The Science
Behind the Story
What is Sickle Cell Disease?
There’s more to it than sickle-shaped cells.
Sickle cell disease is a blood disorder that you are born with that affects the shape of your red blood cells. Sickled red blood cells do not live as long as healthy ones and your body has a hard time replacing them. That is why you may experience anemia. But this is just part of the story. Sickle Cell disease is a rare inherited disease that causes severe, chronic anemia. However, this is NOT caused by low iron.
Sickle Cell Disease
What it is
Red blood cells repeatedly sickle (forming rigid chains inside that distort shape). When they reach areas high in oxygen, they plump backup. This process causes Sickled RBCs to live 10 days instead of 120 days. Bone marrow can NOT keep up with CONSTANT demand for new red blood cells.
Causes
Sickle Cell is a genetic disorder, inherited from BOTH parents. There is no other cause.
Treatment
None. This type of anemia is chronic and lifelong. Iron supplementation can lead to iron overload, which can be fatal.
Iron Deficiency Anemia
What it is
Low ferrite levels and low iron-binding capacity leads to an overall depletion of iron. Iron is essential for blood production.
Causes
Poor diet, limited access to healthy food, unregulated vegetarian or vegan diet, excessive intake of cow's milk.
Treatment
Iron supplements, increased red meat and green leafy vegetables, Blood transfusions in severe cases.
Anemia Without Iron Deficiency
What Happens Inside the Body?
1.
Your blood vessels can become damaged, causing blood cells and blood vessels themselves to become sticky.
Stickiness
The shape and stickiness of the blood cells in sickle cell disease make them more likely to get stuck to one another, forming clusters in the bloodstream.
Clusters
2.
3.
These clusters can block blood and oxygen flow.
Blockages
When blood cell clusters get big enough, they can cause a full blockage that can lead to a pain crisis—possibly causing severe, debilitating pain. Over time, this may lead to other chronic health issues, including damage to your organs.
Pain Crises
4.
Categories 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. This forms hemoglobin known as Hb SS. As the most severe form of SCD, individuals with this form also experience the worst symptoms at a higher rate.
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. However, sometimes the symptoms of beta zero thalassemia are more severe. It is associated with a poorer prognosis.
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. Individuals with Hb SC have similar symptoms to individuals with Hb SS. However, the anemia is less severe.
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. If inherited with the Hb S gene, you will have hemoglobin S beta thalassemia. Symptoms are not as severe.
Hemoglobin SD, hemoglobin SE, and hemoglobin SO
These types of sickle cell disease are more rare and usually don’t have severe symptoms.
Sickle Cell Trait
People who only inherit a mutated gene (hemoglobin S) from one parent are said to have sickle cell trait. They may have no symptoms or reduced symptoms.
Who is at Risk of Sickle Cell Disease?
Children are only at risk for sickle cell disease if both parents carry the sickle cell trait. A blood test called a hemoglobin electrophoresis can also determine which type you might carry.
People from regions that have endemic malaria are more likely to be carriers.
This includes people from:
Africa
India
The
Mediterranean
Saudia
Arabia
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