August 2015
March 9, 2015 is the day that Emmett Jeremiah arrived into this world. He was a beautiful little boy weighing 7 pounds 2 ounces. The delivery was spectacular. Emmett and his mom came through with flying colours. His dad was right there with them. The obstetrician commented that Emmett’s mom was amazing in the delivery room and that she should have many more kids. Emmett’s extended family fell in love with him.
Fast forward to August 18, 2015 when Emmett was just over 5 months old. He was a beautiful little boy weighing 14 pounds and quickly closing in on 15 pounds. Emmett, just like the day he was born, is in a hospital. Spinal Muscular Atrophy, Type 1! Does anyone in your family have a genetic disease? The diagnosis is unquestionable. The hospital walls echoed with words and phrases that pressed in on Alisha and Josh. They could barely breath. “Genetic testing.” “Possibly just SIX months to live.” “Maybe he could live to 4 years.” “You might hear him talk.” “Tracheotomy.” “Gastric feeding tubes.” “No treatment.” “No hope of recovery.” “Experimental study.” Emmett heard all of these words and he still smiled and giggled – he was even content when he got the blood work done – and he still brightened his parents’ world, even though they looked at him through tears in their eyes and with hearts that had just been broken and crushed.
Emmett Jeremiah had just been diagnosed with Spinal Muscular Atrophy, Type 1. No one survives this disease past age 4. No one. It is a fatal genetic disorder. There are some trial procedures going on right now, but they are limited in both availability and what they will accomplish. Our perfect little boy has a genetic condition that none of us even knew existed. Emmett’s extended family fell even deeper in love with him – and yes, it is possible.
This story is to tell you about Emmett and to tell you about this horrific disease, which I’m betting you hadn’t even heard about. Don’t worry! You’re not alone. Some medical professionals have never heard about it! Spinal Muscular Atrophy, Type 1. There are four types and each level reduces in the severity of impact upon the individual. Type 1 is the worst. It is our worst nightmare.
His Parents
Alisha and Joshua Willms are really good people. No, really. I’m not just writing that because I’m biased (of course I’m biased, I’m Alisha’s dad. But they really are). They both attended the “Outta Town” program at Canadian Mennonite University in Winnipeg. In this program they travelled across Canada and were involved in service projects from Ontario to British Columbia in term 1 and then across South Africa in term 2. They fell in love and then lived a long distance romance as Alisha continued at school at CMU and summer work in Ontario while Josh returned to work in BC and, as he could afford it, school at Columbia Bible College. They both wanted to serve God and to serve others. Don’t worry about being preached at! This story is one about loving your child, having faith and being tried beyond what any human should face, not about preaching, and I hope that you keep reading regardless of your belief system.
Married in Ontario, Alisha and Joshua moved to British Columbia. Joshua worked in construction as a drywaller and continued to attend CBC as he could afford tuition. Their goal was to be able to reach out to those in need and support them. Ideally, to help them become self-sufficient. Alisha had completed her B.A. and started working at Cyrus Centre. This organization is designed for street kids to have a safe place to drop in and get food and support. It even has beds to provide short term emergency overnight care. I like the term Urban Youth Missionary to describe her role at Cyrus Centre. While Joshua finished the last year of his B.A., they took on the role of House Parents at a brand new community outreach called Babich House. They had up to four teenage boys living with them (and two university age support workers) in this newly renovated home. 24/7 – an instant family of teenage boys, all bringing with them a wide assortment of emotional baggage. And none of them knew how to clean the kitchen. Or the washroom. Alisha and Josh’s role, to be effective role models and provide guidance and support to these young men as they sought to get their footing and direction in life. Upon graduation, Josh began working as a youth minister at Level Ground Mennonite Church. He has been in that role for just over a year. They have done everything they can to support and give back to their community.
Emmett Jeremiah
Meet Emmett, in late August he is a 5 and a 1/2 month old little boy. His eyes are piercing and they follow you wherever you go. He loves interacting with everyone. Tickle his feet and you will have made a friend for life. Hiking with his family is one of his favourite things to do. He’s been to the top of Whistler and Blackcomb, through Rogers Pass, spent time in Glacier and Banff National Parks, and even spent a week working at the Okanagan Gleaners – preparing food to be dried and sent overseas as a soup mix to feed the hungry. When I say working, he was the team morale builder! Chilliwack Lake Provincial Park in B.C. is his favourite campsite – he’s so comfortable that he can fall asleep almost anywhere!
Emmett is a perfect baby! That may have made his mom start to question when he was 2 or 3 months old, because he would quietly lay while she was changing his diapers. No kicking or fussing or wrestling while she tried to get them on. Alisha started looking up items on the Internet – because at this point her family doctor clinic simply reassured her that Emmett was just fine and all babies develop at different rates. Alisha discounted many things found on the Internet related to ‘Floppy Baby Syndrome’, because two factors that Emmett shone at were sucking and pooping. And these two were high on the list of floppy baby syndrome, so maybe it was just slow development. However, as Alisha and Josh started observing younger babies who had much better muscle control of their head and limbs, they started asking more medical questions. On Thursday, August 13 they were back in to the clinic. This time their doctor saw what they were concerned about. In fact, he may have seen more because he had them in to a paediatrician the next day. That visit was alarming with lots of questions being asked, and it was followed by a trip to BC Children’s Hospital on Tuesday, August 18. It was there, checked out by three specialists, that Emmett was diagnosed with Spinal Muscular Atrophy, Type 1. Alisha and Joshua love their little boy and are beyond devastated, but they believe with all of their heart and soul that Emmett deserves a life lived to the limit. Expect him to go on more camping trips! He will have adventures. They want to see him smile every day. This is partially for them too; after all, you cannot be sad when you’re with Emmett and he’s smiling.
As an entire family, we appreciate your support, warm wishes, and positive energy. If you pray, we are believers who value prayer. We want and need you to pray. Laugh with us – tell us a joke. We will probably laugh, yet be prepared for tears as we never know when they’ll appear. Be also prepared for little reaction, maybe none at all. You may have found us on a day when we simply don’t have anything left. Please, don’t be upset with us. Our world has just come crumbling down around us and there will be days when we have nothing to give. Likewise, you may see us from a distance laughing and acting crazy. Come and join us. We may be trying to make Emmett laugh. We may just need to be manic for a few moments and release some pent up emotions.
If you meet Emmett, I have a few things to request. First, look for hand sanitizer and apply it to your hands. Seriously! Second, if you’re sick or under the weather then please don’t approach them. Sorry, but your illness could have significant implications for them. (Even if Emmett isn’t with them). Phone or email would be best if that’s the case. Third, Please don’t ask if you can hold him. I hope you can understand that this would just be beyond their comfort zone at this time. Fourth, while you may know someone who has an amazing set of exercises that has done wonders strengthening babies’ muscles – don’t share that. In Emmett’s case, it won’t work. Also, don’t tell them that you know someone with SMA and they’re a young adult and moving about reasonably well. We’re so happy for the person that you know is still with you, but please realize, they have a different kind of SMA than Emmett has.
