When you live in the B.C.'s Fraser Valley, you know that the fall means rain. And not just a few drops here and there. Not a large cloudburst and then some blue sky. But day after day of deep grey skies, filled with clouds loaded up with moisture, moisture that never seems to stop falling from the sky. This seemingly endless precipitation provides the perfect opportunity for a water study. Therefore, Emmett is studying water. When we are outside going for walk/rolls or bike rides, we study the rain. For example, yesterday we discussed how rain flows downhill. We saw and studied how the fall leaves got stuck at drain grates and caused the water to build up and find a way around the leaf dam. We laughed together when we broke the dam and then watched the flood dissipate and heard the water crashing down into the sewer. We would have lifted the grate and looked down it, but only a city worker can lift the grate and Gerry, our friend the city worker, wasn't available. (**Please do not tell Emmett that anyone can lift a sewer grate. He already has gone through phases where he requests every sewer grate be lifted, and our response is that only city workers can do that. Fortunately, Gerry the city worker has slightly appeased Emmett's curiosity by lifting the sewer grate for him.)
At the Great Blue Heron Reserve we participated throughout October and early November in a weekly morning outdoor educational session in which our guide, Michael, taught us about water bugs, showed us the many different types of fish that live in the local streams and rivers, discussed all of the birds that depended upon the wetlands for food and shelter, and how animals like the beaver both enhanced the wetland yet perhaps caused issues by blocking pathways used by salmon as they returned to their home stream. With his mom and dad earlier in the fall we played on the beach of a lovely lake that had been created by hydro-electric dams and we discussed how these massive dams created electricity that powered his food pump, bi-pap, oximeter, his dad's coffee maker, and so much more. As a result we have been learning about water and the importance of water for all of us for several weeks. Also, I'm pretty sure that I used one of Emmett's syringe's to spray him with water a few times. Of course, we have to tell you that Emmett loves bath time and 'swimming' in the bathtub.
So with this focus on water and these deep grey clouds overhead, it only made sense to study the water cycle. To do so we went to the recycle bin and resurrected the pop bottle rocket. While it makes sense to do a rocket launch comparison in sunny weather versus rainy weather and see what type of weather is most conducive to rocket launching, that is not what we did. The pop bottle rocket has been retired after several launches and isn't going back into action. We saved most of the customizations for flight that Emmett had done (that is, stickers) but that was all that was left of the rocket. We then used a sharp knife to cut the top off of the bottle. This top part needs to be large enough that, when you invert it, the top friction fits into the bottom of the bottle. Like a funnel. Then we used a ruler and a permanent marker to put a measuring gauge on the side. We did centimetres. This took not much more than 5 to 10 minutes and it was the easy part. The hard part was getting Emmett to select a location for this rain gauge. He quickly discounted the front yard, as he knew that something as valuable as a pop bottle rocket converted to a rain gauge would be at high risk for theft. While he liked the idea of putting it on the back porch because he could get to it easily, he also knew that this would not get much rain because the back porch has a roof. So then he settled on the roof. That's right, he wanted his frail and elderly grandpa to put this rain gauge up on the roof. Meaning that every time it needed to be checked, his wobbly and fragile grandpa would have to climb up the ladder and go onto the roof. Well, I refused that option. Emmett laughed and then we settled on the backyard, right where Emmett could easily see it. Within the first three hours our rain gauge gathered about 1 centimetre of water. After 24 hours it registered 2.2 centimetres of water. As there has been nearly 22 centimetres of rain recorded in Abbotsford for the month of November, our rain gauge is fairly accurate.
While we were collecting rain outside, Emmett and I decided to make it rain inside the house! Emmett's first plan was to take the roof off of the house, but his mommy and daddy said, "NO WAY!" so we couldn't do that. Instead to see how the water cycle works we filled the electric kettle with water and turned it on. Then we filled a bag full of ice. Then when the kettle was close to boiling a grownup held the bag over the boiling hot kettle spout. The kettle was like the heated up earth and the water vapour going up was real. When it hit the bag of ice, that was like being high in the atmosphere and hitting cold air, so the water vapour turned to condensation. When the condensation got heavy it turned to rain! And we made it rain in the house without taking off the roof! We didn't even need rain coats.
This last part has nothing to do with water and rain. It is a reader's theatre that Emmett created with grandma and grandpa. One of our critics pointed out that Emmett wasn't reading everything and that is true. He had a bit of stage fright and anticipated his lines, but I still think that he did a spectacular job. So without further ado, here it is and we hope that you enjoy it. Mo Willems, let us know what you think!
