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.

What to do, what to do...

Steve and I have recently been facing our first decision in reagrds to Thomas' health care. To immunize him now according to the usual schedule or delay it until he's on prophylaxis. It's all about preventing inhibitors. Here's how it works, as it was explained to me:

Now that factor concentrates are safe and the risk of blood borne disease is virtually eliminated, one the biggest complications in hemophilia is the develpoment of inhibitors. An inhibitor is basically like an antibody which causes the person's body to attack the Factor VIII like a foreign substance. For these people the Factor VIII doesn't work any more. Factor VIII is essential for blood clotting so if it doesn't work, where do you go from there? I don't entirely know. There are ways to get rid of inhibitors but no one has explained it to me yet and I'm not about to try to explain it based on my limited research. Long story short: inhibitors are bad.

So, the goal at the moment in Thomas' care is to prevent inhibitors. Our hematologist tells me that there is some evidence to show that early prophylaxis may help. If he receives his first dose of factor in response to a bleed, he's more likely to develop an inhibitor. This is because his immune system will already be hyped up due to the inflammatory response caused by the bleed. In this hyped up state, his body is more likely to attack the factor. This is why the current plan is to start prophylaxis so early. At 6 months he'll be started on regular infusions of Factor VIII. This way his body can get used to it, hopefully in the absence of bleeding.

So what does this mean for immunizations? Well, the immunizations are given by intramuscular injection. This has the potential to cause him a muscle bleed which would need to be treated with a factor infusion. So that's exactly what we don't want. The first infusion in the face of an inflammatory response to the bleed, not to mention the hyped up immune system due to the immunzations themselves. Both these things make his body more likely to attack the factor.

There is another piece to the puzzle. Some people are more likely to develop inhibitors due to their genetics. Our particular gene is associated with a lower risk. Based on this, our hematologist has recommended that we go ahead and do the immunizations now with pressure held to the injection sites for 5 to 10 minutes. Our other option would be to wait until around 8 months of age, when his body has already experienced several doses of factor.

I've gone back and forth so many times on this. I have to admit that I'm as afraid of experiencing his first bleed as I am of developing an inhibitor. I'm sure a time in our lives will come that I'll realize that's ridiculous. Bleeds will certainly be part of our lives and I can't avoid the first one forever. But at this point, I can hardly bear to think of my 2 month old going through the pain of a bleed. Not to mention the trauma of requiring an IV infusion in those chubby little arms to treat the bleed. My first instinct was to delay the immunizations.

Then there is the other side of the argument. What if we delay and he gets whooping cough or meningitis in the mean time? He's home with me but he's got a big brother who brings every germ going home from school. People seem to take vaccines lightly, especially on the internet. If you look around the internet a bit, you'll get the impression that vaccines are evil and no one does it any more. Now that I'm in this position, I just don't get the people who choose not to immunize based on some fear mongering website that bases its "facts" on the study of a doctor who has since been proven to be a fraud. I mean, I'm not sitting here worrying about him catching diptheria but pertussis and meningitis are very real concerns. Kids get these things and they can actually die from them. That's why I just can't empathize with people who are anti-vaccines. If they choose not to vaccinate their kid based on their personal beliefs then "herd immunity" is a little bit weaker, leaving kids who have legitimate reasons for not being vaccinated a little less protected.

Where does Steve stand in all of this? His feeling is that we should do what the doctors recommend but he'll ultimately support whatever I choose. He knows that I drive myself crazy worrying about things. And I can't just defer to the doctors. It was presented to me as a decision so I'll blame myself if things go badly because I made the choice.

So this is where we stand right now. Thomas had his 2 month checkup last week (14 lbs 14 ozs, by the way!) and I did not do the immunizations. I've continued to agonize over it since then so yesterday I made an appointment to get them done next week. My plan is to hold pressure on the sites for 10 minutes, apply ice packs on the way home, and cross my fingers. I'll update after the appointment to let you know if I did it or if I got cold feet.

Diagnosis

I've known for a long time that I was a carrier of hemophila A. My uncle has hemophila and my mom is a carrier. Mom and I both have low levels of Factor VIII ourselves. Mine is 38% which sounds like plenty compared to my son's < 1%. Regardless, I've known for years that I need DDAVP before surgery. Otherwise, it's not something that affects my day to day life.

My older son, Ty, was born on September 3rd, 2004. We found out that he was a boy and we knew that he would have a 50-50 chance of having hemophilia. His dad and I chose not to have any prenatal testing done. It was a stressful pregnancy anyway because I got parvovirus at 18 weeks. I was followed with weekly ultrasounds after that to make sure he was ok. The concern over that seemed to overshadow the concern about hemophilia. When he was born testing cord blood wasn't an option so he had blood drawn at about 1 hour of age. Thankfully, he had normal factor levels.

