What's that? What's that? What's That!
Oh my goodness, we are absolutely into the threes with this three year old. And like many inquisitive youngsters, he asks "What's That!" at least a million times a day. Emmett is growing up. He no longer likes naps in the afternoon. What is life-saving for his mom and dad is that he loves (at least right now he does) quiet play time in his bed. This is also important because it is time to give his lungs some support and he gets a couple of hours of bi-pap time. It's also lunch time as he gets his mid-day meal at about one. The little show-off has started exclaiming "Mmmmmm, Mmmmmm! Delicious!" as he gets set up for his snack.
Of course, that is just silly (or as Emmett would say, 'Sissy Grandpa!' because he hasn't figured out the 'll' in silly and everyone always laughs when he says 'sissy'!) because Emmett never tastes his food. This is probably one fact that surprises people the most. They realize that Emmett is a medically fragile little one. They know that Emmett has Spinal Muscular Atrophy, type 1 (hmmm? Does the classification change as Emmett develops and improves on the drug Spinraza?). But most do not realize that Emmett has not had food of any type nor any liquid by mouth since he was 6 months old. Let's face it. People just don't think about it and we don't really talk about it. Emmett gets fed by a G-Tube so all food and liquid goes directly into his stomach. The advantages - he can keep playing while he eats. If he gets distracted, he doesn't stop eating. No one ever has to say, 'Emmett - eat your food before you leave the table!'. His food goes with him! The disadvantages - never getting ice cream or hamburger or pizza. And Emmett loves pizza! Another advantage is that instead of getting loaded up with chocolate on Easter morning, Emmett got loaded with little toys that were stuffed inside plastic egg shells. He absolutely had the most fun time during his Easter Egg hunt.
Emmett eats three times a day - similar to everyone else. He also has a slow, continuous feeding throughout the night. Obviously his mom and dad don't know if Emmett is hungry. It's hard to say if Emmett even knows what hungry means? His mom and dad monitor his intake and are obviously aware of his output. Dieticians and nutritionists monitor his weight and ensure that, as he grows, his nutritional intake is adjusted to accommodate for his physical development. Emmett loves joining everyone at the table for meals. The difference being that he almost always prefers his plastic toy food to pretend to munch on. You see, he still has a huge aversion to getting his hands dirty or sticky or covered in food. He does like playing with raw carrots - partially because he likes Moose and Robert Munsch has a book where the Moose loves to eat carrots. *Grandpa's note: literally minutes after writing about how fastidious Emmett is, he proves me wrong during a painting session. Here you have access to never before images of Emmett getting dirty! Mind you, every couple of minutes he needed to get his hands cleaned off!
Emmett's ability to control his muscles is increasing with every dose of Spinraza that he receives. He received his last dose by lumbar puncture just a few days after his third birthday. I think it was his tenth dose. Since Emmett was part of the study we really don't know - as he did go through a few sham injections to help protect the validity of the study. He is getting stronger - and he's getting to be a very big boy.
When Emmett turned one year old we wrote a blog post that we hoped would best describe in everyday language what Spinal Muscular Atrophy, Type 1 does to the body's systems. You can read that post here. A person with SMA can be an infant right through to an adult (adult onset is SMA Type 4) and that person is either missing the SMN1 gene or has a mutated version of it. This SMN1 gene produces the SMN protein that motor neurons need to be healthy. Without this protein, the motor neurons gradually come to a stop which means that messages from the brain do not get through to the muscles. As we have said, at six months of age Emmett's body was quickly shutting down. The drug Spinraza works with a similar gene, known as SMN2, to enhance this gene's ability to produce SMN protein. This amazing drug, combined with ongoing therapy and intensive intervention from his mom, dad, and a great medical team, mean that Emmett is continuing to thrive. Will he someday be able to eat one of the cookies that he helps bake? There simply isn't an answer for that, but we hope so. Just like Emmett is now sitting up by himself or holding his head completely on his own. Yes, he does need to use a back brace to help him stay upright but he is fully in charge of holding his head up. Until you have someone struggling to support their own head, you don't think about how bizarrely designed our bodies are resulting in the neck having to hold up a 5 to 11 pound head! It was just two years ago that Emmett started just lifting his own head - he has come so far and it is exciting to think about what may be next.
