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Sickle Cell Crisis

Author's details

  • Hakim Adeniyi Abubakre
  • Ba (Hons) International Business Management, M.Sc. Digital Marketing.

Reviewer's details

  • Dr. Khashau Eleburuike.
  • MBBS (Ilorin) MSc. Global Health Karolinska Institutet. SFAM, Socialstyrelsen.
  • Consultant in family medicine in Norrbotten County, Sweden.
  • Date Published: 2026-06-06
  • Date Updated: 2026-06-06

Sickle Cell Crisis

Key Messages

  • This condition is common in sub-Saharan Africa, and early recognition of pain, fever, breathing problems, weakness, or sudden swelling can save lives.
  • During a painful crisis, start simple measures early: drink fluids, rest, keep warm, and use pain medicines exactly as advised by a health professional.
  • Fever, chest pain, difficulty breathing, severe weakness, stroke-like symptoms, or a painful erection lasting more than 2 hours are danger signs that need urgent medical care.
  • Preventing crises is very important: stay hydrated, avoid extreme temperatures, treat infections quickly, attend clinic appointments, and take prescribed medicines such as folic acid, penicillin (if prescribed), and hydroxyurea when recommended.
  • People living with sickle cell disease can live better and longer with regular follow-up, family support, vaccination, malaria prevention where relevant, and a clear plan for home care and emergencies.
Introduction

Sickle cell disease is an inherited blood disorder. A person develops the disease when they inherit a sickle cell gene from both parents. The commonest and often most severe type is haemoglobin SS (HbSS), although other types such as HbSC and HbS beta-thalassaemia also occur. Sickle cell disease is especially common in sub-Saharan Africa, where it is a major cause of illness in children and adults. In this condition, red blood cells can become rigid and sickle-shaped, block small blood vessels, break down faster than normal, and reduce the amount of oxygen reaching body tissues.

A sickle cell crisis is a sudden worsening of the disease. The most common type is a painful vaso-occlusive crisis, but other serious complications can also happen, including acute chest syndrome, severe anaemia, splenic sequestration, stroke, infection, priapism, and dactylitis (painful swelling of the hands and feet in young children). Pain may affect the back, chest, abdomen, arms, legs, or joints. In many African settings, infections, dehydration, malaria, delayed access to care, and limited emergency services can make these complications more dangerous, so early recognition and treatment are very important.

Diagnosis is usually confirmed with laboratory testing that identifies abnormal haemoglobin. During a crisis, assessment focuses on the severity of pain, breathing difficulty, fever, anaemia, dehydration, and possible organ complications. Triggers for crisis can include infection, dehydration, malaria, very hot weather, cold exposure, stress, strenuous activity, poor sleep, and sometimes no clear trigger at all. In some areas, long travel times to hospital, medicine shortages, and stigma can delay care, so patients and families should know their warning signs and have a plan for what to do.

Discussion

Warning Signs and Symptoms

  • Sudden severe pain in the chest, abdomen, back, arms, or legs.
  • Swelling of the hands or feet, especially in babies and young children.
  • Fever, chills, or signs of infection.
  • Fatigue, weakness, dizziness, or shortness of breath.
  • Persistent headache, confusion, drowsiness, stiff neck, seizures, or fainting.
  • Slurred speech, weakness on one side of the body, or sudden difficulty seeing, walking, or talking.
  • Chest pain, cough, fast breathing, or difficulty breathing.
  • Pale palms, lips, tongue; yellow eyes; or dark urine.
  • Sudden swelling or pain in the abdomen, especially on the left side where the spleen is.
  • A painful erection lasting more than 2 hours.

What You Can Do at Home

  • Drink plenty of safe fluids, especially water or oral rehydration drinks if advised.
  • Rest and avoid strenuous activity until you feel better.
  • Keep warm but avoid overheating. A warm cloth or warm bath may help some people with pain.
  • Use pain medicines exactly as advised by your clinician. Paracetamol may help with mild pain. Ibuprofen can help some people, but it should be avoided in some patients, especially if there are kidney problems, stomach ulcers, dehydration, or a doctor has advised against it.
  • If you have been given a personalised pain plan by your clinic, follow it early when pain starts.
  • Continue prescribed regular medicines unless a clinician has told you to stop them.
  • Seek help early if pain is worsening, if you cannot drink, or if there is fever, chest pain, breathing difficulty, severe weakness, or any neurological symptom.

