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!

Creative and Courageous in Colorado

I recently took a trip that I have dreamed about for a while, and didn't think would ever actually happen. I took a train on an 18-hour ride from Chicago to Denver, and spent a few days in the spectacular mountains of Colorado! On my own, this would be impossible. But the Lord went with me, and he made it possible for two very dear friends to go with us too! 

My brother, Kevan, has taught me that anything is accessible when people are creative and courageous, and I definitely saw that demonstrated on this adventure. Here's some examples...

• First of all, my dad is a true McGyver-genius. This summer, he adapted my portable "Connie lift" (which connects directly to the back of my chair and plugs in to run off the battery) so that it's made of two pieces of light and strong aluminum, making it even easier to pack, transport, and handle (in my preppy-looking golf club bag!). This baby went everywhere with us, and did all the lifting so the girls and I were safe and comfortable the whole time.
• I have to give major kudos to the state of Colorado for their accessible restrooms and transportation. Big, private, family restrooms were basically everywhere, so I never had to cut our outings short to run back to the hotel multiple times a day. Family restrooms in public places are. the. BEST. (Take note, rest of America.)
• The sleeper car attendants on the train were really wonderful and helpful, checking on me frequently, taking room-service meal orders, and providing three extra pillows just so I could comfortably sleep on the cot through the night (which I did... slept like a log!).
• Then there's the waiter at Hard Rock Cafe in Denver... the silverware was too heavy and cumbersome for me to handle, and he couldn't find plasticware in their kitchen. I told him it was fine and I'd make it work, but he ran outside (in the rain) and across the street to get a plastic fork from another restaurant, and brought it back for me anyway.
• Thank you to the National Parks for being wheelchair-friendly! There were paths I could take everywhere we went - paved or dirt, flat or hilly, but always scenic and beautiful. It was such a blessing to feel free to hike to summits, stroll along sparkling lakes, and climb red rocks! 

• My favorite story from the trip was in Estes Park at the aerial tramway, which claimed to be accessible. True, there was a ramp, and true, there were no steps, but... to get from the ramp to the tramcar, there was a pretty hefty gap that my chair wouldn't cross. The two middle-aged ladies in charge did their best but realized the challenge was beyond them. Just when I thought they would say "Sorry, we can't do it," they looked at each other and both said, "We'll call Adrian." A couple minutes later, the tram came down the mountain toward us, and a young guy with sunglasses on could be seen, fists on hips like Superman, and a wide confident grin on his face. Adrian, I presume, I thought, amused but doubtful of his abilities. He came over and took a good look at all angles of my chair, asked a couple good questions about it (NOT including, "How fast does that thing go?") and tested some handholds. Then he gave me a few instructions on how to angle my wheels and a few explanations of what he was about to try, and before I could be scared, I was safely in the tramcar! Adrian is a magician! No, but he's an engineering student who, I'm convinced, has other disabled people in his life... this was clearly not his first attempt. He actually had adapted the platform at the top of the mountain so that it truly was more accessible. So we went to the top, got a coffee and some souvenirs, fed the chipmunks, and then let Adrian accompany us safely back to the bottom.
  
  
  
  
• Last but definitely not least, Pam and Rachel - the most creative and courageous of all! They both were full of energy, strength, optimism, and hope through the whole journey. Nothing phased them. They constantly were paying attention to find the best places, views, and activities that we could do together, so that I forgot that I had a disability at all. On the last day, the only disappointment we faced was that we couldn't figure out how to navigate the mineral hotsprings spa that claimed to be accessible, but really wasn't at all. We'd been looking forward to it all week, but I tried to forget it and hide my disappointment. But these girls took me back to the hotel, ran a hot bath scented with lavender oil, turned on soothing music, and gave me some time to soak and relax. A perfect, peaceful end to a thrilling and adventurous week. Pam and Rachel were the perfect people to join me, and they made this a trip I will cherish forever. 

Whatever limits or challenges you face, remember that God is creative and courageous, and the master of tackling what seems impossible to us. When we put our hope in him and look for people around us who live out these aspects of his image, the possibilities are endless - and adventure awaits!

It's Your breath in my lungs...

I've written quite a bit over the years about breathing, and particularly my struggles to breathe. I've been reviewing some of my old posts, and I listed some of the links below, in case you want a review, too. The interesting thing to me is that most of these posts also include song lyrics - worshipful songs about air, breath, spirit and strength. Singing, to me, is a sort of joyful cry of victory against weakness and despair... it is the evidence of a Power deep inside me that is much greater than me.