This is Emmett's fifth anniversary of the start of Spinraza, which in 2015 was known as SMNrx. This name changed to Nusinersin and now is best known as Spinraza. Emmett had the first of many lumbar punctures on October 7, 2015. This fifth anniversary post got postponed as BC Children's hospital had to reschedule the October 2020 until November. This LP, #19, will take place on Monday, November 2, 2020.
EDIT: Everything went well today at B.C. Children's Hospital. Emmett will be home soon.
There was no treatment available when Emmett was diagnosed with Spinal Muscular Atrophy, Type 1 five years ago. Emmett, however, was just the right age and weight in the fall of 2015 to be accepted into a study for a pharmaceutical drug trial. It was a drug that had a lot of promise but with a drug trial there are so many questions and very few answers. This trial would involve 124 similar aged babies from around the world. All with one common feature - they had SMA Type 1. SMA is the number genetic cause of death for infants - the trial for Spinraza was brought to an early close as those on the drug were improving and those receiving the placebo were failing - they were then given the drug. It was approved by the FDA and Health Canada afterward.
To understand how much Emmett has progressed, you need to realize that Emmett had lost all movement of his legs by 3 months of age. By 5-6 months of age he'd lost the majority of movement in his arms. He could no longer grasp things with his hands and fingers. He'd never been able to hold his head up. Obviously, he could not sit up on his own. Travelling had to switch from a car seat to a car bed - as the seated position caused too many issues with choking on saliva. All nutrients and liquids were by G-Tube (gastronomy tube) as swallow studies revealed that he wasn't swallowing properly. There were a trip or two to the emergency room due to choking/stopped breathing. October 2015 Emmett was accepted into the trial for this drug - it was a blind study in which 2/3 of the children would be administered the drug while 1/3 would get a placebo. Apart from the fact that even with a baby it's difficult to fake a lumbar puncture, small signs of improvement told us Emmett was getting the drug. You see, with SMA an individual may level off in their decline but there was no medical record of any individual with this genetic disease showing improvement. Emmett was improving.
Fast forward five years to 2020. There are now multiple treatments available for SMA. The first is this drug Spinraza, the treatment that Emmett is on, which can be administered to SMA patients at any age. After the initial dosing, it is administered every 4 months by lumbar puncture. Babies who receive this treatment immediately (days old) are walking, eating, and demonstrating incredible movement. There is the gene therapy treatment that has been getting a lot of press lately. It is the $2 million US dollar one time gene therapy treatment called Zolgensma that is for children under 2. Then there is the oral treatment that is taken daily called Risdiplam (Evrysdi). Of these three which are all approved in the USA for use, only Spinraza is approved in Canada. And in Canada provinces still limit, due to financial implications, who may receive Spinraza.
Emmett can hold his head up. He can wheel himself around on smooth, flat surfaces with his manual wheelchair. He can hold a pencil or marker and print his name. When placed in a sitting position, he can maintain it for a considerable length of time. He loves telling stories and playing jokes on people. He loves to have ice cream - he takes very tiny spoonfuls and can feed himself. However, his suction machine is always within reach as choking is still a significant concern. Emmett can usually tell us when he requires suction. While infrequent, there are moments when intense suctioning is required and everyone's heart beats hundreds of times a minute. Once cleared, Emmett returns to play instantly. He's fine, even if we may need a few minutes to recuperate.
This is the fifth anniversary of Emmett being on the drug Spinraza. To celebrate he is getting another lumbar puncture on Monday. More of this drug which has changed his life. More stories of his joy of living to share.
Within our family social bubble, we had a Thanksgiving celebration. Turkey, dressing, and all of the rest for our meal. Fantastic cream puffs that Emmett helped make for dessert. It was memorable. But what was outstanding was our games event that took place between the fabulous main course (that Josh engineered) and the Emmett touched cream puffs for dessert. That is when Josh and Emmett introduced us to Carcassonne. Emmett had played this game once already. Just once. But when Josh started explaining the game by showing a card to us newcomers, Emmett interjected and told us what the card meant and how you played it. So from that point Josh just showed the card and Emmett provided the game play rules. It was fantastic. We are thankful, this thanksgiving season, for a little boy who is so aware of his surroundings, takes in everything, and simply loves life!
