Emmett and I have a secret. We have been planning this for a while. In fact, Emmett told you about it in his blog last summer. After reading the story The Blue Hippopotamus Emmett decided that he wanted to give his mommy a blue hippo on wheels and his daddy a green hippo on wheels. Well, that is our secret project that we are working on. If you see Alisha or Josh, please do not tell them about this blog post or the secret that we are working on. They don't know. If they have been in the room where we have been working on our hippos on wheels, then we either cover them up really quickly OR we tell them to not look OR we tell them to cover their eyes. And so far it is working. We are sure that they don't know about it.
Daddy has been really good about it and he doesn't ask BUT Mommy is always asking what the secret project is. Silly Mommy! But Emmett won't tell her what the present is. And it was very funny because when she was coming close one afternoon, Emmett gave her the best 'what are you doing glare!" It was quite funny.
First though, a video of Emmett playing with his zoo:
And maybe a little bit of music therapy while at Canuck Place in Abbotsford!
Then it was time for both of us to have a nap! We'd been working very hard!
Merry Christmas, Everyone!
Emmett loves December for one reason. Santa Claus.
Yes, Emmett knows that his daddy is a minister and that he works at a Church. Yes, Emmett knows about the baby Jesus and he always checks the nativity scenes to ensure that the baby Jesus is there. He has lots of fun playing with his Playmobil Nativity Scene and the baby Jesus can do miraculous things. But ... Santa Claus ... well, sorry but in Emmett's eyes even grandparents take a back seat to Santa Claus. Ho Ho Ho!
We do not know why Santa plays such a huge part of Emmett's life view. It certainly didn't stem from a family gathering a few years ago when Josh dressed up as Santa and Emmett was absolutely terrified. There was no love for Santa at that point but it has been a developing story every since. You may recall that we mentioned in the summer that there was great wailing and grief in the Willms' home when Emmett's Playmobil Santa was lost. And then great joy when Grandma Willms found one in a toy store at almost the same time that Alisha found the missing Santa at home. (We are blaming Wendell the cat for hauling Santa away!) But now there are two Santas!
Last year before Christmas, in the early fall, and well just about anytime of the year Emmett would look up at the night sky, full of stars, and go "Ho! Ho! Ho!" and search for Santa and his sleigh. He never saw them. This year we haven't heard the "Ho! Ho! Ho!" for a while. But last week, the first week of December, he looked up at the night sky which was a mix of stars and clouds and whispered "Ho! Ho! Ho!" Emmett hasn't seen Santa and his sleigh in the night sky, but he certainly has the real spirit of Christmas.
Last year Emmett was ill around Christmas and missed out on an opportunity to meet Santa and go for a plane ride with him. When Canuck Place in Vancouver announced that it was happening again, Alisha and Josh were very quick to get Emmett's name into the draw so that he might have the opportunity for two of his favourite things. Santa and planes. Because planes are right up there as special things for Emmett and, apart from an emergency air ambulance flight, he has never been in a plane. Nor met a real Santa (because it doesn't seem that his dad dressed up as Santa was very satisfying. The photo at the very top is one of Emmett's many expressions as he was on his one hour plane ride that flew from Vancouver over the mountains to Whistler and then circled back. He loved it and even with the elevation changes his ears didn't even bother him. Think about it, how do you tell a three year old who cannot swallow to swallow and reduce pressure on his ears! He does do a good job of yawning so that was an alternative. The scenery was absolutely fantastic.
Click here for a link to CTV's coverage of the Canuck Place and Air Canada Special Santa Flight. Emmett has a cameo in it.
CBC News has given us permission to share their coverage of the Special Santa Flight. They interview Charlie and her mom. This is the same Charlie that hosts the 10K walk, run, and roll to end SMA. There are also several more clips with Emmett visible!
Well, this trip was even more than Emmett was ready for but there was one huge flaw! As the family headed out of the house for this outing, Emmett had a huge fuss and tons of tears. There was no way that he was getting into his van. After all, Santa was coming to get him and pick him up with a plane! Right! LOL - eventually he calmed down and they were on there way to the Vancouver airport. After a lengthy wait, Santa appeared - with his upper body revealed through a roof top opening in the Dash 8 airplane. "I'm here, Santa! I'm here, Santa!" Emmett shouted! While he's getting louder, Emmett's shout is not too much above most kids inside voice. Even so, Santa heard him. We think. When Santa arrived in the building, Emmett was so excited about Santa that he was hyper-ventilating. Alisha and Josh first went into a minor panic and medical support roles as they weren't sure what was happening at first, then worried that if he didn't settle down that he'd have to miss the flight, and then as Emmett calmed down a bit they realized he was literally just so excited that he couldn't breathe for a few moments. Once he got his second wind, he grabbed his power chairs joystick and went charging off towards Santa - who was on the other side of the room. In process he almost plowed over a couple of other kids in wheelchairs (Canuck Place is a hospice for medically fragile little ones - running over them in a power chair would not have been ideal) because he had to get to Santa.
December hasn't all been Santa. There was an evening opportunity to visit Canuck Place - Abbotsford and to see a bunch of critters up close. If you look on his chair's arm rest you'll see a Tarantula that was crawling about and visiting him. The evening included a wide range of animals like snakes, turtles, rabbits, and other long legged bugs. Emmett also got a trip to Home Hardware to check out their offerings. The face mask is just in case anyone in the immediate vicinity has a cough or bug that we don't want Emmett to catch.
Emmett's Hippos have been left in the house now that December has arrived. Santa, Rudolph, and Frosty are his friends that are accompanying him on his outdoor excursions right now. And even though it is getting chilly out, Emmett is still out and about on his bike. He just upgraded it with a carrying rack that is mounted on the back and can hold his suction machine, food pump, and anything else that he needs to haul around with him.
We will say Merry Christmas and Happy Holidays now
but we're pretty sure we'll have another blog posting before the New Year.
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.