Who Are The Orange Hippos? We are!
Yes, that is us. We are the Orange Hippos. If you're thinking that this is not the most flattering name, well, maybe it isn't. Some might say that it is quite appropriate - although none of us are orange. Personally, I'm incredibly proud to be an Orange Hippo. I honestly cannot think of anything better. For you see, Emmett has a lot of say in our combined households and when he chose the team name, that became our team. He had three names to choose from and I cannot even remember what the other two names were. Suffice it to say that they were not memorable. Plus, Emmett didn't like them. You may have gathered by now, that if Emmett likes and approves something, then I adore it. I'm his grandpa so I can do that!
If you've been reading the blog then you know that Emmett loves Hippos. He went through a number of animals early on, but Hippos have been a favourite for nigh unto two years. (I've always wanted to say 'nigh unto' so I deleted nearly and typed in 'nigh unto'. And now look at how many times I've used 'nigh unto'. Things like this make me happy.) Orange is his favourite colour. It has been for n ... nearly two years. Carrots are his favourite food - because they're orange, of course. They are also a lot easier to hold that peas or watermelon. Plus one of Emmett's favourite squeeze toys is a little orange hippo. So Emmett instantly chose the Orange Hippos as our team name.
You may be wondering what team this is. Well, of course, this is Emmett's Team. We are going to be walking/rolling/running in Florence and Charlie's 10K to end SMA. And we are looking for people to sponsor us - tax receipts are available. This run takes place on August 19, 2018 in Stanley Park, Vancouver. If you want to walk/roll/run with us, you're more than welcome to. We would love to have you join us! Details are in links at the bottom of this blog entry.
What is amazingly special for all of us Orange Hippo's is that it was on August 19, 2015 that Emmett was diagnosed with Spinal Muscular Atrophy, Type 1. Three years ago. Oh my goodness, that is a day that we will not forget. Ever. All of our lives changed in an instant - and we were sort of preparing ourselves for some difficult news. There is absolutely nothing that can prepare you for a five month old getting a diagnosis of Spinal Muscular Atrophy, Type 1. A disease that three years ago had no medical options for treatment. A genetic disease that took away the life of most little ones afflicted with it BEFORE they reached two years of age.
10 K is absolutely nothing compared to the fight that Emmett has had for his entire life. Emmett is looking forward to participating in this walk. He has had his power chair tuned up and the speed limiter has been raised from 50% to 75% - so he can move along at a brisk walking pace. He may tire me out! Fundraising efforts like Florence and Charlies 10K to end SMA have helped with research so that treatment, like the miracle drug Spinraza, has not only been discovered but is the ONLY approved treatment in Canada, the USA, and many other countries in the world. This drug is not a cure, it is a treatment. It is a miracle, but we want the miracle of a cure. So even a small donation to our walk and roll (look at me, did you really think that I would be running!) will help CureSMA Canada. These funds help everyone afflicted by this disease. (*These funds do not go to Emmett. He does benefit from them. These funds go to CureSMA Canada to help with research.)
The walk itself is named after Florence and Charlie, who are two little girls. Florence lost her battle with SMA. Charlie also has SMA Type 1 and is now on Spinraza and is fighting this genetic disease with everything that she has. The girls' parents started this run a few years ago and they are working hard to raise funds to find a cure for SMA. All funds raised go to CureSMA Canada. You see, there are children and young adults across our country that are fighting this disease. And now they're also fighting to get access to Spinraza, the only treatment that is available for SMA. This drug wasn't available in 2015 when Emmett was diagnosed. There was no available treatment. Emmett was lucky in that he was one of 124 kids from around the globe that was invited to participate in a blind study for an experimental drug. The drug that was eventually named Spinraza.
This, by the way, is Po. Po is the latest Hippo to join Emmett's menagerie of stuffed, rubber, and plastic hippos. What is unique about Po is his size! We have decided that Po is a grandpa hippo! Po has guarded Emmett at night. The issue with this is that Po has absolutely startled (scared the pants off, to be truthful) both Alisha and Josh when they checked up on Emmett in the evening. A dark room. A large creature hovering over Emmett. You get the picture. Emmett loves sleeping with a few choice members of his menagerie. He invited Po into his bed once and quickly kicked him out. Po is just too big!
To make a pledge for the Orange Hippos or, even better, to sign up for the run, please click on this link to the Running Room. https://www.events.runningroom.com/site/?raceId=15056, this will take you right to Florence and Charlie's 10K event. Then go to the Fundraising link. There click on "Search by Name" and type in Alisha Willms. She is Emmett's mom and our Orange Hippos' Team Captain. Then you can pledge your amount.
To sign up to participate, click on the Registration link. Select the Team 10K. Then select a username or login with your facebook account. As you work your way through the form, click on Team Member. Another field will appear where you can select the Orange Hippos. The password that you need to complete everything is
Thank you for your words of encouragement, your moral support, your prayers, and for considering sponsoring us for this worthy cause.
Emmett is a busy boy. He is constantly on the go. This month our blog is going to be a photo montage of events Emmett has participated in - things he loves doing. Enjoy! We will start off with our little show off posing for the camera. To help keep things in perspective - when Emmett was one year old and then he would have to be 'posed' for a photo. And he was in that position until you moved him out of it. Now as a three year old he is able to pose himself! What a change!
In this series of photos, Emmett is considering a career in modelling. He's holding his face to, obviously, highlight his cheekbones!
The photos above are all taken in Emmett's front yard. The blossoms have been amazing, although late, this spring. As much as possible, Emmett loves being outside!
A day trip to Harrison Lake allowed Emmett some awesome scenery and to meet new friends.
Emmett loves playing cars ...
and he loved going to the race track to cheer on his grandpa and his grandpa's car. We were quite surprised that the noise did not bother him.
The aquarium is a really great place to visit. Although the Zoo is his favourite. Emmett knows how to spell Z - O - O so his mom and dad can no longer make plans by spelling the word!
The local tulip farm is incredible. A huge thanks to Canuck Place for hosting an evening there!
One last cheesy pose from a little boy who has stolen our hearts!
Emmett has just had his power chair adjusted. Now his third gear is as fast as his fourth gear used to be. His fourth gear has jumped from the speed of a slow adult walk to a brisk adult walk. This little one is getting places faster. And that is a good thing, because Emmett is planning on going places!
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!