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Sickle Cell Disease (Children)

A genetic disorder of hemoglobin structure causing recurrent pain crises and organ damage.

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 Sickle Cell Disease (Children)?

Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy caused by a point mutation in the HBB gene that produces abnormal hemoglobin S (HbS). HbS polymerizes in low-oxygen environments, transforming red cells into rigid, sickle-shaped cells.

Sickle cells block small vessels, leading to vaso-occlusive crisis (VOC), acute chest syndrome, stroke, priapism, and organ damage. Early splenic damage (splenic sequestration and autosplenectomy) leaves children vulnerable to encapsulated bacteria (pneumococcus, meningococcus, Hib).

Hydroxyurea therapy increases fetal hemoglobin (HbF) and significantly reduces crisis frequency and acute chest syndrome. Hematopoietic stem cell transplantation is a curative option.

Symptoms

Recurrent pain crises (hand-foot, bone, chest)
Jaundice and pale appearance (anemia)
Swollen hands and feet (dactylitis — often the first sign)
Splenic enlargement or sudden shrinkage (sequestration)
High fever (bacteremia risk)
Stroke in children (hemiplegia, speech loss)

Risk Factors

Inheritance of the HbS gene from both parents
Families of African, Mediterranean, Middle Eastern, and Indian origin
Lack of screening
Cold exposure, dehydration, infection (crisis triggers)
Travel to high altitude
Stress and strenuous exercise

When to See a Doctor?

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

  • Fever ≥38.5°C (urgent — sepsis risk)
  • Onset of severe pain crisis
  • Stroke signs (facial droop, arm weakness, speech disturbance) to ER
  • Chest pain and shortness of breath (acute chest syndrome) to ER

Treatment Methods

01
Hydroxyurea: increases HbF and reduces crisis frequency
02
Penicillin prophylaxis: daily oral penicillin until age 5
03
Vaccination: pneumococcal, meningococcal, Hib vaccines are mandatory
04
Pain management: NSAID + opioid, hydration
05
Transfusion: for stroke prevention and acute chest syndrome
06
Hematopoietic stem cell transplantation: curative if a suitable donor is available

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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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.