Spinal Muscular Atrophy, Type 1
Spinal Muscular Atrophy, Type 1. The short form is SMA. It’s devastating. Even Type 2, 3, or 4 are devasting and terrible – but Type 1 means that the child’s life expectancy is fragile – his mom and dad have to protect him from germs, from throwing up and choking – possibly inhaling vomit into his lungs, and make sure he’s breathing. Please, don’t ask if you can hold Emmett. Please, give them lots of space if you’re not feeling well. Alisha and Josh have been advised to avoid having Emmett in all busy, congested places – like malls, grocery stores, and air planes. Now, with 20/20 hindsight, we can identify the symptoms of SMA Type 1 which we saw – but couldn’t identify. That was his very weak leg movements (he loved being in water where he could easily move his heavy legs around), delayed grip, little ability to control his head muscles, and breathing with his diaphram (stomach breathing).
This is my simple layman’s understanding of SMA Type 1. Muscular Dystrophy is the group of diseases that cause weakness of the muscles due to a genetic defect. Muscular Atrophy describes the group of diseases in which a progressive degeneration of the spinal nerves causes a wasting of the muscles they control. 1 in 50 people are carriers of the survival motor neuron 1 gene, which decreases survival motor neuron protein, and this decrease causes the destruction of the nerves in the spinal cord that control neuromuscular growth and function. Carriers experience no symptons. Carriers do not develop the disease. When two carriers have a child, there is a 1 in 4 chance their child will have SMA. It can vary in severity.
SMA type 1 – most severe and the life expectancy is two years, exceptions maybe up to 4 years of age.
SMA type 2 – children have greater SMN protein but still have a shortened lifespan and can never stand independently.
SMA type 3 – children have a normal lifespan but have life-long physical disabilities.
SMA type 4 – adult onset.
References:
For more information, visit these links:
Caring Choices: For Families Newly Diagnosed with SMA Type 1
There is always hope …
The purpose of this blog is multi-fold. One is to keep family and friends updated on Emmett. The second is to raise awareness of this serious disease. And it’s also a journal to record history of events that are happening so fast that we may otherwise lose them. We invite you to follow along with us as we laugh and cry. After essentially 5 months of blissful ignorance, there have been three trips (in the first five days after the diagnosis) to BC Children’s Hospital and the supports that are available in the surrounding area. Family from both sides has been there to support them, attend appointments together, and enjoy Emmett.
On August 25th there was a full day in Vancouver. Visits and appointments included a Dietician, Occupational Therapist, Respite Care Home Doctor, Neurologist, and Respirologist. The Respite Home, Canuck Place, is a well-appointed facility that is staffed with a full slate of medical supports and it is designed to provide short term relief for parents who are exhausted and, when necessary, long term care. This facility is for children up to 18 years of age. The team surrounding Emmett has become very large – Doctors, Nurses, Therapists, Social Workers, and Parent Support Groups. They are incredibly caring and also very blunt and honest. There is no mistaking the diagnosis.
There is a 2% chance that SMA 1 has just appeared out of no where; 98% chance that the parents are both carriers. Alisha and Josh will soon undergo genetic testing. After that has been completed, if they are in the 98% realm then the 4 grandparents will all be tested. After a long day in the hospital, back home to eat and debrief. Fortunately, home is only an hour away from the hospital.
Dr. Kathryn Selby is Emmett’s neurologist. Her area of interest is neuromuscular disorders. She is very hopeful that the research will lead to a cure for SMA in the next five years. She is involved in a study that has shown positive improvements in the quality of life of her tiny patients who place their trust in the adults around them. Alisha and Josh, with support and guidance from their medical team and their families, have some very tough choices to make. The trial is not a cure – yet. But there is hope. While searching online, Alisha found the story of a little girl who knocked all odds out of her way and, at 7 years of age, is an example of the need for hope!
The Pacific National Exhibition is on and Emmett went to explore the sites and sounds on the 26th. Time to create some family memories. While they have been advised to avoid crowded places, they were also advised that they cannot live their life in a bubble and that they must carry on and experience memories. As you can see in the candid shot, Emmett was thrilled to be at the exhibition. Alisha was also introducing a bottle to Emmett. Since feeding is one of the challenges that babies with SMA 1 have, there was concern that breast feeding may be limited in the future and alternate methods considered. Some more physically invasive like a surgically implanted gastric tube. Also, some of the testing required the skill of sucking a bottle – which Emmett has not experienced. This caused some frustration for Emmett, for while he loves his soother, he did not appreciate the bottle.
Back in Vancouver on the 27th for the sucking and feeding test, part 1, Emmett passed with flying colours. While the near future holds another test, there was a celebration that Emmett’s immense sucking power (I am sure we could pick him up by his soother and carry him around!) and capable swallowing meant that he was not at immediate risk of aspirating food. This is huge with an illness that has a serious impact upon breathing and swallowing.
Meanwhile, Emmett’s house is bouncing with family and friends. A couple of Grandma’s eagerly waiting to hold him. An Aunt from one side and an Uncle from the other. Many of Josh’s family are in B.C., so he’s had the opportunity to see many great Aunts and Uncles. Even his Great-Grandparents. A great Aunt/great Aunt and Uncle from Ontario have been out to visit. And just prior to the diagnosis Emmett had a chance to visit his Aunt and Uncle in Calgary during a road trip. This photo is classic Emmett! Personally, I think this look is a cry out for his grandpa in Ontario to come and see him!
Blissful Ignorance
I remember thinking, when Alisha and Josh were engaged, how diverse and great their gene pool would be. Pretty bizarre thought for a man whose kid wasn’t even thinking of having a child at that point. But I thought it. After all, Alisha was from Ontario and she is a third generation in Ontario. Josh and his family are third or fourth generation living in British Columbia. That was a struggle – not because we didn’t love Josh – but because it was so far away. Pam and I often joked that either we raised amazingly strong, independent young women (we have two girls) or they couldn’t stand us – because at the time we had Alisha in B.C. and Katie in Australia. One as far away from us as possible yet still within Canada and the other on what seemed like the other side of the world. Probably because Australia is on the other side of the world. Long distance plans, social media, Skype/FaceTime all help shrink the distance. Why, when I was young none of this was possible – it was a mailed letter and maybe an infrequent and quite expensive long distance call. Plus we walked 5 miles to school, in the snow, uphill both ways!
With this diagnosis of SMA 1 our entire family has suddenly become knowledgeable about this genetic disorder. There is no known history of this disease on either side of our families. As far back or as wide a spread as we know about. Silent carriers! No warning signs. We will be having genetic testing to be able to make our respective families know if there is a possibility that they are carriers. Dr. Google has provided much information – yet I caution you as I cautioned my family that you may not want to research it without a box of tissues close by. As we have done so, I think all of us question why this disease is so unknown! In the United States there has been talk that all couples getting married should be screened for the gene that leads to SMA 1. It is far more common than we think. Is it because the most severe cases, SMA 1 and SMA 2, affect only children and they may be gone before their parents have a chance to tell the world about their journey? Emmett’s dad, Josh, wants to make sure that everyone knows. It doesn’t matter if the carriers are couples from opposite sides of the country or have lived in the same village all of their lives. If two carriers come together then they have a 1 in 4 chance of their offspring having this disease. And yes, what you’re wondering is absolutely right. The risk is that high for future pregnancies. Brutal honesty. Alisha and Josh heard this news the same afternoon that they heard Emmett has SMA 1.
