Saturday, January 23, 2016
We’re All On A Journey!
I have been hearing some voices of concern about the lack of posts here on Emmett’s Journey. Please know that Emmett is doing fine. In fact, now that he is ten months old, his two bottom teeth have decided to make their appearance. Whoa, stop the presses. Just got a text that a third bottom tooth has just appeared! Apart from one quite irritable night and tons of drool, Emmett handled it easily. Which is a good thing because his two top teeth are apparently right below the gum line and ready to break free any day. I am so impressed that Alisha has put Emmett in his Jolly Jumper rigging and allowed him to play in it. The bathtub is still one of Emmett’s most active places. As much as the public swimming pool is an exciting place, his activity tends to be limited as his focus is upon the sights, sounds, and activity all around him. It is in the security of his calm and quiet bathtub where he twists and turns, moves all of his limbs, and even more startling, arches his back and neck as hard as he can. This, of course, means that if he is in his special neck flotation ring, that it will pop up and essentially go vertical. It keeps Emmett’s head well above water but has caused minor heart failure for Alisha, Josh, and Rhonda as they’ve been giving him baths and allowing him to play in the water! I think it’s actually pretty cool. I mean, everyone now has to be more cautious about Emmett’s placement, because he’s active. Due to his lack of movement up to now, if he was set on a couch or a table or a counter or virtually anywhere, we didn’t have to worry. Emmett didn’t move. Before. And honestly, he still isn’t moving a lot compared to other ten month old little ones – but his increasing activity means that everyone has to be more aware. Obviously, everyone has always been aware of his need for health safety. If you know anything about his mom and dad, grandparents, aunts and uncles – it’s an obsession. And now we’re branching out with safety!
Some of you picked up on my earlier comment where I said Emmett’s top two teeth are apparently right below the gum line. Pam and I are back in Ontario right now. We have been going non-stop. Late in 2015 we decided that we had to be closer to Emmett, Alisha, and Joshua; but that living in our RV in their driveway or right in their house (once it got freezing cold in the temperate Fraser Valley) was simply too close. We bought a strata (that’s a B.C. term, we Easterner’s would call it a condo), that’s a rancher/bungalow style townhouse. Easy access for Emmett to visit and within an easy ten minute drive for us. While finding the right place was a challenge, thanks to Allan Unrau at Landmark Realty, we got it. Then at New Year’s we came back to Ontario and found that the real challenge was boxing up 29 years of living. Oh my goodness! The transport company is coming this Wednesday and it has been a rush. With multiple trips to drop things off at a thrift store, several trips in our friend Doris’ truck to move larger items around, a few visits from friends who loaded up on car parts from Frank’s bizarre collection, and we are ‘almost’ packed today. Big thanks to Dawn Peace, a Re/Max Twin City realtor here in K-W, for her thoughtfulness and caring throughout the process of listing our home and helping us work through offers and such. It’s not black and white yet, but once a few conditions are satisfied that will happen.
So the blog has been somewhat quiet. That will change soon.
Emmett’s Favourite Toys – by Alisha
Sunday, January 10, 2016
Emmett is ten months old today!!!! (January 9, 2016) In honour of this special day, I (Alisha) have decided to write a post about a few of Emmett’s most favorite things. One of the (many) challenges of SMA is that Emmett is a really smart, intelligent baby and he wants to do the things all ‘normal’ babies do, but his mobility is very limited. We’ve had a hard time finding baby toys that can work for Emmett. For instance, he really loves music, but it is hard/nearly impossible to find a musical baby toy that Emmett is strong enough to press the buttons. Thankfully his Grandma Rhonda found one toy that plays songs and animal sounds just by touch (Emmett is so excited about it that we sometimes can even forget how annoying the sounds are!) On this blog we’ve already shown you some of his favorite things; his bath ring, his balloons, and his jolly jumper that really allow him to play and explore his environment.
Even before Emmett got his SMA diagnosis, we realized that his mobility was less than other babies and we started to make changes to his toys. For instance, he really loved to play with toys that dangled off of his baby play mat, but had trouble reaching up to them, so we just added a few extra plastic rings. When he was about 6 months Emmett started losing even more mobility in his arms so we would have to roll up blankets and prop his up arms so he could still swat at the toys.
Ever since Emmett was 5 months old, feathers have been one of his favorite toys. They are colourful, light, easy to wave around, and they tickle! He also has some weird mini pool noodles things that his Grandma Pamela found and that fit perfectly into his hand. He enjoys hitting his Dad in the head with them. We were even giving him Kleenex, plastic and tissue paper to play with because of how light and easy it was for him to hold on too, but had to stop that once he gained back the ability to put things in his mouth. (For those who have been following Emmett’s Journey, you’ll know just how huge a deal it is that he can reach his mouth again!!!!!!!!). Now we also let him play with cheerios so that he can learn to coordinate his thumb and index finger by picking them up.
Recently Emmett’s discovered that the lids to his bottles fit into his little palm perfectly and make some really cool noises when hit on the floor, you can watch the little movie clip to see how much fun Emmett has with them! We are so happy that we have still been seeing some small improvements in his movement and strength – the other day he rolled his head while he was on tummy time – we haven’t seen him do this since August! GO EMMETT!!!!!!!!!
Emmett loves flashing lights and whirring, spinning toys. Sounds and touch are awesome. He is a little boy who loves going for stroller and wagon rides. Bedtime is one or two stuff animals and his amazing turtle music light. Happy tenth month birthday, Emmett. We love you and are so proud of you!
Sunday, February 07, 2016
We All Cry Together!
Three of Emmett’s friends have left this world in the last month and a half. We are crying for these little ones, their parents, families, and friends.
Emma
February 2, 2015 to December 25, 2015
Addison
February 2, 2015 to February 3, 2016
Atticus
November 25, 2015 to February 6, 2016
The twins – Emma and Addison plus Atticus – are Emmett’s soulmates. Together they shared the diagnosis of the terrifying genetic disease – Spinal Muscular Atrophy, Type 1. They and their parents were occasionally at BC Children’s Hospital at the same time as Emmett, Alisha, and Josh – sharing the heart aches, the highs, and the lows. Sharing together on FaceBook the struggles to stay awake through the day after nights of little or no sleep. Sharing ideas for toys and making plans for play dates. Parents separated by distance yet with a connection created by this disease. Talking about the joys of parenting medically fragile children. Knowing at times the despair of parenting medically fragile children. Parents struggling with a diagnosis that turned their world upside down and inside out. Parents grasping at the possibility that an experimental drug trial offered hope. Yet knowing that not all babies in the trial would get the real drug. Or even if this so called miracle drug would provide the miracle that we all want! Parents struggling to make sense of a world where beautiful babies can be born with a disease that not only takes their ability to move away, it takes their breath away.
Right now, we are all crying. It is not fair. These three angels have left us far too soon.
Tuesday, February 09, 2016
We are celebrating today because this is Emmett’s 11th month birthday. What a joy to spend time with this precious little boy. Emmett is happy and loving being the centre of attention … Well, actually, he was somewhat irritated and aloof when I was just with him. Not interested in anything that was going on around him and certainly not ready to give anyone one of his million dollar smiles. He’s healthy – for a baby with SMA, Type 1! He constantly surprises us. Today, when Alisha asked him to blow bubbles, he did! Without hesitation. When Josh asked what sound a dog makes, Emmett just stared at him. However, earlier today he apparently made the woof woof sound all on his own. I cannot verify that, but I can verify that I saw him blow bubbles! This is a day when we forget about all of the concerns of the world and simply spend time enjoying the miracle of life. And blowing raspberries together! We have all said it before, that when you’re with Emmett and he smiles then the only thing that is possible to do is to smile along with him! And today, we are smiling. Even if Emmett is a bit on the grumpy side.
If you are wondering, the answer is no. Emmett absolutely did not get another helium balloon for this month birthday. I will fix that very soon – maybe with two balloons! Pam and I are keeping busy with getting settled in B.C. and I simply didn’t get out to pick one up. It is unlikely that I will go bankrupt buying helium balloons, but according to some people in our family that is a possibility. Emmett, with his amazing eyes and expressions, has already told me that when he’s older he would like to have enough helium balloons to allow him to float around the house. I said no, that he’d have to wait until he is older to do something risky like that. But that’s likely doable, as long as his mom and grandma’s don’t find out. His dad and grandpa Willms are possibly going to support this endeavour.
Do you think that for his 12 month birthday – his first year – that he would be old enough then?
Emmett’s Occupational Therapist designed and built an ‘arm’ crane for Emmett that is hinged and extends. This allows Emmett to move his arm more extensively. With the weight held by the sling, he can concentrate on movement rather than on picking his arm up. It is great to see the look of concentration but also joy as he explores the unknown. In the pic where he’s sleeping, Emmett is buckled into his car bed. It is, I’m sure, the only way to travel! The last few pics, just a few more opportunities for this little boy’s eyes to cause you to catch your breath! Emmett now wears his bi-pap machine for naps in addition to overnight sleeps. It isn’t his favourite apparatus, but he does love story time and that sort of takes away the irritation of the bi-pap mask.
Stay tuned for an upcoming blog on Emmett swimming – we just have to get some photos that are discreet and won’t embarass him when he’s older!
“Mommy, why can’t I float around the house with helium balloons? Grandpa says it will be safe!”, is what Emmett is thinking!
Bath Time Workout!
Thursday, February 11, 2016
If a picture is worth a thousand words, how much is a video worth!
At four months, even if he was in water Emmett could only make tiny movements with his legs. So imperceptible sometime that if you weren’t touching his legs you didn’t see the muscle contracting. He could manage tiny movement of his ankles. Between five and six months he could not do much more than raise and lower his arms – at one point it was only his right arm. His ability to slightly turn his head was also lost. It was a very challenging time. More challenging than we have ever shared on this blog. However, miracles are happening and look at this little fish! He loves being in water. Our little Nemo! Keep praying for Emmett, other little ones with SMA, and the researchers in their search for a cure.
Here’s a flashback to when Emmett was 6 months old. His 6 month birthday balloon.
Want to learn more about how you can help cure SMA. It takes funds to support the research. But even more, your kind words and thoughtful emails help support the brave families who are involved in the unknown factors of clinical trials. They have hope – but your support offers even more hope! Watch this video. Note: The children in this video may have either SMA Type 1 or Type 2 or Type 3.
Emmett has Type 1.
Emma, Addison, and Atticus each had SMA Type 1.
The following video is produced by Families of SMA Canada. Their website is http://www.curesma.ca/
Tuesday, March 08, 2016
Emmett is ONE!
Every child is precious and every first birthday party is adorable. All of Emmett’s family, however, are significantly biased because we feel blessed to celebrate this first year of life with this little boy. The most special little boy in the world! (Again, we’re biased.) We thank God that Emmett is here with us and we pray for many more birthdays to come. We are thrilled that Emmett is doing well and we anxiously wait for the next signs of progress. Just yesterday Emmett, in the bathtub with his neck ring, rolled over. Literally rolled over from his back onto his stomach and looked like he was ready to do the doggie paddle!
Emmett got to enjoy the texture and feel of his cake but, as he’s still fed by a gastric tube and takes nothing by mouth, the piece on his nose was as close as it got to his mouth. It was fun watching him play with his food. Literally something that this little boy just doesn’t normally get to do! In fact, he hardly ever needs to wear a bib!
It isn’t because he doesn’t have choppers – as you can see here his teeth are present. Two on the top and three on the bottom. They have caused him some restless nights and he drools like crazy! We have also succeeded in spoiling him as Emmett is quite willing to put up a fuss if he doesn’t get something that he wants. Setting him in his crib while grabbing something from his room is likely to create a scene because if it is not his nap-time then don’t even think of using his crib! Or, if he wants more attention than he’s getting Emmett isn’t above using a ‘fake’ cough to cause a crowd around himself! Obviously, he has learned these attention seeking behaviours from his grandpa’s!
Emmett’s first birthday was an immediate family affair. Emmett and his parents, the Willms’ family (except Jennie and Ben in Alberta) and the Ewald family. It’s too extreme to call it reverse isolation, but Emmett is absolutely still being very protected from bugs, germs, and viruses. There are selected outings – like a hike to Cascade Falls that is just a short ride from Abbotsford. And not too many tourists at this time of year. Hint of a cold – put on a facemask and don’t get close. Presents were in abundance – race car floor rug, jack-in-the-box, clothes, and more to celebrate this little man’s first year of life.
One of Emmett’s favourite iPad programs is a Xylophone that reacts when he touches the screen.
