February 2020
February 6, 2020
ICE CREAM FACE!
Is there anything better than a four year old kid (almost five, mind you!) with their face covered with ice cream? Well, yes, there honestly is. It’s a four year old whose face is covered with ice cream that we never thought would get to taste, let alone eat, ice cream! Not to mention use his own arms and hands to get the spoon of ice cream from the bowl to his face … and then into his mouth! In fact, Pam had to go searching for kids’ spoons as all we had in the kitchen were adult sized utensils. What can we say about this miracle drug called Spinraza except that it works! It has never been touted as a cure. It is not a cure. But not only does it stop the disease, in Emmett’s case it has absolutely been rejuvenating the spinal pathways carrying messages from his brain to his muscles. When Emmett started the blind drug trial over four years ago, no one would say for sure what the outcome would be. Certainly the hope was that it would slow down if not stop the disease. Look at Emmett. We have seen babies that have received the same diagnosis but started Sprinraza pre-symptomatically develop seemingly challenge free. With Emmett the disease, SMA Type 1, has been stopped from encroaching upon his capabilities and the REVERSAL of some of the effects of the disease. It is not a cure, but it is a miracle drug. If only British Columbia would make this drug – and yes, it is very expensive – more accessible to the children and young adults whose lives would be changed. Now back to ice cream!
Emmett is still very much fed by a tube that goes directly into his stomach. But for the last six or so months he has been taking small sips of water – and when we say small that is very much what we mean. One milliLitre of water. You see, since he was six months old he has been tube fed as he was not swallowing properly. The risk of him aspirating on food and even his own saliva has been huge. When he was about 6 months old, after a swallow study showed food not being swallowed properly, Emmett was weaned from breast feeding and immediately fed by tube. For over four years he has had no food or liquid by mouth, until recently. His medical team has given the go ahead so he, supervised by Alisha and Josh, is experimenting with food by mouth. And it is both fun and exciting and somewhat nerve wracking!
What you cannot appreciate is the joy that Emmett is experiencing. Photos simply cannot express his emotions. You see, while Emmett has to the best of his memory never eaten by mouth, he loves food. At meal times he is always at the table and part of the action. He has play food and dishes and utensils. He has Melissa and Doug – now I am not being paid to say this but Melissa and Doug stuff is awesome! – pizza set, lettuce set, pots and pans, and more things than I can remember. Suffice it to say, this little boy loves food. And not only playing with it. He has always enjoyed the aroma of what those around him are eating but, until recently, wasn’t even interested in tasting it. That has changed now and, while he loves ice cream, he thinks that everything from apples to hot dogs to licorice to pickles taste great. And ice cream! Well, need we say more. *Note: the food given to Emmett is in minute amounts and pureed. When he tastes something, it is large with no risk of him swallowing it, plus he knows very well to taste and then it’s back out of his mouth. The only thing he swallows is either liquid or pureed.
Emmett loves to play and he can focus upon an activity far longer than any of the adults who are playing with him. With Emmett it is very much directed play, as in he will direct his play partner on what they need to do as part of the play. If he is playing with small toys on a table or tray or on the floor, then he can amuse himself for lengthy periods of time. Of course, if he knows that someone is nearby then he quickly will envelope you into his play. And direct you. By direct I don’t mean just telling you which toys to get from the toy box to play with. He will tell you how to play with the toys. For example, recently Emmett and I went on a walk/roll with many of his favourite stuffies being pulled along in a wagon behind his power chair. Winnie the Pooh was along on this journey (Winnie has been a mainstay of our treks for over a year) and, as to be expected, Winnie the Pooh was hungry and was searching for a honey tree. In his local park (Emmett refers to it as Emmett’s Park). Emmett/Pooh found the tree with honey and then I carried Pooh to the tree and put him up as high as possible. Then behind me Emmett started making a humming/buzzing sound and I wondered if something was wrong with his breathing. I was only a few steps away but I quickly turned and headed back, only for Emmett to exhort me to get back to the tree.Â
“Grandpa, it’s the bees! The bees are coming!”
Grabbing Pooh, I now understood that the buzzing sound coming from Emmett was actually Emmett making the sound of buzzing bees, and with that knowledge we quickly left that area of the park. We did not want to get stung, after all. The bees did come after Pooh and us several more times but we managed to evade getting stung! Probably because it is February and there are not many bees out. They were imaginary.
Another area of play that we are experimenting with is remote controlled toys. For Christmas he got a remote controlled excavator. It has a lot of buttons but Emmett is starting to figure out how to use this toy. Don’t tell him, but for his birthday on March 9 Emmett is going to get a remote controlled dump truck to go with the excavator. This way he can control his own play with toys that are otherwise too big and challenging for him to play with otherwise. Yet every little boy should have an excavator and a dump truck that they can play with. This move to remote controlled toys is an experiment that is worth taking and hopefully he has fun. Of course, while Emmett has stopped marking up the walls with his power chair, this new venture with somewhat large and powerful remote control toys has already left some scars on the drywall. Oops! Sorry Alisha and Josh!
Alisha, Josh, and Emmett are really excited about some upcoming changes. After a lot of talk, planning, and saving they are getting ready to demolish their bathroom and create a more accessible bathroom for Emmett. The floor space cannot change (without drastically impacting the house floor plan) but the layout of the room can be significantly enhanced. First up, the doorway is going to be enlarged so that a wheelchair can enter without difficulty. A roll-in shower is going to be built which will allow Emmett, while in a shower chair (a non-powered chair able to get wet made by Rifton which they already have), to be rolled in without any issues. The room will be designed as a wet room so that no water damage can result. A toilet with a higher seat, similar to what many seniors have in their bathroom, will be put in. A deep soaker tub will be installed where the current tub is – Emmett still does a lot of physio in the tub. Of course, there is going to be a sink also – the aim is for a wheelchair accessible sink that will have adjustable height levels.
The next change is that after going through two loaner power chairs, Emmett is going to be getting his own power chair. The biggest challenge of this is that, like the bathroom, it is expensive. And before you ask, yes the basic power chair is covered under provincial plans. But nothing optional is. You’re probably wondering why Emmett needs options; why can’t we/he be satisfied with the basic chair. Here is one example that will cost in the thousands of dollars extra. That is a seat that raises and lowers – so that Emmett can participate fully with activities that occur around him, such as joining people at the dinner table at an appropriate height rather than having his eye level below the table top. An option like this is not included in the provincial coverage.
Emmett is excited to announce that he is registered for kindergarten next year (See The Pigeon Has to Go to School). On family outings he often goes for a roll/walk to the local neighbourhood school that he will be attending. Everyone is very excited about this. Everyone is nervous about this. Meetings with the school staff have been very encouraging. We are also very excited and happy that the City of Abbotsford is going to be installing accessible sidewalks on the corners between Emmett’s house and the school. Emmett currently has to go to private driveways to safely access the road to cross the street when he’s heading to the school or the local park, so this is a very positive change that will benefit the entire neighbourhood. In the pic just above, Emmett the vandal is trying to push over the stop sign that is currently right in the middle of the sidewalk. We’re positive that this if going to be relocated also. Emmett loves the swing at his new school – although he still has a bit of growing to do.
While Spinal Muscular Atrophy is a rare genetic disease, every individual dealing with it is amazing. One of these is individuals is Kevan who has SMA Type 2. About four years ago, with a group of his friends, Kevan spent three weeks backpacking across Europe. Literally. His friends carried Kevan in a backpack he/his family/they created. The WeCarryKevan backpack is now a product that can be purchased commercially. Emmett is now the proud owner of a WeCarryKevan backpack. We are all looking forward to some amazing journeys into areas that a wheelchair simply cannot access. Stay tuned for more adventures!
Thank you all for your support, your caring, and your prayers. A huge thank you to everyone who donated to the bathroom fundraising campaign in the fall. You’ll be able to see the finished product soon. And if you would like to make a financial contribution to this project, please visit the How You Can Help page for information on making a gift. Please be aware that as this gift is for an individual there are no tax receipts available.
