Shira singing Beth by KISS

Shira Singing I Was Made For Lovin You Baby by KISS

Weight, Height, Growth-Spinal Muscular Atrophy

Almost everything to do with the care of an SMA Type 1 child is up for debate. Except for the Conventional wisdom of Tracheostomy all other modes of therapy are often looked down upon by physicians and other medical professionals. Can you imagine doing double blind studies on terminally ill children allowing some to just die while giving life saving treatments to others. I have observed many children now while caring for my daughter Shira who has SMA Type 1. My greatest frustration is parent's not receiving a list of choices for therapies that would insure their child does not die an untimely death. It's true that a course of action can be strictly palliative and I support parents that want to go that way if they have been exposed to all the modes of therapies available for our children before making their choice. This is a long discussion so I will get to the point I would like to make today. Diet! Yes diet is another mode of therapy for SMA Children that is a heated topic and of great debate. I am tired of doctors telling me that the AA diet or Amino Acid diet is unproven and might be dangerous. I have watched more children suffer from other diets than from this diet. The other diets also available for our children are also unproven in the SMA world and are also just as anecdotal in effectiveness as the Amino Acid Diet. So, if you are butting heads with your nutritionist and doctor over the AA diet just ask them for Clinical or Empirical information Expressly Written that proves that the diet they would like to see your child on has been studied on SMA patients. I can tell you right now no diets have been studied on an SMA Population!

Also, one of the measures doctors and nutritionists use is the traditional BMI or Body Mass Index to chart SMA childrens development. Unless nutritionists and doctors adjust for SMA patients low musculature etc. the outcome will show the SMA patient to not be thriving which just isn't the case. I have recently found an Index that might be more inline and accepted to use for the SMA population than the BMI and that is the Kennedy Krieger Institutes Growth References for Children with Quadriplegic Cerebral Palsy. Growth Charts

If you need more information to back up what you are talking about with your Doctor, Nutritionist and other medical professionals regarding the AA diet you can also go to Shira's web site at Shira's Web Site

Serendipity? Coincidence? Fate?

Yesterday was another really good day for Shira and I. Poor Sammy is starting to feel a little left out so I’m hoping we can move upstairs today or tomorrow. Our close friends from Israel are in town now and I’m really looking forward to spending some quality time with them. Yesterday was a lot of fun. Shira and I decided to go to the Victoria Art Gallery. Shira had more fun playing with some business cards while we walked around the gallery than actually viewing the art ……lol but I thought I would give the gallery a try. Again we are travelling on bipap, laying on her side using the EZon vest. After the gallery we drove down to my mother’s house and hung out there for a couple of hours as she didn’t have any guests and therefore we wouldn’t be exposed to any illness. We went for a walk down to Victoria’s inner harbour to feed more seals. A strange thing happened while down at the harbour. My mother had just given Shira a little stuffed Panda Bear but while on the docks in the harbour I dropped the bear. A First Nations ran up to us to bring the bear back which we hadn’t noticed was gone. This is getting weird all these First Nations People and Bear energy coming into are lives again!!! I’m not a superstitious person but is this more than consequence? We ate dinner on the docks (Halibut Burger Mmm), walked back to my mother’s and headed home once again on bipap. When we arrived home Sammy was so happy to see us. It has been 31/2 years since I have left the house on my own with Shira so unless I had someone go with me to suction Shira or drive so I could Suction Shira we would stay close to home. Sammy never experienced Dad and Shira leaving for the whole day before. I put Shira on bipap for at least 1-2 hours a day to recharge and not get overly tired but yesterday we played it by ear and I just kept an eye on her in case she needed a break but she was fine with 2 short bipap breaks while we drove from point A to B then B to C. All in all another fantastic day mixed with a mystical experience! And let’s not forget feeding the Seals which is always too much fun!!!

