Sickle Cell Crisis

Sickle cell vaso-occlusive crisis requires rapid analgesia titration. Acute Chest Syndrome (new infiltrate + respiratory symptoms) is the leading cause of death — consider exchange transfusion early. Involve haematology for any complication.

NICE NG143 Sickle Cell Protocol

The pathway

1 · Assess for ACS

Any chest pain, fever or falling SpO₂ in sickle cell = Acute Chest Syndrome until proven otherwise. Escalate immediately.

2 · Analgesia within 30 minutes

IV/SC morphine titrated rapidly
paracetamol 1g QDS
NSAID (if tolerated)
opioid
PCA for severe crises

3 · O2 and hydration

SpO₂ target 95%+
Avoid dehydration (promotes sickling)
Avoid overhydration (precipitates ACS)
Target euvolaemia

4 · CXR and bloods

CXR: look for new infiltrate (ACS)
FBC, reticulocytes, LDH, renal function
Blood cultures if febrile

5 · Haematology involvement

Early haematology input for any complication
Exchange transfusion if ACS or stroke
Top-up transfusion for aplastic crisis

6 · Prevent and discharge

On discharge: hydroxycarbamide review. Folic acid 5mg daily. Prophylactic penicillin V (if splenectomy or hyposplenism). Crisis prevention education.

Drugs

Drug	Dose	Route	Notes
Morphine	0.1mg/kg	IV/SC	Titrate to pain. Repeat every 20–30 mins until controlled. PCA for severe/prolonged crises.
Paracetamol	1g	PO/IV	QDS. Regular, NOT PRN.
Ibuprofen	400mg	PO	TDS with food if renal function normal. Avoid in AKI.
Hydroxycarbamide	15–30mg/kg	PO	Disease-modifying. Reduces crises by 50%. Discuss with haematology.

When to escalate

Acute chest syndrome (new CXR infiltrate + respiratory symptoms) — haematology urgently, ITU, exchange transfusion,Stroke — emergency exchange transfusion, neurology + haematology,Priapism >4h — urology urgently,Aplastic crisis (low reticulocytes + falling Hb) — transfusion, viral screen (parvovirus B19)

Reference: NICE NG143 (2021) — Sickle Cell Disease / BSH Sickle Cell Guidelines

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