I'm not a religious person but I have considered that God or the universe or whatever won't give you more than you can handle. When Ty was born, I was not quite 24. I was married but our relationship was on shaky ground. We would go on to separate when Ty was 2 years old and I became a single mother. I was a young nurse, working shift work, my son and I sleeping on the floor at my parents' house before I worked day shifts so my mom could get him to day care in the mornings. Could I have handled a child with hemophila then? Not very well, that's for sure.

So, fast forward to 2010. I was remarried. My husband, Steve, is very much a grown-up. Level-headed, responsible, a real caretaker to Ty and I. After losing a tube to an ectopic pregnancy that summer, I got pregnant again in early September. Steve knew I was a carrier and he knew we had options in regards to prenatal testing but it wasn't something we talked about much. I considered myself extremely lucky to be pregnant again so quickly after the ectopic. I certainly wasn't about to choose to end a pregnancy at that point so any testing was really a moot point. So, on we went.

The first trimester felt very different from my pregnancy with Ty so I was pretty sure this was a girl. Not so! At our 18 week ultrasound it was confirmed that we were having a boy. Again, we decided not to do anything further. We did meet with the pediatric hematologist so we would be on her radar. Our hospital had recently made new guidelines for delivering carriers of hemophilia so everything was clearly laid out. I would have an elective repeat c-section and Thomas' cord blood would be tested. We would have the results within a few hours of his birth.

All this preparation and for some reason, I never pictured getting bad news. When I imagined his birthday, I imagined them coming to me with his results. He would have a full supply of Factor VIII and we would all hug and cry tears of joy. After having Ty, I really believed that I would win the lottery twice. I feel bad talking like that because I wouldn't trade my sweet baby for anything, hemophilia or no hemophilia. But still, the news was devastating.

The cord blood clotted and the test couldn't be done so Thomas had to have blood drawn. Not a heel stick but a real blood draw, from his elbow like an adult. This is really rare for normal newborns and strangely enough, the only time I've seen it done in my 8 years as a labor and delivery nurse were on my sons. Two lab techs came over to do it. It took two attempts and the lab techs commented that it was a good sign that the tourniquet wasn't leaving bruises on his arms. I breathed a sigh of relief, he was ok. I still pictured nothing but good news. I finished my time in the recovery room and just as we were about to be transferred to our postpartum room, the phone rang. My nurse, co-worker, and very good friend Amy answered it and we listened intently to her end of the conversation. It was my doctor. Yes she had the results but no, she didn't want Amy to relay them to me. She wanted to speak directly to me. I was laying on a stretcher, my legs still frozen from my spinal anasthetic and no phone nearby. Amy explained this to my doctor and she still wanted to speak to me. This was bad news. Amy wheeled my stretcher over to the phone. My doctor told me "Your baby does in fact have hemophilia and it's severe".

Severe is such a harsh word. You don't usually hear people talk about severe diabetes or severe epilepsy. But there is a very big difference between severe hemophilia and mild or even moderate hemophilia. My heart dropped when I heard it. How could I be so shocked? We'd gone over this with obstetricians, hematologists. I knew the statistics and I knew that my particular gene was one that would result in severe hemophilia, just like my uncle's. It still felt like the wind had been knocked out of me.

When we got to our postpartum room, a resident came to see us. She started with taking a family history and didn't even acknowledge the diagnosis. Then when we got into the specifics about Thomas she asked if we were planning on circumcision. We weren't and she said that was good because he could die from that. Omg... She needed some work on her communication skills.

They decided they wanted to do a cranial untrasound right away. They needed to rule out a head bleed from his birth and since it was a Friday afternoon, time was running out. It would be too hard to get it done on the weekend. It was arranged within a few minutes and Steve and a nurse took him to have it done.

While they were gone, my parents showed up with Ty. Of course, my mother's first question was "where's the baby?". I told them that we had a positive diagnosis and he was gone for an ultrasound of his head. Mom's eyes teared up and Dad groaned with disappointment. I don't think there was any reaction from them that I would have deemed appropriate in that moment. I snapped. I said something along the lines of "we're not talking about this right now". That was the first of many times that I would feel defensive. I was already in the mindset that this would all be ok and we could handle it. I wanted to be celebrating my baby's birth not mourning the fact that he had hemophilia.

Thomas is 2 months old now and hasn't had so much as a bruise. I'm told that's normal, even with severe hemophilia. The current plan is to start prohylaxis at 6 months. There's a lot to learn in the near future but we're feeling positive about it. Steve and I believe that this boy was given to us because we can handle it.