What is Hemophilia

WHAT IS HEMOPHILIA?

Hemophilia is a rare, in-born, life-long congenital bleeding disorder, affecting mostly males. Due to a defective gene, the body of a person with hemophilia does not produce enough of certain blood proteins, called clotting factors, which prevents an affected individual from forming a strong and stable blood clot. People with hemophilia do not bleed any faster than normal, but they can continue to bleed for a much longer time.

What are the symptoms of Hemophilia?

  • Big bruises
  • Bleeding internally, inside muscles and joints, causing pain and immobility
  • Spontaneous bleeding (sudden bleeding inside the body for no clear reason), characterized by pain and swelling
  • Prolonged nose bleeding
  • Prolonged or spontaneous hematuria (blood in urine)
  • Prolonged bleeding after getting a cut, removing a tooth, or having surgery.
  • Bleeding for a long time after an accident, such as after an injury to the head.

WHAT HAPPENS WHEN THEY DO NOT GET PROPER TREATMENT:

Long term effects of joint bleeds

  • Repeated bleeding into a joint causes the synovium (lining) to swell and bleed very easily. Over time, most of the cartilage breaks down and some bone wears away.
  • Without proper treatment, hemophilia patients develop a form of arthritis, causing joints to be locked in and permanently deformed

Long-term effects of muscle bleeds

  • After repeated bleeds, muscles can become weak, scarred, and shorter than normal (sometimes permanently). They can no longer protect the joints. Joints above and below the muscle cannot move properly. They may bleed more often.
  • If nerves are damaged during muscle bleeds, the muscle may become weak or even paralyzed.
  • Permanent damage to joints, muscles, and nerves affects the way a person sits, stands, and walks.

Serious or life threatening bleeds!

  • Bleeding of the brain (usually resulting from a head injury) is a major cause of death in hemophilia, especially in children. Head bleeds can cause headache, nausea, vomiting, sleepiness, confusion, clumsiness, weakness, fits, and loss of consciousness.
  • Bleeding into the throat may result from infection, injury, dental injections, or surgery. Throat bleeds cause swelling, as well as difficulty swallowing and breathing.
  • Other medical conditions that cause bleeding, such as infections or injury can be aggravated by hemophilia. Bleeding inside the gut, or other internal organs can cause major loss of blood if left untreated over a prolonged period of time.