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 Childrens 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. 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.
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.
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 Childrens 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 ...
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 murmering '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!