In case you didn't realize it, Emmett loves Toy Story. Woody the Cowboy and Buzz Lightyear have been his favourite companions for more than half of his life. This summer when Alisha and Josh started talking about making the move from his crib to a big boy bed there was only one request on Emmett's part. This his big boy bed be a Toy Story bed! Well, fortunately Emmett wasn't talking about a bed that looked like Bullseye's corral or Buzz's space ship. He was fairly open ended on his expectations. Josh and Alisha, however, had a bigger challenge than Toy Story. They had to figure out what to do? You see, a regular big boy bed from any furniture store would mean instant aching backs for any adult supporting Emmett. A hospital bed would provide height and adjustability but is so institutional. So Josh went to Google and started researching bed construction and came up with a plan that had the bed high enough that adults don't have to bend over to work with Emmett. It is still a project in progress as the drawers still need to be built (picture a captain's bed on steroids) and then it will be all set.
So you may be wondering, how is a captain's bed on steroids a Toy Story bed! Well, because it has Toy Story Sheets and a Toy Story comforter. That is everything that Emmett needs to make his big boy bed the best thing ever. Of course, Woody and Buzz pyjamas are also very important for this character bed. Is he happy with his Toy Story bed? One look at his eyes in the above photo should answer that.
Halloween was either going to be Woody the Cowboy or Buzz. The week before Emmett finalized his decision. He was absolutely and without hesitation going for his second year as Woody. Buzz is a fun costume to dress up as, but Woody is the real thing. Emmett dressed as Woody on Sunday, Buzz on Monday, and then Woody on both Tuesday and Wednesday. Emmett had to go out on Halloween in his specially adapted stroller because his power chair was in the shop for repairs. Fortunately, it was back in service before the week was up and Emmett had his independence again.
Emmett got to go out to a pumpkin patch and was overwhelmed by the range of pumpkins that he found. He selected a great pumpkin and then announced that he wanted it carved like a hippopotamus. If you know Emmett at all, then you know that hippos are absolutely another very important facet of Emmett's life. So we got out our pumpkin carving gear and Emmett got what we think was a pretty good hippo pumpkin.
Of course, with some trick or treating it was once again an opportunity to discuss with folks the fact that Emmett doesn't eat or drink anything by mouth. So candies and chocolate bars are like toys for him. Something to play with but not to eat. Smarties boxes are still a favourite because they make great musical instruments. A huge thanks to all of the neighbours who helped make Emmett's evening a great one. And the helium balloon and toys you specifically selected for Emmett certainly allowed him to really celebrate.
Emmett has been back to the Vancouver Zoo - it really is one of his favourite places. The hippos were busy lying in a mud puddle and enjoying the fall sunshine. The wolf was meandering around the enclosed habitat. The grizzly bear was right up at the fence and seemed to take an extremely close interest in Emmett ... maybe Emmett's wheelchair ... no, I think it was Emmett. That grizzly tracked us for a while - we were all glad that there was a significant fence between us and even more to leave that area. We were able to get up really close to the moose, saw lots of flamingos, and were able to see the lions sleeping. The lions always seem to be sleeping! All in all, it was a great trip.
Alisha and Josh recently received word that they won an awesome mobility bike designed for 4 to 8 year olds. Another child had outgrown the bike and the family graciously allowed Canuck Place to have a draw for it - and Emmett was the lucky recipient. This bike's awesome three wheel design was intended from the beginning to meet the needs of individuals who simply could not ride a regular bike. And it means that Emmett has another very safe means of moving about and feeling like other kids. It comes complete with a full safety harness to hold him into the very secure high back seat and foot pedals/straps it allows him to feel part of the bike. Even better, the adult can steer and brake the bike while using the push bar at the back of the bike. Emmett doesn't have a helmet on in this video clip, but believe us when we say that a helmet will be a mandatory part of his safety gear. (Augghhh! His head is too big for a toddlers "CARS" bicycle helmet. It doesn't appear to come in larger sizes. And yes, subversive grandpa that I am, I'm trying to push him from one of my favourite movies, Toy Story, to another of my favourite movies, CARS!)