In the September pics of the first day of Kindergarten Emmett still had one front tooth. This Thanksgiving Emmett has no front teeth and, while the adult teeth are hiding in his gums, it's hard to say when they'll actually make their appearance. Kindergarten has been a lot of fun and Emmett absolutely loves school. As mentioned in the last post, Emmett's kindergarten is a distance education program through Oak and Orca, which provides guidance and support to enhance the education that parents provide. It is going quite well. We have had excursions to the Great Blue Heron Reserve and have been to an apple orchard and a pumpkin patch. The 'we' is intentional, as school is truly a family affair plus, due to the challenging pandemic situation and the lack of social involvement in an actual school setting, everyone in the family is involved. Even if that involvement is story telling via video conferencing. With school we are pleased to say that Emmett is doing very well with his sight words and basic math facts. He is number one in his class - although we're not keeping track. Too often people make assumptions about individuals who are in wheelchairs or whose speech may not be perfect; Emmett is a very bright little boy. Don't believe us - when the pandemic is over come and play a game of Carcassonne with him! Then you'll see.
As we draw closer to the end of the month there are two things that you should realize. The first is that you will see the return of Woody the Cowboy. 2017 was the first year for Emmett as Woody, then 2018. Again in 2019 Emmett returned as Woody the Cowboy. And now for 2020 Emmett has once again decreed that he will be Woody the Cowboy. The costume will be tight as Emmett has outgrown all of the Woody apparel that he has, but it is going to happen. His sidekick, Buzz, will likely be making an appearance also. You will also see an abundance of pumpkins at the Willms' house. Hippo pumpkins. And the bar has been raised as this year it was more than one hippo pumpkin. My niece, Carolyn, pushed the envelope further by sending a pic of a 3D hippo face carved into a pumpkin. Challenge accepted. But then Emmett announced to his mom and dad that he and I would be carving yet another hippo pumpkin. Another search of the internet and we discovered the Team Fiona pumpkin carving stencil. While the first two were freehand, this time the stencil is going to be put to good use. By the way, in the pic at the top Emmett picked the two pumpkins on the table as the ones that he wanted to take home! LOL!
The second thing that we want you to realize is that Emmett will be receiving his 19th lumbar puncture and the corresponding injection of Spinraza just
before after Halloween. There was a change in plans at the hospital and the procedure has been rescheduled to November 2. This will be a full on visit with a wide range of blood work, physio tests, and the nerve tests. All of which make for a miserable day for a little boy. He will received a general anesthetic for the actual lumbar puncture, but he's very alert for everything else. While he absolutely loves his hospital people, he does not look forward to this visit. He knows.
Emmett is working hard on his sight words. Alisha and Emmett came up with this awesome bean bag game to enhance word recognition. Then Emmett and I made the absolutely best ice cream. We will make some for you too! And then Emmett goes for a walk in his Kids' Walk.
School is so much fun. This happened earlier in October when it was still short sleeve weather. We launched pop bottle rockets. We attracted a lot of neighbourhood kids. We tried our best to maintain social distancing ... but Rockets!
First Day of Kindergarten
Yes, with us just passing by labour day weekend the first day of school has occurred for students across Canada and the USA. It has been a very surreal start up as everyone is dealing with Covid 19 issues and concerns. Alisha and Josh have obviously been dealing with those very same issues that are obviously complicated by a little one who is medically fragile. This complication is magnified because they were really anticipating the greater social interaction with other kids. Obviously, even in prior years in our imperfect world that would introduce a range of health issues into Emmett's environment and with Covid that seems like a quick black and white 'No' to school participation. And yet over the months that this pandemic has gripped the world there have been talk of drastically limited class sizes, reduced number of school days per week, and obviously greatly enhanced awareness on the part of most individuals of the need to sanitize, stay home if ill, wear masks, social distancing, and wash your hands! However, as the school year got closer and closer it became more obvious that the school system across Canadian provinces was going to proceed without any significant changes. These health and school challenges meant looking for other options.
The network supporting this family is amazing and one of these partners is Canuck Place. The teacher their has met and been working with Emmett in person (while he's there) and virtually though online video conferences. She made several great observations and recommended some distance education programs that other families had accessed. Long story short, Emmett is now enrolled in the Oak and Orca Bioregional School and has started Kindergarten. Distance Learning education - where he will be at home while supported in his education by Oak and Orca.
On Tuesday Emmett was so excited for his first day of school photo shoot. You may not realize this, as Emmett is in a lot of photos, but he does not appreciate posed photos. Candid shots are by far the best. Yet on Tuesday he was ready to pose and get his photo taken. He was also ready to show off his sole front baby tooth. Months ago Emmett lost his bottom front teeth and just last week his first extremely wobbly top front tooth came out. He's very protective of the last very wobbly tooth that is barely hanging in there - which is making tooth brush time quite an ordeal - but that's just another part of growing up.