Bleeding Disorders

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.

How is it treated?

The Medical Treatment of Hemophilia

• Clotting Factor Replacement Therapy – infusion (injection into the bloodstream) of factor VIII or IX concentrates, or other blood products to facilitate normal clotting.
• First aid of affected area –  PRICE (Prevention, Rest, Ice, Compress and Elevate)
• Pain medication and Anti-inflammatory medication to reduce swelling.
• Tranexamic Acid for surgical, external or oral bleeding.
• Physical rehabilitation of damaged joints and muscles

WARNING: Persons with Hemophilia should avoid using any medications classified as blood thinners such as:

• NSAIDS such as ibuprofen
• Herbal/supplement products such as glucosamine, Vitamin E or gingko biloba

Living with Hemophilia

Hemophilia is a lifelong, chronic and severe disease from birth. Persons with hemophilia often grow up with all the complications and the difficulties of a rare condition. In addition to receiving proper medical treatment, a person with hemophilia also needs lifelong help to become a fully developed and empowered individual.

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A HOLISTIC APPROACH TO CARE:

• Physically active – Regular exercise of safe sports and activities, physical rehabilitation, and proper nutrition.
• Mentally sharp – Continuing education about hemophilia care, and skills within their physical limitations.
• Emotionally healthy – Mindful of their thoughts, feelings, and behaviors, able to cope with challenges and difficulties associated with having hemophilia.
• Spiritually strong – Strong in faith, self worth and awareness of the meaning and purpose of his life.
• Socially involved – Positive, productive and supportive relationships with other people. Patients moving beyond themselves and becoming involved in community.

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