Strength, Courage and The Bear

When you care for children with SMA you have as much routine in your day as a soldier does in the military. Today we went awol from our routine a little with Shira sleeping in an extra hour so we missed our morning Chest Physio session. As soon as Shira was ready I asked her if she wanted to go downtown and have another adventure and her answer was a loud Ahhh Ha! We have now mastered the art of travelling alone with Shira placed on her side in her EZon Vest while on bipap. I advise anyone travelling alone with their SMA child to travel this way as it helps prevent desat situations as well as managing secretions. We arrived downtown parked unloaded etc. which is a chore in itself when you are alone. Amy my hat goes off to you for the years you spent caring for Lily on your own and thank you for teaching me so many valuable skills!!!! Shira loves to walk through stores especially the stores playing funky loud music. It took Shira and I about an hour to walk a mile because we stopped at every street musician so Shira could sing with them. Yes you heard me. Shira has a microphone and speaker so people can hear her speak because her voice is so low in volume. Now Shira is heard very well and she can also hear herself better. Shira has an excellent ear and can match pitch very well with most melodies. So the street musicians got a kick out of Shira humming along with them. I would put a couple of quarters in Shira’s hand and wheel her over to their guitar case and hang her hand over it and she would work very hard to drop the quarters into the case. We worked our way through downtown to the waterfront where there are more street musicians and lots of artists of all kinds. At the end of the row of artists is an embankment where all the First Nations people sit making arts and crafts. Shira loves to with everyone. She is so cute. She loves to say, “Hello, nice to meet you!” It just makes people melt. We walked the line of all the First Nations Artists and one of them a big burly man with hands the size of warn baseball gloves looks deeply at Shira and says, “take the small bear drum there I want her to have it.” Then he says to her, “You need strength and courage little one. The bear will bring you strength and courage.” And he says to me, “She will play the drum for both of you, you need strength and courage too.” Man it brought me to tears. I shook the mans hand and we walked on. As the Oglala Sioux would say charging into battle to count coup, Hoka Hay! My heart was soaring. This man connected deeply with Shira, he could see, no words needed to be spoken between us for understanding. After our spiritual encounter Shira and I headed off towards my mother’s house. On the way we stopped and played with the huge draft horses that pull carriages full of tourists. We only stayed long enough at my mother’s to say hello and take a bag of carrots from her to feed the horses on the way back to our car. While feeding the horses the owner came up to me and basically mistook Shira for Charlotte Hodgson who lives in that area and is often feeding the same horse with her mom Tamara. SMA children have many of the same physical traits and Shira is often mistaken for Charlotte when we are in their neck of the woods. After feeding the horses our bag of carrots and sharing our carrots with another little girl that was there so she could also feed the horse Shira and I headed back to the car. So now not only did we miss our physio session in the morning but we were way past Shira’s respirator break, bad dad!! We got back to the car and I put Shira on bipap and she relaxed and breathed easy all the way home. When we got home and moved all the gear inside I placed Shira back on bipap, I drank a gallon of water and looked in the mirror only to notice that I had a sunburned face that started half way down my forehead because I was wearing a hat. Another adventurous day with Shira!!

