Things just got real

As I write this, we're in the midst of Thomas' first bleed. He had his usual infusion on Tuesday morning (today is Thursday). I poked him twice on the right arm and once on the left and couldn't get it. One of the clinic nurses then poked his left arm and got it. So, it was four pokes total but none of them were any more traumatic than any other times we've poked him. We went home after that and he was normal all day. He crawled around and was maybe slightly fussier than usual but nothing that made me think there was anything wrong. That night though he woke up crying several times. Steve got up with him in the morning and noticed that his left arm was really swollen. He held it limp by his side and cried when he moved it. We called the hematologist and he had us come in to the clinic.

They assessed his arm in the clinic and decided that it was probably a muscle bleed as a result of the infusion the day before. He's been getting 250 units of Kogenate FS for prophylaxis and to treat the bleed they gave him 500 units. With his left arm out of comission, he doesn't have a lot of options left for venous access. He has two good veins in his right arm but I'm terrified that something similar will happen to that arm. If someone is going to cause damage to that side, it isn't going to be me so I had the clinic nurse do this infusion. She missed the one she tried in his arm and ended up going to his ankle. She got that one easily but it's more traumatic for him and it's more difficult to restrain his leg than his arm.

Thomas' arm on Wednesday night

So that was yesterday morning. We came home and he acted quite normal. He ended up playing and crawling like nothing had ever happened. The bruising increased but I thought the swelling went down some. He slept fairly well last night but this morning the arm looked worse than ever. The swelling was significantly increased. We went back to the clinic and they were quite concerned about the increased swelling. Our hematologist thought that it had spread too much to be a muscle bleed and it was more likely a soft tissue bleed. She was also concerned about compartment syndrome considering the huge amount of swelling. She decided that we would treat with another 500 units of factor and he'll likely need more tomorrow as well.

While they were waiting for the factor to come from Blood Bank, Thomas and I went to the playroom. I felt like his arm was changing quickly at the point. The bruising got darker and the swelling increased all within about half an hour. The nurse agreed and decided to keep us around after the factor infusion to see if it continued to change.

Sleeping while waiting to be re-assessed



We spent most of the day there but it never really changed much. They did have the othopedic surgeon come see it and he felt that we could continue to watch it closely and he would see it again tomorrow. If it changes through the night tonight we're supposed to go back for more factor. We're watching for increased pain or swelling and decreased ability to use his hand. If it stays the same, we'll go back in the morning. He's definitely favouring it but he is using it still. He's gone to bed now and unfortunately the only picture we took of it this evening was with a cell phone but I'm going to post it anyway.

Thomas' arm on Thursday night

So wish us luck for tomorrow. And please send us positive vibes that this doesn't turn into compartment syndrome!!

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.