For You

September 11th, 2018 was a significant day. The anniversary of the biggest terrorist act the United States has ever seen, the day I came home from my first international trip, and the day that John H. passed away.

I won't pretend to have been really close to you. If there are pictures of us together I don't know where they are. We never had deep philosophical discussions. We never talked much outside of camp, except for maybe when one of us was in the hospital. Truth be told, I don't even know when it was we first met, you were just always there, a part of either the MDA camp or Camp Promise West family.

I don't really have any stories, just facts and observations. You and I were the same age, 26. As a kid 26 seemed ancient. I lost my first close friend with Muscular Dystrophy when I was 13 and by 20 I had lost 1-3 friends from camp a year. 99% weren't yet 25 and none of them had ever made it to 30. That amount of death at such a young changes you in ways you don't always understand. For example: I don't grieve “normally,” whatever that means. When I find out another person from camp has passed it can take days or weeks before it sinks in. I won't cry until I hear that particular song/story or see that picture. Even then, I only allow myself one good crying session because I feel like anything more damages the memories of my friends. I am more desensitized to death than I should be at this age. Or maybe I'm not desensitized maybe I just don't feel like death really cancels out life.

Anyway, now that I am 26, it does not seem so ancient. There is still so much I have to see and learn and thanks to medical advancements in my particular form of MD, I might actually have time to do said things. So no, 26 isn't ancient but it is lonely. Especially when cabins at camp that used to be full grow more empty by the year.

I said before that John and I weren't close which is true. But there is a special bond you have with someone when you grow up together and both have Muscular Dystrophy. It's being silent and watching the sun set while you sit right next to each other, because sunsets mean a little more when you know you might not make it to 30. It's knowing that our 26 does not emotionally or physically feel the same as the average 26 year old. It's sharing war stories of hospital stays, crazy caregivers/counselors, or the times we almost died. It's sharing a mutual respect for death while simultaneously giving it the finger because neither of us would go without a fight. It's constantly perfecting our personalities because we know it is the only thing we have to draw people in.

Speaking of personalities, yours was 1 in a million, John. When it came to sass and eye-rolling you were my male equivalent. When it came to your accepting heart and perpetual willingness to listen we were not equals; you were the master. You were the only proud, Republican who could make anyone love him and it was because your heart was beautiful. And while you would roll your eyes at my different beliefs you'd never shut down the conversation and that is a rare quality. I will cherish all the moments we spent at camp joking around or just being near each other. I'm not sure what camp will be like without your music, fart machine, t-shirts, and most of all your laugh. The Blue Cabin might just be a bit more subdued.

And if you taught me anything, aside from how to just be a better human in general, you taught me how to appreciate all the moments. Now I suppose I have to figure out how to go hunting, shooting, [insert any other country thing you were into] in Montana without you. Because Angie, Amber, and I owe you a road-trip.

The Side of Treatment we Don't Discuss: My First Dose

[I apologize for my hiatus but I was busy living life]

This is going to be a long one, folks, so buckle up. With treatments and cures comes some unpleasant feelings and I wanted to shed light on some of that.

First a little background: in May I posted this status on my personal Facebook (feel free to skip the quote if you've already read it), “July will mark 25 years since I was diagnosed with Spinal Muscular Atrophy(SMA), the #1 genetic killer of children under the age of two. In 1993 there was no hope or treatment. I was young when I figured out that every day meant I was a little bit weaker than yesterday. I don’t really talk about it, the bad days, the fact that I can only use two fingers, or the fact that there are days I can barely drive my chair. I don’t talk about it because it’s my reality and I’m kind of allergic to pity. I accepted the fact it would never get better and there would never be a cure/treatment.

Well, in December 2016, the first treatment, Spinraza, was approved for all types and people with all ages of SMA. If started at a young enough age it has shown to reverse the disease, the older patients don’t always notice drastic results, everyone is different, but regardless of age it has shown to definitely stop the disease in its tracks.

Well due to politics I have not been able to get the drug. I have been enviously watching kids here, adults nationwide, and worldwide get this drug. I’d lost hope. It’s been hard watching others gain strength while knowing I’m getting weaker.

Well, today I found out I’m one of the handful of adults that Aetna has actually approved the drug for. With a drug that’s 750k the first year and 350k every year after it’s a huge deal. The hospital here is finally dosing adults so I will be getting Spinraza very, very soon! Like maybe a month out.

I feel extremely blessed right now! Here’s to hoping the procedures goes smoothly. I will keep y’all posted." 

