Hypokalemia and The Pitt

Two Thursdays ago, The Pitt, season 2, episode 3 aired. I didn't get a chance to watch it until Friday night.

ICU life with HypoKPP

Now, let me say, I love The Pitt. I love the format in which each episode is one hour of a fifteen hour shift. I loved E.R. back in the day as well. Both shows are insanely medically accurate, which is a pleasure to watch. And, funnily enough, both shows have had a patient with hypokalemic periodic paralysis. I'm always thrilled when Digger's disease is named, because it brings attention to his (mostly) invisible disease. When I first saw the episode, I texted Digger that he was going to love it when had a chance to watch it. (For a refresher on Digger's disease, click here and here.)

He did not love it, and with good reason. He said, "look, my disease is already a zebra, they didn't need to paint more stripes."

As his mom, I was just happy to have his disease mentioned, as it's rare (1 in 100,000). I've spent my time educating medical professionals about the disease, and I love to see it mentioned in an arena in which I know other medical professionals are watching.

When I asked him to explain why he hated it so much, he had some well-developed thoughts.

• In the episode, as they're going over the results, they said the patient had a potassium level of 1.2. Digger's lowest ever was 1.6 and they were planning intubation. The lab techs actually visited him in his ICU room, because they couldn't believe that he was awake and talking with a K+ level that low. His body is well accustomed to low potassium level; someone who has never had an attack would be dead with a K+ level of 1.2. Digger did a little bit of digging, and there is a woman with the disease who has survived a K+ level of 0.9.
• The two times his disease has been mentioned (ER and The Pitt), it's always a "drop attack", where the attack came out of nowhere. Those are so exceedingly rare that they're practically non-existent. In our experience (and that of Digger's entire paternal family), attacks are brought on by stress, sleep, hormone disturbances, food choices, etc. Hormones play a huge part in frequency of attacks, so most attacks begin in adolescence, then subside throughout the mid-twenties and thirties, only to come back again with a vengeance in the mid-forties.
• It's been a few years since I checked for any new/updated information on the disease and I was interested to see that there is a thyrotoxic form that effects primarily Asian males. I believe that's what the writers were aiming for, but they blew it. Let me explain: in the episode, as they were talking about the patient, they mentioned it was hereditary. Digger's biggest beef with this, is that unless the patient was adopted, someone in his family had to have had the disease and they would have been aware of it. The doctors go on to mention the thyrotoxic component of the disease. What it feels like to me is that the writers skimmed the overview of the disease, saw 'hereditary' and 'thyrotoxic' and mushed them together. Thyrotoxic hypoKPP and hereditary hypoKPP are two different things. Is the outcome of paralysis the same? Yes, but the etiology is quite different.

After speaking with Digger, I completely understand his frustration. He was very clear, and correct, when he said they treated his disease, which has greatly impacted his life, as a prop, and failed to bring factual representation of it.

I get it, and he's right. Just because the disease was mentioned, does not mean it was represented appropriately, and did not bring awareness to it.

I watched last week's episode, hoping to see a resolution for the patient, but that hour of the shift did not deal with it.

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.

Hypokalemic Periodic Paralysis

I had a couple of questions the other day about Digger's disease and I realized that I had never addressed it head-on here, just mentioned it in passing in a couple of posts.

All of my kids have an inherited disease called Hypokalemic Periodic Paralysis (HypoKPP) which they inherited from their father, who inherited it from his father, who inherited it from his father...as far back as anyone can research.

It affects 1 in 100,000 people statistically (unless you happen to inherit the lucky gene) and causes signs and symptoms ranging from periodic extremity weakness to full-on paralysis, sometimes involving the respiratory muscles.  It's what's known as an ion channelopathy, specifically affecting the sodium/potassium and calcium channels in the cells.  Potassium must be pumped out of the cell and sodium pumped in.  It's all a very complicated way to maintain homeostasis (or balance) within the body.  Calcium is the necessary ion for muscle contraction, without calcium to activate the troponin and tropomycin, which then allows the actin and myosin heads to be released, no contraction can occur.  When those channels don't work, it throws a monkey wrench into, well, pretty much everything.

In a family with HypoKPP, each offspring has the following chances or not of inheriting the disease:

• 25% will inherit the trait and will exhibit signs and symptoms
• 50% will inherit the train, but will not exhibit signs and symptoms, but may pass on the trait to their offspring
• 25% will be home free; they won't inherit the trait, won't show any signs and symptoms, and won't pass it on to their kids

I knew that my husband had the trait (and exhibited all signs and symptoms) and expected that if I had kids, they would probably have the disease.  The degree to which patients are affected varies.  For example, Digger has mornings when he wakes up a quad, but Ashinator's disease manifests itself via muscle weakness and drop attacks.  Ash tends to recover faster from attacks than Digger, who can take days to fully recover.  Monster complained of muscle weakness when he was younger, but no longer seems to exhibit any signs or symptoms.

It's much, much worse in puberty, but can be brought under control in adulthood.  Digger currently controls his with an assortment of prescription medications designed to help control his potassium balance.  Ashinator and Monster have such mild attacks, that they don't take any daily medications and will take prescription potassium only during an attack.