When to Seek Medical Attention Urgently

  • Signs of stroke: sudden weakness, numbness, facial droop, confusion, difficulty speaking, seeing, or walking.
  • Chest pain, fast breathing, difficulty breathing, or coughing with fever.
  • Fever or suspected infection, especially in a child.
  • Severe pain which is not relieved by home treatment.
  • A painful erection lasting more than 2 hours.
  • Severe headache, neck stiffness, repeated vomiting, confusion, seizures, or loss of consciousness.
  • Severe diarrhoea, vomiting, or inability to drink enough fluids.
  • Sudden tiredness, extreme weakness, fainting, or very pale appearance.
  • Yellowing of the eyes with dark urine, or sudden swelling of the abdomen.
  • If you live far from a health facility, start travelling early rather than waiting for symptoms to become worse.

Prevention and Ongoing Care

  • Drink enough fluids every day, especially during hot weather, fever, diarrhoea, or long journeys.
  • Avoid extreme heat, cold, and sudden temperature changes where possible.
  • Prevent and treat infections quickly. Keep routine vaccinations up to date.
  • In malaria-endemic areas, sleep under an insecticide-treated mosquito net and follow local advice on malaria prevention and testing.
  • Attend regular clinic reviews, even when you feel well.
  • Take prescribed medicines regularly, such as folic acid, penicillin in children where advised, and hydroxyurea if recommended by your clinician.
  • Eat a balanced diet and seek help early for weight loss or poor growth in children.
  • Avoid smoking and second-hand smoke. Alcohol can worsen dehydration in some people.
  • Have a written or remembered emergency plan that includes where to go, transport options, and the medicines you already use.
  • Seek counselling, family support, school support, or community support if stigma, stress, or low mood is affecting daily life.

Over-the-counter pain medicines can be helpful, but they are not safe for everyone. Ask a doctor, nurse, or pharmacist which medicines and doses are appropriate for you or your child, especially if there is kidney disease, stomach ulcer disease, pregnancy, dehydration, or frequent severe pain episodes.

Conclusion

Sickle cell crisis can become dangerous quickly, but early action can reduce complications and save lives. Patients and families should know the warning signs, begin safe home care early for mild pain, and seek urgent medical attention for fever, breathing problems, severe weakness, stroke-like symptoms, severe anaemia, or worsening pain. In sub-Saharan Africa, regular clinic follow-up, infection prevention, malaria prevention where needed, hydration, and a clear emergency plan are especially important. With the right information, timely treatment, and strong family and community support, people living with sickle cell disease can achieve better health and quality of life.

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References
  1. World Health Organization Regional Office for Africa. WHO SICKLE package of interventions for sickle-cell disease management: strategic guidance framework. Module 1. Brazzaville: WHO Regional Office for Africa; 2024.
  2. SPARCo. Standards of care for sickle cell disease in sub-Saharan Africa: home-based version. Accra/Dar es Salaam/Abuja: SickleInAfrica/SPARCo; 2023.
  3. Esoh K, Wonkam-Tingang E, Wonkam A. Sickle cell disease in sub-Saharan Africa: transferable strategies for prevention and care. Lancet Haematol. 2021;8(10):e744-e755.
  4. Ansong D, Osei Akoto A, Ocloo D, Ohene-Frempong K. Sickle cell disease: management options and challenges in developing countries. Mediterr J Hematol Infect Dis. 2013;5(1):e2013062.
  5. Musuka HW, Iradukunda PG, Mano O, Saramba E, Gashema P, Moyo E, et al. Evolving landscape of sickle cell anemia management in Africa: a critical review. Trop Med Infect Dis. 2024;9(12):292.
  6. Federal Ministry of Health Nigeria. National guideline for the control and management of sickle cell disease. 2nd ed. Abuja: Federal Ministry of Health; 2022.

Key Messages

  • This condition is common in sub-Saharan Africa, and early recognition of pain, fever, breathing problems, weakness, or sudden swelling can save lives.
  • During a painful crisis, start simple measures early: drink fluids, rest, keep warm, and use pain medicines exactly as advised by a health professional.
  • Fever, chest pain, difficulty breathing, severe weakness, stroke-like symptoms, or a painful erection lasting more than 2 hours are danger signs that need urgent medical care.
  • Preventing crises is very important: stay hydrated, avoid extreme temperatures, treat infections quickly, attend clinic appointments, and take prescribed medicines such as folic acid, penicillin (if prescribed), and hydroxyurea when recommended.
  • People living with sickle cell disease can live better and longer with regular follow-up, family support, vaccination, malaria prevention where relevant, and a clear plan for home care and emergencies.