His face lit up when one of his presents was a real one! Of course, it’s harder for him to play but that is something to work on in the upcoming months. Emmett loves a crowd and is fascinated by the colours, the sounds, and the activity that engulfs him when he is surrounded by others. We are looking forward to spring and summer when we hope he can be out and about more often.
Of course, Emmett’s birthday wouldn’t be complete without balloons. And there were a lot of them!
Happy Birthday, Emmett. You are God’s precious gift and we love you. Thank you for being such an amazing little boy with such a huge smile!
To all of Emmett’s online friends – thank you for your support, prayers, love and thoughtfulness. We can never tell you how much your support helps during the challenging times. Thank you and God Bless you!
Wednesday, March 09, 2016
A first birthday is exciting, but why are we making such a big deal about this here on Emmett’s Journey? Well, last August one of Emmett’s specialists gave him only five to six months to live. His lead specialist said that his lifespan had the potential to be much longer (well, much longer relative to six months), but certainly five to six months was a timeline that shook us all to the core. The silence was deafening. That is why we have celebrated Emmett’s birthday each and every month since.
September, October, November, December, January, February, March …
When I was a kid in grade school math was a huge challenge to me but I struggled, persevered, and worked hard. As a result my addition is pretty solid and I know that Emmett has passed that first timeline. Unfortunately, it is not because the specialist was wrong; they were probably very right. But, through the miracle of science, the power of prayer, and the love of his parents – Emmett is stronger now than he was at five months of age.
Spinal Muscular Atrophy, Type One is a challenging and terrifying disease. This SMA can strike at any age IF you have the right (I should probably say wrong) genetics. Both parents have to be carriers, and if they are then there is a 1:4 chance that their children will have SMA and a 2:4 chance that they will be SMA carriers but won’t actually have the disease themselves. Scarey odds! If the disease presents itself before the child is six months old, then it is identified as Type 1. Emmett displayed symptoms before his diagnosis at five months but we didn’t know what they were. Neither did the professionals during his check ups. It wasn’t their fault. The highly trained nurses in the baby clinics and the family practice GP’s saw a little boy who was demonstrated delayed development. SMA Type 1 would fall into a category of rare diseases that many medical practitioners may never see in their careers. Parents see their little one 24/7 and must be vigilant and push. And we’ve said before, when their GP did observe what concerned Alisha and Josh, Emmett was in to a paediatrician in less than 24 hours.
The one question that we have heard most often is “What is SMA, Type 1?”. SMA being Spinal Muscular Atrophy. Here is another of my attempts to try to describe it. With almost no scientific phrases.
Picture your local multi-lane freeway. Traffic flowing perfectly both ways. Everything running like clockwork. No accidents. No flat tires. No construction (yes, that is a miracle in itself). Then see that some challenges intrude. Way ahead someone did get a flat tire or they ran out of gas resulting in a slight slow down of traffic. As you get closer to that incident, someone crashes into the rear end of another in the middle lanes. As you can imagine, traffic is slowed right down and comes to a virtual stop. That’s irritating, but you know that in an hour or two you’ll be back on your way. Maybe there’s a bridge that is the only way across the country and it breaks, falling up, so that this main thoroughfare is out of commission or down to one lane for an unknown timeline. Or picture that there was a massive earthquake that has taken out literally kilometres of this highway. Obviously, the entire country is in chaos as a result of this natural disaster. Your car cannot ever traverse this expanse as the massive highway has literally disappeared. Since the government has no money to fix it (doesn’t matter which country, as they’re probably all broke), the only way to get from A to B now is to hike on foot or to take a donkey. But it is very hard to find a donkey, so you’re left on foot. Of the hundreds of people who try to walk the distance, only a few can get through because it’s tough. Climbing cliffs. Jumping crevasses (yes, there’s ice everywhere). Crossing expanses of rushing water that has carried a few of the hikers off to the unknown. Very few of the hikers make it to their destination. Most just stop. The economy in this devastated area shuts down – completely. Until the engineers and construction crews can open up a new route nothing moves – and everyone hopes and prays for that a new route is opened.
This is similar to SMA, Type 1. The spinal column is the highway and it suffers a major blow. It is no longer healthy and the messages simply cannot get through. As a result the muscles begin to shut down.
The light at the end of this tunnel (maybe my analogy should have been a train tunnel that runs through a mountain) is the pharmaceutical trial that Emmett was allowed to participate in, beginning last fall. This is the scientists developing a drug that revitalizes the spinal column so that messages from the brain can get through. If this was our highway that had been devastated by the earthquake, the first step would be to open up some kind of walking path. Maybe with ropes across the crevasses and rushing rivers – if you can go hand over hand on the rope you can make it. Tough and very slow, but possible for some. Then the crew sets up a swinging rope bridge which enables many more to cross. With time, sturdier and more expansive paths are created. This experimental drug in this trial is like this.
Officially we do not know if Emmett is getting the drug or a placebo. We celebrate every day knowing that Emmett is still with us. The messages from his brain are getting to his muscles. This is obvious as he sits in a baby seat and, with no side support but with support behind his head, is able to hold his head up. Something he couldn’t do just a little while ago; he needed support padding on both sides of his head to stay upright. We laugh and cry at the same time as we watch him in the bathtub or pool as he rolls from his back to his front over and over. Or as he moves himself around the pool. Slowly, but he’s doing it! Limited – but it is happening. Maybe it is a swinging rope bridge and a small path right now, but we pray and trust God for a miracle. We pray that the incredible team of miracle workers behind this drug trial make even greater break throughs. We laugh and cry and love every minute that we get to be with Emmett!
Thursday, March 31, 2016
It’s SPRING TIME!
Emmett is already for spring. He’s looking forward to lounging around in the backyard and spending time gardening and getting his hands and feet dirty. He is still not too impressed with bright sunlight so sunglasses go a long way towards making him feel more comfortable in the great outdoors! Of course, once in a while Emmett will show off some of his teeth – two on the top and four on the bottom now. This little boy looks like quite the man with his baseball hat, glasses, and two of his teeth showing.
Like any little boy or girl in spring, Emmett’s thoughts turn to … his wheels! That’s right. Emmett got a new set of wheels. His stroller and wagon are still in regular use but Emmett’s Occupational Therapist has been working with some University Engineering students and have this amazing electric cart/scooter that Emmett can move about with. Right now he’s only using the start and stop button, but eventually it has the potential of forward, reverse, and turn with three buttons that he can activate on his dashboard. To keep things simple, right now only one button is placed on his dashboard. It’s so cute!
Obviously, all of us were very excited to see Emmett mobile. The first two videos were the first time that the four grandparents saw Emmett in action using it. Lots of laughter taking place. Emmett also has some wrist braces to help him extend and straighten his wrists. This little boy takes everything in stride and doesn’t fuss at all with these extras. I guess he knows it’s helping him out!
Tuesday, April 05, 2016
Today, Wednesday, Emmett will be at BC Children’s Hospital for his fifth session in the pharmaceutical trial. Please, your prayers and warm wishes are needed by Emmett, Alisha, and Josh. This is very challenging for all three of them.
Emmett will be going through the trial process and the real drug is administered through a lumbar puncture. I’m cringing just typing that. He will be in the ICU all day. Alisha and Josh will also be at BC Children’s hospital all day. There will be many tests, assessments, and specialists to see and they need to see and hear everything. Except, of course, the test procedure during which they aren’t allowed to be present.
The staff at BC Children’s Hospital are amazing but that doesn’t take away the challenges that they all face. It’s been four months since his last session and Emmett is much more expressive now. If he doesn’t like something, he can readily express himself. And he doesn’t like this procedure. He does, however, love seeing Dr. Selby who is his neurologist. And he enjoys all of the attention. If he is unsure of someone he will act shy and turn his head away, but that does not usually last long.
He spent last night at Canuck Place, a beautiful children’s hospital. Last night he spent almost an hour in the big bathtub there. He was turning 360 degrees, making big waves, and just having fun and wearing himself out. He will be back there tonight. He loves the attention and surroundings there. It is the perfect place for him.
Thank you for reading this blog. Thank you for praying for Emmett plus his mom and dad. Thank you for your concern and warm wishes. On a day like today, the Willms’ family really need it.
oh yes, Emmett will be getting another balloon! Count on it.
Emmett is One Year and One Month Old today!
Saturday, April 9, 2016
There was not a new balloon for Emmett today (Saturday, April 9, 2016), since he just got his Tonka Truck balloon a couple of days ago (Notice this obvious sign of restraint on my part!). Even so, we are celebrating that this is his one year and one month birthday. Emmett did get lots of hugs and kisses from his mommy and daddy. And in the midst of his babbling we are certain that we heard ‘momma’ more than once. Big steps for this little boy.
This next image is a picture that you will have to use your imagination to create, because I don’t have a pic to go with it. In this image you will see Emmett smiling, and with little paper medication cups on his feet. He knows that they’re there and he’s moving his feet about. Not necessarily to knock them off, but rather to amuse the nurses and staff at Canuck Place who put them on his feet. Emmett, in his glory at that moment as he was surrounded by four or five staff (they had a down moment in their hectic pace), was loving being the centre of attention. And he absolutely was showing off!
On Wednesday, just a couple of hours after his procedure, Alisha commented that she believes her son to be an extrovert. Josh agreed. They both pointed out how he simply lights up with both focus, smiles, and attention when someone comes into the room – or in this case the area around his hospital bed. Alisha has also observed that he loves the ICU at BC Children’s Hospital as he gets so much attention there. Even though the procedure is far from comfortable – evidenced as his heart rate is literally a mile a minute – within a couple of hours he is ready to share smiles with his visitors. Dr. Selby is, of course, his favourite guest and she was at his bedside a few times during his stay. As his neurologist she is key to his care and her enthusiasm towards the movement he demonstrates is hard to conceal. In this photo, Emmett is preparing to break out into a grin and it is literally just two hours after his procedure. From the grip he has on his plastic sock monkey, it’s obvious that it was a winning choice! The tiny foam cylinder sticks have been a long time favourite. As his grip on the foam sticks continues to improve, the long awaited episode “The Return of Super Diaper Head Baby” (play the Star Wars theme music in your head) is getting closer and closer to this blog!
Emmett’s dietician has put him onto a new formula. To this point he’s been on breast milk and baby formula, but this new stuff has chicken and veggies included. More fibre! Food that sounds real! Exciting times! (I think I will let others change diapers for the next little while!)
Emmett is absolutely enjoying the lovely spring weather. And spring at Canuck Place is absolutely wonderful. This beautiful Vancouver property is absolutely majestic looking and is wonderfully setup, both inside and outside, to meet the needs of the children who are patients there. And their parents, also. A huge thanks to the amazing staff at Canuck Place. A giant hug to the visionaries who developed this oasis of calm and quiet for medically fragile children and their parents. And thanks to all who make donations to keep this facility running smoothly.
Now some pics to enjoy!
Mother’s Day – Full of Emotions
Sunday, May 08, 2016
It is Mother’s Day. As I am a Dad and a Grandpa and not a mother, I am probably not well suited for writing about Mother’s Day. But I’m not so much writing about Mother’s Day as writing on Mother’s Day. Plus, I will do what I almost always do. I will write about Emmett. This is Emmett’s second Mother’s Day and it is so special. There have been so many highlights and so many challenges in the year since this day last appeared on our calendars. A day to celebrate so many things. With Emmett in our lives, it is even more precious.
It is almost impossible to explain what it is like to live in crisis mode for months on end. Unless you meet someone else who is also in crisis mode – and then no words are necessary. When the crisis is centred around your baby, then Mother’s Day becomes even more special. And even more challenging. It causes you to wonder about the future. To look back at the past. To think about so many things.
This morning some of what I’m thinking about as a Grandpa of an almost 14 (that big day is tomorrow) month old little boy, who at 5 months of age was diagnosed with a terminal genetic disease by multiple specialists and a terribly short lifespan was offered, is how great it is to have us with him. But I also think of some of the well-meaning but misguided offers of comfort that we have heard. Things like – the doctor’s just told you that to scare you. Well … yes, I guess that’s right. They sure did scare us!
Or how this will make us stronger.
Hmmm. Do we really need to be stronger? Is this the only way to be stronger? We want Emmett to be stronger; I don’t need strength but he does. Can you be stronger with a broken heart. We will be different and there is no question about that. I am not sure about the stronger part. Being different can be good. And a broken heart can be mended with a cuddle and smile from Emmett!
Of course, there is this standard phrase – This will allow us to understand and help others. Better. Of course. Dealing with a severe illness does help anyone understand the depths of the challenges that another may be facing. Oh my goodness, this is a tough way to learn understanding and empathy.
