Haemophilia

What is haemophilia?

Haemophilia is a rare genetic disorder of blood clotting. There are many different types of haemophilia, depending on their pathophysiological mechanism. People with haemophilia have fewer blood clotting factors than people in the general population, and as a result they have problems with blood clotting. As a result, they bleed for a longer period of time after injury. The degree of morbidity depends on the extent of the deficiency of clotting factors. The more severe the deficiency, the more severe the disease will be. 

How often does it occur?

According to statistical studies, there are approximately 30,000 to 33,000 people with hemophilia living in the United States. 60% of them suffer from severe hemophilia, 15% from moderate hemophilia and 25% have mild symptoms of the disease. The most common form of haemophilia is Von Willebrand's disease, which affects about 1 in 100 people in the US. Although the disease occurs equally often in men and women, women tend to suffer more because of their menstrual cycles. 

Symptoms of haemophilia

The signs and symptoms of haemophilia vary, depending on the level of clotting factors. In mild forms, the disease can cause bleeding after surgery or injury. In severe cases, spontaneous bleeding may occur without external stimuli, trauma or surgical interventions. In general, some of the most common symptoms are as follows:

• Unexplained and excessive bleeding from cuts or injuries or after surgery or dental work
• Bruises
• Unusual bleeding after vaccinations
• Pain, swelling or tightness in the joints due to haemarthrosis (bleeding within the joints)
• Blood in the urine or stool
• Nosebleeds without known cause
• In infants, unexplained irritability
• In women, prolonged menstrual cycles with increased blood loss
• Bleeding from the gums

Risk of haemophilia in people with severe form of the disease

In people with a severe form of the disease, a simple blow to the head can cause bleeding in the brain. Although this is rare, it is one of the most serious potential complications of the disease. Signs and symptoms include:

• Prolonged headache
• Direct
• Drowsiness or lethargy
• Diplopia
• Sudden weakness 
• Clumsiness in movements
• convulsions or seizures

Types of haemophilia

There are different types of haemophilia. The following two are the most common:

• Haemophilia A. This type is caused by a lack or decrease in clotting factor VIII.
• Haemophilia B. This type is caused by a lack or decrease in clotting factor IX.

Is there a cure?

Treatment of haemophilia is determined by the type of haemophilia and the clotting factors that are in short supply. The main treatment for severe haemophilia involves replacing the specific clotting factor in short supply through intravenous therapy. The treatment is administered either long-term or short-term, as appropriate. 

The Hematology Department of the Clinic treats all blood diseases and disorders, including types of haemophilia, with modern treatments and under the guidance of excellent health scientists who provide high quality nursing care to each patient.