| Summary of evidence-based interventions for children and adolescents with SCA | |||
| Interventions | Recommendations | Strength of recommendation | Certainty in evidence |
| Infection prevention | |||
| Penicillin prophylaxis | Prophylactic penicillin therapy should be given until age 5 years in children with SCD-SS | Strong | Moderate |
| 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 discontinued | Weak | Moderate | |
| Pneumococcal vaccination | All children with SCD should be vaccinated against Streptococcus pneumoniae | Strong | Moderate |
| Malaria prevention | Children with SCD in malaria endemic areas should receive prophylaxis against malaria | Strong | Low |
| Management of acute complications | |||
| Vaso-occlusive pain episodes | The 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. | Strong | Low |
| A short course (5–7 days) of NSAIDs in addition to opioids is recommended for acute SCD pain management | Conditional | Low | |
| A systematic review of the evidence recommends against using chronic monthly transfusion therapy as first-line strategy to prevent recurrent acute pain episodes | Conditional | Low | |
| Acute chest syndrome | Treat 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 hypoxaemia | Strong | Low |
| 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 required | Conditional | Low | |
| 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 levels | Conditional | Low | |
| Acute treatment of suspected or confirmed stroke | For 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 resources | Strong | High |
| 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 planned | Moderate | Low | |
| Stroke prevention | |||
| Primary stroke prevention | Children 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. | Strong | Moderate |
| 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 recommended | Strong | Low | |
| Secondary stroke prevention | For 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 recommended | Strong | Low |
| 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. | Strong | Low | |
| Hydroxyurea therapy | Children with SCA who have severe disease (recurrent pain, dactylitis, ACS and anaemia) should be treated with hydroxyurea. | Strong | High |
| 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. | Strong | Moderate | |
| 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. | Strong | Low | |
| Screening for chronic complications | |||
| Pulmonary disease | Children with SCD with no respiratory symptoms should not be screened with pulmonary function tests. | Conditional | Low |
| Pulmonary hypertension | Screening asymptomatic children with SCD for pulmonary hypertension using echocardiography is not recommended. | Conditional | Low |
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.