At just 5 months of age August 2015, Emmett was diagnosed with Spinal Muscular Atrophy, Type 1. It is the most severe SMA type with life expectancy of about two years. We welcome you to join him on his journey ...
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. (We have learned since that there are absolutely some Type 1 who have lived and thrived well past age 4.) 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.
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 met when 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.
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 medical 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 appointments were made with specialists at BC Children's Hospital on Tuesday, August 18. Talk about a weekend that dragged on and on - this was it. The fear and uncertainty were oppressive. It was at BC Childrens, 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 most likely have a different kind of SMA than Emmett.
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, which most people have heard of and often confuse with SMA, is the group of diseases that cause weakness of the muscles due to a genetic defect. Spinal 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 is the #1 genetic disease that kills youngsters.
SMA type 1 - most severe and the life expectancy is generally two years, however, there are always exceptions. Some youngsters are in their twenties ...
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.
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UPDATE: On June 30, 2017 Health Canada approved Spinraza - the only drug that has demonstrated successful results in patients with Spinal Muscular Atrophy. In the spring of 2017 we were officially told that throughout the global blind drug trial that Emmett participated in, he was getting the drug and not a placebo. He continues to improve.
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