Monthly Journal - May (2019)

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Monday, May 20, Through Sunday, May 26 - This had to be one of the most fulfilling weeks that I've had in the past 2 years. Not only did everything fall into place, the FDA finally approve ZOLGENSMA from AveXis, as the 2nd FDA approved treatment for those of us with spinal muscular atrophy. This historic announcement will dramatically improve the lives of thousands of children with SMA. We knew that the FDA would approve ZOLGENSMA for infants that were 9 months of age or younger with Type I SMA, but we also knew that AveXis had submitted additional data and information to the FDA in hopes that the FDA would broaden the label to include older children. When the announcement took place on Friday, May 24, 2019, the label said that ZOLGENSMA would be available to infants and toddlers up to the age of 2 years old, irregardless of the subtype of SMA they had. This incredible announcement basically turns a death sentence into a possible normal life for thousands of children around the world with spinal muscular atrophy. We are hopeful that ZOLGENSMA will be available to those of us who are older in the future, and while we believe that this will be the case, they still have quite a bit of testing to go through regarding those of us that are older. ZOLGENSMA will be administered intravenously to infants, and will be administered intrathecally to toddlers. Intravenous delivery of ZOLGENSMA to infants is possible because this treatment can get past the blood brain barrier in these little babies. The intrathecal delivery to toddlers will be administered directly into the central nervous system, similar to how we receive our Spinraza treatments. They chose to do intrathecal delivery for toddlers to ensure that there would not be a blood brain barrier problem. We are also thankful and blessed that this treatment has been passed through the FDA, and with the future of potential treatments being strong, our future is getting brighter each and every day.


This past week, when I went for my workout with Emily, she and I worked on my core strengthening exercises. We not only use the resistance bands, we also did isometric exercises on my core muscles and my neck. The resistance bands and isometric exercises that we are doing has not only increased my core strength, it is also the key reason why I am able to work on my balance exercises every other week. Without a strong core, I would not be able to sit on the therapy table and improve my balance. This goes to prove that months of core strengthening exercises has paid off. If any of you reading my journal entries are thinking about working on your balance, make sure that you focus on your core strengthening first. With a strong core, your balance exercises will be much easier and you will find success much quicker than you would without strengthening your core first. Next week, Emily and Vicki will transfer me from my wheelchair to the therapy table. We will first began with my balance exercises, and I will also be sitting on a cushion, which makes these exercises much more difficult. Sitting flat on the therapy table is not difficult for me to do now, so by sitting on this cushion, it not only throws my balance off, it forces my core muscles to work for longer periods of time. After my balance exercises, Emily will work on my range of motion and flexing exercises on my knees, legs, ankles and feet. We will also be taking pictures while doing these balance exercises next week, because Emily will be producing her 2nd article for the SMA News Today HCP site. After Emily writes her article, she will send it to me and I will post it to the HCP site, along with the pictures, and a short video detailing my balance exercises. I will share the pictures with you next week on my website.


In this week’s video, I discussed the recent approval from the FDA of AveXis’s drug known as ZOLGENSMA. The FDA made the official announcement on Friday, May 24, 2019, making ZOLGENSMA the 2nd FDA approved treatment for those of us who suffer from spinal muscular atrophy. I not only discuss the FDA approval of ZOLGENSMA, I also share a document that was written by SMA News Today regarding this historic announcement. Click HERE to view my video.


I've updated the flash briefings that I do for BioNews Services. Click HERE to go the flash briefings.


I hope that all of you had a fantastic week. Do something for yourself this upcoming week that will make you a better person. God bless you and I'll see you soon.

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Monday, May 13, Through Sunday, May 19 - What an unbelievable week. The only time that I left the house this week was when I went for physical therapy on Tuesday. The majority of the time I was back in my home office working for BioNews Services. We are eagerly anticipating the approval of AveXis's drug candidate ZOLGENSMA. We know that the announcement will probably come before May 31, but we are hopeful that this announcement will come during the upcoming week. We also received exciting news regarding an article that was published by BioNews Services. In this article, it states that AveXis has submitted additional information from their clinical trials regarding the IT, or intrathecal delivery of their drug candidate ZOLGENSMA. While we understood that the initial acceptance of ZOLGENSMA would only be for infants with Type 1 SMA who were 9 months of age or younger, we are learning now that AveXis is asking the FDA to broaden the label to include children with Type 2 SMA who are 5 years of age or younger. If the FDA approves this request by AveXis, and the FDA broadens the label to include this new set of patients, this treatment will be available to a wider patient population. My hopes are that if the FDA broadens the label like AveXis is asking for, those of us who are adults who have SMA Type 3 and 4, will possibly be able to receive this treatment earlier than first thought. While nothing is definite as of yet, the excitement level has been ramped up a few notches.


