Daily/Weekly Thoughts

Emmett had one wish for his 8th birthday party - and that was to launch pop bottle rockets with his grade two classmates at his school. So we did. It was a process that may have taken longer than getting his 29th injection of Spinraza via lumbar puncture just a couple of weeks ago on the 16th of March (which went flawlessly, by the way!) Emmett and I discussed how to talk to the class about how to understand about air powered pop bottle rockets and we decided that we would take a class on Tuesday to have a discussion about air pressure, a class on Wednesday to decorated our pop bottles so they looked like rockets, and then on his actual birthday that we would launch the bottles. And Thursday was a picture perfect day - although still a bit chilly. And 22 rocket bottle launches took place with extreme excitement with each launch.

Happy Birthday, Emmett!

So that was the past few months. The 29th lumbar puncture with Spinraza, the excitement of turning eight, and in a few days a camping trip. Well, we hope there will be a camping trip. Right now the provincial park we are going to head to is closed because of snow! It will be better weather in a few days, right!

Just a short update to let you know that Emmett is okay and is now back home, but so far hasn't made it to see the Christmas lights at Canuck Place. He returned home from a Christmas celebration at school (where it was "the best day ever along with the Terry Fox Run day!") just over a week and a half ago - and that night he wasn't feeling good. After dealing with his illness for several days at home where he just wasn't improving the way he should have been, his mom and dad took him in to BC Children's hospital. There he was admitted to the ICU where he spent that last four days/three nights and was diagnosed with having both RSV and parainfluenza.  Emmett was bored at the hospital - and that is always a good sign when a sick kid is bored. After all, boredom while being sick is a sign that the illness isn't overwhelming. (Now, Alisha and Josh will likely say that this health challenge has been overwhelming enough and we would agree! So we will argue with Emmett about this later on!). No one would say that either of these illnesses are trivial and to be taken lightly - they are rough on anyone let along both at once. And of course, everyone who has kids will realize that Theodore is also sick. So far he's handling it well - we just hope that Emmett and Theodore don't play pass the cold back and forth!

We hope that both boys quickly regain their health. - we have lots to do to get ready for Christmas! This is an exciting year because Aunt Katie, Uncle Josh, and cousin Elias are coming for a visit! We cannot wait!

Thanks to all of you, $3225 was raised for Canuck Place this fundraiser. This brings the three year total to $9450!

Pic 1 was taken in 2018 and Pic 2 was at Halloween in 2021. The pic with the hands was from 2015 and the pic with the orange hippo was another from 2018. The picture with Woody the Cowboy and Buzz Lightyear was taken on October 31, 2022. That is Emmett and his little brother, Theodore.

Once again Emmett is holding a fundraiser for Canuck Place. Emmett welcomes you to join his fundraiser to raise money for this incredible organization. Donations made by clicking the link will be eligible for a tax receipt plus every dollar donated by you will be tripled by matching donations. Canuck Place Children's Hospice is there for families when they are overwhelmed by the extreme medical needs that a child in their family is facing. That is how we met Canuck Place in 2015. Emmett continues to be involved with Canuck Place and we appreciate having an opportunity to give back a little bit to them. There are two locations: one in Vancouver just minutes from BC Children's Hospital and one in Abbotsford.

Want to join the Orange Hippos? Well, you're already an Orange Hippo because you're reading this. If you'd like to support the many children and families who are supported by Canuck Place Children's Hospice, you can click here to make a donation: https://my.canuckplace.org/orangehippos

From an early age, as seen in the first picture above, Emmett has loved the colour orange. His favourite animal has been the hippopotamus. In 2018 there was a fundraising event and Emmett gave our fundraising team the name "Orange Hippos" and it has stuck.

Emmett's Goal for this year is $1000 and with your help he can make that happen. Emmett has raised $6225 over the years for Canuck Place. We think that this is the fourth year for Emmett's Orange Hippo's Canuck Place fundraiser and each year Emmett has gifted one of the participants with a special project. That will happen this year too - and you'll have to wait to see what the special project is! Like almost every 7 year old, Emmett loves surprises!

Click here to make a donation: https://my.canuckplace.org/orangehippos

#28

Did you know that Emmett was one of the first individuals in Canada to receive the drug that is now known as Spinraza? You do know that if you've been following along from the beginning of the blog, but if you've just recently started following then this could be news to you. Today, October 19th, Emmett had his 28th "POKE"! That is right, his 28th lumbar puncture. What is a milestone about this lumbar puncture that took place yesterday is that the the drug trial with Biogen, the pharmaceutical company that owns Spinraza, which is also known at nusinersen, has come to a close. For seven years Emmett has been part of this ongoing worldwide study. Before you jump to conclusions, Emmett will continue to receive the drug and require lumbar punctures every four months but from this point on it will be conducted under B.C.'s publicly funded health care system. What a journey it has been since 2015 when Emmett was first admitted to the experimental drug trial where he was 1 of 124 infants worldwide to participate in this blind study, in which only 2/3's of the participants actually got the drug. At that time Spinraza was known as SMNrx and the company that developed it was ISIS pharmaceutical (later renamed Ionis Pharmaceuticals) which has had a long partnership with Biogen. Emmett was fortunate that he was one of the youngsters getting the drug (and not a placebo) starting in October 2015 when he was seven months old.

