Janurary 2019
Merry Christmas and a Happy New Year!
January 1, 2019
Emmett, Alisha, and Josh want to wish you a Happy New Year. Thank you for reading our blog. Thank you for your prayers, interest, and positive thoughts. This little boy has grown so much this past year. In fact, his Orange Hippos T-shirt is now too small but in August it was too big! That is why Emmett is in a big boy bed and, in fact, is even starting toilet training. This is facilitated by an awesome toilet seat setup which will hopefully reduce back strain – for his mom and dad – not Emmett.
Emmett made Hippos on Wheels for many family members as Christmas presents. He loved giving them out – especially his parents. He shrieked ‘Mommy’ and ‘Daddy’ when he gave them their hippos! While Emmett still hates getting dirty, his grandpa somehow got paint all over Emmett’s face. Grandma Pam showed him what he looked like in a mirror. He loved it and left the paint on until his bath!
Emmett got to visit with his great-grandma Willms. Family time is so important!
There was no snow on Christmas Day so it was perfect to go out for a roll and walk to get some pictures!
Emmett has become a fussy little boy when it comes to hats. This year he has not wanted his ears to be covered up. Obviously, that causes some concern in cold weather so Grandma came to the rescue with an ‘awesome sauce’ hat that he allows over his ears. It had matching mittens with a flip opening for his fingers. This is important as he uses his right hand on the joystick and his left hand to change the speed levels. His driving is rather scary when he doesn’t have a good grasp on the joystick.
Opening presents was quite a workout. He’s got pretty good arm movement but ripping paper still is a lot of hard work for Emmett. In the video you can see how he’s turning pages in books. Emmett loves stories! In the video you’ll hear Emmett calling out ‘Look, Po!’ a couple of times – we should explain that Po is his giant stuffed Hippopotamus. This boy has a vivid imagination.
You’ve seen Emmett pull Po around in his little red wagon. This time Po rode on the back of the power chair. This was also the first time that Emmett has allowed Po to come over to grandma and grandpa’s house.
On New Year’s eve Emmett stayed up until the clock hit 9 o’clock. He enjoyed playing KerPlunk and Hungry Hippos!
2018 has ended. 2019 has started. We are looking forward to what is ahead. What we can tell you is that the drug Spinraza (also known as Nusinersen) has changed Emmett’s life. The changes are still happening.
It has been quite a year for Emmett. Here’s a quick recap.
January – 2018 started off well and, with his back brace on he was sitting up by himself.
– but on January 26, 2018 he was admitted to BC Children’s hospital, into ICU the next day and was diagnosed with RSV. We cannot describe how seriously this affected Emmett and all around him. And remind you again, if you’re ill please ensure that you don’t spread your illness with medically fragile (well, honestly, don’t share it with anyone!) He was intubated. He was heavily sedated for several days. He had more tubes inserted into his little body than we could count. It was terrifying. The original blog posts left out so much of the terror we felt. It was too much to share.
February – it was a total of two agonizing weeks that Emmett was in the ICU and on February 9 Emmett was discharged. He bounced back so quickly! Phew.
March – his third birthday and a special ride on a BC Transit bus!
April – “Read a book. Please!” and “What’s That!” seem to be his favourite phrases
May – Emmett’s power chair had it’s speed limits upgraded. Can he ever go fast now!
June – We introduced you to The Orange Hippos! And Emmett went camping! He got to try out a standing walker – which he sort of liked but it hurt his pen… hmmm, can we type that word on a family blog? (he still doesn’t have a permanent standing walker. Maybe in 2019!)
July – Emmett watched the CARS video for the first time. He really wanted to watch Toy Story again! And more outdoor excursions.
August – The Orange Hippos participated in Florence and Charlie’s 10K to End SMA. Emmett’s team, The Orange Hippos, was identified as the team that raised the most funds! Thanks to all of you for your generosity.
September – Emmett went fishing. During tummy time when he was lifted up he HELD UP HIS OWN HEAD! Such a huge accomplishment!
October – Emmett has SMA Type 1 but in October his neurologist told him and his parents that Emmett was now presenting like a child with SMA Type 2. This is a huge improvement. The drug Spinraza is the first approved medical treatment for SMA and IT WORKS WONDERS!
November – Emmett got his Toy Story big boys bed! And he won a second hand Freedom Concepts three wheeled bike specially designed for children with physical needs. It’s awesome.
December – Emmett got to go on a plane ride with Santa Claus. This was organized by the very amazing Canuck Place! Last January we told you how Emmett was sitting up with a back brace. Well, now when he is put in a sitting position he can sit up without a back brace. Plus, during tummy time he held his head up on his own to watch Daniel Tiger. THAT’S ANOTHER FIRST! Who knows what will be next!
Oh yes, Emmett loves telling jokes!
February 2019
Winter: It’ll Be Over Soon
February 9, 2019
Can you believe that it is almost the middle of February! Here in the Fraser Valley we can believe it because winter weather really didn’t get here until February. Our almost springlike January is now a figment of our imagination as we have to deal with temperatures that have actually dipped down below freezing! Of course, the rest of Canada isn’t feeling too sorry for us with our last minute winter. We cannot complain as this winter has been awesome – and for far more reasons than the temperate weather conditions. This winter has been one of really good health and we absolutely celebrate that. Plus Emmett is really enjoying the snow! So how upset can we be over a few snowflakes!
Every time of the year is disinfectant time, but even more during winter which could also be known as cold and flu season. Sneeze near Emmett and he will likely tell you to wash your hands. Cough and you’ll be told to disinfect! This little boy knows that staying healthy is important and he follows the lead of his family. Alisha and Josh really appreciate how everyone who comes in contact with Emmett and themselves takes steps to ensure they all stay healthy!
Now back to winter, while some days have been miserable enough that Emmett stayed inside, others were so incredible that Emmett had to go outside and play. Somedays the snow was just a light covering, but there were also days when it was perfect sledding weather! And Emmett – decked out in the only hat that he allows to cover his ears – was out and taking full advantage of it. Mom and Dad also welcomed the opportunity to play in the snow rather than playing hours upon hours of Fisher-Price, Playmobil, RV, Winnie the Pooh, and doll house!
