Dysfunctional platelets

[Sally C]

I thought this information would be useful to those of you with bone marrow diseases.
My husband Don has MDS. Right now he is totally tranfusion independent with normal HGB (although they are huge), normal whites, and platelets 107,000 after about a year on Promacta. Currently he is on no MDS meds (since 7/12).
He was scheduled for a hernia operation at the end of Oct. Our hematologist as usual, covered all bases and ran a PFA-100 test on his platelets to test their clotting time. While the test isn't perfect it did come back abnormal. The morning before his surgery her office called to inform us they had already canceled his surgery.
From what I understand, MDS can cause dysfunctional platelets - I don't know about PNH or AA.
I wanted to give a heads up for anyone who is contemplating surgery to have this test done. We were thinking since his platelets were over 100,000 there would be no problem. Thankfully she ran the test before he went under the knife.
She is going to test him with a shot of Vasopressin which can be a temporary fix for the dysfunction. The knowledge that it would work could be crucial if he ever needed emergency surgery. Getting this shot would be much quicker than finding platelets and his having to take the pre-meds 30 minutes before the transfusion.
God Bless and Happy New Year to all!
Sally

[Birgitta-A]

Hi Sally,
My platelets are dysfunctional too. Before I had my port inserted I took Amicar during 5 days and I got a platelet transfusion but I still bled a lot.

If I have to have "real" surgery I will suggest a PFA-100 test like Don had.
Kind regards
Birgitta-A

[Sally C]

Hey Birgitta,
I remembered that you had mentioned you have dysfunctional platelets.
Something you may want to consider if it's available where you are is to get the shot that Don is going to get - Vasopressin. The way it was explained to us is that, if the shot does what we want with the platelets, it would make Don's platelets purge their insides into the blood stream which is what makes the blood clot. It is a short term fix as once those platelets have purged then you have to wait for a new bunch of platelets - they can only be purged once. But the value of knowing it would work on your platelets is what I mentioned in my first post - if Don needed emergency surgery, what platelets he has would be made to function with just a shot without having to wait to find platelets, administer pre-meds, and infuse them. In a critical situation the time saved by just getting a shot could possibly mean the difference between life and death.
The only concern with the shot is they have to closely monitor blood pressure as all the purged material is pushed into the blood stream and can run up the blood pressure.
We are meeting with his cardiologist on 2/4 and with his OK, Don will get the injection to see if it works on him. Will let you know.
Take care!
Sally

[Birgitta-A]

Hi Sally,
Very good info about Vasopressin. Thank you!
Kind regards
b
Birgitta-A

[Sally C]

Thank you Birgitta,
And one thing more I want to mention to you or anyone else with dysfunctional platelets...
Don's hema. suggested he put her business card in his wallet and write on the back that his platelets are dysfunctional. If he were to be in a wreck or some situation that I wasn't there to tell them, or he couldn't, they would know about his platelets and also would know to contact his doctor.
Take care,
Sally

[Neil Cuadra]

Sally,

You could go a step further and make a personal "medical information card", as I describe here, so emergency medical personnel will know his age, contact information, diagnosis, physicians, and specific details such as having dysfunctional platelets.

[Birgitta-A]

Thank you very much Sally and Neil. I will control if we have some good cards like that in Sweden - if not I will write my own.
Kind regards
Birgitta-A

[Sally C]

Thank you Neil,
As always you have very good advice!
Thanks!
Sally