ANAEMIA, SICKLE CELL

DESCRIPTION

Homozygous sickle cell anaemia (HbSS: HbS > 50–100%). Individuals with

sickle cell trait have < 50% HbS and are generally asymptomatic.

The disease is characterised by various crises: vaso-occlusive, aplastic,

megaloblastic and sequestration crises, and infection.

The pain crisis/vaso-occlusive crisis

The most common type of crisis is characterised by acute episodes of

severe, agonising and relentless pain. The pain may be localised to a single

long bone, typically in the juxta-articular area. It can be symmetrical in

several limbs or involve the axial skeleton, i.e. lumbar spine, ribs or pelvis,

abdomen, chest or organ systems.

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

Bed rest and/or hospitalisation.

MEDICINE TREATMENT

• Oxygen.

All patients:

• Folic acid, oral, 5 mg daily.

Analgesia

For severe pain:

• Morphine, IV, 10 mg 4 hourly.

Fluids

Keep well hydrated with intravenous fluids.

REFERRAL

» All for chronic management in a specialised centre

Reference and further reading

Standard Treatment Guidelines and Essential Medicines List for South Africa 2012 Edition