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Paediatric Sickle Cell Crisis

Acute painful, vaso-occlusive and aplastic crises in children with sickle cell disease.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Çocuk Sağlığı ve Hastalıkları department. Book Appointment →

What is Paediatric Sickle Cell Crisis?

Sickle cell disease is a hereditary haemoglobinopathy in which haemoglobin S forms sickle shape under hypoxia and obstructs vessels.

Crises in children include vaso-occlusive (most common), splenic sequestration, aplastic and acute chest crisis types.

Triggers include infection, dehydration, cold exposure and high altitude; management requires aggressive pain control, fluid and antibiotics.

Symptoms

Severe long-bone, joint and back pain (vaso-occlusive)
Hand-foot swelling (dactylitis, in young children)
Acute splenic enlargement and pallor (sequestration)
Cough, dyspnoea and chest pain (acute chest syndrome)
Fever and signs of infection (sepsis risk)
Acute weakness and difficulty breathing (anaemia)
Stroke symptoms (hemiparesis, aphasia)

Risk Factors

Family history of sickle cell disease (autosomal recessive)
African, Mediterranean and Middle Eastern origin
Infection (especially Streptococcus pneumoniae)
Dehydration and warm weather
Cold exposure and high altitude
Stress, exercise and surgery
Functional asplenia and immunosuppression

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Emergency hospital presentation is mandatory for fever (>38.5°C), severe pain and difficulty breathing
  • Within 4 hours pain control, hydration and antibiotic must be started
  • Emergency exchange transfusion is required for stroke or acute chest syndrome
  • Long-term hydroxyurea and prophylactic penicillin are essential

Treatment Methods

01
Intravenous fluid (1.5x maintenance) and oxygen therapy
02
Aggressive pain control (morphine PCA, ketorolac)
03
Empirical broad-spectrum antibiotic (ceftriaxone)
04
Exchange transfusion (stroke, acute chest, multi-organ failure)
05
Long-term hydroxyurea and folic acid
06
Prophylactic penicillin (until age 5) and pneumococcal vaccine

Which Department to Visit?

You can visit our Çocuk Sağlığı ve Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Çocuk Sağlığı ve Hastalıkları Department

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You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.