Thank God for technology. Alisha and Josh called us from the BC Childrens Hospital when they got the diagnosis of SMA 1. Within moments of them hearing it, we heard it and shared their grief. Thank God that Josh’s family is in BC and his mom was with them at the appointment. And Katie, Alisha’s sister, was visiting with them and so she was there too. They had family there at this most challenging time of their life. Josh’s dad was there when they returned to Abbotsford from Vancouver. Alisha’s mom flew out the next day. My flight had to be cancelled – on Wednesday I was in to see my doctor and Shingles was diagnosed. My presence could compromise Emmett’s health by causing Chicken Pox – so I’ve been on prescription drugs and impatiently waiting for the contagious period to pass. I will be there soon. Remember in the first post how I requested that you give them plenty of space if you’re not feeling well? Our family is practicing that also! Emmett comes first, not our need to be with him. I do love the way Emmett expresses himself – like this pic where he describes his feelings about SMA!
With this diagnosis of SMA 1 our entire family has suddenly become knowledgeable about this genetic disorder. There is no known history of this disease on either side of our families. As far back or as wide a spread as we know about. Silent carriers! No warning signs. We will be having genetic testing to be able to make our respective families know if there is a possibility that they are carriers. Dr. Google has provided much information – yet I caution you as I cautioned my family that you may not want to research it without a box of tissues close by. As we have done so, I think all of us question why this disease is so unknown! In the United States there has been talk that all couples getting married should be screened for the gene that leads to SMA 1. It is far more common than we think. Is it because the most severe cases, SMA 1 and SMA 2, affect only children and they may be gone before their parents have a chance to tell the world about their journey? Emmett’s dad, Josh, wants to make sure that everyone knows. It doesn’t matter if the carriers are couples from opposite sides of the country or have lived in the same village all of their lives. If two carriers come together then they have a 1 in 4 chance of their offspring having this disease. And yes, what you’re wondering is absolutely right. The risk is that high for future pregnancies. Brutal honesty. Alisha and Josh heard this news the same afternoon that they heard Emmett has SMA 1.
Blissful ignorance. That was us the first five months of Emmett’s life when we did not know what his body was doing to itself. Our hopes and dreams for Emmett continue – even though we know the brutally honest truth about SMA. Blissful ignorance – it is time that our communities are more aware of this disease that primarily strikes children. Little ones without a voice to share the news. A cure must be found!
It’s ironic that August is SMA Awareness month. Wow! Have we ever become aware of SMA. And now as our families grasp with this genetic disease, we must pull even closer together to support Emmett, Alisha, and Joshua. Then when we have time, we are going to make people aware of SMA. Spinal Muscular Atrophy. If you skipped over the first post, Emmett’s Story, my layman’s definition of SMA is found in the last paragraph coupled with links to more professional descriptions.
Families of Spinal Muscular Atrophy Canada Society is an organization committed to finding a cure. Please, consider supporting them in their initiatives and research
Blessings
I know. You’re looking at the title, Blessings, and saying that there is no way. I guess logically there is no way there should be blessing through this, but if we look at it illogically there are. Lots of them. So break out of your rut, and look at this illogically. We are. We have too!
Dr. Selby is the leading SMA neurologist on the west coast, if not in Canada. What a blessing to have someone who knows almost everything there is to know about this genetic disease. The medical team – more on them in a later blog – is extensive and wonderfully caring. CureSMA.ca is a fabulous network to provide support for families who have received this diagnosis. Again, very practical things. From toys that work to help keep Emmett stimulated to ideas and suggestions for the parents.
Emmett loves movement and the adrenaline rush of activity. His lack of muscle control requires that activity be carefully supported. That meant that his Jolly Jumper sat unused, because he simply didn’t have the muscle control required. We did have him in it – his mom supporting his head and his grandma bouncing him. His face literally beamed. But that was a lot of work to do on a consistent basis. A call to my friend, John, who is a key person at Jolly Jumper revealed that they didn’t have anything designed for what Emmett needed. However, on sending in some sketches, John sent out a list of products that he thought could be modified. More on this soon, but John, God Bless you for your help and support!
Support from friends and emails from around the country have been coming to them. Offering support and asking how help could be provided. We hope that through this blog you will be able to stay in touch with Emmett, Alisha, and Joshua. Hopefully over time it will provide you with a means of keeping updated on the ups and the downs of baby Emmett’s life with SMA Type 1. Alisha and Josh have not had time to personally contact all of their friends. Please understand and appreciate that they are focused upon Emmett. As of September 2, they’ve only had two weeks to grasp this themselves and they’ve had to absorb so much information. It is simply too emotionally and physically draining to tell everyone individually about their Emmett. That is one reason for this blog.
Practical things have been so touching. The loan of a travel trailer to handle guests who’ve come from out of town. Likewise, a bed was provided to give extra space. There have been gifts of gas cards/money to help cover the multiple trips to BC Children’s Hosptial. That is absolutely a wonderful to a young family on a tight budget. There have been friends who’ve supported those of us in the East to make it out west with access to flight discounts on airline travel. Gifts from the BC Lions are greatly appreciated – Emmett is anticipating (as much as an almost 6 month old can anticipate) attending a BC Lions game in early October. Josh is also looking forward to this also. The main thing – your prayers, kind wishes, and warm thoughts. We already know that there are people praying and supporting Emmett across North America – and even overseas!
As we’ve mentioned, Josh is a youth pastor at Level Ground Mennonite Church in Abbotsford. They are providing the flexibility with work that this young family so desperately needs. They have also activated a Meal Train! What an amazingly practical and thoughtful way of supporting Emmett. There’s no question that at times they are simply too exhausted to think about preparing meals. Josh will be involved in a Church youth retreat on Labour Day weekend – and a travel trailer was offered so that Alisha and Emmett can travel along to keep this family close together. And to ensure Emmett will be safe and comfortable. All of this is, without question, a blessing.
Family. What more can we say. The support and care from family. Emmett has had wonderful visits throughout the summer with his grandparents and his Aunts and Uncle. He’s even seen extended family. Emails, phone calls, cards and letters, Skype conversations. Those times are a blessing. Also challenging. But a blessing. Alisha and Josh know that some family members are simply too far away for a visit – so this blog is for you to get to know Emmett and enjoy his presence as much as we do.
Most important of all of these, Emmett is an incredible blessing. He is an unbelievably happy little boy. Your spirits are lifted beyond grief when you have a chance to interact with him.
SEPTEMBER 2015
Friday, September 18, 2015
What does a little boy need to learn from his grandpa? Well, so much to teach and share. I pointed out to Emmett that in order to keep the garage neat and clean, that he should take the necessary tools and materials for any project from the garage into the kitchen. Not only will this keep the garage clean but the kitchen has likely got better lighting and heat in cool weather. Then we completed a small project in the kitchen to reinforce the idea.
One morning when only Emmett and I were up, we went around and looked out all of the windows and into all of the mirrors. Since Emmett had not really done this before, it was up to me to show him how to press his nose up against the window pane and the mirror. Sometimes he got carried away and licked the glass. I figured that was okay since the windows were pretty clean – shouldn’t be any germs on them. All little boys need to look out windows to dream about the adventures that lay off towards the horizon!
This morning was a lovely fall morning. Cool, but dry (apparently it’s going to rain for the next three days). So Emmett and I went out for the first time in his little red wagon. It will be a challenge to speed down hills with this as you cannot steer the front wheels, but when he’s ready for that, I’m sure that we’ll find a way. Plus it was time to teach Emmett how to use a camera. Thanks to Families of Spinal Muscular Atrophy Society Canada for this little red wagon – all little boys need a wagon. So do little girls – when Alisha and her sister, Katie, were little we had many excursions in our wagon. I have to warn you – this is a long video and it is not amazing cinematography – but if you love Emmett then you’ll enjoy it!
