Jake Turns Five

As a quick update from my last post Jacob has been doing much better. It seems the botox made an improvement and he’s been in less pain. It does seem to be wearing off, but it is effective. It’s been tough to really guage things lately because of Jacob’s behavior. He seems to get mad at Marcy and me and will scream sometimes for hours on end. He doesn’t do this at school or even with Gina, his nanny since he was first born. However, other part time care givers aren’t so lucky. A very sweet PSW has been working with him one afternoon a week for a couple months. But as soon as he sees her, he starts up and doesn’t stop till he goes to bed.
So was it pain that was bothering him? Was it behavioral? Since a little advil made such a difference, I believe pain was definitely playing a role. However, it’s equally clear to me that he’s giving us a piece of his mind. It hurts watching him when he’s so miserable and angry. 

Meanwhile, he just had his fifth birthday which has been a critical milestone in our minds for several years. Typically, a childs brain develops during the first five years. We also found that Jacob has been developing, albeit slower than other children. Our theory is that his brain has been developing faster than the disease has been progressing and that’s why we’ve been seeing steady progression. In our minds, we gave ourselves a target of his fifth birthday to get some treatment into him while his brain is still developing. This would allow him to develop his brain as much as possible to set him up for the rest of his life.

Sadly, although we first got wind of the experimental drug we’re working with three years ago, we failed to meet our target. We failed Jacob. We have loads of excuses, mostly because we rely on so many others that we cannot control. In fact many of those doctors, researchers, etc. probably also have loads of excuses why they were too busy to make a phone call, or read an email, etc. These excuses will not buy Jacob any increased brain development and are therefore completely irelevant. We failed Jacob.

We need an Ontario MD to help. We want to develop a safe protocol so that we can start Jacob on this drug. We’d like to be able to do this in hospital to mitigate the risks associated with potential unexpected side effects. We’re not wreckless here. We’ve done research into the disease, the drug, and we even funded a scientific study to experiment with the drug in animal models of Jacob’s disease. Right now, given the current state of PMD related research, this drug is Jacob’s best chance for improved quality of life.

Having said that, we’re cautious about the risks and we’re open to disprove our theory if it’s in Jacob’s interest. However, vascilation is unacceptable.

How do you get this done in Canada? Money is not the answer. What’s the problem? CYA. Nobody is accountable to help kids like Jacob. On the other hand, they’re all afraid he might get worse. And that is all the rationalization any of them need to sleep sound at night. If only they had to spend one night at our house.