New Delhi: Sickle cell disease (SCD) is a condition affecting the red blood cells (RBCs). It is an inherited condition characterised by abnormal haemoglobin. The highest prevalence of the disease is in India among the South Asian countries, where over 20 million patients with SCD live. On World Sickle Cell Awareness Day, let’s dive into the nitty and gritty of the disease and how it impacts the growth in children.
Types of Sickle Cell Disease:
1. Sickle Cell Trait (HbAS): Individuals inherit one normal haemoglobin gene and one sickle haemoglobin gene. They usually don’t have symptoms but can pass the gene to their children.
2. Sickle Cell Anemia (HbSS): The most severe form, where both inherited genes are sickle haemoglobin, leading to significant health problems.
3. Other Forms: These occur when a sickle gene is inherited with another abnormal gene like beta-thalassemia or haemoglobin E, causing conditions like HbS beta-thalassemia and HbSE disease.
Common Effects of SCD:
1. Chronic Anemia: Sickle cells have a shorter lifespan than normal red blood cells. Hence, there is a constant shortage in their total number, resulting in chronic anaemia and reduced oxygen in circulation. This causes weakness, fatigue, and difficulty breathing.
2. Pain Crises: Sickle-shaped cells can block small blood vessels, causing severe pain, especially in bones, chest, and joints. These episodes, known as vaso-occlusive crises, can also damage organs over time.
3. Chronic Inflammation and Hemolytic Events: The haemoglobin gene mutations in SCD promote chronic inflammation and hemolytic events, eventually leading to vaso-occlusion. Vaso-occlusion is identified as one of the major causes of pain crises in SCD.
4. Organ Damage: Repeated blockages can harm organs like the spleen, kidneys, liver, lungs, and brain, potentially causing strokes, lung problems, and more.
5. Increased Infection Risk: Damaged spleens in SCD patients weaken the immune system, making them more prone to infections.
6. Splenic Sequestration: Sickle cells can clog the spleen, causing acute splenomegaly (enlarged spleen) and a rapid drop in haemoglobin. This can be life-threatening and requires immediate treatment.
7. Delayed Growth: Children with SCD often grow slower and may have delayed puberty and cognitive challenges due to chronic anaemia and complications.
8. Blood Clots: The abnormal shape of the cells increases the risk of clotting, which may in turn lead to life-threatening conditions like deep vein thrombosis and pulmonary embolism.
9. Hyper-Viscosity Syndrome (HVS): The polymerization of hemoglobin-S and abnormally shaped RBCs in sickle cell disease contribute to hyper-viscosity of the blood. Highly viscous blood cannot flow freely within the arteries, potentially increasing the risk of fatal conditions like stroke and vasoconstriction.
It is essential to understand that the severity of SCD varies among individuals. While some may experience mild symptoms, others might face frequent and severe complications. Effective management and early intervention are crucial.
Sickle cell disease (SCD) is a condition affecting the red blood cells (RBCs). It is an inherited condition characterised by abnormal haemoglobin. On World Sickle Cell Awareness Day, expert shares the impact of disease on the growth and development in children Health Conditions Health News: Latest News from Health Care, Mental Health, Weight Loss, Disease, Nutrition, Healthcare
