January 2017
Million Dollar Baby
Monday, January 09, 2017
Every child is a priceless addition to a family and there is no question that Emmett is absolutely a treasure. He is our million dollar baby! On January 9, 2016 he celebrates a huge milestone. He is officially 22 months old. In this past month he has come to adore his cat, Wendell. He loved Christmas and couldn’t get enough Christmas presents – signing ‘more’, ‘more’, and ‘more’. Then he played with the boxes and wrapping paper and forgot about the contents of the packages. He can “Ho Ho Ho” with the best of them – maybe even better than the jolly old man himself!
There has been a bit of cabin fever going on in Abbotsford, as Emmett hasn’t enjoyed being outside when the temperature is below zero. And since the snow has not left, he gets quite frustrated. You see, his wheel chair has absolutely no ground clearance nor snow tires so if he attempts to head across the lawn, he gets stuck. He is a very bright little boy, but he knows that he can drive his chair on the lawn so that what he tries to do. But there is 15 cm of snow on the lawn. It just doesn’t work! He’s been to both grandparents’ homes. He’s been to the swimming pool. The need for caution on outings is even higher right now as there is a terrible flu going through the area – lots of sanitization and cleaning going on! Thus as you can imagine, cabin fever is very much a factor for the Willms’ family.
Emmett loves knocking blocks, toys, and anything he can onto the floor. He then appreciates someone picking them back up for him – so that he can do it all over again. When is this going to stop! Also, right now he is a drooling little boy because it appears that he has a million teeth that are all trying to appear at the same time. He loves his power wheelchair and every day his driving control gets better. He loves to play hide and seek – except he always wants to be the one who is seeking. When he finds you he will drive up and bump you with his chair so that you know he’s caught you. Obviously, you have to play the game in a reasonable manner so that Emmett can find you and then also get to you! Roger got a video of him in fast forward – which is often how it feels like he is moving to us as we try to keep up with him.
Emmett is also a million dollar baby because the suggested price for a first-year supply of Spinraza, the drug that Emmett has been receiving, is a whopping $750 000 USD or $1 000 000 CDN. The number of doses required reduces for the second and following years with the suggested price being $375 000 USD or $497 000 CDN. Shakes you to the foundation of your being, doesn’t it! Emmett is still involved in the trial throughout 2017 so all costs are covered by the pharmaceutical company. The drug has not yet been approved in Canada – only in the United States – so there are still many, many questions to be answered.
If you’re asking how a drug can be so expensive, Spinraza is literally building new pathways for messages to travel via the nervous system. It is mind blowing what these scientists have developed. We simply hope that once production begins in earnest that the price changes drastically.
After a few months of working with balloon refills, it’s time for a new balloon. Ella the Elephant and her free refills have been great – and Ella has become part of the family – but she cannot hold her gas very well anymore. Neither can Nemo the Clownfish. And since Emmett doesn’t really like the air swimmer idea, we’ll save the shark until he is older. Monday Morning it’s off to find Emmett a new balloon. I think our local store has some Paw Patrol ones! That is the theme that happened this Christmas. Paw Patrol toys. Paw Patrol books. Paw Patrol Jammies. Paw Patrol shirt. Therefore it is time for a Paw Patrol balloon and we’ll all sit and watch a few episodes of Paw Patrol!
I’m a Man!
Saturday, January 28, 2017
Emmett, are you an elephant? Vigorous head shaking to indicate No!
A cow? A bear? A doggie? Same reaction, including verbal sounds that are absolutely indicating disagreement.
A kittie cat? Looks at Wendell and same reaction. Wendell, of course, is his cat.
A girl? Same reaction.
A boy? Same reaction.
Of course, this is where you as a wise reader would be shaking your head and going ‘tsk tsk, what are those parents teaching their little child!’ but it really is not the parents. Emmett has trucks. He has cars. If you remember, his favourite game is to throw things onto the floor for people to pick up … simply to have them tossed to the floor again. Except for his cars and trucks, which he will play with for extended lengths of time. Of course, he has lots of stuffed animals and he has a purple helium balloon elephant called Ella (we think Ella is now maybe seven months old! They recognize us and laugh with us when we stop at the party store for helium refills!). Emmett even has a doll house (hmmm, he doesn’t have many dolls!)
When he goes outside he is really demonstrating that he is a little boy. He was just at the park and we are really noticing his independence. In 2016 (doesn’t that sound like it was a long time ago) Emmett would usually follow his mom or dad (or grandparents) fairly closely when he was out with his wheel chair. He would stay in the same room as the adults. Now, however, his independent character and his stubborn streak is showing itself. He will simply leave. He may start to follow you but if he decides that somewhere else is more intriguing than where you are going – he will go the other way. Or if you ask him to stop beside a January flower – so that you could photograph his smile as he touches the flower in the hope of making everyone else who is stuck in the midst of winter doldrums envious of the springlike weather coming to the Fraser Valley – and rather than stopping he does a 180 and heads off in another direction!
It started in the house when he would just leave the room. Outside, what started as stopping and simply looking elsewhere has progressed to literally heading off in another direction and expecting/knowing (hoping?) you will follow him. And of course, you do because you cannot let a kid who is not even two years old drive his electric wheelchair down the road without adult supervision. That would be wrong (Let alone the need to be close by in event that suctioning is required). So you follow and catch up to Emmett and then you have a discussion about why you should be going the way that you, as an adult, want to go. Sometimes you lose and you go where Emmett has planned. But perhaps you will stand firm and dictate the direction! Last night, for instance, we were heading out to load the wheelchair into the van and then off to grandma’s house (grandpa’s too!). Emmett decided he wanted to go for a walk so he sailed past the van. I steadied his head as he rocketed over the bump from the driveway to the street – it is maybe an inch but at rocket speed [I’d guess maybe 1.5 mph, that is enough of a bump to cause Emmett’s head to be jostled beyond his ability to control it. Obviously at 1.5 mph he is not going to get away. Even at the fastest 2 mph that he is allowed to control on his own, any adult is able to overtake him rapidly. But that doesn’t stop him from attempting a get away. And then, possibly in the middle of the street (there are no sidewalks in their neighbourhood so the chair has to be on the street for walks) you are having a discussion with a 22 month old about why they cannot just stop in the middle of the road, why they should go back to the driveway so that we can load up the van, and why they are going the wrong way. With so many why questions in a row, it is no wonder that two-year olds most frequently asked question is why!
Well, I couldn’t convince Emmett to do what I wanted and needed him to do, so that meant that I needed to wrestle the controls from his grip. You see, I can simply turn the chair off and then Emmett cannot move. If you’ve ever tried to push an electric wheelchair you’ll realize that is not a reasonable option (of course, you can disengage the drive wheels and then pushing is easier). So rather than shut off his chair, Emmett and I wrestled for the controls … I won. Emmett cried. After all, he is just a toddler and he always wants his way. Of course, he forgot this and the cries changed to smiles as we went superfast back to the van (probably 3 or 3.5 mph). The chair only goes superfast when it is in adult mode and controlled by the joystick on the rear handlebars. Emmett may not like losing control, but he absolutely loves going superfast. Then back into the driveway and loaded up for grandma’s house.
Another sign that Emmett is all boy was when we were at the local park. In 2016 when Emmett was still in his teens (months, not years) Emmett would stay on the path and be very conservative with his new chair. Now that he’s in his twenties (months, not years) and the chair is just over six months old, Emmett has become a trail blazer. Off trail driving is his focus. Over tree roots, up and down hills, and then heading from the turf onto the woodchips of the playground. At our local park this is quite an elevation change of probably 6 to 10 inches (15 to 25 centimetres) which truly would have caused Emmett’s head to lose control. Of course, supporting his head while also supporting the chair can be a bit of a handful. This chair is awesome but it is absolutely designed for flat terrain. Plus, in this springlike weather the chair might make it down the incline easily but couldn’t find any traction on the way up and out of the woodchip playground – so a good push is necessary. [Time for the mini-activist in me to say that playgrounds should be developed with wheelchairs in mind. And I’m feeling guilty, because I helped in the design of two or three school playgrounds and I know now that they weren’t as wheelchair friendly as I would now like to see!]
Emmett will not acknowledge that he is a boy. Ask him if he is a man, however, and he will give you the strongest and most absolute verbal acknowledgement that you got it right. Our little boy Emmett is already identifying as our little man! Make sure you get it right!
Now, here’s a video of our little man. Watch it and cheer our little man on! If Emmett could talk, I think he would be saying “Dr. Selby! Nella! Look at me!”
Thank you for the notes and email! It means a lot to us. Whether you’re in another part of Canada, Australia, the United States, England, Ireland, or another part of the world. Thank you for your interest and support. And if you are a part of our extended SMA family – our love goes out to you and we pray for strength for your family as you are fighting for your little one – just as we are fighting for Emmett!
February 2017
23 Months!
Thursday, February 09, 2017
Emmett is really not very sure about the snow. It’s cold … but the awesome thing about being bundled up for the cold weather is that the added layers provide support which make sitting easier. Plus the hood helps support his head – which is necessary even though his neck muscles are getting stronger.
While he doesn’t like the snow very much, that is nothing compared to his distaste for bright sunlight! Combined with snow – it’s a bad day!
Just a few days ago there was no snow in Abbotsford – Emmett was out cruising down the middle of the street (no, this is not acceptable behaviour and he was directed to the side quickly!) with his cool shades!
But while Abbotsford is under a “once a decade” blanket of snow, Emmett gets to be outside with his parents to enjoy the winter wonderland.
Even if the only thing Emmett is wondering is why his parents are tormenting him by making him be outside while cold stuff is falling on his face!
And we should be back to the green stuff soon! After several days of record breaking snow fall, tonight it is raining. Freezing rain – so drive safe – but the spring weather should be back soon. Just like this photo taken last week!
23 months old! Stay tuned for a more detailed blog post about some of the amazing progress we are seeing. The road ahead is long and challenging but Emmett has already come so very far. Thank you for supporting Emmett and all of us on this journey.
There Are Good Samaratins
Wednesday, February 15, 2017
The Good Samaritan in the book of Luke is a stranger who stops and helps another stranger on the roadside. Completely unable to help themselves, beaten up by robbers and left seriously hurt, the injured stranger was at the side of the road and obviously in trouble. That is, until the Samaritan came along and helped him to a place of care and safety. Thank God for good Samaritans.
Today I saw neighbours stop to help a stranger at the side of the road. The stranger – or strangers – were myself and Emmett. First, let me say that Emmett is wonderfully fine now. He spent the evening relaxing in a warm bath and then laughing and reading (rather, having stories read to him!). Of course, he also got to watch several episodes of Paw Patrol in a row. But there were a few moments this afternoon which can only be described as terrifying.
Emmett and I were out on a stroll through his neighbourhood. We went nowhere very slowly. We stopped to look at birds flying past in the sky. The neighbour’s cat walking across the top of a fence. A travel trailer that has pictures of wolves on it – a mandatory stop on all walks. Emmett will often howl like a wolf while he is there. And, of course, we made honking sounds at every pickup truck that was parked on the side of the road as we passed it. Emmett really liked the shiny black one … because he could see our reflection in the paint! When a city bus or truck passed in the distance, Emmett would make deep sounding honks – for the big truck! We chatted briefly with a couple of neighbourhood kids who noted that Emmett had a wheelchair so obviously couldn’t walk. Hopefully we see them again on our next walk. We took a detour down a side street that we never take and, once turned around and heading back I figured that we could retrace our steps and head home. The short route. Emmett, however, turned left and continued on his journey around the long block so that is what we did. It was a lovely day and Emmett was enjoying himself. Me too!