Thank you
February 8, 2020
Emmett’s Reading Corner
Today’s blog post is simply a video. Emmett would like to share with you Mo Willems’ book “The Pigeon Has to Go to School”.
March 2020
March 1, 2020
February Was Busy!
February was an awesome month. Not only is it Alisha’s birthday month but also because Alisha, Josh, and Emmett went exploring with their new “WeCarryKevan” backpack that we mentioned in the last post. The great thing about this WCK backpack is that it is comfortable for Emmett and they can go explore areas that otherwise would be impossible for Emmett to access. All it takes is a few huge roots or rocks and a wheelchair simply cannot traverse that terrain. Sure, it’s possible to just pick up and carry him – but try carrying a heavy weight on a hike. It’s not much fun and that is why backpacks were invented. And the WCK backpack is specifically designed for this purpose. Of course, that doesn’t mean that it isn’t a workout for both dad and son! Mom too – as she was carrying the suction machine, food pump, and other assorted necessities. The short hike that they went on turned out to be quite a gruelling ordeal. Lots of fun but also an awful lot harder than they anticipated. Hiking poles are a near future expenditure.
Meeting Brock Boeser at a skating event hosted by Canuck Place was an awesome experience (for Josh). Brock is a 23 year old Vancouver Canucks’ player (yes, there is a close rapport between the Vancouver Canucks and Canuck Place) who, as a rookie with the Canucks, scored 25 goals in his first 44 NHL games. Eventually Emmett will find that interesting, but at this age Emmett (and Alisha) were far more interested in meeting Fin, the Vancouver Canucks’ mascot. This meeting, however, was like a typical first meeting with Santa Claus. Fin was just too close and startled Emmett! As a mascot at hockey games, Fin does an amazing job of inspiring the crowd with his antics, speed, and dexterity. For a little kid with SMA, Fin skidding to an abrupt stop right in front of him with a spray of ice and snow was just a bit much for Emmett. He preferred Fin at a distance. Or behind him! LOL!
Thank you so much to Canuck Place and to those who support Canuck Place. Events like this are fantastic for the entire family (well, maybe not grandpa who wasn’t invited) and provide a rare opportunity for this little boy who has a rare disease. Canuck Place also provides opportunities for overnight respite and support and so much more. It is an invaluable community support. BTW, did you know that the last day of February is Rare Disease Day. 72% of the rare diseases in the world are genetic. 70% of those genetic rare diseases start in childhood. Spinal Muscular Atrophy is a rare disease. Emmett has SMA Type 1.
You’ll remember how Emmett was trying to push over the stop sign that was in the middle of his sidewalk. And yes, it is his sidewalk as he travels to ‘his park’ that is just down the street. In addition to the stop sign the access to the street was not accessible at all. The City of Abbotsford was out in full force recently, making an amazing spectacle for a little boy to watch. Two dump trucks, two excavators, a small truck and lots of workmen made for an exciting morning. We have to tell you, there was only one city excavator present. the second excavator was Emmett’s! He was supervising and lending a hand with his equipment as needed. This is such a welcome upgrade to Emmett’s neighbourhood. And while he will miss getting to try to push over the stop sign, it will be so much nicer for daily travel.
While observing the city excavator at work, Emmett noted that his excavator was different. That his did not have a shovel on the end. This is quite accurate, as his excavator has grappling claws that are fantastic for picking things up. They are not good for digging up sidewalks. He had forgotten (the Christmas morning bustle makes it easy to forget things) that his excavator did indeed come with a shovel. This meant that tools had to come out and he went to work removing the claw and installing the shovel. Now he is ready to go out and dig up mom’s gardens.
Emmett will be going in on March 5th for his next lumbar puncture. His 17th one. Please remember to keep him in your prayers on that day. You can certainly appreciate that he does not look forward to this event and it does cause him anxiety. He is one smart cookie and he knows the procedure room where it occurs – he calls it the ‘hurt room’. And he knows the doctor too, he gave him the nickname of the ‘hurt doctor’. Obviously everyone tries to minimize this anxiety and the discomfort. We’re hoping to hear that this procedure goes incredibly smoothly.
One of our answers to prayer is that Emmett has had a very healthy winter. Alisha and Josh go to great lengths to keep this little one happy and healthy. That involves sanitizer. If you know Alisha, then you know that their house is cleaner than most hospitals. Guests must use hand sanitizer. Have a cold, then realize that you’re not welcome in the vicinity of Emmett. If you have tried to purchase hand sanitizer recently, you can appreciate the stress that Alisha has been experiencing as the shelves are bare. Product is in short supply and if it does arrive at stores, it is gone before it has time to get comfortable on the shelves. We’ve seen higher than normal prices on Amazon and we’ve had online orders cancelled as the supplier knows that they cannot fill the order. So it was with great relief that we managed to get a case of 12 bottles south of the border to bring home. We are not sponsored by Purell, but hey, Purell, if you want to we’d be happy to accept your offer!
March. Lumbar Puncture on the 5th.
Family Birthday on March 9th. FIVE YEARS OLD. (oh yes, there will be a blog post shortly afterwards)
March 11th – bathroom renovations for an accessible bathroom begin. (Emmett, Alisha, and Josh will move in with Grandma and Grandpa for a while.)
March 14th – kids birthday party with some of Emmett’s friends. And family too.
Here are a few more pics. The WCK backpack comes with an awesome neck support to hold up heavy heads. As Alisha and Josh were just taking Emmett on a short hike they left it at home. Everyone underestimated the challenge of this short hike and all felt it, including Emmett who just couldn’t hold his heavy head up anymore! Sweet little kid!
March 10, 2020
5 Years Old: Strength in Community
Emmett is FIVE years old!
Birthdays are always exciting. Monday, March 9th was Emmett’s fifth birthday. But when your birthday also coincides with being the Junior Chief of Police at Abbotsford Police Department, then all of a sudden, your birthday is that much more exciting. Police Chief Serr welcomed Emmett to the department and there was an official swearing in ceremony. It was quite fabulous. Chief Serr talked about the department’s vision statement, which is Strength in Community. That is absolutely what the fabulous Abbotsford Police Department has demonstrated to Emmett, pardon me, Junior Chief Emmett. That our community is one of strength. In his five years Emmett has absolutely seen how the community has rallied to support him and his family. Chief Serr also talked about how Integrity, Honour, Courage, and Service are the APD’s values. And that courage is a keystone of the police officers and the police department – and then he commented on how Emmett’s courage in his five years surpasses theirs. Huge recognition for a very brave and courageous little boy. Thank you, Chief Serr. We certainly have seen first-hand how your amazing force demonstrates these values.
The entire Abbotsford Police Department made Chief Emmett’s day a great one, but there are a few that I’m going to mention by name (at the risk of forgetting someone important). Obviously, Chief Serr for making time in his day to visit with Emmett. Sgt. Joiner and Sgt. Bird for coordinating this celebration and ensuring that everything worked together. And especially Constable Kern. Emmett met Constable Kern at the Abbotsford Air Show in the summer of 2019. I apologize to the air show for saying this, but the Snow Birds and the aerobatics paled beside Constable Kern, his police cruiser, and the attention and care that Constable Kern shared with Emmett. There is no question that a bond was formed that day.
Abbotsford Police Department – thank you for this very memorable day. Pizza and ice cream in Chief Emmett’s new office, fantastic. There were a few things that Emmett wanted to sign into official business. They were something like this, a) every Wednesday was ‘Talk like a Pirate Day’, b) quiet time in the afternoon, c) pizza day once a week, and d) once a month his grandpa (and Emmett, of course) gets to race around in a police cruiser with the lights and sirens on (This last one may be his grandpa’s idea). And thank you for this coming Saturday. Blog readers – stay tuned for that!
