Anaemia, sickle cell

D57.0-3/D57.8

DESCRIPTION

Homozygous sickle cell anaemia (HbSS). Individuals with sickle cell trait have <50% HbS and are generally asymptomatic. Milder sickle cell disease occurs in individuals with HbSC.
The disease is characterised by recurrent acute vaso-occlusive episodes (“sickle crises”) and chronic haemolytic anaemia.
Adults develop hyposplenism, predisposing them to infection with encapsulated bacteria.

Vaso-occlusive episodes

Vaso-occlusion can involve any part of the body, especially the skeleton. Episodes may be triggered by dehydration, infection, stress or menstruation. The most common presentation is with acute episodes of pain, varying in severity, in the affected areas.

Investigations

The diagnosis is suspected from the history, peripheral blood examination, and/or screening tests for sickling.
Diagnosis is confirmed on haemoglobin electrophoresis.

GENERAL MEASURES (SEVERE VASO-OCCLUSIVE EPISODES)

Keep well hydrated with intravenous fluids.
Transfusion is only indicated for severe episodes with severe anaemia – discuss with a specialist.
Pain must be controlled.

MEDICINE TREATMENT (SEVERE VASO-OCCLUSIVE EPISODES)

  • Use of Oxygen to maintain adequate saturation.

To prevent venous thromboembolism:

  • Low molecular weight heparin, e.g.:
  • Enoxaparin, SC, 40 mg daily.

In morbid obesity dosing of LMWH should be individualised, in discussion with a specialist.

LoEIII [8]

In renal failure (eGFR <30 mL/minute), the recommended dose of LMWH is 1 mg/kg daily.

LoEIII [9]

OR

  • Unfractionated heparin, SC, 5 000 units 12 hourly.

LoEIII [10]

Analgesia

Refer to Anaesthesiology and intensive care.

MEDICINE TREATMENT (CHRONIC MANAGEMENT)

All patients:

  • Folic acid, oral, 5 mg daily.
  • Vaccination against infections due to pneumococci and haemophilus (see Adult vaccination).

Hydroxyurea (specialist-initiated) is the mainstay of therapy in severe disease. Typical indications include:

  • frequent painful vaso-occlusive episodes,
  • severe vaso-occlusive episodes (e.g. acute chest syndrome, stroke), and
  • severe symptomatic anemia.

REFERRAL

  • All patients, for chronic management in a specialised centre.
  • Vaso-occlusive episodes should be managed in consultation with a specialist.