One thing we know for certain on this Mother’s Day is that we love Emmett. I see that every day in Alisha and Josh’s conversations, texts, and phone calls. I see that in our extended family and in the Willm’s extended family. That we will do anything we can to support Emmett and to brighten his day is undeniable. His smile, and I am quite positive of this, can move the entire world! His eyes will not only captivate you but they will pierce your heart. And when you see movement where before there was no movement, you will marvel at science and what man has accomplished. You will believe in God. And you will realize that the doctor’s were not trying to scare anyone – they were simply doing their best to prepare a family for the reality of Spinal Muscular Atrophy, Type 1. The number 1 genetic cause of death in infants.
Our hearts are broken on this Mother’s Day for our friends who have lost their babies to this disease. Or to other causes. We do not understand why this had to happen to them anymore than we can understand why it happened to Emmett. We do see that Emmett is showing progress during this experimental drug trial. And we know that other babies did not have that benefit and God has taken them back to be with Him in heaven. The real thing or the placebo – that is painful for us. And we don’t even begin to understand how painful it is for them.
A disease that kills off the nerves in the spinal column. Yes. We are scared. Yet at the very same time we have hope that fills our eyes with tears. On this Mother’s Day we can tell you that Emmett has demonstrated some superhero moves! This little boy has begun lifting his head up. Yes, he has started LIFTING HIS HEAD! Then just a few days ago, during a nap, Emmett rolled from his side onto his back. This is beyond huge! Emmett may move his arms while sleeping, but he is always in the exact same position when you check him. He does not move. He did not move. UNTIL NOW! Oh how much we want to know what will be next.So, as a Grandpa on this Mother’s Day, my heart goes out to all Mothers! But especially to the mothers who are close to me. And especially today, to my daughter Alisha, a mother whose love and devotion for her little boy makes me so proud. I thank God for her and for her husband and soulmate, Josh, who goes above and beyond to be with Emmett and Alisha. Bless you both.
Wednesday, May 11, 2016
Emmett’s 14th month birthday flew by and, on May 9, we missed getting a post up about it. Emmett missed out on much of his day also, because he was under the weather. He had a busy weekend with some trips to see family and, absolutely adorable photos here, an opportunity to play with some kittens. It could have been all of this activity. Maybe it was that he’s preparing to add a 7th tooth to the mix. Or maybe just a spring cold, but Emmett’s chest was quite congested and he had to be suctioned frequently. So much so that it was very concerning to Alisha and Josh.
A call to the paediatrician resulted in direction to go right to the ER to get checked out. After the typical ER wait – that was atypical as they had the stroller loaded down with his suction machine, his oximeter, his bi-pap machine, and his feeding pump – they saw the paediatrician on call. Blood work was ordered and a call to Emmett’s specialist at BC Children’s Hospital took place. It was decided that an overnight stay would be appropriate but this added to the challenge of the day. You see, no one at the local hospital was trained on the Bi-Pap machine and while in the hospital Alisha and Josh weren’t allowed to set it up. In fact, if it required adjusted settings, that was beyond the parents’ knowledge.
Well, after a bit of a wait for a transfer to Vancouver, Alisha and Emmett were taken to the airport and flown to Vancouver. It’s just too bad that Emmett was asleep for the entire flight. His first plane ride, in an ambulance plane no less, and he missed the entire thing. Emmett is still in the hospital but he’s doing well. They’ve run a series of tests on him and things are looking good. It was likely a virus, but he is past the worst of it and we are hoping that he is home soon. Maybe still tonight; probably tomorrow. Thank you to all of the medical professionals for their very good care!
Stay strong, little boy! We will see you soon!
Edit: Emmett was home about 8 p.m. Pacific Time
The Many Expressions of Emmett!
Tuesday, May 31, 2016
Emmett got a fantastic new chair. This custom made chair offers him support while providing a wide range of reclining positions for him to enjoy. Waving is one of the latest moves that we’ve been seeing – with Emmett usually waving with both hands at the same time!
Emmett enjoys laughing. He has an awesome fake laugh which you’ll hear shortly.
Expression: Okay, this is pretty cool. Why can’t I take it home?
Emmett recently got to try out a properly sized wheelchair. Alisha and Josh said that it was simply incredible to watch him manipulate the joy stick and move himself about. As he is going to be growing so quickly over the next few months/years, we are hoping that he is able to get a loaner wheel chair to start off. Making long range plans is exciting. And challenging.
Emmett loves the freedom that the pool offers and he thinks his bathing suit is quite lovely. However, no one enjoys the shock of a first dip into cold water.
Emmett is doing pretty good. He is almost over the cold that he had at the beginning of the month. He does, unfortunately, now have an ear infection which has made him rather irritable. This little cutie is getting bigger and bigger – he’s quite a handful to carry around!
June 2016
I Cannot Believe It!
Thursday, June 02, 2016
Literally, I cannot believe it! Emmett has never been able to support his head and yet, over the past month, we have seen him demonstrating neck muscle strength that we have never seen before. From tummy time as a three month old right past his first birthday. Then at the end of April, while reclining on his mom’s chest, he lifted up his head to look at her. And now what you see in this video clip, taken on May 31st.
That’s just incredible.
Worried that Emmett hasn’t had a new balloon for a bit? That’s because we haven’t shared this awesome sea turtle balloon that Emmett has been playing with for the past couple of weeks. It’s pretty cool!
Emmett still loves his Jolly Jumper! And his posture is so much better than a few months ago.
Emmett’s Favourite Things
“Balloons!” Some balloons last forever while others barely at all. His almost new sea turtle balloon is already losing a ton of altitude.
“Bubbles!” Emmett has a powered bubble machine and loves getting splattered by bubbles – and especially loves if they land intact near enough that he can swat them!
Mommy and Daddy, not so many kisses! “Emmett doesn’t like too many cuddles or kisses.” He’s a big boy now.
“Poking at bugs. Playing with branches.”
“Being the centre of attention!” It’s tough having a medically fragile little one. More than anything Emmett loves a crowd and, especially, being right in the centre of that crowd. Recently Emmett was at Mill Lake Park where the Abbotsford Run For Water took place. The crowds, the noise, the colours, and the atmosphere was so much fun for Emmett. A huge thanks to everyone who interacted with Emmett but respectfully gave him necessary space!
“Dropping toys on the floor over and over and over again. It’s a great game to watch mommy or daddy have to pick it up again and again!”
His favourite song is now “If you’re happy and you know it …” Then insert things that he can do (wave hands, laugh out loud, wiggle fingers, blow bubbles, make a monkey sound, …). Emmett loves interacting.
“Swinging in the backyard hammock” which, Alisha, is really a swing and not a hammock.
“Backyard pool time. At first it was not one of Emmett’s favourite things as it was cooler than the bathtub or the rec centre pool – but it’s so much fun that he adjusted quickly!”
Wednesday, June 08, 2016
June 9th – Emmett is 15 months old!
Alisha’s Reflections Of The Past Month
“Ohhhhh, he just gave me a kiss on my cheek for the first time!!! You can put that in too.” Alisha exclaimed, as we were discussing this blog post just as Emmett, who so far has really only done air kisses, planted one on her cheek.
The moments with Emmett are so exciting. You never know what might be next. Anything in quotes in this entry will be Alisha’s words and thoughts – probably typed by her also. There are the typical youngster moments like Emmett filling his diapers and just picking him up means that you will be covered with poop. Then there are the moments when you just want to be like a normal person and take your baby out to mingle with other parents and youngsters – but … the risk of an infection is simply too great so when you do go out it is with a laminated sign attached to the stroller asking people not to touch. Because people just touch babies and toddlers. There are moments like a plane ride to BC Children’s Hospital – an exciting moment that no one wants to experience. And there are moments that just seem to take forever and that no one else understands. There are moments like watching Emmett intently focus upon a bug that is crawling on the grass near him. Seeing him move himself about the pool with so much more activity versus when he is on land. Listening to him laughing in perfect timing to a comment that he’s just heard. Seeing him wide awake one moment and then peacefully asleep mere moments later – without any fuss as his bi-pap apparatus goes on. Seeing his techni-colour smile when someone he knows enters the room! And then there is a first kiss and that is just absolutely overwhelming. That is exciting.
Sometimes boys just want to cry!
May 9-June 8 Memories:
“Your Great Aunt Debbie and cousins Julia and Jason came to visit. They were here over Mother’s Day and you had a busy day! We had breakfast with Grandma and Aunt Katie, went for lunch at Grandma Rhonda’s, and then had supper again at Grandma Pam’s with the Dippels.”
“Unfortunately you weren’t feeling well (This was early in May) and you came down with your very first cold ever. That night you had a low fever, two vomits, a high heart rate, and a very restless night. In the morning you had trouble clearing your throat after suctioning so I called the doctor, who suggested we go to the ER. There we spent hours waiting with you, while you were hooked up to all of your machines. When you were admitted they realized that they could not have a patient with Bi-pap – so had to transfer you to Vancouver.”
“It was already 8 p.m. by this time and you were so tired. An ambulance team came to pick you up but they were not a paediatric team so they refused to take you. Then the infant transport team had to come get you – in an airplane! Your first airplane ride, but you slept all the way through it.”
“In ICU at Vancouver you already were doing better, but they kept you for two nights to make sure. They confirmed that you had a cold. We are so thankful that you are not afraid of the hospital and that you like all of the staff there! Dr. Selby thought it might take you about 4-6 weeks to completely recover, but we are thankful that it only took about 3! You had to spend a lot more time on bi-pap.”
“Grandpa Frank was spending two weeks in Ontario while you got sick, but was very happy to see you when you got home!”
“May 13th was a milestone. It was the last time getting mommy’s milk through your g-tube. Now you’re completely on your paediatric formula.”
“You got a custom made seat.” It has a wood base that allows you to have a variety of reclining positions. The padding and covering were custom fit for you. They took measurements and then made and upholstered it in a van parked in our driveway. You love sitting in this chair. You want to be upright like everyone else, but we have to be cautious as you get congested. “But we’ve gone for several days over the last two weeks without the need for suction assistance!”
“Last week you got your first ear ache. You were miserable.”
“You had your first eye doctor and your first dentist appointment last week. Your eyes look great. Your teeth are pretty cool but you don’t have as many as other fifteen month olds. Is it because you don’t chew?”
Your dad had his 28th birthday and that was exciting. Soon you will be saying da-da. You’ve been saying momma for a couple of months now.
In the backyard reclining in the swing, with one foot dragging through the grass! Fun sensation.
“On Tuesday you had a great clinic day at BC Childrens Hospital. Your specialists (neurologist, respirologist, orthopedist, nurses, and therapists) were all very impressed.” You showed them how you can clear your own throat – a big step. You still need help, but there are times when all by yourself you clear your own throat. And there were many other things you showed them. Of course, Emmett does have wrist braces to stretch his hands, as they’re turning in.
This month had many milestones!
“Got to try out a power chair and drive it around the facility!” We know some of you saw the picture and thought it was Emmett’s chair, but it is not. Soon we hope he has something like that.
“Emmett can laugh on command!” It is so funny and his timing is also amazing. It is like he already has a sarcastic sense of humour!
“Emmett can YELL on request!” This is pretty cool.
“He can blow kisses. Make squishy faces. He loves the song Head and Shoulders. He knows and will touch eyes, ears, mouth, nose, finger and toes. And knees. He knows elbow and tongue too.” Of course, he knows tongue. And since he has a sarcastic sense of humour, I’m sure he’ll be putting this new found knowledge to use soon.
Emmett can hold his elbow up off of the ground (while lying on the floor) for long periods of time.
“Emmett balanced his head – holding it upright – for three minutes. He’s learning to better balance and support his head every day.” Believe me when I type that this caused more than a few tears. Holding up his head is a literal miracle.
“His grip is getting strong and he can hold onto his toys (when he chooses too) when they are moved around.”
“He can sit in his stroller or chair for longer and longer periods of time without getting choked up.” Of course, we tend to get choked up watching this!
“Emmett only uses his soother at night or maybe during a nap. He doesn’t want it while he’s awake!”
A Great Birthday Surprise
Thursday, June 09, 2016
A huge thank you to the Getty Owl Foundation. Emmett’s name was submitted to an online give-away specifically for families dealing with Spinal Muscular Atrophy. The Getty Owl Foundation was started by parents of a little girl who has SMA – what a fantastic outreach and global service they are providing.