Emmett got to have four Christmases that went almost to the middle of January. For a three year old who loves everything about Christmas, this was absolutely fantastic. Of course, he also got to see family from all over. Aunt Jenny, Uncle Ben, and cousin Henry came to visit from Calgary. Aunt Katie and Uncle Josh were here visiting from Melbourne, Australia – apart from video chats they haven’t seen Emmett in 2.5 years so that was a great reunion. Uncle Ben and pretty soon to be Aunt Alesha were around a lot. Of course, his grandma’s and grandpa’s were around to visit also.
As you can imagine, four Christmases led to a great number of awesome presents. And Emmett probably got spoiled. He misses out on a bunch of other things in life, so a bit of spoiling here and there is okay. Playmobil camping toys, lego, clothes, stuffies, a doll, and I’m sure there is much more that I haven’t listed. (BTW, If you are nearby and have a 3 D printer could you contact us? Cliff needs some custom AFO’s (ankle and foot orthotics). Cliff is the doll that Emmett got for Christmas and he has wonderful hair! But a set of AFO’s would be awesome. Let me know if you think you can help us out.)
Emmett has had a really good winter. We are happy to report that he has been healthy. His voice is getting stronger and stronger with his language becoming even more understandable. He is able to stick his tongue out now – something he could never do before. SMA not only affected his limbs, ability to swallow, and his breathing, it affected his facial expression and ability to control his tongue. Obviously that affected his speech so this chatterbox is becoming even more of a chatterbox. His imagination is incredible and he simply loves playing. He plays almost everything. The different voices that he gives his giant hippopotamus Po, along with Winnie the Pooh and friends, is fantastic. Emmett is also continuing to grow. He will be four next month and he is tall for his age plus has a solid build. As a result the homemade wheelchair that he has is gradually becoming less comfortable for him. Emmett’s support team came to the rescue with a loaner mini-wheelchair which is absolutely incredible but, believe it or not, almost too small for him. This little boy is really not so little any more. Alisha really feels it as she is lifting him multiple times each day.
Adding to the back strain is that Emmett is having success (some days) at potty training. Some of you are likely saying isn’t that late for a three year old while others are likely wondering how it is possible. Sure it may be a bit late, but considering the physical challenges that Emmett has sitting up it is understandable. He had to wait for the appropriate ‘potty seat’ that wouldn’t wear out the care giver. Once again Emmett’s team came up with a great solution. There aren’t any photos although the other day Emmett, as he was sitting on the potty seat, ordered his mom to take a photo and post it on FaceBook! How does he even know about posting pictures on FaceBook! Smart kid! But there will not be any potty photos!
Next month Emmett will be turning four. He’ll also be visiting BC Children’s Hospital for another Lumbar Puncture and injection of Spinraza. The progress that we have seen over the last 3.5 years since Emmett began receiving this drug has been amazing. Thank you for your support, your calls and emails, your prayers and your best wishes. They help and are appreciated more than you can guess!
A Winter Video
February 16, 2019
March 2019
March 5, 2019
Does Spinraza Work – YES!
On Wednesday, March 6th Emmett will be at BC Children’s Hospital for another treatment of the drug Spinraza. This is going to be his fourteenth lumbar puncture where they withdraw spinal column fluid and replace it with fluid containing the drug. This is also a day which is full of Emmett meeting all of his friends from his medical team – plus likely a few new faces.
Before we get into all of that, we want you to know that Emmett is already looking forward to going camping this summer. He knows the campground he wants to go to. He knows that he wants his parents and all of his grandparents to join him (of course, Emmett is not old enough to go camping by himself!). And he’s planning on going to the race car track with his grandpa. He recently was talking about this and then talked about how last summer his caregiver (I don’t want to identify Alisha so I’ll just say his caregiver to be anonymous) forgot to pack his hearing protectors. So we didn’t spoil his day or his earing, he used some of my soft ear protectors that fit into the ear canal. They were orange – he remembered that too. He literally remembered all of this and was talking about it without any prompting from the adults around. Alisha, laughing, asked him how he remembered this from so long ago. His response was a typical EMMETTism – “It’s just been stuck in my head for a long time!”. What a memory this kid has. A memory that could be helped by the fact that he has a huge brain! Literally – his head is gigantic. We bought him a bike helmet for 5 year olds. Not big enough so back it went. Bike helmets for 8 year olds. Too small. We have to get him a youth sized helmet. What is really ironic about this, is that this kid with the large head is afflicted with a disease that affects all of his muscles. It is incredibly hard work for him to hold his head up (his neck muscles are getting stronger and stronger) and how much easier would it have been with a normal or smallish sized head! Sigh. Well, his neurologist told Alisha and Josh early in his life to ensure that they made memories and so they have. And Emmett with his large brain has the amazing capacity to remember an incredible amount of these family memories. Every day is fantastic. Every day is a challenge. But still fantastic.
Emmett does not like the lumbar puncture – and he is old enough to recognize the tests/visit which means it is a lumbar puncture day. He’s a smart kid. He does not like the blood tests. He really does not like the nerve conduction study – fortunately that now only happens once a year. Unfortunately that ‘once a year test’ is this visit. Emmett really enjoys most of the rest of the sessions that happen during the day. They put him through a physio therapy workout which, if he’s in a good mood (he will be if it happens before the nerve conduction study and the blood work), he loves showing off his moves. Of course, he doesn’t always do what is expected. Par for the course at his age. If he’s had the blood work already, he’ll tell the therapist about it and show the bandages. Indicating and saying that this is why he won’t move that arm! He loves talking with the therapists, the nurses, the staff, and the doctors. They are his medical team. So as you go through your day, say a prayer for Emmett. Think about him, his mom and dad, as they spend the day at the hospital. Think about others that you know who spend so much time at the hospital that everyone knows them and they know everyone. That is probably not how they planned to spend that time.
14 lumbar punctures with this drug called Spinraza. Emmett has been receiving this drug as long as, if not longer, than anyone else in Canada. He is still part of the study being held to study the effects of this drug, which is now owned by the company Biogen. When Emmett began this study no one really knew if the drug worked. It literally was an experiment. It did and it does work. Not only did Spinraza STOP this disease known as Spinal Muscular Atrophy (of the commonly identified four types, Emmett has Type 1 which is the most severe) but it has ACTUALLY reversed the disease. Emmett has absolutely improved. What is even more amazing is that the improvement that Emmett has demonstrated simply did not happen with SMA prior to Spinraza. Children with SMA could be supported and cared for, obviously, but there was no means of changing the ongoing degeneration of this disease. That was the message received when Emmett was diagnosed in 2015 – there was no known medically approved treatment for SMA. But there was a trial. And now there is a treatment – Spinraza.