Eventually, a bit more than half way around the block, I took over the rear controls so that we could make a bit better time. We got to the corner that turned onto his street, looked both ways and headed across. In the middle of the street I noticed that Emmett stopped chattering … stopped his hand movement. Peering into his face I could see that he was choking. It happened that fast – while crossing the street. Right to the side of the road and got the suction machine out and running. But it didn’t clear it. This was not a typical Emmett choking moment – one that happens way more often than we ever write about – that is cleared quickly with Emmett carrying on with his activities like nothing happened. Because to Emmett, that is a normal part of life. This moment, however, was not normal. I tipped the chair back so Emmett was parallel to the ground and more suction. Still nothing. Well, there was gunk coming out but nothing as far as opening his airway better. Got Emmett unstrapped, out of the chair, and lying down on the grass right beside the curb. More suction. While doing this I used voice activation on my phone to call Alisha on speaker phone (I do appreciate technology). I’m still suctioning. Patting Emmett’s back to loosen whatever is there. Told Alisha where we were, I needed help, and to call 911. She came running. In sock feet and on the phone with 911 the entire way. Due to a hill in the street, she couldn’t see us from her house but she got to us in record time.
As Emmett’s airway was blocked and suction wasn’t clearing it, I did the reverse of artificial respiration. Rather than blowing in (compounding the blockage?), I sucked out. Did that at least twice. I’m still not seeing the reaction that I wanted, which was his little tummy going in and out like an old-fashioned bellows, but I think Emmett’s eye’s started following me better. I thought I was hearing breathing. Very shallow, but something. Emmett is still lying on the grass and I’m beside him, one knee on the grass and one foot on the road. Empty wheelchair on the side of the road.
And neighbours we didn’t even know came running. A man in a red car (too vague, it was a BMW Mini. Red, with stripes. I think it might have been a Mini Cooper but I’m not sure. Pardon me for being a car guy and just seeing things that aren’t important). He asked if we needed help and 911 – I’m sure I said yes. A lady came from the house and asked if we needed a blanket – Alisha was already here by that time – and we said yes. Alisha took over suctioning and I continued lightly pounding Emmett’s back. I picked Emmett up – butt higher than his head to help him drain. It was more than saliva – thick. The Mini driver could hear the ambulance but thought it went past – so he drove off to flag them down at the main road. The Ambulance team arrived before he got five houses away. By this time Emmett was okay. Alisha thought the worst was over as the paramedics rushed to Emmett. They took Emmett and Alisha to their house where – with his Bi-Pap and Oximeter and stuff – Alisha had almost everything needed for Emmett’s care. His face was back to rosy red and flushed cheeks. They were obviously checking his lungs and they were crystal clear. No sounds of aspirating anything – which would have meant a trip to ICU. The oximeter was reading 100% – cannot get any better than that. The paramedics were awesome and, recognizing Alisha knew more about caring for Emmett’s needs than they did, followed her lead. Professional, caring individuals who recognized the child’s needs over protocol. Close monitoring throughout the evening. His nurse was in early and checked him out!
It took the wheelchair and I a few minutes to get back to the house. Arriving we maneuvered around the Fire Truck and Ambulances which were now gathered at the front of the house. There were at least 6 paramedics and firefighters in Emmett’s room. As I arrived at his doorway, Alisha was now calm(er) and Emmett was looking at the team of strangers who were there to support him. His stats were coming back up. Not too much movement, but as the emergency was over and the team started to disperse, Emmett gathered enough energy for a wave. After at least three episodes of Paw Patrol – and it may have been six to ten episodes – Emmett’s voice was back and he was complaining loudly when Marshal, Rubble, and the rest were silenced. Nothing like hearing that voice of complaint. It was great to pick up Emmett and feel him squirming because, earlier, he was so much more a rag doll than we now expect.
Whenever you see photos of Emmett if you look closely, you’ll probably see a blue and white machine. If he’s outside that machine is housed in a black case and likely sitting on the back of his wheelchair. That is his suction machine and, while Emmett is doing incredibly well, it is his lifeline. It gets used regularly because Emmett cannot swallow. As a result, dealing with choking situations is something that Emmett faces daily. Multiple times a day but they are usually cleared very quickly. In fact, Emmett is getting better at coughing and clearing his own airway. You may hear us laughing about spitting and blowing bubbles – teaching Emmett to help keep his airway clear and this amazing little boy sometimes clears things up before we even get the suction machine turned on. We have been very blessed that Emmett has only had a few chokes that took more than suction to clear. For a baby/toddler with Spinal Muscular Atrophy, this is amazing. This was actually the first time that 911 has been called to support Emmett. And what fantastic support we got.
Thank God for the paramedics and the fire crew that showed up to help us out. They have my respect and thanks for the great work that they do day in and day out. Thank God for these kind neighbours who recognized that the man and little one on the side of the street needed help. Thank God for Alisha’s presence of mind to be able to give directions to 911. And thank God that Emmett is doing fine.
Emmett – bounced back and it’s like nothing happened.
The rest of us … terrified at the fragility of life. Knowing that we looked both ways before we crossed the street and yet trouble still hit us! And if it isn’t raining tomorrow, knowing we will be going for another walk. Just maybe not quite so far – half a block instead of a whole block.
See the Difference!
Saturday, February 18, 2017
Emmett’s Uncle Ben captured this classic shot. Emmett has begun extending his arm and Ben caught it just as Emmett grabbed for his daddy’s nose!
It’s time for some Emmett clips that show his growth in stature but also in capability. The video on the right he was 16 months old while on the left he is 23 months old. Things to watch for are his head, arm, and finger movement. This one doesn’t really allow you to see his feet too well. And isn’t he pudgy as a 16 month old! And we will caution you right now, this is a family photo/video essay so get ready for it!
Those of you who know me well will appreciate this video clip. Believe it or not, Alisha asked for Mr. Potato Head. Apart from limited exposure at our house and a couple of stuffed Mr. Potato Heads at his house, Emmett has not been force fed this toy! Near the end of this video there is a cameo featuring Wendell the astronaut cat!
Ella the Elephant jumped into bed with Emmett to wake him up from his nap! While Ella has lost two of her legs, she’s still a great source of amazement for Emmett (and Wendell!).
It has been very exciting to watch Emmett’s hands and fingers as his dexterity improves. In the pic on the left (below) from April 2016 you can see how his wrists aren’t held as you would expect and his fingers are quite curled. Now his wrists are doing much better and, when he tries, even his baby fingers can be straightened.
Watching Emmett play with toys is so much fun. Watching him supporting his head is absolutely fascinating. And then wait until he has a temper tantrum (we don’t have that on video yet), where he will purposely toss his head off of the side of the head support and be stuck on the side. Scary at first but now that we know it is happening on purpose, it’s a bit frustrating. And funny!
Of course, then there’s taking advantage of Paw Patrol to get a fuss free tummy time workout. When Emmett was a baby and prior to being diagnosed with SMA, tummy time was encouraged to help strengthen his obviously weak muscles. Of course, the poor little kid couldn’t do anything and would cry like crazy at what he must have felt was akin to torture. Now, he’s literally making the beginning moves of crawling! Very beginning, mind you, but so exciting to watch.
It was sunny yesterday so Emmett was out for a neighbourhood roll! He had a lot of fun! The snow is also gone from his front yard so he was able to head out onto the grass. Next Tuesday his loaner Power Tiger chair will be exchanged for a little bit bigger chair. It will be more toddler friendly in that it will handle the bumps and lumps of a grassy terrain much better than this chair. The battery life should also be much improved.
Nothing like a storybook workout before bedtime!
March 2017
Happy Second Birthday! Emmett’s SMAzing!
Wednesday, March 08, 2017
On March 9, 2017 Emmett will be two years old and the celebrations started last weekend. Here in beautiful British Columbia our trip to the Vancouver Zoo was altered due to the temperature hovering around zero with a mix of rain and snow falling. Terrible weather. An outdoor trip was absolutely not a good idea so we did the next best thing … we went to a pet store! For Emmett this may have been even better. You see, Emmett really hasn’t been in a store since he was about five months old so this was an exciting experience! The sensory stimulation entering the store was fantastic. Lights, colours, fish in aquariums, rodents (in cages), amazing little song birds, and … giant ceiling fans! Plus huge aisles that, once Josh felt Emmett was beginning to pay attention to what was immediately around himself, Emmett got to cruise up and down in his chair. Some aisles more than once but pretty sure that Emmett got to all of them! Like many of our videos, this one is long and rambles about. You’ll see Emmett selecting a fish to take home. There’s some suctioning. And at the very end you’ll see Emmett getting strapped into his car bed. He cannot stay in his chair while being transported.
Roger and Rhonda captured this video of Emmett raising his hands and arms! Don’t you just love how he’s watching his hands and arms! How he’s literally willing them to go up! He knows! Just like when he was 6 months old and I could tell by the look in his eyes that he knew that his hands and arms used to work but then they stopped. He knew!
We are winning this fight right now but the battle against Spinal Muscular Atrophy is a lifelong one.
We all love someone who’s SMAzing. Aunt Jennie, Uncle Ben, Aunt Katie, and Uncle Josh weren’t able to join us for the birthday celebrations but we know they were celebrating with Emmett. Aunt Katie and Uncle Josh were planning on visiting the Melbourne Zoo on the same day we were at the Vancouver Zoo – but obviously that didn’t happen. Emmett, meanwhile, was mesmerized by the camera which was using the auto-timer (flashing in advance) to snap the photos.
At the end of the day Emmett was getting tired of having his photo taken but then he’s having fun playing hide and seek.
Emmett, you truly are SMAzing and we all love you!
To all of you who follow Emmett’s Journey, we truly thank you for your prayers and support. Your kindness and thoughtful comments and emails truly are appreciated. Two years old! What is next for this SMAzing young man!
Off To See His ‘People’!
Sunday, March 19, 2017
Visiting BC Children’s Hospital and Canuck Place
Update from Alisha and Josh at 1 P.M. PST Wednesday.
“Long day! Neurologist, blood work, nerve conduction tests, EKG, physio evaluation, and lumbar puncture, all by 12:00! Now time for a nap…“
Emmett is heading to BC Children’s Hospital where he will have a variety of appointments and, most important, another lumbar puncture and injection of Spinraza. We are praying and hoping that this goes smoothly for you, Emmett! And you too, Alisha and Josh.
Meanwhile, it’s time for a short clip of Emmett doing his favourite things. Playing in the bathtub and driving his chair outside!
Have as much fun as you can at the Children’s Hospital and Canuck Place. Enjoy visiting your friends! While we are cringing at the thought of the lumbar puncture, we are optimistic about what the results will be.
April 2017
Tuesday, April 04, 2017
That’s One Small Kick Inspite Of SMA,
and One Giant Kick At SMA!
On Friday Alisha called out, “Look at what Emmett’s doing!” Believe me when I say that we still drop what we’re doing and head to see what this little boy is up to now.