March 17, 2020
Self-Isolation
What a week! There’s an internet meme floating about right now that says, “Anyone else feel like life it being written by a 4th grader right now? And there was this virus and everyone was scared. And then the world ran out of toilet paper. And then there was no school for like a month. And then it snowed!” As a retired teacher/principal – several years of 4th grade – I do think that this is what has happened!
Well, there’s no snow here in Abbotsford but we did have a significant wind storm with temperatures at or just below freezing. We even saw, if you can believe it, icicles. Today, however, the weather is awesome and that is Emmett’s coat casually tossed on the back of the power chair. The meme writer, and I cannot confirm the source so if you know please let me know and I’ll give credit, hit it right on. But we’re going to add to it. How about fit in we demolished our bathroom! Because last Wednesday the long-awaited accessible bathroom renovations began at Emmett’s house. So not only is there no toilet paper, but there’s no bathroom either! As a result, there was a planned move to grandma and grandpa’s house. And that is when the North American Covid 19 virus awareness began in full force. As a result, this giant sleepover at grandma’s house became a giant sleepover at grandma’s house.
That’s right. No change. You see, for Emmett every day is a virus scare. He literally hasn’t noticed anything different. He is five, of course, and that makes it easier than if he were fifteen. But Emmett’s daily life is one of social distancing and avoiding crowds. During this cold and flu season, Emmett was able to get out more than previous years but his parents still were very cautious about events he attended. Visits include direct investigation of potential sources of illness. Visitors – absolutely screened for illness. Wash hands and apply sanitizer upon arrival. You’ve read here before, if you sneeze Emmett will not say “Bless you” but he will say “Sanitize”! Self-Isolation doesn’t mean we cannot get outside – as we are absolutely taking advantage of the awesome weather – as you can see in some of these photos – to enjoy the outdoors and local forest trails. Isolation does mean that play dates and having anyone else in his immediate circle is simply not happening.
So Emmett hasn’t noticed the self-imposed isolation. We managed to slide past the missed birthday party (the rest of us missed the crowd of police cars and the friends and the BBQ) by playing with his Air Swimmer (named Shark-Kaaah; and don’t say it wrong or you will be corrected) and his birthday presents, which included a remote controlled dump truck to accompany his excavator Christmas Present (just wait until his Easter present – which is a new thing but it was a super Black Friday sale and I couldn’t resist). The Air Swimmer wasn’t a birthday present but a sleepover present. It is a great distraction and, even better, Emmett isn’t afraid of it. You may recall that 2 or 3 years ago he had another air swimmer – a clown fish. He loved that fish from a distance, but it was just too large and terrifying up close. At first there was hesitation but we built it together (and yes, you have to build an air swimmer and it takes a long time) and he loves it. We have to force Emmett to leave the fun and games inside to explore the outdoors: we get to go on walks now that the weather has warmed up and in the evening we play games like Zinga. A good round of Uno has to happen soon too.
Meanwhile, work continues on the bathroom. The floor joists have been strengthened and modified to accept the roll in shower. The plumbing and electrical rough in work has been completed. The flooring is going down today and the drywall is starting tomorrow. So right now this is moving ahead as planned. Hopefully it is a pace that can be continued. Josh – loaded up with face masks, sanitizer, and with full social distancing in effect – is interacting with the workers to ensure that any of their questions are answered. Even though Emmett isn’t there, the contractors have been asked to wash hands and sanitize on a regular basis throughout the day. Keeping a medically fragile youngster healthy is an everyday, long term project.
For us adults, this self-isolation and the Covid-19 virus has put us all into hyper-vigilant germ avoidance mode. Our hands are raw from hand washing and sanitizing. There’s no going out to any public venues – except to Emmett’s house in the evening to check on progress and grab more supplies. Good thing that there are some lovely walking areas right in our neighbourhood, like the forest walk pics you see above. Panic set in when some of Emmett’s supplies were not delivered on time. Hours were spent on the phone getting everything sorted out and a huge sigh of relief was heard from Alisha when she heard that the courier had dropped everything off. While Grandma Pam almost always has a great supply of stuff in her fridge and pantry (and don’t even get me started on toilet paper – it’s like Pam has been in training for this pandemic for decades. Now I’m not going to be able to tease her about it as it has certainly paid off!). Huge thanks to Grandma Rhonda and Grandpa Roger who are acting as our personal shoppers. Unfortunately, with this isolation they are not able to visit. Porch drop-offs and FaceTime right now. And while paper towels may not be the most environmentally friendly, they are a necessity to limit germ spread from hand towels.
So things haven’t changed and yet they have. It was just two weeks ago that Emmett was getting his lumbar puncture. The procedure didn’t go as efficiently as most previous ones. As a result, Emmett had a tender back for a few days afterwards. Part way through the specialists moved him from the procedure room to the OR so that they could put him to sleep. And Emmett is literally amazed that, in the middle of the day, he was awake and then he fell asleep and then he was awake. Even better, everything was done. Emmett told his mom and dad that he much preferred going to sleep to have the lumbar puncture – and that is what his medical team is going to do from now on! While this may not be far from Emmett’s regular normal, that the rest of the world is now moving towards our lifestyle is shocking. Going for walks and wanting everyone to remember social distancing. Normal is now abnormal, I guess.
Now for a typical day with Emmett while in self-imposed isolation. Alisha and Josh made the tough decision to cancel their night nursing support. This was a tough decision as some of their nurses have been with them for four years. However, the risk that they could bring a virus into contact with Emmett is just too great. As a result, Alisha and Josh are monitoring Emmett overnight. Night includes ensuring the bi-pap is on, functioning within proper specifications, and that the mask hasn’t been jarred out of position causing an air leak. Hi oximeter monitors his oxygen levels and his heart beat. The suction machine (which temporarily was malfunctioning but now seems to be working again; plus there is a back-up machine) is never far from Emmett. While he is practicing swallowing, this suction machine is still used daily; there are still some significant episodes where our hearts are given a workout. Then Grandparents took over in the morning while Alisha went to get an hour or two of uninterrupted sleep. Emmett woke up in a great mood and began the morning telling stories. He would tell one and then get us to tell as story. And this went back and forth for at least 45 minutes – the stories were hilarious. Next Emmett and I repaired the drawers in the bureau that is being used for his clothes. Emmett thought that the screws were very sharp. Then we went upstairs for breakfast. After breakfast, Emmett went into his standing frame and played fishing with Paddington cat. Of course, grandpa got a bunch of sharks and they surrounded Paddington Cat and eventually they tipped Paddington’s boat. Emmett switched to his manual wheel chair and then he played with his mommy. It was a really cool Owl present that he got from Felicity, Brendan, and Aiden. He loved creating an awesome owl, read an owl story, and played with little owls for the rest of the day. (The little owls are Billy, Percy, and Sarah.) He also watched the Cincinnati Zoo live presentation of the Brazilian Porcupine (yesterday he watched his favourite, Fiona the Hippo).
Then we had lunch and Emmett helped grandma by stirring batter for banana bread. And chocolate chip cookies. Emmett loved the aroma of the fresh baked cookies. And grandpa loved eating them. Then Emmett and mommy went outside and lay on a blanket in the sun in the back yard while reading stories. It was about 15 Celcius – so perfect outside weather.  Then Emmett had his quiet time. This is time for bi-pap and Emmett played with his Toy Story characters plus Billy, Percy, and Sarah (his new owls). Emmett hasn’t had afternoon sleeps for months – but quiet time is still an important aspect of his day (for his mommy’s sanity too!). After quiet time Emmett came out to help grandpa work on his race car. Yesterday we put on a rear wing – which Emmett thought was really funny. He wondered if it would make the car fly, but now realizes that it pushes the back of the car down. Today we put on the front splitter and air dam. This also pushes the car down – so you can go faster through corners. Emmett asked questions non-stop about everything. Literally. Then we went on a walk to grandma’s forest (which really isn’t grandma’s but we call it that anyhow). We walked to the bridge in the forest and then turned around and came back. Then Emmett helped me remember today’s events for the blog when he heard his daddy coming back from the bathroom project. He and his daddy talked all about what happened at the bathroom – including getting the new bathtub installed. Then it was just chatting time, stretching time, story time, and bed time. No games tonight – that will happen tomorrow.
