HEMOPHILIA

Uncontrollable spontaneous hemorrhage

Hemophilia is an inherited bleeding disorder in which blood doesn't clot normally because it lacks sufficient blood-clotting proteins (clotting factors). This leads to internal and spontaneous bleeding following an injury, trauma, or surgery, mainly targeting the articulations (knees, ankles and elbows) as well as the muscles.

Small cuts usually aren't much of a problem. Most of the danger comes when vital organs as stomach, liver and brain are subject to internal bleeding.

FACTS
• Generally Hemophilia affects males only.
• Hemophilia is usually diagnosed at birth in case of genetic history. .
• It is very rare for a girl to be born with hemophilia, but it can happen if the father has hemophilia and the mother carries the gene for hemophilia.
• Hemophilia is an inherited genetic condition.
• Hemophilia isn’t curable.
• Symptoms may occur around age of 2 or in case of circumcision.

SIGNS AND SYMPTOMS
People with a mild deficiency may bleed in the case of trauma.
People with a severe deficiency may bleed for no reason.
Spontaneous bleeding can cause the following:

• Tight joints
• Deep bruises
• Bleeding gums
• Pain in the joints
• Blood in the urine
• Psycho- social problems
• Blood in the stool
• Excessive bleeding
• Frequent nosebleeds
• Irritability (in children)
• Large, unexplained bruises
• Work and social integration

TYPES OF HEMOPHILIA

Type A
• Hemophilia A is the most common type. It is caused by a deficiency of factor VIII, one of the proteins that help blood to form clots.
Type B
• Hemophilia B is caused by a deficiency of factor IX.

STATISTICS
1 out of 5000 new-born male is attained by Hemophilia A.
1 out of 15.000 to 20.000 new-born male is attained by Hemophilia B.

GENETIC CAUSES
• Hemophilia is an inherited genetic condition, it is usually passed down through families.
• According to the World Federation of Hemophilia WFH, scientific research stated that due to a new genetic mutation, 30% of hemophilia cases are not hereditary.
• A woman can be a carrier and pass the gene for hemophilia on to her children without having herself the disorder.
According to Cleveland clinic, there is a 50% chance that any of her sons will inherit the gene and will be born with hemophilia.
• There is also a 50% chance that any of her daughters will be carriers of the gene, without having hemophilia themselves.

DIAGNOSIS
Hemophilia is diagnosed through a blood test.
Mild hemophilia is indicated by a clotting factor in the plasma that’s between 5 and 40%.
Moderate hemophilia is indicated by a clotting factor in the plasma that’s between 1 and 5%.
Severe hemophilia is indicated by a clotting factor in the plasma of less than 1%.

TREATMENTS
Therapies to stop bleeding depend on the type of hemophilia
• Receiving clotting factors replacement therapy.
• Regular infusions of DDAVP or clotting factor
- Instantly and within a 2 hours delays following the hemorrhage
- Regular treatment on weekly bases, 2 or 3 times a week, in order to limit the hemorrhage risks.
• Physical therapy, treatment of joints and muscles.
• Treatment for other problems associated with hemophilia
• Vaccinations.

Lebanese Association for Hemophilia (LAH)
Established in 1992 and recognized by decree 106/AD, LAH is a member of the World Federation of Hemophilia and collaborates with the Ministry of Public Health and the Ministry of Social Affairs, but does not receive any governmental grants.
The strategic objective of the association for 2011-2020 is to insure the accessibility of adequate treatment for all.
LAH aims at improving the medical, educational and social status of the Person with Hemophilia (PWH).
All the HTC services are free of charge.

Credit: LAH, Cleveland Clinic, Mayo Clinic, WebMed, Health line.