An inhibitor changes everything... Sort of

This post has been in my head for a long time but life is busy. Thomas recovered well from the arm bleed that I wrote about in my last post. I went back to work after maternity leave at the end of April. Hard to believe I've been back for eight months already!

Thomas is in daycare now and doing really well. I can talk more about that transition in another post but his teachers have been great. We encouraged them to call us with any injury and they do. The calls have decreased in frequency as they've gotten used to him and we feel really comfortable with him there now.

After that arm bleed we started having more and more trouble with IV access. Around June it got to the point that infusions became impossible. I think we went three weeks without getting a full dose into him. I would hit the vein and then lose it less than half way through the infusion. In conjunction with his hematologist and nurse we finally decided to get him a port a cath. Steve and I had the teaching to learn how to use a port and Thomas went to the OR to get it on July 19th. Holy stressful experience. He was infused right before his surgery. I can't remember how much they gave him but it was a big dose. Much more than we were using for prophylaxis at that time. After he got the factor they did blood work to check his Factor VIII level but because of time constraints he went to the OR before the results were back. Steve and I waited and waited. Finally the surgeon came out to talk to us. Thomas was under anesthesia but the surgery was never started because his Factor VIII level hadn't come up high enough. Huh?? He told us that they were keeping him asleep, giving him another dose, and rechecking his level. I asked what his level had come up to and he really dodged the question. Again we waited. Finally the anesthetist (a colleague of mine) came out and said that they had woken him up without doing the surgery because again, his level didn't come high enough. I couldn't even wrap my mind around it. I asked him why and he said "well, they think he has an inhibitor". My heart dropped. There it was. The thing we had tried so hard to avoid and there it was.

It turned out that Thomas' inhibitor titre was 28 BU. That's considered a high titre. High being bad. The plan was to start immune tolerance induction (ITI). ITI involves giving frequent, high doses of Factor VIII to overwhelm the inhibitor. There's a good explanation of it here: http://www.hemaware.org/story/immune-tolerance-induction-can-eliminate-inhibitors We were presented with two ways of doing it: 1) Lower doses three times a week. or 2) Much higher doses everyday. They are equally as effective but the higher dose treatment tends to work faster and children on it tend to have fewer bleeds. We chose the higher dose. We also chose to go with a peripherally inserted central catheter (PICC line) over a port. Why we went that route is sort of a post in itself so I'll plan to write about that soon.

Thomas got his PICC line on August 28th and started his ITI the next day. So we're now over 5 months into it. Some kids have gotten rid of their inhibitors in that time frame but we're not there. He's had his level checked twice since starting and the results were 28 BU and 32 BU. We'll be checking again on Feb. 4th.

The inhibitor diagnosis was pretty devastating but life goes on. It's amazing how well a toddler can adapt to daily infusions. Steve infuses him six days a week (I do Sundays) and does an amazing job. Who knew that in a couple where one person is a nurse, the non-nurse would be the one to take responsibility for that stuff? Five months in and we're still on that same PICC line which is fantastic. And the most important thing is that Thomas is still just Thomas. He's a clumsy toddler who throws himself head first into everything and he's just the exact same kid he was before the inhibitor was diagnosed.  So yes, an inhibitor sucks and it's all bad news but the truth is not much has changed for us.