Sickle cell disease (SCD) is one of the most common serious inherited conditions in the world. The majority of affected babies are born in sub-Saharan Africa, with Nigeria having the largest number. Without care, many children with SCD die in early childhood, often from infections or severe anaemia. With simple, proven interventions, most can survive to adulthood and live full lives. Nurses play a central role in that long-term care.
For management of painful crises, see our sickle cell crisis guide. This article focuses on keeping children well.
1. Early diagnosis
- Newborn screening allows care to begin before complications appear. Many high-income countries screen all newborns; several African countries are expanding programmes. Point-of-care tests make early diagnosis more feasible.
- Encourage genotype testing for couples. See premarital health checks.
2. Preventing infection
Children with SCD have reduced spleen function, making them vulnerable to serious infections, especially pneumococcus.
- Penicillin prophylaxis (oral penicillin V daily) from early infancy, typically from around 2–3 months to at least 5 years, as per national guidelines.
- Vaccinations: complete routine immunisations, including pneumococcal conjugate, Hib and others; additional pneumococcal and meningococcal vaccines may be recommended.
- Malaria prevention in endemic areas: bed nets and chemoprophylaxis as recommended. Malaria can trigger crises and severe anaemia.
- Folic acid is commonly given, according to local practice.
3. Hydroxyurea
Hydroxyurea (hydroxycarbamide) is a daily oral medicine that increases fetal haemoglobin, reducing pain crises, acute chest syndrome, transfusions and hospital admissions. Studies in sub-Saharan Africa have shown it is safe and beneficial, including in malaria-endemic settings. Many guidelines now recommend offering it to children from around 9 months of age.
- Needs regular blood count monitoring for low white cells or platelets.
- Dose is adjusted by weight and response.
- Teach families the importance of daily adherence and keeping clinic appointments.
- Access and cost remain challenges in many countries; advocate for affordable supply.
4. Stroke prevention
Children with SCD (especially HbSS) have a high risk of stroke. Transcranial Doppler (TCD) ultrasound screening, typically yearly from age 2 to 16, identifies children at high risk who benefit from treatment (regular transfusions or hydroxyurea). Teach families stroke warning signs: sudden weakness, facial droop, speech difficulty, seizures. See BE FAST.
5. A fever plan
Fever (38.5°C or above, or as per local threshold) in a child with SCD is an emergency. Families should know to seek care immediately, day or night. Delays can be fatal due to sepsis.
6. Teach families the danger signs
- Fever.
- Severe pain not relieved by home measures.
- Breathing difficulty or chest pain (acute chest syndrome).
- Sudden pallor, weakness, or a swollen tummy (splenic sequestration: teach parents to feel for the spleen).
- Neurological symptoms.
- Painful erection lasting more than 2 hours (priapism).
7. Daily living
- Plenty of fluids; avoid dehydration and extreme cold.
- Balanced nutrition and growth monitoring.
- School support: access to water and toilets, rest, catching up after absences.
Families may face stigma, blame (especially mothers), financial strain and fear. Support groups and accurate information help. Prepare adolescents to transition to adult services and manage their own care.
Based on WHO guidance on sickle cell disease, American Society of Hematology guidelines, NICE and national guidelines, and evidence from African hydroxyurea trials. Educational content: follow national guidelines.