Just recently one of Emmett's parents noticed that Emmett was sleeping on his back, a position that they usually avoid as his drool cannot drain out by gravity. Wondering why the other had left him like this, they went in and rearranged him. You see, while Emmett has gained a lot of movement that he demonstrates in his daily activities, in his sleep or semi-sleep all you observe is a shape that doesn't move. Or you hear "Other side. OTHER SIDE. OTHER SIDE!", which can get quite loud as Emmett's voice and lung power are gaining strength. Every couple of hours he needs to be moved to ensure that he has a change of position. Well, back to that slight frustration about positioning which quickly changed to wonder as they watched Emmett, in his sleep, roll himself back onto his back. In his sleep. Simply Wow! Way back in the blog we described the nervous system as being like the 401 highway - the largest highway in the world - with massive traffic travelling on it while SMA was a blockage that, with Type 1, shut the highway right down. In fact, SMA Type 1 would be like a giant sinkhole that swallowed up the entire highway and all alternate routes. Traffic literally stops moving. And until 2016 that is where the disease was left - traffic possibly at a standstill. Then came Spinraza, a drug that started traffic moving again. It wasn't as good but if you've ever been stuck in a traffic jam for hours at a time then you know how good it is to see movement. And slowly there is more and more movement.
It was in the August of 2015 that Emmett was diagnosed with Spinal Muscular Atrophy, Type 1. Ironically, August is traditionally the month in which CureSMA Canada and CureSMA (USA) focus upon raising awareness of this genetic disease. It is recognized as the number one genetic disease that takes the lives of children under the age of two. This is a disease that is sometimes (and mistakenly) described as being a 'Lou Gehrig's disease' (or Amyotrophic Lateral Sclerosis) for kids. Neither SMA nor ALS are contagious. There are obvious similarities - in both diseases the brain is unable to communicate with the muscles which causes the individual to lose the ability to walk, talk, eat, swallow and breathe. In the movie "The Theory of Everything" you see the dramatic changes that ALS imposed on the young and healthy Stephen Hawking as a university student. And the incredible health and mobility challenges that affected his adult life. There is no cure for ALS and 80% with this diagnosis die within five years. There is only limited treatment for ALS. This is where the similarities end. There is no specific cause of ALS; it can strike anyone although most often between ages 40 and 60. SMA has a definite genetic cause. SMA primarily affects children. There was no approved treatment for SMA until December 2016 when the US FDA approved it. Canada approved it in June 2017 but access is still limited with some provinces providing greater access than others.
We do not have a movie depicting Emmetts struggle although you can follow a significant history though this blog which began in 2015 shortly after his diagnosis. The cause of SMA is almost 100% when two carriers have a child - they have a 1 in 4 chance their child having SMA. While there was no medically approved treatment available when Emmett diagnosed, he was quickly involved in a world wide study of 124 babies with the genetic disease Spinal Muscular Atrophy,
Type 1. This drug, trademark name Spinraza and study name Nusinersen is an antisense oligonucleotide (that's for the scientific types reading this). He was one of four precious little ones with SMA Type 1 involved in this Canadian study at BC Children's Hospital. He is also the only one of the four who survived. At six and seven months of age Emmett had lost almost all ability to move. Prior to this drug, Emmett's loss of ability may have slowed or stopped, but individuals with SMA did not improve. Period. Emmett could no longer travel in a normal car seat as the position would cause him to choke on his own saliva. He had to use a specially approved car bed for transportation; and in most activities he would need to be prone. Emmett was never able to sit up on his own nor could he sit up without being held up. He was never able to support his own head. By three months of age he had lost the ability to move his legs. At six months of age he had lost almost all arm movement. At nine months of age he was on a bi-pap machine to assist his breathing - this equipment pushes air into his lungs to expand them and then has a lower pressure that allows him to exhale (different from a cpap machine which is one constant pressure and for Emmett would make him work too hard to exhale).