Every four months he gets this injection of Spinraza which is like an injection of power juice that allows a secondary path to carry his brain's commands to his muscles. You see, Spinal Muscular Atrophy causes an issue with the nervous system and not the muscles themselves. The muscles atrophy because they do not receive messages from the brain.

Spinal Muscular Atrophy Type 1 first made itself evident in Emmett quite early on - as a newborn he was able to kick his legs and move his arms quite well. By three months he had lost the use of his legs. By six months he had lost the use of 95% of his arms. Between five and seven months he began to demonstrate signs that he was losing the ability to swallow. He got a G-Tube to allow tube feeding. His breathing was compromised so he got a Bi-Pap Machine to assist as it supports his breathing in and out (a C-Pap machine only pushes air in, the Bi-Pap is a two way air flow). Spinal Muscular Atrophy affects all muscles - except the heart.

Spinraza was the first drug (there are now more) that was approved to treat Spinal Muscular Atrophy. The intent of the drug was to stop the regression of this devastating genetic disease but in Emmett's case (and many others) it has actually reversed the effects of the disease. And today's procedure went perfectly and Emmett is back home sleeping in his own bed.

During the drug trial the infants were older and had to be a specific weight to be accepted - Emmett fortunately fit all of the requirements. Today, if the disease is caught early enough intervention can begin almost immediately. As of September 30th, 2022 BC has begun newborn screening for SMA along with 26 other treatable conditions. This is fabulous news and we have been advocating for  BC to include this screening test.

You've been asking what Emmett has been up to recently, and the huge step for the Willm's family is that Emmett is attending Grade 2 at his local public school. After Distance Education at home for Kindergarten and Grade 1, last February Emmett announced that he was ready for public school. He loves being at school and, as you can imagine, has picked up all kinds of things. Playing tag with his classmates, reading with his buddies, participating in the Terry Fox Run, getting colds/flu/stomach bugs! It has been chaotic and exciting all at the same time. In addition to EA support while at school, Emmett also has nursing support to accommodate his medical requirements. It sounds like his support team got quite a workout during the Terry Fox Run. Emmett's power chair got a workout too - it required a full service to lube up the wheels, axles, and hubs after the intense used he's put it through this fall.

In early October we had our regular guys (Emmett, daddy, and grandpa) camping trip and thanks to the drought that BC is experiencing we had our first ever rain-free guys camping experience. It was unbelievable. Campfires, BBQs, games, hikes, stories, and also Theodore, mommy, and grandma came up to the lake to have picnics with us at the beginning and end of our excursion.

Emmett is continuing hippotherapy - you'll remember that this is horse therapy - this year and once again Fiona is his trusty steed. Other sessions for Emmett include speech, physio, and occupational therapy.  Emmett has started reading chapter books on his own and his current favourite series is Mercy Watson: Adventures of a Porcine Wonder. He also loves being read to and recent books include Watership Down, Call of the Wild, Hatchett, Lord of the Rings, and Tom Sawyer. This boy loves books.

Life hasn't been perfect. Like any little kid in school Emmett has gotten colds and stomach bugs - it just hits him harder. And he gives it to his little brother and then they trade germs for a while. Unlike his classmates who might cheer when they hear that they have a sick day and miss school, Emmett is very upset about missing any school time. Also, his treasured orange power chair has been in the shop for service for over two months. Of course, the failure happened literally days after the warranty expired and while we are hopeful that it is fixed soon, no timeline has been provided. Fortunately, we have taken the old but reliable power chair that we use for his power chair soccer events so he is still mobile. Also, he has his "Purple Chair", which is his manual wheel chair that Theodore loves and has just figured out how to climb into on his own. The challenge with the purple chair is that Emmett has grown so much in the last six months that he's outgrown it! The chair company has a 'growth kit' for the chair but Emmett has been waiting over 6 months for that to arrive. His hopes were raised in July when they came with parts to expand the chair - but the expansion kit was missing multiple key parts so he's still using his too small purple chair.

Oh, and just before school we got permission to go out onto the race track in my race car - Emmett loves watching me racing and this time he got to actually experience it!