Anaemia, Sickle Cell

Description

Diagnostic criteria

General and supportive measures

Medicine treatment

Referral

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ICD-10 code: D57

DESCRIPTION

Haemolytic anaemia due to homozygous inheritance of sickle cell mutation.

Patients may experience complications:

• Painful vaso-occlusive crises
• Haemolytic crises (usually secondary to infection)
• Aplastic crises
• Thrombotic crises, e.g. acute chest syndrome, priapism or stroke
• Splenic sequestration
• Severe infections

DIAGNOSTIC CRITERIA

Clinical

• Pallor, jaundice, fatigue all of which may worsen abruptly (sequestration crisis, aplastic crisis)
• Features of complications:
  • painful swelling of the hands and feet (dactylitis)
  • bone pain, abdominal pain
  • chest pain, fever, dyspnoea (acute chest syndrome)
  • convulsions, hemiparesis
  • priapism

Investigations

• Laboratory features of haemolytic anaemia. See: Anaemia, haemolytic.
• Haemoglobin electrophoresis shows an SS pattern (both parents will be AS).

GENERAL AND SUPPORTIVE MEASURES

• Avoid exposure to cold, dehydration and stress.
• Increase fluid intake during painful crises.
• Heat and/or massage for pain.

MEDICINE TREATMENT

For sequestration crisis or aplastic crisis:

• Packed red cells, IV, 15 mL/kg.
  • Do not transfuse Hb > 13 g/dL as this may increase blood viscosity and consequently raise the risk of vasculopathy.

If hypoxic:

• Oxygen, by face mask.

Exchange transfusions may be used to treat severe complications (see referral criteria).

Prophylaxis against infection

Prophylaxis is given to all children because functional asplenia is present by 1–2 years of age.

• Routine vaccinations during infancy.
• Catch up conjugate pneumococcal vaccine:
  • If < 12 months of age: 3-dose series.
  • If 12 months of age and older: 2 doses, 8 weeks apart.
    • Pneumococcal polysaccharide vaccine at 2 years (at least 8 weeks after conjugate vaccine). Repeat vaccination as a booster 5 years after the initial dose.
• Annual influenza vaccination.
• Haemophilus influenzae, type B (Hib) booster, IM, 0.5 mL.
• Meningococcal conjugate vaccine (MCV), IM, 0.5 mL.
• Phenoxymethylpenicillin, oral, 12 hourly.
  • < 5 years: 125 mg
  • > 5 years: 250 mg
  • Give indefinitely.

Treatment
Analgesia as required:

• Paracetamol, oral, 15 mg/kg 6 hourly.

AND

• Ibuprofen, oral, 10 mg/kg 8 hourly.
• Hydroxyurea, oral, 15 mg/kg.
  • Increase by 5 mg/kg every 12 weeks.
  • Maximum dose: 35 mg/kg daily.

Infections:
All children with axillary temperature ≥ 38° C

• Ceftriaxone, IV, 50–80 mg/kg/dose once daily.

Acute chest syndrome:
Consult a paediatrician.

REFERRAL

• All children with sickle cell anaemia should be managed in consultation with a paediatric haematologist or paediatrician.
• All children with severe complications that may benefit from exchange transfusion or intensive care, e.g. stroke, severe vaso-occlusive disease and acute chest syndrome.
• All cases of stroke should be referred for a regular transfusion program.

Click here to access the pdf.

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Standard Treatment Guidelines and Essential Medicines List for South Africa Paediatric Hospital_2023 edition_Updated February 2025

Chapter 3: Blood and Blood-Forming Organs, 3.2.2 Anaemia, Sickle Cell