Today I had my 6-month pulmonary check-up. I did the obligatory annual breathing test and let the doctor listen to my lungs. The conclusion was that my respiratory strength is the same as it was last year, and to me that was really wonderful news.  See, in the past year, I've had two surgeries in which I had to be intubated, and I was in the hospital with pneumonia only four months ago. I really expected that my strength would have weakened with all this drama.

When I originally talked with my neurologist about what Spinraza might do for me, she said, "At the very least, it should stop the progression of the disease." At the very least?? a disease that has slowly chipped away at my strength and health would be stopped?? Wow, what a miracle. I wonder if Spinraza is working to stabilize my lungs. I wonder how that happens, how protein injected in the spinal chord is able to do this, and I wonder what exactly "this" is that it's doing.

I wonder about science and research and medicine, and the mysterious role it might play in reversing bits and pieces of the curse of Sin... But I don't wonder about Jesus. If I am certain of anything, it is that He is my Strength and my Portion and my Song. He is the breath in my lungs, and I pour out my praise to him only.

Past posts about breathing:

Sept 15, 2011
Nov 1, 2011
Jan 25, 2012
April 8, 2012
April 27, 2012
June 21, 2013
Dec 5, 2017
Mar 2, 2018

Dose #6 of the Super-Soldier Serum

Today I got my sixth dose of Spinraza - it's been 10 months since I began this journey with the first dose!

The lab work was a success - as in, it only took three attempts to find and retrieve two tubes of my platelets... So that's good, right? I think I'm becoming notorious around the lab. The lady who called me back actually remembered me from four months ago, and promptly rounded up three other phlebotomists to assist her. Special thanks to the one who gave me a "Skylander" Band-Aid, and major kudos to the feisty little lady who stabbed me fearlessly and wouldn't take "No" for an answer from my shy and elusive veins. I saw my neurologist on the elevator afterward, and observing my multiple bandages and bruises, he casually said, "So... I see you got your labs done." Mom treated me to a cherry danish and tea afterwards, which is becoming my regular post-blood-draw reward for NOT crying or biting anyone. Overall, not a terrible morning.

Three hours later, I was in Diagnostic Neurology, signing my name in acknowledgement that this treatment could result in headaches, bleeding, infection, or DEATH. My doctor is always quick to add that the "death" part hasn't happened to any of his patients so far, blah-blah-blah... I waved him off and signed with a flourish. Please, give me this moment to face Death and feel just that brave! Again, that epic scene from Captain America flashed through my memory, and I smiled as I told myself that it's "super-soldier serum" time. I was really quite proud of how cool I was being about the whole thing.

One more hour later, and I'm laying on my side, facing a taupe-colored wall ("They say taupe is very soothing"). I can't see what's going on, and no one is giving me any verbal hints. I know there's a small needle in my lower back, and we're somewhere in the process of either sucking out cerebral spinal fluid or injecting Spinraza. It doesn't hurt me at all... I felt the initial stick of the needle, but honestly that is it. My doctor is very careful and cautious and takes his time to make sure everything goes all right, so I just need to lay still and stay calm and not be the annoying kid who keeps asking "are we there yet?". So while I try to find patterns and count the various shades of brown in the wallpaper, I reflect...

Last year at this time, I was still in consultation mode with the neurologists. Lots of unknowns, but also lots of optimism. I knew there would be risks, but I didn't know how complicated, long-term, or painful it would be. The past year has been quite a roller coaster ever since... as the Lord uses this medicine to strengthen and sustain my body, he seems to have a correlating (if not increased) interest in the condition of my heart... to remind me to find my strength, purpose, joy and hope in Him. Again and again, he proves himself almighty and faithful to me.

Today's treatment went well, with no problems. I had a little trouble with my breathing shortly afterward, and felt tired and emotional the rest of the afternoon, but the doctor thinks that's all due to stress relief... I guess I was more anxious and stressed out beforehand than I wanted to admit. But all in all, I'm curious, and yes, even excited to see what happens in my life in the next four months before my next injection... I'll be sure to keep you posted!

I have this hope...

I had the privilege to go to a special luncheon last Saturday for local families of Spinraza patients. I met a man in his 50s who started taking Spinraza at about the same time I did, a mother whose 7-year-old daughter is on Spinraza, and a mother whose 3-year-old daughter was one of the earliest Spinraza patients. It was really wonderful to meet these people, hear their stories, and get to share my story with them.

The thing that brought us all together was Spinraza, and I know one of the main purposes for bringing us together was to talk about how great it is. And there is nothing wrong with that - I'm a big fan of this treatment, and I'm excited to see how it will help me and lots of other people. I am thrilled to know that young children with SMA now have the chance to develop their motor skills, and that adults with SMA can maintain and rebuild their strength. I am so thankful to know that Spinraza is just the beginning - it has opened doors and increased research opportunities for other treatments and possible cures to be discovered. It's a miracle, and it is changing lives all over the world.

But my hope is not in a drug called Spinraza. Long before an answer was found, long before the FDA approved it, long before doctors were able to conclude their diagnoses with: "...but a treatment is coming soon," I found peace and hope and joy. It came in the form of a relationship and a promise - and it didn't ignore my disease, but it also wasn't contingent upon it. I chose to take hold of it as a small child, but it's taken a lifetime to understand. I've wrestled with it, desperately clung to it, and almost lost it at times... it's stretched and grown and deepened, at about the same rate as my muscles have tightened, shrunk and atrophied.

No matter how small, weak, sick, or unable I am or become, Jesus is strong and constant and great and mighty to save me. He promised never to leave me and never to let me go, and after all the things we've been through together, I can't help but trust him. He promised that he knows and understands me fully, and when I read and remember all that he sacrificed and suffered for me, I know it is true. No matter what else changes in my life and my world, his promises stay the same. His love, mercy, and faithfulness to me has never, and will never fail - this is my hope! 

I'm thankful God has given me the chance to experience a treatment for my disease, and that I get to see his creativity, intelligence, and ingenuity through medicine and science. But whether or not Spinraza has any effect on my body, I know my life is purposeful and beautiful and valuable, because Jesus has saved me and made me whole.

Setbacks and Progress

I had my first maintenance dose of Spinraza on February 5, and with that milestone I set some personal goals. See, I've learned that this treatment provides potential - potential for muscles to strengthen and rebuild - and I needed to start doing some serious work to live up to this potential. More stretching, more exercising, more intentional diet, more breathing... basically just moving and working out whatever I can. I kicked off this knew season with a little trip to Florida on Feb 8-14, to soak up some much-needed vitamin D and deep-breathe that salty sea air and digest as much seafood and fresh fruit as possible. Mission accomplished, I came home determined to get to work.

But five days later, on Feb 19, I woke up with a sore throat and hacking cough, and three days after that, I found myself in the ER, getting chest X-rays, blood work, IVs, and without much hesitation getting admitted with pneumonia. I felt weak, exhausted, dehydrated, congested, and discouraged.

Setbacks. That was the title that overwhelmed my mind for the four days I spent in the hospital, and to be honest, it's continued through some darker moments since I've returned home this week. Wheezing and coughing without much in the way of results and relief eventually burns a hole or two in the human spirit. It felt like I was wasting away, shriveling up, getting weaker at the time I should be getting stronger. I knew that taking Spinraza wasn't going to automatically make me immune to illness for the rest of my life, but it still made me disappointed to face my old enemy again so soon.

Progress. In my brighter moments of optimism and clarity, I have noticed some things that were different about this experience. I've been hospitalized for pneumonia many times throughout my life, but I believe this was the first time I was never put on oxygen support. The whole time I've been sick, my O2 saturation levels have been 96% or higher, with no outside assistance! The nurses told me this probably was a big factor in me being able to go home so soon - they didn't have to ween me off the oxygen. I was only in the hospital for four days - which is a noticeable improvement to the two-week stints I've had to endure in recent years. My hospital stay also did not include the rather uncomfortable measures of a bronchoscopy or nasopharyngeal suction, which have been necessary in the past. And the respiratory therapists utilized a High-Frequency Chest Wall Ossilation vest, which I've not been strong enough to handle in the past, but worked really well for me this time around. I have an idea that Spinraza has something to do with these remarkable bits of progress.

It's been over a week, and I'm still coughing, still fighting, still working toward recovery. But I'm doing better and getting stronger every day. Thankful for minimal setbacks and encouraging progress.

Handicrafts

One more week until my first post-loading-dosage treatment! Medically speaking, my life has been pretty quiet for the last three months, which I am incredibly thankful for. There's been no more swelling or leaking. I've dealt with some sciatic nerve pain, but that is improving with stretches and a new seat cushion.

The big question people constantly ask me is: Do you notice any improvements or change? It's hard for me to identify specific things, but others have told me that my neck seems stronger and I'm able to bend my arms easier.

Over Christmas break, I tested my ability with some hand-crafted projects: a cross-stitch "baby's first Christmas" ornament for my darling baby niece, Josie, and this nifty crochet hat for Kevan, which he said reminds him of old-school John Reuben:

I was pleased that I was able to complete both projects, and in a matter of days instead of weeks. I haven't been able to do these kinds of projects for about three years. One thing that made me laugh is that while I was working on them, my eyes got tired before my hands did! Being able to do these things is a huge encouragement to me, because it means my fingers, wrists, and arms are getting stronger. I am so thankful to be able to use my hands to create things that are beautiful and useful.