I always want to begin talking right as you press record
I know
But then I'm like no, let her get settled
Thank you, thank you
Hi, do we have any announcements?
Other than the fact that we filmed this video yesterday, Shane?
Oh, yesterday was a frustrating day on our Youtube world
Yeah, that's the first time that's happened
It is
Filmed a video and it has not been useable
We've already passed our two year anniversary of our..
No, we haven't
Yeah
No, the 26th is when we posted
Really, of June?
Yeah, of June
Oh, okay
That was when we posted, we started filming two years ago
It took us like 3 weeks to post our first video
Oh, yeah, alright so we're almost at our two year mark of being Youtubers
And never had to completely scrap a video that we filmed
Yep
Yesterday it happened for the first time
Well also our standards have raised, in the past we have posted the videos
So the problem with this one was wind, we filmed outside, we played it back
H: And it was just like... S: The whole video was just (static noises)
The wind was horrible
And we were so angry that we almost used it
Yeah
But this is an important video, and we figured...
We wanted to be professional
We wanted to avoid all of you not listening to the video because of the wind
So, here we are, we were gonna film again yesterday, but it was just...
Halfway through the video we had moved inside because of the wind
It was a whole big deal, and then two hours later we go to watch it and it's not useable
So we just decided to put it off until today
Also, for everyone that's gonna tell us to get a mic that has a windguard
We know, we have a mic, it hasn't worked for the last year
I think out of principle we're just not getting another one
Oh, yeah
Because we're angry that the first mic didn't work
It's kinda funny, we don't have a microphone so right now we're just filming with our camera
And I feel like most Youtubers would have a microphone on the camera
But we don't, we're literally just using our camera and the microphone built into it
So we should buy a microphone
We need to be better Youtubers
Meh
I vow from now on, I will be a better Youtuber
Okay, today's video is actually good news even though the title may not imply that
So let's jump in
(intro song) Hello, hello, welcome to the show, welcome to the show
Welcome to the show, it's time to talk about our lives....Nope!
So I think all of you know this, but just in case you're new
Shane was born with a disease called Spinal Muscular Atrophy
That's very important background information for what we're gonna talk about
We don't start every video that way
I can't walk, so actually to prove it to you I'm gonna attempt to walk right now, okay?
Ready?
(sounds of exertion)
Nope, there's no... the legs didn't even twitch
Can't walk, I've never been able to walk
Okay, we'll move on past that point, Shane, there's more
Let's give them a quick background about everything that's been going on
With my disease and my treatment so that people that don't know can follow along
Yeah, quick recap
Yeah, fast, fire away Hannah
Oh, I'm doing the recap, okay
Tell them about what my disease does to me
I am, that's where I'm beginning
S: Tell them about how it just... H: Ravages?
Ravages my body (both laugh)
So SMA is a progressive neuromuscular disease
Oh, I love to hear you say that
So it is basically, Shane gets weaker as he gets older
And that is because his motor neurons don't have a protein that they need to survive
So his motor neurons die, and once a motor neuron you can no longer move that muscle
And the muscle just withers away
Atrophies, yes
Atrophies, Spinal Muscular Atrophy, yep
Yeah, so I have lived my entire life, most of my life
With the knowledge that as I get older, I will lose more and more function
And that goes for every voluntary muscle in my body, like my hands, obviously
My arms, but it affects other functions like breathing as well
Because when you think about it, you use muscles in your chest to breathe and cough
And that's why most people with SMA have to really worry about respiratory illness
As they get older, and unfortunately many of them until recently, passed away early in life
Because of respiratory illnesses
Yep
Fun!