And that is a brief overview of Emmett’s day in isolation.
March 29, 2020
21 Days and Counting
The past 21 days have been some of the most unique that North Americans have seen. Unfortunately, other parts of the world saw it so much earlier. So many businesses and venues have been closed down. So many people are sick. So many people are worried. It’s terrifying! This is the stuff that April Fools day jokes would have been made of before – except that today’s reality is too bizarre for anyone to even have suggested it.
As you sanitize, wash, and look over your shoulder at someone coughing, you are at the beginning of an understanding of our world. The world around a little one with a rare disease. Now you understand why we moved away when you coughed or obviously had a cold. Now you know why we left early if we were in a crowded room and people were sniffling/blowing their noses/coughing. Now you understand why we constantly used hand sanitizer – even though it offended you when we applied it shortly after shaking your hands. Now you understand why from October to April you rarely ever saw Emmett outside of his home.
Now you understand why this is 21 days and counting that we’ve been in self-isolation. Why Alisha and Josh cancelled their nursing support. Why if we meet you outside while on a walk, we quickly back away if you move into our space. Two metres? We prefer three or four, thank you. You’re reading stories about people removing their clothes, bagging them, and showering before interacting with their families. While we didn’t bag our clothes, they were absolutely removed, we showered, and changed into fresh clothes before interacting with Emmett after being out in public places. If you thought we were crazy about hand washing and sanitizing before, you should see us now! Plus we’ve shared before how, due to carrying Emmett all of the time, we stopped the practice of coughing into our elbow and only cough into our hands; you cannot clean your elbow and that is exactly where Emmett’s head would be.
It wasn’t that we did not want to interact or be sociable, especially Emmett, who loves interacting with others. But like you’re probably doing right now with this Covid-19 virus, we were and are protecting those we love. Surfaces, light switches, door handles are disinfected daily – that’s normal routine every day around Emmett. What is abnormal is that we literally are not going out – apart from neighbourhood walks on very broad pathways. (The forest path in the last blog, too narrow. It’s not happening any more!) We are having Roger, Rhonda, Ben, and Alesha do our shopping – that’s not normal. Not going to Canadian Tire. Oh my, that’s so abnormal. My Canadian Tire fix has to come from online searches as I cannot go to the store. In fact, I cannot even look at the newspaper flyer as it’s not allowed in the house until it’s been sitting for days. And for Emmett, the prospects of entry to kindergarten this fall – with health experts indicating that there will be another Covid-19 wave happening then – have changed from excited anticipation to seriously considering planning out a home schooling program. And after beating us two rounds straight, we’ve decided we don’t really want to play Uno with Emmett! He really is good at this game.
Shark-Kaaah is still floating. There is a tiny amount of ballast still in the ballast hold, but after 22 days of floating about we really do not anticipate that Shark-Kaaah will last the week. And we cannot risk getting a helium refill at this time, so time is limited for this galeocerdo. This is humourous, obviously, yet we are quite aware of the serious challenges that are facing families right now. Our family, just like yours, is in the midst of challenges that we can do little about. Emmett’s Great-Grandpa Willms has just had a fall and a broken hip – and family cannot even go and see him. The hip has been replaced, but for a man closing in on 90 there are most certainly complications. Great Grandma Willms is facing lung cancer issues – again on her own, without family to support her, and not even able to see and support her husband. Kilometres away in Ontario Emmett’s Great Grandpa Ewald has had a minor heart attack (if any heart attack is minor when you’re in your 90th year) that coincided with the first of two falls – the first garnering a broken collarbone. This led to a bump up the “needs ladder” and admission into a nursing home. Great Grandma Ewald is now separated from him, still in her assisted living room where she is as comfortable as possible. The challenges of an extended family – exacerbated by seniors’ facilities having shutdowns so that family cannot visit – these are being faced by all of us throughout the world.
The world is full of daily challenges as everyone tries to adapt to and deal with Covid-19. So what does our day entail?
We wake up and go and do some baking. In our PJ’s, of course. Why put clothes on! These sour dough glazed doughnuts were to die for! And the Raisin Carrot muffins we made today – so much healthier! And still really good. Emmett likes the texture of flour; that is feels so soft! We purposely put flour on our faces to show to everyone how hard we bakers were working!
And then we do some gardening – like planting a mock orange bush in grandma’s garden. This was a lot of fun as Emmett asked all kinds of questions – especially when we dug up some construction refuse from years ago. Then you can see and hear that Emmett is thrilled to participate in home videos with his family:
Then we have some physical education activities like soccer:
It is possible that Emmett could be an Elasmobranchologist when he grows up – but right now he’s just a boy with a shark! With a robot voice.
It is possible that Emmett’s home school teacher will have to pull up her socks and be more enthusiastic in upcoming lessons – have to keep this little one inspired! But, as you can see, there are days when we change out of PJ’s and into real clothes.
And while current affairs are more bizarre than any movie, Emmett had a lot of fun with April Fool’s Day jokes. Telling us that spiders were on our heads, ants were in our pants, and that sharks were coming to eat us. My personal favourite – beating on a steel bowl with a wooden spoon while telling grandma that it was time to get up! LOL! I wonder who suggested that to this impressionable little boy! Then he proclaimed “April Fools!” and that it wasn’t really her alarm clock! Emmett was quickly forgiven. Me, not so fast.
What will day 22 and following bring? Well, more progress on the bathroom renovations. The next blog post will have some updates on the progress that is happening there! Potentially more antics as we try to keep each other sane and occupied while we’re isolated. There are articles floating about on social media that indicated that many of the deaths that are attributed to Covid-19 have underlying causes thus, the articles imply, Covid-19 is not so dangerous. This type of article absolutely angers me! That is the point! Covid-19 attacks those who are at risk and they for so many reasons they cannot overcome this disease. Emmett’s lungs are so much stronger than they were just a few years ago, but this virus absolutely terrifies us. We have seen Emmett on a ventilator, with deep suctioning happening to clear his lungs and airways. We never want to see it again. We never want to see someone else, healthy or frail, having to undergo those procedures just because someone else was thoughtless and carried the virus to them. So practice social distancing. If you see someone in a wheelchair or other mobility device, GIVE THEM ROOM! That device is not a mobile as you are – it cannot go down the curb to get away. Thank you for being considerate of others. Wash your hands. And stay at home. We so appreciate everyone’s support. Thank you for thinking about and praying for our family – and we’re doing the same for our family and friends. Stay Home. Keep everyone safe!
April 29, 2020
7 Weeks!
When the bathroom renovation project started, no one realized that a pandemic was going to affect the entire world. Here we are now in the middle of May and the bathroom (except for minor things like towel racks and a mirror) was finished almost three weeks ago. This has been a long time coming and Emmett is very proud of his newly renovated bathroom. As mentioned already, this was making the best use of the existing space and ensuring that it is accessible. Entering the door is now much easier as there is a new pocket door that is much wider than the previous door – plus it tucks out of the way when it is open which is fantastic. The main bathroom light is motion activated so goes on as soon as one enters the room; plus the switches are low so that they can be easily accessed from a wheel chair. The tiled floor leads right to the roll in shower at the end of the room. The floor is level until you get to the shower and then it slopes to the drain. A ceiling heat lamp there and one near the bathtub ensure that everyone will stay warm when they get out of the water. The new, deep tub is available for baths and for physio sessions – as Emmett still loves playing in the tub. The biggest change in plans, maybe even the only significant change, is that the sink is fixed and cannot be raised/lowered. The sink is fixed at at height that Emmett can access in his manual wheelchair and his KidsWalk standing walker. It has an automatic faucet so that Emmett can now wash his own hands.