They realize that families with children with SMA are tied to their medical equipment – effectively tethering them to their power source. Alisha and Josh were certainly not sure what their summer would look like. If you know them, you know that hiking and camping in the B.C. mountains is a big part of their life. They knew that a little one would change that, but with Emmett’s complex medical equipment it just couldn’t happen apart from very short excursions.
Then they saw this give away for a back-up power system to be able to greatly increase the tether. And they are one of six lucky families to receive a battery/inverter system and a getaway quilt to allow them a greatly expanded movement range. We so appreciate the practical way that Getty’s family is reaching out to help other families.
Emmett is fortunate in so many ways with his medical care and the great supports available to him in our wonderful province of British Columbia. As Getty’s parents point out, there are many out of pocket expenses that add up and are a burden. This device is one that we’ve talked about but just was not going to happen. And now it has!
Getty Owl Foundation – You Rock!
‘We Are Out To Discover’ Giveaway Announcement
July 2015
More Balloons & Home Today!
Tuesday, July 05, 2016
Emmett just got a new balloon from his cousins, Kole and Khloe, that has a big smily face on it. Plus, at the BCCH Gift Shop they had a Sesame Street balloon that I simply couldn’t pass up. Obviously, Emmett really enjoys this BUT the big news is that he is on the upswing and quickly becoming himself again. Emmett has a rhinovirus. Sounds like an ominous illness that you’ve never heard of before, but it’s commonly referred to as a cold – which you have heard of and have likely had without thinking twice. Before it sounds like I’m passing it off lightly, a cold for a youngster with Spinal Muscular Atrophy, Type 1 can have terrible consequences. Emmett will stay in the PICU at BC Children’s Hospital for another day or two while they ensure that his lungs are cleared out. And it is because we often don’t think twice about having a cold that Alisha and Josh are so protective of Emmett and keep him away from crowds and mandate that everyone who enters their home washes their hands and use hand sanitizer. No colds allowed.
While we were there yesterday (taking much needed fresh clothing and shower supplies), Emmett got chest physio and deep suctioning (that made me cringe but Emmett handled it like a pro) to assist his breathing. He was actually off of his BiPap machine for almost two hours. BiPap is Biphasic Positive Airway Pressure and this is a machine which provides a pressure controlled ventilation. It’s more advanced than the CPap machine that you may have seen someone use for sleep apnea. On a normal day Emmett only uses his BiPap while he’s sleeping at night or napping during the day. Since he got this cold, he has been on it much more. In fact, for 22 of yesterday’s 24 hours. The medical staff is pleased with how Emmett is responding.
Upon arrival at the hospital, he got an IV to get some extra fluids and drugs quickly. That’s not needed right now but is still there ‘just in case’. His little foot looks so cute!
Emmett, we love you and are praying for you. We look forward to having you home soon. Your 16 month’s birthday will be happening in just a few days! You need to spend it playing on your swing!
Alisha just sent an update that Emmett is doing even better today (Tuesday) and will be coming home on Wednesday! Way to go, Emmett.
Second Update: Emmett is coming home today – Tuesday!
16 Months Old!
Saturday, July 09, 2016
Saturday, July 9th and Emmett is 16 months old. Thank you for all of your prayers and warm wishes over the journey that Emmett has been on. While the diagnosis of Spinal Muscular Atrophy, Type 1 did not occur until late August, it was around this time last year that family concerns about Emmett’s lack of movement began. We were all researching the floppy baby syndrome – but our medical professionals’ perspective was that all babies develop at a different pace with Emmett just needing more time. Fast forward twelve months and there are probably even more questions. But there are some answers. More important, there has been so much joy (mixed with huge doses of anguish) as we share moments with Emmett. Last weekend you saw in our blog how our hearts missed several beats as he was taken to BCCH in the middle of the night where he was admitted to the PICU. He has quickly beaten the cold that he came down with last weekend. As he had a buildup in his lungs it was close to pneumonia, but now just a week later that is all behind us and we are looking ahead to the next adventure. And there will be some challenges too – we’re all too sure of that.
Pushing personal limits. That is the challenge for Emmett. This unusual system is a way to get vertical and have his feet planted. That is the challenge and push for Emmett. So much of his time is spent seated and reclining or lying down. Even when he goes for car rides it is in a specially designed car bed that takes up most of the back seat of their car.
This setup was customized for Emmett and, especially when a drill had to be used to finalize the fitment, he was not too thrilled with that. He was quite happy to have the attention and enjoyed being upright. Obviously, his cold put a temporary stop to being upright but as that’s now gone, he’ll be going vertical again. We really cannot say enough positive things about the support that he is getting through his team of occupational and physiotherapists.
Bottle lids continue to be one of Emmett’s favourite games. Picking them up and dropping them onto the floor is not as easy as it sounds. The blue mat has a light adhesive on it so they don’t slide off. Emmett really has to work to reach them and then to pick them up. Of course, dropping them onto and hearing the bang on the wood floor always brings a smile to Emmett’s face. If they land on his tummy without hitting the floor, that causes a look of consternation. Clearing the table of all of them causes giggles as then mom or dad need to pick them all up again.
What is next? Well, a few posts ago you saw Emmett trying out a toddler sized electric wheelchair. That is on its way to Emmett and can you imagine him roaming the house and neighbourhood. It certainly won’t be on his own! I’m curious to see if he will take after Josh and be a fast-moving toddler or if he will take after Alisha and stop every second step (do you call it a step if you’re in a wheelchair?) to examine his surroundings. Emmett loves the outdoors. Especially the trees and watching the sunlight filter through the leaves. So his backyard is a perfect haven for him as it is full of trees, plants, and flowers. While he loved the hanging out in the single hammock, he always had to be with someone (great cuddle time) but now with his giant green saucer swing – right beside the hammock swing hanging from great apple tree branches – Emmett has a perfect play spot.
Thursday, July 14, 2016
Emmett Is On The Move!
As you know, Emmett is 16 now (months, not years) so it was only natural that Emmett gets his own set of wheels! Believe it or not, it wasn’t just Josh pushing for Emmett to get behind the controls. It was Alisha too! In fact, it was all of us! Everyone surrounding and supporting Emmett. You will remember that he had an innovative little electric ‘cart’ that allowed him to move about. While it was a curiosity, in practice it didn’t work effectively. Rather than a joystick it had four buttons and that was simply too complex for this little guy (even younger at that point). But more challenging was that the wheels were only friction fit to the drive axles meaning that any incline or complex terrain (like carpet) and the axles would slip while the wheels stood still. (Yes, I could have fixed it BUT I was under strict instructions from the occupational therapist not to modify it. Or future equipment loans could be affected.)
Then you saw the photo of it a while ago where Emmett got to try the power chair out. That was the start of Emmett’s involvement in a mobility study. We are so pleased with the support that Emmett is receiving through British Columbia Medical Services Plan. His medical team has been doing so much for this little boy. Today’s focus is going to be upon his Occupational Therapists. Emmett has three. In BCCH Emmett met his first OT who has stayed involved throughout – mainly during visits to BCCH. His local OT is here and supports him actively with home visits and comes up with ingenious devices to encourage Emmett to move and get vertical. She got Emmett involved with the OT who’s working with kids from a wide geographic area in this mobility study – who then saw that Emmett had great potential for effectively using a chair. The result, he was accepted as a candidate for this mobility study and at 16 months Emmett is the youngest person in B.C. that we know about with a power chair. And Oh My Goodness – Emmett Loves It!
Now it wasn’t all peaches and cream. Either Emmett remembered that he got to sit in this chair (actually, a similar one) and didn’t get to keep it (quite a sad day) OR he simply didn’t like the two or three hours that he had to stay awake and miss his nap time while the technician was fitting him and his chair into/onto the power chair with wrenches, drills, and other power tools. Yes, the same technician who worked on his previous equipment and has done such a great job with the custom work to make Emmett’s adaptive equipment comfortable for him. In fact, it is one of these chairs that is actually fitted to the power chair. Emmett was quite cranky with all of this sitting and strange mechanical noises going on behind him. Even worse, when the joystick was active Emmett did a 180° turn and found himself nose to nose with the technician. He was so startled that he started a 30-minute crying session. Alisha and Josh had to get him out of the chair and let him have some quiet time in his own space.
When he was settled down and ready for some more seat time, his power chair was ready for him. Look at the pic to the right where he’s fussy. Even there you will see his right hand on the joy stick. This little boy was ready to move. He is alternating between using one finger on the joystick to having his palm on it. Just as exciting but easily overlooked, Emmett has been holding his wrists straight on his own. That probably makes it even easier for him to control his new chair.
Like most novice drivers, Emmett’s experiences behind the controls have not been flawless. In fact, he’s going to make his mark on the world. Or at least, on Alisha and Josh’s house. If you’ve followed Emmett’s Journey then you know that one of his favourite games is dropping things on the floor. Not only does he enjoy the activity and having someone pick the items up for him, but he loves the sound of the item hitting the floor. If it’s one of his helium balloons then it’s the sight and sound of this hitting the ceiling. Unfortunately, his Seasame Street balloon was released while he was out on the back porch. While it hit the ceiling the wind current also caught it and Emmett watched it sail away (start humming the soundtrack to the song ‘Sail’ here). Back on topic, he has quickly transferred that love of banging to his wheels – and giggles when he crashes into the wall … then does it again! Josh is absolutely hoping to quickly teach Emmett that this is not acceptable! Of course, it is very hard not to laugh as he is exploring his surroundings and banging into things. Simple things, like being able to touch something that he wasn’t able to get to previously. Turning around … and around … and around. No question, doing circles is the easiest thing for Emmett to accomplish. He is, however, seeming to understand how to use the joystick to make the power wheelchair go where he wants to. Remember, this 16 month old has only had this for two days. We’re pretty impressed with what he’s able to do.
This is so exciting but also has brought a huge number of challenges. Alisha and Josh have temporary ramps to get this wheelchair in and out of the house. They can also use them to get the wheelchair into a van or truck. It may be small but it is quite heavy so physically lifting it is not recommended. Funding is available to obtain a permanent ramp to their house. Upgrading their compact car to something like a van is going to require some help. Stay tuned for further information about a van fundraising initiative to help this young family out. They’re trying to figure out what is best – a full mobility van now or just a larger vehicle for now that can handle all of the equipment Emmett needs when he leaves the house. There are so many unanticipated expenses with a medically fragile toddler. The exciting thing – Emmett is a toddler now. Our baby is growing up and we will do everything we can for this little man.
Learning How To Move
Friday, July 22, 2016
Bumps and Bruises are part of growing up and learning to move. In Emmett’s case, the bumps and bruises are hopefully mainly on the objects around him as he is exploring with his electric wheelchair. As soon as he is set in his chair seat he starts going for the joystick. We quickly learned that it must be positioned out of his reach until he is fully strapped in with his five point harness and that the gear selector has been positioned at very slow. Or maybe one level up at slow. We aren’t ready for him to explore his environment at top speed – which must be about 3 mph. I must warn you up front about these video clips. They are slow. Very slow. Only Emmett fans will be able to sit through the whole thing. (And if you’re left wanting more, I have a couple of hours of video that I can show you. Just come on over!)
Slow. Yet at the same time it is so exciting to see that after just one week Emmett is doing quite well in his chair. Most of all, he loves it and his face lights up when he is asked if he wants to go in his chair. Once in his chair, he is quite focused and working hard so you won’t see the same expressions that we do. His grip on the joystick changes from moment to moment. Sometimes it is one finger on the top – either the index or middle finger – and other times it is a firm grip with his whole hand. Or some combination. Once in a while he will stop and give his hand a rest by twirling it about – just like race car drivers reduce tension by twiddling their fingers on straightaways. Sometimes you’ll see his steering hand trembling – because he’s working so hard.
Emmett is a pro at doing circles but there are highlight moments when he is able to head straight on to a target place. Or when you see him alter direction to get to where he wants. Even more, he runs right into garden plants, trees, automobiles, houses, and fences. Obviously, adult supervision is required.
Enjoy the video. In the first one the camera is mounted low on the chair. In the second it’s mounted in several places – behind his right shoulder, left shoulder, on his thigh looking back, arm looking back, belly harness looking up. This is our driving Emmett.
In just a few days Emmett is going to be the ring bearer in the wedding of his Aunt Katie and his future Uncle Josh. Hope he doesn’t lose the ring! Stay tuned for more of this handsome young man!
August 2016
17 Months (and 3 days) Old!
Thursday, August 11, 2016
Yesterday Emmett’s Great-Grandmother asked me if it wasn’t Emmett’s birthday already. And since it had passed, asked why there wasn’t his normal birthday blog? Well, I was camping in the U.S. near Mount Baker so I didn’t have access to the Internet let alone my computer. But, two days late here it is! Happy 17 months and 3 days, Emmett. We are so proud of you.