Emmett is looking forward to this weekend. All of his Willms’ family will be here as his Aunt Jennie and Uncle Benjamin are flying in from Alberta today. Party time! Stay Tuned for some stories of the Willms’ gathering.
Sunday, September 20, 2015
Emmett has had a busy weekend. He had the opportunity on Friday evening to spend a bit of time watching his dad at the MCC Relief Sale in Abbotsford.
Saturday he spent the day at his Grandma and Grandpa Willms’ house. There he got to see his Aunt and Uncles. Everyone got to eat lots and lots of great stuff – while Emmett watched and, I’m pretty sure, drooled. He doesn’t know what he’s missing, but he knows that the big people are getting stuff he doesn’t. Still, breast milk is pretty good stuff. Emmett gets all of the nutrition but none of the taste as it all goes in via his G-Tube. We are getting pretty good at this system. Even though we’ve sometimes gravity fed him faster than we planned (the new setup won’t allow that to happen) or once we’re not sure how much he got – as a port was open and the bedding was soaked. Emmett is very patient with us and he knows that we have to learn.
Waffles with strawberries, whipped cream, and ice cream – I think that we need to blend some up and feed Emmett some awesome stuff like this. Emmett loves his family and he loves crowds (even though his doctors have advised that he avoid them. Then on Sunday he had a huge number of extended family from Josh’s side visiting. It was a special service at Level Ground Mennonite Church. Emmett was invited to the front with his mom and dad as it was his baby dedication service. The message was great – about building community by being a Christian who follows Christ’s example of going the extra mile. Then after the service everyone was invited downstairs for an amazing soup and buns meal – with donations being accepted for Emmett’s anticipated medical and care costs. Some of Emmett’s Ontario family watched via Skype and others will have a chance via YouTube – depending upon technology.
The Church basement was packed. What an outstanding turnout. Throughout the service and the following lunch, Emmett was on his best behaviour. Barely a whine from him throughout. It was, however, quite a hectic day. When he got home he fell asleep and had a nap – that was at least four hours long.
Emmett got a second helium balloon this weekend. His look was one of dumb-founded amazement, as he tried to figure out how his balloon had expanded into two. It was worth every penny to watch him try to figure this out. He actually liked his two balloons better than a bubble maker that makes hundreds of bubbles in seconds. And like millions of babies before him, Emmett has figured out that when he can get his thumb into his mouth that it is a pleasure that his parents had previously denied him.
Distance Makes The Heart Grow Fonder
Sunday, September 27, 2015
Pam and I are having a challenge. We’ve been back home in Ontario for not quite a week and it will be another week before we head back to BC and it is true. Distance makes the heart grow fonder! However, our hearts cannot get their fill of Emmett’s smiles and expressions and cuddles from half-way across Canada. We will be driving back and, like Emmett as a new born coming home from the hospital in his first car ride, we will be anxious for it to be over.
Rhonda and Roger are supporting Alisha and Josh and keeping Emmett very busy. In addition, Emmett had a visit from a physiotherapist who watched Emmett play one of his favourite games – bouncing a helium balloon. The first balloon is starting to lose it’s ability to float but the second one is still going strong. And a third and fourth will be sure to be added soon! Outdoor walks and indoor baths are both highlights for our little man. Soon Alisha will be giving him permission to go in his Jolly Jumper again. It has been off limits while the area that the G-Tube surgery took place heals. Of course, then there are photo opportunities and this little boy absolutely shines when a camera is close by.
Soon we will have a guest blogger doing some writing here. Stay tuned.
Emmett had some further testing at BC Children’s Hospital last week and he will be back this week for some more tests. I cannot say that he loves the tests, but he certainly loves everyone who is around him, supporting him, and even those administering the tests! It has come to my attention that people are concerned that I’m not sharing the full picture, because Emmett always appears to be happy. And usually he is, but I searched through my picture files and I found a few examples of Emmett and his sad face.
Of course, it could be that I’m sad and missing him which caused me to search out these pics. In reality, he’s still a very happy little boy and his every wish will be, if we possibly can, granted!
Wednesday, September 30, 2015
Alisha, Emmett’s Mom, Writes …
While my Mom and Dad, Emmett’s grandparents, are reluctantly back in Ontario for a short time, my Dad informed Josh and myself that it is now our responsibility to take over the blog. And, since it is getting pretty close to Thanksgiving, I just wanted to express our thanks and appreciation to our family, friends, church community, and even to some kind strangers who have been so very generous to us. We cannot fully express how grateful we are to have such a loving community that is holding us steady right now. One of Emmett’s lovely (great) aunties made him a blanket and embroidered on the back are the words, ‘Emmett, loved beyond measure’, and I am so thankful because I know that this is true for him.
The day that Emmett was diagnosed with SMA Type 1, my thoughts were filled with grief and fear and confusion. We had never heard of SMA before and could not understand how our healthy baby could actually have a terminal neuromuscular disease.
Then skip forward to today. Today, for the first time in over a month I got to spend a day with just the two of us, just my son and I. We read books, blew spit bubbles (Well, he did. Not Me!), went for a stroller ride, looked at trees …
When Emmett was born, Josh and I would argue about who would get to hold him. When he sleeps, I have to patiently sit on my hands while I wait for him to wake up again, because I can’t wait to cuddle him again. Nothing in the world makes me happier than when I see how much Emmett admires his dad. Emmett knows that whenever his dad is around something fun is about to happen. I am so thankful for my son, who couldn’t be a more cheerful and happy baby. It’s hard to grieve when a chubby, toothless, grinning face is gazing up at you.
Right now Josh and myself have a lot of different options to wade through regarding how we will proceed with Emmett’s treatment. We are starting to organize a therapy program for him, which will eventually include water therapy, physiotherapy, and occupational therapy. We are working with a social worker and the Infant Development Program to find funding for these therapies. This week we also will learn if Emmett is eligible to be entered into a clinical trial for a drug that could potentially mean a substantial improvement in his quality of life. We realize that this drug is only in testing stages, but it has done some pretty amazing things for a few other babies with SMA.
We are praying that he will be in the trial and that he’ll receive the drug and not the placebo (66% of babies in the trial will actually receive the drug). The trial is set to start on Oct.7th. I am grateful that we’ve been given this little bit of hope. According to researchers, SMA could be a curable genetic disease within 5-10 years. We don’t really know what this means for Emmett, but we are going to do whatever we can for him right now.
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(Photography by Jocelyn Isaak)
OCTOBER 2015
Monday, October 19, 2015
Emmett has had his flu shot. So have his mom and grandma. His dad, myself, and his Aunt Katie (who’s looking for a job in BC) are off to get ours this morning. You should consider getting one too! Maybe after you vote today you can stop off and get your flu shot!
And now, the harsh reality.
Please, don’t even consider a visit if you have any type of illness. Not even a scratchy throat. Emmett is extremely susceptable to any type of bug. He isn’t paranoid about germs, but all of the adults around him are. You may wonder what the big deal is – so look up on Google or your favourite search engine what a ventilator is. When you or I or a child we know gets a cold or flu, we’re miserable. We may even need to go and see our doctor. Maybe even a hospital visit.
For Emmett, that same bug could mean that he requires a ventilator to assist his breathing. Or breathe for him. So, stay away. We’re going to be harsh about this to protect this little boy!
