Sickle cell disease — MSF Clinical guidelines

What this guideline says

GMJ summary

This MSF clinical guideline provides comprehensive management protocols for homozygous sickle cell disease (SCD), a genetic haemoglobin disorder common in sub-Saharan Africa (1–3% of births), the Americas, India and the Mediterranean basin. The guideline addresses major acute manifestations including vaso-occlusive crises, infections, acute anaemia, stroke, acute chest syndrome and priapism, with detailed treatment algorithms adapted for resource-limited settings.

The guideline emphasises early recognition of life-threatening complications, aggressive hydration (minimum 100 ml/kg daily in children, 50 ml/kg in adults), appropriate use of antibiotics (ceftriaxone for suspected bacterial infections), and judicious transfusion thresholds (Hb < 5 g/dl or drop ≥ 2 g/dl below baseline). Pain management follows a stepwise approach from paracetamol/ibuprofen to morphine for severe crises.

Prevention strategies include lifelong phenoxymethylpenicillin prophylaxis until age 15 years, comprehensive immunisation including pneumococcal conjugate vaccine, folic acid supplementation (5 mg daily for adults), malaria chemoprophylaxis with mefloquine in endemic areas, and structured patient/family education on crisis triggers and warning signs requiring urgent medical attention.

Key points

For clinicians — doses and thresholds as stated in the guideline

1. Minimum daily hydration for vaso-occlusive crises: 100 ml/kg in children, 50 ml/kg in adults (2.5–3 litres); warm compresses and stepwise analgesia from paracetamol/ibuprofen to morphine for severe pain
2. Admit all children < 2 years with fever, children with fever ≥ 38.5 °C and adults with fever ≥ 39.5 °C; treat with ceftriaxone 50 mg/kg once daily (< 20 kg) or 1–2 g once daily (≥ 20 kg)
3. Transfuse packed red blood cells if Hb < 5 g/dl or drop ≥ 2 g/dl below baseline; start with 10–15 ml/kg over 3–4 hours (raises Hb by ~2.5 g/dl per 10 ml/kg)
4. Acute chest syndrome: oxygen to maintain SpO₂ 94–98%, ceftriaxone plus azithromycin (10 mg/kg daily for children, 500 mg then 250 mg for adults) for 7–10 and 5 days respectively
5. Osteomyelitis: ceftriaxone 50 mg/kg every 12 hours plus cloxacillin 50 mg/kg every 6 hours IV for ≥ 14 days, then oral ciprofloxacin 15 mg/kg twice daily plus co-amoxiclav for 14 days
6. Phenoxymethylpenicillin prophylaxis until age 15 years: 62.5 mg twice daily (< 1 year), 125 mg twice daily (1–5 years), 250 mg twice daily (5–15 years)
7. Folic acid 5 mg daily lifelong (2.5 mg for infants < 1 year); mefloquine 5 mg base/kg weekly for malaria prophylaxis if prevalence ≥ 5%
8. Stroke: exchange transfusion is treatment of choice; if unavailable, transfuse if Hb ≤ 9 g/dl targeting 10 g/dl, oxygen at ≥ 5 litres/minute, transfer to specialised facility

Laboratory tests and indications in sickle cell disease

Table extracted from the guideline

Source: Médecins Sans Frontières, Sickle cell disease — MSF Clinical guidelines. Reproduced in summary form.

From the guideline

Verbatim

“All children less than 2 years; Children with fever ≥ 38.5 °C and adults with fever ≥ 39.5 °C; patients who are critically ill appearing or have acute anaemia.”

Editor’s perspective

Why this matters for Georgia and the region

Applicability

Georgia and the South Caucasus

Questions this guideline answers

Quick answers

Related on GMJ

Interconnected resources

GMJ Guidelines Library records are original summaries written by the GMJ editorial team from the issuer’s published document and reviewed by a physician. They do not replace the full guideline. The document is the copyright of its issuer and is linked, not hosted. Spotted an error or a newer edition? Tell the Guidelines Desk.