Canada has approved this drug. So have many other countries. Other countries have approved it for many if not all individuals with Spinal Muscular Atrophy. But NOT Canada – except for the province of Quebec. After consideration, Quebec has approved it for all individuals with SMA. Quebec has a different process of recommending approved drugs for individuals than the rest of Canada.
Emmett has undergone so much to help prove to Canada and the world that this drug works. It was a risk that Alisha and Josh took. Obviously, we’re all glad that they did. Spinraza is literally a life changing and miraculous medication. Yet in Canada there are individuals who cannot access this approved by Health Canada drug. It has only been approved for a minority. The rationale given for limiting access to this drug, by the committee that makes this recommendation, is that there is not enough data to prove it works. Technically correct. I believe that it is also correct to say that drugs, like Spinraza, which are developed for rare diseases like SMA mean that typical benchmarks must be set aside and other factors considered.
There is not enough data because the formal testing and the scientific blind study was stopped early. One of these was the study of which Emmett was a part. A study that we, individuals who knew nothing about the scientific trials and discoveries that were taking place, in which we knew Emmett was getting the drug rather than a placebo because he was improving. The drug company STOPPED the test and then provided the drug to all participants BECAUSE participants on the placebo were dying. The vast majority of those on the drug were improving. It was not humane to continue with the blind study when facts overwhelming proved the drug worked. This is not to say that everyone with SMA will be cured. Spinraza is not a cure. Emmett is not cured. Every day is a struggle for this little boy. But access to this drug Spinraza is an incredible opportunity for Emmett and for everyone with Spinal Muscular Atrophy.
This drug is expensive. Understandable when you take into consideration that it reconfigures the nervous system to transmit messages to the muscles. That takes some work. Understandable when only a hundred people worldwide are getting the drug. But the price must come down when the numbers of users is increasing. The Canadian government got a discount – although we don’t know what that figure is. Certainly it is expensive. This is a rare disease and an even rarer drug discovery. That changes lives!
If you would like to support CureSMA Canada as they seek to get improved access to this drug, please consider writing your MP, MLS/MPP, your provincial Health Minister and Deputy Health Minister. We are not asking this for Emmett. Emmett meets the current requirements. We are asking on behalf of Emmett’s friends and individuals that Emmett doesn’t even know. We are asking on behalf of Emmett’s friends who have already lost this battle. Emmett, Alisha, and Josh participated in this study with the hope of a better life not just for themselves, but for everyone stricken with this disease. The potential for that better life is right there in front of many of these families who know that this drug works – if only this drug was available to them. If you want to send a letter to encourage the government to reconsider, below is text that you can copy and update with the information for your province or territory.
Emmett thanks you. So does all of his family.
(Date)
Dear (insert name)
Please support reimbursement for all patients who require the drug Spinraza. You are already aware of the pressures that are being applied to the Spinal Muscular Atrophy (SMA) patient community in Canada. These are people attending school, university, volunteering in their communities, hold careers and have families. However, with SMA, the body becomes weaker and weaker, patients lose lung function and motor function, but it never affects their cognitive abilities. These people laugh, learn, love, and cry. They know that they have a terrifying genetic disease.
Until now this serious, life limiting neuromuscular disease has never had the benefit of a treatment. Biogen’s Spinraza (Nusinersen) has been developed and is the only treatment that has ever been proven effective and come to market for SMA. Spinraza was approved by Health Canada, however, the original recommendation from CADTH was only to approve treatment for a small subset of type 1 patients. After a resubmission and appeal by Biogen, CADTH has offered a broader coverage, however, still only encompassing type 1 patients and type 2 patients up to the age of 12. This narrow selection of patients recommended for reimbursement is a devastating blow to the entire SMA community and its supporters. The remaining patients affected by SMA will steadily decline as the disease progresses and they will eventually die from the disease.
Everybody should have the right to be all that they can be, to achieve career and personal goals, to have hopes, a future, an improved quality of life, as well as longevity. How can we as a country allow a disease to cause even one person to lose their functionality and die when there is a proven safe and effective treatment to halt the progression! This disease has always been considered a tragic diagnoses, but in Canada it is even more devastating because there is a treatment that many are unable to access. 45 countries plus the province of Quebec have approved broad access. The same data was submitted to all of these countries and now, due to the delays within Canada, there is even more information available of the benefits of Spinraza for all ages and types. This overwhelming evidence should not be overlooked or dismissed, but it has been!
We implore the provincial governments to approve reimbursement for all patients. Those with SMA and their families have been waiting and hoping for many years for this day that there is medical hope. We have a treatment now and it is an incredible injustice that access for all patients is not available. There are no other treatment options for SMA.
Make the right decision, the clock is ticking for individuals with SMA – without treatment it gets louder every day.
Help us change the outcome to one that saves lives!
Sincerely,
(your name)
(your contact info.)
March 10, 2019
Happy 4th Birthday, Emmett! (March 9th)
A few weeks ago we started talking to Emmett about how his birthday was going to be happening soon. Emmett was quite concerned about this, as he had already had a birthday party. Emmett has very clear memories of his third birthday where he got to ride around the block in a city bus! Emmett wasn’t quite sure why he would need another!! It took a while to convince Emmett that he actually gets more than one birthday party – one every year in fact. Fortunately by the time his actual birthday came around last Saturday, Emmett was more than certain that he wanted another party – and more presents!
Many of you know that Emmett’s Birthday is a really huge deal in our family. When Emmett was 5 months old a neurologist told us that Emmett likely wouldn’t even live to see his first birthday. The doctor told us that Emmett would never hold up his head, sit up, walk, or talk, and that he would continue to lose his ability to move, to breath and even to smile. During the two months leading up to Emmett’s diagnoses we had recognized that something was not quite right in Emmett’s little body – but could never have imagined that it would be as terrible as SMA Type 1…
For us, Emmett’s birthday isn’t just a special day, it’s a reminder. A reminder that miracles do happen, a reminder of the joy and the love that is in our family because of Emmett. This is why we go all out for Emmett’s birthday, convincing the Abbotsford Fire Department to bring a fire truck and fire fighters to make a little boy happy. This is why we spend hours baking and decorating the coolest firetruck birthday cake – even though Emmett can’t actually eat it. This is why Emmett’s birthday is the most special day of the year to us…. We were never supposed to experience it. Every birthday Emmett has gives us hope for the next one, and the next one, and the next one. Here’s to many more special days with you, our wonder boy!
