WHO consolidated guidelines for the management of common childhood illness: management of sickle-cell disease in children and adolescents

Overview

Sickle-cell disease (SCD) is the most common inherited blood disorder worldwide and a leading cause of preventable child death and disability, particularly in low- and middle-income countries (LMICs). The highest burden falls on sub-Saharan Africa, with substantial prevalence across other historically malaria-endemic regions, including the Eastern Mediterranean Region, the Caribbean, South Asia and parts of Latin America and growing recognition of SCD among diaspora populations in high-income settings.

This publication is the first WHO normative guideline on the diagnosis, prevention and clinical management of sickle-cell disease in children and adolescents (0–19 years). It closes a longstanding gap in global guidance and supports Member States in delivering equitable, evidence-based care for children and adolescents living with SCD, advancing universal health coverage and the Sustainable Development Goal targets on noncommunicable diseases (SDG 3.4) and ending preventable child deaths (SDG 3.2).

Developed using WHO standard methods and the GRADE approach, the guideline issues 15 recommendations across seven priority clinical areas:

• Point-of-care diagnosis and early identification, including lateral flow immunoassays and micro-engineered haemoglobin electrophoresis.
• Infection prevention, including penicillin prophylaxis and links to pneumococcal vaccination
• Disease-modifying therapy with hydroxyurea.
• Pain management, including NSAIDs, paracetamol, opioids and transfusion therapy.
• Acute chest syndrome (ACS) management with antibiotics and blood transfusion.
• Stroke prevention and management, including transcranial Doppler (TCD) screening and exchange transfusion.
• Screening for SCD-related complications, including echocardiography and pulmonary function testing.

The guidance is intended for policy-makers, programme managers, paediatricians, haematologists, nurses, midwives, primary-care providers and frontline health workers at all levels of the health system. It is designed for feasibility, affordability, equity and contextual adaptation, with explicit attention to implementation in resource-limited settings.

The recommendations form part of the evidence base for updating WHO child health tools and will be integrated into the WHO Pocket Book of Hospital Care for Children and the Integrated Management of Childhood Illness (IMCI) package, supporting implementation at primary care and first-referral hospital levels. They also strengthen integration of SCD into national noncommunicable disease platforms, including WHO PEN and PEN-Plus.