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World Hemophilia Day 2023 | Care, Type, Definition

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16 Apr, 2023

 World Hemophilia Day

World Hemophilia Day
World Hemophilia Day(Getty)

On April 17th each year, World Hemophilia Day is observed with the goal of increasing awareness about hemophilia and other bleeding disorders. Hemophilia, a genetic disorder, can hinder the blood's ability to clot, resulting in prolonged bleeding and spontaneous bleeding in severe cases.


In 1989, the World Federation of Hemophilia (WFH) first celebrated this day to pay tribute to WFH founder Frank Schnabel on his birthday. Schnabel was instrumental in drawing attention to the disease and establishing institutions and clinics to aid those affected by it.


The WFH employs World Hemophilia Day to inform individuals about the importance of early detection and access to care. They also lobby for improved treatment choices and assistance for those with hemophilia and other bleeding disorders. The day provides a chance to acknowledge the obstacles faced by those with these diseases and to demonstrate support for the hemophilia community.


What is Hemophilia


Hemophilia is an inherited condition that impairs the blood's clotting ability, causing prolonged bleeding and, in severe cases, spontaneous bleeding. Clotting factors, which are essential for the development of blood clots, are lacking in this condition, resulting in blood not clotting effectively.

World Hemophilia Day
World Hemophilia Day(Getty)

There are two primary forms of hemophilia, hemophilia A and hemophilia B, each caused by a deficiency of clotting factor VIII and clotting factor IX, respectively. Hemophilia is a rare disease, and it predominantly affects males since the gene responsible for the condition is located on the X chromosome, making it an X-linked recessive disorder.


People with hemophilia can experience bleeding into their muscles and joints, leading to discomfort, swelling, and decreased mobility. They are also at a higher risk of bleeding into vital organs, which can be life-threatening. A diagnosis of hemophilia is typically confirmed by blood tests that measure clotting factor levels. The standard course of treatment for hemophilia is regular infusions of the missing clotting factor. With proper treatment and care, people with hemophilia can live healthy and satisfying lives.


Hemophilia care

Hemophilia care involves managing and treating the bleeding episodes and other complications associated with the condition. Here are some essential aspects of hemophilia care:

World Hemophilia society
World Hemophilia society(Getty)

Early diagnosis: Early diagnosis of hemophilia is crucial in preventing severe bleeding episodes and complications. Diagnostic tests such as coagulation factor assays and genetic testing can determine the type and severity of hemophilia.


Regular monitoring: Regular monitoring of clotting factor levels and clinical symptoms is important to detect bleeding episodes early and prevent complications.


Treatment: The primary treatment for hemophilia is factor replacement therapy, which involves infusing clotting factor concentrates into the bloodstream. In some cases, other treatments such as desmopressin, gene therapy, and bypassing agents may be used.


Prophylaxis: Prophylaxis involves regular infusions of clotting factor concentrates to prevent bleeding episodes. This is typically recommended for people with severe hemophilia.


Management of bleeding episodes: Prompt management of bleeding episodes is essential to prevent complications. Treatment may include factor replacement therapy, rest, immobilization, and physical therapy.


Management of joint and muscle bleeding: Joint and muscle bleeding are common complications of hemophilia. Proper management involves early diagnosis, treatment, and rehabilitation to prevent permanent damage.


Comprehensive care: Comprehensive hemophilia care involves a multidisciplinary team approach, including hematologists, physiotherapists, social workers, and other healthcare professionals, to address the medical, social, and emotional needs of people with hemophilia and their families.



Hemophilia type

There are two primary types of hemophilia: hemophilia A and hemophilia B.


Hemophilia A: Also known as classic hemophilia, hemophilia A is caused by a deficiency in clotting factor VIII, which is essential for blood clotting. Hemophilia A is the most common type of hemophilia, accounting for about 80% of all cases.


Hemophilia B: Also known as Christmas disease, hemophilia B is caused by a deficiency in clotting factor IX, which is also necessary for blood clotting. Hemophilia B is less common than hemophilia A, accounting for about 20% of all cases.


Both types of hemophilia are inherited in an X-linked recessive pattern, which means that the genetic mutation that causes the condition is located on the X chromosome. Because males only have one X chromosome, they are more likely to develop hemophilia if they inherit the mutation. Females can also carry the gene mutation and pass it on to their children, but they are typically not affected by the condition themselves. In rare cases, hemophilia can also occur spontaneously due to a new genetic mutation.



Hemophilia A


Hemophilia A is a type of hemophilia caused by a deficiency in clotting factor VIII, which is essential for blood clotting. Hemophilia A is the most common type of hemophilia, accounting for about 80% of all cases.

Hemophilia A is an X-linked recessive disorder, which means that the genetic mutation that causes the condition is located on the X chromosome. Males who inherit the mutation are more likely to develop hemophilia A, as they only have one X chromosome. Females can also carry the gene mutation and pass it on to their children, but they are typically not affected by the condition themselves.

People with hemophilia A have a reduced ability to form blood clots, which can result in prolonged bleeding episodes, spontaneous bleeding, and internal bleeding. Bleeding can occur anywhere in the body, but it commonly affects the joints and muscles, leading to pain, swelling, and limited mobility.

The treatment for hemophilia A involves regular infusions of clotting factor VIII concentrates to replace the missing clotting factor and prevent bleeding episodes. Prophylactic treatment, which involves regular infusions to prevent bleeding episodes, is also recommended for people with severe hemophilia A. Other treatments, such as desmopressin, gene therapy, and bypassing agents, may also be used depending on the severity of the condition.


Hemophilia B

Hemophilia B is a type of hemophilia caused by a deficiency in clotting factor IX, which is also essential for blood clotting. Hemophilia B is less common than hemophilia A, accounting for about 20% of all cases.

Hemophilia B is an X-linked recessive disorder, which means that the genetic mutation that causes the condition is located on the X chromosome. Males who inherit the mutation are more likely to develop hemophilia B, as they only have one X chromosome. Females can also carry the gene mutation and pass it on to their children, but they are typically not affected by the condition themselves.

People with hemophilia B have a reduced ability to form blood clots, which can result in prolonged bleeding episodes, spontaneous bleeding, and internal bleeding. Bleeding can occur anywhere in the body, but it commonly affects the joints and muscles, leading to pain, swelling, and limited mobility.

The treatment for hemophilia B involves regular infusions of clotting factor IX concentrates to replace the missing clotting factor and prevent bleeding episodes. Prophylactic treatment, which involves regular infusions to prevent bleeding episodes, is also recommended for people with severe hemophilia B. Other treatments, such as gene therapy and bypassing agents, may also be used depending on the severity of the condition.
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