If you are well, you’ve heard us talk about hand sanitizer and washing your hands before you interact with him. This is mandatory at the Willms’ home. Common practice over the last few years has been to sneeze into your elbow as this is more sanitary than using your hand – and it’s true. BUT don’t hold Emmett if you’ve been doing that. After all, guess where his head will be when you’re cradling him in your arms!
Emmett’s outings have been drastically reduced. We appreciate restraint. For example, the other day we were out for a walk and one of Alisha’s friends and neighbours started to come over to see Emmett – but stopped herself. She looks after her own child and several others. I’m sure she decided in that moment it was better to talk from across the lawn than risk exposing Emmett to something. Even though she wasn’t sick! Wow, we so appreciate and respect that.
Emmett is a champion! Thank you for caring about him.
Wednesday, October 21, 2015
Today is the second part of the ISIS Pharmaceutical’s drug trial. Again, the neurosurgeons doing the injections do not report to Alisha and Josh because this is a blind study. They won’t know if Emmett is getting a lumbar puncture or just a sham needle poke. Following the same protocol with all participants who went through the first part flawlessly (as he did), Emmett will only be in ICU for 6 hours today. After that he will be released from the hospital and will be staying at a local hotel, courtesy of ISIS Pharmaceutical, overnight so that he will be near BC Children’s Hospital. Just to be safe.
Emmett, Alisha, and Josh – we are all praying for safety.
EDIT 7 p.m. Pacific Time
Emmett came out from the procedure angry! He wasn’t too happy at all with what went on … and then he fell asleep. He’s back to himself now and waiting for a final check before the three of them can leave the hospital. It’s been a long day and they’re looking forward to crashing back at the hotel!
Go Canucks Go
Tuesday, October 27, 2015 written by Josh, Emmett’s Dad
Hey Everyone, Josh here. Emmett and Alisha had the chance to attend their first ever NHL game last week. Even though the boys in blue didn’t get the win we still had an awesome night.
Thank you so much to the Klassen family for the tickets and the Vancouver Canucks for making the night unforgettable! We were even taken downstairs after the game to meet a few players.
Thank you Bo Horvat, Jared McCann, Ben Hutton, Richard Bachman, Ryan Miller, Jacob Markstrom, Adam Cracknell, and Chris Higgins for taking the time to make Emmett feel so special! Fergal Dempsey was an astounding guide. Emmett is a Canucks fan for life now 🙂
Go Canucks Go!
Wednesday, October 28, 2015
Emmett gave me a raspberry. And it startled me.
You know what a raspberry is – when you blow out air and bubbles through your lips and make a blubbering sound! Emmett was sound asleep while I finished filling his feeding bag with milk and reset the pump for the next 168 millilitres of fluid that would be pumped in. Then I rolled him on his back for a diaper change and, thinking I was fortunate as he hadn’t awakened, I proceeded to change the diaper. And that’s when he blew a raspberry at me! At 3:30 in the morning! So don’t adjust your set – these photos are from the middle of the night and, if they look blue and dark, that is how they are supposed to look! The brilliant light is Emmett’s night light turtle – this creates awesome patterns on the wall and ceiling for about 20 minutes – combined with soothing sounds – before automatically going off.
Just like that he had become a wide awake and alert little boy, and the best way he knew to tease and startle me was to blow a raspberry. I know he was teasing me because if he just wanted attention he would have used his voice. Raspberries are his way of communicating that he is happy and fooling around. With a sparkle in his eye, I could tell that the next move that he was considering was whether or not he should unleash a tsunami like flow of pee that would make Superman proud (speaking of Superman, how is it that no one has written about how many bathroom fixtures Superman destroyed while peeing? I mean, wouldn’t the force have been beyond what mere mortal porcelain could withstand? But I digress – and yet hope someone answers that for me, someday.) Fortunately for me, the new diaper was right at hand and quickly taped into place. Once again, a fluid catastrophe had been diverted, all was once again safe, and Grandpa’s record of not being pee’d upon is still spotless.
Alisha frequently manages to go through this 3:30 a.m. process without Emmett opening his eyes; me, I’m not sure what I’m doing differently but frequently Emmett wakes up and we get some early morning quality time. This morning he was really awake; what else to do but grab a camera and take some photographs. No flash was used so this is really as close as I can provide as to what Emmett sees between 3:30 and 4:30 in the morning. No selfies of me, because this camera is too large to hold and point at myself! And that would be too scary!
Emmett has two attachments overnight. The first, obviously, is his feeding tube, as he gets a slow feed throughout the night. No worries about needing a nighttime snack for this little one – everything is taken care of automatically. Of course, automatically means that at 11:30 p.m. and 3:30 a.m. someone replenishes the milk in his food bag. If that doesn’t happen, the pump alarm goes off because there’s no food available. Or if you’re late getting there, the pump alarm will sound because the set allocation has been pumped and a big person has to reset and restart it. No raspberries from Emmett when this happens – he can and does sleep right through these alarms. Then around 7:30 a.m. the overnight meal finishes and the pump must be turned off and the tubing disconnected. Then it is free time as his next session will be Emmett’s 9 a.m. breakfast.
The second attachment is the Oximeter. Attached to a thumb or a toe – maybe a palm or a foot (the later two actually work better) – this monitors Emmett’s oxygen levels and his heart rate. You’ll see it in the pictures as a glowing red attachment on his hand. If the oxygen level drops below 92 an alarm – at least double in volume of the food pump’s alert – sounds and everyone nearby jumps. This alarm could mean that breathing has stopped – so all adults in the vincinity have their own breathing stop while rushing to check on Emmett. So far it is always a false alarm. Medical opinion is that most likely the false alarms are caused by movement – and so it is not something to worry about. Regardless, even though the alert sounds at least once every night there is no way to get used to it! As startling as this machine can be, it is a God send. Otherwise you’re constantly on edge wondering if he’s breathing or not. Most monitors on the marketplace don’t work for a baby that has limited movement. This is a hospital grade machine. Before we knew it was coming we ordered an Owlet monitor which is a consumer device that became available on October 1 and were quite excited about; it’s design and sensitivity should have worked well with Emmett. We have returned it as we no longer need it (the hospital Oximeter arrived the last week of September), but when you have a medically fragile child you need the peace of mind that this equipment can bring. And you live with the startling alarm.
Tonight is not a night of alarms. It’s a night of a little boy who is awake and wants to grin and have fun. Of course, we have to play quietly because the night vision baby monitor has a great microphone and, if we’re too loud, we will wake up Alisha. If we’re really loud, we’ll wake up Josh. So far we haven’t disturbed Josh! And Emmett knows that I’ve got a camera! He recognizes the shutter click and, I think, wakes up a little bit more so that he can be sure that I get his best side. We didn’t really play apart from making faces at each other – this was a time to cuddle and put him back to sleep.
This is a time when I pray with and for Emmett. It’s easier, for some reason, in the early morning hours rather than in daylight. Maybe I’m too negative in the daytime. Too angry. Too hurt. I try not to be but I’d be lying to say I’m not. Just like Alisha and Josh are strong for Emmett, Rhonda, Roger, Pam and I have to be strong for them. That doesn’t mean we cannot share our feelings – we have to. And it’s easier for me to pray in the middle of the night. Seriously, though, all of that negativity fades when you spend time with Emmett and his outlook on life – so sunny, positive, and optimistic – literally pushes all of the gloominess away.