Emmett has been a very busy boy with little down time. At the end of July, he was the ring bearer in his Aunt Katie’s wedding to his new uncle Josh. This wedding took place at Squamish, B.C. on top of a mountain. Fortunately for everyone, rather than climbing the mountain to get there the Sea to Sky Gondola was there to transport everyone to the top. It was a very intimate family wedding and the setting was spectacular. Emmett missed out on a few pictures as the hectic pace of the day and the normal hustle and bustle of a wedding simply was too much for him. Combined with more heat than we’ve usually seen here in B.C. this summer and Emmett was simply not at 100% and, shortly after the rings were picked up from his care, he had to be excused from the ceremony. Fortunately, he bounced right back once he got into some air-conditioned comfort and he could tell that everyone around him was relaxed.
Last Sunday Emmett participated in Charlie and Florence’s 10K Run/Walk/Roll to End SMA. It was a fantastic weekend and over $52,000 was raised for SMA Canada to support the search for a cure. Emmett was out and about prior to the race start in his power chair – demonstrating his moves to anyone who would watch but mainly simply enjoying the movement himself. Charlie and Florence’s 10K event took place in beautiful Stanley Park. Emmett chose to go against the flow and did the course backwards from the start, meaning that he got to spend lots of time on the waterfront. This also meant that he got to cheer all of the runners as they came past. Emmett didn’t take his power chair as he would not have stayed on course (He is good, but that’s expecting too much of a 17 month old). Alisha and Josh pushed him in his stroller. Emmett learned to cheer whenever a runner or walker passed him – usually the cheer came after the runner was already out of sight – but it is the thought that counts, right!
While he is demonstrating some amazing skill with his chair, he also demonstrates that he is only 17 months old. For example, at Canuck Place there is a two metre tall statue of a bear. Emmett loves bears and loves growling like a bear. This colourful statue is like a magnet for him and, ignoring the flower bed at the foot of the statue Emmett powers right up to the bear. If he hit it at the right angle, I’m sure that the chair would go right up on the base and topple backwards. Good thing that his parents realize that Emmett cannot be trusted to drive on his own. He is absolutely still considered a novice and an adult must be with him at all times. Canuck Place is so amazing and Emmett is treated like a rock star (they treat all of their children like rock stars!) and while there Emmett got to have a luxurious bubble bath. Which he absolutely hated. His face was a big frown. His arms and legs were pulled into a fetal position (good moves here Emmett) and he didn’t move. He just wanted out! That was one new experience that will take a few more attempts before he learns to enjoy. Another experience that was fantastic was the snoezelen therapy room. This multisensory environment of light and sound is just a wonder for Emmett and makes his sound and light turtle or his Disney light wands pale in comparison. Fortunately for Alisha and Josh, (if you’re wondering, the answer is yes. Alisha married Josh. And Katie just married Josh. LOL!) Emmett still loves his turtle. But the snoezelen therapy room was awe inspiring.
There’s no doubt that you’re likely wondering what kind of birthday helium balloon Emmett received. It was one day late (he got it yesterday) and it is a cool elephant balloon with paper ears and legs. The paper weights it down so that rather than floating on the ceiling it actually floats near floor level and looks like it is standing on its own legs. Speaking of standing, Emmett had his legs casted yesterday so that they can make AFOs (ankle foot orthotics) that he will use in his standing frame. It is important to keep stretching the horizons of this little one by stretching his muscles. As you can tell, this has been a tremendously busy few weeks for Emmett. Especially when you consider that between the wedding and Charlie and Florence’s Run, Emmett was in BC Children’s Hospital for the final part of the study that he is involved in with the drug study. We won’t be getting an official answer as to whether Emmett is getting the real drug or not yet because Emmett is now involved in another blind study. Children in the first study go through the same process as the first study, except everyone gets the drug. But if you got the drug in the initial study, these children will get a placebo in the rapid phase and only get the real drug during the four-month intervals. Just to maintain suspense and test the effects of the drug. Which we do believe Emmett has been receiving.
Not Feeling Good!
Saturday, August 13, 2016
The last couple of days Emmett has been quite irritable. Yesterday wasn’t sleeping and more irritable. Fever. Threw up in the morning – something he rarely does. Alisha got him an appointment with his paediatrician who could tell he was uncomfortable, ordered a few tests as benchmarks but also to see if they revealed anything, and set it up so that Emmett could go right to the paediatric floor. He got the best care and treatment on Abbotsford Hospital’s 4th floor, tests done quickly, and sent home. Blood work results were clear.
However, little Emmett was still miserable. Holding him wasn’t comfortable, a bath wasn’t fun, his swing just okay, and he wouldn’t sleep. The only thing that would make him laugh was ‘Paw Patrol’ episodes. We watched a lot of Paw Patrol. This morning Late last night he was throwing up more so Alisha, Josh, and Emmett made an early morning late night trek to BC Children’s Hospital. He just won’t settle. Praying that they find what is bothering him!
UPDATE: Emmett’s lungs are clear and they’re coming back home. He’s still irritable, but after a session of throwing up there was a huge worry of something being aspirated into his lungs. Fortunately, that’s not the case. Now to live through this stage of irritability!
“New to Emmett” Van
Tuesday, August 16, 2016
Emmett is feeling fine now and thanks everyone for their prayers and concern.
A few blog posts ago I mentioned that Alisha and Josh had found a solution to their transportation concerns – a wheelchair accessible van.
Wheelchair vans are expensive. Plus they are very time consuming to make. Yet the 4 door compact car that Alisha and Josh were transporting Emmett around in was simply stuffed to the roof with his equipment for just a short journey. There was barely enough room for luggage for an overnight stay while fitting a wheel chair next to impossible. A power chair – not a chance. This family had to go shopping for an automobile. The question being ‘what’? Simply a larger vehicle to accommodate the volume of material required or some type of mobility vehicle? If a mobility van, how long would it take to get? and how much?
While contemplating what to do for a more spacious vehicle to haul themselves around, the need for a wheel chair accessible van became a priority when Emmett got a power chair. Even though he has only had it for a few weeks, this chair has become an important part of Emmett’s life. To put it simply, he loves it! Maybe even more than swimming!
Josh heard from a long-time friend, who’s an occupational therapist, about a family with a potentially available wheelchair accessible van. It was a terrible tragedy for them, as their ten year old child had recently passed away. We could empathize with the pain they were experiencing, yet also so appreciated what they were doing. They wanted their 2010 Dodge Grand Caravan – equipped with a SideWinder rear entry ramp – to go to a family that needed a mobility van for a child. Their tears and sadness were hopefully lightened a little knowing that another little one, Emmett in this case, could make use of the vehicle that they had maintained so diligently.
This six year old van is exactly what a budget minded family would want. It is in excellent shape and, if necessary, can seat six plus the wheelchair. It has a manual ramp which is unbelievably easy to deploy and is also low maintenance. A heavy-duty spring means that with just one hand it can easily be opened and closed. Emmett has already been for a couple of rides in his chair in his new van. Without doubt, he loves it! He can sort of look out and up through the front windshield which absolutely makes car rides more intriguing. For most trips he’s secured in his car bed, which fits perfectly into this spacious van. That’s what they used when they recently made the trips to Vancouver to BC Children’s Hospital.
While the van was purchased for a great deal, it is still a huge expense for this family. Donations to date were being saved up for a big event – and fortunately the big event was getting this van with donations covering more than half of the expense. Thank you so much – we simply cannot say thank you often enough. We want you to know that your continued best wishes, prayers, positive thoughts are by far the most important thing we need from you. We thank God daily for the progress that we witness with this little boy. He is our little miracle. We hope that sharing Emmett’s Journey with you provides you with inspiration, happiness, and joy. We know that there have likely been some tears too! That’s part of this journey.
Of course, now that Emmett is upright, he can see that his mom and dad are driving. And that almost knocks his pants right off! I love this look of semi-terror as Josh is getting ready to move the van!
One Year Later!
Thursday, August 18, 2016
Thoughts from Alisha and Josh one year after the diagnosis of SMA Type 1.
“One year ago a neurologist told us that our Emmett has Spinal Muscular Atrophy Type 1 … But today we are thrilled and amazed that Emmett has the chance to be the most cheerful toddler who loves to swim, drive his chair around, and play with his balloons. Emmett, you are our greatest adventure!”
September 2016
This Boy Can Drive!
Thursday, September 01, 2016
Put Emmett in his Power Tiger chair and he’s off. He has moved way beyond circles and he can manipulate the joystick to get exactly where he wants to be … which may not be where the adult with him wants or expects! In fact, he even demonstrates street safety. Towards the end of the video, he is on the driveway and heading towards the street – but he stops and turns back! Is it because the driveway is a bit bumpy and he doesn’t like it? Or is it because he has heard ‘stop’ so many times as he has gotten to that point on the driveway? Either way, it is impressive.
Emmett has come so far and in this blog we have certainly focused upon that. Playing catch/soccer by using his chair to capture the ball! Wow. This is literally the first time he’s played with the beach ball. Waving bye to his mommy and daddy as they head out – just so cute! Yes, Emmett has come so far. The reality is pretty evident, however, that Emmett faces huge struggles – even as a toddler. However, rather than focus upon these very obvious challenges we love highlighting the fantastic things that we see. Accentuating the positive!
But being positive is a challenge for Emmett today, because his Power Tiger chair is malfunctioning and there’s no one to contact on Labour Day weekend.
18 Months
Saturday, September 10, 2016
With a shout of hooray, Emmett celebrated his one and a half year birthday on September 9 – the same day his Auntie Katie was celebrating her birthday. There was no joint party, as Aunt Katie is now living in Australia. To celebrate this milestone Josh’s cousin, Jocelyn (Jocelyn Isaak Photography), captured a number of amazing photographs for us to share. Emmett was also visiting BC Children’s Hospital this past week – don’t worry, nothing was wrong. It was just time for a series of tests and observations as this was the finish of the first part of the Ionis Pharmaceutical drug study. He is now moving into the second stage. Plus he got to visit with his crew. Emmett loves his crew (his medical team) and will gladly skip his naps so that he can visit with his hospital people. Another benefit of this trip to Vancouver was that his wheelchair was dropped off at the shop (bypassing wait time for a home visit) and they got it sorted out and set up better than ever. Last night Emmett went for a stroll in his power chair – it took maybe 30 minutes to go 400 metres. Circles. Backwards. Sideways. Stop to watch the cars. Stop to watch the clouds. Stop to just stare! Go backwards because mom wants him to go forwards. It was a typical toddler stroll – except Emmett was in his newly functioning power chair.
While in Vancouver Emmett got to stay in a fancy hotel and he got a huge bed all to himself. He quite loved the luxury and, if he could have, would have been on the phone to room service. Emmett can be quite demanding, as I’m sure you can imagine. He always wants to be active and doing things, so keeps mom and dad pretty busy. Of course, even when demanding he’s a great baby and his volume is much lower than most fussy 18 month olds. But he’s still demanding!
At the hospital he was poked and prodded while they were checking out all aspects of his physical ability and responses. The usual blood tests which you never get used to. A nerve study which really sounds uncomfortable. And observation … lots of observation.
They liked what they saw. While Emmett is not necessarily doing things that are evident for the casual observer, he is absolutely showing giant steps to all of us. Especially his medical people. For instance, Emmett’s legs three months ago were barely moving unless he had the benefit of being in water. Today, Emmett moves his legs and if they are placed in a bent knee position he can hold them there for seconds, correction here, minutes at a time. Two or three months ago, they would instantly fall flat to the table. Compared to one year ago and the differences are almost beyond belief. In fact, they are beyond belief. One year ago his movement was so drastically limited that many of us doubted we would see this birthday milestone!
Yet here we are. This is a miracle. This drug trial from Ionis is a scientific wonder and miracle. That Emmett is one of 120 kids worldwide in this study is a miracle. While we would love to see it available to all children with SMA, the process of approving a drug and making it available to the public is painfully slow. Beating the odds of colds, flu, avoiding pneumonia, and all of the other normal childhood ailments that would be life-threatening for a little one whose respiratory system has been significantly compromised. Yes, it is a miracle! Continue to pray for Emmett. Continue to pray that this drug is made available to all children who need it.