Ever wonder what a corduroy road feels like? Then take a wheel chair for a spin on a wooden pier! Emmett was enjoying some ocean air by heading out onto the pier at White Rock, B.C.
On Friday Alisha called out, “Look at what Emmett’s doing!” Believe me when I say that we still drop what we’re doing and head to see what this little boy is up to now. For instance, several weeks ago he learned to cross his eyes at will to get a laugh out of others – to all of our friends who are eye specialists believe me when I say I’m discouraging this. And he loves doing circles in his wheel chair or coming right up to you and then stopping. Or going ‘ssshhhh’ with his finger to indicate being quiet. This wasn’t one of those. He was sitting in his power chair and swinging his feet. That’s right. SWINGING HIS FEET! He will frequently move his toes. He will slide his foot off of the foot rest. He has swung his feet a little bit. He shows us a lot of leg movement when he is floating in water. But this time he was simply swinging his feet around and enjoying it. Can you imagine how huge this is and how cute it was to see him sitting there kicking and swinging his feet! There simply are not words to describe this moment. Perhaps a video will help. And isn’t he cute in his ‘jammies’!
Emmett loves toys. He has a lot of them just like every other two year old. Emmett is incredibly fortunate to have gotten a power wheelchair at 16 months of age and, while at first it was cute watching him try to manipulate the joy stick as he really didn’t have a clue how to operate it those first few days, it didn’t take very long before he was getting to places that he wanted to go. 9 months later and his power chair is in use daily. It is a part of his life and we are, without doubt, so lucky to live in a country and a province that supports the mobility needs of little ones. This chair is a tool that provides this little boy with the ability to interact with others. While the chair is not a toy, there are many toys which help him to expand his horizons and to stretch his physical limitations. Play and interaction with others are a key part of socialization and learning. Toys and games are an integral part of learning. We are looking forward to the end of flu and cold season to allow Emmett greater interaction with others. The ball on the rope is really to indicate stop the car when it touches the windshield – but it is the perfect height for Emmett when he is in his chair. And Emmett has learned how to push the chair’s control buttons to change speeds (also to go up and down). He’s such a smart little boy and learned this from watching adults (I only gave him one formal lesson).
Of course, then there’s Emmett’s neck and head control which is continuing to develop. Emmett requires support to sit up, however, the amount of support has reduced significantly. Plus he is able to hold up his head for longer and longer periods of time. What is really cool is watching him while Alisha is supporting him – and he tilts his head back to look at his mommy! Then he physically pulls his head back into an upright position – talk about strength!
Emmett’s Journey has made a point of celebrating his birthday every month. Now that he has had his second birthday we are going to continue with the blog and the posts will likely happen on his birthday. However, we are not going to openly celebrate his month birthday. We will be celebrating and sharing special moments and thoughts that arise as we’re laughing, playing, and probably even crying (at times) while we watch Emmett on his journey. Of course, there will be blog postings at other times too. Thank you for following with Emmett on this Journey. Thank you for your prayers, your support, your warm wishes, and the love that you have for our little Emmett!
P.S., not sure what a corduroy road is? Your homework is to find out. Ask someone who looks old (but don’t tell them they look old) or do a search on Wikipedia. And while Emmett is almost always happy, he does have his moments! But even amidst all of these tears, he’s holding his head up on his own!
May 2017
What do you do when it rains? Go outside and have fun!
Friday, May 05, 2017
It is no surprise that it rains in British Columbia. After all, the largest temperate rainforest in the world is found here in British Columbia. This spring also seems to have brought an abundance of rain to many regions across Canada. Emmett usually scrunches up his face and anxiously waits to be carried out of this ridiculous wet stuff. Alisha and Josh are gently pushing this little prima donna into exploring new horizons. Basic stuff like getting his hands dirty (guaranteed tears when that happens) and getting outside in the rain. Of course, with his first power chair we quickly learned that it was just as temperamental in rain as Emmett was – it would just stop working for a day or so if it even sensed inclement weather. This second chair is more robust but no chances are being taken so, with a giant blue poncho that protects both of them, we ventured out into the light rain. Emmett went a metre or two and just stopped. Unsure of what he was unsure about, we checked to make sure all health needs were okay and they were. Then realization dawned … Emmett was listening to the raindrops pitter patter on the poncho. Just like you’ve probably listened to rain falling on a tent (which it always does when you go camping) or a barn tin roof (which if you’ve never experienced that, find a farmer and ask if you can sit in her/his barn the next time it rains). After a few moments of listening and contemplation, Emmett was off and then it was a challenge to get him to come back inside! In fact, when we finished our outside play and took off the poncho in the garage, Emmett turned his chair around and headed right back out into the rain where he just kept on going. Running after him with the poncho, we got them both covered up and then stayed outside for another 15 or 20 minutes of enjoyment.
With the occasional arrival of wonderful spring weather, Emmett has been taking more and more excursions. Neighbourhood walks, excursions to hardware stores, and to local parks. Believe it or not, Emmett attracts attention. It is quite something to see this little boy proficiently wheeling his chair about and walks often take longer due to conversations. While Emmett still plays bashful with strangers, he is getting to be more and more of a showman. He does love people and attention. Alisha and Josh were a bit wary, though, when a group of boys approached Emmett in a park. All was well as they were quite excited about this little ‘baby’ and his awesome wheelchair. They coined the term ‘techno baby’ and asked a lot of questions about Emmett and his chair that Josh and Alisha answered. Another outing was to the local Tulip fields organized by Canuck Place Abbotsford. It was a beautiful day and the colours were fantastic. (Remember, click on the photos to get a larger version.)
Another excursion was to our local library. They have a wonderful ramp for ease of access and Emmett hasn’t been to the library since before his diagnosis, so this was a special moment. The library staff was wonderful and Alisha left with several new books for this voracious listener. I’m pretty sure that we’ve mentioned before that Emmett simply loves books. The best was Emmett waking up the other day, looking at his mom and wailing ‘boo(k)’. The ‘k’ was maybe more implied than pronounced – but the intent was quite clear. He wanted a story and he wanted it now! This little boy is an avid listener (we don’t think he can read yet!). And he loves to talk. Emmett is quite verbal when he warms up to people and so he responds too – not quite in a form that is understandable yet. His vocabulary is increasing and we are hearing more and more words every week. His two year old talk is incessant at times – so as he continues to jabber and get words out, we truly anticipate that sentences will follow.
Of course, family photos would not be complete without a pic including Wendell the cat! Emmett also had a great time finding Easter Eggs – so much so that after finding every single one, they had to be removed from his Easter basket and re-hidden. Just so that Emmett could do it all over again!
Alisha and Josh also got the news that we have really known all along. The study was unblinded and Emmett has been receiving the actual drug throughout the trial period. As I say, we have known simply because of the miraculous improvements we have seen. Of course, we have to caution you that miracles when dealing with SMA Type 1 are small. Small, but when compiled together they are absolutely HUGE! Yes, small miracles are so fantastic. For example, I was preparing to pick him up the other day and my hand was underneath him when he arched his back and there was literally air between my hand and his back as he was stretched out on the table. Simply incredible. Not to mention holding him and while supporting only his body and back, he holds his head on his own! Each of these little steps, so often taken for granted with little ones, is a major feat for Emmett.
Mother’s Day
Sunday, May 14, 2017
Mother’s Day – a time for mom’s to sit back and relax, enjoying the adulation of their spouse and child (or children). There may even be a pet in the picture. But mom’s don’t sit back for very long. Alisha learned a lot from her mom, Pamela. She’s learned from her grandmothers. She’s learned from Rhonda, Josh’s mom. And now Alisha is teaching all of us what it means to be a mom and to fight for every move that your little one makes. Alisha, Emmett is so blessed to have you as his mommy! We are blessed to call you our daughter and Josh our son-in-law.
In this photo you can probably see that Emmett is sitting with only his lower back supported, supporting his own head, and petting Wendell the cat! Thank God for the strength He has given to this family, for BC Childrens Hospital, Canuck Place, and the drug trial for Spinraza in which Emmett, one of 124 little ones world wide participating in this blind study, was getting this miracle drug. Only two out of three children in the study got the actual drug. Three little ones in the study at BC Children’s lost the fight with SMA – our hearts and prayers go out to their mom’s and dad’s this day.
The future ahead is full of challenges but the present is full of smiles – unless a precocious two year old tries to squash you with his several hundred pound power wheelchair in which case you likely won’t be smiling, but that frown only lasts a moment or two.
Spinal Muscular Atrophy, Type 1 – a fact of life for our family. We are fortunate that we can and do smile every day!
June 2017
Let’s Go To The ZOO!
Thursday, June 08, 2017
You can see it in his every move as he contemplates life and then acts upon it. I wonder if any of his actions are random – or if all of them are for a purpose. For example, the other day Alisha was putting him down for a nap. He had a fish, his favourite toy of the day, in his hand (I say that because his favourite toy of the last couple of months is still a soft, pliable plastic hippopotamus). Emmett was waving it around while Alisha was getting him ready for his sleep time when Emmett started fussing. Looking to see what was wrong it was obvious that the toy was missing. Emmett pointed to the wall side of the crib – a place similar to the Bermuda triangle that has swallowed up innumerable soothers, toys, and syringes. (Yes, syringes! Emmett is surrounded by syringes on a daily basis so of course he wants to play with them. That’s what little kids do. None of them ever have needles attached – just the blunt plastic end. Emmett doesn’t need daily injections but the syringes are used multiple times a day to flush his G-tube, which is his feeding tube). Well, Alisha got down on her hands and knees to search under the crib for the toy – but no sign of that fish within easy reach. A moment later when she was fully committed to the task of crawling under the crib to get the fish, she heard Emmett giggling. Not finding the fish, she popped back up to see Emmett into a full on giggle and waving the plastic fish which was now tightly held in his fist! The little turkey had pulled a fast one on his mommy! So smart and so funny.
If you have been following the blog you know that a visit to the Vancouver Zoo has been in the works for months – well, it finally has happened. Lions, Tigers, and Bears! Oh My. And most important of all – a hippopotamus! Of course, it was a hot day so the hippo’s just sat in the water and stared. But, ask Emmett if he saw them and he will respond with a resounding yes. Of course, the photo to our left where Emmett is obviously enthralled by some exotic animal … it was a squirrel! LOL!
Too often people assume – incorrectly – that since Emmett has Spinal Muscular Atrophy that his mental capacity is limited. That is absolutely not the case and he is an extremely intelligent little one. SMA traps these little ones’ bright minds inside bodies that simply don’t respond. This disease has limited him in so many ways, but certainly not his ability to think. And with the help of Spinraza, his medical team, and his mom and dad – Emmett is working hard to regain the abilities that he lost. He has a new speech therapist who, after just meeting Emmett, commented that if it weren’t for his disease he felt that Emmett would be chatting a mile a minute. Words are coming out more and more often. Grandma and Grandpa are quite clear! We are looking forward to even more chattering taking place.
