World Health Organization · 1st edition, 2026 · 129 pages
WHO consolidated clinical guidelines for diagnosis and management of SCD in children and adolescents aged 0–19 years
- Issuer
- World Health Organization
- Edition
- 1st edition, 2026
- Audience
- Policy-makers, clinicians, health workers responsible for care of children and adolescents with SCD, particularly in low- and middle-income countries
- Evidence
- GRADE-rated systematic reviews; 15 recommendations (13 conditional, 2 strong) based on RCTs and observational studies
- Reviewed
- 14 Oct 2026 · GMJ
What this guideline says
GMJ summary
This guideline provides 15 evidence-based recommendations for the diagnosis, prevention and management of sickle-cell disease (SCD) in children and adolescents aged 0–19 years. It addresses priority clinical topics including point-of-care diagnostic tests, antibiotic prophylaxis against pneumococcal infection, hydroxyurea therapy, pain management, acute chest syndrome, stroke prevention and screening for complications.
The guideline includes two strong recommendations: universal hydroxyurea therapy for children and adolescents aged 9 months to 19 years with sickle-cell anaemia regardless of clinical severity, and annual transcranial Doppler ultrasound screening for primary stroke prevention in children aged 2–16 years. Most other recommendations are conditional, reflecting low or very low certainty of evidence and the need for shared decision-making and contextual adaptation.
The guideline emphasizes feasible interventions for low-resource settings, including lateral flow immunoassay and micro-engineered haemoglobin electrophoresis point-of-care tests where standard laboratory diagnostics are unavailable, penicillin prophylaxis in children aged less than 5 years, and a stepwise approach to pain management starting with NSAIDs before escalating to opioids. Implementation requires health system strengthening, capacity-building and ensuring availability of essential medicines and diagnostics.
Key points
For clinicians — doses and thresholds as stated in the guideline
- Point-of-care tests (lateral flow immunoassay, micro-engineered haemoglobin electrophoresis) suggested for diagnosis where standard laboratory haemoglobin fractionation or DNA-based tests unavailable
- Prophylactic penicillin (oral or intramuscular) suggested for all children with SCD aged <5 years to prevent pneumococcal infection, regardless of pneumococcal vaccination status
- Hydroxyurea therapy recommended for all children and adolescents aged 9 months to 19 years with sickle-cell anaemia regardless of clinical severity (strong recommendation)
- For acute pain: NSAIDs suggested initially; for moderate to severe pain, NSAIDs plus opioids suggested rather than opioids alone; reassess every 15–30 minutes
- Regular (chronic) blood transfusion with iron chelation therapy suggested for recurrent pain, with shared decision-making and monitoring for iron overload
- Antibiotics (third-generation cephalosporin plus macrolide) and blood transfusion suggested for acute chest syndrome in addition to standard supportive care
- Exchange transfusion suggested rather than simple red blood cell transfusion for suspected acute symptomatic stroke
- Annual transcranial Doppler ultrasound screening recommended for primary stroke prevention in children aged 2–16 years with sickle-cell anaemia (strong recommendation)
Summary of recommendations by clinical topic
Table extracted from the guideline
| Topic | Recommendation | Strength | Evidence certainty |
|---|---|---|---|
| Diagnosis | Lateral flow immunoassay and micro-engineered haemoglobin electrophoresis POCTs where standard tests unavailable | Conditional | Low |
| Infection prevention <5 years | Prophylactic penicillin (oral or IM) regardless of pneumococcal vaccination | Conditional | Low |
| Infection prevention ≥5 years | No penicillin if vaccinated and no risk factors; suggest if unvaccinated, splenectomy or prior invasive pneumococcal infection | Conditional | Low |
| Disease modification | Hydroxyurea therapy for all children aged 9 months–19 years with sickle-cell anaemia | Strong | Low |
| Acute pain | NSAIDs initially; NSAIDs plus opioids for moderate to severe pain rather than opioids alone | Conditional | Low to very low |
| Recurrent pain | Regular blood transfusion with iron chelation therapy | Conditional | Very low |
| Acute chest syndrome | Antibiotics (cephalosporin plus macrolide) and blood transfusion plus standard care | Conditional | Low to very low |
| Acute stroke | Exchange transfusion rather than simple RBC transfusion | Conditional | Very low |
| Secondary stroke prevention | Regular blood transfusion with iron chelation rather than hydroxyurea alone | Conditional | Very low |
| Stroke screening | Annual transcranial Doppler ultrasound for children aged 2–16 years | Strong | Very low |
Source: World Health Organization, Management of sickle-cell disease in children and adolescents. Reproduced in summary form.