Emmett's new bike is another great form of exercise for his legs. You see, Emmett's brain messages are gradually getting through to his limbs and, you may recall, Emmett's legs stopped responding when he was about three months of age. Please note that Emmett is not powering this bike in this video - Alisha is pushing it and the direct drive wheels of this Freedom Concepts bike mean that the pedals turn in conjunction with the wheels. This in turn exercises Emmett's legs. We hope and pray that in a future post we can exclaim that Emmett is powering himself! Those legs of his just aren't strong enough yet. In water his legs have been quite active for a long time - as the water reduces the impact gravity has upon him. His bathtub is filled to the top daily for a physical outlet as Emmett floats, swims, and kicks his legs. Alisha exercises his legs multiple times a day. During his quiet time he is in his 'legzerciser 2018' for about 1.5 hours daily getting more movement happening. The legzerciser is a homemade device that gets Emmett's legs up in the air where he can freely move them about. Recently while working out with his mom during stretching exercises, he pointed to his left leg which, when mom puts it into an upright position that he can hold upright and vertical for a few minutes, he said "This leg is perfect!". Then he looked and pointed at his right leg, which he cannot hold up nearly as long, and pointed out that "This leg is not perfect". Alisha certainly assured him that she loves everything about him and especially both of his legs. The bottom line: Emmett has always known that his body is different than other kids and, even at his young age, that he is unique.
There are so many joys with Emmett and we love sharing those with you. There are also challenges. For instance, Emmett is currently on his second wheelchair. While he has been involved in a mobility study with power chairs since he was 16 months old with chairs that have been provided for him to use, his next chair will be 'his' chair. This is both awesome and a challenge, as a power chair can cost as much as and probably more than many families would spend on a car. The basic cost of the chair for Emmett will be provided but extras - which will be very important for Emmett to have - are the responsibility of the family. We mentioned that his chair was in for some work - the technician indicated that this chair (second hand when Emmett got it) is nearing the end of it's serviceable life.
Another challenge is public accessibility. This includes our church. When it is not cold and flu season - which does seem to be most of the year - Emmett loves getting out. He really enjoyed being at Church VBS this year. The main floor is accessible and all of the activities that Emmett's age group was involved with took place in the sanctuary, the foyer, and outside. The congregation is looking at the challenge of how to provide accessibility for Emmett and others who require it, but for a building with three levels it is not going to be an easy task. Yet it is an important task to allow Emmett independence and the opportunity to participate. At least, participate when it isn't cold and flu season.
Of course, their house does not have a handicapped washroom. That is something that will have to be in future plans. Right now, Emmett has just finished getting another dose of Spinraza. I think that this might have been injection number 13. This took place on Wednesday, November 7th Emmett at BC Children's Hospital - another injection of Spinraza. Another lumbar puncture, to be exact, which is certainly far more invasive than just an injection. The neurologist removes 5 ml of spinal fluid and replaces it with 5 ml of fluid containing the drug Spinraza. Emmett was not looking forward to this. As much as he loves being at the hospital, he recognizes the tests and process that takes place before this procedure. Which he obviously does not like. It went very well and Emmett handled it like a champ. This was a huge relief for Alisha and Josh - because he was very upset in July for his last lumbar puncture.
There have been three or four trips to Vancouver over the last two weeks. Check-ups, getting fitted for new orthotics, getting his body brace adjusted, and of course his lumbar puncture for Spinraza. This little boy has a very full calendar. This little boy is growing so much. He is a tall boy and my goodness he is getting heavy!
Your thoughts, best wishes, and prayers for Emmett are truly appreciated. Emmett is such a happy little boy. Thank you for loving him with us! He is incredibly special.
Definitions of Spinal Muscular Atrophy Types
What is SMA 1, 2, 3, and 4. And Type 0!
In the last blog post we made a huge proclamation, that Emmett has progressed to presenting as a toddler with Spinal Muscular Atrophy Type 2 versus Type 1. However, I did not go into details nor did I provide any definitions. My friend, Sharon, commented that I made her do some research to figure out what was the difference, so here is a recap. *Please note that the descriptions of Emmett that follow are going back in time.
Layperson definition of the Types of Spinal Muscular Atrophy - PRIOR to SPINRAZA:
Spinal Muscular Atrophy describes the group of diseases in which a progressive degeneration of the spinal nerves causes a wasting of the muscles they control. 1 in 50 people are carriers of the survival motor neuron 1 gene, which decreases survival motor neuron protein, and this decrease causes the destruction of the nerves in the spinal cord that control neuromuscular growth and function. Carriers experience no symptons. Carriers do not develop the disease. When two carriers have a child, there is a 1 in 4 chance their child will have SMA. It can vary in severity.
SMA type 1 - most severe and the life expectancy is incredibly short. There are some amazing exceptions. Infants identified immediately upon birth may be referred to as SMA type 0.