We love you Emmett,
Josh & Alisha
Check back frequently throughout this week as more photographs will be added.
Huge thanks to Engine #6 from Abbotsford Fire Station #1 that came out to celebrate with Emmett. It was awesome, unfortunately, they had to respond to a call so they didn’t get any birthday cake. As Emmett lacks lung power, he blew out the candles by waving paper plates!
May 2019
Book Review
May 5, 2019
Emmett loves books, stories, and expressing himself. He loves telling jokes and stories – and as he gets older his stories are getting more and more sophisticated. (Remember, I’m a grandpa so there’s a slight chance that I’m biased!) Just the other night his mom asked a question about the location of one of their friends and Emmett piped up that he knew. Then he went on to tell us that this person was on a boat trip and they were going for a long time but then the boat sank, in very deep water, and that this individual then had to swim to California! Oh my goodness, he was funny. Very serious and straight faced as he told us this fantastic yarn. Perhaps his fascination with ships comes from his recent appreciation of the book Elephant and Piggy Go For A Drive. Do not let the title mislead you, but rather than me telling you about the story I’m going to let you join Emmett as he reads/tells this story to you. His obvious enjoyment of the story indicates that he’s giving this book two thumbs up. (Please excuse the very poor video work – but I needed more hands! There will be better video soon.)
What is incredible is that when Emmett’s journey started, his specialists couldn’t say if he would be able to speak or not. Obviously, SMA affects the muscles that are involved in speech. The progress that this little boy is making is so wonderful; the joy that he is sharing is contagious. Emmett has had a wonderfully healthy winter and that is such a blessing. This past week he has had a touch of the stomach flu which obviously put everyone on edge. He’s spent a lot of time on his bi-pap and, while he still requires close monitoring and a lot of suction, he’s doing much better. With no trip to the hospital.
Emmett is proud to announce that he has a new aunt. This was a massive event and Emmett was one of the ring bearers in the wedding of his Uncle Ben to his new Aunt Alesha. There are no pictures yet as the bride and groom have not released them. That will happen soon though! We are very proud of Emmett’s work and he overcame a few road blocks. First and foremost, his electric wheelchair malfunctioned exactly one week before the wedding (on a Friday night, of course) and the technician was sick so couldn’t come and work on it (remember, no sick people near this little boy). As a result, Emmett had to go to the rehearsal in his manual chair and there was absolutely no time for even a practice run – as the chair got fixed just 24 hours before the wedding. Bottom line, he did fantastic. Congratulations to Ben and Alesha!
Emmett has been getting his speed fix too. Whether it has been on his bicycle (hmmm, it has three wheels so should it be called a tricycle!), his manual chair, or his power chair – even riding in his van – Emmett loves the sensation of going fast.
There will be a lot more photography in a day or two!
Emmett’s Corner
May 15, 2019
Oh boy! There’s nothing quite as hilarious as getting on Emmett’s bad side. While this little one is a very cheerful little boy most of the time, he does have his moments! Maybe you didn’t read a story … or you couldn’t understand what he wanted … or he just woke up in a grumpy mood! Whatever it was, it is a momentary reminder of another aspect of this little guy’s very expressive personality.
Daily workouts and physio sessions with mom are a key component of daily life. Tummy time is something that has always been a frustration point for Emmett. Bowling is much more his style – and gives whoever he is playing with an extensive workout.
Emmett’s uncle Ben (affectionately called ‘Ubba’ got married recently to Alesha. Emmett was one of two ring bearers and he did a fantastic job. Congratulations to Ben and Alesha!
Unfortunately Emmett picked up a bug over that happy and exciting time. This meant a lot of time on bi-pap and resting, but it was also a bug that Emmett was able to fight at home without a trip to BC Children’s Hospital. He was under the weather for over a week yet his personality was back and he was feeling a bit better after about three days.
There’s a new addition to Emmett’s menagerie of animals – please welcome Ted E. Bear to the family. Ted E. Bear was made by volunteers at an organization called Tubie Friends. Tubie Friends aim is to help children with a feeding tube – and Emmett certainly has an instant connection with Ted E. Bear. What’s fantastic is that Ted E. Bear’s feeding tube port is orange! Further helping Emmett feel at home with himself is that Cliff now sports a fantastic pair of AFO’s. I put on a call of FaceBook for help from someone with a 3D printer and a huge thanks to Dylan who designed and got Cliff’s AFO’s printed!
Spring has led to finding beautiful flowers and some wonderful hikes. The suspension bridge at Cascade Falls is a short and very steep hike to an awesome view. Please note, it is not wheelchair accessible.
Playing cards is a new activity for Emmett. Playing RV, Toy Story, Cars, and on and on are still a huge part of Emmett’s daily activities.
Keeping Emmett occupied is easy as he always loves and wants to be doing things. The bigger question is whether or not you can handle playing Winnie the Pooh and the Hundred Acre Wood or RV and a family trip for as long as Emmett wants to!
You may recall that in the past Emmett hated getting his hands dirty. In fact, when playing with his mini-sandbox he wouldn’t touch the sand but only use his small plastic implements to move things around. Well, here is photo proof that Emmett is getting his hands dirty! Hurray! Emmett also got to sit in his grandpa’s race car and this was a first. Not because grandpa hasn’t let him sit in it before, but even though Emmett loves looking at the race car and talking about it, up until now he has declined every invitation to sit in it.
Emmett is still very much in an Elephant and Piggy phase of his books right now. Here’s another story that Emmett would like to share. He’s wearing his bi-pap which impedes his enunciation a bit, but we hope you enjoy another moment of reading at Emmett’s Corner!
July 2019
Another Lumbar Puncture!
It’s the Little Things!
When you say the phrase Lumbar Puncture out loud, does it make shivers go up and down your spine. Because it does to me. Literally, I just cringe and yet I have never had one done. Today Emmett will receive his 15th lumbar puncture where the specialists will remove a tiny amount of spinal fluid and replace it with an equal amount of fluid that contains the drug Spinraza.
