July 2017

There is nothing like a hug and in the last few weeks Emmett has started to give hugs! Another miracle first.

Emmett had another great trip to BC Children's Hospital (July 19th) which included a couple of nights stay at the amazing Canuck Place Children's hospice. Some parents of SMA children are posting that they've had their first, second or whatever number of injections of Spinraza their little one has had. We were going to post a number and then we realized that we do not know. It is not that this isn't important to us, because it truly is. The fact is that Alisha and Josh were intentionally kept in the dark about this because Emmett was part of a blind study that, for him, started in the fall of 2015. And even though they have been told that Emmett has been receiving the drug from the beginning, this study with Emmett has included some 'sham' procedures so we simply don't know. But we did ask and we will find out.

This was also the first time that Emmett's family was able to be present for the injection. While the honour of being present for a lumbar puncture may not be what every parent wants to attend, it was important to be there for Emmett. Of course, the doctors and nurses are all so caring and gentle with Emmett. The Doctor doing the procedure was one of the specialists who initially diagnosed Emmett and it has been fantastic to have him following Emmett throughout. He has been present for all of Emmett's procedures to date. The good thing about family being present was that the procedure was not as bad as what imagination leads you to believe. What Emmett disliked the most was the position that he had to be held in. Phew! Imagination is so much worse than real life - sometimes.

Prior to the lumbar puncture Emmett went through a morning full of tests and appointments. A physiotherapy session to do a standardized series of tests mandatory for all patients in the study. We were thrilled to see Emmett show off and also trying to encourage him to do things that we know that he can absolutely do at home! But of course, we couldn't do anything that would throw off the standardized testing so Emmett left some of his 'capabilities' on the table. For next time. At six months of age Emmett's physio study had quite a few zero's because he simply had no muscle reaction. You've heard us say he was a floppy baby, well, he absolutely was and the brain's messages simply never got through and the muscles just sat there. This time around Emmett was getting some great scores. In fact, if he continues on this pathway in the next year (maybe a bit sooner) he is going to pass this assessment level and be given a more challenging set of tests. That will be both exciting and challenging, as we know that it will take some time for him to develop the nerve pathways and strength to accomplish these more challenging tests. From the Physio study he went off to another section of the hospital where a nerve study was conducted. This  is one session that Emmett is always happy to leave behind as they 'stimulate' the nerves and it is not something that he enjoys. They loved seeing him and were very happy with what they saw through the testing.

Throughout the course of the day we saw two of the study coordinators a couple of times and, as they have also been following Emmett from almost the time of his diagnosis, it is fantastic to see their reaction as Emmett moves about. The study coordinators (there are two - one who was blinded from the testing just like us and another who was unblinded and very aware of the process throughout. But I haven't asked for permission to say their names so right now you are going to have to stay in the dark about who they are!) are such key members of our Emmett medical team and we love them. Of course, Emmett saw his neurologist a couple of times for assessment before and after the lumbar puncture. His specialist was so pleased with the performance that this little boy was exhibiting with his muscle tone. In fact, just before he was discharged all of us (except Emmett) giggled when he held his arm bent while his doctor was trying to extend it! Usually you want your child to cooperate with their specialist, but when your little one who usually has no strength is fighting to keep the Dr. from straightening his arm, you simply have to cheer him on! We're pretty sure that he did all of the other assessments that were completed, but at the end of the day he simply was done and there was no way that he was going to allow his arm to be straightened. And he demonstrated his strength and will power by not letting it happen!

Just holding Emmett now, you can feel the muscle tone in his body. There is muscle tone throughout his core. You can feel him flexing his leg muscles and, as evidenced in the pics in the last blog, watch him lift his legs. Tightening his core tummy and butt muscles - even lifting his butt from the floor mat. And of course his neck muscles are getting stronger and stronger. It is so fantastic to watch. And feel. Like this next paragraph.

The latest milestone that Emmett has accomplished is that he is giving hugs. Alisha and Josh have never, until recently, had a hug from their little darling boy. The first one to get a hug was Alisha and Emmett 'finger crawled' (see the photo and his crawling fingers!) his arms up her shoulder and around her neck. Talk about an earth moving and teary eyed moment, this was one! Getting a hug from Emmett - simply wow! He gives them out very sparingly.

Other news was the very huge update that Emmett will continue in this drug study for another five years. What is so momentous about this is that while the drug, Spinraza, was recently approved for use in Canada it is still too early to know how this extremely expensive drug will be covered. According to the financial website "The Motley Fool", Spinraza is the third most expensive drug in the world [https://www.fool.com/investing/2017/04/18/the-7-most-expensive-prescription-drugs-in-the-wor.aspx].  This is beyond the ability of a young family to handle thus the knowledge that the testing is ongoing into the 2020's is fantastic. By then we hope that the province and insurers will have the funding question sorted out.

The trips to Vancouver are numerous so it is wonderful that Emmett lives as close to the city as he does. It looks like he will have one or more appointment in the city every week through the month of August and then a few in September also. The wheelchair van that Alisha and Josh purchased last summer has been an absolute gift. There is no way to transport people along with all of Emmett's equipment, not to mention his power chair, in a vehicle any smaller than a van. Alisha's mind is an absolute whirlwind of lists and checks as she gets ready for a trip to grandma and grandpas for the evening - so you can imagine when it's an overnight trip to the hospital. And on this most recent trip Emmett's nasal mask on his BiPap broke. Just from normal wear and tear but it is something that has never broken before so the spare had been left at home. Ironically, Alisha has had a spare one for every other trip except this one! This BiPap breathing machine provides Emmett with support probably 12 to 15 hours daily during a normal day. Longer if he's having breathing challenges so absolutely a must have after a significant hospital procedure like this. After a lot of calls and moments of panic one was located at BC Children's hospital so all was well (and saved us a 3 hour trip to Abbotsford and back). And another  "cannot leave home without it" item was added to Alisha's mental and physical checklist of what is necessary to keep her little boy safe and secure.

Of course, the safe haven called Canuck Place is simply wonderful. This majestic old mansion has an interesting history with a few twists and turns, but it is such a wonderfully caring place for medically fragile little ones. There are beautiful gardens, accessible play areas, and wheel chair accessible pathways for Emmett to visit and explore. They have an awesome fish tank that he loves to watch. Poppy the service dog is a great favourite - although Emmett is still rather reserved around Poppy. He loved counting the bees that were visiting the flowers - we cannot make out the numbers yet but his pointing and verbal intonation clearly indicated what he was doing. The awesome staff there cheers Emmett on and, once he moves past his bashful mode, he is quite a showman and a chatterbox. It is impossible to say just how important all of Emmett's caregiver's and medical team are to us. From the bottom of our hearts, thank you so much for everything you do to support Emmett. And to all of you who are supporting Emmett on his journey, thank you for following along. And don't forget, many of the photos will expand when you click on them.

The next adventure - Emmett is going camping in the wilds of B.C.'s mountain country ... well, Grandma and Grandpa Willms' driveway will seem like mountain country to Emmett!