So, three ish years ago, around three, three and a half years ago
Three or four, yeah
The FDA approved the first ever treatment for Shane's disease, and it was called Spinraza
Yeah, we made videos about it, it was a big big big big development
Yeah, huge
In my life and the SMA world
Yeah, and so Spinraza is a every 4 month spinal injection
And Shane gets his in the side of his neck to access his spinal cord
Behind his neck spine, cervical spine
It is a gruesome procedure
Yeah, it's a long needle towards your spine
I'm not sedated or anything, but it's not fun, they hit nerves
There's all sort of possible complications
Well, remember when you got your first ever one
You know when you get a procedure done they have to tell you this is the risks, whatever
Shane's doctor came in and he was like this is how I like to describe the risks
It's like when you get on an airplane, most of the time the flight is gonna be just fine
But if something goes wrong and you go down it's gonna be really catastrophic
And we were just like.. huh?
And then he was like so let me know, do you want to go ahead with the procedure?
I was like, and I have to do this every 4 months forever?!
And that was the first ever one, and at the beginning of it you have to do loading doses
So it was every two weeks
Oh, yeah
So it was just like, do you want to get on this airplane every two weeks?
So, I mean all that kind of aside, Spinraza has been helpful for me
And for many people that live with SMA, I've been on it for about two and a half years now
You all know I have been gaining back some strength, I feel stronger than I ever have
And the coolest part of a drug like Spinraza is that it stops me from getting weaker
As I get older, so all those fears about not being as physically able to breathe
Ten years down the road, are now a thing of the past, great development
Recently, I've been having issues with my Spinraza injection
Yeah, so Shane has been getting his injections here in Minnesota
At one hospital, you've done like seven of them there, like a lot
A lot, yeah
And it's been going wonderfully, we loved that hospital
It's Gilette, the region, I don't know why I'm keeping it a secret
The region's Gilette, yeah
Anyway we recently found out four months ago, around four months ago
Around that, yeah
That Shane could no longer get his Spinraza injections there
So we had to switch to the Mayo clinic which is far from our house
But obviously a well known hospital, we were like okay this is fine
So we went there for the first establishing care visit
And that was right before the Coronavirus happened and we went there for that visit
And then the next week they called and they were like, we're cancelling your appointment
Shutting it down
Yeah
So the Mayo clinic put all, what are they, elective procedures on hold due to the virus
Which makes sense, but I began, as you know, to get later and later on my injection
And that physically was very difficult because I now rely on that synthetic DNA
To keep my strength where it is
Yeah, and then after, once you're two months late on your injection
It has like a half life of six months or whatever it's called
So once you're two months in on your injection you're supposed to get every four months
You have to start over, and get the loading doses again
One, that's horrible, and two, insurance would not approve that
I cannot imagine insurance... cause the doses if you don't know are $125,000 each
So for us to be like, well we missed it, can we start over?
They'd be like uh, no
It was just this whole big mess, many of you have been commenting
Asking, is Shane doing okay? He seems a bit weaker
And you are very right about that, I have been feeling extremely weak
It's been a very frustrating three or four months for a variety of reasons
But the Spinraza complications were a big one
And then we had an idea
We have been aware of another drug for SMA that is coming through FDA approval right now
And it was supposed to be approved in mid May and that was pushed back
Because they submitted new data, I don't know if the FDA's behind because of Covid
But they pushed it to August, and that was upsetting
Because Shane was sort of relying on that coming because Spinraza was no longer happening
Yeah, this new drug called Risdiplam is an oral option for SMA treatment
And it's a once a day liquid, and I was so excited because I knew approval was coming
Or I suspected that approval was coming in May, and I was like well, I can get to May
And then they announced that they were pushing it back, I was like oh man
I can make it to August but I'm gonna be so... I just don't have function right now
Or I didn't, so we looked into it a bit more
And we discovered what is called an expanded access program
I think in Europe and other countries they might call it compassionate access
It's basically giving patients access to a drug prior to FDA approval
When it's their only option, and when their care team can prove that this a need
So Shane talked to his neurologist and his neurologist was like, that's a great idea
The studies look wonderful on this drug, I think it would be great
So he applied, he sent in the application for Shane
Shane had to get a blood test and an EKG before, and the EKG was the longest part of this day
The guy came in and was like oh my gosh this will take 30 seconds, hang in there, one second
This is the thumbnail that you see
Yeah, and those sticky things that you see on Shane's chest
For some reason Shane's skin repels sticky objects
I have baby smooth skin, and nothing adheres to it, it's not wet
I don't know why but he could not get it to stick
And it took like fifteen minutes and this guy at the end was like oh my god
It was like playing whackamole, like he would put on 11 and then the 12th would fall off
He'd put that on, another would fall off
Oh, it was so frustrating to watch
I almost thought I wasn't gonna be able to apply for the expanded access
Because I couldn't get the stupid EKG, but finally we got a successful test
So we applied and it was a long process of waiting
Although, I say long because I felt like tired and every day felt long
But all things considered..