This past week, when I went for my workout with Emily, she and her assistant, Vicki, transferred me from my wheelchair to the therapy table. This was the first week in nearly 2 months that I was able to do my workout with Emily and Vicki. They used to set me on the edge of the therapy table so that we could work on my balance exercises, but since my balance is improving, Emily is now having me sit on a cushion that makes it much more difficult for me to gain my balance and remain in an upright sitting position. After positioning me on the cushion and after I gain my balance, Emily stood behind me and Vicki stood in front of me. Emily began pushing me forward, backwards and from side to side, and sitting on this cushion made this exercise much more difficult. Emily didn't have to push me near as hard to make me lose my balance. We worked on this for nearly 15 minutes, and by the time we finished, my abdominal muscles were screaming. Overall, Emily and Vicki said that I did a great job and that it would get easier as time goes on. After finishing my balance exercises, Emily and Vicki laid me down on my back and Emily began working on the range of motion and stretching exercises on my legs, knees, ankles and feet. Along with stretching my legs out straight, Emily is now sitting on the table and placing the heel of my foot on her shoulder. She then places both of her hands on the upper portion of my thigh, just behind the knee cap. She will then pull my leg as straight as possible, which really works the hamstring muscle. After stretching my legs out, Emily places my feet flat on the table and begins pressing down on my knees. This not only brings my foot up toward my body to correct some of the dorsiflexion, it also stretches the calf muscle and the Achilles tendon. This particular exercise is not overly enjoyable, but it really stretches these muscles and tendons out, and I can tell after I finish my workout, these muscles feel much better. Emily is also bringing my knees up toward my chest, and then pulling my knee in a downward position which really stretches the muscle along the side of my hip. This is a great exercise, and I can tell you, my hips have never felt better. After doing these particular exercises, I'm pretty much pain-free for the rest of the week.


 While I only do my videos for my YouTube channel every other week, I’m producing another video this week because of some exciting news regarding AveXis’s drug candidate ZOLGENSMA. We have received information that AveXis has provided additional information to the FDA, and have asked the FDA to broaden the label of their potential new drug therapy. The initial request was for SMA Type 1 infants, who were 9 months of age or younger. Now, AveXis is requesting that the label also include children with SMA Type 2, who are 5 years of age or younger. Since this is exciting news for all of us who have spinal muscular atrophy, I thought I should produce another video to keep everyone updated with regards to the latest news. Click HERE to view my video.


I've updated the flash briefings that I do for BioNews Services. Click HERE to go the flash briefings.


I hope that all of you had a fantastic week. Do something for yourself this upcoming week that will make you a better person. God bless you and I'll see you soon.

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Monday, May 6, Through Sunday, May 12 - It seems as if each week tends to go by faster and faster. I was hoping that this week would follow suit. To my surprise, it did. While I usually spend the majority of the days in my home office, on Wednesday, my house was swarmed with carpenters putting down nearly $4000 of new carpet. I had my house built in 2000, so I guess it was time for some new carpeting. A few weeks ago, someone rear-ended my caregiver while she was driving my van. While there wasn't a great deal of damage done to my van, it was still in the shop for 5 days. The insurance company provided me with another accessible van that we can use while mine was being repaired, but since it only took 5 days to get my van repaired and back to me, I never had to use it. My caregiver told me that my 2006 Toyota Sienna minivan drove better than the 2016 Dodge Caravan that they gave him as a loner. Suffice it to say, I was glad to get my van back in the garage where you belongs. One day turned into the next and before long, my week was over. 


This past week, when I went for my workout with Emily, she performed her 4 week reevaluation on me. The only thing that went wrong during this reevaluation was the measurements on my feet and ankles. My left foot, the one that I injured a few weeks ago, actually got better, and my right foot, was a little bit more swollen than what I would've liked, and the test results were not as good. My strength tests were much better with regards to my core muscles, so overall, I was pleased with the results. I'm back on my hydrochlorothiazide, and will stay on this until I get the swelling in my feet and ankles down to a more manageable level. After performing our reevaluation, Emily and I worked on my core strengthening with the resistance bands and isometric exercises. Now that Emily's physical therapy students are gone, I will resume working out with Emily, and her assistant Vicki. While I enjoyed working out with her student therapists, I've missed working out with Emily. You get used to working out with someone, and you don't really realize how much you enjoy their company. It is been a few months since I saw Emily's assistant, Vicki, because I was working out on Wednesdays, and this was usually her day off. Vicki said that she was excited that I was back on my Tuesday schedule, and I'm looking forward to my workout this coming week. When I go for my workout with Emily and Vicki next week, they will transfer me from my wheelchair to the therapy table, and we will do all of my normal balance exercises along with the stretching and range of motion exercises. Instead of sitting on the therapy table, Emily is now placing a therapy cushion under me. This is the type of cushion that they use for patients who have had foot injuries and are rehabilitating their ankles and feet. Two weeks ago, she had me sit me on this cushion, and it made my balance exercises much more difficult. By the time my balance exercises were over, I was sweating like a stuck pig. Maintaining my balance on this therapy cushion was a lot more difficult, and it definitely gave my abdominal muscles a great workout. Emily said that it would get easier over time, and I'm sure it will. My balance has definitely improved, and my core muscles are a key reason to my success. Emily did say that she was going to focus a lot more attention on the Achilles tendon's. She does this by placing my foot flat on the therapy table, and by stabilizing my foot with one hand and pressing down on my knee with another hand, this not only stretches the calf muscle, it also stretches the Achilles tendon. We had been working out my left Achilles tendon quite a bit due to my injury that I sustained a couple weeks ago, and she said that's probably the reason why my reevaluation on my right foot was not as good as my left foot. Emily almost had a sheepish grin on her face when she said that she was going to pay more attention to the Achilles tendon on my right side, so I'm sure that my workout on Tuesday will be one that I'll remember for quite some time. To be honest with you, I'm actually looking forward to it.


