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June 19th is world sickle cell day!

Today June 19th is the day that we create awareness on sickle cell disease. Majority of people do not really understand what sickle cell disease is. Sickle cell disease affects people of all races and continents. 

What is sickle cell disease(SCD)?

Sickle cell disease refers to a group of inherited red blood cell disorders. Our red blood cells are responsible for carrying oxygenated blood to our organs, tissues and cells. Those who suffer from sickle cell disease therefore have an abnormal hemoglobin, referred to as hemoglobin S

Sickle cell anemia is the most common form of sickle cell disease. It occurs when an individual inherits two hemoglobin S genes (one from each parent).

Normal Red blood cells Versus Sickle red blood cells

The image below, depicts what happens in the blood vessel of a person suffering from sickle cell disease.

Image source:National Heart, Lung and Blood Institute

Figure A shows normal red blood cells flowing freely in a blood vessel. The inset image shows a cross-section of a normal red blood cell with normal hemoglobin. Figure B shows abnormal, sickled red blood cells blocking blood flow in a blood vessel. The inset image shows a cross-section of a sickle cell with abnormal (sickle) hemoglobin forming abnormal stiff rods

How does it feel to have sickle cell Disease?

Those who suffer from sickle cell anemia go through a lot of challenges. However, with our support and love, they can make it in life up to an old age. 

1) Our normal red blood cells live for about 120 days before dying but the red blood cell of a SCD individual lives for only 10-20 days. What does this mean? It means that those suffering from sickle cell disease will pay frequent visits to the hospital for possible blood transfusion so that they can have more red blood cells to keep up with oxygen supply to the body.

2) They are likely to suffer from anemiaAnemia is a condition where the level of hemoglobin in blood is lower than normal. Our bone marrow is responsible for the production of red blood cells. The red blood cells of those suffering from sickle cell is very few in number resulting in a decrease in the number of red blood cells, causing anemia. Therefore, they are always looking weak and tired as a result of anemia.

 
3) This constant shortage of oxygenated blood over time can cause failure in the following organs: 

• Brain

• Skin

• Bones

• Joints

• Kidneys

• Penis

• Heart

• liver

• lungs

• Eyes

4) Painful crisesThis occurs suddenly and without any warning. The person feels pain all over his body. Painful crises can cause them to stay out of school or work. Children may be free from pain in between the painful crises but some adults/adolescents may have ongoing pain. 

5) Swelling at the hands and feetThis is caused by a blockage of blood flow to these areas. 
Some Myths and Facts about Sickle cell disease

Some misconceptions about sickle cell disease

1)Myth:There is no cure for sickle cell disease.Fact: Bone marrow transplant can cure someone suffering from sickle cell disease. The person who donates the bone marrow is usually a sibling having the same bone marrow type as the patient. Sadly, only 1 in 10 sickle cell patients can find a matching donor.

2) Myth:Sickle Cell Disease affects only blacks or African AmericansFact: Sickle cell disease can affect anyone of any race 3) Myth: children who suffer from sickle cell disease are witches sent to drain the money of their parentsFact: Sickle cell patients are not witches. They inherited it from both parents.

4) Myth: Sickle cell patients always die before their 21st BirthdayFact: In the past, sickle cell patients used to die because of the complications associated with it. As of today, sicklers can live a normal life with proper care and medical checkups

Cure for SCDBone marrow transplant/stem cell transplant is the only cure available for SCD. Apart from this, it is only managed. 

Management of SCD

1) Eating a healthy Diet: this will supply our bone marrow with the necessary folic acid and vitamins to manufacture new red blood cells.
2) Blood Transfusion: This is done when there is severe anemia
3)Folic acid supplements: This will add to the folic acid being consumed through fruits and vegetables.
4)Drinking enough water: This will help keep the individual hydrated and prevent a crisis from occurring. Adults should drink about 8 glasses of water daily.
5) Emotional support from the community: Sickle cell patients should avoid stress because this can trigger painful crises. If your class mate is suffering from sickle cell disease and if out of school for a few days or weeks, help her catch up by helping with the lessons she missed. 

6) Taking some pain medications like Ibuprofen

PreventionIn order to prevent this condition, always do your hemoglobin electrophoresis test before marriage. This is because if both parents are carriers then there is a 25% chance that they will deliver a sickle cell patient. Remember that this 25% chance is for every pregnancy and all your children may suffer from sickle cell disease as well as they may not. 

Sources:

Mayo Clinic: Sickle Cell AnemiaNational Heart, Lung and Blood Institute: Sickle Cell DiseaseNHS Choices: Sickle Cell DiseaseSickle Cell Association of ontario

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