Hemophilia – A Blood Disorder

Queen Victoria was a carrier of Hemophilia and then it was passed to her children who were royal family’s members in Russia, Germany and Spain.

Nicholas II (Russian) suffered from Hemophilia; he was a descendant of Queen Victoria.

Hemophilia

Hemophilia is a blood disorder in which blood does not clot properly.
Bleeding disorders can be due to blood vessels defect, the coagulation or the blood platelets. A patient having Hemophilia may bleed continuously for an extended time than a healthy person after a surgery or an injury.
The blood coagulation is a process that changes the blood from liquid state to the solid state and it involves many clotting factors. This mechanism produces fibrin which combines with the platelet to stop the bleeding.
When coagulation mechanism is not effective, the blood does not clot and the bleeding continues.

Patients with Hemophilia A or B have genetic defects in them that prevents the blood clotting factors.

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What is Coagulation? Or Clotting of Blood

Coagulation

Coagulation is process by which blood is clot and then heals a cut and stops the bleeding. It is also a complicated part of Hemostasis. It stops blood loss from damaged blood vessels. In Hemostasis a damaged blood vessel is blocked by a Platelet and clot of fibrin stops the bleeding and the damage is repaired.

Coagulation consists of Platelets and the Protein components.

When a lining of the blood vessel is damaged, Platelets form a membrane at the place of injury and proteins in the Blood Plasma chemically reacts to form fibrin that forms the Platelet covering.

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An Overview of Hemophilia

Hemophilia

Hemophilia is one of the famous bleeding disorders that are often well defined as an inherited coagulation disorder.

When you cut your hand, the blood that oozes out of your hand should stop within a few seconds, which is an indication of normal metabolism, owing to the presence of clotting factors in the blood to stop bleeding. This has been identified that there are lowered amounts or absence of these clotting factors in hemophilic patients. There is a difference between bleeding faster and bleeding for a longer time.

Hemophilic patients are those who bleed for a longer time. In addition, this is a real dangerous situation, if the hemophilic patients experience internal bleeding and most the time will go unnoticed until is too late.

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Some Facts about Blood

Why is blood required ?

Modern medical science is based on the availability of human blood. Blood is required by :

  1. Patients of genetic disorders like Thalassaemia, Haemophilia andHuman Blood Bank Sickle Cell Anaemia.
  2. Patients undergoing major surgeries.
  3. Cancer patients.
  4. Victims of major accidents or injuries.
  5. Burn victims.

Women in childbirth.

In the city of Mumbai alone, everyday there are more than 600 people who are in need of life-saving blood for one of the above reasons. Continue reading “Some Facts about Blood”

Haemophilia: Genetic disorder affecting blood clot formation

haemThe human body is a very complex system. A lot of people have devoted their lives to the study of the human body and the various ailments affecting the human body. The recent years have seen a lot of research on the all-important genes and how they affect the offspring. Experts showed a great interest in studying about ancient practices and how a condition passes on from the family to the offspring. Genes play a very important role in the acquisition of characteristics by the offspring.What is haemophilia?

The human body has 23 pairs of gene groups or chromosomes, out of which 22 are known as autosomes and the remaining pair (called sex chromosomes) is X-Y in males and X-X in females. Autosomes are common to both males and females. Experts have clarified that haemophilia is a submissive condition and a hereditary defect. Haemophilia is a genetic defect that affects the X chromosome. In normal people, if there is a cut on the skin, the blood clots and the wound dries up in some time. Haemophilia represents the failure of the clotting system to completely arrest the flow of blood when the skin is cut. There is no clotting of blood when a haemophilic person is wounded, hence the bleeding is continuous and the person loses a lot of blood.

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