Table 2
Summary of evidence-based interventions for children and adolescents with SCA
InterventionsRecommendationsStrength of recommendationCertainty in evidence
Infection prevention
 Penicillin prophylaxisProphylactic penicillin therapy should be given until age 5 years in children with SCD-SSStrongModerate
In children with SCD-SS who have not had prior IPD or splenectomy and have completed the recommended pneumococcal vaccination series, prophylactic penicillin can be discontinuedWeakModerate
 Pneumococcal vaccinationAll children with SCD should be vaccinated against Streptococcus pneumoniaeStrongModerate
 Malaria preventionChildren with SCD in malaria endemic areas should receive prophylaxis against malariaStrongLow
Management of acute complications
 Vaso-occlusive pain episodesThe WHO strategy recommends acetaminophen together with ibuprofen (an NSAID) for mild pain, and a strong opioid (morphine as first choice) for moderate-to-severe pain.StrongLow
A short course (5–7 days) of NSAIDs in addition to opioids is recommended for acute SCD pain managementConditionalLow
A systematic review of the evidence recommends against using chronic monthly transfusion therapy as first-line strategy to prevent recurrent acute pain episodesConditionalLow
 Acute chest syndromeTreat children with SCD who have ACS with an intravenous cephalosporin, an oral macrolide, supplemental oxygen (to maintain saturation >95%) and close monitoring for bronchospasm, acute anaemia and hypoxaemiaStrongLow
Give simple blood transfusion to improve oxygen-carrying capacity to children with moderately symptomatic ACS whose haemoglobin >10 g/L below baseline. If base haemogobin ≥90 g/L, blood transfusion may not be requiredConditionalLow
Perform urgent exchange transfusion when, despite simple transfusion, there is rapid progression of ACS with oxygen saturation <90% despite supplemental oxygen, increasing respiratory distress, progressive pulmonary infiltrates and/or decline in haemoglobin levelsConditionalLow
 Acute treatment of suspected or confirmed strokeFor children with SCD and acute neurological deficits including TIA prompt blood transfusion is recommended. The transfusion should be given immediately on recognition of symptoms without delay beyond 2 hours of acute neurological symptom presentation. The type of transfusion (simple or exchange blood transfusion) is dependent on individual patient factors and local transfusion resourcesStrongHigh
For children with SCD and acute neurological deficits including TIA, exchange blood transfusion (EBT) is recommended versus simple blood transfusion. When exchange blood transfusion is not available within 2 hours and haemoglobin is <85 g/L, simple blood transfusion can be preformed to avaoid delays in treatment while EBT is plannedModerateLow
Stroke prevention
 Primary stroke preventionChildren with SCA who have abnormal TCD screening should receive regular blood transfusions given every 3–4 weeks aimed to maintain HbS level <30% and haemoglobin level >90 g/L and <130 g/L.StrongModerate
For children with SCA who have abnormal TCD screening and live in LMICs, where regular transfusions and chelation therapy are not available or affordable, hydroxyurea therapy with dose ranging from 20 mg/kg/day to maximum tolerable dose is recommendedStrongLow
 Secondary stroke preventionFor children with SCA and a history of prior stroke regular blood transfusion with goals to increase haemoglobin >90 g/L and maintaining HbS level at <30% is recommendedStrongLow
In LMICs where regular transfusion therapy is not available or affordable, hydroxyurea therapy is an alternative—inferior to transfusion therapy but better than no therapy at all for secondary stroke prevention.StrongLow
Hydroxyurea therapyChildren with SCA who have severe disease (recurrent pain, dactylitis, ACS and anaemia) should be treated with hydroxyurea.StrongHigh
Infants 9 months or older, children and adolescents with SCA should be offered hydroxyurea therapy regardless of clinical severity (depending on patients’ or families’ values, drug availability and costs) to prevent SCA-related complications.StrongModerate
For children with SCA who need primary and secondary stroke prevention in LMICs where regular transfusions and chelation therapy are not available or affordable, hydroxyurea therapy is recommended.StrongLow
Screening for chronic complications
 Pulmonary diseaseChildren with SCD with no respiratory symptoms should not be screened with pulmonary function tests.ConditionalLow
 Pulmonary hypertensionScreening asymptomatic children with SCD for pulmonary hypertension using echocardiography is not recommended.ConditionalLow
  • ACS, acute chest syndrome; HbS, sickle haemoglobin; IPD, invasive pneumococcal disease; LMICs, low-income and middle-income countries; SCA, sickle cell anaemia; SCD, sickle cell disease; TCD, transcranial Doppler; TIA, transient ischaemic attack.