SMA type 2 - children have greater SMN protein but still have a shortened lifespan and can never stand independently.
SMA type 3 - children have a normal lifespan but have life-long physical disabilities.
SMA type 4 - adult onset.
Prior to the drug Sprinraza there was No Treatment. Parents were told to love their little ones, provide them with as many experiences of life as they could, but realize that respite care was the only available care. Emmett was born in 2015 and he was involved in a worldwide trial of the drug that involved 124 little ones around the world. 2/3 of the little ones got the drug/1/3 of them got the placebo. The kids and families on the placebo were promised the drug after the test if the drug proved successful. The drug was amazing - the kids receiving the drug were improving. The kids on the placebo were literally dying. The company ended the test early, pushed for early approval of the drug from the USA FDA, Health Canada, etc.
At about three months of age Emmett lost the ability to move his legs. He never developed the ability to hold up his own head. His grip was weak and his wrists curled. He was a stomach breather. He was an incredibly floppy baby. And he was incredibly cute!
On August 18, 2015 Emmett was diagnosed with Spinal Muscular Atrophy, Type 1. By late September his alert eyes were rapidly looking about but his side to side head movement was virtually non-existent. He was losing the ability to control his muscles very quickly - with both arms failing to respond by the end of September 2015. Literally one day he could reach out and grab the ribbon from his helium balloon and then the next day he could not. It was heart breaking and I swear that even at his young age, Emmett knew that something was wrong with him. In October - I believe it was October 7, 2015 - Emmett got his first injection. At that time the drug was known as SMNrx. The drug was later called Nusinersin and, when it came to market, was called Spinraza. He was in the blind trial so we did not officially know if he was receiving the drub or a placebo until almost two years later - unofficially we started seeing improvements rather rapidly. (Interesting fact: the company that developed the drug was called ISIS. They changed their company name to IONIS. The drug was later purchased by BIOGEN.
Here's a blog post when Emmett turned one. The italics are a section copied from that post or you can click the link to read the entire post.
Take a look at Emmett when he was 12 months old.
"Spinal Muscular Atrophy, Type One is a challenging and terrifying disease. This SMA can strike at any age IF you have the right (I should probably say wrong) genetics. Both parents have to be carriers, and if they are then there is a 1:4 chance that their children will have SMA and a 2:4 chance that they will be SMA carriers but won't actually have the disease themselves. Scarey odds! If the disease presents itself before the child is six months old, then it is identified as Type 1. Emmett displayed symptoms before his diagnosis at five months but we didn't know what they were. Neither did the professionals during his check ups. It wasn't their fault. The highly trained nurses in the baby clinics and the family practice GP's saw a little boy who was demonstrated delayed development. SMA Type 1 would fall into a category of rare diseases that many medical practitioners may never see in their careers. Parents see their little one 24/7 and must be vigilant and push. And we've said before, when their GP did observe what concerned Alisha and Josh, Emmett was in to a paediatrician in less than 24 hours.
The one question that we have heard most often is "What is SMA, Type 1?". SMA being Spinal Muscular Atrophy. Here is another of my attempts to try to describe it. With almost no scientific phrases.
Picture your local multi-lane freeway. Traffic flowing perfectly both ways. Everything running like clockwork. No accidents. No flat tires. No construction (yes, that is a miracle in itself). Then see that some challenges intrude. Way ahead someone did get a flat tire or they ran out of gas resulting in a slight slow down of traffic. As you get closer to that incident, someone crashes into the rear end of another in the middle lanes. As you can imagine, traffic is slowed right down and comes to a virtual stop. That's irritating, but you know that in an hour or two you'll be back on your way. Maybe there's a bridge that is the only way across the country and it breaks, falling up, so that this main thoroughfare is out of commission or down to one lane for an unknown timeline. Or picture that there was a massive earthquake that has taken out literally kilometres of this highway. Obviously, the entire country is in chaos as a result of this natural disaster. Your car cannot ever traverse this expanse as the massive highway has literally disappeared. Since the government has no money to fix it (doesn't matter which country, as they're probably all broke), the only way to get from A to B now is to hike on foot or to take a donkey. But it is very hard to find a donkey, so you're left on foot. Of the hundreds of people who try to walk the distance, only a few can get through because it's tough. Climbing cliffs. Jumping crevasses (yes, there's ice everywhere). Crossing expanses of rushing water that has carried a few of the hikers off to the unknown. Very few of the hikers make it to their destination. Most just stop. The economy in this devastated area shuts down - completely. Until the engineers and construction crews can open up a new route nothing moves - and everyone hopes and prays for that a new route is opened.
