• The commonest presentation to acute hospital services in sickle cell disease is with pain.

• Pain should be assessed/reassessed regularly using a validated pain tool appropriate for patient’s age

• Pain should be assessed and analgesia given within 30 minutes of arrival in the department.

• Simple analgesia (most commonly paracetamol and ibuprofen) should be prescribed regularly rather than as required.

• Most children will have received simple analgesia at home so should be escalated to oromorph quickly.

• If pain is not controlled adequately with oral medication escalate to subcutaneous morphine or PCA.

This is a controlled document. If you are using a printed copy, check it against the guidelines site to ensure you are using the latest edition. Print copies must be destroyed after use.

Abbreviations

This guideline is intended for use in managing children with Sickle Cell disease (all genotypes, including but not limited to HbSS and HbSC) presenting to hospital with acute painful crisis.

• Sickle cell disease is the most common serious genetic disease in England.

• It affects one in 2436 live births (2016-17 data), with prevalence of 1:300 in some areas.

• Highest prevalence is in people of African and African-Caribbean origin, but it is also seen in people from the Middle East, India and the Eastern Mediterranean.

• Improvements in newborn screening, early initiation of prophylactic antibiotics, early recognition of acute crisis’ and screening for those at risk of stroke have all helped increase life expectancy.

• The latest standards and recommendations have been released in response to two national peer reviews of clinical services for those with SCD.

• Acute pain crises are caused when deformed sickled RBC occlude small blood vessels, and can vary in frequency, severity and duration from patient to patient.

• Pain is the most common cause of acute morbidity.

• Recurrent painful episodes have a negative psychological impact and may lead to a higher incidence of chronic pain and difficulty developing long-term pain coping strategies

Sickle related pain is a medical emergency to be addressed within 30 minutes

• Make a pain assessment using a tool appropriate for the child’s needs and age: Visual descriptor scale: 0 – No pain 1 – Mild 2 – Moderate 3 – Severe

• Initiate urgent analgesia within 30 minutes – paracetamol AND ibuprofen/diclofenac AND oramorph

And

And

Follow on analgesia

• Regular paracetamol and ibuprofen

• Maintenance opioids – initially dose every 4 hours, titrate to response

• Re-assess pain at least every 4 hours

• If repeated doses of strong opioid needed within 2 hours of initial pain control, consider PCA if available

Additional medical management for admissions

• Acute sickle admissions bloods panel – search under ‘during visit orders’ in EPIC

• Hydration – ensure euvolaemia with a low threshold for IV fluid. Do not aim for hyperhydration.

• Warmth

• Incentive spirometry – under Epic ‘Orders’, obtain from physio

• Do not transfuse without consultant authorisation

Presentations of particular concern - check specific regional protocols (see below)

Acute chest syndrome(if hypoxic): Consider exchange transfusion

Priapism: Contact urology urgently

Stroke: Consider exchange transfusion

Abdominal pain and jaundice: Consider cholecystitis and ascending cholangitis

Worsening anaemia (drop of 20 g/l): - Consider aplastic crisis and sequestration. Please only transfuse with consultant authorisation

Escalation to Primary Treatment Centre:

Severely unwell patients may need to be discussed with or transferred to the tertiary centre that typically oversees their care. At Wexham, this will usually be the John Radcliffe in Oxford. At Frimley, this may be the John Radcliffe, St George’s, King’s or the Evelina. Each patient’s centre will be recorded in their clinic letters and in their diagnosis overview.

• https://nssg.oxford-haematology.org.uk/red-cell-paeds/documents/acute-scd-management/PH2-acute-management-of-scd-crisis.pdfNICE guideline June 2012: Sickle cell acute painful episode: management of an acute painful sickle cell episode in hospital

• http://www.nice.org.uk/guidance/cg143

• Rees, D.,, Olujohungbe, A., Parker, E., Stephens, A., Telfer, P., Wright, J., Guidelines for the management of the acute painful crisis in sickle cell disease, British Journal of Haematology, 2003, 120, 744–752

• Cooper TE, Hambleton IR, Ballas SK, Cashmore BA, Witten PJ. Pharmacological interventions for painful sickle cell vaso-occlusive crises in adults. Cochrane Database of Systematic Reviews 2019, Issue 11. Art. No.: CD012187

• Uwaezuoke, S., Ayuk, A., Ndu, I., Eneh, C., Mbanefo, N., Ezenwosu, O., Vaso-occlusive crisis in sickle cell disease: current paradigm on pain management; Journal of Pain Research 2018:11

• No one’s listening: An inquiry into the avoidable deaths and failures of care for sickle cell patients in secondary care, Sickle Cell Society

This guideline has been registered with the Trust. However, clinical guidelines are guidelines only. The interpretation and application of clinical guidelines will remain the responsibility of the individual clinician. If in doubt, contact a senior colleague or expert. Caution is advised when using guidelines after the review date.

This guideline is for use in Frimley Health NHS Foundation Trust hospitals only. Any use outside this location will not be supported by the Trust and will be at the risk of the individual using it.

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