Those who frequent our blog often know that it is a glorified family home video time, and here in video you see Emmett painting and then outside rolling about. He can now start rolling the chair forward and backwards on his own. He can turn. And he loves sitting in it and playing catch. He will literally play for hours (of course, that means that someone else is literally playing catch for hours too!) But his excitement when he realized that there was a slight slope on his sidewalk was, well, turn up the volume and watch the video clip to see and hear what he thought about rolling on his own. Thank you for your patience with our family film night!
Then there's his language. Say the wrong thing and you're going to regret it, because even if you're in another room this little boy will hear it and repeat it perfectly. Not to say that anyone in our extended family will ever say the wrong thing. This little boy is like a parrot. But he does far more than just mimic us. He chatters and jabbers non-stop when it is his quiet time. Along with the "What's This?" we hear throughout the day to which Emmett fully expects an answer. And it had better be a reasonable one, too. For weeks as we've walked around our condo complex we have counted the numbers from 1 up to our unit 18. He really likes numbers that have a 3 but his favourite number is 8. Inside he loves playing with toys but he simply loves books. He literally can almost tell us his favourite stories. And the infamous 'Book! Book! Book!' has now been upgraded to 'Read a book ... please!' Usually the please is an afterthought or has been prompted by an adult. Regardless, it's pretty thrilling to hear the language pouring out of this little one.
This coming summer Emmett is going to be participating in Charlie and Florence's 5/10 K roll/walk/run to end SMA. It will be held on August 19, 2018 in Stanley Park. You should start making plans to join us. Or sponsor Emmett as he rolls through the 5 K. It was August 2015 that Emmett was diagnosed with Spinal Muscular Atrophy, Type 1.
Emmett loves life and he thrives on new experiences. Of course, as you realize a simple cold can mean an ICU stay for Emmett so he does not always get to do things that most kids can do. Emmett loves buses. He sees them. He hears them. And he loves them. However, riding on a public bus would be so risky due to the challenge of picking up a cold so that simply has never happened. So for part of his birthday celebration we asked BC Transit if they could help us out - thinking that one of the small handicapped busses would make Emmett's day. Well, BC Transit and First Transit Canada had a better ideal. A BIG BUS! It was fantastic. The team at First Transit Canada - BJ, James, and Calvin (to name a few) - were so enthusiastic about giving this little boy a special day. The big bus showed up and Emmett's face was one of surprise as it rounded the corner and then pulled up to a stop in front of him. Then he rode around the block! Literally, around the block. Emmett had so much fun. And he had a bunch of his people, including his cousin Henry, along on the ride with him.
BC Transit and First Transit Canada - Thank you! You have given this little boy a memory that he won't soon forget.
Emmett got to take his power chair right onto the bus. Driver Calvin ensured that Emmett and his chair were securely fastened in place. Not only does the bus lower down for entry, but it has a power ramp for incredibly easy access. Then a fully flat floor that was so easy for Emmett's chair.
First Transit Supervisor James presented Emmett with a hat, sunglasses, and his very own bus. Even better, this bus is cardboard meaning that Emmett can easily pick it up. He drove it back and forth on his lap while waiting for the bus trip to start. Along with Emmett for the ride were his mom and dad, both sets of grandparents, his Aunt Jennie and her son Henry, and his Uncle Ben. Missing from the trip were Aunt Katie and Uncle Josh (Australia) and Uncle Ben (Jennie's husband) in Alberta.
