It's finally out!

It's been loose for months. We've wiggled it every morning for weeks. We've shown it to everyone around. Jacob's front tooth fell out tonight. And it couldn't have been timed any better! Around dinner time, Bracha called unexpectedly to see if she could stop by for a quick visit with Jakey. As we were finishing our supper, I heard someone call me from upstairs. It wasn't one of those panicky calls, but there was something in the voice that made me head up to check it out. Sure enough, there was Jacob, in Bracha's arm, with her other hand using a kleenex to dab at his mouth, his tooth in her hand. While we were very excited, Jacob had the strangest expression on his face; it looked like he didn't know how to react - should he laugh, should he cry? But the more fuss we made, the more he seemed to enjoy his new source of attention. And to make it an even bigger deal, this happened when his Bubby and Grandpa were over for dinner.

What is ok to ask?

People often ask how Jacob is doing and I'm not really sure how to answer them. Should I simply say "he's doing well, thanks for asking" or should I tell the truth, which might make them a bit uncomfortable? I believe most people really want to know how he is and truly hope I have some great, or at the very least, good news to report. Sometimes when I give some detail as to what we've been going through lately, the 'asker' gets very uncomfortable and there is an awkward pause because the response wasn't what was expected.

For those of you who are unsure of what is ok to ask, you should know that both Andrew and I welcome your questions and interest. There is very little about Jacob's care and illness that we don't discuss. If you are curious and want to share in his life, then thank you for your concern and please, ask how he's doing. Most of the time you'll get the current status, it's too tiring to try to hide the details.

Without going into too much detail (only because that's not the point of this entry), Jacob has been having a rough time lately. We're not sure what's going on but we're in the process of investigating a few avenues and hope we can get to the bottom of it soon.

To our friends, thank you for taking the time and interest in our son and his progress. We truly appreciate your involvement.

Brief update

Well, it's been a long time since we've posted an update on Jacob. He had a great summer, (with the exception of low-grade fevers almost every week and a quick trip to the ER for an ankle xray) and school is off to a good start. And he's lost his second tooth, with a third one wanting out too!

During the last 2 weeks of summer, Jacob attended Camp Robin Hood with his best pal Kelly by his side. It was there he met Taryn who quickly decided Jacob was her #1 best friend. After a few short days she changed her mind and Jake became her boyfriend. As you can see by the picture, they make an adorable couple! They had their, according to Taryn, "first date" in September when they drank some milk together (Taryn from a cup, Jake through his tube). Taryn is an amazing little girl who loves playing with Jacob and does a great job of making him smile.

Jacob lost his first tooth!

It was loose since his birthday, and on Thursday night (July 18), Jacob's loose tooth fell out! He was showing his 'Poppy' how he can wiggle his tooth with his finger (with mom's help) and the next thing we knew, the tooth was out. Jacob was so excited, and a bit surprised with the hole it left behind. When he moves his tongue to the spot where his tooth was, he gets a funny look on his face as if to say "something feels different here".

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.

Speech given by Marcy at Zareinu Golf Tournament May 29,2007

Like most parents, I love talking about my children so when Randy asked if I’d speak to you about my son, I welcomed the opportunity.

My son, Jacob, is an adorable 5 year old with a great sense of humor. Like most boys his age, he loves the rides at Canada’s Wonderland and funny noises.

Unlike most kids, Jacob can’t sit by himself; he can’t walk, can’t eat and can’t speak. But when he’s with his teachers and therapists at school, the only focus is on what he can do and what his latest achievements have been.

The first few years of Jacob’s life are like very blurry images in my mind. It was a very difficult time, especially when we didn’t know what was wrong with him. One of the clearest memories I have of that time was walking into Zareinu for the first time. I came to check out the Infant Intervention Program to see whether it would be appropriate for Jacob. This stands out, in my mind, as one of the most difficult things I have ever done. The staff could not have been warmer or more welcoming but all I could focus on were the wheelchairs and funny-looking equipment in the hallways. I remember not wanting to stay. I also remember realizing that Jacob needed to come and benefit from what they were offering.

Jacob started coming to Zareinu when he was 16 months old. Because he has a weak immune system, we were worried that his exposure to other kids would be very dangerous for him. Ellen understood this and set aside toys that were for Jacob’s exclusive use while he was in the program.

Jacob graduated from the infant program and moved into the nursery classroom where he has been for the past 3 years. Jacob gets so much love, attention and therapy, more than I could describe. His teachers joke that he should have a pager because he spends so much time out of the classroom, either having 1:1 therapy or zooming around the halls in his powerwheelchair and walker.

The African proverb “it takes a village to raise a child” is so true for us. The staff at Zareinu have become part of our family. They share our joy at Jacob’s accomplishments, worry with us when he is sick, and for the past 2 years, celebrate his birthdays with us at our home.

Jacob is an inspiration to so many around him. He works harder in a day than most people do in a week, and he never gets frustrated. He tries so hard and gets really excited when he masters a task. Jacob gets the opportunity to learn, interact with other kids and staff and really thrive at school.

When I pick him up at the end of the day, I don’t notice the wheelchairs in the hall anymore. I only notice the big smile on my son’s face.

Pain

How do you know when your child is in pain? Well, maybe we're not the swiftest because it took us several weeks to figure out that Jacob was in pain all those difficult evenings. It's a bit embarrassing how many smart people, parents, therapists, doctors, etc. it took to realize that Jacob was in pain due to spasticity.

I guess we knew this day would eventually come, but we hoped that Jacob would be different. We hoped that with all the special therapy, nutrition, and experimental treatment would somehow protect Jacob from the inevitable effects of this degenerative disease. This may be the first lost battle in the race to treat Jacob before the degenerative effects catch up. Clearly, we can't afford to lose any more battles.

On the bright side, we finally got to the local botox clinic yesterday to help relieve Jacob from the pain from spasicity. We've had him on pain medication for the past couple weeks which has certainly helped. In a few days we'll wean him off these meds in hopes that the botox will be enough to do the trick. Jacob got eight shots in his calves. It will take some time to take effect and we may have to do some more muscles to relieve his pain. In any event, it looks like we're back on top of this one.

Time to get back to the bigger picture and get some experimental treatments underway. Next stop, seizures. What will we do? Do we treat him for seizures even though he doesn't have clinical seizures? Looks like we need some more investigation.

Maybe we'll try anti-seizure meds. Maybe we'll get to try the experimental treatment in a hospital. Maybe we'll do both together. Keep your fingers crossed for our little Jacob!