Sickle Cell Disease

What is Sickle Cell Disease?

Sickle cell disease (SCD) is a group of inherited red blood cell disorders caused by a mutation in the hemoglobin gene. Normal red blood cells are round and flexible, allowing them to move easily through blood vessels. In SCD, red blood cells become crescent or sickle-shaped, making them rigid and sticky. These abnormal cells can block small blood vessels, reducing oxygen delivery and causing episodes of severe pain known as vaso-occlusive crises. In the UAE and Eastern Province of Saudi Arabia, sickle cell trait prevalence is estimated at 2-6% among nationals, making it a significant public health concern alongside thalassemia.

Symptoms

• ●Episodes of severe pain (pain crises)
• ●Chronic fatigue and anemia
• ●Swelling of hands and feet
• ●Frequent infections
• ●Delayed growth in children
• ●Vision problems
• ●Jaundice (yellowing of skin and eyes)

What Causes Sickle Cell Disease?

Sickle cell disease is caused by a mutation in the HBB gene that provides instructions for making the beta-globin subunit of hemoglobin. This mutation produces an abnormal hemoglobin called hemoglobin S (HbS). It is inherited in an autosomal recessive pattern; a child must receive the sickle cell gene from both parents to have the disease.

Risk Factors

• ⚠Both parents carrying the sickle cell trait
• ⚠African, Middle Eastern, Mediterranean, or Indian ancestry
• ⚠Consanguineous marriages
• ⚠Family history of sickle cell disease or trait

How to Prevent Sickle Cell Disease

Premarital and newborn screening programs in the UAE are critical for early detection. Genetic counseling for carrier couples helps in family planning decisions. Staying well-hydrated, avoiding extreme temperatures, and preventing infections can reduce the frequency of pain crises.

When to Get Tested

Newborn screening is standard in the UAE. Adults with unexplained anemia, recurrent pain episodes, or family history of sickle cell disease should be tested with a CBC and hemoglobin electrophoresis.

References & Sources

• [1]
  WHO — Sickle Cell DiseaseAccessed: 2026-02-24
• [2]
  Mayo Clinic — Sickle Cell AnemiaAccessed: 2026-02-24
• [3]
  CDC — Sickle Cell DiseaseAccessed: 2026-02-24