This blog has said many times before that we knew Emmett was not on the placebo because we started seeing signs of improvement almost immediately. To say that this experimental drug stopped the regression of this disease is an understatement - it reversed it. Obviously, it is a long process and we do not know where this will lead. We do know that infants who have received this drug pre-symptomatically appear to be developing with few symptoms of SMA. Another new treatment is the apparently one time injection of Zolgensma (onasemnogene abeparvovec-xioi for those
who need to know) that has already been approved in the USA - this treatment appears to offer even greater potential. However, back to Emmett and Spinraza. It has not been an easy haul for Emmett and his family. That may be partially the fault of the blog as we tend to highlight the forward steps that Emmett makes, but there have been health scares and hospital stays. Fortunately, the past year and a half have been so much better but a simple cold can still cause the entire household to go into a crisis prevention mode. Emmett's lungs are stronger and so is his ability to clear his own saliva. Emmett is in the beginning stages of learning how to swallow again. He has been tube fed for four years so this is going to be a slow process about which we are all cautiously optimistic.
Emmett just spent several days attending Kid's Camp at his church. He has been getting out more and interacting with other kids, including Sunday school almost every Sunday. It is easier in the summer as flu and cold season is not surrounding us. It doesn't mean that caution is tossed to the wind; but it does mean that Emmett can explore and interact. Playing Duck, Duck, Goose - Emmett would roll around the circle calling duck or goose while I tapped the others on their head. When he called Goose, Emmett needed the kids to circle the group twice to his once in order to make it close to even. It worked! Emmett had a lot of fun playing this and games like "Red Light/Green Light or What Time is it Mr./Mrs. Wolf". Obviously the other kids would look while Emmett was being suctioned or his feeding tube was connected/flushed/disconnected. Adults look too! Shortly Emmett will be heading off to SMA camp with his mom and dad. There they will have several days of interacting with other families that are also dealing with SMA. And Emmett, who was at this family camp for two days last year, is already looking forward to spending time with other kids in wheelchairs. Just like him. He is not, however, looking forward to the mosquito's!
Emmett loves going fast! In his van down a hill. Going fast and high on his swing! Down hills in his power chair. Down hills on his pedal bike especially when Grandpa lets go so that he is on his own and everyone else is freaking out that this little boy is going to crash but fortunately he didn't because his grandpa grabbed the rear controls in time to avert such a catastrophe which was never going to happen because everything was under control all along! Phew! But not just going fast physically, but thinking fast. In Sunday School we were watching a video with a group of kids ages 4 to 10 and, during one section with humour geared more for the adults observing, Emmett giggled. There wasn't a sound from anyone else. His mom and dad having a conversation spelling words to avoid detection only to have Emmett join in the conversation with a response that indicated he was fully aware of what was being said. He rolls his eyes like a teenager - especially when grandpa suggests something that he considers unworthy of a response.
Back to our comparison to the super highway; Spinraza is slowly but surely allowing this little boy to regain strength and mobility that he has lost. We are realistic and understand that Emmett is always going to need support. We are also not giving up on miracles! Every roll and turn and lift is a miracle. Did we tell you how Emmett lifted his hand and held it out the van window! The look on his face was incredible. Emmett will finally be getting a kid's walker/gait trainer. He just got a lift in his bedroom that will make it easier on mom and dad to move Emmett about. His
second hand wheelchair has been very reliable this summer with the 'MacGyver' fixes but plans are underway for a new model. Bathroom renovations to be more accessible are still in the planning phase. And Wendell the cat got sick and had to go to the vet. He's back home now and recuperating. All of this is just a fact of life when living with Spinal Muscular Atrophy. (Wendell and Emmett are best friends, so Wendell the cat being sick was pretty challenging!) Emmett doesn't go anywhere without his suction machine. He doesn't go anywhere far without his bi-pap. Sneeze and Emmett will tell you to sanitize. Even with these new, exotic and expensive drugs that are a miracle, Spinal Muscular Atrophy is a terribly challenging disease. August is Spinal Muscular Atrophy awareness month. Want to know more, just ask.
After watching the bike video, now go back with us to 2016 and watch a video clip from that era.
It's the Little Things!
When you say the phrase Lumbar Puncture out loud, does it make shivers go up and down your spine. Because it does to me. Literally, I just cringe and yet I have never had one done. Today Emmett will receive his 15th lumbar puncture where the specialists will remove a tiny amount of spinal fluid and replace it with an equal amount of fluid that contains the drug Spinraza.