Emmett was in really high spirits yesterday. There were a few tests at BC Children’s Hospital with more this morning. Some of the tests today are not ones that he appreciates and he seriously doesn’t like. He knows what is going to happen. I seriously think that he looks forward to it … BUT he also knows that it is going to hurt. And that causes his anxiety and apprehension to build. That is normal. Hang in there Emmett, we’re so supporting you! *N.B., All went well and Emmett is back home! Plus he got to watch Cars 3 and Mary Poppins Returns!
Emmett was just camping at Alice Lake Provincial Park. This was his second time camping there in RV who he calls RV (stands for recreational vehicle – our motorhome who Emmett sometimes makes talk) and he has literally been waiting all winter for this to happen. Even better was that Grandma Rhonda and Grandpa Roger were there with their RT (recreational trailer – so Emmett can easily identify and differentiate between the two). Emmett had so much fun that we’ll talk about in just a bit. Right now it is time to talk about mosquitos. First off, you need to know that in an amazing twist of nature that there are really not that many mosquitos in the lower mainland area of British Columbia. It is important to say that so that people in the rest of the country can be even more envious of this geographical area which truly is God’s playground (you’ll see some of that in some upcoming photos). However, while there are few mosquitos all it takes is one and while camping Emmett got three mosquito bites. One on his tummy, one on his shin, and one on his forehead. You need to know that for some reason a lot of individuals with Spinal Muscular Atrophy react adversely to mosquito bites. That is Emmett. A bite causes him to have the area swell up, often break out in blisters, and be incredibly sore and painful. In fact, a mosquito bite is literally worse for Emmett than a Lumbar Puncture. Don’t believe me! Then stay in the RV with Emmett overnight when he has had a mosquito bite. Oh my goodness! The pain and agony that this little guy has to endure from a mosquito bite is one that he ensures that everyone within earshot also endures. It is intense!
Soothing cream. Antihistamine. Cool cloths. Ice Packs. Heaven help you if you move that ice pack in the middle of the night because then all four adults (the tent got rained out so it was four of us plus Emmett) heard the very loud cries to get that “ICE PACK!” back on that mosquito bite. If it wasn’t that Emmett was really uncomfortable and none of us were getting any sleep, it would have been rather humorous. As much as Emmett doesn’t like getting Lumbar Punctures, and he obviously gets upset and says “Ouch” many times before and after the L.P., he truly is incredibly stoic about the entire procedure. While it slows him down for a day or two, within hours he is his normal happy self. In fact, one of his play stations is a hospital setup where he has been known to perform lumbar punctures. In comparison, while he plays RV an incredible amount of time throughout the year, I don’t think he’s ever included mosquitos in his play time! Fair to say that he really and intensely dislikes mosquitos! (Obviously, while posing for a photo while daytime it was time for Emmett the photo ham to pose for his audience.) When talking with Emmett just hours after the Lumbar Puncture we asked Emmett which was worse, the blood test (which he also doesn’t like) and Lumbar Puncture OR a mosquito bite? With the logic that only a four year old can have, he looked at me like I was silly (I’m often referred to as silly grandpa) and told me that there were no mosquitos there today! LOL! But truly it is the little things, like that mosquito bite, that can sometimes just push you too far.
Mosquito bites are bad! So are Lumbar Punctures! Emmett, we are praying for you today. Thinking about you today. Bounce back quickly and we’re looking forward to seeing what your next adventure will be. *N.B., Emmett has bounced back and is happy to be home.
Now to highlight some fantastic summer moments: Alice Lake Provincial Park is a campground that Emmett literally hasn’t stopped talking about since his camping experience there last June. This camp is a preferred one for this family because it has full mobile phone coverage, has electrical hookups, is just outside of Squamish so in a beautiful area that is close to Vancouver, and the park roads are paved while the main paths are wheelchair accessible. All are important – but mobile phone coverage, electricity, and near Vancouver are all essential “Just in Case” items. You see, when you have a medically fragile child everything that you do has a “just in case” clause. Once you’re relatively satisfied that the “just in case” scenarios are covered then you can go out and enjoy life. You build memories. You take some calculated risks to ensure that your little one is living life to the fullest. Building memories of camping, campfires, marshmallows, fishing, and canoeing that will last a lifetime. Even mosquitos add to these memories with new experiences. The bite on his shin which blistered has now turned into a scab. Emmett really dislikes this scab – it’s also the first scab he’s ever had. After all, he’s never fallen down – at least, not like that!
Alice Lake Provincial Park was a great trip. It was cool and wet – but fortunately most of the rain took place at night. A trip to the Sea to Sky Gondola, where his Aunt Katie and Uncle Josh got married three years ago in July, was very special for all of us. While Emmett doesn’t really remember the wedding (he was only one), he will certainly remember this trip!
You can click on any of the pics to get larger ones!
August 2019
August 8, 2019
Spinal Muscular Atrophy Awareness Month
Just recently one of Emmett’s parents noticed that Emmett was sleeping on his back, a position that they usually avoid as his drool cannot drain out by gravity. Wondering why the other had left him like this, they went in and rearranged him. You see, while Emmett has gained a lot of movement that he demonstrates in his daily activities, in his sleep or semi-sleep all you observe is a shape that doesn’t move. Or you hear “Other side. OTHER SIDE. OTHER SIDE!“, which can get quite loud as Emmett’s voice and lung power are gaining strength. Every couple of hours he needs to be moved to ensure that he has a change of position. Well, back to that slight frustration about positioning which quickly changed to wonder as they watched Emmett, in his sleep, roll himself back onto his back. In his sleep. Simply Wow! Way back in the blog we described the nervous system as being like the 401 highway – the largest highway in the world – with massive traffic travelling on it while SMA was a blockage that, with Type 1, shut the highway right down. In fact, SMA Type 1 would be like a giant sinkhole that swallowed up the entire highway and all alternate routes. Traffic literally stops moving. And until 2016 that is where the disease was left – traffic possibly at a standstill. Then came Spinraza, a drug that started traffic moving again. It wasn’t as good but if you’ve ever been stuck in a traffic jam for hours at a time then you know how good it is to see movement. And slowly there is more and more movement.
