Female Speaker: Young Iowa resident Philip Parker is a spunky 12-year old, who loves playing basketball in his spare time. Philip also has a blood disorder called von Willebrand disease.
Philip Parker: There's a whole bunch of stuff in my body sticks together when I bleed, and I don't have it working right.
Female Speaker: Philip isn't the only one in his family with von Willebrand, his mother Denise was diagnosed with the disease 25 years ago, after her arm became bruised and swollen following a prior blood test.
Denise Parker: Whenever I have to have a procedure done, it requires treatment beforehand and usually something that which has been outpatient surgery requires a hospital stay, so it just kind of makes everything more complicated. Philip, has just been kind of the evolving experiences or him understanding what it means when he hits a bruise, that's a bleeding inside under his skin.
Female Speaker: Our bodies have developed an efficient mechanism to control bleeding. When the system is defective it throws the process off balance. This is what happens with von Willebrand disease. When a blood vessel is damaged and starts bleeding it triggers a three step process. First, the vessel constricts, slowing the flow of blood to the injured area, then platelets in the blood starts sticking to and spreading on the walls of the damaged vessel. Finally a clot forms on the surface created by these platelets stopping the bleeding.
Dr. Jorge Di Paola: I always tell my patients that blood vessels are like water pipes, if you have a crack in a water pipe you leak water. In this case if your blood vessel is damaged, you're going to bleed. We have a system that is a clotting system where the platelets are part of it that is going to come and repair the blood vessel and repair the leak. The von Willebrand factor is almost like a glue that allows the platelets to stick to each other and to stick to the damaged wall and eventually make a plug and stop the leak. This is the family that I was telling before this viability of von Willebrand disease--
Female Speaker: The von Willebrand disease is potentially fatal in rare instances, 70% to 80% of cases are classified as mild to moderate. Typical symptoms include frequent nose-bleeds, bloody gums and easy bruising. Philip has mild von Willebrand, he knows about the dangers surrounding his blood disorder, but that hasn't stopped him from playing.
Denise Parker: Just last week was he was at the hospital because he crashed, fell on his knees really hard and hit his face and hit his wrists and now he's at school in the gym.
Philip Parker: I jumped backwards to avoid a ball and smashed.
Female Speaker: Stopping and getting an ice pack and pressure on the injury as time away from the game.
Denise Parker: Sometimes he is kind of like mom; just go let me play basketball.
Female Speaker: Current treatment options for patients are medications that boost the body supply of von Willebrand factor. These medications include an easy to use nasal spray for mild von Willebrand. Individuals with more severe symptoms can receive infusions loaded with von Willebrand factor, known as replacement therapy.
Paul Monahan: For a short period, at least, you can often double or triple the von Willebrand factor level for the patient.
Denise Parker: Yeah, I really don't spend a lot of time thinking about the disease, it's really quite manageable --
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