SICKLE CELL ANEMIA - Cause, symptoms and complications

       Sickle cell anemia is a disorder of red blood cells. It is also called as Sickle cell disease. In this disorder, there is a decrease in the number of healthy red blood cells to carry oxygen to your body tissues.

Normally, the red blood cells are biconcave in shape. This makes them flexible to move easily through blood vessels. In sickle cell anemia, the shape of red blood cells changes from biconcave to sickle shaped or crescent moon shaped.

This change of shape makes the cells rigid and sticky and they get stuck in small blood vessels, which can slow or block blood flow and oxygen supply to various parts of the body. 

Normal RBC's in blood

CAUSE OF SICKLE CELL ANEMIA:- 

Inside each red cell, there is a hemoglobin molecule present. The work of this molecule is to transport oxygen to all parts of body through blood. This hemoglobin molecule is made up of 2 alpha chains and 2 beta chains.

Normally, On the 6th position of this beta chain is an amino acid present called Glutamate. In Sickle cell disease, glutamate is replaced from it's position by another amino acid called Valine. This substitution leads to change in normal structure of hemoglobin. This abnormal hemoglobin causes red blood cells to become rigid, sticky and sickle shaped. 

Sickle cell disease is an inherited disease. If two people carrying the gene of sickle cell disease marry each other, there is 25 % chance of baby being born with sickle cell disaese. 

Normal RBC

    

                    Sickle shaped RBC 

SYMPTOMS OF SICKLE CELL DISEASE:-

 • Anemia - 

Due to disruption in shape, Red cells become weak and die before their normal life span of 120 days. This leads to decrease in the number of Red cells and hence body cannot get proper oxygen for proper fuctioning. This creates a condition of fatigue and weakness. 

• Jaundice - 

Due to early death of of red cells, the content of red cells are expelled out and get mixed in the blood. This leads to Jaundice. 

• Splenomegally (Enlarged spleen) - 

Rigid and sickle shaped red cells are trapped in the small blood vessels of spleen. This accumulation leads to enlargement in the size of spleen. 

• Growth is retarded due to less oxygen availability to the body tissues. 

• Pain crisis - 

Due to less availability of oxygen at tissue level, occurs infarction of organs. This causes pain in the affected organ. 

COMPLICATIONS OF SICKLE CELL DISEASE:- 

Sickle cell anemia can lead to a number of complications, including:

• hand-foot syndrome - 

Also known as dactylitis. In children, painful bone crises of hands or feet, or both are extremely common. 

• Acute chest syndrome - 

This crisis involves lungs, presenting with fever, chest pain, cough and inflammation of lungs. 

• Non healing leg ulcers. 

• Multiple organ damage - 

Sickle cells that block blood flow to organs deprive the affected organs of blood and oxygen.This lack of oxygen-rich blood can damage organs, including kidneys, liver, brain and penis. 

• Loss of visual acquity and even blindness. 

• Priapism - 

Prolonged erection of penis without sexual arousal.

• Cells that are progenitors of red cells get infected by parvovirus. This causes Aplastic crisis.