Emmett was in really high spirits yesterday. There were a few tests at BC Children's Hospital with more this morning. Some of the tests today are not ones that he appreciates and he seriously doesn't like. He knows what is going to happen. I seriously think that he looks forward to it ... BUT he also knows that it is going to hurt. And that causes his anxiety and apprehension to build. That is normal. Hang in there Emmett, we're so supporting you! *N.B., All went well and Emmett is back home! Plus he got to watch Cars 3 and Mary Poppins Returns!
Emmett was just camping at Alice Lake Provincial Park. This was his second time camping there in RV who he calls RV (stands for recreational vehicle - our motorhome who Emmett sometimes makes talk) and he has literally been waiting all winter for this to happen. Even better was that Grandma Rhonda and Grandpa Roger were there with their RT (recreational trailer - so Emmett can easily identify and differentiate between the two). Emmett had so much fun that we'll talk about in just a bit. Right now it is time to talk about mosquitos. First off, you need to know that in an amazing twist of nature that there are really not that many mosquitos in the lower mainland area of British Columbia. It is important to say that so that people in the rest of the country can be even more envious of this geographical area which truly is God's playground (you'll see some of that in some upcoming photos). However, while there are few mosquitos all it takes is one and while camping Emmett got three mosquito bites. One on his tummy, one on his shin, and one on his forehead. You need to know that for some reason a lot of individuals with Spinal Muscular Atrophy react adversely to mosquito bites. That is Emmett. A bite causes him to have the area swell up, often break out in blisters, and be incredibly sore and painful. In fact, a mosquito bite is literally worse for Emmett than a Lumbar Puncture. Don't believe me! Then stay in the RV with Emmett overnight when he has had a mosquito bite. Oh my goodness! The pain and agony that this little guy has to endure from a mosquito bite is one that he ensures that everyone within earshot also endures. It is intense!
Soothing cream. Antihistamine. Cool cloths. Ice Packs. Heaven help you if you move that ice pack in the middle of the night because then all four adults (the tent got rained out so it was four of us plus Emmett) heard the very loud cries to get that "ICE PACK!" back on that mosquito bite. If it wasn't that Emmett was really uncomfortable and none of us were getting any sleep, it would have been rather humorous. As much as Emmett doesn't like getting Lumbar Punctures, and he obviously gets upset and says "Ouch" many times before and after the L.P., he truly is incredibly stoic about the entire procedure. While it slows him down for a day or two, within hours he is his normal happy self. In fact, one of his play stations is a hospital setup where he has been known to perform lumbar punctures. In comparison, while he plays RV an incredible amount of time throughout the year, I don't think he's ever included mosquitos in his play time! Fair to say that he really and intensely dislikes mosquitos! (Obviously, while posing for a photo while daytime it was time for Emmett the photo ham to pose for his audience.) When talking with Emmett just hours after the Lumbar Puncture we asked Emmett which was worse, the blood test (which he also doesn't like) and Lumbar Puncture OR a mosquito bite? With the logic that only a four year old can have, he looked at me like I was silly (I'm often referred to as silly grandpa) and told me that there were no mosquitos there today! LOL! But truly it is the little things, like that mosquito bite, that can sometimes just push you too far.
Mosquito bites are bad! So are Lumbar Punctures! Emmett, we are praying for you today. Thinking about you today. Bounce back quickly and we're looking forward to seeing what your next adventure will be. *N.B., Emmett has bounced back and is happy to be home.
Now to highlight some fantastic summer moments: Alice Lake Provincial Park is a campground that Emmett literally hasn't stopped talking about since his camping experience there last June. This camp is a preferred one for this family because it has full mobile phone coverage, has electrical hookups, is just outside of Squamish so in a beautiful area that is close to Vancouver, and the park roads are paved while the main paths are wheelchair accessible. All are important - but mobile phone coverage, electricity, and near Vancouver are all essential "Just in Case" items. You see, when you have a medically fragile child everything that you do has a "just in case" clause. Once you're relatively satisfied that the "just in case" scenarios are covered then you can go out and enjoy life. You build memories. You take some calculated risks to ensure that your little one is living life to the fullest. Building memories of camping, campfires, marshmallows, fishing, and canoeing that will last a lifetime. Even mosquitos add to these memories with new experiences. The bite on his shin which blistered has now turned into a scab. Emmett really dislikes this scab - it's also the first scab he's ever had. After all, he's never fallen down - at least, not like that!
