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.

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.

First Family Weekend

A couple of weeks ago we went to our first family weekend with the Nova Scotia chapter of the Canadian Hemophilia Society. It was held at Brigadoon Village which was really nice. We met a lot of really nice people. Like, the kind of extra nice people who are more than happy to share their own stories and listen to ours. It was really good to feel part of this whole community of support.

Ty fishing at Brigadoon

Thomas was a great boy while we were there and got lots of hugs and kisses from his new friends. He's becoming such an amazingly good baby. He had a rough period from about a month and a half to three months but now he's just a pleasure to be around. Ty LOVED the camp, too. He had so much fun and it was such a relief to us to see Ty playing with the boys with hemophilia and to see that there was really no apparent difference between him and them. If anything, Ty was probably the most timid of the boys his age. Actually, there were a few boys very close to his age which made me think that if he had been born with hemophilia, he would have been lucky to know other boys locally. Thomas might not get that.

Sleepy Thomas and I at Brigadoon

As we were leaving, we realized that we didn't explain to Ty what the point was of the weekend. I told him on the way home that those boys he was playing with had hemophilia like Thomas and that's what everyone there had in common. He asked why he got to go and I explained that he'll always get to go to those things because he's a family member of someone with hemophilia. Sometimes I feel like I need to be careful to not make it sound to Ty like Thomas is special. I don't want him to somehow resent Thomas because he gets to do special things or has access to things he doesn't. It's kind of a hard balance to make Ty understand that Thomas is different without making it seem like Thomas is different... if that makes any sense at all! My hope is that hemophilia can just be a small part of our lives with Thomas not feeling like he's any different and Ty not treating him any differently. It will probably be something that will just naturally happen but for people with unaffected siblings, feel free to weigh in on that.

In other news, we've gotten through another round of immunizations. We did the exact same as last time, held pressure then wrapped little dressings around his legs and put ice on the sites for about 10 minutes. We had a late appointment so we left the dressings on until bedtime. When we took them off his right leg was bleeding just a bit. It was really superficial, just from the insertion of the needle but we wrapped it up again and left it for the night. It did end up bruising but nothing major at all.

Our next challenge coming up is our hematology appointment on November 3rd. He needs bloodwork which will likely be a disaster considering those roly-poly arms. He's 23 pounds now at four months old!!

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?

Immunization Update and Finding Childcare

It's been a week and a half since we went ahead and did Thomas' immunizations. I'm happy to report that it went perfectly. He had one injection in each thigh. It was so sad of course to hear him scream afterwards. I was pretty tense about the whole thing. Steve came with us so he held him afterwards and I held pressure on the sites. Then I made little pressure dressings for his legs which was suggested by a lovely reader of my blog, "sarasarasara". Thanks for the tip, Sara! I folded a 2x2 gauze, put that over the site, and wrapped the whole thing with Coban tape. That's the stretchy tape that sticks to itself, not skin. Here's a pic of the poor little guy after he got all bandaged and breastfed.

I put ice on his legs for ten minutes also. It may have all been overkill but he didn't get so much as a bruise so you can bet I'll be doing the same thing next time.

Several people suggested getting the shots subcutaneously rather than intramuscularly. For those wondering, subcutaneous injections go just into the fat under the skin and are unlikely to cause trouble with bleeding. Intramuscular injections go into the muscle which can cause a bleed. Anyway, our hematologist looked into it and said only the Hepatitis B vaccine could be given sub-q. I asked our family doctor as well and she said she had never heard any evidence that giving them sub-q would be effective. So, that's why we went with IMs.

With that all behind us, we've moved on to arranging childcare for when I go back to work. That isn't until April of next year but I wanted him in a centre and they can be hard to get into. We decided to first try for the centre where Ty had gone. It's familiar and we had a good experience there overall. Still, because Thomas is different, it's pretty stressful. When I was still pregnant, my biggest worry about daycare was whether they would use our cloth diapers. That seems pretty trivial now. Well, kind of. It was still our second biggest concern.

The Canadian Hemophilia Society has really thought of everything. We were given a booklet about choosing childcare and also one that's meant to be given to schools to explain what hemophilia is and what the school needs to look for. There's also a grant available to the daycare centre for any extras they may need to look after Thomas.

