Clinical Counseling and Care for Hemophilia

Mar 14, 2017
The specially trained pharmacists and nurses in the Accredo Bleeding Disorders Therapeutic Resource Center counsel caregivers and patients about the different types of hemophilia and how to best manage their condition.
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  • Hemophilia

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Accredo’s specialist pharmacists and nurses can answer questions and counsel patients about the use and benefits of prophylactic treatment.

Of the 20,000 people with hemophilia in the U.S., roughly 80% have factor VIII deficiency, also known as hemophilia A.

Though the severity and symptoms are the same, factor IX deficiency – or hemophilia B – is less prevalent and affects fewer than 4,000 Americans.

Understanding the Symptoms

People with either deficiency have a similar physiological response to trauma and similar symptoms. Severity of symptoms can vary depending on how much clotting factor a person has. A full complement of clotting factor is not necessary to live a relatively normal life, as is evident in people with mild hemophilia. Most bleeding due to hemophilia actually is internal – as the result of trauma injury to a joint, for example.

The specially trained pharmacists in the Accredo Bleeding Disorders Therapeutic Resource Center counsel caregivers and patients about the different types of hemophilia and how to best manage their condition.

  • People with greater than 5% clotting factor are considered to have mild hemophilia. They tend to bleed only with significant trauma or during surgical/dental interventions.
  • Moderate hemophilia is diagnosed when the individual has 1% to 5% clotting factor. People with moderate hemophilia do not bleed spontaneously; however, they do bleed with minor trauma.
  • Those with less than 1% clotting factor are considered to have severe hemophilia. They can experience bleeding anywhere in the body without an obvious injury. They are known as spontaneous bleeds. Joints are the most frequent location of these bleeds. It is not unusual to see many large, knotty bruises in children with severe hemophilia. Bleeds can also cause joint, muscle and even brain damage in the long term.

Fortunately, hemophilia treatment has made great strides in the last quarter century. Bleeding episodes, whether spontaneous or caused by trauma, are now much less frequent due to the advent of prophylactic treatment. Prophylaxis is the administration of the deficient clotting factor to the individual in amounts to help prevent – rather than stem – spontaneous bleeding.

Most bleeding due to hemophilia actually is internal.

Prophylactic treatments can help significantly improve the quality of life, especially in children and adults who grew up using them to prevent bleeding.

There are several advantages to such treatment:

  • Consistent administration, several times a week, prevents spontaneous bleeding in people with severe hemophilia.
  • Joint problems are significantly lessened in those who use prophylaxis.
  • Chronic pain is minimized due to healthier joints.
  • Started early in life, around age 2 or 3, prophylaxis enables individuals to lead a more normal, active life.
  • Prophylaxis reduces the need for joint surgery, physical therapy and assistive devices.

Given the benefits, factor prophylaxis has come to be known as the gold standard of treatment for hemophilia. The National Hemophilia Foundation’s Medical and Scientific Advisory Committee has recommendations to help patients and caregivers evaluate the use of prophylaxis for healthier hemophilia outcomes.

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