Alice Lake Provincial Park was a great trip. It was cool and wet - but fortunately most of the rain took place at night. A trip to the Sea to Sky Gondola, where his Aunt Katie and Uncle Josh got married three years ago in July, was very special for all of us. While Emmett doesn't really remember the wedding (he was only one), he will certainly remember this trip!
You can click on any of the pics to get larger ones!
Necessity is the creator of many inventions. Another instigator is that so many things are so unreasonably priced that one has to find another solution. With us, that has led to the creation that was actually a 'stolen' idea (perhaps borrowed idea is a better thought) of the 'Bumbo chair'. Emmett loves it but has seriously outgrown this cool device. Then there was the 'Legzerciser 2018' to help him move his legs about. It also has been outgrown and his need for it reduced. There is the backup power supply, which will provide a couple of days of electricity for medical equipment and will always be very useful.
The latest is a wheelchair seat cooler which is absolutely needed for hot summer days. As Emmett cannot twist and turn to shift positions, he is literally planted in one position in a hot, black wheelchair seat. As much as he loves his wheelchair, the heat is sometimes just too much and he is drenched with sweat. This is the current challenge - wondering how to cool down a little boy who gets drenched with sweat in his power chair during the hot days of summer. There are cooling pads that you can leave overnight in a fridge or freezer / immerse in water and then place on the seat - they do help - but they have a limited window of effectiveness. There are cooling shirts and vests that you can purchase - they are rather expensive. And then there is ingenuity. For about $60 we have created a cooling pad that effectively assists in keeping Emmett's seat cool.
Get an aquarium pump, a battery, some pipe fittings, tubing, and some material and 'Presto!' - you have a seat cooling pad that costs about $60. Less if you have some of the materials sitting unused around your house.
Here's what we did.
1. First we found an aquarium pump. Local pet supply stores weren't able to help with the battery powered requirements so we turned to Amazon and found a Robolife Mini Brushless 'Submersible' Water Pump. Having the pump submersed just makes it easier. Having it DC powered is a must as you don't want to be trailing an extension cord along behind the wheelchair. And don't limit this to a wheelchair, it could be almost anything. This is a small pump and we were concerned that it might not be able to push water through the 25' of tubing that we used. The flow is reduced when the 25' is compared to just a couple of feet, but we'll let you know how that tiny pump handles the ongoing pressure. UPDATE: it was a 27°C day and the pump and seat cooler performed just as we'd hoped.
2 Month Update: the pump quit so we're trying a different type. It will be arriving shortly and we'll update this with more details. But this does work, this is just a small kink in our project!
2. This pump had a 5/16" inlet and outlet. That piping was simply too big to be comfortably sitting on for any length of time. I couldn't find a pump with a smaller setup so went to a local auto parts store and purchased some reducers so was able to take that 5/16" outlet down to a 1/8" outlet. That should be better for long term seating applications - we will let you know. First trials there were no indications of pressure marks.
3. The adaptors we found were brass and they were ideal to secure in the lid of the plastic container we are using as a water container. It's a snap lid type of container that we hope will reduce the number of spills! We drilled three holes in the lid. The first hole was one that we could fit the brass adaptor into. This hole was drilled so that the fitting actually had to be threaded into the lid (again, will stop water leaks especially when coupled with a bit of silicone). Next we drilled about a 1/4" hole so that the return water tubing could friction fit through this hole. The third hole was a very tiny hole to fit the power lines through. The container we got is about is about 8" by 5" and 4" deep. Again, we will have to let you know if that is large enough to keep cool for long periods of time. The good thing about this is that you can switch to a larger container at any time for more cooling capacity for those really hot summer days.
4. To connect the motor to the battery we chose simple alligator clips. That way you can just connect and disconnect in an instant. Rather than installing an on/off toggle switch we are just disconnecting the alligator clip. Note that the motor requires consistent power so you have to know which is your battery's positive and negative connection. To make life simple - mark them. Red on red and black on black. Or do what we did which was tape the black one into place to make it impossible to mix up.