It was three weeks
I was approved in three weeks
And you received the drug in three weeks from applying
And going back to Spinraza, it took 9 months to get insurance approval
From when Shane first submitted for it, to when he was approved to get the drug
Yeah
So this didn't have to go through insurance which is why it was so quick
It was just the doctor being like, we have this, and the drug company was like, yep
And they sent it, so that was amazing
Yeah, so I was approved for Risdiplam and it arrived, my first order arrived last week
Thursday, so today's Sunday, it's been about a week, over a week
Hannah's been giving it to me with a little syringe in my mouth
Syringe sounds like, odd
It is, though
It's like what you use to give a kid medicine
I do the baby bird and I go (bird noises)
Can you put my head back? (both laugh)
Just squirts it into my mouth, it tastes really good
It smells really good, every time I squirt it into the syringe I want to try a little bit
And the fact that it tastes good, it's strawberry flavored
Is like icing on the cake that it's not a spinal injection
Yeah, in the three weeks leading up to the approval, though
You were like what if I throw up and gag every time I try it?
You were expecting the worst
That would still be better than the spinal injection
Yeah
And I was preparing myself, like I'm just gonna gag this down
I'll get through it every day, and then it tastes like dessert
Like a melted icy is what he says
She gives me a little bit and I'm like more please
(both laugh)
It's really good
That's great
The physical changes so far have been very gradual
I think that's to be expected with a daily smaller dosage compared to Spinraza
Yeah, giant injection into your spine
Yeah, I do not feel the overwhelming sense of tiredness anymore, yay
I was hoping you might clap for me
Yay!
I feel like I'm chewing and swallowing with more gusto
Yeah, you said that like three days in, you really felt the difference
So yeah, right now during expanded access, I'm receiving the drug outside of insurance
When that all becomes approved by the FDA, if it becomes approved
I will have to apply for it through my insurance which might be an obstacle
But honestly I think that it will be priced to be competitive with other available treatments
And we hope that my insurance will pay for it
Yeah
We'll cross that bridge down the road
So yeah, we're pretty surprised, like when you started Spinraza
We really thought it was gonna be a part of our lives forever
It was so big, and all of you know about it, we get so many comments like
How's Spinraza going? Like it's such a part of our lives
And it's so it's really strange for it to not be anymore
Yeah, it feels like a chapter of my life closing, and a new one opening
Yeah, okay
But yeah, I always assumed it would be kinda something we had to do forever
Yeah
Going for spinal injections, it complicated our plans
Yeah, it was really hard if we heard about a conference in August
And we would count four months from Shane's injections
And be like oh, your injection's in August but they don't schedule it until 2 weeks before
Do you think we can do it? Is it the beginning of August?
It was just hard to balance that, make sure we're there for it
We never really knew when exactly it would be, so this is nice that we can take medicine with us
They can probably ship it to wherever we are
I mean I don't know why they would ship it, we have like six weeks worth, but still
Yeah, so things are really good, and I know the title says I'm stopping my treatment
And I am, I'm stopping one of the treatments
And we made that title so you would watch this
I'm just saying, I'm not gonna lie about that
Oh yeah, I know
Because I want you to know that now I'm starting Risdiplam
And really excited it seems to be working well
We'll keep you updated
If I'm able to walk, I'm gonna let you know as soon as the feet start moving
Wait a minute, my foot's moving
Shane, your foot has always been able to move
(Shane laughs)
Okay, thanks everyone
Bye