Thank you. Literally, so much thanks to all of you for your support and donations to this project. We also thank CKNW Kids’ Fund and Muscular Dystrophy Canada for their generous financial donations to this project. Also, huge thanks to Micah Hooge, the project manager from Highplace Homes, and his incredible team who brought this accessible bathroom dream to fruition.
As a result, after living with Grandma and Grandpa for seven weeks, Emmett is back home and loving it. Seeing toys that had been left at home and untouched for nearly two months. As you can imagine, it was better than Christmas. Of course, it also coincided with some amazing weather. That has resulted in bike rides, hill rolling, and long walks/rolls through the neighbourhood. The heat wave didn’t last and now there is more seasonal weather, but it is still awesome. During the heatwave temperatures were 29°Celcius. That meant it was time to hit the sprinklers!
Like many of you, Emmett has had many meetings using video conferencing. And we are not talking about family – meetings with his occupational therapist, his physiotherapist, the teacher at Canuck Place … this boy is busy. In fact, his Aunt Katie has even been sending him some kindergarten preparation work to give him a head start on school for next fall. Of course, we don’t know what that is going to look like! Even more good news, the City of Abbotsford has returned to the corner near their house, where Emmett frequently rolls, and did the final touch. They moved the stop sign from the middle of the sidewalk (okay, it was maybe 15cm off centre but that’s close enough to the middle) to beside the sidewalk. As a result strollers, wagons, and power chairs can easily move down the sidewalk and to the awesome accessible curbs. Thank you City of Abbotsford, Emmett really appreciates this. Of course, Grandma time is always fun. Grandma Pam is still in isolation mode so able to interact with Emmett. Grandma Rhonda is interacting with Emmett all of the time, just not by being physically present. Visits at a distance and using social media are the best that can happen right now.
CureSMA Canada is an amazing organization that works endlessly to help individuals with SMA and their families. One of the things they did during this pandemic time was to send a care package (pic on left) to put a smile on faces of those dealing with the pandemic but also with SMA. And it did give Emmett a smile. The second pic is Emmett in his backyard helping his mom with gardening. The third pic is just when Emmett moved back home and got to hang out with his favourite toys that he had left at home while he was at Grandma and Grandpas. He had barely been back at his house for any time at all when he announced to his parents a schedule outlining which toys he would play with and when. Who knows how long he had been planning this.
In the next pics we start off with Emmett being mommy’s helper and sorting and folding socks. Then out for a roll on a typical rainy BC morning. And then playing with his remote controlled excavator, dump truck, and front end loader.
This has not been the spring that anyone expected, but in so many ways it has been a fabulous spring for the Willms’ family. We know others have not been so fortunate and are hearts are heavy for them, but we also have to celebrate when things are good. And, for Emmett, things are good.
Thanks to a great project team. If you’re in the Fraser Valley, remember these businesses:
Highplace Homes – Micah Hooge
Legacy Plumbing
Valley Imperial Enterprises (Electrician)
And again, a huge thanks to all of you. Your support, whether financial or spiritual or emotional, is so very important to all of us.
God Bless!
May 30, 2020
Josh Carries Emmett!
BC’s Provincial Parks recently opened for day use and, after a wait to allow the first rush of hikers to subside followed by a wait to allow the downpour to stop, we headed to Rolley Lake Provincial Park on a cloudy morning. With the weather promising us a day of sunshine ahead while seriously looking like another downpour could begin at any moment, we were off on the 30 minute drive to this park. As we were getting ready, I commented that this was a ‘we carry Emmett’ hike and he quickly corrected me that it was a ‘We Carry Kevan’ hike. And he is correct, because the backpack that Emmett sits in for hiking is a unique people carrier. It was designed and made specifically for carrying those who cannot walk into places that they’d never be able to go with a wheelchair. We’ve mentioned it before, and Kevan is a young man who has Spinal Muscular Atrophy Type 2; his friends carried him around Europe in a custom backpack. That prototype is now the WeCarryKevan backpack that is available online. Emmett, as you know, also has Spinal Muscular Atrophy. Emmett has Type 1, however, with the Spinraza doses that he has been getting since before he was one (and he’s now five) Emmett has progressed and now presents as SMA Type 2.
With the backpack resting on the ground, Emmett is set into the seat and strapped in. It is very secure and the backpack even has hand holds for him to grab onto when he needs a bit more support. Then with the help of one other person, Josh (Happy Birthday today May 30th, Josh!) picks up the backpack containing Emmett and straps it onto his back. It’s perfect. Emmett sits up nice and high so this was ideal for grabbing wet tree branches and shaking the rain water onto his mommy. We were very pleased to note that do to social distancing and personal safety, the trails were labeled for foot traffic to go in one direction only and that 2 metres must be maintained upon approaching another family/group. As we were there mid-morning we had most of the trail all to ourselves (apart from a few people fishing and a couple of other hikers).
The first part of the trail is very accessible and Emmett has done that in his chair, but after about 500 metres the tree roots stop all wheeled conveyances. This is where the WeCarryKevan backpack shines. If Josh can walk there, then Emmett can go there. It was quite funny as Emmett realized this and began giving orders, “Go Up!” and “Go There!” and “Go Down!”. Emmett just giggled as they were tramping through the forest. By the way, the up and down is referring to going up steep hills and down hills to the lake. Of course, Emmett did think that his daddy could take him up a tree … but that didn’t happen.
Emmett is a very brave kid, but there was absolutely no way that he would even think about touching the huge banana slug that was crossing the path. Fortunately, it was travelling pretty close to the appropriate path direction so we didn’t have to report it to the park ranger. We did see a park ranger and asked if they had seen any bears. Emmett’s eyes popped wide open when the ranger responded affirmatively – but we relaxed when she said that the bear had been near her house which was a long distance away from the park we were visiting.
After our hike we had a picnic lunch near the lake and then we sat on the beach. Emmett was throwing stones into the water (possibly to scare the fish away from where daddy was fishing) and Emmett also spent a bit of time fishing himself. He has an amazing Lightening McQueen fishing pole and we saw a minnow chasing after his lure. Today neither fisherman caught anything so it was a good thing we had sandwiches and weren’t expecting a fish fry for lunch. While Josh was carrying Emmett, Alisha had a backpack full of gear plus a u-shaped pillow that, when Emmett tires and cannot hold his head up well, comes with the WeCarryKevan backpack and offers both support and comfort on those longer trips. Or those more rugged trails where Emmett gets jostled more than he is used to. Pam and I had the suction machine and water bottles in our backpack. So all of us were carrying a bit of stuff. Josh did find that a walking stick helped – we’re going to have to get some proper walking sticks for future hikes.
From Emmett and all of us,
Happy Birthday, Josh!
July 6, 2020
It’s Spinraza Time … but first Came Camping Time!
July 9th Update: Emmett is back home. The procedure went well. It is a tough day with a lot of anxious moments – because Emmett knows everything there is to know about lumbar punctures. This was his 18th lumbar puncture – they’ll be happening every four months for life, unless a new method or a new drug is discovered. Emmett wants you to know that he does like going to sleep when this happens.
Every four months Emmett receives a lumbar puncture where a tiny amount of spinal fluid is removed and a tiny amount of Spinraza is injected. This is the drug that has reinvigorated the pathways in his spinal column so that his brain can talk to his muscles. So that messages like, “Hey, swallow now!” and “Emmett, hold your head up!” or “Reach out and turn the page of that book!” get through. And Emmett does it. It is a miracle drug – a miracle that requires a huge amount of work. Recently on Canadian news there have been reports about kids’ parents seeking funding for a gene therapy treatment. A one time treatment – but it is only for children under the age of two and only in Canada under unique circumstances. Health Canada hasn’t approved it yet. Obviously, not something that is available to Emmett. We are, however, so thankful for the drug that is available. The healthcare system that is helping him. And Spinraza is an amazing miracle drug that is absolutely working for Emmett.