We take so much for granted. We’ve said before that what happens with little ones with SMA, Type 1 is that the nervous system in the spinal column is quickly being eroded just like a mountain road can be washed away during spring floods. Sometimes so severely that it can never be repaired. Except in this case Emmett is a wonderful miracle baby involved in the drug trial for what is now called Spinraza. Approved by the FDA in the USA and hopefully approved in Canada by the end of this summer. This drug allows the messages from the brain to travel the spinal column network and make their way to Emmett’s muscles. And not just to his limbs – but that is most evident. This goes to the muscles that tell his lungs to work, that tell his throat to swallow or cough, that tell his lips and tongue how to form words. SMA is the #1 genetic killer of children because it is such a terrible disease. Spinraza is the first medical approach to REVERSE the impact of this disease. There are many who have survived through significant medical intervention and who have reached a point where their regression has halted and for a time plateaued, but this is a degenerative disease. There is no evidence of a child or adult with SMA (there are different levels depending upon the severity of the disease and the age at which it is discovered) have improved. Until this amazing drug and, hopefully, even more amazing drugs that are going to be discovered in the future.
A year ago Emmett was checked out to see if a power wheelchair was a viable option. In May 2016 (the photo on the left is from May 2016! Isn’t he cute!) he was just starting to lift his own head but he could not support it at all. He fully needed the head support. Any seams in the floor would jostle the chair and cause his head to flop and an adult would need to support it for him. If his arms fell from the chair arm rest, he could not pick them back up. He used one finger to manipulate the extremely sensitive joy stick – because he could not grasp it in his hand. What a difference a year makes. What a difference!
Emmett is getting ready to move back to a car seat from his car bed. He’s meeting (so are Alisha and Josh) with part of his team of specialists to see if a car seat is an appropriate option. He cannot travel seated in the wheel chair yet – His neck strength is simply not strong enough – and he is outgrowing his car bed. Plus he cannot see anything from his car bed which is strapped to the floor. Alisha has found a rear facing car seat that can handle Emmett’s size and weight for a few more months – and then it can be forward mounted. The wheel chair van needs a new exhaust – just one of those things. That van has been in the family for almost a year and it is invaluable for transporting Emmett, his wheel chair, and all of his gear. Hmmm – maybe we should have a blog post about all of the stuff that must go with Emmett.
Enjoy this collection of photos and video clips. We hope that, like us, you marvel when you see the movement that simply was not present just twelve months ago!
In this video clip you may watch and yet not realize what miracle you are seeing. Emmett is reaching straight out in front of himself to grab the tinfoil shells. It wasn’t that long ago that Emmett had to lift his arm from the side and then attempt to move it to the front – often an impossible task. Yet here we see Emmett reaching out straight in front. Passing things from one hand to the other. Doing things we could barely dream of him doing a year ago. Things that in the fall of 2015 we couldn’t even comprehend.
Okay, this next one is a longer one. Just so you’re prepared before you start to watch it.
Father’s Day
Monday, June 19, 2017
One of Emmett’s first words was ‘Da-da’. When Emmett sees family photos – even now – the first thing he sees and says is ‘Da-da’. If a white car coes down the street, ‘Da-da’ comes out of Emmett’s mouth. When a car pulls in the driveway and Emmett hears it inside the house, ‘Da-da’ is his first thought and expression.
Emmett loves his daddy. Emmett also loves his mommy and there is no question about that. No reason for mommy insecurities with this little boy. The reason for this focus upon Daddy is that daddy leaves for work … and church … and other activities. Mommy almost never leaves so the security is there. But Emmett’s daddy has to leave! And all of us surrounding Josh, Alisha, and Emmett know how very hard it is on him to be away from his little boy. We also know how hard he works to arrange and rearrange his work schedule so that he can be there for as many of Emmett’s crucial moments as he can be!
Josh is a fantastic daddy and when you see the two of them together you will recognize that. Josh reads stories and allows Emmett to get out of some stretching exercises. Emmett and Josh play catch and explore the back yard and neighbourhood. And they go swimming together in the big local pool. There are so many things that they do together! It is, however, tough to watch hockey together as Emmett so far chants ‘Off!’, ‘Off!’, ‘Off!’, ‘Off!’, ‘Off!’, ‘Off!’, ‘Off!’, ‘Off!’, ‘Off!’, over and over again. He will learn, but right now hockey is simply not as good as Paw Patrol!
What is the sign of a great daddy? I am not qualified to answer that question, but according to Emmett, it is his daddy!
Josh, thanks for being a fantastic daddy.
Love from Alisha, Rhonda & Roger, Pamela & Frank
Up! Up! Up!
Wednesday, June 28, 2017
IMPORTANT UPDATE:
Health Canada APPROVED Spinraza today – June 30, 2017.
Up! What a common word for a little one around the age of two. It is used so often. To get mommy or daddy to pick them up. Perhaps to describe what needs to happen to a toy that has fallen. Maybe to express the feeling of making their way up the stairs … or being carried up the stairs. After going down a slide it is very appropriate for Up! Up! Up! to describe the need to go back up and down the slide again. Emmett and I used to have the routine of reaching up to the ceiling (sort of like in the Lion King) so that Emmett could touch his ceiling airplane light in his bedroom. It was a must happen ritual when I had the opportunity to put him in bed for his afternoon nap or to sleep for the night. It was repeated for many days … but Emmett has moved on from that. So when I heard this refrain as he was in bed and I was setting up his equipment for nap time, I wondered if he wanted to reclaim this ritual that has passed by the wayside. But then quickly set that aside with the realization that this two year old simply wanted to get up out of bed to continue playing. Imagine my surprise to turn and see him smiling at me, repeating Up! Up! Up! and touching his knee, which was raised up towards his chest as he was lying on his back. Very proud of himself and wanting me to see it. Maintaining his raised knee, with his foot off of the mattress, he then followed suit with his left leg. He’s done this before, of course, and his mom and dad have observed it many times. Pam and I have seen it in water, but not like this. It was pretty exciting. To top it off Emmett raised both hands up into the air in a form of celebration and pure enjoyment of life. [Normally we do not show diaper pics, but this was such an amazing moment that we hope you will pardon the additional exposure that we are sharing in these photos.]
Enjoyment of life … Emmett truly enjoys life. The drug that provides Emmett with these new pathways for the messages to traverse the nerves in his spinal column is still experimental in Canada. We hope that it is passed soon. That is a major hurdle. It may pale beside the next hurdle which is finding out how this extremely expensive drug, the only one that works on this rare genetic disease, will be funded to allow Emmett to continue to receive it. The trial he is a part of continues through to 2018. What happens after that? Will the drug be approved in Canada? Will there be funding for it? We don’t know. There are no answers to those questions yet.
What we do know is that this drug, Spinraza, is life changing for those afflicted with Spinal Muscular Atrophy. Emmett was diagnosed with this genetic disease at five months of age. Between four and six months Emmett had lost a huge amount of muscle control with only minimal ability to turn his head while lying down or raise one arm. Before his diagnosis Emmett was a ‘floppy’ baby with weak muscle tone. He was a tummy breather and had a bell shaped body – his abdomen was much larger looking than his chest. The seriousness of this disease was evident when Emmett began having issues with choking on his own saliva. You see, it isn’t just limbs that this disease affects. The muscles that enable swallowing. The ability of the lungs to function so breathing can take place. If a child gets this far, the formation of words and speech. Spinal Muscular Atrophy is the number one genetic killer of children. Period.
Spinraza is the first drug proven to affect positive change in those afflicted by SMA. Emmett has been enrolled in a trial program for this drug (which has had at least three names during this time) since the fall of 2015. Earlier this spring our assumptions that he was getting the real drug all along and not the placebo were confirmed (we were sort of in suspense for over 16 months waiting to hear). The changes have been small and have taken place slowly but cumulatively the changes are absolutely incredible. This drug has been approved in the United States. This drug has been approved in Europe. This drug currently has a special access program in Canada as Biogen, the drug company that owns this wonder drug, seeks for Health Canada approval and that provides hope. Hope for a medical treatment that was not available when Emmett was born. Except, of course, this trial study in which he was one of 124 children worldwide participating. A study in which only 2 out of 3 of the participating children received the drug; 1 out of 3 got a placebo.
This is not an instant cure. Emmett is still a ‘floppy’ child. He cannot crawl, stand, or walk. He has trouble picking up heavy objects but he is picking up all kinds of things that at one time we never thought would happen! As we’ve already said in previous blogs, he is sitting with limited support. He’s going to be moving from his car bed to a car seat (well, not for long trips). And he can lift his knees up to his chest! Wonder what will be next? Believe me, so do we! What we do know is that as long as Emmett is able to continue treatments, then Up! Up! Up! is the long term view.
And yes, Emmett has watched the video “Up”.
Visit Cure SMA Canada to stay up to date on the approval process of this amazing drug, Spinraza, here in Canada.
IMPORTANT UPDATE:
Health Canada APPROVED Spinraza today – June 30, 2017.
July 2017
Nothing Like A Hug!
Saturday, July 22, 2017
Emmett had another great trip to BC Children’s Hospital (July 19th) which included a couple of nights stay at the amazing Canuck Place Children’s hospice. Some parents of SMA children are posting that they’ve had their first, second or whatever number of injections of Spinraza their little one has had. We were going to post a number and then we realized that we do not know. It is not that this isn’t important to us, because it truly is. The fact is that Alisha and Josh were intentionally kept in the dark about this because Emmett was part of a blind study that, for him, started in the fall of 2015. And even though they have been told that Emmett has been receiving the drug from the beginning, this study with Emmett has included some ‘sham’ procedures so we simply don’t know. But we did ask and we will find out.
This was also the first time that Emmett’s family was able to be present for the injection. While the honour of being present for a lumbar puncture may not be what every parent wants to attend, it was important to be there for Emmett. Of course, the doctors and nurses are all so caring and gentle with Emmett. The Doctor doing the procedure was one of the specialists who initially diagnosed Emmett and it has been fantastic to have him following Emmett throughout. He has been present for all of Emmett’s procedures to date. The good thing about family being present was that the procedure was not as bad as what imagination leads you to believe. What Emmett disliked the most was the position that he had to be held in. Phew! Imagination is so much worse than real life – sometimes.
Prior to the lumbar puncture Emmett went through a morning full of tests and appointments. A physiotherapy session to do a standardized series of tests mandatory for all patients in the study. We were thrilled to see Emmett show off and also trying to encourage him to do things that we know that he can absolutely do at home! But of course, we couldn’t do anything that would throw off the standardized testing so Emmett left some of his ‘capabilities’ on the table. For next time. At six months of age Emmett’s physio study had quite a few zero’s because he simply had no muscle reaction. You’ve heard us say he was a floppy baby, well, he absolutely was and the brain’s messages simply never got through and the muscles just sat there. This time around Emmett was getting some great scores. In fact, if he continues on this pathway in the next year (maybe a bit sooner) he is going to pass this assessment level and be given a more challenging set of tests. That will be both exciting and challenging, as we know that it will take some time for him to develop the nerve pathways and strength to accomplish these more challenging tests. From the Physio study he went off to another section of the hospital where a nerve study was conducted. This is one session that Emmett is always happy to leave behind as they ‘stimulate’ the nerves and it is not something that he enjoys. They loved seeing him and were very happy with what they saw through the testing.