It was in the August of 2015 that Emmett was diagnosed with Spinal Muscular Atrophy, Type 1. Ironically, August is traditionally the month in which CureSMA Canada and CureSMA (USA) focus upon raising awareness of this genetic disease. It is recognized as the number one genetic disease that takes the lives of children under the age of two. This is a disease that is sometimes (and mistakenly) described as being a ‘Lou Gehrig’s disease’ (or Amyotrophic Lateral Sclerosis) for kids. Neither SMA nor ALS are contagious. There are obvious similarities – in both diseases the brain is unable to communicate with the muscles which causes the individual to lose the ability to walk, talk, eat, swallow and breathe. In the movie “The Theory of Everything” you see the dramatic changes that ALS imposed on the young and healthy Stephen Hawking as a university student. And the incredible health and mobility challenges that affected his adult life. There is no cure for ALS and 80% with this diagnosis die within five years. There is only limited treatment for ALS. This is where the similarities end. There is no specific cause of ALS; it can strike anyone although most often between ages 40 and 60. SMA has a definite genetic cause. SMA primarily affects children. There was no approved treatment for SMA until December 2016 when the US FDA approved it. Canada approved it in June 2017 but access is still limited with some provinces providing greater access than others.
We do not have a movie depicting Emmetts struggle although you can follow a significant history though this blog which began in 2015 shortly after his diagnosis. The cause of SMA is almost 100% when two carriers have a child – they have a 1 in 4 chance their child having SMA. While there was no medically approved treatment available when Emmett diagnosed, he was quickly involved in a world wide study of 124 babies with the genetic disease Spinal Muscular Atrophy,
Type 1. This drug, trademark name Spinraza and study name Nusinersen is an antisense oligonucleotide (that’s for the scientific types reading this). He was one of four precious little ones with SMA Type 1 involved in this Canadian study at BC Children’s Hospital. He is also the only one of the four who survived. At six and seven months of age Emmett had lost almost all ability to move. Prior to this drug, Emmett’s loss of ability may have slowed or stopped, but individuals with SMA did not improve. Period. Emmett could no longer travel in a normal car seat as the position would cause him to choke on his own saliva. He had to use a specially approved car bed for transportation; and in most activities he would need to be prone. Emmett was never able to sit up on his own nor could he sit up without being held up. He was never able to support his own head. By three months of age he had lost the ability to move his legs. At six months of age he had lost almost all arm movement. At nine months of age he was on a bi-pap machine to assist his breathing – this equipment pushes air into his lungs to expand them and then has a lower pressure that allows him to exhale (different from a cpap machine which is one constant pressure and for Emmett would make him work too hard to exhale).
This blog has said many times before that we knew Emmett was not on the placebo because we started seeing signs of improvement almost immediately. To say that this experimental drug stopped the regression of this disease is an understatement – it reversed it. Obviously, it is a long process and we do not know where this will lead. We do know that infants who have received this drug pre-symptomatically appear to be developing with few symptoms of SMA. Another new treatment is the apparently one time injection of Zolgensma (onasemnogene abeparvovec-xioi for those who need to know) that has already been approved in the USA – this treatment appears to offer even greater potential. However, back to Emmett and Spinraza. It has not been an easy haul for Emmett and his family. That may be partially the fault of the blog as we tend to highlight the forward steps that Emmett makes, but there have been health scares and hospital stays. Fortunately, the past year and a half have been so much better but a simple cold can still cause the entire household to go into a crisis prevention mode. Emmett’s lungs are stronger and so is his ability to clear his own saliva. Emmett is in the beginning stages of learning how to swallow again. He has been tube fed for four years so this is going to be a slow process about which we are all cautiously optimistic.
Emmett just spent several days attending Kid’s Camp at his church. He has been getting out more and interacting with other kids, including Sunday school almost every Sunday. It is easier in the summer as flu and cold season is not surrounding us. It doesn’t mean that caution is tossed to the wind; but it does mean that Emmett can explore and interact. Playing Duck, Duck, Goose – Emmett would roll around the circle calling duck or goose while I tapped the others on their head. When he called Goose, Emmett needed the kids to circle the group twice to his once in order to make it close to even. It worked! Emmett had a lot of fun playing this and games like “Red Light/Green Light or What Time is it Mr./Mrs. Wolf”. Obviously the other kids would look while Emmett was being suctioned or his feeding tube was connected/flushed/disconnected. Adults look too! Shortly Emmett will be heading off to SMA camp with his mom and dad. There they will have several days of interacting with other families that are also dealing with SMA. And Emmett, who was at this family camp for two days last year, is already looking forward to spending time with other kids in wheelchairs. Just like him. He is not, however, looking forward to the mosquito’s!
Emmett loves going fast! In his van down a hill. Going fast and high on his swing! Down hills in his power chair. Down hills on his pedal bike especially when Grandpa lets go so that he is on his own and everyone else is freaking out that this little boy is going to crash but fortunately he didn’t because his grandpa grabbed the rear controls in time to avert such a catastrophe which was never going to happen because everything was under control all along! Phew! But not just going fast physically, but thinking fast. In Sunday School we were watching a video with a group of kids ages 4 to 10 and, during one section with humour geared more for the adults observing, Emmett giggled. There wasn’t a sound from anyone else. His mom and dad having a conversation spelling words to avoid detection only to have Emmett join in the conversation with a response that indicated he was fully aware of what was being said. He rolls his eyes like a teenager – especially when grandpa suggests something that he considers unworthy of a response.
Back to our comparison to the super highway; Spinraza is slowly but surely allowing this little boy to regain strength and mobility that he has lost. We are realistic and understand that Emmett is always going to need support. We are also not giving up on miracles! Every roll and turn and lift is a miracle. Did we tell you how Emmett lifted his hand and held it out the van window! The look on his face was incredible. Emmett will finally be getting a kid’s walker/gait trainer. He just got a lift in his bedroom that will make it easier on mom and dad to move Emmett about. His second hand wheelchair has been very reliable this summer with the ‘MacGyver’ fixes but plans are underway for a new model. Bathroom renovations to be more accessible are still in the planning phase. And Wendell the cat got sick and had to go to the vet. He’s back home now and recuperating. All of this is just a fact of life when living with Spinal Muscular Atrophy. (Wendell and Emmett are best friends, so Wendell the cat being sick was pretty challenging!) Emmett doesn’t go anywhere without his suction machine. He doesn’t go anywhere far without his bi-pap. Sneeze and Emmett will tell you to sanitize. Even with these new, exotic and expensive drugs that are a miracle, Spinal Muscular Atrophy is a terribly challenging disease. August is Spinal Muscular Atrophy awareness month. Want to know more, just ask.
