I casually came to know his name.
He was next up to get dosed after my trial at the hospital.
When I bid my awesome nurses goodbye until my next maintenance dose, I cheered on Joe (not his real name) and wished him (in absentia) and the team of nurses well and good luck. If the medication worked half as well for him as it did for me, I knew what kind of life changing experiences he was lined up for.
On this most recent dose, I excitedly asked how things went for him…
It was hard to learn that his loading doses were rough going and that his kidneys were/are struggling.
It was even more difficult to hear the reason why:
he did not want to drink water, because he did not want to pee.
The thing that gets glossed over with this treatment is the sheer volume of water that you have to drink for two reasons:
1) keeping super hydrated ensures that you don’t get a wicked post lumbar (or cervical) puncture headache.
Every time this medication is administered, they literally remove a requisite amount of CSF (cerebral spinal fluid – the liquid that surrounds your brain and spinal cords) in order to make room for the medication.
If I am only slightly dehydrated when they do this, I get a headache like no other. The fringes of which I have only touched vaguely and once. It feels like my literal brain and spinal cord has a headache. Let me tell you – that DOES NOT feel any kind of good. The more you’re hydrated the less of a chance this will happen.
2) The second reason you need to drink so much water is to keep your kidneys flushed. There is the protein-to-creatinine ratio (PCR) that we keep an eye on to determine how hard of a time my kidneys are having with this medication.
I can’t tell you the exact mechanics on this because I don’t know the whys. What I can tell you is when my PCR reached 875 (!!!) [normal is 61-160] once after one of the loading doses, the administering doctor called me personally to cancel my appointment and in no uncertain terms told me to go directly to my PCP to check how my kidneys were functioning.
After running a kidney function test (and drinking gobs more water) my PCR came down and my little powerhouse filter for kidneys revealed themselves to be quite efficient, if only I would drink water to help them Be Great.
So I drank water.
Lots more water.
But drinking lots more water isn’t as simple as it seems. Because after drinking all that water, I’d need to pee it out. And that’s where things get complicated.
I need assistance to use the restroom. Even for something as simple as peeing. Knowing this, I requested extra hours from my long term care insurance for someone to come out in the middle of the day to help me.
Explaining to them the importance of this, I was cautiously hopeful that they’d at least meet me half way and give me some of the additional hours I requested.
They denied me.
Spinraza treatments started about a week or so after these denials. There was no way I was putting them off. So I proceeded, unsure on how I would resolve the peeing situation.
When I got my 875 PCR, that lit a fire under me to make something happen. But not without help. Seeing as how the home health agency was not being cooperative, I had to get Bethany involved to help advocate for me.
A lot of back and forth but finally a schedule was agreed upon with my current allotment of hours and staff quickly arranged to have a person come out in the afternoon for one hour, literally to help me pee. She’d help me get other things done, like laundry and putting my Instacart purchases away and helping me heat up and arrange my arms to eat dinner. But her main reason for coming? To help me pee so my kidneys don’t call it quits.
And before anyone asks, No. An adult diaper is not sufficient to hold the amount I am peeing. And WHO would want to sit in that all day?! That would bring problems of its own (pressure sores on my butt) and I’d still need someone to come and change me anyway, so…??♀️
My water schedule looks like this:
10:30am – Eat breakfast and drink 3/4 of a bottle of water through breakfast.
2:45pm – Drink the remaining 1/4 bottle of water PLUS an ENTIRE bottle.
4pm- Do my best to wait until my aide gets to my house at 4pm to help me pee.
On a not great day, I am outputting 200-300mL of pee. On a particularly stellar day, I’m 500mL easy. For reference, a bottle of water is 500mL.
Imagine having to hold that until the person arrives. Imagine them being late. Or not showing up.
Between 5pm and 7pm I have to try and squeeze in two more bottles of water because my next aide comes at that time. I usually only manage one because my belly will also be full of dinner and there is only so much room I have in there.
My real work though? That begins overnight.
When I have my bed fixed, there is a layer of chux lining the mattress, the fitted sheet, then I use a draw sheet that doubles as a cloth chux, an actual cloth chux and then me in an adult diaper because there is no funding for an overnight person to help me pee periodically at night.
Somewhere around 2am, I awaken, PARCHED and I down an entire bottle of water. Sometime after that in my sleep, I down another bottle. And before 8am I down as much of a third bottle as I can.
I am generally pee-soaked at this point, as the diaper has long ago given up. My best hope is that things didn’t make it down to the chux protecting my mattress.
I’ve managed to soak down to that last layer more times than not lately. Which means I have to do laundry just about everyday.
For those keeping count, that’s 6-7 bottles per day. 12-14 cups. Nearly a gallon.
Do YOU drink a gallon of water everyday?! Can you imagine how many bathroom trips you’d make if you did? Imagine having to only be able to use the bathroom 3 times. How would your life be?
So when I heard it said that Joe’s kidneys were struggling because he didn’t want to drink water because he didn’t want to pee?
What I really heard was:
I don’t want to drink water because there isn’t anyone to help me pee.
I don’t want to drink water because I don’t want to bother my family members to help me more than they do.
I don’t want to drink water because I’m embarrassed to need so much more help.
I don’t want to drink water because having to pee more would be an extra burden on those who I rely on to help me.
And I get it.
The thing that the Spinraza folx and the healthcare folx don’t realize, is as adult SMA patients, the help we need often isn’t out there. We’re not well funded for services like SMA children are. We often don’t have willing or available family members there to help us.
And so we do the best we can but it’s almost always at a loss: we don’t eat enough cause there’s no one to help when we’re hungry, we don’t pee enough for the same reason. I’m not saying all of us are like this but I suspect Joe might be.
I’m fortunate that my morning aide fixes me breakfast and positions my arms so I can eat. Once I’m done, I am now able to move my arms by myself to drive my chair and putter about. Some SMA folx can’t do that.
It was bitter sweet when the nurse who told me about Joe stated that I was the only compliant patient thus far.
It felt… wrong to hear that. When the treatments began, I was Joe. I was not wanting to drink water because I too didn’t want to pee for all of not more of the reasons I mentioned above.
But between living more years doing this treatment and dying early because my kidneys crapped out from not drinking enough water, I pushed (and got help advocating) to see what I could get done to help support my body through these injections.
It’s an awful decision to face – potentially having to stop treatment that will give me a better quality of life because I don’t have the resources/support to pee.
I suspect Joe may be facing that decision.
I wish folx knew. I wish they understood.