Editor’s perspective
Why this matters for Georgia and the region
Prof. Giorgi Pkhakadze, MD, MPH, PhD
Editor-in-Chief, Georgian Medical Journal · Chair, Public Health Institute of Georgia · WHO consultant, health and migration
As Georgia experiences increasing migration from sickle-cell endemic regions, this WHO guideline provides essential clinical pathways our paediatricians and primary care physicians must understand. I would emphasise to Georgian colleagues the strong recommendation for annual transcranial Doppler screening in children aged 2–16 years, as stroke prevention is achievable with existing infrastructure in our regional centres. However, we must be realistic that establishing reliable hydroxyurea supply chains and training sufficient personnel in transcranial Doppler technique will require dedicated investment and at least 18–24 months of systematic implementation across our national health system.
ORCID 0000-0001-7609-4515 · Perspective written for the GMJ Guidelines Library, 14 Oct 2026
Applicability
Georgia and the South Caucasus
This is the first WHO guideline on sickle-cell disease management in children and adolescents, highly relevant for Georgia and the South Caucasus region. While SCD prevalence data for Georgia are limited, migration patterns and population diversity mean cases will be encountered. National programmes should assess local SCD burden, ensure point-of-care diagnostic capacity at primary health care level, include hydroxyurea and penicillin on essential medicines lists, establish protocols for pain management and transfusion services, and integrate SCD into noncommunicable disease strategies and child health programmes. Implementation will require training health workers on SCD recognition and management, ensuring blood transfusion safety and supply chain for essential medicines and diagnostics, and establishing referral pathways for specialist care including transcranial Doppler screening where feasible.
Questions this guideline answers
Quick answers
Which children with SCD should receive hydroxyurea?
All children and adolescents aged 9 months to 19 years with sickle-cell anaemia (HbSS or HbSβ⁰-thalassaemia) should be offered hydroxyurea therapy regardless of clinical severity. This strong recommendation aims to reduce acute complications, hospitalisations and improve quality of life. Appropriate dosing and regular monitoring are essential, and adolescents should be counselled on contraception needs.
When can penicillin prophylaxis be stopped in children with SCD?
Children aged <5 years should receive prophylactic penicillin regardless of pneumococcal vaccination. In children ≥5 years who have completed pneumococcal vaccination and have no additional risk factors (no prior invasive pneumococcal infection or splenectomy), prophylaxis is not recommended. However, continue prophylaxis in unvaccinated children ≥5 years or those with history of splenectomy or prior invasive pneumococcal infection.
What is the stepwise approach to managing acute pain in children with SCD?
Start with NSAIDs alone for mild to moderate pain. Reassess every 15–30 minutes; if pain not controlled within 30 minutes, add opioids. For moderate to severe pain, begin with NSAIDs plus opioids combination. Continue reassessment and taper opioids as pain subsides. If only NSAIDs or opioids available, use the available agent. Treatment should begin within 30 minutes of arrival at the health facility.
How should clinicians monitor children receiving regular blood transfusions for recurrent pain?
The guideline recommends monitoring for iron overload in children receiving chronic blood transfusion therapy, with concurrent iron chelation therapy to prevent complications from repeated transfusions.
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- Issuer
- World Health Organization
- Type
- Guideline
- Edition
- 1st edition, 2026
- Status
- Current
- Published
- 2026-05-12
- Languages
- English
- Scope
- Diagnosis, prevention and management of sickle-cell disease in children and adolescents aged 0–19 years; focus on primary and secondary care levels
- Audience
- Policy-makers, clinicians, health workers responsible for care of children and adolescents with SCD, particularly in low- and middle-income countries
- Pages
- 129
- Licence
- CC BY-NC-SA 3.0 IGO
- ISBN
- 978-92-4-012266-6
- Evidence
- GRADE-rated systematic reviews; 15 recommendations (13 conditional, 2 strong) based on RCTs and observational studies
- Record history
- Created 14 Oct 2026 · updated 14 Oct 2026 · next check Apr 2027
WHO consolidated guidelines for the management of common childhood illness: management of sickle-cell disease in children and adolescents. Geneva: World Health Organization; 2026. Licence: CC BY-NC-SA 3.0 IGO.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.
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