Throughout the course of the day we saw two of the study coordinators a couple of times and, as they have also been following Emmett from almost the time of his diagnosis, it is fantastic to see their reaction as Emmett moves about. The study coordinators (there are two – one who was blinded from the testing just like us and another who was unblinded and very aware of the process throughout. But I haven’t asked for permission to say their names so right now you are going to have to stay in the dark about who they are!) are such key members of our Emmett medical team and we love them. Of course, Emmett saw his neurologist a couple of times for assessment before and after the lumbar puncture. His specialist was so pleased with the performance that this little boy was exhibiting with his muscle tone. In fact, just before he was discharged all of us (except Emmett) giggled when he held his arm bent while his doctor was trying to extend it! Usually you want your child to cooperate with their specialist, but when your little one who usually has no strength is fighting to keep the Dr. from straightening his arm, you simply have to cheer him on! We’re pretty sure that he did all of the other assessments that were completed, but at the end of the day he simply was done and there was no way that he was going to allow his arm to be straightened. And he demonstrated his strength and will power by not letting it happen!
Just holding Emmett now, you can feel the muscle tone in his body. There is muscle tone throughout his core. You can feel him flexing his leg muscles and, as evidenced in the pics in the last blog, watch him lift his legs. Tightening his core tummy and butt muscles – even lifting his butt from the floor mat. And of course his neck muscles are getting stronger and stronger. It is so fantastic to watch. And feel. Like this next paragraph.
The latest milestone that Emmett has accomplished is that he is giving hugs. Alisha and Josh have never, until recently, had a hug from their little darling boy. The first one to get a hug was Alisha and Emmett ‘finger crawled’ (see the photo and his crawling fingers!) his arms up her shoulder and around her neck. Talk about an earth moving and teary eyed moment, this was one! Getting a hug from Emmett – simply wow! He gives them out very sparingly.
Other news was the very huge update that Emmett will continue in this drug study for another five years. What is so momentous about this is that while the drug, Spinraza, was recently approved for use in Canada it is still too early to know how this extremely expensive drug will be covered. According to the financial website “The Motley Fool”, Spinraza is the third most expensive drug in the world [https://www.fool.com/investing/2017/04/18/the-7-most-expensive-prescription-drugs-in-the-wor.aspx]. This is beyond the ability of a young family to handle thus the knowledge that the testing is ongoing into the 2020’s is fantastic. By then we hope that the province and insurers will have the funding question sorted out.
The trips to Vancouver are numerous so it is wonderful that Emmett lives as close to the city as he does. It looks like he will have one or more appointment in the city every week through the month of August and then a few in September also. The wheelchair van that Alisha and Josh purchased last summer has been an absolute gift. There is no way to transport people along with all of Emmett’s equipment, not to mention his power chair, in a vehicle any smaller than a van. Alisha’s mind is an absolute whirlwind of lists and checks as she gets ready for a trip to grandma and grandpas for the evening – so you can imagine when it’s an overnight trip to the hospital. And on this most recent trip Emmett’s nasal mask on his BiPap broke. Just from normal wear and tear but it is something that has never broken before so the spare had been left at home. Ironically, Alisha has had a spare one for every other trip except this one! This BiPap breathing machine provides Emmett with support probably 12 to 15 hours daily during a normal day. Longer if he’s having breathing challenges so absolutely a must have after a significant hospital procedure like this. After a lot of calls and moments of panic one was located at BC Children’s hospital so all was well (and saved us a 3 hour trip to Abbotsford and back). And another “cannot leave home without it” item was added to Alisha’s mental and physical checklist of what is necessary to keep her little boy safe and secure.
Of course, the safe haven called Canuck Place is simply wonderful. This majestic old mansion has an interesting history with a few twists and turns, but it is such a wonderfully caring place for medically fragile little ones. There are beautiful gardens, accessible play areas, and wheel chair accessible pathways for Emmett to visit and explore. They have an awesome fish tank that he loves to watch. Poppy the service dog is a great favourite – although Emmett is still rather reserved around Poppy. He loved counting the bees that were visiting the flowers – we cannot make out the numbers yet but his pointing and verbal intonation clearly indicated what he was doing. The awesome staff there cheers Emmett on and, once he moves past his bashful mode, he is quite a showman and a chatterbox. It is impossible to say just how important all of Emmett’s caregiver’s and medical team are to us. From the bottom of our hearts, thank you so much for everything you do to support Emmett. And to all of you who are supporting Emmett on his journey, thank you for following along. And don’t forget, many of the photos will expand when you click on them.
The next adventure – Emmett is going camping in the wilds of B.C.’s mountain country … well, Grandma and Grandpa Willms’ driveway will seem like mountain country to Emmett!
August 2017
Monday, August 07, 2017
August is SMA Awareness Month
Spinal Muscular Atrophy – did you know that 1 in 40 people is a carrier of this rare disease. Did you know it was that common!
Feel free to share Emmett’s Journey with others so that others know about this rare but all too common disease.
Cups. Plates. Utensils. Plastic food. All of these things are fascinating to Emmett. He loves to join us at the table and when we pray for our meal he closes his eyes (sort of, cause he likes watching everyone else) and reaches out his hands to hold hands with everyone else around the table. And then he starts to play with his toy setup. Right now he is really into cups. Of any size. Of course, Emmett doesn’t get any food or liquid by mouth. It is all by G-Tube directly into his stomach so all of this food is just pretend. Taking sips from his cup of coffee – just pretend. Give him real food to play with – and his face contorts and he will push it aside with disgust. He doesn’t even want a taste. His hands must get cleaned off if the food was slimy feeling. Give him a plastic pizza, hot dog, or fries and then he’ll eat. Just don’t give him the real stuff!
Emmett wants you to know that he loves books. He wants you to know that big trucks go ‘Honk’ and little trucks go ‘Beep’. He wants you to know that cows go ‘Moo’, horses go ‘Neigh’, cats go ‘meow’, geese ‘honk’, ducks ‘quack’, pigs ‘grunt’, and hippos ‘hiccup’. You don’t have to agree with him, but that is what they do. On Sunday his daddy was telling the story of the prodigal son – who had a job feeding pigs – and managed to keep a straight face when Emmett started grunting like a pig. Emmett was sitting right in the front of the church! Emmett wants you to know that he pays attention to everything.
Camping at Grandma and Grandpa Willms house was so much fun. Out in the country and able to enjoy the countryside. Wild deer visiting and walking right past them! Getting to go to the lake and see ducks and geese – which started the infamous phrase ‘ Duck. Duck. Goose!’ Getting lots of time with daddy as he was on holidays. Lots of trees and lots of rocks (Emmett is still fascinated by rocks!) to view and play with. It was an incredibly relaxing time for all three of them. And camping at the grandparents gave a sense of security that was necessary – you simply double think everything that you used to do when you have a medically fragile little one.
We all want you to know that August is SMA awareness month. The Willms and Ewald families first learned about Spinal Muscular Atrophy two years ago in August. Emmett was diagnosed with SMA Type 1 in August of 2015. None of us had ever heard of this disease. It’s genetic and our families have been silent carriers – there’s no history in either family of this disease. When two carriers have a child, there is a 1 in 4 chance that the child will have SMA. 2 in 4 chance that they will be a carrier. 1 in 4 chance that they will not carry any aspect of this disease. Odds like this are overwhelming and terrifying. Fortunately for Emmett, since September 2015 he has been part of a trial study which has led to the approval of the only drug that has been proven to improve neuron pathways in the spinal column thereby allowing the brain to communicate with the muscles. It is not a cure, but it is certainly giant steps in the right direction. This is the first treatment available. Up to now it has been physio, hope, and prayer. These still play a huge part in the care plan of any SMA youngster.
There are four basic classifications of Spinal Muscular Atrophy.
SMA Type 1 is the most significant and identified before 6 months of age. Physical challenges include muscle weakness and trouble breathing, swallowing, and coughing. SMA Type 1 is the number 1 genetic cause of death of infants. This is the diagnosis Emmett received and, with the help of the drug Spinraza, that he is defying!
SMA Type 2 is usually diagnosed after 6 months but before 2 years of age. Individuals with Type 2 can usually sit up but unable to stand or walk.
SMA Type 3 is usually diagnosed after 18 months and before age three. It may not be diagnosed until the teenage years. Individuals with Type 3 are initially able to walk but have increasingly limited mobility as they grow.
SMA Type 4 is rare (actually, all who are afflicted with spinal muscular atrophy are considered to have a rare disease) and it affects adults. Usually the symptoms are minor motor impairment.
Emmett wants you to know that he loves to interact with others. It is summer right now so that is so much easier because the risk of colds and flu are simply not as common. His family want you to know that disinfectant is everywhere around Emmett to assist them in reducing the risk of catching a cold. You see, if a child has trouble breathing and coughing and swallowing then a simple cold could be devastating. The flu – overwhelming. Even so, Emmett loves interaction. And Emmett wants you to know that he really doesn’t like wearing his bi-pap breathing machine! This is a must to give his lungs support and ensure his oxygen levels are great while sleeping. If he is extra tired or congested, he wears it during his waking hours too.
Talking. Emmett wants to talk and he has a pretty good vocabulary already – but you need to be used to listening to him. SMA also impacts his ability to form words. It affects the volume of his voice. But that doesn’t stop Emmett from communicating. All along he has used his eyes, his expressions, and his ability to point. Sound has played a big part and more and more words are coming out. Just recently he has learned how to make a ‘TH’ sound and his favourite word is ‘THIS’. Unfortunately, those around him do not always recognize what is the ‘this’ that Emmett wants. After all, there are a lot of this’s in his house, let alone in the world. He has also been saying his own name! You see, while Emmett’s body is physically very limited his mind and aptitude is incredibly active and sharp. I think that he is brilliant.
Activity is incredibly important. Emmett undergoes a daily physio routine with his mom and his dad. Stretches. Movement. Water therapy in the bathtub. Emmett needs to work his muscles. He is so proud when he reaches out and grasps something. He knows that when he pulls his knees up to his chest that he is doing something monumental. That when he stretches his arms up into the air that it is a huge accomplishment. You see, I know that when Emmett was only 6 months old that he knew his arms wouldn’t do what they used to. That he couldn’t hit his balloons the way that he did just a few weeks earlier. And now he can and he knows that this is something to celebrate. Yet it is also going to be a challenge because the more he interacts with others and sees them running and jumping and rolling around, the more questions Emmett is going to start wondering about and asking. We will face those together, right now we will continue to celebrate every improvement. It may be miniscule compared to others, but for a youngster with SMA Type 1 it is huge!
Emmett is now using a rear facing car seat – a Graco 4 in 1 – in combination with his car bed. It will take him a while to get used to it, but there is no question that he loves it. This is an off the shelf item which offers great head support and offers among the longest times that a child can stay in it and stay facing the rear. Very important to protect his head and neck. The first time he rode in it he was so happy and chanted “Bus. Bus. Bus.” over and over. You see, the car bed is strapped into the floor of the van where it is impossible to see out the windows. One more step to help Emmett feel more and more a part of everyone else’s world.
