If you've been wondering where we've been, you can relax. No news has been good news. Emmett has been working diligently in kindergarten in his distance education program (which takes place at home). He loves reading. He's pretty good at mathematics - especially addition and subtraction. He endures art and crafts. He loves excursions and outdoor/garage projects. The biggest project that we had this spring was building a cardboard box boat. From the beginning Emmett was quite clear that this boat had to be for both of us - that he would not go in it alone. As you can imagine this led to a lengthy search to find the appropriate box. Which was discovered at a fitness store - the right size and very sturdy. Sturdy was needed to hold the weight of both Emmett and I - this little boy is growing like crazy. He's almost 120 cm/4 feet tall and his weight is getting up there! (and my weight is super way up there!)

The construction of this craft took place initially at AES Auto in Burnaby (I picked the box up at the fitness equipment outlet next door) as I had to cut the box down to fit in our tiny SUV. The rest of the build took place in our garage. Emmett helped by first ensuring that we cleaned up the workspace and then offering suggestions. And holding the duct tape. Emmett was impressed that we used almost an entire roll of duct tape. Since we weren't building for a competition we added large plastic recycling bags for added waterproofing - and this was likely a wise addition to our build. But that is enough discussion. It's time for the video!

After you've finished this video, maybe you'd like Emmett to read you a story? Get comfortable, this is a long clip!

Emmett is SIX today - on March 9, 2021.

Birthdays are very special. Emmett's birthdays are even more so. Last fall when it was obvious that the pandemic was going to have an impact upon our birthday celebration, we were not worried. Because this birthday the big surprise was to be in a big truck! And as Emmett's grandpa Roger works for Valley Carriers in Abbotsford, we had access to lots of big trucks. Emmett got to go to Valley Carriers' yard - when everything was closed down - and race around on his power chair and look at all of the heavy equipment. Grandma Rhonda had an amazing cake that was designed as a work area with trucks and heavy equipment. Uncle Ben ran the loader and put topsoil into the dump truck, and then Emmett got to ride in the dump truck (fully secured in his car seat, of course). Then he got to watch the dump truck dump the load onto his driveway. Then he got to work with the dirt (it was more than play and no, we really didn't get in the way. At least, not too much!) All in all, it was a fabulous 6th birthday!

Happy Birthday, Emmett!

#20 - March 3, 2021 is when Emmett received his 20th injection of Spinraza by lumbar puncture.

Also on March 3, 2021, Emmett's Aunt Katie and Uncle Josh had a baby boy who is named Elias Mark. Elias lives in Australia! Elias, mom and dad are now at home and all are doing well!

At BC Children's Hospital, everything went well. As you can appreciate, the POKE (as Emmett calls a lumbar puncture) not a highlight of Emmett's week, but he did enjoy seeing a lot of his favourite people at BC Children's Hospital. Plus, he got presents - a Woody the Cowboy and Buzz Lightyear figure set! He doesn't have a dozen of each yet, and he loves them! Seriously, the little kid who has been Woody the Cowboy for the last four Halloween's loves Toy Story toys and he will never have enough!

But enough of that. Did you know that Emmett is a soccer player. Did you know that 2 days after his lumbar puncture that Emmett was out playing soccer? Well, not on a team yet but maybe that will happen soon! You know how last blog we showed you a picture of Emmett playing soccer with a cardboard soccer bumper. Well, that proved to be a bit more dangerous than deemed safe. We were going to rethink our approach when we found out about Sportsablitly BC and we reached out to them. They have provided us with a loaner wheelchair soccer ball and a universal power soccer guard - one that won't allow the ball to go under the chair and risk causing a roll-over (that did not happen with the homemade cardboard one - but it was close!)

Huge thanks to Cheryl who introduced us to Jade at Sportsability BC, Emmett was very patient while Jade was hooking up the universal soccer guard to his power chair, but as it was almost bolted on Emmett said, "Let's Go! I want to play soccer!". And while it was a wet and windy day, we went out and had a lot of fun playing soccer. It was fantastic. Sportsability BC - thank you for making a little boy's day!

March 9th is Emmett's 6th Birthday. Stay tuned because you know that we will be celebrating - in a very pandemic responsible manner!

February 28, 2021 is Rare Disease Day.

If you know this, then you are one of a minority. Six years ago I had no idea that there was a day that sought to focus the attention of the world on rare diseases.  There may be as many as 7 000 rare diseases that affect millions of people around the globe. These rare diseases may not be well known, but their impact is significant.

The most common characteristic of a rare disease is the small number of people that it affects. For example, in Europe a disease is considered rare when it affects fewer than 1 in 2 000 people. That's 1 000 people in a city of 2 000 000. That is a small group, but if you are one of those people then you know that it is a significant number. Rare diseases are often mis-diagnosed, or not diagnosed at all, by medical professionals. These rare diseases often have limited treatment, if any, and this leads to long term challenges for the health and care of individuals with rare diseases. And their families too.

Emmett has Spinal Muscular Atrophy, Type 1. It is a rare disease. In the first half of 2015 our families were not aware of it and had never heard of it before. Now we have. Emmett arrived in the world on March 9, 2015 but his diagnosis was not made until that August. There was no treatment available in 2015 BUT there was a drug trial and Emmett met all of the requirements for admission. This drug, now called Spinraza, worked. Emmett's disease does not allow the messages from his brain to be transmitted effectively to his muscles. So basic things like walking, holding things, supporting his own head, sitting up, swallowing, breathing were at risk. When he was seven months old he could not roll over, move his legs, hold up his head, sit up, and was losing the ability to move his arms. He was having trouble swallowing. He had to be lying down as a sitting position would cause him to choke. Sometimes when I say or write this, people think that I'm exaggerating. If I am exaggerating, it is probably reverse exaggeration. I am understating the challenges that Emmett and others with SMA Type 1 face. That is, this disease is probably worse than what you're reading in this blog.

The benefits of Spinraza are not something that are understated. The trial was a success and the drug was approved in Canada, the United States, and many other countries. Two other newer drugs have been approved by the FDA in the United States and hopefully we will see them available in Canada also. But don't relax and think that all is now a rose garden. The physical improvements that Emmett has demonstrated over the past five years are fantastic; from the drug and the fact that he works incredibly hard for all of his gains. His mom and dad do daily physio sessions with him; he has regular speech therapy sessions to work on tongue control, and he has occupational therapy sessions to enhance his large and small motor skills (by the way, during this pandemic season all of these sessions are currently online). For example, he can now hold himself (while wearing his back brace) in a sitting position with no back or side support for almost forever. While lying on an exercise mat he can roll from his left side to his right side - and if it wasn't that he intensely dislikes being on his tummy, he could probably roll right onto his tummy. He can swallow tiny amounts of liquid and pureed food; he absolutely loves tasting every kind of food and his eyes absolutely light up when someone mentions ice cream. Emmett loves painting and drawing but we have had to find the right tools; he cannot use regular children's wax crayons as they require too much pressure to leave marks on paper.

What do you need to do for rare disease day? The starting point is to be more aware. That you're reading Emmett's blog is a sign that you are becoming more aware. Thank you. You can also check this link about rare disease day.

On March 3, 2021 Emmett will be at BC Children's Hospital for his 20th Lumbar Puncture. On March 9, 2021 he will be celebrating his sixth birthday.

February 28, 2021 is Rare Disease Day.