So this week, Wednesday July 8th, Emmett will be getting a lumbar puncture and for the first time Emmett will be anethesized prior to the procedure starting. Yes, that does mean that he has been awake and alert for every other lumbar puncture, however, for the last one he was anethesized part way through. You can appreciate that Emmett is quite looking forward to “falling asleep in the middle of the day!”. This is something that this five year old simply doesn’t do any more.
Four months is not a long time, but four months ago when Emmett had his last lumbar puncture the world was a different place. There was a virus overseas but nobody on this side of the ocean was really in a panic, it was just a concerning situation. Emmett was Abbotsford’s Police Chief for a day. Then in the intervening time everyone was suddenly welcomed into a version of Emmett’s world. Sanitize! Wash your hands! Social Distancing! Don’t go to the grocery store! Stay away from anyone who has a cough! On and on it goes – a new daily plan for everyone. Schools closed. Work places closed. Everyone’s life became a closer version of what Emmett has been living since he was six months old. There have been changes for Emmett too. Tough changes.
His family bubble was instantly closed up. Family members who were still working were not able to be inside his social bubble. The rare visits meant being on opposite sides of the yard. Video conferencing became the main stay of story time and more. Physiotherapy via video conferences? Yup. Speech Therapy – hmm, curiously Emmett doesn’t hear from them much. Occupational therapy – absolutely. In fact, there have been days when Emmett has been booked for 2 and 3 video conferencing meetings. So incredible. Pam and I have stayed in that family bubble – we’re not working outside of the home. We’re not going into grocery stores. We are extreme social distancers – if that is even a word.
Yet it is the start of summer, so when BC opened their provincial park system we logged in and booked a campsite for the five of us. Emmett had already agreed that he was ready for something new. Alice Lake has been a spring go-to place. It has cell phone service. Electricity. And the road ways are paved. It is ideal for camping and it was not even an hour away from BC Children’s Hospital. Emmett and his family have camped at Rolley Lake and loved it. We’ve done day trips to Golden Ears. But this year was going to be a stretch – we booked a few nights at Sasquatch Provincial Park. It is about 10 minute drive outside of cell access. There is no electricity (don’t worry, our RV has been fitted with a small inverter that can power all of Emmett’s medical equipment). And it is the furthest that Emmett has been from BC Children’s since he was five months old. Emmett and Grandpa were up for it; everyone else was optimistic with a large side of anxiety.
We choose provincial parks for two reasons. First off is that they are large and spacious sites with plenty of room for Emmett and us to roam without bumping into someone at a neighbouring campsite. Second, with a medical note, children and their parents get the base campsite rental free. This is a huge bonus for a family that is always challenged by the expenses that sometimes never end as you accommodate someone with medical requirements. The large sites also typically mean that there is a lot of trees, birds, and nature trails to explore. You can believe that the power chair batteries were fully charged and that the WeCarryKevan backpack was ready to go.
Emmett was so excited – just seeing the RV arrive in his driveway brought on a fantastic response. The morning that we we heading out, Emmett played on his own all morning while his mom and dad packed. Emmett is a great little boy but it is very rare that he will spend that amount of time without insisting that someone play with him. Once at Sasquatch Provincial Park Emmett wanted to do it all. He wanted a campfire. S’mores. Roasted marshmallows. Hot Dogs. Swimming time. Fishing. Playing at the beach. Going for a hike. And he did it all and then some.
This camping trip was fantastic. And it will give Emmett great memories to dwell on while he is undergoing procedures that he doesn’t enjoy at the hospital. We do have to say that the hospital is still one of Emmett’s favourite places. There are so many people who know him there. His friends. There’s staying overnight at Canuck Place – another fabulous group of people who go out of their way to support and assist Emmett and his parents. Thank you for supporting Emmett. You can send him a message on FaceBook through his parents – or his grandparents. This lumbar puncture is a tough go – but Emmett is even tougher! Literally, the strongest and toughest boy we know.
And now for some photos:
Deer Lake is one of the lakes in Sasquatch Provincial Park. It’s a beautiful park. However, Emmett found a pothole with his powerchair and he got stuck! Fortunately, we didn’t need to call in a tow truck.
The WeCarryKevan backpack was certainly put to use – and not just for wide trails. This backpack is made for going deep into the forest (so far that means within metres of the main trail!) which is so much fun for Emmett.
We found Eeyore’s House! It is always exciting when we find Eeyore’s house. Emmett loves his Winnie the Pooh stuffies – and when Emmett is on a hike he is always keeping his eyes open for Eeyore’s house. After a hike there is nothing as relaxing as lounging near a campfire.
While fishing during a canoe ride, Emmett caught his first fish. And after fishing it was refreshing to go for a swim in Hicks Lake – only Josh and Emmett were brave enough for that!
Campfires are so much fun – but finding rocks is even better!
Returning home involved getting cleaned up (we had good weather but there was one rainy day) and then having a swim in the backyard pool. Lots of fun stuff so far this summer. After this next injection of power juice is completed, we’re sure that Emmett has plans. Because he always has plans!
September 10, 2020
First Day of Kindergarten
Yes, with us just passing by labour day weekend the first day of school has occurred for students across Canada and the USA. It has been a very surreal start up as everyone is dealing with Covid 19 issues and concerns. Alisha and Josh have obviously been dealing with those very same issues that are obviously complicated by a little one who is medically fragile. This complication is magnified because they were really anticipating the greater social interaction with other kids. Obviously, even in prior years in our imperfect world that would introduce a range of health issues into Emmett’s environment and with Covid that seems like a quick black and white ‘No’ to school participation. And yet over the months that this pandemic has gripped the world there have been talk of drastically limited class sizes, reduced number of school days per week, and obviously greatly enhanced awareness on the part of most individuals of the need to sanitize, stay home if ill, wear masks, social distancing, and wash your hands! However, as the school year got closer and closer it became more obvious that the school system across Canadian provinces was going to proceed without any significant changes. These health and school challenges meant looking for other options.
The network supporting this family is amazing and one of these partners is Canuck Place. The teacher there has met and been working with Emmett in person (while he’s there) and virtually though online video conferences. She made several great observations and recommended some distance education programs that other families had accessed. Long story short, Emmett is now enrolled in the Oak and Orca Bioregional School and has started Kindergarten. Distance Learning education – where he will be at home while supported in his education by Oak and Orca.
On Tuesday Emmett was so excited for his first day of school photo shoot. You may not realize this, as Emmett is in a lot of photos, but he does not appreciate posed photos. Candid shots are by far the best. Yet on Tuesday he was ready to pose and get his photo taken. He was also ready to show off his sole front baby tooth. Months ago Emmett lost his bottom front teeth and just last week his first extremely wobbly top front tooth came out. He’s very protective of the last very wobbly tooth that is barely hanging in there – which is making tooth brush time quite an ordeal – but that’s just another part of growing up.
October 2020
Thanksgiving! Then Halloween! … Then a Lumbar Puncture!!!
Within our family social bubble, we had a Thanksgiving celebration. Turkey, dressing, and all of the rest for our meal. Fantastic cream puffs that Emmett helped make for dessert. It was memorable. But what was outstanding was our games event that took place between the fabulous main course (that Josh engineered) and the Emmett touched cream puffs for dessert. That is when Josh and Emmett introduced us to Carcassonne. Emmett had played this game once already. Just once. But when Josh started explaining the game by showing a card to us newcomers, Emmett interjected and told us what the card meant and how you played it. So from that point Josh just showed the card and Emmett provided the game play rules. It was fantastic. We are thankful, this thanksgiving season, for a little boy who is so aware of his surroundings, takes in everything, and simply loves life!