Water physio is a daily part of Emmett’s routine. Usually it takes place in a bathtub. Once every couple of weeks in a community pool. And when there is a heat wave like the past few weeks here in B.C., in his kiddie pool outside. Which he hates because it is cold. And he loves because once he gets into it and used to the cold he has room to move about and have fun. And so does the local wild life! One morning Alisha and Josh found all of Emmett’s pool toys scattered around their back yard. One of the sponge balls – a Paw Patrol sponge figure head – had the nose and face chewed off. The pool was almost empty of water. It wasn’t until a day or two later that Alisha caught the culprits. A family of raccoons came into the yard while she was watering her garden – and looked at her like she was invading their space! Needless to say, they don’t leave toys in the pool anymore.
Splish and Splash at the Lake
Friday, August 25, 2017
At Chilliwack Lake Emmett was content to toss rocks into the lake – especially after his toes were dipped into this glacier fed lake in the mountains. The second lake was Rolley Lake (both are provincial parks) and the water temperature was fantastic. Emmett quite enjoyed himself.
The video says it all! Emmett’s lake crawl. Spinning around. Going for his first canoe ride.
Emmett stopped being able to use his legs between 3 and 4 months of age. His arm movement diminished greatly between 4 months and 6 months of age. LOOK AT HIM NOW! Support CureSMA as they fight to find a cure. CureSMA Canada CureSMA USA
September 2017
Emmett is in the Hospital
Tuesday, September 05, 2017
Emmett has a cold. Alisha knew that something was starting mid-week but it was just a slight fever. Saturday he was a miserable little boy. Sunday he seemed to be bouncing back but late Sunday night he took a turn for the worse. There was no question that he had a cold. One that caused him to have a bit of a frog in his throat which, honestly, made him that much more adorable. But late Sunday night there was nothing adorable but only the urgency to get him into medical care. The van was packed and Emmett was on his way to Vancouver and B.C.’s Children’s Hospital. The advantage of travelling in the middle of the night on a long weekend was that the normally always busy highway 1 was almost empty so no traffic issues at all.
The Emergency Department was a bit more populated but still not bad. Emmett was immediately in a room and being checked out. He was upset when they checked his blood pressure and took his temperature. He doesn’t like the blood pressure cuff. Or the thermometer which has to be held under his arm pit for a few seconds before it registers. He did, however, love seeing the hospital staff. It was not too long before they determined that he did have something in his lungs and he was admitted to the ICU.
A slight cold. ICU! We have said before that a cold can be incredibly threatening for a little one with SMA. Emmett hasn’t been rushed to the hospital since this past November. We’ve been lucky but there is no question that he needs to be there now. Obviously, his sleep patterns have been drastically disrupted. Into an isolation room at ICU where his nurse is literally right outside his window. He’d just fallen asleep again when a respiratory technician came to check him out. Almost instantly made the move from Emmett’s little elephant nose bi-pap mask to a full face mask that was obviously connected to hospital equipment. This woke Emmett up and not only was he grumpy but he began fighting the mask. This was looking like it was going to be a long night! Then we looked into Emmett’s eyes and told him it was a Buzz Lightyear helmet. Almost instantly he stopped fighting the mask. You see, for the past two weeks Emmett has been on a Buzz Lightyear kick. He has been watching the movies on every car trip. The stories have had to be read to him over and over. His favourite word right now is “Buzz”! Emmett realized that this mask was indeed like Buzz Lightyear and, at 4:30 in the morning with a look of recognition in his eyes, he stopped fighting and shortly fell back asleep. And without much fuss he has been wearing the mask ever since. Hmmmm. Was that a lie or merely misdirection?
Emmett has a chest infection. They are not saying pneumonia because that is a specific type of chest infection. His lungs are full and that is a serious concern. But he is stable right now and throughout this past night has been having RT’s doing deep suction of his lungs every three hours. And they have been getting a lot of secretions out! For the medically bright folks reading, his regular bi-pap settings are still being used and he is not getting additional oxygen. He’s just getting normal atmospheric air. He is showing strength and fighting this. For all of the rest of us, this is a sign that he is a strong little boy and fighting this the way that he should be doing. And that mom made a timely call to get to the hospital.
Emmett ‘Buzz Lightyear’ learned how to make elephant sounds with his mask. He started doing this mid-day yesterday and Alisha wondered what was wrong. Nothing, actually, except that the mask was a little loose and Emmett had figured out that he could make these elephant sounds so was purposely expelling more air in quick succession to sound like a very noisy elephant. His little tummy inflating and deflating in quick succession. If you were there you would have laughed with us, because he was having fun and loved being an elephant. That was somewhat short lived as his mask was adjusted so that he couldn’t force the air past the mask seal and his forehead anymore.
He will be in ICU for a couple of more days. While the deep suctioning makes him quite mad, he is doing okay. He is placing orders and making requests. For example, through the window into the next child’s room he saw they were watching TV. While it took the adults a while to figure out what he was pointing at, it was eventually quite clear than Emmett was not pointing at the child in the next room (there are large windows between the rooms) but their active TV and his inactive TV. We got the nurses to turn it on and then went through a range of channels. He very clearly was saying no, no, no as we flipped through the channels until we arrived at an obviously child’s show. And then he was content.
Alisha is beyond exhausted as she hasn’t been getting much sleep. Josh is not too far behind. Emmett’s sleep patterns are very disrupted. These are things that we would appreciate you praying for. And especially pray that his lungs clear quickly. A cold and chest infection during B.C.’s hottest summer on record – we want this cold gone very quickly.
So Emmett is stable. Every once in a while he raises his arm with his index finger pointed upwards. We fill in the words for him – “To Infinity and Beyond” and then Emmett ‘Buzz Lightyear’ puts his arm back down. If you know Toy Story then you will know and realize that he is in character as Buzz! Get well quickly, Buzz, as we want to see you back home!
Emmett is Improving
Wednesday, September 06, 2017
Emmett is improving! He will be in the hospital in ICU for a few more days, but as you can see in the photo the full face mask has been replaced with an elephant nose mask. This means that his expressions and vocalizations will be much clearer. It also means that his lungs are not as stressed as they were before. This little mask makes it much easier on Emmett. You can also see that Buzz and Woody, along with Emmett’s faithful bunny, are there helping Emmett along. His lungs are gradually making less and less sound as they are clearing up. His oxygen levels are improving and his heart rate is slowing back down.
Your prayers and warm wishes have been so gratefully appreciated. And are still needed. Alisha and Josh are exhausted. We need Emmett to get all better so that he can return home!
Emmett Is Home!
Tuesday, September 12, 2017
After 9 days in the hospital, Emmett is home. The last day and a half Emmett was really getting fussy and irritated – showing that his health had returned and that he was ready to go home.
He has really missed ‘vroom vroom vroom’, which is what he calls his wheelchair. Today (Tuesday) was the first day that he got to go wheeling around in it. He still needs to use his Bi-Pap and he has to be carefully watched, but he is better and he is home. Thank you so much for all of your prayers and best wishes and support. Alisha and Josh and Emmett really thank you for your support.
September – What a Month!
Thursday, September 28, 2017
This has been quite a month for little Emmett. Of course, you’re very aware of his lengthy stay in ICU as he overcame a cold. It was hard on him but he was really a happy little camper throughout the ordeal. But that all changed just a day or two after he was released. He came down with an infection that was not easily identified and he was simply miserable. He didn’t want to be touched, picked up, or held! While the last blog post shows him in his wheelchair, he really hasn’t been in it much since then. It literally seemed like he was crying and moaning all of the time. This little boy whose normally brilliant smile is everywhere was miserable and simply had trouble smiling. His sleep was disrupted. Everyone’s sleep was disrupted. Finally, after a couple of doctor visits, Emmett got on an antibiotic for what Emmett could now describe by pointing to his ear as a very sore ear. He had a bad ear infection. And just now, at the end of September, is he finally bouncing back.
The ongoing challenge is that Emmett has really lost a lot of strength over this past month. Even as he is bouncing back there are obvious concerning factors. He does not like sitting up right now. This was a huge milestone that he had attained and we know it will come back, but right now sitting up is obviously very uncomfortable for him. He is still bringing his knees up, raising his arms, and rolling to his side. Obviously, we are concerned when we see anything that seems to be holding him back.
The good thing is that Emmett is smiling again. And talking. Unless his cousin Henry is nearby. Or if he’s on FaceTime with his Uncle Josh. Or meeting with his speech pathologist. Or virtually any guest/stranger. Then Emmett’s eyes become fixated upon someone that he knows and he clams up. Once they leave, however, then a stream of two year old language lets loose! I can understand not talking with Henry, because baby Henry is going to be laying claim to Emmett’s toys so he is already showing dominance by being aloof. And Uncle Josh does have an American/Australian accent which is confusing. Why he won’t talk to his speech therapist when he loves to chat with his nurses and doctors – mystifying.
If you were wondering, the Buzz Lightyear and Woody the Cowboy phase is still going strong. Emmett has claimed my Buzz Lightyear and, gracious grandpa that I am, I have loaned Buzz to Emmett. On condition that I get to play with him (Buzz Lightyear) when I come and visit. Fortunately, Emmett has his own Woody the Cowboy so I can keep mine. The thing is, my Woody the Cowboy talks and Emmett’s doesn’t. I’m wondering if the writing is on the wall …
There is a lot of anticipation about Emmett trying out the MegaSeat wheelchair that we built for him. Unfortunately Emmett is not ready to use it. He has to be comfortable sitting with limited support before he can use this homemade device. So right now it looks cute and Winnie the Pooh has claimed it as his spot! But whenever Emmett is ready for it, we’ll give it a go. Several of the photos included have Mill Lake in the background. We had a lovely stroll and roll with Emmett the other evening. He loved seeing the water, the ducks, and the geese. He’s relatively comfortable in his electric wheelchair now – because it has significant back support and, if required, head and neck support also.
This weekend he’ll be heading to Camp Squeah with his parents and us for Level Ground Mennonite Church’s fall retreat. The weather forecast is not the most promising, so we will let you know about our weekend retreat soon. Until then, Emmett’s going to be rolling along. But a little bit slower for the immediate future.
If you’re curious about the DIY Wheelchair click this link, then there are more details and a parts list on MotoIQ.com – great to see an online automotive publication take an interest in a project like this. I’m glad to be associated with them.
October 2017
31 Months
Tuesday, October 10, 2017
There was no post on October the ninth, which was the start of Emmett’s 31st month. It was also Thanksgiving and a time for gathering with family and friends and thinking about all of the things that we are thankful for. And there are a bunch. But that is not why we missed getting a post out on the ninth. And Emmett is doing very well – that didn’t stop the post. He’s feeling very good and has bounced back from his September health issues. This is a good thing because we want him to be strong as we head into flu and cold season. Emmett got to go out to a corn maze where they didn’t get lost, but for Alisha and Josh it seemed like it as Emmett would have stayed all day. Searching for pumpkins was also great fun. He found it so intriguing. His mom and dad had to literally drag him away! LOL!
Earlier this month Emmett was back at the Greater Vancouver Zoo. While there his favourite animal, the hippopotamus, was swimming around and submerging and then popping its head back above water. It even yawned once – but Emmett had his chair turned so didn’t see the yawn. Plus Emmett spent a couple of days at Camp Squeah, just outside of Hope, B.C. Lots of trees, fresh air, and he got to see a deer family eating lunch in the camp’s garden. He also got to wheel around in the gym while everyone else was having meals. The great thing about a G-Tube is that Emmett isn’t tied down by three meals a day – which meant great times in the empty gym.