In this week’s video, I discussed the latest with regards to AveXis’s drug ZOLGENSMA. While there is not an exact date as to when the FDA is going to make their announcement, I do share a little information that I received about a possible date, along with discussing what’s been happening in my life for the past 2 weeks. Click HERE to view my video.


I've updated the flash briefings that I do for BioNews Services. Click HERE to go the flash briefings.


I hope that all of you had a fantastic week. Do something for yourself this upcoming week that will make you a better person. God bless you and I'll see you soon.

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Monday, April 29, Through Sunday, May 5 - This past week, I spent quite a bit of time working on the Achilles tendon in my left foot. If you read my journal entry last week, I told you that I almost snapped my ankle, when I got it caught in a doormat in my podiatrist's office. I was actually surprised at how quickly my ankle started feeling better, given the fact that I came close enough to doing damage that could've required surgery to correct. Overall, my week went well, and this upcoming week is filled with events that will definitely keep me busy.


This past week, when I went for my workout with Emily, this was the last week that she would have her physical therapy student Derrek to work with me. As soon as I got there, Derrek and Emily transferred me from my wheelchair to the therapy table, and we began doing my balance exercises. Three weeks ago, when I went through my 4 week reevaluation, Emily told me that she was going to change up the way that we did my balance exercises. She told me that my balance was getting better, so instead of sitting flat on the therapy table, she was going to have me sit on a cushion that they use for balance exercises for people that have had a foot injury. While I laughed at her when she brought the cushion over to show me, I thought to myself, how hard could this be? Well, my questions were answered. They put the cushion on the therapy table and transferred me from my wheelchair and got me centered on the cushion. As soon as they were trying to help me gain my balance, I realized just how much more difficult this was going to be. It took me a minute, but I finally got my balance and I can tell you from first-hand experience, this definitely made the exercise much more difficult. With Emily in front of me and Derrek behind me, Derrek began pushing me forward, backwards and from side to side. Sitting on the cushion did make it much more comfortable on my hip, but by making it more comfortable on my hip, this meant that we were going to work longer on the balance exercises. After nearly 15 minutes, and after I was sweating like a stuck pig, we decided it was time to lay me down on my back and began the range of motion and flexing exercises on my legs. My abdominal muscles definitely felt this new exercise, but Emily just smiled at me and told me that it would get easier as time goes on, and that she was already thinking of other exercises to do to make it more challenging for me. While Emily is an excellent physical therapist, she's definitely not going to take it easy on me, which, is probably exactly what I need. My flexing and range of motion exercises went well, and we incorporated a new exercise in my flexing that involved an exercise called a figure 4. If you look at the photograph just to the left of my journal entry, you can see what this exercise looks like. This really works the muscle that runs along the side of your hip, and while it is uncomfortable to do, this particular exercise has really helped to stretch that muscle out, and it make both my hips feel much better. Next week, I will go through my next 4 week reevaluation, and then we will start the process all over again. For the past 2 months, I've been going to my workouts on Wednesday. I did this so that Emily's physical therapy students could work with me. Now that both of them are gone, I will go back to my normal schedule which is on Tuesdays. I'm actually glad to go back on Tuesdays, because Emily's assistant, Vicki, will be working with me again, and she's a lot of fun to work with.


I've updated the flash briefings that I do for BioNews Services, and will begin working on my next video, which should be up on my YouTube channel next Sunday. Click HERE to go the flash briefings.


I hope that all of you had a fantastic week. Do something for yourself this upcoming week that will make you a better person. God bless you and I'll see you next Sunday.