Alisha, Josh, and Emmett celebrate the miracle of life each and every day. Emmett puts his hands up in the air and cheers! He started doing this at ‘Charlie and Florence’s Run to End SMA’ and it continues. He loves to cheer. He loves to wave to people. If you see him, please wave. In fact, if you see any toddler in a wheelchair, then wave and say hi! Wave long and hard. And be patient – as it will likely take Emmett quite a few moments to get his body to respond and wave back. But he will wave back! And you will make his day! Alisha and Josh’s too!
September 2016
Wow! Earthshaking News.
Thursday, September 29, 2016
Since being in beautiful British Columbia last fall, Pamela and I have felt two earthquakes. Light tremors that made you sit/stand up and say “What was that!”. Prior to that in Ontario, I can only recall feeling one tremor. But, feeling these tremors of the earth I am positive that it is what Luke, Yoda, et al were experiencing when they would exclaim that they could feel the force.
The Spinal Muscular Atrophy Community is currently abuzz with reactions to a tremor that has positively shaken all of us. A force that is very positive and worth noting. You see, this disease which medically had no options and which doctors’ best advice (for Type 1) was to help make your little one comfortable and happy as there was not a medical option — now has an option looming in the not too distant horizon. Medical hope. It isn’t that there wasn’t hope before – the human spirit is massive and there is always hope. However, little ones with SMA have had massive odds stacked against them. There was literally no medical cure. Indeed, when Emmett was diagnosed just over a year ago that is what his parents were told. But they were also told about an experimental drug trial that might be a possibility – and they latched on to it. I must tell you, I was not in favour of our little grandson being part of a medical experiment. The medical team at BC Children’s Hospital was so welcoming and caring that they convinced Alisha and Josh – who convinced the rest of us – that Emmett had to be in this trial. We are so happy that he is.
At the beginning there were so many questions about this blind study. Much heartache as we dealt with the effects of SMA on Emmett’s tiny body. Much Joy as we saw a reversal of the decline. For those of you who have been following Emmett’s Journey, you have seen the growth and development of Emmett’s body as the messages from his brain started getting through to his muscles.
There were about 120 little ones, all no more than seven months of age, from around the world involved in this drug trial. One third of the participants got a placebo and not the drug. We do not know for certain if Emmett got the drug – because that was part of the blind study. The primary physicians did not know. We do know, however, that we all have seen the progress that Emmett has made throughout this past year. In this disease in which children do not improve, during this blind study we have been non-medical observers and noted that Emmett HAS improved. Our guess is that yes, he got it. We can tell you that Emmett is in an extension study (called SHINE) for patients involved in the previous study. There is no question, he is now getting Nusinersen.
Biogen and Ionis (two pharmaceutical companies) have completed the rolling submission of a New Drug Application to the U.S. Food and Drug Administration. Biogen is also planning to make submissions in other countries. This drug, Nusinersen, means that parents will no longer hear that there is no medical hope. Nusinersen is HOPE! That is the tremor that has been felt around the world. This drug cannot be made available fast enough. And this is where the entire SMA community has felt the tremor. Nusinersen means so much. The need to see this drug made available quickly is so necessary.
Link to CureSMA’s Update on Nusinersen.
October 2016
19 Months Old!
Friday, October 07, 2016
October 10th is the day that Canadians celebrate Thanksgiving. This is the day after Emmett turns 19 months old. It is a day that the Willms and Ewald families are certainly expressing our thanks. Emmett is getting stronger and stronger. Last Tuesday and Wednesday he was in at BC Children’s Hospital for a series of check ups and the second session of the second phase/year of the experimental drug trial. While we can only guess that he was getting Nusinersin during the first year as it was a blind study – his development suggests he was. This second year he is absolutely getting Nusinersin. His neurologist, Dr. Selby, looked at all of us and commented that she has never seen a little one with Spinal Muscular Atrophy demonstrate improvement like Emmett has. Our miracle baby on his miracle drug. It is fantastic. Right now Emmett is the only child in the experimental program at BCCH – we are hoping that other little ones soon get the benefit of this drug. Even with this drug, Emmett has to fight and work hard for everything. Just watching him work to throw his blocks on the floor is a challenge that he joyfully tackles. We are so happy that Emmett is a happy and outgoing little boy.
Here is a video of Emmett from just over a year ago, September 2015.
And video from Emmett today:
Like the giant saucer swing that Emmett is using? You can get one too – and save 20% – visit https://www.swingingmonkeyproducts.com/ and on checkout enter the following in the coupon code: EMMETTSJOURNEY This swing was amazing to find and it provides Emmett with lots of enjoyment!
His Pulmonologist, who only sees Emmett a couple of times a year, was visibly impressed when he saw Emmett. He literally stopped in the middle of the examination room and watched Emmett. He stared as Emmett jabbered, squirmed, and moved his limbs about. You see, medical experts know that for a child with a diagnosis of SMA Type 1 and a timespan of a few months between visits, that they will note a significant decline. Emmett is the only patient currently in this experimental drug study at BC Childrens Hospital and he is stunning the medical world here with his obvious improvements. Emmett, proud to show off, lifted his left elbow more than ten centimetres off of the examination table, snorted like a pig, gave a Tarzan yell, and then pretended to blow out candles!
So we are thankful – even though if we are honest being thankful is so very challenging. Heading to this visit meant packing up his formula, his food pump plus tubing, the bi-pap machine, the oximeter, his suction machine, his stroller plus his wheelchair. I’m sure that I forgot a few things. We are absolutely thankful – but still have those days when we wonder why! Why Spinal Muscular Atrophy, Type 1! Why Emmett!
And it wasn’t only these two specialists – the other doctors, the residents, the nurses, the program staff – everyone in contact with Emmett literally have huge smiles as they see this little fighter. So this Thanksgiving we are thankful that Emmett is with us and that he continues to grow and strengthen. His favourite activity right now is taking everything that is near him on his tray/bed/etc. and tossing it on the floor. He loves to hear that bang on the floor and, with his great sense of humour, loves to give the sign for ‘more’ to make whomever is nearby pick up all of his toys/blocks/etc. up just so that he can instantly toss them on the floor again. He literally never tires of this game. Alisha and Josh, however, are wishing (sometimes) that they’d never shown him the sign for more!
Of course, it is time for another helium balloon as they always coincide with a hospital visit but especially his month birthday. Helium balloons in all shapes and sizes have been presents for Emmett over these past 19 months. Some normal sized ones and some have been rather large. None of them, however, prepared Emmett for his Nemo the Clownfish air swimmer. This balloon filled up the entire backseat of our mid-sized car and while Emmett’s eyes bugged out when it first came through the door of his house, once he saw how gigantic it was up close he absolutely played shy. There was no way he wanted that fish close to him. At the same time, he wanted to be able to see part of it. It is going to be a case of want overcoming fear! So here’s a video of Nemo without Emmett – we’re giving him time to get used to this giant Clownfish! He’s allowing his mom and dad to figure out how it works. Of course, they only play work with it to be ready for when Emmett has overcome his hesitation.
As you can appreciate, it was a huge day when Emmett went to Lamp Post Hair Studio and Kim gave Emmett his very first haircut. He is such a fine looking young man … and his curls are still there … just shorter. At 82 centimetres tall, with his new haircut, and vertical in his standing frame – what can we say except that Emmett looks like a grown-up almost two year old! You may recall how Emmett had a special harness and helmet set-up to allow him to use his Jolly Jumper. This held up his upper body and specifically his head. Look at the photo of Emmett in his standing frame. Yes, it has harnesses but they are there to hold him into place. And it has a neck/head support to allow him to rest/stop his head from falling backwards. Emmett is, however, holding his own head up as he turns from side to side and looks up and down. Wow! We are thankful for that huge sign of improvement.
While out and about Emmett has surprised many observers who just assumed that dad or mom was controlling his power chair. The reaction is priceless when they realize that Emmett himself is at the controls. Obviously, there are many times when it is an adult providing the inputs but even more when it is Emmett. Bumping into things still happens however he is getting better and better at understanding what he can and cannot do with his chair.
Obviously we are incredibly thankful for all of the prayers and support that are offered from around the world. Just as obvious is that Emmett is still incredibly medically fragile. As we move into cold and flu season this is a stressful time for Alisha and Josh, as for Emmett a simple cold will have such a huge impact upon his weak respiratory system that is already working so hard.
The challenge of letting your little one explore and be a part of the everyday world BUT the urgent need to keep him safe from commonplace, normal childhood illnesses. A battle that Emmett makes more challenging because he wants to be out and about to interact with others. He is, quite seriously, a social butterfly. In fact, while in Vancouver he enjoyed a night time trip up and down part of Davie Street where he could absorb the noise, lights, and smells. Before you get worried – he was back inside before 8 P.M.
It is a challenge to be thankful. Yet it only takes a smile, a cuddle or a giggle from Emmett to push aside all challenges and cause us to see how blessed all of us are.
Emmett – you truly are an adorable little boy who lights up the room with your smile and interactions.
Alisha and Josh – you are amazing parents and your care and devotion for Emmett are a joy to see. That you fight for him every day is not something that you let many others see, but we know you do. Thank you.
The final instalment of our birthday blog is a lengthy video of Emmett using his power chair at Camp Squeah – just outside of Hope, B.C.
November 2016
20 Months
Monday, November 07, 2016
In this blog I have to tell you that Emmett does not have a new balloon! Even though it is Emmett’s birthday – no new balloons. Even though he is signing for more in the above photo, there comes a moment when you have to be tough and say ‘No’! After all, should we continue to spoil him? give him everything he wants? make sure he’s always inspired? entertained? Tough Love – even for the little boy whose eyes will cause you to bend to his every wish. Tough Love – stand firm on the stance of no balloons for his 20th month birthday, a day on which Emmett is showing continued signs of strength and genius. In the photo below we’ve just told Emmett about our no new balloon plan!
Yup, no new balloon for him. As we start an austerity program just in case the world economy crashes drastically, Emmett is only getting hand-me-down balloons. We’ve refilled Nemo the Air Swimming fish (which costs more than a regular helium balloon, but that’s another issue) – but Emmett still isn’t really to excited to have this giant orange clownfish swimming around the room. He loves it when it’s not moving. Emmett hits it on the nose (or the eye) and then chases after it in his chair. And since the clownfish came in a twin pack with a shark – when Nemo bites the dust then the shark will come out. We will find out if little ones have preconceived ideas about creatures of the deep with sharp teeth. Of course, Emmett has a couple of weeks old Paw Patrol balloon that is a regular sized normal balloon that is fun. He still loves letting balloons go and watching them hit the ceiling.
And then there’s Ella. Ella is Emmett’s best inanimate object friend. Initially Pamela thought I was crazy to buy Ella the purple elephant – because Ella was at that point (before the air swimmers) the most expensive balloon that we have purchased. Emmett can say ‘Ella the Elephant’. Now, maybe the last two words you have to have a really good ear – but there is absolutely no question about Ella! The other night he went to sleep murmuring ‘Ella Ella Ella’ over and over again. I burst out laughing on Monday because Alisha texted me how she sent Emmett off to find Ella. He was in his chair – and he’s really quite good at finding his way about – so off he went searching. There was a problem, however, as Ella was off with us to get a refill of helium so was not anywhere to be found in the house. Nemo was also gone – so Alisha had a frustrated little boy on her hands! LOL! Or if I want to be hip, then ‘ha ha ha!’ (Alisha tells me that LOL is so old school that I’m dating myself. My Bad.) (I hope that My Bad is also past it’s prime – I’ve never used it because I think it’s a silly saying, but if I irritate Alisha a tiny bit it may be worth it!). The best thing about Ella the Elephant is that she comes with a lifetime of free helium refills! We’ve been worried for Ella’s life a few times, as Emmett has literally squashed Ella between furniture and his power chair! Ella’s life flashed before our eyes, but she remains full of gas! Emmett is getting lots of value with Ella! If you live near us, stop in at EZ Party on George Ferguson. Tell them Emmett sent you – they are great at filling helium balloons!
You know, we do laugh. Emmett is so much fun. Alligator Baby has been his favourite book for a few weeks now although he may be tiring of it. It is unfortunate that he is tired of it, because he knows all of the actions and sound effects. On cue he goes ‘whaa, whaa, whaa’ to show the baby is crying. He shrieks when the Alligator baby bites the mom and then the dad on the nose. He uses his hand to signify knocking when the parents go blam, blam, blam on the door. Of course, Emmett’s ‘blam, blam’ on the door is quite soft. But now he is tired of it. And when he’s tired of a book, he’s quite able to show it. He shakes his head from side to side and expresses his displeasure until you set the book aside. If you ask Emmett if he wants to read a book that he doesn’t like – you’ll get the shaking head. If you suggest a book that he wants, you get a positive expression and his head vigorously nodding yes. Mind you, it might be twenty books he doesn’t want before you get to one that he likes. Alisha has narrowed it down to stories with animals, stories with rhymes, and stories that aren’t too long. Of course, like everyone else, he likes his readers to use a lot of expression. Right now his favourite book is I know a Rhino – and at the part where there is a tea party he holds up two fingers and then three to show the number of spoonfuls of sugar required. The Rhino is the one who likes three spoons!