I mentioned Spinraza that one time and then I never mentioned it on public social media again. I think part of the reason I struggle with keeping up a blog is I am in constant debate about how much of my life belongs to me and my circle and how much I should share with the world. I shared that status without much thought because I was so flippin' excited. But then I felt a lot of other things I had not been able to put into words.

Hundreds of people liked/loved and commented on that status saying things like, “I am so excited for you. I hope you get better/stronger.” In my heart of hearts, I know that they meant well. But then there's this other part of me, the part that has spent my entire life learning how to love myself because of and not in spite of my disability that had a problem with all the well wishes. You see, I can count on one hand my friends I actually get to hang out with in person and on the other hand I can count the men I have dated. What that means is I spend 95% of my time with caregivers (basically being alone) or family members. I spent my entire youth wondering if I was less disabled if people would actually want to hang out with me. Hell, I spend a lot of my adult life in that mind-set, too.

To see all these people that I grew up with that never took the time to hang out with me, yet they were so excited for the opportunity to see me get better, it just confirmed what I had always felt, which is I would be more lovable if I were more physically whole. That pure ableism put a damper on my excitement. Even though I told people the only guarantee with the treatment is that it would stop the progression of my disease (which is more than enough if you ask me) I now had all these other people's hopes and expectations that I would get stronger. It felt suffocating and because of it I shut down.

Then there's the fact that the procedure itself is terrifying. In layman's terms the injection is administered via your spine like an epidural. In the average person it takes 5 minutes in an out patient clinic. But if you're one of the many adults with SMA who has metal rods attached to your spine holding it straight, chances are the normal method is not going to work. You'll have to do it under a live CT scan and if you're like me with 16% lung capacity you might choose to do it without sedation because sedation weakens your respiratory even further.

I had heard a lot of horror stories. Anywhere from the doctors aggravating nerves with the needle to getting a debilitating spinal headache post injection that could last hours or days. And the worst case scenario would be that my back would be too complex for them to do the procedure. So yes, I was a nervous wreck.

By the time July 2nd came I was more than ready to get my first dose out of the way. After waiting my whole life for this day, trying not to have expectations, dealing with everyone else hopes, plus accepting the fact that I actually had hopes, and dealing with the whole unknown of the actual procedure I was a mess. But the procedure came and went without any complications or side effects and it was only about 70% as painful as I was anticipating. There were drugs on standby and I didn't even need them.

7/2/18 post injection #1, my spinal fluid is now worth 125K (something like that, I do words, not numbers), holy canolie! 

When it comes to Spinraza there are 3 types of people. 1. The people who have been doing it for over a year and feel nothing because, remember it's only supposed to stop the disease from progressing, anything else is a bonus. 2. People who needed a few doses to feel any changes. And 3. People who felt changes within 24 hours or less of the first dose. Truth be told I am in group 3, it took maybe 15 hours and I have never felt more excited or hopeful.

With that overwhelming hope though, came the decision that I am not sharing every gain on social media. SMA has been a disease where I have had to deal with the losses mostly on my own and with a small group of people and I will deal with the gains of it that way too. Thus far, it has only been small gains but small things will add up into bigger things and maybe one day I will talk about it more freely. But right now this is where I am. If you're curious to see the things Spinraza can do Google/Youtube it; tons of people are sharing their journeys.

This is a moment of change in my life and while I welcome change it can be emotionally taxing. By my 26th birthday I will be receiving my 3rd dose of Spinraza. And I'm realizing a huge part of my identity is loss and learning to cope with it. In all honesty I do not know who I am if I don't have another lost ability looming over my head, if I'm not fatigued from the moment I wake up to the moment I go to bed, if I'm not working on a significantly shorter time-line than my able-bodied peers. I am turning 26 with all the possibilities I had given up years ago and it is as exhilarating as it is frightening.

What I need in this time of flux is support, comfort, and normalcy. Please do not ask about the changes I see if I have not brought it up with you or if you do not hang out with me enough to actually notice. Whatever positivity is happening it is strictly for me and my support group that have always been there. That may sound odd or rude or whatever but I am too old and lack the energy to spare everyone's feelings. I appreciate everyone's support and well wishes up until this point, I do, but I also feel overwhelmed with expectations and the ableist notion that I will get significantly better. If I don't continue getting better I need to know/feel that that is okay, that I am still enough and I want people in my life that make me feel enough as I am today. Not everything "broken" is inherently less valuable and needs to be fixed. And if for whatever reason, I one day decide to stop this treatment, I want support in that decision as well.