Guideline

WHO consolidated guidelines for the management of common childhood illness: management of sickle-cell disease in children and adolescents

Year of Publication

2026

Issuing Institution

World Health Organization

Links

WHO IRIS

Recommendations

15 included recommendations

In children and adolescents (aged 0–19 years) with suspected sickle-cell disease (SCD), the use of lateral flow immunoassay and micro-engineered haemoglobin electrophoresis point-ofcare tests (POCTs) is suggested for diagnosis in settings where standard laboratory-based haemoglobin fractionation or DNA-based tests are not available.

Status

New

Recommended in favor

Conditional

Certainty of evidence

Low

In children aged less than 5 years with sickle-cell disease (SCD), prophylactic penicillin (oral or intramuscular (IM)) is suggested to prevent pneumococcal infection, including in those who have received age-appropriate pneumococcal vaccination.

Status

New

Recommended in favor

Conditional

Certainty of evidence

Low

In children aged older than 5 years with sickle-cell disease (SCD) who have completed pneumococcal vaccination series and do not have additional risk factors, such as prior invasive pneumococcal infection or history of surgical splenectomy, prophylactic penicillin is not recommended.

Status

New

Recommended against

Conditional

Certainty of evidence

Low

In children aged older than 5 years with sickle-cell disease (SCD), who have not completed pneumococcal vaccination series, or who have a history of splenectomy or prior invasive pneumococcal infection, prophylactic penicillin is suggested, regardless of vaccination status.

Status

New

Recommended in favor

Conditional

Certainty of evidence

Low

In children and adolescents (aged 9 months to 19 years) with sickle-cell anaemia (SCA), hydroxyurea therapy is recommended regardless of clinical severity.

Status

New

Recommended in favor

Strong

Certainty of evidence

Low

In children and adolescents (aged 0–19 years) with sickle-cell disease (SCD), experiencing acute painful crises, the use of non-steroidal anti-inflammatory drugs (NSAIDs) is suggested for initial pain management.

Status

New

Recommended in favor

Conditional

Certainty of evidence

Very low

In children and adolescents (aged 0–19 years) with sickle-cell disease (SCD), the use of non-steroidal anti-inflammatory drugs (NSAIDs) in combination with opioids is suggested for the management of moderate to severe acute pain, rather than opioids alone.

Status

New

Recommended in favor

Conditional

Certainty of evidence

Low

In children and adolescents (aged 0–19 years) with sickle-cell disease (SCD), the use of opioids alone is suggested for acute pain management rather than paracetamol plus opioids.

Status

New

Recommended in favor

Conditional

Certainty of evidence

Very low

In children and adolescents (aged 0–19 years) with sickle-cell disease (SCD), regular (chronic) blood transfusion with iron chelation therapy is suggested for the management of recurrent pain

Status

New

Recommended in favor

Conditional

Certainty of evidence

Very low

In children and adolescents (aged 0–19 years) with sickle-cell disease (SCD) and a clinical diagnosis of acute chest syndrome (ACS), the use of antibiotics in addition to standard therapeutic and supportive interventions is suggested.

Status

New

Recommended in favor

Conditional

Certainty of evidence

Low

In children and adolescents (aged 0–19 years) with sickle-cell disease (SCD) and a clinical diagnosis of acute chest syndrome (ACS), blood transfusion in combination with standard therapeutic interventions is suggested rather than standard therapeutic interventions alone.

Status

New

Recommended in favor

Conditional

Certainty of evidence

Very low

In children and adolescents (aged 0–19 years) with sickle-cell disease (SCD) and suspected acute symptomatic stroke, exchange transfusion is suggested rather than simple red blood cells (RBC) transfusion.

Status

New

Recommended in favor

Conditional

Certainty of evidence

Very low

In children and adolescents (aged 0–19 years) with sickle-cell disease (SCD) at risk of recurrent stroke (e.g. history of stroke, haemoglobin S >30% and haemoglobin <9 g/dL), regular blood transfusion with iron chelation therapy is suggested rather than hydroxyurea therapy alone for secondary stroke prevention.

Status

New

Recommended in favor

Conditional

Certainty of evidence

Very low

In children and adolescents (aged 0–19 years) with sickle-cell disease (SCD) who have no symptoms of pulmonary hypertension, routine echocardiogram screening to detect pulmonary hypertension is not suggested.

Status

New

Recommended against

Conditional

Certainty of evidence

Low

In children and adolescents (aged 2–16 years) with sickle-cell anaemia (SCA) and no prior abnormal transcranial Doppler (TCD) results, annual TCD ultrasound screening is recommended for primary prevention of stroke.

Status

New

Recommended in favor

Strong

Certainty of evidence

Very low