Long overdue update

I've really neglected this poor little blog. Around the time I gave up writing Thomas was teething and his sleep went all to hell. I was tired and it was hard to sit down and focus on anything. Anyway, I'm still tired but that's no excuse now.

So, a much needed update on lots of things. These days hemophilia is certainly part of our lives to an extent that it wasn't when he was littler but it's definitely manageable. I think things have been easier than we expected to this point. I'll get to some specifics.

Immunizations
Thomas is up to date on his immunizations. We haven't had any bleeds as a result of them. Each time we've made little pressure dressings and applied ice. It may be overkill but since they've all gone so smoothly, I'm not about to mess with success so we'll continue doing all those things every time.

Bleeds and bruises
We haven't dealt with an actual bleed yet but it seems as he's getting older, he's bruising easier and easier. He's looked pretty shocking at times. He army crawls instead of hands and knees crawling so his knees are being spared but his elbows and forearms really take a beating. The worst bruise we've seen so far was on the back of one of his thighs. It was really big and dark purple. It took weeks for it to heal and even now, probably two months later, you can see a light brown mark where it was. We're pretty sure it was from sitting in a shopping cart so now we line carts with his blanket before we sit him in them. That seems to have fixed the problem. It's really unpredictable what will cause a bruise and what won't. We pick him up under his arms all the time (and he's really heavy) but he's never bruised there. Meanwhile, right now he has a line of three bruises on one of his legs that totally looks like fingerprints. Those are the kinds of things I would prefer that strangers not see. I can only imagine what I would think if I saw a kid with what looked like a handprint shaped bruise on his leg.

When we first started prophylaxis (which I'll talk about in a minute) we saw an immediate improvement in the bruising but now it seems to be increasing again.

Prophylaxis
When I last updated, the plan was to start prophylactic infusions of Factor VIII at 6 months. Well, at about 5.5 months we went for a clinic appointment and had some blood work done. It was traumatic to say the least. He's always been a chubby baby and it took three attempts to get the blood drawn, ending with getting it from his ankle. We all decided then that venous access was just too difficult at that point to start trying to do regular infusions. I've been asked why a port wasn't suggested at this point. Our hematologist made it clear that she wouldn't consider any sort of venous access device at that point and while I didn't specifically ask why, I thought it was because the risks of doing that just weren't warranted based on the evidence for starting prophylaxis at that age. I talked about this before but up until very recently, kids usually started prophylaxis around 18 months to 2 years. There is some new evidence to suggest that earlier may be better but I don't think it's terribly convincing. So our plan at that time was to reassess in a few months.

By the time we went for our next appointment I was more than ready to get started. We had some blood work done in the outpatient lab and it went extremely well. Thomas was also starting to bruise a lot more and I really wanted to get him on some factor. He had his first dose on on February 13th at almost 9 months old. It was difficult but not terrible. Two nurses poked him one time each. The second infusion was worse. And the third time, I did it! I was on a high after that because I got it on my first attempt and he did awesome. I thought this was wonderful. I was awesome at it and he was never going to have to go through the trauma of anyone else doing it again. Unfortunately, the times since then haven't been as great. I'm usually pretty good at IVs and venipuncture at work and I really think that people can get themselves psyched out and start missing just because they think they're going to miss. That's about where I am right now. I've put a lot of pressure on myself to get us to a point where we can do it at home and now after struggling through doing it the last three weeks, I'm feeling a little defeated. We go in to the clinic again tomorrow morning and I'm going to do everything I can to talk myself into believing I can do it.

At this point we're doing a 50% dose once a week. I'm not sure how things are going to progress from here. The last time we talked to the hematologist she talked about increasing it as he has joint bleeds. I don't like the sound of that and I would rather prevent joint bleeds altogether. We should have an appointment with her soon so we'll see what the plan is then.

Daycare
My maternity leave is quickly coming to an end and Thomas is starting daycare orientation on April 20th. My heart skips a beat just thinking about that. Our clinic nurse has offered to do an education session with the daycare and they are very receptive to that. I have a hard time talking to them about it because there are so many unknowns. We know what Thomas is like now but we really can't say what things will be like when he starts walking. Who knows what issues might come up when he's playing with other kids and stuff. We aren't really that careful with him right now. He has a soft helmet and knee pads but we never use them. I'm not sure how we want to use those things at daycare. To be honest, my bigger concerns at this point are whether he'll nap there and drink milk from a cup.


So, that's where we are right now. He's such a great little guy. He's had a cold all week and even though he's obviously been miserable, he's still so nice and usually really easy to get along with. I'm looking forward to getting back to work but it's going to be really hard to leave him.

First Bruise

Two days ago, Thomas and I were getting ready to go get Ty at the bus stop after school. I put Thomas in the Ergo and as I was doing that, put my hand behind the back of his head like I always do. I felt a little lump there, almost at the base of his skull, right in the middle. At first I described it as the size of a marble but really it was more like a pea. I was rushing to get out of the house so we wouldn't miss the bus and since I had Thomas inward facing in the Ergo, I went and looked in the mirror. There was a little purple bruise right on top of the lump. It was a little bigger than the tip of a pencil eraser.

I called Steve as we were walking up the street. This was the first time we've seen any bruise on Thomas. It worried me that I didn't know where it came from and it worried me even more because it was on his head. We agreed that I would call the doctor as soon as I got back home.

We're lucky to live close to a children's hospital with an excellent hemophilia treatment centre (HTC). I called the clinic and left a slightly panicky message saying if they didn't call me back in a few minutes I would page the hematologist. I don't know if all HTCs work this way but we're able to page the hematologist directly 24 hours a day and they encourage us to do so. So, after waiting about three minutes, I had him paged. He asked me a few questions and I described the lump to him. He told me to keep a close eye on it and if it changed at all or if Thomas behaved differently, to bring him in for factor. Eek. You can read my post about immunizations to learn about my concerns about factor at this point but the bottom line is this: we want him to have his first dose of factor in the absence of a bleed. That puts him at a lower risk of developing an inhibitor.

We did keep a close eye on it all evening. I lined up my mom to look after Ty in the event that we had to go to the hospital. But it didn't get any worse. Thomas acted normal. He fussed when I touched the lump but no more than anyone would if someone was poking at a bruise. Here's what it looked like that evening.

At this point it was already starting to look more red than the purple it was earlier.

Last night when I was putting him in the bath, we folded up a towel to go under his head. The edge of the infant bathtub lined up perfectly with the bruise so I'm quite confident that's where it came from. It makes me feel better to know what caused it and that it's not just random. Today the lump is still there but the bruise is kind of a light brown. It's healing just like I would expect anyone's bruise to heal.

If there's anyone reading this with a kid with severe hemophilia, is this normal? I would have expected worse. When do severe hemophiliac kids become symptomatic? Or maybe this is symptomatic. Maybe a normal kid wouldn't have gotten a bruise from an infant bathtub at all.

Thomas has had his bloodwork done only once, at birth. Steve has been wanting his factor level rechecked for a while and our hematologist has plans to do it in November before we start prophylaxis. I've heard stories of kids who were found to have lower levels than they had at birth so I guess it's possible that his level could be higher than it was. I try to not even think about that. Trust me, I'm not in denial. But I do tend to develop unrealistic expectations about things sometimes. My fear is that when we have the bloodwork done in November and it comes back as <1% again, it will be like getting the diagnosis all over again. I'm trying hard not to expect anything other than <1% but he just seems so normal. Is this still some sort of newborn protective period? Or do all hemo babies do this well in the beginning?