Sickle cell anemia (sickle cell disease) is a disorder of the blood caused by an inherited abnormal hemoglobin (the oxygen-carrying protein within the red blood cells). Normally, the flexible, round red blood cells move easily through blood vessels.
In sickle cell anemia, the red blood is shaped like sickles or crescent moons. These rigid, sticky cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body.
Symptoms of Sickle Cells Anemia
Symptoms of sickle cell anemia usually show up at a young age. They may appear in babies as early as 4 months old, but generally occur around the 6-month mark.
While there are multiple types of SCD, they all have similar symptoms, which vary in severity. These include;
- Excessive fatigue or irritability, from anemia
- Fussiness, in babies
- Bedwetting, from associated kidney problem
- Jaundice, which is yellowing of the eyes and skin
- Swelling and pain in hands and feet
- Frequent infections
- Pain in the chest, back, arms, or legs
Types of Sickle Cell Disease
Hemoglobin is the protein in red blood cells that carries oxygen. It normally has two alpha chains and two beta chains. The four main types of sickle cell anemia are caused by different mutations in these genes.
- Hemoglobin SS disease – Hemoglobin SS disease is the most common type of sickle cell disease. It occurs when you inherit copies of the hemoglobin S gene from both parents.
- Hemoglobin SC disease – Hemoglobin SC disease is the second most common type of sickle cell disease. It occurs when you inherit the Hb C gene from one parent and the Hb S gene from the other.
- Hemoglobin SB+ (beta) thalassemia – Hemoglobin SB+ (beta) thalassemia affects beta globin gene production. The size of the red blood cell is reduced because less beta protein is made.
- Hemoglobin SB 0 (Beta Zero) Thalassemia – Sickle beta-zero thalassemia is the fourth type of sickle cell disease. It also involves the beta globin gene. It has similar symptoms to Hb SS anemia.
Treatments
Management of sickle cell anemia is usually aimed at avoiding pain episodes, relieving symptoms and preventing complications. Treatments might include medications and blood transfusions. For some children and teenagers, a stem cell transplant might cure the disease.
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