In the September pics of the first day of Kindergarten Emmett still had one front tooth. This Thanksgiving Emmett has no front teeth and, while the adult teeth are hiding in his gums, it’s hard to say when they’ll actually make their appearance. Kindergarten has been a lot of fun and Emmett absolutely loves school. As mentioned in the last post, Emmett’s kindergarten is a distance education program through Oak and Orca, which provides guidance and support to enhance the education that parents provide. It is going quite well. We have had excursions to the Great Blue Heron Reserve and have been to an apple orchard and a pumpkin patch. The ‘we’ is intentional, as school is truly a family affair plus, due to the challenging pandemic situation and the lack of social involvement in an actual school setting, everyone in the family is involved. Even if that involvement is story telling via video conferencing. With school we are pleased to say that Emmett is doing very well with his sight words and basic math facts. He is number one in his class – although we’re not keeping track. Too often people make assumptions about individuals who are in wheelchairs or whose speech may not be perfect; Emmett is a very bright little boy. Don’t believe us – when the pandemic is over come and play a game of Carcassonne with him! Then you’ll see.
As we draw closer to the end of the month there are two things that you should realize. The first is that you will see the return of Woody the Cowboy. 2017 was the first year for Emmett as Woody, then 2018. Again in 2019 Emmett returned as Woody the Cowboy. And now for 2020 Emmett has once again decreed that he will be Woody the Cowboy. The costume will be tight as Emmett has outgrown all of the Woody apparel that he has, but it is going to happen. His sidekick, Buzz, will likely be making an appearance also. You will also see an abundance of pumpkins at the Willms’ house. Hippo pumpkins. And the bar has been raised as this year it was more than one hippo pumpkin. My niece, Carolyn, pushed the envelope further by sending a pic of a 3D hippo face carved into a pumpkin. Challenge accepted. But then Emmett announced to his mom and dad that he and I would be carving yet another hippo pumpkin. Another search of the internet and we discovered the Team Fiona pumpkin carving stencil. While the first two were freehand, this time the stencil is going to be put to good use. By the way, in the pic at the top Emmett picked the two pumpkins on the table as the ones that he wanted to take home! LOL!
The second thing that we want you to realize is that Emmett will be receiving his 19th lumbar puncture and the corresponding injection of Spinraza just before after Halloween. There was a change in plans at the hospital and the procedure has been rescheduled to November 2. This will be a full-on visit with a wide range of blood work, physio tests, and the nerve tests. All of which make for a miserable day for a little boy. He will recieve a general anesthetic for the actual lumbar puncture, but he’s very alert for everything else. While he absolutely loves his hospital people, he does not look forward to this visit. He knows.
Emmett is working hard on his sight words. Alisha and Emmett came up with this awesome bean bag game to enhance word recognition. Then Emmett and I made the absolutely best ice cream. We will make some for you too! And then Emmett goes for a walk in his Kids’ Walk.
School is so much fun. This happened earlier in October when it was still short sleeve weather. We launched pop bottle rockets. We attracted a lot of neighbourhood kids. We tried our best to maintain social distancing … but rockets!
Emmett critiquing the elephant he just drew for the story he just dictated to his mom. And he’s using the homemade Rainbow Marker Holder – when the caps have been loosened Emmett can access them on his own. Up to now, someone has had to remove the caps for him. While they still have to be loosened, they’re not drying out and Emmett can use them, put them back, and have a ton of independence while being creative. *If you want to read Emmett’s Elephant story, just let us know.
October 31, 2020
5 Years!
This is Emmett’s fifth anniversary of the start of Spinraza, which in 2015 was known as SMNrx. This name changed to Nusinersin and now is best known as Spinraza. Emmett had the first of many lumbar punctures on October 7, 2015. This fifth anniversary post got postponed as BC Children’s hospital had to reschedule the October 2020 until November. This LP, #19, will take place on Monday, November 2, 2020.
EDIT: Everything went well today at B.C. Children’s Hospital. Emmett will be home soon.
There was no treatment available when Emmett was diagnosed with Spinal Muscular Atrophy, Type 1 five years ago. Emmett, however, was just the right age and weight in the fall of 2015 to be accepted into a study for a pharmaceutical drug trial. It was a drug that had a lot of promise but with a drug trial there are so many questions and very few answers. This trial would involve 124 similar aged babies from around the world. All with one common feature – they had SMA Type 1. SMA is the number genetic cause of death for infants – the trial for Spinraza was brought to an early close as those on the drug were improving and those receiving the placebo were failing – they were then given the drug. It was approved by the FDA and Health Canada afterward.
To understand how much Emmett has progressed, you need to realize that Emmett had lost all movement of his legs by 3 months of age. By 5-6 months of age he’d lost the majority of movement in his arms. He could no longer grasp things with his hands and fingers. He’d never been able to hold his head up. Obviously, he could not sit up on his own. Travelling had to switch from a car seat to a car bed – as the seated position caused too many issues with choking on saliva. All nutrients and liquids were by G-Tube (gastronomy tube) as swallow studies revealed that he wasn’t swallowing properly. There were a trip or two to the emergency room due to choking/stopped breathing. October 2015 Emmett was accepted into the trial for this drug – it was a blind study in which 2/3 of the children would be administered the drug while 1/3 would get a placebo. Apart from the fact that even with a baby it’s difficult to fake a lumbar puncture, small signs of improvement told us Emmett was getting the drug. You see, with SMA an individual may level off in their decline but there was no medical record of any individual with this genetic disease showing improvement. Emmett was improving.
Fast forward five years to 2020. There are now multiple treatments available for SMA. The first is this drug Spinraza, the treatment that Emmett is on, which can be administered to SMA patients at any age. After the initial dosing, it is administered every 4 months by lumbar puncture. Babies who receive this treatment immediately (days old) are walking, eating, and demonstrating incredible movement. There is the gene therapy treatment that has been getting a lot of press lately. It is the $2 million US dollar one time gene therapy treatment called Zolgensma that is for children under 2. Then there is the oral treatment that is taken daily called Risdiplam (Evrysdi). Of these three which are all approved in the USA for use, only Spinraza is approved in Canada. And in Canada provinces still limit, due to financial implications, who may receive Spinraza.
Emmett can hold his head up. He can wheel himself around on smooth, flat surfaces with his manual wheelchair. He can hold a pencil or marker and print his name. When placed in a sitting position, he can maintain it for a considerable length of time. He loves telling stories and playing jokes on people. He loves to have ice cream – he takes very tiny spoonfuls and can feed himself. However, his suction machine is always within reach as choking is still a significant concern. Emmett can usually tell us when he requires suction. While infrequent, there are moments when intense suctioning is required and everyone’s heart beats hundreds of times a minute. Once cleared, Emmett returns to play instantly. He’s fine, even if we may need a few minutes to recuperate.
This is the fifth anniversary of Emmett being on the drug Spinraza. To celebrate he is getting another lumbar puncture on Monday. More of this drug which has changed his life. More stories of his joy of living to share.
November 20, 2020
Water Study
When you live in the B.C.’s Fraser Valley, you know that the fall means rain. And not just a few drops here and there. Not a large cloudburst and then some blue sky. But day after day of deep grey skies, filled with clouds loaded up with moisture, moisture that never seems to stop falling from the sky. This seemingly endless precipitation provides the perfect opportunity for a water study. Therefore, Emmett is studying water. When we are outside going for walk/rolls or bike rides, we study the rain. For example, yesterday we discussed how rain flows downhill. We saw and studied how the fall leaves got stuck at drain grates and caused the water to build up and find a way around the leaf dam. We laughed together when we broke the dam and then watched the flood dissipate and heard the water crashing down into the sewer. We would have lifted the grate and looked down it, but only a city worker can lift the grate and Gerry, our friend the city worker, wasn’t available. (**Please do not tell Emmett that anyone can lift a sewer grate. He already has gone through phases where he requests every sewer grate be lifted, and our response is that only city workers can do that. Fortunately, Gerry the city worker has slightly appeased Emmett’s curiosity by lifting the sewer grate for him.)