This is similar to SMA, Type 1. The spinal column is the highway and it suffers a major blow. It is no longer healthy and the messages simply cannot get through. As a result the muscles begin to shut down.
The light at the end of this tunnel (maybe my analogy should have been a train tunnel that runs through a mountain) is the pharmaceutical trial that Emmett was allowed to participate in, beginning last fall. This is the scientists developing a drug that revitalizes the spinal column so that messages from the brain can get through. If this was our highway that had been devastated by the earthquake, the first step would be to open up some kind of walking path. Maybe with ropes across the crevasses and rushing rivers - if you can go hand over hand on the rope you can make it. Tough and very slow, but possible for some. Then the crew sets up a swinging rope bridge which enables many more to cross. With time, sturdier and more expansive paths are created. This experimental drug in this trial is like this.
If you are wondering what an EMMETTism is, then I'm guessing that you haven't read this blog too often. Allow me to provide a definition. An EMMETTism is a phrase or comment from Emmett which makes us smile - possibly even laugh. Unfortunately we have forgotten several EMMETTism's, but we have a few here to share with you. N.B., EMMETTism must be typed/written with the first six letters capitalized.
There's nothing like freewheeling down a hill. This isn't an EMMETTism - it's just a little boy enjoying life!
Emmett loves having a bath. Not just because he loves being clean, because he is fanatical about being clean, but also because it is great physical exercise. One night, while having a bath, Emmett wanted his hair washed with his mommy’s shampoo rather than his baby shampoo. After the bath he was all excited to see himself in the mirror. When he did, he burst into tears. When finally calmed down the reason for the tears came out. Mommy’s shampoo bottle was orange. Emmett thought his hair would be orange.
Watching TV, videos, or YouTube is not something that Emmett gets to do very often. It is quite a treat, in fact. In early September Emmett had gotten the sniffles - which he probably caught from me - and was feeling a bit under the weather. One afternoon he looked at his mom and told her that he needed a rest on bipap ‘and a little bit of veggie tales’. Alisha couldn't pass up this request for video time.
As we've already indicated, Emmett is a clean fanatic. So much so that if someone else plays with his toys he will ask if they've been sanitized before he will play with them. He's never liked touching sand or doing finger painting - although we've been doing that. It's sort of like Star Trek - You know, the one that goes 'Dirt. The messy stuff on your fingers. These are the adventures of Emmett, his mission, to explore dirt and actually get it on his fingers without crying'. *You have to say that with the music of the Star Trek theme playing in your head. Well, just last week Emmett wasn’t letting Alisha brush his teeth. They had just been at the dentist and he loved it (they were at Allwood Dental, a new clinic with awesome staff and a cool TV setup that moves up over your head so you can watch without interruption), so to get his teeth brushed Alisha told him they were playing pretend. That she was the dentist and he was her patient. Emmett looked at her and said “You a dentist, mommy? Well, where’s your TV????”
Emmett loves Toy Story. It is literally his favourite. He gets to watch the DVD while driving to and from BC's Children's Hospital - which will be happening on October 9 (today) as he is heading in for a bunch of assessments and tests - and he does know the story inside and out. He has miniature Toy Story characters that he loves playing with. At almost any time, he can tell you exactly where in the house his Toy Story tupperware container is so that you can find his toys. In the video the toys are only animated when the 'humans' are not around and that includes the boy Andy that the toys belong to, so when the 'humans' are around they stop and drop. One of Emmett's favourite things is shouting "Andy’s coming"! When Emmett does that, you have to stop, fall over and be still.
A less challenging command from Emmett is that he will shout "Family Hug!" and Josh, Alisha, and Wendell (the cat) have to stop everything and immediately join with Emmett in a giant bear hug.
Of course, there are the longstanding favourites. Carrots. Emmett loves carrots. It will be fantastic when the time comes that Emmett can eat food by mouth versus his tube feedings. We are all, however, quite worried that when he finally tastes carrots that he may be somewhat disappointed! Of course, they will always be orange. His favourite animal is most certainly still the Hippo. And based upon the picture book he knows that Hippos hiccup and giraffe’s laugh. And he still loves orange hippos. We actually found some orange hippos on Amazon and ordered them. They never arrived. After some emails, the seller re-sent the order. And it never arrived. We were quite disappointed but four months later both orders arrived - we now have lots of orange hippos.
