It's been a few weeks since my appointment at UC Davis and so much has happened that I've just now allowed the information from my appointment to really sink in. I was lucky enough to spend an amazing week in Sacramento, California and catch up with friends and family. The day after we arrived was the day of my appointment and to be completely honest, I was terrified. I have had such a negative experience throughout the last few years that the thought of having to go to a hematology appointment just throws me into an anxiety tailspin.
I was lucky enough to be accompanied by both of my amazing parents. Since diagnosis, it has been my mom and I primarily dealing with appointments and infusions while my dad worked his butt off to provide for us, so I was really glad that my he could be there also.
Walking into the area where they hold clinic I was immediately met with positive energy. Everyone was so amazing and kind. It was a breath of fresh air. We initially met with a very nice doctor who kind of did an overview of everything. I rattled off my story like I have done so many times before. I had my list of questions I have asked for the last three years, I had all of my research, I was as ready as I could have been. After taking my history and hearing my symptoms the doctor asked if I had ever heard of Glanzmann's Thrombasthenia. I explained that yes, I had heard of it and I have connected with individuals that have GT and I always noticed how similar our symptoms were but no, I had never been tested. She didn't really say much about it after that and she left the room. As she left I couldn't help but think about all of the times I have specifically questioned my hematologist on whether or not I needed to be tested for GT and the consistent one word answer I have always received. "No." Hm. Interesting, isn't it?
About ten minutes later we met the pediatric hematologist and RN. The following hour was the most validating hour of my life. Every question that I have asked over the past year or so, knowing the answer because of the many hours of research I have conducted , has always been met with that all so familiar one word answer... "No." No explanation, no reasoning behind it, just no. So you can imagine that I found it fairly interesting that this time around, when asking my questions, I was met with a new one word answer..."Yes." For the first time since being diagnosed I finally felt like the many hours spent trying to figure everything out had paid off.
I was told that, YES all of the hormone I have been on would in fact affect my VWF. I was also informed that YES, stress does impact my VWF. The RN actually said that they could pull my labs but that if I was upset--even if I didn't feel upset--the tests wouldn't be accurate. The doctor also agreed that, YES, it was time for a new DDAVP challenge.
Beside getting my questions answered, I also was given some very valuable information, some that reinforces just how difficult platelet disorders are to manage and treat, contrary to popular belief within the community. As of right now, my diagnosis is still a Non-Specific Platelet Storage Pool Defect. There are NO proven treatments for a disorder such as mine. Treatment for SPD patients is a guessing game. Stimate can work, but unless there has been a Stimate challenge done to see if the Stimate directly affects platelet function there is no guarantee that it works. I was also told that Tranexamic Acid, in my case one of the two only medications I have to help with bleeding, is basically irrelevant to what I'm dealing with. I asked about Novo7 and inquired as to whether or not I might be a candidate.. I was told that, again, there is really no guarantee that it will work, that it either does or it doesn't. The doctor also explained that I am in a 'gray zone' that they see in which I am very difficult to treat and that the same treatment doesn't always work. One day Novo might work, the next maybe not. One day platelets might work, the next maybe not. He also explained that even if I was diagnosed with GT the only thing it would change is the name of my diagnosis. It wouldn't impact my treatment plan very much due to said 'gray zone'.
So where are we now? Well, as of right now I am scheduled to go back to UC Davis mid-August for a Stimate challenge and more labs. We are still waiting on some of the lab work, specifically the test to see if I have Glanzmann's. Overall, the prognosis isn't great...It's all still day by day and a guessing game and it probably always will be. To be honest though, knowing and feeing confident that I finally have people who want to help me figure this out is the best feeling in the world. Knowing that I'm not insane and that all my hard work has paid off is so validating that I can't even begin to describe it. I'm looking forward to seeing where this road takes me. A huge thank you to everyone who continue to support me through this crazy journey. I couldn't do it without you.
I was lucky enough to be accompanied by both of my amazing parents. Since diagnosis, it has been my mom and I primarily dealing with appointments and infusions while my dad worked his butt off to provide for us, so I was really glad that my he could be there also.
Walking into the area where they hold clinic I was immediately met with positive energy. Everyone was so amazing and kind. It was a breath of fresh air. We initially met with a very nice doctor who kind of did an overview of everything. I rattled off my story like I have done so many times before. I had my list of questions I have asked for the last three years, I had all of my research, I was as ready as I could have been. After taking my history and hearing my symptoms the doctor asked if I had ever heard of Glanzmann's Thrombasthenia. I explained that yes, I had heard of it and I have connected with individuals that have GT and I always noticed how similar our symptoms were but no, I had never been tested. She didn't really say much about it after that and she left the room. As she left I couldn't help but think about all of the times I have specifically questioned my hematologist on whether or not I needed to be tested for GT and the consistent one word answer I have always received. "No." Hm. Interesting, isn't it?
About ten minutes later we met the pediatric hematologist and RN. The following hour was the most validating hour of my life. Every question that I have asked over the past year or so, knowing the answer because of the many hours of research I have conducted , has always been met with that all so familiar one word answer... "No." No explanation, no reasoning behind it, just no. So you can imagine that I found it fairly interesting that this time around, when asking my questions, I was met with a new one word answer..."Yes." For the first time since being diagnosed I finally felt like the many hours spent trying to figure everything out had paid off.
I was told that, YES all of the hormone I have been on would in fact affect my VWF. I was also informed that YES, stress does impact my VWF. The RN actually said that they could pull my labs but that if I was upset--even if I didn't feel upset--the tests wouldn't be accurate. The doctor also agreed that, YES, it was time for a new DDAVP challenge.
Beside getting my questions answered, I also was given some very valuable information, some that reinforces just how difficult platelet disorders are to manage and treat, contrary to popular belief within the community. As of right now, my diagnosis is still a Non-Specific Platelet Storage Pool Defect. There are NO proven treatments for a disorder such as mine. Treatment for SPD patients is a guessing game. Stimate can work, but unless there has been a Stimate challenge done to see if the Stimate directly affects platelet function there is no guarantee that it works. I was also told that Tranexamic Acid, in my case one of the two only medications I have to help with bleeding, is basically irrelevant to what I'm dealing with. I asked about Novo7 and inquired as to whether or not I might be a candidate.. I was told that, again, there is really no guarantee that it will work, that it either does or it doesn't. The doctor also explained that I am in a 'gray zone' that they see in which I am very difficult to treat and that the same treatment doesn't always work. One day Novo might work, the next maybe not. One day platelets might work, the next maybe not. He also explained that even if I was diagnosed with GT the only thing it would change is the name of my diagnosis. It wouldn't impact my treatment plan very much due to said 'gray zone'.
So where are we now? Well, as of right now I am scheduled to go back to UC Davis mid-August for a Stimate challenge and more labs. We are still waiting on some of the lab work, specifically the test to see if I have Glanzmann's. Overall, the prognosis isn't great...It's all still day by day and a guessing game and it probably always will be. To be honest though, knowing and feeing confident that I finally have people who want to help me figure this out is the best feeling in the world. Knowing that I'm not insane and that all my hard work has paid off is so validating that I can't even begin to describe it. I'm looking forward to seeing where this road takes me. A huge thank you to everyone who continue to support me through this crazy journey. I couldn't do it without you.
Comments
Post a Comment