Table 3

Management of sickle cell anaemia in hICs and lmICs

	HICs	LMICs
NBS and early intervention	Universal NBS programme includes SCD	No universal NBS system Standalone local SCD screening programmes POC testing Linked to immunisation clinics and primary health centres During healthcare encounters
	Penicillin prophylaxis	Penicillin prophylaxis Malaria prophylaxis
	Pneumococcal immunisation	Pneumococcal vaccines- Global Alliance for Vaccines and Immunisation (GAVI) supported primary PCV
	Splenic palpation Fever and pain management Parental/family education	Splenic palpation Fever and pain management Parental/family education Integration into primary healthcare
Comprehensive care	TCD screening Primary stroke prevention Hydroxyurea therapy Chronic blood transfusion Exchange blood transfusion Haematopoietic stem cell transplant	TCD testing if available Hydroxyurea therapy Primary stroke prevention Widespread use beneficial in the absence of routine TCD testing Nutritional support
	Acute care Acute pain episodes Sepsis Acute chest syndrome Acute splenic sequestration Acute stroke	Acute care Acute pain episodes Sepsis and malaria Acute chest syndrome Acute severe anaemia Acute splenic sequestration Acute stroke
	Health maintenance SCD centres linked to lower levels of care Screening for cerebrovascular disease MRI and Magnetic Resonance Angiography (MRA), retinopathy, renal disease Psychosocial support of patient/family	Health maintenance SCD clinics in district hospitals linked to primary and tertiary levels of care Screening for cerebrovascular disease using validated clinical tools (if MRI/MRA not available), retinopathy, renal disease Psychosocial support of patient/family
Prevention of SCD	Awareness in at-risk population	Public awareness and education Engagement of community leaders
	Genetic counselling	Training of genetic counsellors Improve access to genetic counsellors
	Premarital testing and counselling Prenatal diagnosis PGD	Premarital testing and counselling Prenatal diagnosis and PGD (not widely available)

HICs, high-income countries; LMICs, low-income and middle-income countries; NBS, newborn screening; PCV, pneumococcal conjugate vaccine; PGD, preimplantation genetic diagnosis; POC, point-of-care; SCD, sickle cell disease; TCD, transcranial Doppler.