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Hemophilia is a rare inherited blood disorder in which blood is unable to clot due to which the patient suffers profuse bleeding after an injury or surgery.
The disease is more prominent in males. Normally, when a person gets an injury or a cut, there are some clotting factors in the blood that along with platelets form a clot around the wound to stop bleeding. People with hemophilia lack few clotting factors in their blood due to which they bleed for a longer duration compared to normal people. The main clotting factors that are missing in hemophilic people are factor VIII (8) or factor IX (9).
In cases of Hemophilia, small cuts aren't much of a problem but people with a severe form should be concerned since the bleeding may also be inside your body, especially in your knees, ankles and elbows. Internal bleeding can damage the organs and end up being life-threatening in few cases.
Let's know about the causes, symptoms, diagnosis, and treatment of Hemophilia in detail.
When a person bleeds due to an internal or external injury, the body pools blood cells to form a clot to prevent further bleeding. Clotting is caused due to the proteins in the blood that work with cells known as platelets to form clots. Hemophilia occurs when a clotting factor is missing and there is continuous bleeding.
Congenital hemophilia is usually inherited, meaning a person is born with the disorder and in this type the clotting factor is low.
Acquired hemophilia is a type that develops after birth in people with no family history of the disorder. Acquired hemophilia occurs when a person's immune system attacks clotting factor 8 or 9 in the blood.
According to the National Organization for Rare Disorders, signs and symptoms of hemophilia include:
Bruising
hematomas, bleeding into the muscle or soft tissues
Bleeding from the mouth and gums
Bleeding after a circumcision
Bloody stool
Blood in urine
Frequent nose bleeds
Bleeding after vaccinations or other injections
Bleeding into the joints
Bleeding following dental or other surgical procedures or injury
Symptoms in people suffering from Hemophilia will vary from person to person.
According to doctors of Mayo Clinic, diagnosis of hemophilia involves:
Physical examination of symptoms
A clinical evaluation
Reviewing medical history
Blood tests
Blood tests can provide information regarding the duration f blood clots, levels of clotting factors, and clotting factors that doctors cannot detect physically. Blood tests can also help identify the type of hemophilia and its severity.
According to MedicalNewsToday, there is no cure for hemophilia but doctors can successfully treat the disease. Major treatment option includes replacement of missing protein to prevent complications which tackles replacement of clotting factors.
Doctors also consider recombinant clotting factors as their first treatment choice since this treatment option reduces the risk of transmitting infections through human blood. Moreover, modern screening techniques have reduced the risk of disease transmission from human samples.
The two main forms of replacement therapy are:
Prophylactic therapy for patients who require regular replacement therapy to prevent bleeding. This is a treatment recommended for severe forms of Hemophilia.
Demand therapy is recommended to stop the bleeding on-demand and is perfect for people with mild hemophilia. It is given by doctors only after bleeding begins and remains uncontrollable.
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