And Thanksgiving at his Grandma and Grandpa Willms home was lovely. It started off with an amazing scavenger hunt in which Emmett had to scour their large property to find toy animals that were hiding everywhere. There had to be two dozen or more of the little critters waiting for Emmett to find them. Then great family time that included his Great-Grandma and Great-Grandpa Willms. Emmett napped while all of us enjoyed a turkey feast.
As both Woody the Cowboy and Buzz Lightyear are still very much a part of Emmett’s daily activities, you’re going to have to wait until the end of the month to see which of these characters actually shows up at the Willms’ home. Emmett doesn’t know, but even if he did he wouldn’t tell you! He’s great at keeping secrets. Especially if he doesn’t know you well, then his lips will be sealed.
Thankful – for a medical community that supports Emmett and his family in so many wonderful ways.
Thankful – for the drug trial that Emmett has been part of for over two years now. This drug, called Spinraza now (it’s had a couple of names), literally amazes us.
Thankful – for every smile that Emmett shares! You cannot believe how adorable he is. Or how big he is getting to be.
Thankful – for his power wheelchair which gives him great joy and freedom to move about. And amazed that the moment he was set up in his colourful wheelchair, he grabbed the wheels and started to attempt to move them. He needs back support right now plus doesn’t have the upper body strength to get it rolling on his own, but he’s trying. With a little nudge he can keep it moving for a little bit.
Thankful – that his speech is getting better every week. Obviously those familiar with him understand him much better than strangers, but he is certainly making progress. And you should hear him sing! Of course, he still is in a shy mode so there is no singing or talking or verbalizing anything when strangers are around.
Thankful – for so many things. We cannot recount them all.
But you know, even though we are thankful for so much this is still tough. Would we be more thankful if Spinal Muscular Atrophy Type 1 hadn’t had it’s grip on Emmett’s body? Would we be less thankful? Would I know my grandson as well as I now do? You know, I am so thankful for this little boy, his mommy and his daddy. But this disease, Spinal Muscular Atrophy, this is a terrible disease. It affects babies and, when it is at its worst, SMA stops them from moving and swallowing and breathing. It changes families. Every aspect of Alisha and Josh’s life revolves around how SMA has affected their family. They cannot run out to the store without making plans … the store! Seriously, they cannot even run out into the front or back yard to do yard work.
Why wasn’t there a blog post on October 9th, Thanksgiving day? Here I must put in a disclaimer and specify that this is me and me alone talking. Not Emmett’s mom or dad. Not his other grandparents. Me. There was no post because I wasn’t sure that I was thankful. I was and am angry with SMA. I’m angry at how it affects not only Emmett, but so many other little ones around the world. It shouldn’t happen. Of course, that can be said about all of the other diseases and natural disasters and horrible things that happen in this world we inhabit. It shouldn’t be. But with SMA, it is right here with our family. We see it every minute of the day. And as a grandparent I’m not even right there – I still do my own stuff. Emmett cannot. He will need ongoing support. Period.
So this blog is a few days late … but I can say that I’m thankful. I’m still angry – but I’m thankful. I am thankful for this little boy that we love with all of our hearts. This little one who marches to his own drummer. Who has started doing scissor kicks in the bathtub. Who tries to roll his own wheelchair. Because … There is hope. There is always hope. SMA – well, we will temporarily forget about you. Because we have a little boy to read books with, to count with, to practice talking with, and to laugh with! Laughing with my grandson – there is nothing better. And I’m very thankful for that!
If you look behind Emmett in the above photograph, you’ll see his tracks in the grass. He wanders. Goes left and then goes right. Follows the sounds and sights on his journey. His path, like any other two year old, is rarely a straight line. And that path Emmett created is an analogy of life. We may want the straight pathways like Tom (Tom, thank you for the ramp you made for Emmett and his wheelchair. We’re thankful for that too!) made while he was cutting the grass on this large field; Emmett’s path will likely always be like a wibbly-wobbly line, it is going to take longer to get from A to B, but perhaps along the way everyone with him will shout ‘Yee-Haw’ like Woody the Cowboy and Hiccup like a Hippopotamus* or caw like a crow. And I’m thankful for those precious moments.
*Don’t understand, then you need to read the book Hiccupotamus by Aaron Zenz.
November 2017
The Family Fall Festival – which in simple terms is Hallowe’en!
Sunday, November 05, 2017
So how does a little boy who gets rather bashful about meeting new people handle Hallowe’en? Well, first he gets lots of practice driving through leaves. Then he dons the costume of his alter-ego. That is none other than “YEE-HAW!” – which is two year old talk for Woody the Cowboy from Toy Story. Then you go out to the Chilliwack Corn Maze with your church family to check out the animals and pumpkins. Alisha and Josh decided to avoid the actual corn maze simply because when they checked out the maze in early October they had great difficulty getting Emmett to leave and go home.
Then Emmett had a few trial runs in his Woody the Cowboy costume just to ensure that it would fit. And which hat he preferred to wear! This little man is very particular about his head wear and won’t allow just anything on his head. The cowboy hat, however, was barely allowed to come off. Of course, in a huge gust of wind the hat was lifted from his head and went flying. I went to grab it and almost got it but the wind caught it again. At that point Alisha directed me to go back to my position with Emmett while she captured the wayward cowboy hat. And the costume and character was whole once again.
Then it was trick or treating time on the big day. The first house was prepped for him and book (5 little pumpkins) plus a stuffed Hippopotamus just made his day (yes, we were waiting for this moment!). The second house was some neighbours who always see Emmett out for walks. Emmett has been thrilled to see their display of pumpkins and ghosts on our weekly walk and, upon ringing their bell (well, we rang it because Emmett couldn’t reach it) they popped out with an awesome helium balloon that was an awesome jack-o-lantern! (There were a few chocolates for Emmett’s grandpa, too!) Next was Emmett’s great-grandparents and grandparents. Emmett loves his great-grandparents place as there’s an elevator and long hallways that he can race his chair down!
Finally it was back to his own neighbourhood and Woody the Cowboy was now completely into the groove. Up close to the door, get mom or dad to ring the bell, and then politely indicate that of all of the goodies available that his preference was a shaker. Now, normally a kid doesn’t get to choose when they’re out trick or treating, but that seems to be one advantage of being Woody the Cowboy (or maybe it’s being a cute little kid wearing a Woody the Cowboy costume while in a wheelchair). You get a bit of preferential treatment while trick or treating. So Emmett got a ton of shakers! (Not really a ton, because he didn’t even make it to a dozen houses). And at one house he got an awesome glow stick.
There was a sad moment when he dropped a shaker and then ran over it with his chair. As all of us have had our toes run over, we can tell you that the shaker didn’t stand a chance. Emmett was quite distraught about that – until the next house and his shaker supply was replenished. So that’s it until next Hallowe’en – oops, until next year’s fall Festival. Are you wondering what a shaker is? Well, if you’re a kid who cannot eat anything at all then you’re creative – and SMARTIES are awesome Shakers! But is this really it until next year … not when there are post Hallowe’en sales! To Infinity and Beyond!
Meanwhile, Emmett is busy helping out around the house. Need a garbage can to fill up with leaves? Emmett will get it for you! Does it have leaves in it already? Not a problem, Emmett will dump them out and spread them across the yard. Have a pile of leaves in the middle of your yard? Not for long if Emmett is around – he’ll plow right through them and spread them everywhere. Such a happy little helper!
Of course, when it’s over and time to stop the fun, then Emmett has a few thoughts. Fortunately, he is pretty good at going with the flow and moving on to the next task! Even though at the time he may not appreciate the change.
Are you trying to remember last Hallowe’en? Here’s a glimpse back at 2016.
Visiting the New Wing of BC Children’s Hospital
Friday, November 10, 2017
I love this photo that Alisha captured on her iPhone. The joy and excitement in Emmett’s expression. The way that he’s leaning forward. How his hand is gripping the joystick and urging the powerchair to go even faster. Clutching his stuffed hippo in the other hand as he drags it along on his journey. His costume – which I truly believe transports him to the Wild West where he is Woody the Cowboy on an adventure.
Every day in Emmett’s life is an amazing adventure. Through this blog we have been sharing some of the heart highs and some of the lows. Emmett’s love of life shines through everything. God has truly blessed us with this little boy. On the other hand, you already know that I do not view SMA Type 1 as a blessing.
On the ninth Emmett was 2 years and 8 months old. Right now he is at BC Children’s hospital for assessments, tests, and then his lumbar puncture and injection of Spinraza, the super cool drug that is changing the way little ones with Spinal Muscular Atrophy live their lives. This will also be the first time that Emmett, Alisha, and Joshua have been able to see and appreciate the newly built wing – the Teck Acute Care Centre. This facility is, from all reports, simply above and beyond in what it offers to the little ones and their parents. We were amazed with the care and support at the old facilities, but this new building has windows, facilities to allow parents to be at their child’s bedside in some comfort, and has bright, airy hallways. Simply wow!
Remember Emmett today and tomorrow. There will be all kinds of tests and assessments. Some are fun. Others are not. The lumbar puncture absolutely not. Yet Emmett looks forward to these visits. It makes me wonder if this little one remembers that, at six month of age before these lumbar punctures happened, he could barely move. What do little ones remember!
Praying for you, Emmett.
December 2017
I Sneeze Into My Hand! This is why I don’t sneeze into my elbow!
Saturday, December 09, 2017
And cough too! If I have a tissue that I sneeze into that – obviously that’s much preferred. Then I wash and disinfect often. This weekend Emmett is off limits to me. I have a cough. I cannot see him. We can talk on the phone. Maybe a video call. Just not in person. And all of us who care for Emmett sneeze/cough into our hands. You’re probably thinking how uncivilized. Haven’t we seen the videos and public health presentations about sneezing into your sleeve, the nook of your elbow, to reduce the spread of germs? And of course we have and we all taught ourselves to do that. But think about it – the nook of your elbow is EXACTLY where Emmett’s head goes. Every time you pick him up! Every time. So we retrained ourselves back to sneezing into our hands. And then washing or using sanitizer. If that makes us uncivilized, then so be it.
Right now I’m teaching a group of grade two kids so there are a lot of coughs and germs rampant in the classroom. This teaching position sort of dropped into my lap and when it ends on December 22 then I will have more free time. Hopefully less coughing too. Because right now I do have a cold just like many of the seven year olds in this classroom. The teacher that I’m filling in for set up a washroom system whereby the child who heads out to the washroom puts either the girls’ or the boys’ sanitizer dispenser on their desk. That way you know who’s at the washroom plus the kids know if the dispenser is on a desk, then they cannot go to the washroom. (Unless it’s an emergency – please run out fast if it’s an emergency.) And they’ve been taught to use it, in addition to washing their hands in the washroom. Some of the boys caught me using the girls’ sanitizer dispenser on my hands yesterday. They laughed at me and I told them sanitizer is all the same. Of course, the dispenser is not – and I prefer using the girls’ dispenser over the boys. Just because.
