Sickle cell disease (SCD): standard treatments

Surveillance

• Annual transcranial Doppler (TCD): from the ages of 2 to 16 years
• Regular iron overload assessment
• Echocardiography: to monitor for pulmonary hypertension

Treatment of vaso-occlusive crises

Hydroxyurea: indications for adults

Treatment of avascular necrosis

Other interventions

Treatments of doubtful benefit

Exagamglogene autotemcel (exa-cel)

Investigational treatments for vaso-occlusive crises

References

1. Yawn BP, Buchanan GR, Afenyi-Annan AN, et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA 2014; 312:1033-1048.  
2. Hardouin G, Magrin E, Corsia A, Cavazzana M, Miccio A, Semeraro M. Sickle cell disease: from genetics to curative approaches. Annu Rev Genomics Hum Genet 2023; 24:255-275. 
3. Dixit R, Nettem S, Madan SS, Soe HH, Abas AB, Vance LD, Stover PJ. Folate supplementation in people with sickle cell disease. Cochrane Database Syst Rev 2016; 2:CD011130. 
4. Soe HH, Abas AB, Than NN, Ni H, Singh J, Said AR, Osunkwo I. Vitamin D supplementation for sickle cell disease. Cochrane Database Syst Rev 2017; 1:CD010858.
5. Swe KM, Abas AB, Bhardwaj A, Barua A, Nair NS. Zinc supplements for treating thalassaemia and sickle cell disease. Cochrane Database Syst Rev 2013; 6:CD009415.
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Related checklists

• Sickle cell disease (SCD): genetics and pathology
• Sickle cell disease (SCD): systemic features
• Sickle cell disease (SCD): neurological features
• Sickle cell disease (SCD): investigations
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