Emmett loves to laugh as much as we do. His giggles are infectious. When he fills his diapers with poop he giggles about the stinker that he has become. When the adults tell a joke it is amazing how often Emmett will giggle just a moment before the adults in the room have understood the punchline. He does it so often that it cannot be chance. Alisha’s friend, Kate, has been visiting and captured Emmett’s antics with her camera for all of us to view. Kate is also a speech therapist and is quite impressed with Emmett’s attempts at verbalization. You can imagine that we are looking forward to more communication as Emmett gets older and his muscles get stronger. You see, SMA affects speech too. There’s literally nothing that this disease doesn’t affect.
The sense of wonder that Emmett has is fantastic. He loves watching. He watches birds outside his window playing in the trees. Seeing cats and dogs playing and walking in the distance – he’s not ready for close up just yet. He has a fascination with his bedroom light – shaped like an airplane – and a highlight is being held up high so that he can spin its propellor or touch the fuselage. This fascination was captured in this photograph when, possibly for the first time, Emmett saw an airplane flying high over his house. The look of astonishment on his face is incredible. There is no way that the occupants saw this little boy in his wheelchair waving at them, but that didn’t dampen his spirits or smile. Nor did it stop him from waving. I think Emmett’s going to have to go to work at church with his dad soon. Not because Josh is a Youth Pastor but because the church is right beside the airport. Time for Emmett to watch planes land and take off. Curiously, when Alisha was little she liked watching trains. Planes were a second choice.
Emmett’s mobility continues to amaze us – have wheelchair will travel. At home, to make things easier, all of the grandparents got together (and uncle Ben and 2nd cousin Jason) to build some ramps at the back door and in the garage. Emmett can now drive his chair out the back door, onto the covered porch, then through the garage and into the front yard. This is not only easier for Emmett. It’s easier for Alisha and Josh. That chair is tiny but it sure does weigh a ton.
Emmett was at Canuck Place for some down time and a chance for him to experience a change of surroundings, plus a bit of respite for his mom and dad. The staff there were quite amazed at how well Emmett maneuvered around items in his power chair. It is really quite amazing to see what he can do. While there he got to hang out with Nicholas. I didn’t hear if they had races in their chairs but they have a lot in common. You see, Nicholas is a four year old little boy who has SMA. He also has a smile that will move mountains.
Emmett, happy 20th month birthday. There are extended trials for the drug Nusinersen – what a fantastic birthday present that other little ones with SMA are now getting an opportunity to have this drug.
From Emmett, Alisha, and Josh to all of you – thank you for your support and love!
Cold and Flu Season
Monday, November 14, 2016
I have a cold. As a result I haven’t seen Emmett for over a week because at the first sign I stopped visiting. Today I’m going to wave at him from the front and back yards through the windows. For me, a cold is irritating and my nose gets sore. Everyone around me (by that I mean Pamela) knows that I am sick and that I am in dire need of assistance and support. Back rubs and cuddles would be great. Of course, that doesn’t happen because I have a cold.
Josh had a cold. Not too horrible but enough to have to wear a mask around the house and keep his distance from Emmett. Even from Alisha – because Alisha does not want a cold. The question is, did I give Josh his cold? Or did he pick it up elsewhere?
Emmett has a cold. Yes, Emmett. It happened Friday. He’s been very congested and required a lot of suctioning. The first day of his cold he couldn’t sleep. If he started to fall asleep then he would cough and wake himself up. Even with the bi-pap machine breathing with him his oxygen levels are lower. Even before Emmett’s cold, Alisha was cleaning and sanitizing the house daily. How do you keep cold germs out of your child’s lungs when you cannot see them. It’s impossible. Every mom knows that but every mom still tries. And Alisha tries harder than most. You have heard us say that when other kids get the ‘green elevens’ it is tough but sometimes comical. Absolutely gross. With Emmett his weak lungs cannot cope. The Bi-Pap machine helps. Fortunately, he has already started showing progress; we don’t require more invasive measures like intubation and oxygen.
Alisha and Josh decided to deal with this cold at home rather than going to the PICU. Even though Emmett loves all of the nurses and doctors there, the battery of tests and needles isn’t fun. Plus the risk of catching something else is also a possibility. Hospital trips are only when it’s beyond in-house support. Emmett’s night nurses are kept busy with suctioning and observation. A bit of chest physio. Saturday morning Emmett got a treat by being allowed to watch TV all morning. The compromise – chest physio, lots of chest physio.
And now it’s Monday morning. Emmett at 8:30 a.m. he was still sleeping. A good sign. He slept well yesterday afternoon also. His oxygen levels and heart rate are closer to normal than they’ve been all weekend. He is still coughing a bit. Worrisome, but you know what? Coughing is also part of the miracle because that shows his lung muscles are improving. You see, he didn’t and couldn’t cough before. The message that he needed to cough never reached the muscles around his lungs. So this cold, as terrible as it can be, also shows how Emmett continues to improve. The messages are travelling from his brain to his muscles. We just hope and pray that this rebuilding continues – faster and faster.
So as soon as I heard that Emmett was awake, I was at his window to see him. BUT since I have a cold, I stayed outside and waved through the windows. It looked odd to the neighbours, but I’m not worried about that. And Emmett doesn’t care what the neighbours think! In fact, he’ll probably think it’s wonderful if they call the police and I get arrested. The activity will be fantastic. And my family will bail me out – once I no longer have a cold!
We went for a short walk. Encumbered by his bi-pap and oximeter and suction machine – everything needed to monitor Emmett. Being sure that all is well. And on our short walk, all was well. Emmett is bouncing back from his weekend cold. Alisha and Josh – well, they will recover too. It’s just that when Emmett is sick it takes a lot out of them. A cold for this little boy is tough on everyone.
Edit:
At 4 p.m. Emmett was still looking and sounding good, however, his specialists wanted him checked out. So the van was packed up and off to BC Children’s Hospital in Vancouver. After an evening of tests, Emmett was cleared and sent home. So around midnight they were back. And everyone’s schedule was in upheaval. It also meant a lot of work disinfecting everything that had been at the hospital. Just in case …
Another Lumbar Puncture!
Tuesday, November 22, 2016
Update: Everything went perfectly (apart from some scheduling delays) and Emmett is happy and at home.
Pray for Emmett today. He’s back at BC Children’s Hospital for the scheduled visit and next injection of Nusinersen. Interestingly, this new drug is now going onto at least it’s third name, Spinraza. We don’t really care what it is called – all we know that it is amazing science that is working!
Most people go through their entire life without a lumbar puncture. All they know about it is that it is something that is very uncomfortable – painful even. Wednesday, November 23 – Emmett is having another Lumbar Puncture. He’s lost count of how many he has had … of course, he hasn’t learned how to count yet. Alisha and Josh have not lost count. They’ve felt every one!
Thanks to your prayers and positive thoughts, to a supportive family, to his incredible parents and, at just over twenty months, Emmett’s indomitable optimism and enthusiasm – Emmett is able to have a lumbar puncture and if past practice continues within hours seemingly has forgotten it. He just bounces back! Some kids would be in tears if they were back at a place that hurt them. Not our little Emmett. He literally views the hospital as a place where he meets his friends. We are so blessed. Emmett is over the cold that he had last week. Like previous colds, he still requires a bit more suction than usual but this healthy little kid beat his cold in less than half of the time that it has taken me! Alisha and Josh will be watching him closely over the next couple of days. Not only because he has had the lumbar puncture but also because he has been interacting with a whole bunch of people that are somewhat new to him. And as much as he loves new people and his doctors and nurses, there is always that risk of germs.
All Emmett needs is a brief nap and then he’s ready to love his nurses and doctors all over again. Give them a smile or two. Show them his grip and how he is doing at holding his head up. And if allowed, he will show everyone how amazing he is at controlling his power wheelchair. If asked he may even sign an autograph or two. (Of course, Emmett hasn’t learned cursive writing yet. That’s not his fault – it’s not on the curriculum anymore!).
A highlight for me this week was being over and visiting Alisha and Emmett. Alisha had me wait on the back porch and then opened the door and came out. Following behind her and driving his chair was Emmett. Out the doorway, across the porch to come to me, and then down the ramp into the garage and then out onto the front yard. Impressive skills! Emmett literally would drive right up to me before stopping. No fear. But you know what – he did the same with my car and their van. Without touching the vehicles except, of course, with his hand. He loves touching cars and trucks. He loves waving at his reflection in the paint. This little boy loves waving. And his control of his wheelchair is incredible. Of course, he will still run over feet and toes. And if he’s really happy to see you he may just run into and almost over you!
This is where I must confess that I get angry. You see, there are things that Emmett and I cannot do together. If I was a really together Christian then I’m sure that I wouldn’t be angry, but I am. Angry, that is. Because I want Emmett to experience all of those things. I’m also worried because I know that Emmett and I will probably eventually try to do most of these things that people will think that we shouldn’t be doing. In fact, we can do some of those things very soon, maybe even right now – we just have to plan it out more thoroughly. Yet even with planning I’ll get in trouble with my wife, my daughter, my son-in-law (unless he’s in on it with me), and probably lots of other people. If it makes Emmett’s day, it will be worth it.
Then that anger is pushed aside and, with Emmett nodding his head like he did this morning as he’s asking for another story, we will read a story together and laugh together. Oddly enough, Emmett didn’t say no to any story this morning. From the first book he enthusiastically indicated he wanted it read to him. “If you give a mouse a cookie”, “Paper Bag Princess”, and the list goes on. He wanted them all! We read stories while his mom and dad packed up for the trip to Vancouver.
We’re praying for you, Emmett. Have a safe procedure and we’ll see you back home soon! (And don’t worry about breaking your wheel chair! I used to break my bikes, my dad’s tractors, snowmobiles, cars and trucks, and then all of my own vehicles. I think it runs in the family!)
Now, back to Nusinersen now also called Spinraza. Last night Avis Favaro on CTV news did a segment on Spinal Muscular Atrophy and Nusinersen. It is really great to see the National Media share this story. Emmett is one of only a few kids in Canada who was part of this study — and now it’s getting administered to SMA kids in the extended program. See the CTV National segment here.(Sorry, it appears that this news clip has vanished.) As we’ve said before, we are so hopeful that this drug changes other lives the way it has changed Emmetts. Even better, the drug is being given to children much younger than when Emmett started at Seven months. And that just means it can start working that much earlier!
UPDATE 12:45 PM PST:
The procedure went very well. Emmett is growling like a bear – he’s happy! Emmett, Alisha, and Josh will be home soon.
December 2016
21 Months!
Friday, December 09, 2016
Milestones! Each month is a milestone. And December 9 is 21 months for Emmett! We are so excited! And tired. As we have been on this journey with Emmett, we have realized that the specialists’ initial statements that we may not ever see Emmett in pre-school, hear him talk, or live much past the age of two were the general understanding of youngsters with Spinal Muscular Atrophy Type 1. And while very fact based are not going to be the outcome for our little Emmett. He is powering his way through life – albeit with muscles that aren’t very powerful. But his antics and expressions make up for his lack of movement.
You may have heard that the weather in BC has been rather rainy. I think the weather report was two days each in October and November with no rain. Balance – rain! So when it was sunny out then Emmett and Alisha had to get outside and play. Walks take quite some time but they’re worth it.This was a local park that is fun to visit.
Emmett’s Christmas tree is up with lights and decorations on and his house gets lit up at night with some artistic light displays (set up by both grandpa’s) that he enjoys admiring. Emmett does not tire of decorating the tree. Literally. He has multiple times had the ornaments removed from the tree so that he can look at them up close, one at a time, before they go back on the tree. He is fascinated by them. So much so that we wonder if he will allow the tree to come down after Christmas!
Emmett, Alisha, Josh, and Rhonda went to Canuck Place in Abbotsford where they got to get up close to a bunch of reptiles. Emmett really wasn’t sure about them. If you’re wondering what Rhonda is laughing about, let me just say that she does not own a snakeskin scarf! Brave woman!