At the Great Blue Heron Reserve we participated throughout October and early November in a weekly morning outdoor educational session in which our guide, Michael, taught us about water bugs, showed us the many different types of fish that live in the local streams and rivers, discussed all of the birds that depended upon the wetlands for food and shelter, and how animals like the beaver both enhanced the wetland yet perhaps caused issues by blocking pathways used by salmon as they returned to their home stream. With his mom and dad earlier in the fall we played on the beach of a lovely lake that had been created by hydro-electric dams and we discussed how these massive dams created electricity that powered his food pump, bi-pap, oximeter, his dad’s coffee maker, and so much more. As a result we have been learning about water and the importance of water for all of us for several weeks. Also, I’m pretty sure that I used one of Emmett’s syringes to spray him with water a few times. Of course, we have to tell you that Emmett loves bath time and ‘swimming’ in the bathtub.
So with this focus on water and these deep grey clouds overhead, it only made sense to study the water cycle. To do so we went to the recycle bin and resurrected the pop bottle rocket. While it makes sense to do a rocket launch comparison in sunny weather versus rainy weather and see what type of weather is most conducive to rocket launching, that is not what we did. The pop bottle rocket has been retired after several launches and isn’t going back into action. We saved most of the customizations for flight that Emmett had done (that is, stickers) but that was all that was left of the rocket. We then used a sharp knife to cut the top off of the bottle. This top part needs to be large enough that, when you invert it, the top friction fits into the bottom of the bottle. Like a funnel. Then we used a ruler and a permanent marker to put a measuring gauge on the side. We did centimetres. This took not much more than 5 to 10 minutes and it was the easy part. The hard part was getting Emmett to select a location for this rain gauge. He quickly discounted the front yard, as he knew that something as valuable as a pop bottle rocket converted to a rain gauge would be at high risk for theft. While he liked the idea of putting it on the back porch because he could get to it easily, he also knew that this would not get much rain because the back porch has a roof. So then he settled on the roof. That’s right, he wanted his frail and elderly grandpa to put this rain gauge up on the roof. Meaning that every time it needed to be checked, his wobbly and fragile grandpa would have to climb up the ladder and go onto the roof. Well, I refused that option. Emmett laughed and then we settled on the backyard, right where Emmett could easily see it. Within the first three hours our rain gauge gathered about 1 centimetre of water. After 24 hours it registered 2.2 centimetres of water. As there has been nearly 22 centimetres of rain recorded in Abbotsford for the month of November, our rain gauge is fairly accurate.
While we were collecting rain outside, Emmett and I decided to make it rain inside the house! Emmett’s first plan was to take the roof off of the house, but his mommy and daddy said, “NO WAY!” so we couldn’t do that. Instead to see how the water cycle works we filled the electric kettle with water and turned it on. Then we filled a bag full of ice. Then when the kettle was close to boiling a grownup held the bag over the boiling hot kettle spout. The kettle was like the heated-up earth and the water vapour going up was real. When it hit the bag of ice, that was like being high in the atmosphere and hitting cold air, so the water vapour turned to condensation. When the condensation got heavy it turned to rain! And we made it rain in the house without taking off the roof! We didn’t even need rain coats.
This last part has nothing to do with water and rain. It is a reader’s theatre that Emmett created with grandma and grandpa. One of our critics pointed out that Emmett wasn’t reading everything and that is true. He had a bit of stage fright and anticipated his lines, but I still think that he did a spectacular job. So without further ado, here it is and we hope that you enjoy it. Mo Willems, let us know what you think!
December 12, 2020
A Time to Give!
Canuck Place Fundraiser!
Click on this link to go to Emmett’s Canuck Place Fundraiser and make a donation to Canuck Place.
All Funds raised in Emmett’s Fundraiser go directly to Canuck Place. Tax Receipts are available. Emmett will select two people from everyone who makes a donation to his fundraiser for Canuck Place to receive one of his Santa’s Sleigh creations. Thank you for supporting Canuck Place, an organization that means so much to us.
Recently a Canuck Place van came down the street to Emmett’s house. Christmas music blaring through the van’s windows. When it stopped at his house several elves and a Christmas tree rushed out of the door bearing gifts for Emmett. It absolutely made his day. And then as fast as they appeared, they vanished. Off to the next medically fragile child so that they could bring Christmas cheer. This socially distanced Canuck Place drive-by was a fantastic 2020 opportunity for Emmett.
Canuck Place is far more than a place that provides Christmas cheer. Every day, Canuck Place is there for children with life-threatening illnesses and families across BC who require support to navigate through one of the hardest times in their lives. Emmett was just 6 or 7 months old when he first visited Canuck Place, which provides clinical care at two BC locations as well as consultations in home or in hospital. Canuck Place provides space for families needing to manage their pain, create special memories, and share love – all at no cost to families.
Thank you. Emmett and his entire family appreciate your support.
To make a donation to Emmett’s fundraiser, click here!
Dec. 13 update: $125 has been raised. Thank you!
When Emmett was 5 3/4 months old (he’s now 5 3/4 years old) Emmett was a little boy who could not support his own head, roll over, or sit up. He couldn’t move his legs and the loss of arm movement was so massive that it was inevitable. Emmett was also losing the ability to swallow and sitting in a car seat/baby seat was a danger – he had to be lying down or he would choke on his own saliva. At that very difficult time with multiple appointments at BC Children’s Hospital, Canuck Place was there. Recently, Emmett’s food pump stopped working – he cannot swallow so all of his nutrition and liquids are provided through a G-tube (gastronomy tube) – and Canuck Place was there and a supplied a loaner pump almost instantly. This is a fantastic organization and we hope that you support it. If you feel that it’s too far away from where you live that is understandable and appreciated, but then support a similar hospice near you. Thanks.
A Time To Give! Part 2
December 17, 2020
Thank you to everyone who supported Canuck Place through Emmett’s fundraiser.
$1950 was raised for Canuck Place, and a Canuck Place benefactor doubled that to make the donations worth a total of $3900.
Two of Emmett’s Santa Plaques were awarded: one is going to Anne in Ontario and one to Alesha in B.C.
Thank you for your support! Happy 2021!
Orange Hippos – you’ve got to be wondering what this has to do with a little boy with Spinal Muscular Atrophy, his Canuck Place Fundraiser, and anything to do with reality. The fact is, absolutely nothing and yet absolutely everything. Because this team name/phrase is from Emmett’s incredible imagination. In this pic, taken in 2018, it shows Emmett when he still had front teeth and when his Orange Hippos t-shirt still fit him. Emmett is holding the original Orange Hippo, which for several months was his most favourite possession, and playing with his giant hippo, Po. Even now, when I asked Emmett and his mom where the original orange hippo toy was for the headline photo that is above, he could direct me to the exact location where this prized toy was located. No help was required to find Po!
Emmett has many favourite animals, but without question the Hippo is the absolute and ultimate favourite. And while he will rattle off several colours that he likes, there is no doubt that Orange is his ‘top of the charts’ favourite. In 2018 when we were getting ready to participate in a roll/walk/run for Spinal Muscular Atrophy that was held in Stanley Park, we had to come up with a team name. While the adults bounced several ideas around, Emmett’s suggestion was “The Orange Hippos”! That name stuck! The t-shirt was created. And the rest is history.
So there you have it, the name the orange hippos represents a little boy’s incredible imagination, sense of humour, and love of life. This is Emmett’s fundraiser – and the Orange Hippos started from his imagination but has morphed into the team that includes all of his family, friends and supporters.
If you make a donation to my.canuckplace.org/orangehippos then you will be making a donation to support many medically fragile youngsters and their unique imaginations. Plus if you make a donation this month (December 2020), your donation will be matched so every $1 you donate will mean $2 for Canuck Place. As of now, the fundraiser is at $1100 – we are really close to our goal of $1225.
Emmett loves the orange hippos and would love to see every room in the house decorated with them. This, of course, is maybe going too far for some members of the house! LOL!