Traveling Solo With Spinal Muscular Atrophy Type 1

The last few weeks have been tough. Sammy was endlessly sick the last part of the winter and now Maxine is very sick. Maxine went in to have swabs and tests done so we will hear on Monday if she has anything nasty. Today was a reprieve for Shira and I that have lived in our basement way too long this winter. A Channel news that helped us in the beginning by putting pressure on the Health Minister and Doctors here called us to come down and speak at a rally. The rally was for the television that was trying to make a point as to how important the news was beyond entertainment. Canadian television companies are not doing well these days because of the slow down in the economy and they are fighting with the cable service providers that charge them a fortune to carry their signal. Long story short Maxine and Sammy did not come with us because they are sick. So Shira and I went for our first solo car ride. We followed Lily Barnett’s car travel protocol by laying Shira on her side and traveling while on bipap. Usually I only travel with another person so that Shira can be suctioned but this time we had to go solo. Everything worked out without a hitch and all the way there and back Shira kept saying, “I’m having fun daddy!” “I’m ok daddy!” She was so funny! When we got to the event I was surprised to see the street blocked off, a jazz band playing, food, mascots etc. The journalist that has helped us out many times greeted us as well as the anchor. I thought I was just going to be interviewed and it would be on the air but it turned out that I was to speak in front of 250 people. Shira was great and had a lot of patience while I spoke. When I was done Shira just wanted to watch the band play. After about an hour Shira said, “Daddy walk around.” So we left the event and walked around downtown. We went to the Gap where Shira picked out an outfit (Gap Girl athletic lime green jacket and pink pants) and then we walked around downtown. We stopped to listen to a guy playing guitar on the street and Shira sang along with him using her microphone and speaker. After about an hour of walking around and talking to people Shira and I headed back home. The ride was un eventful and fun all the way home. When we got home Shira rested for about 2 hours on bipap and then we headed out to watch baseball until 9 at night. Sammy came with us which is not usual as he hates to walk but he did tonight. Of course when we got to the game Sammy just wanted to leave and he hounded me the entire time for pop which I wouldn’t buy for him. On the way home Sammy wanted me to carry him on my shoulders of which I refused so he started telling me he was going to have a heart attack he was so tired. I didn’t give in and carry him this time and we had a race for part of the way back, stopped at a park to play for a and chatted up a storm. 6 year olds are hilarious. We talked about a wide range of subjects from brain damage, how clouds are made, why people of color aren’t as famous as white people (then he remembered about Barack Obama), who the most famous person in the world was, why the giant boulder he climbed on was hot, why the two men that walked by us were different shades of brown, why we can’t take flowers out of peoples yards and on and on and on. Now Shira is on bipap and tucked away in bed and I’m writing this. It was a good day!!

It's Never Too Late

I'd be lying if I didn't say that things have been tough for me hearing about all these children and people dying from SMA. Some parents and children I have met only briefly through email and telephone conversations some more intimately through video conferencing. It's hard to experience the pain of children dying over and over again, trying to understand the suffering of parents, syblings and close friends. There are so many things I don't like anymore that once I enjoyed like war movies, violent action movies and other art forms that push the buttons of conventionality. I'm always on edge, on high alert for Shira and every time I hear of another child sick, even regular kids something inside me makes my sences more heightened. When I see a parent scold a child outlandishly, spank a child, smoke in the car, or parents not closely watching their kids as they play in the park I feel fear for those children. I never felt this way before, not even when Sammy was born. Sure, I wore my heart on my sleeve a little more when Sammy was born but I came from that school of "Let the boy go he'll learn." or " what doesn't kill you will make you stronger mentality." I just finished watching The Curious Case Of Benjamin Button while Shira lays in her bed on bipap not feeling 100%. The movie is a must see and I won't watch and tell other than to say, watch it. Here is an excerpt from the movie that I liked about facing life:

Excerpt from The Curious Case Of Benjamin Button



“For what it’s worth it’s never too late to be what you want to be. There’s no time limit, start whenever you want. You can change or stay the same, there’s no rules to this thing. We can make the best or the worst of it. I hope you make the best of it. I hope you see things that startle you, feel things you never felt before. I hope you meet people with a different point of view. I hope you live a life you are proud of. If you find that your not, I hope you have the strength to start all over again.” – Benjamin to his daughter.


I can honestly say that I have never been so proud of myself as I am now for the way Maxine, Sammy and I care and deal with and for Shira and each other and how proud I am to know so many people that put so much energy, love, compassion and care into their children or friends children like the people on smasupport. There is a real energy on smasupport chat that keeps so many families going inspiring them to deliver intensive multidisciplinary care to their kids or others day after day after day. Here's a big pat on all of our own backs or as many of you put it, "Hugs!" Brad