Emmett has recently discovered he is more into warm blooded creatures. His early Christmas present (or is it his daddy’s?) is a seven month old cat that they’ve named Wendell. After a couple of hours this creature changed from a scaredy cat into a purring machine – Wendell is absolutely at home. While he is still getting used to Emmett, Emmett absolutely adores his kitty cat. They are going to be great buddies. I love the expression on Emmett’s face the moment Wendell jumped from beside him to the back of the sofa. Wonderment!
What an amazing time of year. Emmett is 21 months old! Lots of laughs and giggles from the Willms house to yours. (Oh, and Emmett didn’t get a new balloon this month either. But Ella the Elephant did get a refill! Here are a few more videos of Emmett … if you have time!
Emmett was ready for his nap as he headed back into the house from a long walk/roll. As such he exhibits characteristics of a tired driver!
And finally, here’s a rather fussy Emmett chattering non-stop! He wants something and I’m not getting it!
Reality Can Be Harsh!
Thursday, December 22, 2016
On our journey of finding out more about this genetic disease called Spinal Muscular Atrophy, we have found on FaceBook and other sources inspirational success stories of youngsters who have beaten the odds and are in University – and that is without the amazing drug, Nusinersen, that Emmett is receiving. At the same time, our journey has placed us beside others who have little ones have not been able to survive this disease which literally shuts down the nerves in the spinal column and causes all muscles to atrophy (stop working). That’s bad when it’s your arms, hands, and legs. Terrifying when it involves the ability to swallow and breathe. That is a challenging part of this journey; seeing parents and families grief-stricken as their cherished little one has moved on from this life to the next. And that is hard. Very hard. It simply isn’t fair. Watching little ones struggle with overwhelming physical challenges and knowing that the best scenario is a life dependent upon others for basic necessities will be a fact of life. It simply isn’t fair.
Not fair. Just like we ask why our little Emmett had to be born with this most challenging genetic disease, we have asked why many times. Why – the question that cannot be answered. Another question is why didn’t everyone in the drug trial for Nusinersen get the real drug. Again, there isn’t an easy answer. Sure, the question may scientifically be easy to answer. After all, it’s a scientific study and that is what happens in studies … but does that make it fair! But not when the drug is working yet other little ones were getting the placebo. Then it is a horrible reality! And obviously that happened. What we can say is that because of the kids (and their parents) around the globe involved in this trial and showing how successful it has been, this trial resulted in an earlier than anticipated wider release of the drug. No more placebo going out – the drug was simply too positive to be giving some little ones the placebo. Fantastic news. Of course, the next harsh reality will be getting the drug approved. And once approved what will it cost! Suggestions and speculation are that it will be extremely costly. What then?
Recently I was asked if it wasn’t frustrating to be in a blind study when we didn’t know if Emmett was getting the drug or not. If you’ve been reading from the beginning you know that initially, I (remember, I’m his grandpa) was against Emmett being in a drug study. I didn’t want him to be put through the challenges and the unknown factors involved in the testing of a drug that was literally unknown. What kind of side effects? What would he have to endure? So many questions. I’ve said before, Alisha and Josh convinced us. Dr. Selby and her team convinced them. And the medical team at BC Children’s Hospital has been so amazing in their support. They are like family!
Talking about family, his team of night nurses are also family. The care and love that they provide Emmett is something for which we are all so thankful to have. They are there for the mundane things like diaper changes through to the extreme moments (fortunately these don’t happen very often) of ensuring that his airway is open. They help him by turning and repositioning him – which he literally cannot do on his own (have you ever woken up in the middle of the night and felt you couldn’t move? That’s Emmett!) – and, when he wakes up at night they read to him, sing to him, and pat him on the back. Of course, they follow his monitors – the oximeter, the bi-pap, the food pump. Suction as needed and change sheets if he’s drooled so much that his face is almost a puddle. This team of awesome individuals is critical to Emmett’s care – and his parents’ peace of mind.
Another reality is we do not know what will be next for Emmett. We are optimistic about him speaking and looking forward to that. He’s very communicative with his chattering, his eyes, his facial expressions, and his hands. He knows a couple of sign language phrases but he cannot control his fingers enough for most sign language. He’s getting more movement – his physiotherapist and occupational therapist have observed this. Yet we want so much more movement. Obviously with time and more medication and more prayer there will be more changes. Alisha and Josh constantly work with Emmett on all of his skills. It’s a task that goes on almost every moment that Emmett is awake. Questions. Will he be able to take food by mouth again? He mimics us when we’re eating but that is such a challenging thought. He has to be suctioned for saliva – he’s not ready to swallow food! Will he continue to gain strength in his arms and legs. Will his grasp develop even further? Will he start talking? Only time will tell. We keep praying! And working.
Back to the question about if it was frustrating to be involved in a blind drug trial. When Emmett was diagnosed his strength was declining at a very drastic rate. It was beyond alarming as in September 2015 and early October 2015 we were all terrified of what faced this fragile little one so when the only potential hope offered was an experimental drug study without knowing if he would get the drug or not – or if the drug would even work – with assurances from Dr. Selby that they, Alisha and Josh, were in the position of control with Emmett’s procedures and could withdraw if they felt it was too challenging for him, they literally jumped for the opportunity. Parents do everything for their kids – that’s just how it is. Emmett was stable but very weak when he began the trial. Between 4 and 6 months he lost a lot of movement. With the trial injections, we saw him showing signs of improvement. However, we also saw his respiratory system continue to drop over the first few weeks of the treatment. Small signs of improvement yet also signs that the disease was still taking more away from his tiny body. Yet, we were witnessing a miracle and while no one could tell us that Emmett was on the actual drug that first year, as weeks turned to months we knew. (In fact, as Emmett is now in the second year of the study where we know he is getting the drug – we are still blind about the first year. We assume he was, but as the study Emmett is participating in has been extended we still do not know for certain about year one.) Improvement is an amazing adjective for a genetic disease that usually only gets to use adjectives like decline and, hopefully, stabilized. Up until now, improvement has simply not been used with little ones dealt the genetic cards that have given them Spinal Muscular Atrophy Type 1 (or Type 2, 3, or 4). SMA is the Number 1 genetic cause of death for a reason. It takes beautiful little babies away from their families. As our family saw Emmett improving, we had to deal with the grief and difficulty that other families were having as their little ones lost the battle with SMA. Words do not describe this difficult challenge. We simply thank them for supporting us as we sought to support them.
Unfortunately, that is the harsh reality of a drug trial. All of the parents knew the risk. All knew that their little ones had a significantly high risk of death without the trial – and this trial has now already resulted in many children now getting the drug. In fact, at least three older kids that Emmett has seen at Canuck Place will be getting Nusinersen, now called Spinraza, starting early in the new year. This is exciting. There is still much sadness, however, as in our FaceBook extended SMA family we read of little ones’ deaths every week. Parents contacting us about the drug – some able to access it but others not. Excitement on one hand for friends getting the drug and sorrow for those do not. And then there are Emmett’s friends who were in the trial but did not survive. Emmett didn’t get to know them – none of them were strong enough for playdates, Emmett included – and that is so very sad. But at one time or another Emmett was at BC Children’s Hospital at the same time as they were. I don’t recall if all four were ever there all at the same time – but I think it happened. We will tell Emmett about these friends of his – close friends that he never got to have over for playdates. The twin sister’s Emma and Addison and the little boy from the Okanagan, Atticus! Obviously, we don’t know any of the details – if it was because they got the placebo or if there were other complications. But we celebrate their lives and the fact that they helped bring this drug to the world. When we talk with Emmett about these three little angels, we will marvel about the similarities in their names. We will likely share some tears too.
Emma, Addison, and Atticus! You are heroes and we miss you. All of us supporting Emmett are thinking of each of you and your families.
We must cure SMA!
Merry Christmas
Sunday, December 25, 2016
Merry Christmas from the Willms’ Family! Happy New Year too!
Emmett loves Christmas. It is a time of wonder. A time of colour and a bit of extravagance. A time to talk about Baby Jesus. The tree is awesome and when it first went up he had mom, dad, or whoever was close by pull the decorations off one by one so that he could touch them and then get them back on the tree. And then he went through the ritual again! He loves looking at nativity scenes and is especially intrigued by the baby Jesus. Presents … we’ll have to get back to you on that because we don’t know yet. Pam got an idea from the internet about using ribbons as something for him to grab onto and rip open the present. Great idea – we’ll have to see how it works. And in keeping with Emmett’s needs, I used a tenth of the scotch tape that I would normally use to seal up presents. Emmett is still not sure about the snow, however, and while he does eventually warm up to it, his first reaction is that of tears.
Reasons for not liking the snow? First of all, it’s cold. All little kids are affected by the cold more than adults. Then you bundle them all up and they can barely move … well, bundle Emmett up and guaranteed he cannot move! Second, power wheelchairs don’t travel well in snow. Makes sense, but it also means that Emmett’s movements are limited. Here in beautiful BC we’re now into the third week of snow – unexpected and unusual. Third, a combination of the first two – you cannot fit into a wheelchair all bundled up. When getting strapped into the car bed for transportation all bulky outerwear is to be removed – meaning you get a shock of cold. Three good reasons for Emmett not to like snow. The best reason for this pic is the little tear that is rolling down from Emmett’s eye! But don’t worry, he was laughing soon afterwards. By the way, Abbotsford is now into the third week of having snow. Locals are in shock. Right now it looks like it will be a white Christmas.
Emmett is surrounded by people who love and support him – and want him to get into the Christmas spirit. But Emmett does not like weird things on his head! Give him a jaunty cap and he’ll flaunt it everywhere. Put a Santa hat on him and the faces he makes are awesome in revealing his distress. This little boy is a “clothes horse” whose fashion sense dictates what naughty or nice … and a Santa hat is pure distress! I wonder what he would do if he were to meet Santa Claus! We won’t find that out this year – too many germs hanging out around Santa Claus displays so they will get a wide berth!
In an earlier post we commented how Emmett not only loved his Christmas tree, but that he was fascinated by nativity scenes. Here at Grandma’s house he was taking his chair closer and closer to her Charlie Brown’s Christmas nativity scene. Then he’d wander off and explore the house, but then back to the nativity scene. I think Alisha and Josh were worried that he was going to knock them over, so Josh picked up the pieces and let Emmett get a closer look. Snoopy dressed up as a lamb is really pretty cool! It is very interesting to note that last year Emmett’s favourite Christmas toy was a feather (a bunch of feathers, actually) because it was easy for him to pick up and play with. This year Emmett only grabs a feather to tease Wendell and, when Wendell steals the feather, he cries. His favourite toys are toy cars and airplanes that he loves to pick up. He has even held Baby Jesus from the nativity set (I’m not sure if Grandma knows that!). His head control is so much better and his head is rarely resting on his chair’s head support.
Of course, it isn’t truly a family gathering without scaring a little one. Purposely! Just like knowing that a Santa hat will cause grief, we have all learned that while Emmett loves a partially deflated giant Nemo the clownfish balloon the opposite is also true. When Nemo is newly filled he causes Emmett slight consternation. When Nemo is activated and swimming around the house, then Emmett fusses and panics. In his chair he will try to get away. Of course, if his mom is driving Nemo/swimming Nemo/flying Nemo (not that I’m saying that Alisha is purposefully chasing her son’s wheelchair with a giant, remote-controlled clownfish that she knows causes him to be afraid) there’s not a place of safety anywhere until Grandpa takes the remote away from her! And threatens her with a time out!
Emmett loves Wendell, his early Christmas present. They are absolutely best friends – at least, as long as Wendell doesn’t invade Emmett’s space and control his private property. As Wendell is a cat, you know that he views other’s private sanctuaries as his own. Battles result.
Of course, a Christmas blog wouldn’t be complete without a video of Emmett in water showing off his moves. Of course, while he will usually spin around in multiple 360° circles – as he knew the camera was on there was not as much action as normal. Emmett was recently at the public pool, and he just floated. He was mesmerized by all of the sounds, lights, and actions of other swimmers.
The USA Food and Drug Administration APPROVED Spinraza on Friday, December 23, 2016. This is the amazing drug that Emmett has been receiving (it has had three names since we first heard about it) and now, when a little one is diagnosed with Spinal Muscular Atrophy their parents will be given an option for medical treatment. This is huge and it is the first drug approved to fight SMA! What an amazing Christmas present!
This photo has Emmett with his Grandma and Grandpa Willms and his Great Grandma and Great Grandpa Willms! Special times!
Josh, Emmett, Alisha, and Wendell (the cat) wish you a very Merry Christmas. Thank you for your support, prayers, and interest in how Emmett is doing. We wish you the very best in 2017!