After watching the bike video, now go back with us to 2016 and watch a video clip from that era.
September 8, 2019
Time Flies!
Can you believe how swiftly summer has flown past! It’s now autumn! In fact, on the rare day that it isn’t raining or foggy here in the Fraser Valley, the distant mountains – which were almost bare – are now wonderfully covered with snow. The temperatures are falling. So many changes! Plus so much activity! Aunt Katie from Australia came to visit and smothered Emmett with kisses! Emmett was off to Camp Squeah near Hope for a Church family camp. He’s been out to the pumpkin patch where he selected a pumpkin – that he announced would have a Hippo carved into it. Just like last year. And just like last year, and the year before that, he proclaimed that he would be Woody the Cowboy for Hallowe’en. Of course, that meant a desperate search of local thrift shops and stores to find a costume that would fit. As this little boy has outgrown all of the Woody the Cowboy costumes that he owns. And that is a few. Perhaps we’ll have a Woody the Cowboy pumpkin too! Emmett was out at the Chilliwack Corn Maze where it was pouring rain. As a result, he wasn’t out in the maze itself. His power chair would have been so bogged down in the mud that it would have never gotten clean. He did, however, dress up as a Giraffe for this outing. This creature of habit is willing to change things up. In fact, he’s already agreed that next spring the family camping trip should be somewhere other than Alice Lake Provincial Park!
Time flying by also means that it has been almost four months since his last lumbar puncture and dose of Spinraza. His next one, which will be number 16, will be on October 30 at BC Children’s Hospital. And yes, Emmett is still planning on dressing up and heading out trick or treating on Hallowe’en. He wants to get some more shakers! Shakers, if you’re wondering, are Smarties. You may have forgotten that Emmett doesn’t eat anything by mouth, so Hallowe’en treats all get passed on to grandpa (if I’m lucky). Except for Smarties, which make a wonderful noise when shaken thus Emmett knows them as shakers! Of course, this is where we can let you know of a tiny step for all of us but a huge leap for Emmett. He is experimenting with swallowing just a millilitre or two of water. So on the 30th please remember Emmett. While playing with his dolls one of them recently went to the hospital for spinal muscular atrophy. Then Emmett gave his doll a dose of Spinraza. That hurt. Emmett knows this and, knowing that this is just a week away for himself, really isn’t looking forward to this hospital trip.
As Emmett matures and grows, he is getting stronger and stronger plus bigger and bigger. And heavier and heavier. As a result, some modifications are required. He has a lift now in his bedroom that his parents can use to transfer him from bed to wheelchair and whatever is necessary. This will soon be added to their main living room, where it will be easily accessed for most daily activities. As all of you who follow along on FaceBook know, there are plans to modify their bathroom to make it much more accessible. In fact, on FaceBook we had a somewhat spontaneous fundraiser and with your generosity over $5,000 was raised in one week for this very important project. As you can imagine, this is a huge financial gift that will cover about a third of the anticipated cost of this renovation project. Utilizing the existing space plans have been drafted that provide for a roll-in shower, maintaining the tub (used for light physio and water play), and obviously an accessible sink and toilet. Hopefully January is going to be the bathroom renovation month.
Emmett has another great new piece of adaptive equipment. A walker. This unit has a bicycle style seat that supports his weight while allowing his feet to touch the floor and his hands can power the wheels. So far it is more comfortable than a loaner one that he had tried out, but it’s certainly not his favourite device. We also got a light switch extender – a unit printed off on a 3 D printer – that Emmett can grasp when he is in his wheel chair. He doesn’t have the strength to use it yet, but it’s another goal to have and an opportunity that, without this extender, would have been well beyond reach. There are so many things that are simply overlooked by able bodies – yet are a huge obstacle for those with physical challenges.
On October 22nd individuals and families affected by Spinal Muscular Atrophy marched in Victoria at the parliament buildings. BC was an early adopter of Spinraza for individuals with SMA Type 1 but has still not approved this incredible drug for patients with Type 2 and Type 3. As Spinal Muscular Atrophy is a progressive disease, these individuals will really benefit from this drug that will stop and possible reverse the disease. Emmett has Type 1 but with this drug has improved to where he now presents as an individual with Type 2. The changes that this drug has on individuals is incredibly positive. Emmett meets all of the criteria that BC has for accessing Spinraza but this drug should be available to everyone.
According to Emmett, I am “Silly Grandpa”! No amount of coaxing and suggestions like Super Grandpa or Awesome Grandpa have caused him to deviate from this moniker. This little boy is stubborn – thus “Silly Grandpa” it is. Some could suggest that it is elder abuse! Teasing that borders on bullying! I, however, think that is it awesome. Fair play for a little boy who in the past I’ve called ‘Super Diaper Head Baby’ (don’t believe me, there are a few blog posts that even feature S.D.H.B. and I’m pretty sure that I’ve promised that there would be one more! Emmett!) and tease on a regular basis. Together we push the boundaries. We do stuff like swing higher on the swing than his mom believes is appropriate. Silly Grandpa. Go outside together and put him down on the grass (which he hates touching) so that we can look at the clouds together. Silly Grandpa. I let go of the three wheel bicycle handle while going downhill so that Emmett experiences freedom plus the exhilaration of knowing that Grandpa is running beside him so he is on his own and his grandma and mom are shouting in the background that I should stop immediately! Silly Grandpa!
Silly Grandpa is the truth. Emmett and I have a history of doing silly things. I pick him up off of the floor and bump his head on the ceiling! Or the door frame or wall as I’m carrying him around. ** IMPORTANT NOTE: very GENTLE bumps.** I roll him over on his gym mat and push him onto the floor. I carry him outside and we roll down the hill together. We make giant splashes in the bathtub and get water everywhere! I’ve bounced large, inflatable balls off of his head and face. We have gone up and down steep hills on his power chair. We’ve gone through culverts (big ones in parks!) together. We splash in puddles! Yes, I’m Silly Grandpa. Because, like everyone else who surrounds him, there is nothing better than hearing Emmett laugh. That is the best sound in the world.
December 2019
Merry Christmas
December 25, 2019
“Sure, Charlie Brown! I can tell you what Christmas is all about!” Linus van Pelt.
