Wednesday, January 30, 2013

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.


Rachel said...


Seeing Ashinator's status with him at the hospital freaked me out apparently way more than it did you.

I'm sure all your medical training makes you far more levelheaded in these situations than most. But still... there comes a point when you just wish you could FIX whatever stupid thing is happening to your kid.

I am so glad they are getting him back on track with meds. Now... point us to the sperm donor slash deadbeat insurance provider...

Jamie Kennedy said...

I just came across your blog and my Heart Aches that Digger is going through this. I was googling trying to find a doctor in Colorado who will Treat HYPO KPP since the Periodic Paralysis Association does not show any on their website I myself suffer from HKPP but my first full attack wasn't until I was 30 . I have found wonderful support groups on FB and via I am not sure if you are aware PPA will be having a conferance for not only patients but for familys in Orlando where you are able to meet the doctors and researchers along with others who suffer with Periodic Paralysis. My family and I live in Colorado Springs currently my husband is Active Duty Military.

Jamie Kennedy said...

I came across your Blog while googling to find a Doctor in Colorado who treats hypo KPP. My heart Aches for what your son is going through as my family knows how hard it is in Colorado for managings this horrific disease. I myself was diagnosed this past August in 2012 finding support through outside sources through Periodic Paralysis Org., and recently on FB. I hope your son is able to maintain his medication. I know for me without them I would have major Attacks and be unable to care for my young son. If you are not a memeber of Periodic Paralysis Association I strongly recommend it, if you have the means they are having a conference in Orlando the 11-13th October where the top doctors and researches for Periodic Paralysis around the world will be not only in attendance but will available to speak to and may have ideas how to help your son afford his much needed medication. My Family and I currently reside in Colorado Springs on order with the Army. I knew I could not be the only one suffering here in CO, although there are not many doctors who will see or even treat this condition. Have a wonderful week and hope your son is doing much better especially with the weather warming up.

Jamie Kennedy

Gareth Evans said...
This comment has been removed by the author.
Gareth Evans said...

I am sooo glad I came across this. Also up front let me apologize for my succinctness. I had a better comment but it got deleted when the site asked me to sign in. I have Hypo KPP and at 6'5" 285lb I can sympathize with the monumental task of getting up the stairs. Digger has incredible fortitude, I still get scared when ever I have an attack. Also thank you sooo much Jamie Kennedy I didn't know about the Periodic Paralysis Association and believe me I am currently signing up. Does anyone else have a hard time getting the doctors to listen to you about the disorder?

Jamie Kennedy said...

It is very difficult to get doctors to listen. It seems like because HKPP affects so many of us differently most of the time they see what is black and white and nothing in the grey area. Between being persistant in finding a doctor who will listen but one who is willing to treat and speak to other doctors. I have found most of my strength in confronting doctors from joing HKPP groups. one is on linkserve the other I have found is on Facebook. I have met some awesome people. We all may have many similar symptoms but share only one or so with someone else. In knowing your not alone has made all the difference for me. I have also found a difference between Neurologists and Neurphologist. Neurphologist was my life line who not only diagnosed me but gave me hope I can one day over come to the point of having a some what normal life again free of worry and stress. I pray for that day. My Neurologist I can say he tries he had not diagnosed me even with all my symptoms matching because he believed it was a young mans disease mostly of asian dicent. Once I was diagnosed he has helped treatment. My wonder is how many are not treated because of these fallacies. Gareth Evans if you are able and anyone with Period Paralysis (any form)I hope you are able to attend the conference in Orlando to meet the Doctors and others with our Disease. I know I will be attending. Dates are 11-13 October and information is on the PPA website.