Category Archives: Hemophilia

Where did this come from?

Hemophilia, I understand. I get the genetics of it. I’m not quite sure if I mutated or if we’ve had it in the family all along, but at least I understand. I know that 1/3 of all cases are spontaneous mutations. My mom only had girls. My grandmother had 5 girls and 2 boys. My great grandmother had 3 girls and 1 boy. Of course, there should have been some carriers in there to have sons with it. But, I only have 2 boy cousins from biological aunts on my mom’s side. Again, a carrier should have shown up in there somewhere. My nephew doesn’t have it. My niece might  be a carrier, but she’s not been tested. So, it looks like the buck probably started with me. But I have the tools and to figure that out. It gives me an idea of how I ended up with two boys with hemophilia.

I don’t get type I diabetes. Here’s what I found on the American Diabetes Association website:

Type 1 Diabetes

In most cases of type 1 diabetes, people need to inherit risk factors from both parents. We think these factors must be more common in whites because whites have the highest rate of type 1 diabetes. Because most people who are at risk do not get diabetes, researchers want to find out what the environmental triggers are.

One trigger might be related to cold weather. Type 1 diabetes develops more often in winter than summer and is more common in places with cold climates. Another trigger might be viruses. Perhaps a virus that has only mild effects on most people triggers type 1 diabetes in others.

Early diet may also play a role. Type 1 diabetes is less common in people who were breastfed and in those who first ate solid foods at later ages.

In many people, the development of type 1 diabetes seems to take many years. In experiments that followed relatives of people with type 1 diabetes, researchers found that most of those who later got diabetes had certain autoantibodies in their blood for years before.

(Antibodies are proteins that destroy bacteria or viruses. Autoantibodies are antibodies ‘gone bad,’ which attack the body’s own tissues.)

Let’s break it down.

  • Need to inherit it from BOTH parents. Neither of us have any family history of diabetes in our families anywhere, that we are aware of. This explains why I was met with dumb founded stares every time I said we had no family history.
  • Triggers of cold weather or a mild virus. Well, we had the most mild winter ever this year. Well, almost. Certainly more wild than we’ve been accustomed to in Illinois. A virus? I guess it’s possible, but she never really showed anything. And I think triggers work in conjunction with the genetic factors anyway.
  • Early diet? Really??? Girl was breastfed until 19 months. Earlier than other kids weaned, but there were extenuating circumstances at that point. Besides, that should have been way long enough to reduce her risk. I did start solids earlier with her than some of the others. But even so, she was 6 months old. I only did it then because my pediatrician was kind of bullying me into it. Again, 6 months isn’t 6 weeks, which is when her older sister started cereal (20 years ago, doctors thought differently than today).
  • So that leaves us with the autoantibodies. My personal belief on this is they are caused by vaccines. Hannah was vaccinated until 6 or 9 months of age. Can’t remember exactly when her last shots were. When I was a kid, I seem to remember kids were diagnosed with diabetes in the 8-12 year old range. Now, it’s very common for it to happen at around age 1, and again in kindergarten. Huh. What’s happening around those ages? Oh, I know! Lots of shots. Yes, I realize that correlation is not causation. But just in case? Makes me glad we decided to stop vaccinating.

Ok. Off my soap box. I haven’t even really been researching long enough to have one. But I just feel so lost since I can’t trace this to its origins. I’m the kind of person who needs to know why for everything. In fact, I even got a talking to at work during orientation as a result. My preceptor thought I was criticizing their policies/procedures because I kept asking why and bringing up how previous employers had done things. It’s just my method and need to understand. I’ve never been one to follow blindly. My hematologist in Illinois can attest to this as well. I don’t really do anything “by the book” where hemophilia is concerned. I picked my own prophy schedule (we infuse 2x/wk instead of 3x/wk). I adamantly refused the hepatitis B vaccine for the boys (seriously. You can’t brag about how no one has acquired HIV or Hep B from factor products for 20 years, and then convince me I need to vaccinate against the risk of Hep B from factor products; and now, we use factor that never even touches human blood products).

Well, off to do more research. I have to get to the bottom of this.

D-Mom

This is what I’m called now, apparently. I acquired that title Monday. I get to tack it on to “hemo-mom.” That’s hemophilia. I have two boys with severe hemophilia, so I’m no stranger to life long health issues that require lots of needles to manage. Well, in the case of hemophilia, it’s only two needles a week, but they have to find a vein rather than just some skin. I used to have people compare hemophilia to diabetes when talking to me. Like they thought they had some understanding of my life because their child had diabetes. I scoffed. Yeah, right. Pricking a finger or sticking a tiny needle into a belly is does NOT compare to trying to get a larger needle into a vein, digging around, having to stick again (6 was the most times I ever had to) trying to find a vein. The whole time, my child is fighting and screaming at me as I torture him. Many infusion days ended in tears for both me and my son. Yeah. Diabetes does not even come close.

Except now, we have diabetes. I am thankful that she’s old enough that I don’t have to fight with her the way I did my boys. I am also thankful that as an RN, I do have some small working knowledge of how this works. I’ve cared for patients with it. I’m not anywhere near as green as I was when I was handed the diagnosis of hemophilia.

But, man, this is still terrifying! I worry about her constantly now. I worry she’s going to collapse walking home from school I worry about everything. This sucks.

So, we had her appt. with the endo (that’s endocrinologist for the uninitiated, the specialty that manages diabetes) today. It was a lot. She got her meal time insulin added to the routine. She also added three more fingers sticks, at least for now. So, we are currently at 12 sticks a day – five insulin and 7 finger pokes. We expect to be able to drop one insulin and hopefully 3 finger pokes after her next appointment (in 10 days).

At the appointment, they gave her more of the long lasting insulin, which she’s been taking at night, and her first dose of the meal time insulin to try and catch up to what she had for breakfast. By her pre-lunch BG (blood glucose, the finger prick) was down to 152! That’s as low as we’ve seen. Her fasting (first thing in the morning) has been in the 200+ range. Our goal for right now is 100-200. I was so excited. After lunch, it was still under 200. Woo hoo!! Then, tonight, before supper, it was 348. Sigh. This is what it is, I guess. Up and down until we get her stabilized. So, a call to the nurse practitioner with instructions to tack 3 more units on to her supper insulin. The NP gave me her personal cell phone number, by the way. I’m to call with any questions, day or night. I heart her.

I’ve been poking her in the upper arm. Tonight, I suggested we try her thigh because she can reach that to do it herself. She was very concerned. She’s very thin. Finding enough fat to stick her in is tricky. But, we did it and she decided it was actually better than the arm. So, one step closer to self injecting. I have to work tomorrow night. Not sure how that’s going to go with her bed time shot. Maybe during the 4 all day long, she will be able to do it herself by the end of the evening.

So, back to the hemophilia vs. diabetes thing. Yes, getting meds in is WAY harder with hemophilia. But, at least with the boys, once they get factor, I know they are good to go for around 48 hours. I can totally let go of any concerns for bleeding. Normal concerns of course. If ANY of my kids slammed into a brick wall, I’d do something about it. But as far as everyday activities, no worries. But with diabetes, while meds are easier, if more frequent, the worry never goes away. Before she’s treated, I worry she’s too high. After she’s treated, I worry she’ll go too low. And, at any time during the day or night, she could react one way or the other. Apparently, kids die in their sleep from this. Thanks other blogger, for putting THAT fear in my head. If I were to pick one or the other, I’d pick hemophilia, hands down. But I don’t get to pick. God has seen fit to allow BOTH in my life. And so it goes….

Oh, and happy world hemophilia day.