The ride around the block was superb. Emmett loved it and, back at the party room, regaled us with stories of his adventures that were combined with exaggerated head movements to show his audience just how exciting his ride was. He literally tossed his head to both sides and forwards/backwards with great enthusiasm in his retell. We want you to know, however, that Emmett's head control was really good and he handled the turns, stops, and acceleration very well. This was Emmett's first time being transported in his chair and facing forward. In his van, Emmett still uses either his car bed or his car seat. He doesn't ride in his chair - yet.
While a bus is exciting enough, Emmett went back for even more party fun. A Daniel Tiger hat, freeze dance, peek-a-boo, and pin-the-tail-on-the-donkey were just some of the incredibly exciting activities.
Paw Patrol Masks and Disney character masks were a lot of fun ... but so was an orange pie plate!
Emmett and Henry were too distracted to look at the camera. This birthday was made more special celebrating with family. It was awesome for Emmett to have his Aunt Jennie visiting from Calgary and then to chat on the phone with his Aunt Katie and Uncle Josh!
Three year old Emmett has a busy week ahead of him. Right now he is staying at Canuck Place in Vancouver - with his mom and dad - as he has a number of appointments in Vancouver at BCCH, getting his chair adjusted, and then on Wednesday it is time for another lumbar puncture and injection of Spinraza. All good things but certainly not the most fun. Emmett's questions about this hospital trip? Were they going to put tubes up his nose? What about his mouth, were they going to put a tube down his mouth? Alisha and Josh have reassured him that that won't happen this time. He doesn't have a cold or RSV or anything like that. Phew! Wednesday is the big day and Emmett, all of us are here for you and supporting you. You've got this!
EMMETT IS THREE!
Wow! Simply wow! Yesterday if you asked Emmett how old he was he was say two. When you asked him how old he would be tomorrow (which is now today) and he would respond three! Every now and then he will sing Happy Birthday to himself. And when you ask him what he wants for his birthday he will go "Ho! Ho! Ho!" because he wants Santa for his birthday. Well, unfortunately he is not going to get Santa but he is going to get something big and surprising. We will have a blog post in a few more days after his Saturday birthday party.
Today is low key. He's going to heat to the Vancouver Zoo to enjoy the animals. If he's lucky, maybe the hippopotamus will be active today! The bear close to the fence. The giraffe peering over the fence at him.
This is also a day to remember everything that has happened since his diagnosis two and a half years ago. How he had lost so much ability to move as Spinal Muscular Atrophy had such a grip on his body. And yet now he is stronger than ever. Sitting up in his Mega Seat for hours at a time playing catch and playing with his mom's doll house. Travelling in a car seat after years of only being able to travel in a car bed. Hearing his excitement as he observes trucks, construction equipment, and buses through his van window. So many positive things have happened.
Emmett has blessed our family. He is such a joyful little boy.
Ask him to pose for a photo and he'll give you a cheesy smile! Eyes closed too!
We have frequently heard from family and friends who, after reading Emmett's Journey, literally do not know what to do. That is understandable - Spinal Muscular Atrophy is a disease that defies understanding. Emmett has been receiving a drug that is now approved by Health Canada. It is now being considered by provincial governments, however, due to the drug's exceptionally high cost, the drug is only being recommended to a very small subset of patients. Our government needs to approve this drug for all - it will improve the quality of life and literally save lives. Our government also needs to negotiate a much better price for this life saving drug, Spinraza.
Want to know what to do - take a couple of minutes and sign this petition. Share it with your friends.
Want to know why? Because Emmett!
Emmett is still part of the long term study of Spinraza. This means that for him the drug is provided at no charge and this will continue for the next few years. When Emmett began this drug trial, we didn't know if he was on the real drug or a placebo. We didn't know if it would work or not. We just knew that Emmett was losing strength and capability rapidly. His intelligent eyes asked us what was happening to him. And now we see him continuing to make progress and approaching his third birthday. We want him to see many more.
And we want other families facing this rare genetic disease to have hope. Spinraza is a treatment. It is not a cure. But it is hope.
Please fill out this petition.