I know you’re wondering about the trial that Emmett is involved in. Believe me, we are wondering about this experimental drug treatment also. As we have mentioned already, it is a blind study. Emmett could be on a placebo, could be on the drug – but it will be months before we know. 14 months from start to finish. That doesn’t stop us from looking and wondering if we are seeing a reversal of the weakening that has been so obvious in Emmett’s little body. Wondering if the wrist movement that we’re seeing is more active than before – in fact, hadn’t he stopped moving his wrists for a while? Moving his fingers in his sleep – he was just doing that five minutes ago. Usually in his sleep he doesn’t move a muscle – except for sucking on his soother. Oh dear, what would we do without his soother. I have no idea how many Alisha and Josh have bought, but I’m positive that whenever they see this brand they get another one. It’s the only soother Emmett will allow in his mouth – and he loves it. Way more than he loves his helium balloons, which he does seem to be moving more energetically than a couple of weeks ago. His grip also seems to be much stronger than it was. Is this evidence of regeneration of nerves? Is he getting the real drug and not a placebo? Is he just in a good spot? Are we just looking for signs!
Of course we are. But here’s the thing, we think we are seeing them! Little things that are quite hard to gauge. Time. Time will tell. Time is why Emmett is going through this trial process – because Alisha and Josh want more time with Emmett. We all do.
NOVEMBER 2015
Drug Trial – Part Three
Wednesday, November 04, 2015
Our dear little Emmett is back to BC’s Children’s Hospital for the third part of his blind drug test. If it’s the placebo then it is just a needle prick; if it is the real thing it is a lumbar puncture. All we know for certain is that Emmett really gets worked up with these tests and this is the third one in four weeks. After this they will be spread out over a much wider time span.
We’re here for you, Emmett! And we’re praying lots! (I will update this post late today about how it went.)
UPDATE:
Emmett has had a great afternoon, well, apart from the experimental drug procedure where he was separated from his parents. He did not enjoy that but, after a bit of anger and fussing, Emmett had a little nap and woke up happy and enjoying everyone around him. Over the past two weeks we’ve seen Emmett improve his movement and his strength. Babies with SMA Type 1 lose strength – they don’t gain it – and this was confirmed by his doctors. That Emmett has gained strength was also confirmed. Apart from the hope gained by entering the trial, this is a bright moment for Alisha and Joshua. This is cause for hope – never give up! Thank God for the miracle of modern medicine.
Aside:
After the study, Emmett and his parents went back to their hotel, where we had Emmett’s fourth helium balloon waiting for him. It was a ‘Cars’ balloon and you should have seen his face. He absolutely knew that it was a new balloon. I loved it! They’ll all be home later today!
And here are a couple of pics from Hallowe’en. (Did you know, if you click on the pictures they will open up into a larger sized picture so that you can really see Emmett? If you didn’t, try it!)
Looking for Trouble
Thursday, November 05, 2015
There is no question, this little boy is planning some mischief! Emmett’s Grandpa Roger took this picture a couple of days ago and we’re pretty positive that Roger is in on the plans!
Have a great day!
Friday, November 20, 2015
MORE BALLOONS!
It has been two weeks since the last blog post, and we have heard that some of you are concerned and wondering where Emmett is. There is no need to worry as Emmett is enjoying quality time with his family at home and has continued to provide us with little signs that the communication from his brain to his bodies’ various muscles is improving. We are so pleased and, with an illness that we were initially told there was no hope, we are absolutely hopeful. To put this into a better perspective for you, at the beginning of October, Emmett had lost a lot of strength in his neck. He used his eyes, of course, but if there was a noise on his right and he was looking left, he couldn’t move to see it. We had to turn his head. That is something that you cannot see in the pictures of this cute little darling. His arm movement had become very weak – basically moving up and down. He couldn’t grab and hold his own hands. If you wrapped a helium balloon ribbon around his wrist, he could move it, but small, slow movements. It was a very challenging time. Of course, we have honestly always been hopeful but hope is easier when there are positive signs.
Fast forward to today and Emmett is actively turning his head from left to right. His arm movement is, amazingly, likely back to the same level of movement he had in late August or early September. He will firmly grasp your finger or, when it’s placed near him, he will grab the helium balloon ribbons and vigorously yank it around. All the while making faces like in the picture on the left – that is absolutely his balloon face. One of concentration as he tries and succeeds in moving the balloon and excitement as he observes it bouncing around. He was in his special harness to use his Jolly Jumper and, while in it, was turning his head left and right. Before he would possibly swat at something with his entire hand/arm; now he reaches out to his pinwheel with his pointer finger (that same finger that’s reaching for the leaves above) and spins the wheel using his finger! These may not seem like much, but he has never done that before. His eyes would look around, but this time it is absolutely his head that is turning. Going back to October, I was holding Emmett one afternoon when a solitary tear rolled down his cheek. It is presumptuous to make assumptions about what he was thinking, but I am positive that he knew that he couldn’t reach the (toy, balloon, hand, soother, …) that was right beside him and that he knew just a few weeks earlier that he could. That was such a powerful moment for me – and one that wiped out any questions I had of this experimental ISIS drug trial. Not only is it very much an experiment, but needles and lumbar punctures have to hurt; however, for the chance of improvement it is something that we must put Emmett through. Especially now that we can see something happening.
But why no Blog updates for two weeks? That is because we’ve been on the search for better and bigger helium balloons. We found some, but that’s not the biggest change. In just two weeks, Emmett has changed. He’s growing. He’s simply bigger. No teeth yet, but they’ll arrive when they’re ready. Pam and I gave Emmett, Alisha, and Josh a bit of space and we’ve been travelling for the past two weeks. Since Emmett was in a good place, it was time to give this family a little bit of room and independence. Coming back it is so interesting to see the changes in this little boy. Josh had changed too – his beard was gone and he only had a Movember moustache. He’s (Emmett, not Josh!) also more verbal – making sounds all the time. And his arm and hand movements are almost constant. We are seeing movement that we haven’t seen since late summer. Emmett’s head must be constantly supported, but even here there is a difference, as I said already. Emmett is moving his head left to right more than before. There appears to be more support. While Emmett only gets to the community pool once in a while, he does get swimming exercise daily in the family bathtub. This ‘weightless’ environment is where Emmett is really beginning to show movement. This is so incredibly awesome.
Oh, and we did find some new helium balloons! Pictures of them in a later blog post.
Yes, Emmett has MORE Balloons!
Wednesday, November 25, 2015
Even More Balloons!
You may remember that I promised a long time ago, well just a few weeks ago probably, that I would make sure Emmett had helium balloons. The expression on his face is absolutely priceless. While there are some who have felt that I’d already gotten enough balloons, I have been on the hunt for more. I thought that DisneyLand would have some interesting balloons. And they did, sort of, but not ones that looked like they would last and not cause potential issues. For obvious reasons, latex style are being avoided and only foil will do. No foil balloons at Disney! That surprised me. So the search was on throughout the Pacific west coast for a ‘better’ balloon (well, we really only stopped at one store, but through the GPS we sort of looked!).