While camping this summer we were out for a stroll with Emmett. (I like stroll better than walk. Obviously, Emmett doesn't walk. In his power chair he rolls. And stroll has roll in it - so I think it's fitting and inclusive to say we were out for a stroll). As we went around Emmett observed everything from the bugs to the RVs to the other people out for a stroll. He meanders. He does circles. He is a typical three year old. But as we came up to a small parking lot his irregular path suddenly straightened and he went like an arrow straight towards a beautifully restored classic Dodge muscle car. He stopped right at it and rotated his chair to look at us. Then he exclaimed, "This is me!" The car - a brilliant orange.
As a three and a half year old Emmett obviously has an early bedtime. Couple that age with SMA and that early bedtime is an absolute necessity as he is exhausted at the end of the day. This led to the next EMMETTism which occurred while camping during the fall. All spring and summer Emmett has been going to bed while the sun has still been up high and bright. Sitting beside the campfire as darkness settled in, Emmett was enjoying the sights and sounds of the campfire. He loved watching others roast marshmallows and enjoyed squishing his own right out of the bag marshmallow. Out of the blue - or rather out of the dark - he exclaimed, "Look! Dots!" as he starred up at the night sky! It has been months since he saw stars and his words failed him. So dots it was!
After reading the book the Blue Hippo, in which a real hippo turns into a toy hippo on wheels so he can be close to the little human girl that he loves and then after being forgotten when the girl grows up, becomes the girl's little baby boy, Emmett has decided that he wants an orange hippo on wheels for Christmas, that his mommy wants a blue hippo on wheels, and his daddy wants a green hippo on wheels.
Christmas is important for Emmett year round. It was a disaster for Emmett when he lost his playmobile Santa figurine. Fortunately, his grandma Rhonda found one in a thrift store. Hurray! After his fishing excursion with his daddy, Emmett has also added an orange fishing rod to his Christmas wish list!
Emmett's Favourite Jokes: These are pretty corny and remember they come from a three year old.
Why does Santa have three gardens - So he can hoe, hoe, hoe!
Why does the cow cross the road? To get to the moooooo-vies.
Why did the banana have to go to the doctor? He wasn't 'peeling' well!
Finally, Emmett will always try to prolong play time, story time, or anytime that he is having fun with the phrase "Just a couple more minutes". Not two minutes. Or five. And if you say two more minutes that is absolutely wrong. It has to be a couple more MINUTES! And yes, he will start to shout minutes if you don't agree with him right away.
The blog is finished, but if you have a couple of more minutes, Emmett has finished all of his check-ups at the Children's Hospital today. His neurologist told him and his parents that Emmett, who has SMA Type 1, now presents as having SMA Type 2. We have felt that for a while, but now it's official. This is a pretty big Spinraza moment for us. This drug is literally a miraculous and life changing five millilitres that Emmett gets every four months. WOW!
It was a little over two years ago that Emmett picked his head up off of his mommy's chest all by himself. Now he's holding his head 'while' being picked up by his daddy 'WHILE' lying on his tummy on the floor! This is an absolutely amazing first. Emmett, you are such an inspiration!
This next video is a little bit on the long side, but I love seeing his sense of humour. I love seeing how he conveniently ignores requests - in other words disobeys - in order that he can help and prolong staying out of bed! Ha ha ha. And most of all, I love watching Emmett wheel himself around. Emmett is wearing his back brace in this video clip. He can sit up but for longer periods of time the back brace is essential.
Of course, what does every little boy love to do when they go to the park ... you will notice at the end of the video clip that as strong as Emmett's neck now is, he does have some moments when he hits tree roots while off roading! But that didn't stop him. He just needed an adjustment ... and then he headed back to the smoother ground of the pathway.
We want to give you an update on the Orange Hippos fundraising results for CureSMA Canada at Florence and Charlie's 10K to End SMA. Our $6200 fundraising total was increased to over $8200 as Valley Carriers - where Emmett's Grandpa Roger and Uncle Ben work - made a substantial donation to CureSMA Canada. Thank you so much!
While we are saying thank you's we also have to thank Canadian Tire Abbotsford for supplying us with a Motomaster Pure Sine Power Inverter and a Motomaster deep cell battery. We have set this system up to provide 24 to 48 hours of electrical power to run Emmett's medical equipment in the event of a power failure.
While looking at the last couple of pictures with Emmett, the one on the bridge with the water is Mill Lake. Then we flipped to the next one and I said, 'Emmett, look! The Bridge at Mill Lake!' and he corrected me. 'No, grandpa. Bateman Park.'
And he was right! Smart kid.