Lightning Learning: Sickle Cell Crisis

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STOP!

Acute sickle cell crisis⁽¹⁾ is when the sickling of blood cells blocks vessels by causing endothelial damage.

The result is a vaso-occlusive crisis which causes pain and organ ischaemia. If missed, this can lead to lifelong disabilities – even death.

In ED the main goal is to prevent complications of sickle cell crisis (see LOOK section for more examples).

It is imperative to achieve effective pain control by starting analgesia within 30 minutes of hospitalisation.

PRECIPITATING FACTORS⁽²⁾

• Cold weather
• Infection
• Stress
• Hypoxia
• Drugs

LOOK

COMPLICATIONS^(3,4)

Acute chest syndrome: can be difficult to distinguish between pneumonia and vaso-occlusive infarct. Consider CXR, ABG, blood transfusion and invasive ventilatory support.

Abdominal crisis: think splenic and hepatic sequestration. Consider CT abdomen.

Stroke: can occur in adults and children. Need to distinguish between other causes (e.g. meningoencephalitis). Consider CT head.

Avascular necrosis: Bone and joint pain, commonly in the hip. Consider x-ray of limbs.

Priapism: prompt recognition required, urological emergency. Consider urgent aspiration.

Aplastic crisis: Parvovirus B19, causes a life-threatening drop in Hb level. Consider red cell transfusion.

LEARN

TREATMENT⁽⁵⁾

Refer to local hospital guidance:

• Oxygen (15L via non-rebreathe mask)
• PROMPT Analgesia: e.g. morphine, manage every 30 minutes until pain settles (refer to patient’s haematology pain management plan if available)
• IV fluids
• Consider IV Antibiotics if suspecting underlying infection as the trigger
• Keep the patient warm (e.g. warmed fluids/bair hugger)
• Refer to hematology team for admission

REFERENCES

1. Clinical manifestations, diagnosis, and management of the cardiovascular complications of cocaine abuse (NICE)
2. Sickle Cell Crisis (LITFL)
3. Overview of the management and prognosis of sickle cell disease (UpToDate)
4. Acute ischemic and hemorrhagic stroke in sickle cell disease (UpToDate)
5. Guideline for Adults with sickle cell disease: acute presentation (UHL/Local)