Table 2.
Drugs used in sickle cell disease.
| Drug | Recommended dose | Frequency | When to stop | Comments |
|---|---|---|---|---|
| Phenoxymethyl penicillin (Pen-V) | <1 year: 62.5mg | Oral, twice daily, beginning at 3 months of life | 5 years, unless child had splenectomy or invasive pneumococcal infection | Prophylaxis against encapsulated bacteria |
| 1-3 years: 125 mg | ||||
| ≥3 years: 250 mg | ||||
| Erythromycin | Same dose as Pen-V | Macrolides for penicillin allergic patients | ||
|
| ||||
| Sulfadoxine-pyrimethamine (e.g., Fansidar) | <2 years: ½ tablet | Oral, once every month | Life-time | Prophylaxis against malaria Begin from age >2 months |
| 2 to 5 years: 1 tablet | ||||
| >5 years: dose based on weight | ||||
| Proguanil | <1 year: 25mg | Oral, once daily | Prophylaxis against malaria. Based on country's national guidelines Encourage other malaria prevention strategies |
|
| 1-3 years: 50mg | ||||
| 3-6 years: 50-100mg | ||||
| >6 years: 100-200mg | ||||
|
| ||||
| Folic acid | <1 year: 62.5 – 2.5 mg | Oral, once daily | Life-time | All children aged <3 years may receive 2.5mg daily |
| 1-3 years: 2.5 mg | ||||
| ≥3 years: 5 mg | ||||
|
| ||||
| Hydroxyurea | 15-20 mg/kg/day initial dose (max=35mg/kg/day) | Oral, once daily | See section 10.5 | Escalate starting dose by 2.5 to 5mg/kg every 8 weeks until clinical response or hematological adverse effects |
|
| ||||
| L-glutamineª | <30 kg: 5g (1 packet) | Oral, twice daily | Given to patients aged ≥5 years | |
| 30-65 kg: 10g (2 packets | ||||
| >65 kg: 15g (3 packets) | ||||
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| Crizanlizumabª | 5 mg/kg/dose | Intravenous, repeat dose after 2 weeks of the first, then every after 4 weeks | Given to patients aged ≥16 years | |
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| Voxelotor | 1500 mg | Oral, once daily | Give to patients aged ≥12 years Improves anemia |
|