When Emmett looks up, he now sees what you see in the picture above! Four Balloons! As you can appreciate, when the older ones fade away, they are retired because they’ve completed their tour of duty. The expression on Emmett’s face when the Mickey balloon appeared in his line of vision was priceless. And a couple of hours later when the giant Lightening MacQueen balloon arrived, it wasn’t just Emmett’s expression. It was also Alisha and Josh! More priceless looks – but no kodak moment as we were too busy watching to take pics at these moments. That last balloon is almost big enough to lift Emmett off of his crib. It blocks the sun like an eclipse … we need to get a couple more!
Adults have a maze of balloons and ribbons to meander through to get any where in the house, but a promise is a promise. I will continue to keep getting balloons. I really don’t think that there will ever be a time when Emmett has too many! Of course, I’m probably in the minority on this perspective, but without and rationale but for some sort of reason, they will keep coming!
Of course, Emmett also needed to spend some time in his Jolly Jumper. He is so much more comfortable in it that it absolutely makes us all smile. The opportunity for him to be vertical is something he enjoys. And who doesn’t like to bounce. So here are a few more pics of this special little boy. Plus a video of him playing with his balloons.
Friday, December 25, 2015
DECEMBER 2015
Tuesday, December 08, 2015
Super DiaperHead Baby was working out in his private lair in the mountains. Even heros like Super DiaperHead Baby have to exercise to stay in tip top shape. Super DiaperHead Baby finds that moving his arms about on a regular basis is a great way to exercise. He doesn’t really need the weights, they’re more to impress the ladies than anything else. Like every other superhero, Super DiaperHead baby is almost always surrounded by ladies when he’s out and about. Here, however, in the calm and quiet domain that is his private lair there is no audience around (except the photographer and paparazzi, of course)! Without his superhero costume, he’s just like every other baby with SMA Type 1. But with the costume – the diaper on his head, sleepers buttoned around his neck but otherwise flowing in the wind, and no pants but his bottom fully covered by a diaper – Super DiaperHead Baby can do anything he or you could imagine. It was about this time that he got a message (was it telepathy? a beacon shaped-like-a-diaper beamed into the sky? or does he just have a really good mobile phone plan?) that his mommy was getting swamped by paperwork.
Sure enough, Super DiaperHead Baby was right on the money because, through the superhero technology he has in his superhero lair (that’s secluded in the distant mountains) he could see the look of frustration on his mommy’s face about all of the paperwork. He sensed that she was about to give up and that is not something that he could allow. Super DiaperHead Baby knew that part of the problem was that daddy had the MacBook and mommy was once again forced to use the Window’s laptop. Which can be frustrating even without bills and stuff like that.
Tossing the weights aside like they weren’t even there (and in actual fact, they weren’t there. It was a foam bar that was about 30 cm long and Emmett can toss it easily).
Blasting out of his secret lair, Super DiaperHead Baby flew around and over trees without even noticing the mountains surrounding him. He wasn’t bothered by the weather or the music that was playing in the distance – although he prefers classical to hip hop or rap. Super DiaperHead Baby really doesn’t appreciate rap at all.
But, flying at speeds that would bring tears to a mere mortal’s eyes, Super DiaperHead Baby arrived at mommy’s and as I drew in my head and was turning around, down the chimney Super DiaperHead Baby came with a bound.
Of course, Super DiaperHead Baby was travelling so fast that mommy didn’t see or hear him coming. She was still bemoaning the fact that the MacBook would have made life so much more bearable. Super DiaperHead Baby had some kind of guidance system because he truly never looked where he was going. Instead he was focused upon ensuring that his photographer captured his best side! Yet, in spite of not looking, he never crashed into anything – except maybe a helium balloon or two.
Super DiaperHead Baby’s super power is causing everyone around him to forget their worries and concerns. Typically they are so caught up in enjoying being with Super DiaperHead Baby – giving him lots of cuddles and kisses – that they mess up his costume. But that is of no concern to Super DiaperHead Baby. He doesn’t care what he looks like as long as he can make others smile and laugh. Which in turn makes him smile even more and, if you’re really lucky, you’ll get a small giggle out of him! This costume mishap, with the tab being out by his face, made Super DiaperHead Baby wonder if this would perhaps improve his aerodynamics while he’s flying at super fast speeds?
His eyes, how they twinkled. His dimples, how merry! His cheeks were like roses, his nose like a cherry! Don’t be deceived because he’s chubby and plump, he speaks not a word, but goes straight to work. With the diaper tab pushed a little bit back from his face, Super DiaperHead Baby’s work was done. Laying his finger aside of his nose, and giving a nod (more like side to side, cause he cannot nod yet), up the chimney he rose and then, sleeper cape flowing in the wind, soother close by, and diaper planted handsomely on his head – off he flew back to his secret, secluded, and very distant lair in the mountains!
Thank you, Super DiaperHead Baby! Until the next time!
Super DiaperHead Baby Stays Home!
Thursday, December 17, 2015
There is no question, the winter doldrums and cabin fever have struck in beautiful British Columbia. The promise of hardly any snow was smothered today. Smothered by the falling of large, fluffy, white snowflakes. What is happening with BC’s weather? We Easterner’s were promised cool summers and warm winters. The past two summers have been blazing hot with heat waves that, such as this past summer, caused Super DiaperHead Baby to cast off most of his costume and only wear his diaper. And not the one on his head! In fact, if he could get away with it he would wear even less than that. And winters? Snow today in BC yet well above zero in Ontario! Has the world gone mad? We have no answers for serious questions like that because Super DiaperHead Baby could care less about getting into a debate about weather, politics, or anything else. His task, to share his magic touch with everyone. To brighten their lives when they feel like they’re stranded like an alien on a strange planet. When that happens, Super Diaperhead Baby is there in a heartbeat to reach out and touch you! To make you smile again. To make you realize that there is so much joy in life!
In everyday mode, Emmett does many things like every other baby does. Such as filling his diapers and peeing on stuff. As a result this creates a huge amount of laundry which must be washed, dried, and then folded. Of course, grandpa doesn’t really do much of this but he was the only one willing to allow his photo to be taken. Emmett, of course, always wants his photo taken. So here in our made up story I’m dealing with the tedious and never ending chore of putting away clean laundry. Of course, I don’t realize that Super DiaperHead Baby has snuck into the laundry basket. His sleeper cape was in the wash so he wasn’t able to fly about, but somehow he got there!
Of course, as soon as I found Super DiaperHead Baby then all of the challenges and frustration of doing laundry vanished as Super DiaperHead Baby’s smile burst out of the laundry basket – with a few giggles that he had been able to surprise me. However, he was a little bit put out as his favourite sleeper/flying cape was not in this load. It was in the next which wouldn’t be done until after his bedtime!
Bursting out of the laundry basket and scattering all of the nice and freshly laundered clothes all across the floor, Super DiaperHead baby made me giggle and laugh. And then we left the scene and probably grandma and mommy stayed behind to clean up the mess that we had made. It was time for Super DiaperHead Baby to revert back to his normal baby self, have a bath which he absolutely loves (on the other hand, Super DiaperHead Baby absolutely abhores water! It simply makes his costume look and feel horrible. His head becomes so heavy!), and then go to bed.
Stay tuned for the next episode – Super DiaperHead Baby Awakens!
Bit by Bit!
Wednesday, December 09, 2015
“What is REAL?” asked the Rabbit one day …
“Real isn’t how you are made,” said the Skin Horse. “It’s a thing that happens to you.”…
“Does it hurt?” asked the Rabbit.
“Sometimes,” said the Skin Horse, for he was always truthful. “When you are Real you don’t mind being hurt.”