5. The choice of battery size we are still experimenting with. We have a 6 volt 'flashlight' battery that does power our pump. It's light and easy to move about. Internet research indicates that this 6 volt battery will power the 12 volt pump but at a slower speed thus obviously pumping less volume. Then we have a larger 12 volt battery which, even though it's small, is rather heavy. The advantage of this is that it can be recharged with a standard automotive battery charger. This makes an obvious difference in the water flow and speed. Again, this part is trial and error as we don't know if the 6 volt battery will cause more wear and tear on the motor thus premature failure.
Update: July 27th - the 6 volt battery drains very quickly so unless you really need lightweight and don't mind the ongoing expense, buy the larger and rechargeable 12 volt battery. More expensive to start but cheaper in the long run BUT you will need a battery charger to maintain it!
6. Mounting the pump was much simpler than I expected. It literally hangs off of the brass adaptors that are threaded into the container lid. Time will tell if this will need to change, but right now it is working. Obviously, you need to ensure that the container has enough water in it that the pump does not start to suck air when you are on uneven ground. Try to have the pump located as close to the bottom of the container as possible, without touching. Just because touching will likely increase the motor's noise level. Notice that the return tubing is not connected to the motor but just returns the water to the container. The motor inlet is left open for the water to enter.
7. Alisha then sewed a rectangle shape with columns that are about 1.5" apart. The ends were not closed. This allowed the tubing to serpentine through the pad. As it's flexible, no couplers were required (couplers would be horrible to sit on). We made it large enough so that it will wrap around his body to provide some cooling on the sides also. For our situation, that meant that the fabric was about 28" long and 14" wide.
8. In addition to sewing the sides from top to bottom, then columns were sewn into the fabric that we kept 1.5" apart. The top and bottom were left open to provide access to threading the tubing through the columns. The columns were started about 1.5" down from the fabric and ended 1.5" from the other end. The reason for this is to provide room for the tubing to bend and enter the next column.
9. Hopefully you will have a helper. Feeding the tubing through the columns goes much easier when you have multiple sets of hands to work on this project. It also helps if the fabric material reminds you of Charlie Brown's shirt - simply because that's cool. Also, choosing a light colour will assist in the entire aspect of cooling (but it will need to be washable as it will stain easier than a black wheelchair seat.
10. We had a used soft-sided cooler that was a perfect fit for out snap top container and either one of the batteries. This is then hung off the back of the power chair. We still had enough room for a food pump/bag and the suction machine. It would be a challenge to hang a spare suction machine but if required then we're sure you can figure out a way to make that happen.
11. Here's a view from the top and from the back of the chair. We have the inlet and return tubing both coming out of the top of the pad and then into the snap-top container. We've started out using the 6 volt battery to test the effectiveness of this lightweight battery. You'll notice that we taped one of the alligator clips to the battery. This is to ensure that the +ve and -ve clips don't touch but also makes it easier to avoid connecting it wrong (and it won't work if the polarity is reversed).
If it is a really hot day, then add more ice to the container (or swap to a larger container). The capacity is only limited by the space you have available. Cooling is only limited by the availability of ice (fast food restaurants are a good source).
Motor: $15-$35, Battery: $12 (for the 6 volt or $25/$50 for a 12 volt), Tubing: $18 (there is cheaper stuff available, this is more expensive but heavy duty automotive tubing that should outlast the chair. Not that that is a challenging feat as chairs don't last that long!), Connectors: $4, Alligator clips: $3, Snap top container: $7.50
Click on the photos to get an enlarged view (except for the photos of Alisha sewing, I was not even supposed to take those photos so you cannot get enlargements of those).
Emmett has been playing RV all winter and spring. In just a little bit Emmett and his mom and dad are going camping with both sets of grandparents. It will be an awesome time for this little one who so loves camping and time in the RV. We will give you an update on camping in our next blog post. This post is about Emmett and his athletic escapades. First off check out Emmett as he expresses an interest in baseball. In previous years he has come to games but hasn't shown any interest. Not so this year. So his daddy, Uncle Ben, Aunt Alesha, and a bunch of friends from Level Grounder's baseball game helped him experience a game.
Emmett has also been really working hard at sitting up. And that includes swinging!
Sometimes kids ask Emmett why his mouth is open. That is something that he wants to show you that he can now close, but he wants you to know that it takes a lot of muscles and a lot of work to keep his mouth closed. (We love his expression!) So Emmett will often have his mouth open! That's just one of the little challenges that SMA causes.
