We were hoping that this would be our last update on the subject of von Willebrand but it seems that we have a few more labs and tests to do. Our appointment today was with Dr. Torkelson, the hematologist, who confirmed the Type 1 vWB diagnosis. He said that he has had two other cases where the Type 1 diagnosis was made because the child had ITP (a low platelet count of unknown origin) too, which led to vWB factor to be measured. He still is not sure that Jack had/has ITP so he will do a CBC next week to check Jackson's platelet level again. If his platelet count is back to normal, then we'll know he had ITP but if it is still low we will have to keep looking to figure out why.
The doctor then talked about the treatments for von Willebrand, and said that Jackson will not be restricted in any way physically. It is recommended that we not give him Motrin, which is a bummer since it works so well for him, but hopefully since he has the ear tubes we will not have such trouble with high fevers. The only treatments that Jackson will have to worry about are if he has to have surgery or gets into a major accident. There are two options for him at this point, he either can get something called Factor 8 which is a blood product that will help him with clotting. This has it's own risks, however, since it is harvested from donor blood. So, instead, the doctors like to use a synthetic hormone called DDAVP. This medication will actually help pull von Willebrand factor out of the cell lining of his blood vessels and increase his blood volume of von Willebrand, normalizing his ability to clot for 24-48 hours.
On Tuesday the 16th, Dr. Torkelson has ordered for Jack to have an IV dose of the DDAVP, with labs drawn before and after to make sure that he responds to the medication. (They will also be getting his CBC at this time). If he responds, then we know that he can have this med before surgery and after a major trauma if he were to ever need it (hopefully not).
The other labs that will be drawn are on Larry and I, which I have to have my O.B. order since Dr. Torkelson said that insurance companies don't like to pay out when pediatric doctors order labs on adults. Hopefully I can get my other lab work that has been ordered for me at the same time for the baby. I'm sure these labs will take another 2 1/2 weeks or so to get these results so I hope to get them done soon. To give some indication of how mild this vWB thing is, it is likely that Larry or I also have this issue, but neither of us have had any problems with it. The other possibility is that a genetic mutation occurred when Jack was developing in utero that caused this issue to develop...if this is the case, please refrain from calling him 'the mutant,' or 'mutant baby' or 'the little mutant boy,' etc. ;-)
He is gonna be just fine... Look at that face, how could anything happen to that little man. He is going to have a sister to protect pretty soon. I sure love you all.
ReplyDeleteI better not hear anyone call him mutant boy! I might have to make them look like one!!!!
ReplyDeleteHe is too adorable in his 2 year pictures, he looks like a little man on the couch.
Congrats on getting the diagnosis so quickly for your son. Please consider contacting your local Hemophilia Association to get additional support from parent groups. They know the ins and outs of vWD that Doctors and Nurses have only heard about.
ReplyDeletewww.hfnv.org is the local association website.