Bleeding Disorders: It's in the Genes

Feb 6, 2014
Pharmacists and nurses in the Bleeding Disorders TRC help patients understand the complexity of their disease and manage the treatment plan.
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  • Hemophilia

Hemophilia can be a difficult diagnosis for a family to receive, especially because a majority of the newly diagnosed cases are in babies and children. Often, there is a lot of misunderstanding associated with bleeding disorders such as hemophilia. Many years ago, people with these disorders were commonly referred to as “bleeders” because of the ongoing external and internal bleeding episodes that were difficult to control and extremely debilitating.

Bleeding disorders can be complex – and expensive – diseases to manage. Therefore, it’s important to have a specialized medical and pharmaceutical team to help prevent episodes and manage the symptoms and therapy. Bleeding disorders are not curable, but they are treatable. The experienced specialist pharmacists and nurses in the Express Scripts Bleeding Disorders Therapeutic Resource Center (TRC) are dedicated to counseling patients and their families. With their disease-specific expertise, they can help patients manage their conditions in order to lead full, normal lives.

First, some basics about bleeding disorders.

What Are Bleeding Disorders?

They are rare conditions characterized by a deficiency or malfunction of clotting proteins that control bleeding. People who are affected do not bleed any more rapidly than anyone else; they have prolonged bleeding which, if not treated, can be severe.

Hemophilia, which is one type of bleeding disorder, primarily affects males, including approximately 20,000 patients in the U.S. These individuals lack significant proteins that are crucial in the clotting process.

Challenges With Bleeding Disorders

Hemophilia is one of the top five most expensive diseases to manage – with the average cost being about $50,000 per year. Because clotting factor dosing is based on weight, the costs increase with age.

Individuals with severe hemophilia are treated intravenously with the clotting factor they specifically need to prevent and manage bleeding episodes. Typically, the joints are affected first in early childhood and, if not treated appropriately, can result in severe chronic joint problems for life.

Value of Clinical Specialization

The pharmacists and nurses in the Bleeding Disorders TRC can help patients understand the complexities of the disease and manage medication changes to ensure healthy outcomes.

It’s important that extended family members, teachers and others who care for children with these disorders understand how to spot and prevent bleeding episodes. Here are a few tips from TRC specialists that can be shared with caregivers:

Bleeding episodes are likely to be fewer in infancy but also can be harder to spot. Some signs and symptoms of pain may include fussiness, crying inconsolably, not sleeping, not feeding well, grimacing, failing to move or use a part of the body, or crying when a part of the body is moved.

  • Toddlers move and tumble more than infants, so they’re much more likely to have frequent bleeding episodes resulting in superficial bruising on the sternum or chest. These may not require infusions of factor, but it is important to check with the doctor or nurse. Buttock bleeds are typical in the initial stages of learning to walk and should be recorded and reported immediately, as they frequently need to be treated with clotting factor.
  • Have a clinician examine any bruising, “knots,” hardened areas under the skin or swelling.
  • Once a toddler begins to walk, prevent injuries to the mouth that can result from falling while carrying a bottle or sippy cup. Reserve bottles for times when the child is being held and cups for sitting at a table. Otherwise, serious injuries may require intravenous infusions of clotting factor and often hospitalization.
  • Learn and commit to memory the areas of life-threatening bleeds, their symptoms and how to treat them. All of these must be addressed by a hematologist or at an emergency room.
  • Above all, remember that having a bleeding disorder is the “norm” for these children. They don’t know any different life. Parents and caregivers who have an accepting, calm and proactive attitude toward care will help them become responsible adults with positive outlooks on a future that just happens to include a bleeding disorder diagnosis.
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