Hemophilia B

What Is Hemophilia?

There are several bleeding disorders like hemophilia, purpura, and Von Willebrand disease. There are a total of 13 clotting factors that are identified in the blood. Hemophilia refers to an inherited blood disorder. Inheritance refers to the obtaining of a condition from their parents. Males are more commonly affected than females. But, the problem with the feminine gender is that they are known to be the carriers. The carriers of the disease are known to transmit a condition from one generation to another. Depending upon the deficient clotting factors, hemophilia is classified into three types. They are:

• Hemophilia A.

• Hemophilia B.

• Hemophilia C.

What Is the Epidemiology of Hemophilia?

Hemophilia is known to affect one out of 5,000 live births. People of all races are known to be affected. Reports from the Centers for Disease Control and Prevention say that there are approximately 20,000 people in the United States of America with hemophilia. Hemophilia A is four times more prevalent than hemophilia B.

What Is Hemophilia B?

Hemophilia B is also called Christmas disease or factor IX deficiency. It was first recognized by Stephan Christmas. This disease is named after him. Among the types of hemophilia, hemophilia A and hemophilia B are the common types. Hemophilia A is more severe than hemophilia B. In hemophilia B, factor IX is an important protein that will help in a blood clot. It is a condition in which the child suffers severe bleeding. The blood has not clotted after any injury or fall. This will make the person suffer severe blood loss. Parents of children with hemophilia should be very careful because a small cut or bruises can result in bleeding.

What Are the Causes of Hemophilia B?

Genetic reasons are the main cause of hemophilia B. There are two chromosomes called X and Y chromosomes. They are referred to as the sex chromosomes. The gene that is responsible for causing hemophilia is found on this X chromosome. Females are known to get two X chromosomes. One is known to source from their father and the other from their mother. In males, only the X chromosome is obtained from the mother, whereas the Y chromosome is obtained from their father. With this, we can identify that if a mother who is having hemophilia transmits the X chromosome, then the son who inherited it from the mother will have hemophilia.

What Are the Symptoms of Hemophilia B?

Patients who are affected by hemophilia B suffer the following problems:

• Severe loss of blood from minor cuts.

• Passing of blood in the urine or stools.

• Large bruises.

• A bleeding from the site of injury that might continue to bleed even after stopping of the blood.

• Bleeding of the nose for no proper reason.

• Dental procedures can be crucial for the patient as the procedures like extraction can cause bleeding that could never stop.

• If the child is suffering from joint or muscle bleeding, it might cause severe pain to the patent, especially when they move from one place to another. It might be accompanied by swelling, and the warmth feeling will be seen on touching.

If the patient experiences a minor trauma in the head, then you should seek immediate medical attention. The following signs should be checked in the patient:

• Headache.

• Problems while walking.

• Bleeding in the brain.

• Stiffness of the neck.

• Neck pain.

• Vomiting.

• Sudden weakness.

• Sleepiness.

Depending on the severity of the symptoms, hemophilia B is categorized as:

• Mild Hemophilia B: In mild hemophilia B, the time taken for the expression of symptoms is huge. The first appearance of symptoms might take up to adulthood. Women who are known to suffer from mild hemophilia B have heavy bleeding during their menstrual cycle. They might also experience hemorrhage after childbirth.

• Moderate Hemophilia B: In moderate hemophilia B, bleeding will be seen right after the injury. If bleeding occurs without any proper cause, then it is referred to as episodes of spontaneous bleeding.

• Severe Hemophilia B: Patients with severe hemophilia B are the people with the highest risk. They might have frequent bleeding from the muscles and joints.

How Is Hemophilia B Diagnosed?

The doctor will request the following test to identify the deficiency of the clotting factors:

• Complete Blood Count (CBC): Complete blood count gives the evaluation of circulating cells in the blood. It might include white blood cells (WBC), red blood cells (RBC), and platelets.

• Factor IX Test: This test will determine the quantity of clotting factor that is deficient in the blood.

• Fibrinogen Test: With the help of a fibrinogen test, the doctors can easily identify the body's ability to form a blood clot.

• Prothrombin Time Test: In a prothrombin time test, it is possible to identify how quickly the blood can clot. A prolonged prothrombin time will indicate the presence of bleeding disorder.

What Are the Treatment Options for Hemophilia B?

It is very difficult to treat hemophilia B. The following treatment protocols are followed to manage the symptoms of hemophilia B. They are:

• Treatment of the Wound: If you have a mild type of hemophilia B, then the doctor will recommend using Desmopressin acetate. It is a medication that should be applied to the wounds in order to stop the bleeding. If you are suffering from any internal bleeding or larger wounds, then such medications might not be helpful, and you would require emergency medical help.

• Factor IX Injections: Factor IX injections are available that are given to prevent the bleeding of blood. It is known to be derived from a human donor. It can be artificially made from the laboratory also. The artificial ones are called recombinant factors. It is considered a safer option. Blood derived factor IX can be harmful to the patient because the risk of getting HIV and hepatitis is higher. For overcoming this, many blood screening procedures should be implemented.

What Are the Precautionary Measures of Hemophilia B?

If you are suffering from a severe type of hemophilia B, then you might require a profile access treatment. Blood transfusion is recommended as a precautionary measure. This will provide a way of preventing heavy bleeding. Suppose you are going to receive a blood-derived factor or any form of blood transfusion. In that case, it is necessary for you to get vaccinated for hepatitis B. Patients with hemophilia B should avoid taking medications like Aspirin because it is known to disrupt the platelets' functioning.

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