When I have a cold, not only do I miss seeing Emmett but I don’t see Alisha or Josh. Were I to give my cold to them, then Emmett’s main care givers have a serious challenge. They have to stay healthy. So I stay away, because I have a cold. Even when I didn’t have a cold – just a few days ago – I did not go to see Emmett until I’d showered and changed clothes. And I would often wear a medical mask. You see, we’re germophobic. At school where I’m working (and my entire career has been working in schools) it is not reasonable to expect that a child will not arrive without a cold – schools seem to be a hot bed for germs during some times of the year, thus we need to be germophobic.
In spite of that, Emmett has a cold. Not a hospital stay cold, but a cold that causes him to have more secretions than normal that then require more suctioning than normal and then more bi-pap time than normal. If it wasn’t so worrisome, it would have been extremely cute when Emmett’s voice went hoarse earlier this week. He actually was laughing at himself, because he thought his voice was hilarious. We had a nervous laugh – worried that this cute voice might result in something scary. Emmett has his voice back but this cold is still there. It’s not bad. He’s fighting the bug. And that in itself is amazing. Before this amazing drug called Spinraza, this little cold would have been much more challenging for Emmett and most likely would have meant a stay in the ICU. Even now, it is scary. His little lungs simply don’t have the strength to handle a bad cold.
If you have a bad cold or some other potentially infectious ailment, steer clear of Emmett and his family. You’re a risk that they cannot afford. In fact, steer clear of all medically fragile little ones. While you’re at it, steer clear of medically fragile large ones, senior citizens, and … well … steer clear of everyone. I know, it’s not always possible to steer clear of everyone. I was coughing at school yesterday. Into my hands. And washing them often and using sanitizer. So often, in fact, that the boys caught me using the girl’s sanitizer container!
I sneeze into my hand – because Emmett’s head is going to be cradled in the nook of my elbow.
This message has been approved by Emmett!
In March I Will Be Three
Sunday, December 10, 2017
If you didn’t realize this already, I think that Emmett is extremely adorable. And smart. And cuddly! This past November in the beautiful Fraser Valley of British Columbia it has rained and rained and rained. Emmett’s outside time has been severely curtailed. Yes, there is a giant poncho to cover up Emmett and his power chair but it is still not as great as awesome sunshine. The result – more reading time. “Book! Book! Book!” is Emmett’s favourite phrase. And he got lots of reading time this past month. And reading time means cuddle time!
His speech is getting more and more recognizable. And like other toddlers he picks up on the most adorable things – or things that parents absolutely wish hadn’t been heard. Recently we were all getting ready to go outside but I couldn’t find his hat (it was packed in a container already in the van) so I put one of mine on him. Then perhaps I commented about him having a ‘fat head’. It’s quite possible someone else said that as it was a long time ago and I’m having trouble recalling the facts. At any rate, Emmett said ‘Fat Head’. Someone laughed. I’m pretty sure it wasn’t me. But that started it. The chant was even more pronounced than “Book. Book. Book.” Except it was “Fat Head! Fat Head! Fat Head!” over and over again. He would finally stop but then if he saw me the chant would start up again. Sigh! I’m working hard to ensure that I don’t teach him any more unique phrases.
Yesterday Alisha and Emmett surprised me with a question and answer session.
Alisah, “Emmett, how old will you be in March?”
Emmett held up three fingers and exclaimed, “Three!”
“How old are you now was followed with a two and two fingers held up. We are so excited and happy for the speech and the finger control that Emmett has. We pray for more and more!
Emmett is also thrilled that it is Christmas time. His fascination with the Baby Jesus, in Nativity scenes, has carried over from previous Christmas seasons. However, we have to tell you that it is minor when compared to his desire to see “Ho!Ho!Ho!” who, according to Emmett, is visible every evening in the night sky. Emmett was supposed to have been on an excursion with Canuck Place in which Santa Claus was going to be a prime feature – but the cold that he has put a stop to that outing. Emmett loves his Christmas tree and checking out the ornaments. Wendell the cat also likes the Christmas tree and, believe it or not, also likes checking out the ornaments.
Emmett does love his power chair! The speed! The freedom! The flexibility! That is, until he does something like get stuck or jam a wheel. Then it is a catastrophe of the largest order. Even worse, a time out because he ran into someone. That qualifies as an instant time out in which his joystick is deactivated and he has to sit and contemplate his actions. My goodness, the alligator tears are huge when this happens. Fortunately, his control is quite exceptional and he doesn’t usually run into people or things. But when he does, it is quite possible that it was intentional! Of course, there are accidents like yesterday, when he was having fun outside and he accidentally rammed a concrete step. This jammed a wheel and brought his fun to a stop. With a bit of help the wheel was freed up and Emmett was rolling again, chasing a ball around, and then showing some neighbours his new headlight and flashing tail lights. He’s visible when he’s out for late afternoon strolls!
On the ninth Emmett was two years and nine months old. The milestones continue!
It Was Christmas!
Monday, December 25, 2017
Emmett has been so excited about Christmas for weeks. At night while outside he would look up into the sky and exclaim, “Ho! Ho! Ho!”. Inside he would want to have parts of the Nativity scene – to hold and play with, specifically Mary, Joseph, and the baby Jesus. So when he had the chance to meet Santa face to face, well, he did what most every other two year old would do! Cried and tried to get away! Ha Ha Ha! Even though Santa was his dad … and even though Josh had put on the Santa suit right in front of Emmett … it was pure childhood terror! Honestly, there must be more adults with Clausophobia (it’s real, look it up on Google if you don’t believe me!) than with Coulrophobia. Fortunately, it’s only if Santa is up close that Emmett has a negative reaction.
Of course, if Santa is at a distance or a small toy figure then Emmett is all over it! Emmett was right into the Christmas spirit. Helping his dad pass out presents! Telling his mom what was in the presents that she was getting! And he was right, too! Josh handed Alisha one of her presents and Emmett piped up, “Tea Pot!”. Then of course there was present time – Emmett loved presents. He would sit and point at the present that he wanted to open next. His expressions were fantastic – especially when the present was clothes! After the first sweater, some disappointment was evident until prompted by mom or dad to express joy and happiness.
This was also a white Christmas in Abbotsford, so Emmett got to head out on his new sled. He handled it incredibly well while it was stationary. Once it started moving, however, he simply couldn’t keep his head held upright. He was quite fine and not injured at all – except for his feelings as everyone who witnessed it live or video couldn’t help but laugh.
The conundrum of dealing with a medically fragile child. For example, little ones often grab at and sometimes can break necklaces, glasses, and other items often worn by an adult. Of course, with Emmett that has not been an issue. He either couldn’t reach or, even if he did, didn’t have the strength to do anything. Well, the other night he grabbed my glasses and hauled them over to his face. That was so awesome and I was so proud of him. In fact, I looked through his handprints on my lens all night long rather than pulling a tissue out of my pocket and cleaning them. Then as we were getting him ready for bed, he started grabbing diapers and tossing them onto the floor. Perhaps his mom and dad would have stopped him, but he was having so much fun and I loved watching him grab a diaper with his left hand, pass it to his right hand, and then toss it onto the floor! So I let him continue to do it. (And since his grandma picked them up, I didn’t even have to clean up the mess that the two of us made!) This grabbing certainly came in handy with Christmas presents! In the video you’ll note that Emmett does a pretty good job of grabbing onto tissue paper and pulling it out of the way. What really made us laugh was his impatience when we were chatting and not passing him presents. You’ll hear a very distinct, “Hey, Daddy!” coming out of this little one. His personality is so charming! There may be a little bit of spoiling going on, but that is something that Grandparents are supposed to do.
From here on it is simply pictures! Merry Christmas from Emmett, his mom (Alisha) and dad (Josh), and his grandparents Rhonda and Roger Willms and Frank and Pamela Ewald. Have a Happy New Year too!
Canadian Decision Shocks SMA Community
Monday, December 25, 2017
The Canadian Agency for Drugs and Technologies in Health just published a report that recommends only allowing the miracle drug Spinraza to be made available to a very small subset of Canadians who have Spinal Muscular Atrophy. As you can imagine, this is a heart wrenching decision that has filled many Canadian parents and individuals with SMA with fear and sadness. CADTH doesn’t make the decision, that’s up to each province, but the provinces widely listen to and often accept CADTH’s recommendations.
We literally do not know what to say.
If you have been following Emmett’s Journey, then you know that he was one of 124 infants worldwide who were part of a blind study and that he joined this study in the fall of 2015. In the care of professionals at BC Children’s Hospital, we quickly started to see improvements. This was a blind study, but when improvements begin happening in an 8 month old who had lost all movement in his legs, had minimal arm movement, with very limited movement his head, and was being fed by G-Tube, then you quickly notice. Spinal Muscular Atrophy is a degenerative disease. There may be plateaus but evidence demonstrating improvement is simply not there. We saw and still see improvement so we know that this drug, Spinraza, works.
Emmett’s parents were concerned about him participating in a medical study. I’ve shared before that as grandparents we thought it was a crazy idea. I’ve also shared that we were wrong and that we’re so glad that Alisha and Josh agreed to have Emmett participate. He was one of four kids at BC Children’s Hospital who were part of the trial for this drug that came to be known as Spinraza. The three other children, sadly, did not survive. You see, Spinal Muscular Atrophy is a genetic disease that takes the life of more babies than any other genetic disease. It literally shuts down their respiratory system. This is after it has shut down their ability to move their limbs. It is a terrifying disease.
That is why we are terrified by this decision. The drug is already horrendously expensive and that is obviously a challenge to families dealing with SMA. Yet this drug, Spinraza, is the absolute first treatment option available to individuals with SMA. Emmett has SMA Type 1. Some little ones are identified even younger than Emmett – who was officially diagnosed at five months. Some have Type 2 which is a bit later onset. Then there’s Type 3 and Type 4. This decision, if the provinces act upon it, bars Type 2, Type 3, Type 4 and even a large number of Type 1 kids (and adults) with SMA from having access to this drug.
Now, because Emmett was and is a part of this study he probably fits all of the criteria that CADTH has set out for access to this drug. That, however, is not the point. Obviously, we want Emmett to improve and that is not in question. But this drug works for all SMA types, so why is it being limited? It is life changing for anyone with SMA and their families. Emmett was not in this trial for himself – he was in it for everyone. Think about it, when Emmett got involved in this it was an unproven drug. No one knew what would happen – although there were indications and a lot of hope that it was positive. But Emmett’s parents took a leap of faith – hoping beyond hope for a miracle for their son.
Emmett unknowingly worked very hard to get this drug approved. He has benefitted from this drug, Spinraza, which I absolutely call a miracle drug. That it was approved in the USA, Canada, and several other countries is a miracle. It is available now in the U.S.A. for all types of SMA. Now we need another miracle – a Canadian miracle. We need the experts at CADTH that look at drugs to realize that they need to expand their point of view and recommend full access to this drug. The decision they have made is based upon scientific fact but to the narrowest of definitions. As a result there will be more Canadian babies who die of Spinal Muscular Atrophy. Children and parents who have been waiting years for a treatment will be denied access – their hopes dashed.
To those who question the expense of this drug, please realize that I question the expense too. It’s literally beyond belief. Then again, creating new pathways for the brain to be able to speak with muscles that control lungs and muscles is absolutely beyond belief.
Please watch this blog – we hope to be able to share ways in which you can be an advocate for wider access to this drug here in Canada.
Links:
https://www.cadth.ca/nusinersen – nusinersen is one of the early names of the drug Spinraza