Sickle cell disease (SCD) is an inherited blood disorder in which red blood cells become rigid and sickle-shaped, blocking small blood vessels. WHO estimates that most babies born with SCD are born in sub-Saharan Africa, and Nigeria has the largest number of people with sickle cell disease in the world. People of African, Middle Eastern, Indian and Caribbean heritage are also affected in the UK, US and elsewhere. Nurses care for people with SCD in every setting, and the way they are treated in a crisis shapes whether they trust health services.
Types of crisis
- Acute painful (vaso-occlusive) crisis: the most common. Severe pain in bones, joints, back, chest or abdomen. Triggers include infection, dehydration, cold, stress, exertion, and low oxygen.
- Acute chest syndrome: a leading cause of death; see below.
- Splenic sequestration (mainly young children): blood pools in the spleen, causing sudden anaemia, an enlarging spleen and shock.
- Aplastic crisis: often after parvovirus infection; the bone marrow temporarily stops making red cells.
- Stroke: particularly in children.
- Priapism: a painful, prolonged erection: a urological emergency.
Managing a painful crisis
Fast pain relief
NICE guidance for the UK says people presenting with an acute painful crisis should be offered analgesia within 30 minutes of arrival, and their pain reassessed every 30 minutes until controlled. Sickle cell pain is among the most severe pain known. Believe the patient’s pain score.
- Strong opioids (e.g. morphine) are often needed, as prescribed, with paracetamol and NSAIDs where appropriate.
- Many patients have an individual care plan stating what works for them. Check it.
- Monitor for opioid side effects: sedation, slowed breathing (respiratory rate, SpO₂), itching, constipation, nausea.
- Don’t label patients as “drug-seeking”. This is one of the most common complaints from people with SCD and causes dangerous delays.
Supportive care
- Hydration: oral fluids, or IV fluids as prescribed. Avoid overload.
- Warmth: keep the patient warm; avoid cold packs.
- Oxygen only if SpO₂ is below target.
- Incentive spirometry or deep breathing every 2 hours while awake helps prevent acute chest syndrome.
- Look for and treat the trigger, especially infection and malaria in endemic areas.
- Observations: temperature, pulse, BP, RR, SpO₂, pain and sedation scores.
Red flags: escalate urgently
| Sign | Possible problem |
|---|---|
| Chest pain, cough, fever, breathlessness, falling SpO₂, new chest X-ray changes | Acute chest syndrome |
| Weakness of one side, speech problems, seizures, severe headache, reduced consciousness | Stroke |
| Fever 38°C or higher | Sepsis: people with SCD have a damaged spleen and are highly vulnerable to infection |
| Pallor, enlarging spleen, rapid heart rate, low BP (especially in a child) | Splenic sequestration |
| Sudden severe tiredness and pallor without jaundice | Aplastic crisis |
| Prolonged painful erection (over 2–4 hours) | Priapism |
Long-term care and prevention
- Newborn screening allows early diagnosis and care.
- Penicillin prophylaxis and vaccines (including pneumococcal) to prevent infections, especially in young children.
- Malaria prevention: bed nets and chemoprophylaxis where recommended.
- Folic acid supplements.
- Hydroxyurea (hydroxycarbamide) reduces crises and acute chest syndrome and improves survival. It is increasingly available in Africa, and WHO includes it on its essential medicines list.
- Transcranial Doppler screening in children to detect stroke risk, where available.
- Teach patients and families to recognise early signs of a crisis, keep well hydrated and warm, and seek help early for fever.
Fighting stigma
People with SCD often face stigma, discrimination and doubts about their pain. In some communities, children with SCD are wrongly believed to be cursed. Nurses can challenge these myths, support families with counselling (including genotype testing before marriage, a common practice in Nigeria), and advocate for respectful, timely care.
Related: Blood transfusion · Severe malaria · Sepsis Six
Based on WHO sickle cell disease information, NICE CG143 (Sickle cell disease: managing acute painful episodes in hospital) and the NHS Sickle Cell Society standards. Educational content: follow individual care plans and local protocols.