“Does it happen all at once … or bit by bit?” (asked the Rabbit) …
The Velveteen Rabbit by Margery Williams
Today Emmett is nine months old. He will spend most of this birthday as he spent his eighth month and seventh month birthdays, in ICU at BC Children’s Hospital. When he comes home he will have another helium balloon to add to his collection (no, it won’t be as big as the last one!) I almost typed that it’s not because he’s sick, yet that’s not right. At the same point, he is NOT sick. He has a genetic disease. It is simply part of him. Emmett is! Rather, it is not because of a crisis that he is at BC Children’s Hospital but it is the trial. He will be going through the fourth part of the ISIS Pharmaceuticals experimental drug trial today. If you are new to Emmett’s Journey, you won’t know that the babies who get the placebo will go through a sham procedure that mimics the real thing while the babies that get the real thing will receive a lumbar puncture. And it hurts.
It hurts Emmett. Alisha. Joshua. It hurts everyone who has had the privilege of holding and cuddling Emmett. I dare say it’s hurting everyone who is reading this! Yesterday Emmett had a shot to prevent RSV (Respiratory Syncytial Virus) that can be very serious for high risk babies. It was his second shot for RSV and he didn’t mind it at all. Then he had blood work done and he expressed his outrage. It hurt. Emmett. Alisha. Josh. All joking and fooling around aside – Emmett is a medically fragile baby and not only the world, but also the hospital, can be a dangerous place for him. Alisha has Emmett behind barriers fortified by disinfectant and she and Josh are virtually keeping him in isolation to ensure the safety of his health. Cold and flu season is terrifying for a family whose baby, on the best of days, is already struggling to breathe. Have a cough – we’ll be vacating the area quite quickly. That is real!
And today is the fourth session since the beginning of October in this clinical trial of this miracle drug by ISIS Pharmaceuticals in which, after every session, we all are asking “Is It REAL!” Your prayers and warm wishes are coveted – this will probably happen around 12 noon Pacific Standard Time.
But no one will tell us! After all, it is a clinical trial. A blind test. A secret test. One that is taking place around the globe with babies who are dying from Spinal Muscular Atrophy Type 1. Just over a hundred babies get to take part in this test. Emmett is one of the lucky SMA Type 1 babies that was selected – he met all of the criteria. So he, in what can be a cruel world, is a lucky one. Of these lucky ones in this study, one third receive a placebo. Two thirds receive the real drug. A drug that is designed to regenerate the dying nerves in the baby’s spinal column so that instead of their muscles atrophying due to the message not getting through, the muscles return to life. What also hurts is knowing that many babies and families don’t even get a chance for this trial. And that friends – that have been made along this challenging journey – may not be getting the real thing. Is Emmett? No one will say, but like the Skin Horse who with wisdom saw everything that happened in the nursery, we are scrutinizing Emmett’s actions in his nursery and believe it is Real.
Bit by bit, not all at once, we have been chronicalling his improvements in this blog. I’ve said before, babies with SMA don’t improve and yet – is this real – we are seeing improvement. A return of movement and strength that Emmett demonstrated in the summer. And this week there were his ankles. His ankles are cute! There – I know you were waiting for me to say cute. Because I cannot help it. Emmett is cute! His ankles are cute. And this week he was rotating his ankles and feet. Not just up and down toe extensions, but rotating them. This was and is very exciting because this isn’t a return of movement. It’s brand new. Emmett has never before rotated his ankles. And then there’s his frowny face – it’s not because he’s upset or hurt. It’s because he can do it! We love it! Is it REAL? Does it HURT? Absolutely – but the Skin Horse and the Velveteen Rabbit will tell you so eloquently and, I’m positive with strong English accents, that it is so entirely worth it!
Alisha and Josh are so strong and they so love their precious little boy. The hurt that they go through during every one of these hospital visits is beyond description. Like the Velveteen Rabbit, they’d do anything that they could to avoid these uncomfortable things happening and yet, right now, they are enduring the hurt that bites and tears at them while Emmett undergoes these drug test activities because, bit by bit, this is becoming real. And the words they heard that indicated that there may be as little as six months (yes, that’s what they heard in August) for their baby with this diagnosis of SMA Type 1 are being pushed further away. It’s getting brighter and the load is slightly easier to bear. Especially with so many friends and family helping to bear the load. Bit by bit, what appears to be a miracle is unfolding right in front of our eyes. One ankle rotation at a time. We will continue to pray without ceasing for this little angel!
Merry Christmas!
Alisha, Josh, and Emmett are so thankful for the support, friendship, and love that you have given their family this year. It has meant so much to them. What a fabulous outpouring of love, prayers, and positive thinking. Each and every contact is so appreciated. Thank you and keep it up – short notes via social media are ideal! On this day they are celebrating the birth of Jesus – the babe in a manger who is the reason we celebrate Christmas.
Christmas eve was a great time and a full Willm’s house. Josh’s parents, brother Ben and grandparents (Emmett’s Great Grandparents) came over after their Church’s Christmas Eve Program. Alisha, Josh, and Alisha’s sister Katie went to Level Ground’s Christmas Eve program while Alisha’s parents (one being me) stayed with Emmett. Then everyone gathered at Emmett’s Place for a noisy game of Catch Phrase. While Emmett welcomed everyone into his home, he quickly fell asleep inspite of the raucous party in the living room.
Every parent celebrates so many things with the birth of a child. Likewise, Alisha and Josh are celebrating with Emmett. Celebrating Emmett’s birth and life. So many things have happened that it is hard to keep up – and hard to keep you updated. Emmett is doing very well on his Bi-Pap machine (similar to a sleep apnea machine for adults). At the same time as this machine arrived, Emmett was approved for overnight nursing support. This has been a great relief – as everyone was getting stretched thin due to lack of sleep. Not only the challenge of a restless nine month old, but also one who is connected to a feeding pump, an oximeter, and now a Bi-pap machine (which in itself requires constant monitoring).
Emmett loves the Christmas season. While he wasn’t thrilled to meet Santa Claus, he is absolutely mesmerized by the lights on trees and houses (we may have to keep them up longer than is socially acceptable!). British Columbia, unlike most of the rest of Canada, has been having snow! It hasn’t stayed where Emmett lives, but from the expression on his face we can tell you that, like his mom when she was little, Emmett does not like snow! We are sure that this will change, but at the moment it is stuff that falls on his face and he disapproves. He does, however, love when the cameras are out and he is the centre of attention! He searches out the paparazzi. The question is, will he like his presents Christmas morning or will he prefer the wrapping? My vote is the wrapping.
Emmett got one of his Christmas presents early. It is a specially designed swimming ring that securely fastens around his neck – supporting him in water (a pool or a full bathtub) – allowing him the freedom to move his limbs. We’ve said before that he loves water and this gives him freedom like nothing else. Before you begin to worry about his safetly while using this, it has the full approval and support of his physiotherapists. In fact, last week Alisha and Emmett were in the pool with two physiotherapists who were checking him out. It is wonderful to see him twist and turn; make kicking motions with his legs; backstroke movements with his arms.
There are so many facing challenges this Christmas. We share our love and prayers with you. We hope and trust that you reach out and accept support – it is available in every community. We know some of your stories but obviously not all of them. Alisha and Josh are community care-givers. Now they’re also the recipients of care from others and it is such a blessing. We cannot say thank you enough!
Emmett is a gift from God. The best Christmas present ever!
S