Hyperkalemic Periodic Paralysis Sucks.

I know, I know, a couple of years ago I said Epiploic Appendagitis Sucks, and it does, but HypoK PP also sucks.

It might actually suck more because it's an inherited disease (autosomal dominant, if you care) and it doesn't go away like EA does.

Digger's been dealing with it most of his life.  We suspected he had inherited it when he was a toddler and complained of his legs being "sleepy".  It didn't hit full force until puberty hit.  And then it hit like a damn steam-roller.

I know a lot of you are familiar with the equine version of HypoKPP.  The human version is a bit different.  When the body's potassium drops, paralysis sets in.  Most of Digger's teenaged years were spent with him not knowing if he was going to wake up a paraplegic or quadriplegic.

So that sucked.

As he got older, it leveled out a bit and was mostly manageable as long as he stayed on his meds.  However, his dad dropped him from this insurance, which is a whole other story.  What it meant, though, was that I was the one paying for Digger's meds.  Until his doc finally put his foot down and wouldn't fill any more until Digger was seen in the office.

I totally get that.  DEA regs, and all, docs can't just write scripts forever without ever seeing their patients.

No insurance and no job means no doctor's visit and no refills on the meds.

Digger's paternal family has been self-treating with No-Salt, the salt substitute.  It managed to keep him fairly stable for about six months, but last week he hit his tipping point.

We've been dealing with his attacks for so long that they are really no big deal.  In fact, they're kind of a pain in the ass at this point.  We're well beyond the "oh my God, Digger can't move, what can we do to make him more comfortable?" stage and into the "aw, hell, you need us to move you again?" stage.  It's just the ugly truth.  Any family who has dealt with a chronic disease would be lying if they denied it ever gets to that stage.

So when Digger started texting me to tell me he had an attack and needed his brother or sister to go move him, I called them and told them to go help their brother.  They were only about ten feet away from where he was on the couch in their respective bedrooms, whereas I live in a whole different town now.  Monster grumbled, but went and helped Digger get situated again.

A couple of hours later, while I was driving in to work, Monster called and told me that Digger was having trouble breathing.  Crud.  That had happened one other time and I just carried him out to the car and drove him into the ER myself, so I sighed heavily and told Monster I would come on over and take Digger to the ER like I did last time.

I hung up, called work and let my boss know that I would be in much later because I knew it would take some time in the ER to get his potassium level back up.

And then I remembered that Monster and Ashinator live in a basement apartment and that there would be no way I'd be able to get Digger up the stairs and out to the car without hurting one or the other, and quite possibly both, of us.  I called Monster back and told him to call an ambulance.  I'm not one to take calling an ambulance lightly, but sometimes it just makes sense to call in the people who have the tools to take care of the job.  Even knowing that, I felt kind of guilty.

I arrived at the kids' apartment just as the ambulance pulled up, so I gave the paramedic a brief history as we descended into their place.  The fighterfighters (Digger's word since he was a toddler) were already in the apartment taking care of him.  Their eyes were huge and they look kind of lost.

I can't blame them.  HypoKPP is a form of Muscular Dystrophy that only affects 1 in 100,000 people.  As far as I know, my ex-husband's family is the only one in Northern Colorado that has it.  Everyone in the family is pretty well-versed in the disease out of necessity.

Last time Digger was having trouble breathing it was really no big deal.  It pretty much resolved by the time we got to the ER.  This time?  Holy shit, he looked like death warmed over.  His oxygen sats were okay, but he looked like he was headed for some time on a ventilator.  Apparently the paramedic thought so, tooo.  She wasted no time having the fighterfighters load him into the magic wheely chair with the tracks to go up the stairs to the cot.

The trip up the stairs was tough for everyone, despite having the magic wheely chair.  I bet there are a couple of fighterfighters who still have bruises from trying to wrestle the chair up those steep, narrow stairs.  Personally, I think it would have been easier to just throw Digger over a shoulder and fighterfighter carry him up the stairs.

They wasted no time transferring him to the cot and into the ambulance.  Monster and I hopped in my car and couldn't even keep up.  They ran him hot all the way to the ER.  I guess he really upped their pucker-factor.

His STAT potassium was 2.2 mEq/L.  Normal is 3.5 - 5.5 mEq/L.  Patient's whose potassium drops to 1.9 only have a 15% chance of surviving.  Maybe the paramedic was right to run him hot.

I have a lot of respect for the paramedic who responded.  She was one that I did my ride-alongs with when I was an EMT student twenty-three years ago.  (No, she didn't recognize me, it was twenty-three years ago.  I can't even imagine how many snot-nosed EMT students she's seen in that time.)  I had mad respect for her then and have even more for her now.  She listened to what Digger had to say and made sure the ER staff paid attention.  She refused to go clear and back to base until she started seeing some improvement in his condition and she was sure that he was on the rebound.

The miraculous thing about HypoKPP is that once you start the potassium, the paralysis begins to resolve.  It's like giving a diabetic sugar - when their blood sugar is low and you give them some oral glucose, the change is almost immediate.  With HypoKPP, it's not quite as immediate, but it's still pretty amazing.  Digger was taken into the ER with difficulty breathing as a full quad, and within just a couple of hours of IV and oral potassium, had full movement.

He was admitted overnight to make sure his potassium levels stayed stable.  Digger had zero complaints about that.  He became the darling of his floor and his CNA was super hot.

Gotta give him props for that.  The kid gets hospitalized and isn't upset - he's thrilled that he has a hot CNA.  In fact, for the most part, his attitude about his disease is pretty good.  It gets him down sometimes, but his sense of humor about it keeps us all going.

The great thing with being hospitalized was that he got back on his medication regime and is feeling better than he has in months.

But HypoKPP still sucks.