We went to meet with the director this week since Steve was on vacation. I was nervous. I guess on some level I worried that they may not even accept him. Well, I didn't have to worry too much. When we explained Thomas' condition, she didn't seem concerned at all. She said they have had all kinds of special needs there. Tell the teachers what they need to know and they'll look after it. She was quite laidback about it. At first I thought that maybe it was because she didn't get it but by the end of our meeting I think she did. She said they are an "inclusion" centre which I think means that since they get government funding, they can't turn away kids with special needs. (Don't be fooled, government funding doesn't equal cheap. We may go broke paying for childcare.) It's a bit hard to tell someone what to expect from Thomas by the time he starts there because we just don't know. Maybe he'll have a port by then or at some point after that. I did tell her that and she said that was fine, too.

So overall, I felt pretty good about the whole thing. We paid the enrollment fee to hold his spot so he'll be starting in mid-April. When I asked about cloth diapers she actually said that we could just write out exactly how they wanted us to handle them to make things easier for us. HA! Easier for us? I had planned on making things as easy as possible for them by saying they could just fold up the diaper and put it in the wetbag. But maybe I should take full advantage of this and have them dump out the poop!

The director was going to look into applying for the grant. I have no idea how much money it might be but any suggestions for what we should ask them to get with it? She said they have first aid kits in each classroom but I didn't look at them. I was thinking maybe a really good one just for Thomas? Ice packs? I don't know what else...

Why I chose c-sections

I came across a blog called Bubble Wrapped Birth and it got me thinking that I should explain why I made the birth choices I did. I realize most people reading this will be friends and family and will already know why I made my choices. But if you've found my blog while researching the best ways to birth a hemophiliac baby, I have to make a disclaimer here. The following is based on my personal experiences and gut feelings only. I'm not at all suggesting that anyone should make the same choice I did. If you are trying to make that decision, listen to what your doctor says, do your own research, and do what you're most comfortable with. I don't think there's any right answer. With both of my pregnancies I felt that my doctor gave me a lot of flexibility in making the decision I felt best about and I think that was because he didn't have the right answer either.

When I was pregnant with my older son the recommendation for carriers of hemophilia with a male baby of unknown status was to attempt a vaginal delivery, just like anyone else. Internal fetal monitoring (a clip attached to the skin on baby's head to monitor the heartrate), vacuum delivery, and forceps were to be avoided. If, in an emergency, an assisted vaginal delivery became necessary, forceps were considered preferable to a vacuum.

I went through much of my first pregnancy knowing these recommendations and accepting that was my plan. No one had told me it could be any different. I know exactly when I started doubting that plan. I had been a labor and delivery nurse for two years at that point. One day I had a completely normal patient who had a normal vaginal delivery and the baby came out with one arm covered in bruises. Now that I think about it, that baby may have had some kind of undiagnosed bleeding disorder but at the time, all I could think was "what if that was my baby?". There was nothing unusual about her delivery. She had the kind of vaginal delivery that would have been ideal for me but still her baby was all bruised.

I spoke to my obstetrician after that and he felt it was perfectly reasonable if I wanted to opt for a cesarean section. I might not have done it if I wanted more than two or three kids but it was right for me. My doctor and I discussed the delivery and I made it clear that if he struggled to get the baby out I was more than comfortable with him making a vertical incision on my uterus. I wanted my uterus damaged before the baby was damaged.

I had a good experience with my first c-section. Obviously, it's no treat for the first couple of weeks but I had no complications and by the end of six weeks I felt pretty normal. When it came time for my second delivery, I had no interest in trying for a vaginal birth after cesarean (VBAC). We still had an ultrasound to determine gender for planning purposes but it had nothing to do with my decision for delivery. My recovery this time was similar, no complications.

If you really want a "natural" delivery, don't listen to a jaded labor and delivery nurse like myself. I work in a centre where a very high proportion of patients are legitimately high risk. I will admit that it can get you in a mindset where you can see risk in every delivery. I've seen patients go from normal to an emergency in a second. The scenario I would worry about most for myself would be the one in which the head is crowning, the heartrate is down, and the baby has to come out. It's too late for a c-section in that case and that's when a vacuum or forceps might be necessary. Or what if I had a shoulder dystocia? That's a true obstetrical emergency that you can't always predict. I've seen moms and babies both get injured in that scenario, just to save the baby's life. That sounds dramatic and I'm aware that the chances of those things happening in any given delivery are low. But it's for reasons like that why a scheduled c-section is my comfort zone.