Wednesday, June 5, 2019

The Not-So-Normal Life

I got my #9 Spinraza dose last Friday!

I think I've got the formula for successful blood-drawing finally figured out: 1) Drink A LOT of water beforehand, 2) Keep my hand VERY warm (wrapped in a heating pad!), 3) Stay SUPER calm.  It's worked for me the past three times - one-and-done stick, in-and-out in 5 minutes. So if anyone else out there has a lot of trouble in that realm, maybe you can try my formula and see if it works for you!

We're still working on finding the most efficient/least uncomfortable way to dose. I think we figured out the best position for me to be in this time, and we may have found the best size needle to use... though we'll do it all next time and see if it works again. Tenth time is the charm!


I've been thinking about prognoses and predictions lately, especially related to kids with disabilities. It's come up several times in the past week, because people say that now, because of Spinraza, kids have a chance at a "normal life," which kind of makes me sad. Let me explain...

Often a doctor or caseworker or someone "who knows things" says that a child with a diagnosis (like cerebral palsy, spina bifida, Downs syndrome, or SMA) or who has been through horrible, traumatic experiences will "never have a normal life." This news is broken to family very solemnly, usually with sad eyes or a shaking head, like it is a shame and a tragedy. But I want to respond to that by saying, "Amen, and praise God!" Who wants a normal life anyway? Seriously, that sounds so... boring. Why would anyone expect (or even hope) that a child who has faces extraordinary adversity, obstacles, challenges, suffering - pick a word, any word! - will at best move forward in an ordinary way?

These are the people who have the potential to make a dent in the universe!
They have incredible insight, creativity, and resiliency when problems arise. They have the unique super-ability to notice, relate to, and speak into the pain and suffering of others. And they are the ones who can inspire and encourage and challenge those people in the world who are unfortunate enough to be living "a normal life."

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Some quick examples of amazing people who had a challenging childhood are Helen Keller, Nick Vujicic, and Jean Driscoll, Joseph (from Genesis), Malala Yousafzai, and Bruce Wayne... but I'm sure if you think about it for a minute you will think of more, whether from the history books, story books, or your personal life. Imagine what our world would be like without their leadership and influence!

So many people in our world have a backward perspective. They think "normalcy" is a dream to be achieved and suffering is a nightmare to be avoided at all costs. But I know that isn't the way God sees it; every life is valuable and precious to him, and he can take every weak and broken piece and transform them into powerful and beautiful things.

Whether a child ever speaks a word or takes a step, their lives can speak volumes and have a butterfly effect that touches hearts and shakes the world.
Don't despair for the children in your life who endure painful and difficult things; help them to be courageous, strong, and full of hope. Remember - and remind them often - that this is evidence that God has BIG plans for their lives and there is EXTRAORDINARY work he wants to do through them.

And thank God for every child you know who is not "normal."

Tuesday, February 5, 2019

Keep it going

It's February now, and I think it's time for an update.

I was able to do 10 weeks of physical therapy this winter, twice a week! I loved it. It felt good to have a regular "workout time" scheduled into my week, both mentally and physically. My joints ached a lot less, I found that I could just move easier, and I felt more purposeful in my self-care. I liked being able to see quantitative, measurable improvements, even if they were small, but I got excited when they were significant, too. We saw more significant progress in the first five weeks, and then it kind of leveled off over the last five weeks.

When I began, and I was told to kick my leg out or lift my arm, nothing happened. But as time went on the correct muscles began to respond - fire, activate, engage, whatever you want to call it - they were obeying my brain's command to "move" in a way they haven't done in years. So even though I still can't visibly kick or punch, my muscles are training themselves to do those things.

A couple new things I have noticed that I can do now: tear away Christmas present wrapping paper and slowly set down a mug of hot tea (instead of dropping it). A dear friend told me last week that she noticed that I am doing more things and my energy lasts longer throughout the day; I used to do less and get tired a lot faster. 

On January 28, I went in for my #8 treatment. I asked my neurologist if he thought I should continue with PT, and he said, "If you think it's helping, keep it going." In other words, "Whatever makes you happy." He didn't have a whole lot of new insights for me, and I was a little frustrated... I don't want to waste time, energy or resources on something like this. If I'm going to keep it up, I want to know it is making a difference - fast or slow, small or significant, it's about forward motion, people!

So, I called the Biogen representative, whom I affectionately refer to as "my drug guy." As usual, he was incredibly helpful. He said that the research has shown on-going significant progress in Spinraza patients. If you look at the graph at the bottom of this page, you'll see that the progress isn't steady and constant, though. There are times when it levels out or slows down and there are times when it makes big leaps. But no matter the rate, it's upward, forward, increasing progress. So my drug guy encouraged me that even though my progress in PT seemed to plateau after five weeks, that doesn't mean I've maxed out my potential; the potential for growth increases over time, just as the potential for deterioration increased over time before Spinraza.

I've decided to resume PT in March. I think what may happen is that I do a month of PT and take a month or two off, and then do another month... or something like that. I don't know for sure yet, I want to consult with my therapist. But on my "off" time, I don't want to lose momentum. At home in the mornings and evenings I'm doing the exercises I've learned, a little bit every day. I'm hoping to maintain what I've gained, and when I go back in March, we can add in some new things and slowly build it up.

I'm learning the value of steady continuity, in intentionally using muscles and pushing them a little further all the time. Keep it going!

Friday, November 2, 2018

Push, Pull, Squeeze, Hold

I've taken my Spinraza treatments to the next level!

In October I received dose #7, and realized I've been doing this for a year now. I feel more energetic and have longer-lasting stamina. I breathe well without being daily dependent on Albuterol. I have more movement in my fingers.

But I feel that there is a lot of unknown and untapped potential for my muscles. For 25 years, I've been wheelchair-bound, unable to bear weight, and slowly using my arms and legs less and less. Things are tight and small and weak, but I wonder if that could change now... What if I could open doors again? What if I could brush my own hair again? What if I could help with my own transfers? No one seems to know what is possible, so let's find out! It's a bit like Spider-Man, getting new superpowers and figuring out what he is capable of and how to use them...


I talked with my neurologist about this, and he agreed and prescribed physical therapy. Last week, I went to a consultation, and explained my situation to the PT. After an interview and exam, and copious note-taking, he seemed really excited to take on this challenge with me, and said we should start with 2 sessions a week for 8 weeks, and see what happens. So, after basically 25 years, I am back in PT!

My PT assigned me some homework to do in between sessions. These are muscle contraction exercises I can do in my chair or in bed, by myself. In our sessions so far, we've been doing isometric exercises, which means he will raise my arm or leg to a certain suspended level and tell me to try to hold it up, or at least support it as it slowly falls. We also do resistance exercises. It doesn't look like I'm doing anything, but it's hard work for me! Even though I'm not actually moving much, my heart rate goes up and I break into a sweat because I'm focusing on trying to get my muscles to respond - push my fists against inanimate objects, pull my foot up and down on the mat, squeeze my shoulder blades together, and hold... everything. I think the holding is the hardest part so far; with every exercise, I'm supposed to hold the position for 10 seconds. That tension is difficult and exhausting to maintain. (It feels like there's a life metaphor in there, doesn't it?)

I've completed 2 sessions (1 week) in the schedule now, and I'm sore. But I'm also excited and hopeful... I'll let you know how things process over the next few weeks!