Management of Acute Presentations of Sickle Cell Disease in the Emergency Department - Guideline

Authors: Tom Bannister / Codes: CP1, CP2, HC8, HP1, IP1, NeuC12, ObC11, PC1, PC3, ResP1, ResP2, SLO1, SLO3, SLO5, UC4 / Published: 17/06/2024

Scope / Reason for development

This guideline provides recommendations on the initial care of patients presenting to the Emergency Department (ED) with acute sickle cell disease (SCD) and its complications.

The aim is to increase ED awareness of SCD, optimise and standardise the management of such patients, and ensure equity of access to acute care and specialist services. Racial bias and stigmatisation of predominantly young black individuals living with SCD has been recognised. Patients presenting in extreme pain are often accused directly or indirectly of drug seeking and report high levels of anxiety and a lack of empathy and respect when accessing emergency care.

Summary of Recommendations

All Emergency Departments should be aware of sickle cell disease, be able to recognise cases and have knowledge of the potential complications.
All patients presenting acutely with sickle cell disease should be triaged urgently, aiming to assess within 30 minutes.
All Emergency Departments should have a locally agreed care pathway for the management of sickle cell disease.
Recognition and alleviation of pain should be a priority. All patients should be assessed and offered analgesia if required within 15 minutes of arrival, and reassessed within 15 minutes if in severe, or 30 minutes if in mild-moderate pain.
Ensure that all acutely unwell sickle cell patients are cared for in an appropriate and warm environment, or have warming measures provided (such as a Bair Hugger), with provision of drinking water.
Initial assessment and decision regarding the identification of a sickle cell disease complication or severe pain crisis should occur within one hour of presentation.
Decision-making for uncomplicated cases regarding discharge or appropriate disposition such as a haematology day care unit or agreed Same Day Emergency Care pathway (where available) should be made within two hours.
Identification of a significant sickle cell disease complication or severe pain crisis warrants prompt discussion with haematology services and prompt referral and discussion with an admission team such as critical care, if required. Such patients should be referred and seen by the relevant speciality within 30 minutes of recognition of the potential complication.
If specialist haematology services are required and cannot be provided on-site, the patient will require urgent transfer of care to a centre with appropriate facilities and expertise. Transfer agreements with the local ambulance service should be in place with an agreed pathway and effective communication process.
All decisions concerning treatment and transfer should, wherever possible, be made in conjunction with the patient and/or their carer/family.

Ensure that you read the full Management of Acute Presentations of Sickle Cell Disease in the Emergency Department Guideline

Key Information

Acute vaso-occlusive episodes (VOE) are caused by blockage of capillaries by sickled cells, with subsequent tissue infarction. Precipitants include dehydration, hypoxia, and infection. VOE can occur unpredictably, sometimes without clear precipitating factors, and with marked variation in frequency, duration, and severity.

The primary goal in the management of a VOE is to achieve effective prompt and safe pain control. Offer analgesia within 15 minutes of presentation. Re-evaluation should occur within 15 minutes for severe pain and within 30 min for minor-moderate pain. There is no requirement to wait two hours before repeating an opiate dose, provided there is no clinical evidence of opiate toxicity.

Situations suggestive of a more complex SCD presentation requiring ongoing care and admission include: