A SPINRAZA UPDATE: TO BE CONTINUED

Right about now, I should be laying on the operating table.

I should be clenching my mother’s hand and taking slow, meditative breaths as the surgeon carefully stings my lower back with precision. I should be listening to every click and every spin as a fluoroscopy machine rotates around my body. I should be receiving my fifth dose of Spinraza.

But, instead, I am here. Writing to you from my couch and listening to the rain fall, wishing things could have panned out a little differently today.

“I’m not sure if Monday is in my best interest,” I painfully said to my surgeon over the phone on Friday evening. The words tasted sour as they slipped off my tongue. How did I get here? I worked so damn hard to stay healthy this winter, then something completely out of my control decided to dictate this heart-wrenching decision. But, despite wanting to go for another treatment in the worst way, I knew I had to listen to my body, first.

“You’re a very unique case, and it’s always hard to determine what’s wrong with you,” he reminded me over the phone, advising against a lumbar puncture today. Just to confirm, he dialed in my neurologist who also agreed.

Of course, I’m a unique case, I thought- it’s been the same story for 26 years. But, as much as society tells you that being unique makes you admirable and different, every once in awhile, I wish I could just conform to the norm. I wish I could go to a doctor, have them tell me exactly what is wrong, and send me on my merry way. I wish I wouldn’t self-diagnose on WebMD and work myself into an absolute panic on all the possibilities that could be going wrong. I wish I had answers.

In my previous post, I mentioned how I almost passed out at the dentist. Well, what I thought had been a fluke had become an unfortunate routine for me. Three and a half weeks later, I continue to feel lightheaded. I continue to be sensitive to light. The slightest motions continue to set me off. And, these mini fainting spells continue to occur.

Worst of all, I’m not me. I’ve learned to put on a great show for others, and at times, I do have glimpses of my old self. I’ve learned the triggers and how to respond to them. But, it’s not enough. I’m generally a positive person. I like finding the good in every bad situation, but I’ve exhausted all my attempts at trying to make sense of this. I’ve reached a point where I don’t care about what’s wrong with me, and I’m tired of wasting my days in an ER or doctor’s office. (Doctors have some ideas, though.) I just want to get better. 

Amidst trying to find answers, though, I remind myself that this is all, and forever will be, in God’s hands. He is in charge of guiding the way, and I am in charge of following by faith. Today’s procedure wasn’t a part of His plan, and despite wishing it had been, I put my trust in Him knowing that letting go and putting my faith in God is the only type of control I will ever have in my life.

The past four months I’ve been extremely fortunate. Although I haven’t gained any strength since my last update, I’ve MAINTAINED, and everyone in the SMA community knows how victorious this sweet, sweet word is. Maintaining is good, and maintaining is promising. It means Spinraza is still residing in this little body of mine and working its magic. And, it means I still have time.

Time to fight whatever it is I’m fighting. Time to recuperate afterward. And, time to let God lead the way to my next appointment.

To be continued.

WHAT THE MOST SIMPLE, YET POWERFUL, ADVICE FROM MY DOCTOR TAUGHT ME

Emotional pain is like a summer storm. As you’re enjoying the warmth radiating from the sun, a dark cloud-covered sky arrives and indicates what’s about to come. Lightning strikes. Thunder claps. The earth shakes. And, you scramble to find shelter to keep yourself safe. Pain often works the same way. It’s unpredictable, damaging, and springs upon you when you least expect it.

A year ago today, April 2016, was when I experienced one of my most painful periods and deepest lows to date. Just like a storm, this period of my life left a mark- one that I have not been able to shake.

I had been hospitalized for another illness, but this time, it took a huge emotional toll on me. Over the past couple of years, my health had taken a steep decline as my disease progressed rapidly. (Thanks to Spinraza this is no longer the case!) I lost the ability to speak a single sentence without feeling winded. The energy it took just for me to speak affected my life greatly as my body was working in overdrive to do a simple, daily task. I was exhausted both physically and mentally and somehow coaxed myself into thinking that the next time I was to get sick would be my last.

I cried every single day in that hospital bed. I was irritable. I was in physical pain. I had tubes all entangled in me making me feel uncomfortable.Worst of all, I actually felt sorry for myself. I wondered what was even the point in trying. I was just so beyond tired of fighting.

However, several days into my stay, my SMA critical care specialist walked into my room, his eyes wandered around, and spoke the most simple, yet powerful, words that suddenly changed my perspective. (To this day, they still resonate with me.)

He looked at me and said, “I know this isn’t fun. You’ve been sick for awhile, haven’t eaten in days, and have a really uncomfortable tube up your nose and in your stomach. But, it’s 2pm, and it’s pitch black in here. Let the sun shine in. It’ll make you feel better.”

He walked to the back of my room, threw open the shades, walked back to me, and said, “I know this isn’t where you want to be. I get it. But, I also know how strong and stubborn you are. So, promise me you’ll keep those shades open, and I promise you’ll be out of here soon.”

He was right. I was strong (and stubborn as hell), but I had somehow allowed circumstances that were out of my control to control me. For the first time in my life, I felt like a stranger in my own body. I wasn’t that girl nor did I want to be her. And, as the sun began to radiate into the cold, dismal hospital room, I began to feel lighter- a shift of energy began to take place. That was the moment I realized my thoughts and feelings needed to change. I needed to stop playing the victim and start recognizing the resilient power I had hidden inside of me.

Of course, it’s important to let your negative emotions ride out. Scream, cry, feel sorry for yourself, and just plain ol’ hate life. Be bitter. Be angry. Be anything you feel like being because you’re entitled to feel whatever it is you want to feel. But, there comes a point when you have to realize that these emotions you’re feeling are not in control of you. Instead, you are in control of them. Knowing this, I came to realize from his advice, is the foundation for overcoming any and every “storm” of life.

Storms, just like pain, are messy, but that’s just their nature. They wreak havoc, cause destruction, and do everything in their power to destroy what once was. Every now and then, getting caught in the middle of a storm is a fact of life, but fortunately, they don’t last forever. The raindrops will taper, the clouds will begin to part, and the sun will start to shine again. Only then will you decide to rise above those negative feelings.

And, only then will you decide to open the shades and realize you are strong enough to let the sun shine in again.

GUEST BLOGGER: FROM A NEWLY DIAGNOSED MOTHER

This month’s guest blogger comes from a mother whose son was diagnosed with SMA almost two years ago, Lynne Vaudry. Meet Evan!

I am honored to write as a guest blogger for Alyssa. As a mother of a young child with Spinal Muscular Atrophy, I have learned so much and been inspired by Alyssa’s posts. My son, Evan, was diagnosed with SMA type II at 2 years old after many months of doctor’s appointments and testing. Although it wasn’t easy news to hear about his diagnosis, my husband and I always tried to remain positive. I did know of a young lady in my own hometown who had Spinal Muscular Atrophy so, a few months after Evan’s diagnosis, I wrote to Alyssa Katherine Silva. I asked her if we could meet to talk, and she answered my request the same day I wrote with an enthusiastic, “Yes, I’d love to meet and chat!”
My husband, Steve, Evan and I met Alyssa and her mom, Dori, at a local coffee shop one March afternoon. Evan was wheeling around in his little manual wheelchair while the adults all chatted over coffee and donuts. We talked about how we shared the same doctors and how much is known about the disease today. Alyssa also reflected on her time in school and how important it was for her to play and socialize with friends. She told me about her wonderful experience of having a service dog and where the most accessible beaches were with beach wheel chairs and playgrounds for Evan. I went home that day with a new feeling of HOPE which was exactly what I needed at the time.

My husband and I still took Evan’s diagnosis one step at a time in order to fully understand all we could about SMA. We connected with the organization, Cure SMA, and read their information for newly diagnosed families. We learned about the appropriate physical therapy, equipment and home modifications necessary for a child using a wheelchair. We attended our first national conference for SMA in Washington DC, the same one attended by Alyssa and her family. We even joined the fundraiser committee for Working on Walking created by Alyssa.

Evan is now four years old and doing well. The shock of his diagnosis is gone as we’re focusing on doing the best we can for him as parents. Even though Evan uses a wheelchair, he wants to do the same things his peers do, just in a slightly different way. Evan swims, goes horseback riding, sledding, ice skating, and will try skiing next year. He’s not afraid to try anything!

During these past several years with Evan, I’m grateful that Alyssa started Working on Walking because it’s helping to raise money for SMA research, create awareness and provide hope to my son and others afflicted with this disease. Thank you, Alyssa.

GUEST BLOGGER: WHAT A LIFELONG FRIENDSHIP LOOKS LIKE

Last month, I introduced you to my very best friend, Amanda, in a post titled “To the Girl Who Picked Up My Pencil“. Amanda is a pretty remarkable girl and the type of friend everyone needs in their life. I’m extremely fortunate to call her my best friend. After receiving so many heartwarming messages on that particular post and the relationship the two of us share, I asked Amanda if she’d like to be this month’s guest blogger. She happily accepted, and here’s what she had to say.

 

In one of her recent blog posts, Alyssa shared the story of how we became best friends back in kindergarten. I was truly flattered by all of her kind words, but cannot begin to tell you how lucky I feel that Alyssa came into my life and has never left my side. You often hear people say, “Everything happens for a reason.” Although it may sound cliché, I strongly believe in my heart that Alyssa did come into my life for a reason. She has made such an enormous impact on my life, and words cannot express how fortunate I feel to have her. People have often come up to me with questions about Alyssa and her disease, and I feel that people are curious about what it is like to have a best friend with a disability. Although Alyssa’s life is very different from most, in many ways, she and I are just like any other pair of 24-year-old best friends. For example:

1. We spend our days talking about anything and everything.
2. We convince each other that we really need to buy that new shirt (even if we own 25 others just like it).
3. We have about 100 nicknames for each other, and can’t remember the last time we actually called the other by their first name.
4. We like to go out on the weekends, but are sometimes even happier spending Friday nights in sweatpants watching reruns of ‘The Office’.
5. We know exactly how to cheer each other up when we are feeling sad (for Alyssa, anything in the dessert category should do it).
6. We tease each other, but all out of love, of course.
7. We spend a ridiculous amount of time together, and will often send “I miss you texts” when we haven’t seen each other in a while (so, basically two days).

The list could go on and on. Alyssa’s life may be different, but despite the pain and fatigue she often faces as a result of her disease, Alyssa never lets that hold her back, especially when it comes to being a friend. I went to college at Sacred Heart University in Fairfield, CT, and, of course, I always missed Alyssa when I was away at school. For five years, we were two and a half hours and a hundred miles away from each other. One day during my senior year, Alyssa told me her New Year’s resolution was to take a trip to Connecticut to visit me at school, and, just a couple weeks later, I was woken up to find Alyssa sitting in my living room ready with a camera to capture my surprise! Despite how difficult and tiring it must have been, Alyssa drove two and a half hours each way just to spend the day with me and to see my school for the first time. This will always be one of my favorite memories, and I am so happy I had the chance to share my school with her. 

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As we are approaching our 20th year of being best friends, I feel as though I can’t pick just one “Alyssa story” to tell. In fact, I could probably write an entire book on how much she has inspired me, and changed my life. Having a best friend with a disability teaches you a lot of things. Alyssa has taught me the true meaning of strength, perseverance, and hope. She overcomes great obstacles, and never loses the desire to live life to the fullest. When I am having a rough day and feeling unmotivated, I often think of Alyssa and all she overcomes which helps motivate me to be better. I am so unbelievably proud of her and all she has accomplished and cannot wait to see what amazing and inspiring things she does in the future. There is truly nothing on earth like a lifelong best friend.

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