crises then HbSS is unlikely. A normal hemoglobin may be seen in HbSC but the blood film will show characteristic changes. The hematology team should be contacted for advice. If surgery is urgent it should proceed and the patient should be treated as if they have SCD until confirmatory tests are available.

All patients with SCD who need general anesthesia should be discussed with their hematologist prior to planned surgery to co-ordinate management. Suggested preoperative investigations are outlined in Table 17.1.