Look closely and you’ll see a Charlie Brown nativity scene!
At our Church Emmett was one of dozens of kids who answered questions about Christmas. Emmett was not in a talking mood and to several questions his response was “Ti Ti”. This is his nonsensical response with no meaning that he is fond of giving, usually with a smirk, and especially when he just doesn’t want to answer. Obviously, none of his ‘Ti Ti” responses made it to the video but his final answer did. The question was ‘what is the importance of Christmas’ and what Emmett shared with the world was, “Lots of presents!” And this is so true. Emmett does like presents! What little kid who is honest would not say presents. However, limiting the importance of Christmas to presents would be vastly underestimating this little boy.
You see, for Emmett Christmas is so much more. Much more than presents! After all, Emmett is a hard-core fanatic about Santa Claus. He adores Santa Claus. He talks about Santa Claus throughout the year! It could be a blistering hot summer evening and Emmett will look up into the night sky and exclaim, “Santa Claus!”. It isn’t that he is brainwashed into believing about Santa. Or that he is surrounded and overwhelmed from birth by Santa figurines. In fact, there are multiple Nativity Creche’s in his grandparents’ homes and in his own home. Emmett has his own Playmobil Nativity Creche. At his grandparents’ houses he very reverentially picks up and looks at the baby Jesus before gently replacing the baby in the manger back in the proper spot in the stable with Mary and Joseph. The Baby Jesus in his PlayMobil creche is far more active and, one could even say, miraculous as it often flies about the stable. Lest we forget, his daddy is a pastor so, of course, Emmett knows the nativity story of the Baby Jesus.
Yet still, it is Santa that Emmett searches for in the night sky. When Emmett had the opportunity to participate for a second time in the Santa Flight hosted by Canuck Place and Air Canada, there was no question about it. Emmett was going. It was great fun – after about 10 minutes of flying on a typical B.C. overcast day, Emmett asked his parents when they would start flying! Ha ha ha. Santa was on the plane, and that was awesome for Emmett the Santa searcher. He has, in fact, actually asked his mom if Santa is real. Put on the spot, Alisha kindly and carefully discussed and chatted with Emmett about the concept of Santa. Mommy and Daddy bought the presents. Grandparents bought the presents. Santa Claus lives in all of us through the gift of giving. But there is no Santa. It was clarified. There was now no question about the reality of this jolly fellow. The facts are the facts. In his 4 years and 10 months, Emmett has come face to face with many facts that most kids never have to realize. Facts like not being able to walk, not being able to swallow, not being able to – well, these facts go on and on. This is the harsh reality that Emmett lives daily. So when it comes to facts about Santa Claus, a fictional being that Emmett loves, then suddenly the truth and the facts are completely irrelevant – as even after this careful discussion he still absolutely believes in Santa Claus. He just wants to.
Emmett is focused on one word right now, constantly asking every adult’s favourite question, “Why?”, over and over until he is satisfied with the response. Just as large as his desire for factual information is his love of play. His imagination is incredible and the fictitious worlds that he creates in play are immense. The untold hours that we have spent searching in the 100 Acre Wood for Christopher Robin, whose voice we can hear (it’s actually Emmett pretending to be Christopher Robin) but C.R. himself remains elusive and unfound, are seriously endless. (Did you know that it is very challenging to find a Christopher Robin doll? But even if we did find one, that would spoil the game so we don’t want one.) In this case, he wants nothing to do with the facts. For the same reasons, Emmett wants Santa Claus – just not up close and personal. That is too much. From a distance only, please. Otherwise, he will simply look the other way so that Santa Claus cannot be seen.
It is Christmas, however, and we have to tell you that Emmett absolutely got some presents. Some would say he was spoiled with presents. I would say that in some cases, perhaps the term spoiled would be appropriate but here, with my grandson Emmett, I will become quite defensive, protective, and even angry should you say he’s spoiled. Bringing joy to a little boy who cannot walk isn’t spoiling. Giving laughter to a little boy who cannot reach out and grasp something due to his limited movement isn’t spoiling. Expanding the imagination of a little boy who is fed through a tube is not giving too much. You may disagree with me and that is fine. When you’ve had nearly 20 lumbar punctures like Emmett has, come and talk to me and maybe I’ll be more willing to listen to your perspective! Don’t get me wrong, Emmett has an amazing life and he loves it, but that doesn’t mean that it is not a challenge. This Christmas Emmett’s favourite present was a tea set because, even though he has never eaten food, he does love playing with food. His kitchen, however, was missing a tea set so until now he hasn’t been able to properly serve tea or hot chocolate. This gift has rectified that untenable situation. Presents also include a mini-Winnie the Pooh set (his third and by far the tiniest) that he already loves and will be great for car trips. The stuffed Woody the Cowboy, Buzz, Jessie and Bullseye are already in their place on Emmett’s bed. And among all of his other presents, which were plentiful, my favourite was a remote-controlled excavator. He says he really likes it. All of the adults wanted to have a turn with it. Hopefully it will provide lots of play for Emmett as he learns how to use the complex controller. And strengthens his fingers to push the buttons and switches.
As we close off this Christmas Day blog, we encourage you to look beyond the presents. Look to the presence of family and friends. Embrace that. Remember, maybe even in the voice of Linus during the Charlie Brown Christmas special, that good news that came 2000 years ago:
[Linus drops his security blanket on purpose]
Linus Van Pelt: “for behold, I bring unto you good tidings of great joy, which shall be to all people. For unto you is born this day in the City of David a Savior, which is Christ the Lord. And this shall be a sign unto you; Ye shall find the babe wrapped in swaddling clothes, lying in a manger.’ And suddenly there was with the angel a multitude of the heavenly host, praising God, and saying, ‘Glory to God in the highest, and on earth peace, good will toward men.’”
Linus Van Pelt: [Linus picks up his blanket and walks back towards Charlie Brown] That’s what Christmas is all about, Charlie Brown.
Christmas, that’s what it is all about.
Public Service Announcement: When you are out and about this Christmas and New Years, remember, if you’re sick with a cold then don’t go out. Stay home. Sure, you’ve been worse and want to be the life of the party. But your cold could be the death of the elderly person you see or the medically fragile individual that you cross paths with. So stay home. Keep that gift to yourself. Wash your hands. Use sanitizer. Stay home until you’re well and help keep others well too!