Emmett wants to be a helper when he grows up. Here he is checking out Cliff to ensure that he is healthy and happy. Emmett also likes going outside for picnics with Ted E. Bear. As long as he doesn't have to touch grass. Emmett still doesn't like the feel of grass and is not really sure that he appreciated when his grandpa (yes, that would be me) took him out and lay him down on the grass. First to look up at the sky and see shapes in the clouds. Then another day to lay down on a hill to watch grandpa roll down a hill ... however since grandpa lay Emmett down on the grass on the hill it was actually Emmett who rolled down. And then when I picked him up and set him down again, it was still on the hill so he rolled down again! And it was awesome to hear him giggle. But if you ask him, he will tell you that he doesn't want to do that again (but he did whisper to me that I could roll him on the hill again, he just won't admit it out loud! Ha ha ha!)
So Emmett has decided that he wants to be a helper when he gets bigger. I have to tell you that he already is a helper. He helps everyone who spends time with him appreciate life so much more. When you see his expression as he realizes that he has just completed something that he has never done before, you cannot help but appreciate the everyday things that you do without even thinking about it. Simple things like closing your mouth and holding your lips together! When you hear him giggle as you climb up creative playground equipment and then slide down the slide - or maybe even going down one of those twisty, enclosed tube slides with your grandson and getting stuck in the middle of it (and hoping that you get out before Alisha or Josh find you there with their son!) as he laughs and calls out 'Again! Again!), then you find enjoyment in so many more things. (Emmett and I weren't really stuck, I just stopped us so that I could rearrange both of us so that we exited the slide in a dignified manner.)
Emmett loves his cat, but there's certainly something wonderful about being able to take Lucy, Grandma Willms' dog, for a stroll! Emmett absolutely loves animals.
Sometimes Emmett watches baseball from right behind the backstop netting. Other times he sits with his mom and grandma and friends. Other times he forgets about baseball and heads over to the playground to do some playing. Here he and grandpa were testing out a spinning thing. Grandpa got dizzy. His friends wondered why he couldn't walk and needed a wheelchair. This spring Emmett has been going to Sunday School and playing with the kids. It's a great opportunity for him to get out! Life is a balance between heading out and exploring and staying safe. Obviously, there is sanitizer everywhere at our church and the kids are great at using it. If there is someone who is obviously dealing with a cold, then we have to leave or steer clear. The church sent out a lovely letter to all of the parents in the Sunday School class about how illnesses can be extremely challenging for Emmett (for that matter, for new borns and elderly and lots of people in between) as they assist us in trying to stop the spread of germs. It is a learning opportunity for all of us.
Daniel Tiger is a great incentive to stay on his tummy and hold his head up. Otherwise, he cannot see the screen to know what is happening to Daniel Tiger!
While spending some time at Canuck Place in Abbotsford, Emmett got to check out a McLaren. It got his approval! Emmett loves his wheels. In the next post we hope to tell you about the next chair modification, a seat cooler! As you can imagine, sitting in this chair during a hot summer day can get incredibly hot. Cool vests/shirts are available at a rather high cost, so we're going to see if we can build a cool seat cover for him. Stay tuned for updates on this project. Emmett's own set of wheels, his power chair, has been causing some problems. It has been in and out of the shop for repairs and technicians have been out to both Canuck Place Abbotsford and Emmett's house as they try to solve the issues. They are getting closer, but the bottom line is that this second (or third) hand chair has been well used over the years and it is either going to need a complete rebuild or Emmett is going to need a brand new chair. There is funding available for a basic setup, but Emmett is going to need so much more. For example, Emmett still requires a parental joystick at the back of the chair for things like crossing busy streets. That's optional. Being able to change the seat height from a child sized table to an adult table, that's optional. Yet these optional items are really mandatory for Emmett to participate fully in daily activities. That is so crucial as we all want Emmett to be involved in life.
There are so many people and organizations that are involved in supporting Emmett and we so appreciate them. Emmett is still part of Biogen's drug study on Spinraza and we cannot thank Biogen enough as he has been receiving this medication for 3.5 years now. This drug is truly an amazing and miraculous drug and slowly Canadian provinces are making it accessible. It is exciting to see that another even more innovative drug has just been approved in the US for treating SMA.