Context:
World Sickle Cell Day is being observed on 19 June.
More on the news:
- In December 2008, the United Nations General Assembly designated June 19th as World Sickle Cell Day.
- It is an international awareness day commemorated to alert the global public about sickle cell disease.
- This year’s theme, “Hope Through Progress,” reflects the ongoing fight for better treatment and a cure.
Aim of Sickle Cell Day :
- Increase awareness: Educate the public about Sickle cell disease and the challenges faced by patients.
- Advocate for better care: Promote early diagnosis and access to quality healthcare.
- Celebrate progress: Recognize advancements in treatment and research, offering hope for the future.
About Sickle cell disease (SCD)
- It is a group of inherited red blood cell disorders that affects haemoglobin, the protein responsible for carrying oxygen throughout your body.
- Dr. James Herrick was the first to officially describe SCD in 1910. It is one of the most prevalent monogenic diseases, especially in low- and middle-income countries.
- India has the world’s second-largest burden of sickle cell disease, predominantly impacting socially, politically, and economically marginalized populations.
Cause and Effects:
- Sickle cell disease (SCD) is inherited from parents to children. When an individual inherits two copies of the abnormal gene, one from each parent, they develop the disease.
- Inheriting only one copy results in sickle cell trait, which does not cause SCD but can be passed on to future generations.
Unlike normal red blood cells, which are flexible and disc-shaped, sickled red blood cells are rigid and sticky. This can cause several issues:
- Blockage of Blood Flow: Sickled cells can clump together and block small blood vessels, preventing oxygen-rich blood from reaching various parts of the body.
- Breakdown of Red Blood Cells: Sickled cells break down prematurely, leading to a shortage of red blood cells (anaemia).
Symptoms:
- Severe Pain: Pain episodes due to blocked blood flow.
- Anemia: Lack of healthy red blood cells leads to fatigue and weakness.
- Infections: Spleen damage increases infection risk.
Treatment:
There is currently no cure for SCD, but treatments focus on managing symptoms and preventing complications:
- Pain Management: Medications like opioids and pain relievers are used to manage pain crises.
- Hydration: Staying hydrated helps prevent sickling of red blood cells.
- Hydroxyurea: This medication helps increase the production of fetal haemoglobin, a different type of haemoglobin that can reduce sickling.
- Blood Transfusions: Used in severe cases to replace sickled red blood cells with healthy ones.