Advice for ED staff on Sickle Cell

Acute sickle cell pain episode (“crisis”) is the most common reason for patients with Sickle Cell Disease to present to a healthcare provider. However, the standard of care provided in the ED can vary significantly across institutions and provinces. CanHaem would like to offer some resources to assist you in providing the best possible care.The mainstay of managing a sickle cell crisis is the administration of rapid, sustained and effective analgesia. Pain should be assessed objectively using a validated and age appropriate pain score along with documentation of the distribution of pain sites throughout the body, and any analgesia taken so far during the episode. Knowing what works well for the patient in previous crises can assist you in the selection of an effective analgesia protocol this time.

Alongside this a focused clinical assessment should exclude any serious complications such as infection, Acute Chest Syndrome, stroke or priapism, as well as surgical emergencies, e.g. acute cholecystitis.

Investigations will often not yield much additional useful information beyond a CBC and reticulocyte count to see if anemia is due to accelerated hemolysis or an aplastic episode (e.g.parvovirus-induced).

The British Society for Standards in Haematology (BCSH) has published widely accepted guidance on the management of a Sickle Cell Crisis (linked here). Please note these are currently undergoing revision, as are the NIH sickle cell guidelines.