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7 Points of Hemophilia: A Royal Disease, Rare Disorder

Hemophilia: A Royal Disease Hemophilia,

According to a cautious estimate, about 20,000 children are infected in Pakistan.

Hemophilia: A Royal Disease Hemophilia

Hemophilia: A Royal Disease hemophilia… Hemophilia was regarded as “Royal Disease.” It was because the Hemophilia gene was passed to the ruling families of Russia, Spain, and Germany by Queen Victoria, who became Queen of England in 1837.

Spontaneous mutation triggered the Queen Victoria hemophilia gene. Outspread this disease through two of her five daughters of Britain’s Queen Victoria.

Hemophilia

Hemophilia is a hereditary disease, which can be called simply bleeding disease.

Our body has the ability to make blood clot naturally, but the blood clotting particles in this disease are reduced to such an extent that when an individual receives an injury or wound, it takes a long time to stop or clot, and in this case, if excessive bleeding takes place, the chances of death are increased.

Definition:

Hemophilia is a rare genetic disorder that impairs the process of blood clotting.  

Types:

There is three types of haemophilia worldwide

Haemophilia A, haemophilia B, Haemophilia C

These types depend on the level of factors which involve in the process of blood clotting.

The most common form of hemophilia is hemophilia A, caused by factor VIII deficiency and is called classical Haemophilia

Hemophilia B, also known as Christmas disease, is due to a factor IX deficiency.

Hemophilia C is a moderate form of the disorder caused by factor XI deficiency.

Individuals with this unusual form of hemophilia also have no spontaneous bleeding. Blue tongue typically occurs after a wound or surgery.

Symptoms:

Signs and symptoms of hemophilia vary, depending on your level of clotting factors.

if you have a mild deficiency of clotting factor in blood then you may bleed only after surgery or trauma.

If you have a severe deficiency in blood clotting factor then you have no reason to bleed. This type is also called spontaneous bleeding.

Following signs in Spontaneous bleeding are included:

Excessive bleeding

Frequent nosebleeds

Bleeding gums

Pain in the joints

Tight joints

Deep bruises

Large, unexplained bruises

Blood in the urine

Blood in the stool

Causes:

A “coagulatory cascade” mechanism in your body usually prevents bleeding. Blood platelets coagulate or accumulate to form a clot at the site of the wound.

Then the body’s coagulation factors function together to make the wound more permanent.

A low volume of or lack of these coagulation factors triggers bleeding.

It is due to a gene mutation that determines how factors VIII, IX or XI are formed. The X chromosome contains these genes, which make hemophilia an X-linked recessive disorder.

Diagnosed:

Hemophilia is diagnosed by a blood test. A small sample of blood will be collected and measure the amount of Plasma coagulation factor. The sample is then listed so that the severity of the factor deficiency is determined:

Mild hemophilia: Between 5% and 40 % clotting factor in the plasma.

Moderate hemophilia: Between 1% and 5% clotting factor in the plasma.

Severe hemophilia: Less than 1% clotting factor in the plasma.

Complications Associated:

The complications include:

Deep internal bleeding

Joint damage from repetitive bleeding

Neurological symptoms from bleeding within the brain

In fact, by receiving donor blood, you are at elevated risk of other infections including hepatitis.

Hemophilia Cure:

No treatment for hemophilia actually exists. 

Doctor can use a prescription hormone to treat hemophilia A. The hormone is called desmopressin, and can be injected in your vein as an injection. This stimulate the factors.

doctor can treat hemophilia B, by infusing your blood with donor coagulation factors called “recombinant clotting factors.”

Hemophilia C can be treated by plasma infusion by the doctor. The infusion prevents excessive bleeding. Hemophilia C’s deficient factors are only available in Europe as drugs/ medications.

Through a treatment known as gene therapy, there is still great hope.

Hemophilia has been treated in a limited number of hemophilia people with liver transplants. The clotting factor is developed in the liver.

Worldwide, the number of hemophilia patients is about four million.

Eight out of 10 hemophiliacs have hemophilia A, according to the National Heart Lung and Blood Institute (NHLBI) Reliable Source.

Data from human health and treatment organizations and various organizations show that in the largest city of Karachi alone, about 3,000 children are affected by the disease.

However, hundreds of patients are not registered at the government level, which is aggravating problems and such patients do not have the necessary and proper attention, medical help and treatment facilities.

Report on Hemophilia: A Royal Disease Hemophilia

What do you think?

Written by Hamid Aziz

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