Friday, August 28, 2015

I am back!

It has been a long time since Endre or I have written on this blog. Life gets busy. But recent events (I'll get to it) caused me to reflect on the boys early experience with hemophilia.

Zander is now six, he is starting the first grade soon. I am still amazed at how fast this time has gone. Zander hasn't had any major issues at school (pre-k and kinder) and I anticipate we will continue on that same path. He attends a small charter school and the teachers are aware of his bleeding disorder and they called me a couple of times last year to ask questions but I never had to go to the school for infusions.

Zander is the same as he was when he was little (because six is big??), he still bleeds a lot. His port really is our life saver. He gets break through bleeds all the time (at least a couple times a month, sometimes more, never less) It still takes us a couple double doses a couple times a day for a couple days to get bleeds under control. This is our normal. Usually, at his yearly comprehensive visit, we up his dose. Sometimes we see that higher dose cut down on the amount of spontaneous bleeds, but that is like a honeymoon phase and it ends quickly, then we are back to lots of bleeds.

Zander takes this all in stride. He doesn't complain (about needing infusions), he knows when he is having a bleed, as I looked back on the blog, I was amazed that at age 2 1/2  he was asking for a needle day. This is Zander's normal.

Much to our surprise at our comprehensive appointment earlier this week, I saw one of Zander's original nurses, Cyndi. She was there when Zander started prophy (9 months old) she was there when he was diagnosed with his inhibitor, when he bled out during his PICC line placement, when he had his port placed, she removed that same PICC line. She taught us to use his port and was a huge part of our early journey with hemophilia. I was so sad to see her go, although happy she was having a baby and she was able to be at home with her daughter. At that early stage we needed strong nurses, we needed these women who helped us navigate our way. Then, after Zander had his port, things changed. We didn't need to go to the center, except one time a year for a comp visit. We didn't call and ask questions. We just did what we needed to. Needless to say when I saw Cyndi, I was confused and then excited and then really excited. She looked through my list of Zander's bleeds for the year and commented about how he is still bleeding the same as he did as a baby. She was not happy with this. She was not willing to accept this as normal. She had several ideas, first of which was to do a recovery study to see how well his factor was working. She didn't just want to increase his dose, she wanted answers. I can't say it enough, I love her. I didn't realize I had become so complacent about Zander's ability to bleed. I really had accepted it as life, I know which activities will cause bleeds (the beach without shoes), again this has always been Zander's pattern of bleeding.

We are going in next week to do a long recover study, longer than we have ever done. Our last recovery study was 24 hours. We saw that Zander, as he always has, metabolizes his factor really fast. Interestingly enough, when they did a blood draw this week, she ran a level on his factor (which we gave between 3-4 hours earlier) he was at just 49 percent. We were both amazed at how low he was. So, with the recovery study we will see if his 100 percent dose is even getting him up to 100 percent. We will see how long he is protected (I was always amazed he could get a bleed on a prophy day, but it happens not infrequently), we will run another inhibitor test. Then after the 36 hour recovery study, we will discuss our next steps. Do we want to switch products, do we want to use a plasma based product (research shows it is more effective in children).

I am excited to get answers. I am excited Cyndi pushed me to not accept his bleeding as normal. We can do better. Zander deserves better.  I will post updates as we receive them, Even if no one reads the blog, I am glad we have it. I was able to go back and read about Zander's early journey and see that he really has had a pattern that he has always followed. It is good to write things down. I am glad that I keep records as closely as I do. I can track bleeds and compare old records with current records. I am glad that even though Zander does every other day prophy and a lot of infusing in between, that he doesn't know it isn't normal. He is happy. He is healthy. He is "all boy." It takes a lot to slow him down, we learned recently that he can even go pretty fast on crutches.