I worry about him living so far away with his roommates because I've seen the worst of his attacks and they're not pretty.  However, he's managing them pretty well for now.

We've developed a pretty good sense of humor about the whole thing - in fact, Digger's got about a 10 minute routine in which he re-counts the story of the day Ashinator tried to move him from his bedroom to the couch using a skateboard.  If I can get him to let me film him telling it, I'll be sure to put it up.

Monkey's Monday Minute

Ian at The Daily Dose of Reality asked several of us bloggers to join in on this extra special edition of the Monday Minute.  Michelle, one of our blogging buddies over at My Pixie Dreams, just received news that no parent should have to hear - her 16 month old baby, Monkey, has been diagnosed with a rare form of cancer.
 
1.  How old do you act?
I've been thinking about this one for a couple of days.  I think I act old, or at least older than I am.  I don't mean to act old; it's just that I sometimes feel like I've got the weight of the world on my shoulders, even though I don't.  I think was born old.


2.  As far back as you can remember, what did you want to be when you grew up?
That's easy.  Firefighter, just like my daddy.  Guess what I'm not?


3.  If you were to write a book based on your life, what would the title be?
WHICH END IS UP?  I feel like I've spent my life trying to follow the bubbles to the surface.


4.  What's something you do that's considered "childish" by most?
Remember question #1, where I've always acted old?  I don't know that there's a lot that I do that's considered childish, but I do sometimes get all "cutesy" with RCC.  It makes me sick and I can't believe that it's me doing it.


5.  Write a story of a time when you or someone you know overcame great adversity. (Warning, very long story.)


Once upon a time a child called Digger was born.  Digger's daddy, granddaddy, aunt, great aunt and cousin all have a horrible inherited channelopathy called Hypokalemic Periodic Paralysis (HypoKPP).  HypoKPP causes paralysis, either partial or complete, due to a lack of intracellular potassium.  Sometimes the attacks last a few hours, sometimes a few days.  And sometimes, it requires hospitalization and a ventilator if the respiratory muscles are affected.  In addition to not being able to move, the attacks and recovery from the attacks are excruciatingly painful.

Digger's mommy was on OrthoNovum birth control pills.  The pills failed and Digger's mommy ended up pregnant.  His mommy and daddy knew that Digger had a 25% chance of exhibiting the trait, 50% chance of carrying the trait, and a 25% chance of not inheriting the disease at all.


When Digger was a toddler, he used to complain about his legs being tired.  His mommy and daddy worried that Digger was exhibiting symptoms of HypoKPP, but then, all toddlers complain of their legs being tired.  Right?


Digger outgrew toddlerhood and ran headfirst into childhood.  He never stopped moving and his complaints about tired legs went away.  His mommy and daddy breathed a sigh of relief.  By this time, Digger had a sister, Ashee-butt, and baby brother, Monster.  His parents were worried about all of them having the dreaded HypoKPP and carefully watched his siblings for signs of the disease.  


Neither Ashee-butt nor Monster exhibited any signs of the dreaded disease in toddlerhood and with Digger's symptomatic complaints gone, their parents put the disease out of their mind.


The kids' childhood, despite their parents' divorce, was fairly uneventful.  They laughed, they grew, they played all manner of sports without a problem.


Digger loved wrestling and soccer and was exceptionally good at both.  When puberty hit, that all changed.  One morning while his Uncle Deejo was visiting, Digger woke up with his first ever attack.  It was a doozy.  The poor kid woke up a quadriplegic.  His mommy's worst fears were realized.  Yes, Digger had inherited his father's horrible, horrible disease.  His mommy and Uncle Deejo loaded him into the car and took him to the doctor, who knew that there was a chance he'd have the disease.  Digger and his family spent hours in the doctor's office as they tried to raise his potassium.  Slowly, very slowly, he regained control of his body.


That first attack was the beginning of a very long, very hard road for Digger.  The onset of attacks is always sleep, so the poor kid never knew if he'd wake up a paraplegic or quadriplegic.  Nor did he know how long he'd be a para or quad.  Sometimes it would be a couple of hours, sometimes it would be a couple of days.  Thankfully, he was never hospitalized for an attack, though there was a close call one night when he complained of shortness of breath.

The number and severity of his attacks took a toll on everyone in the house.  Sometimes his mommy was very mean because  she was exhausted from working all day and then being woken up in the middle of the night to give Digger his medicine and to re-arrange him into more comfortable positions.  Sometimes his mommy blamed him for not taking care of himself and taking his medications correctly, even though she knew it wasn't really his fault.  She was just tired and angry and hurt to see her child helpless and in so much pain.  On those nights when she was mean to him, she cried herself to sleep, but never let him know.  She was always very careful to apologize to Digger, because it wasn't his fault that he had inherited his father's disease.  

His siblings were also affected; they helped out a lot when Mommy wasn't home.  When she wasn't home, it was up to them to help Digger take his medicine and move him when he needed it.  It was hard on everybody, but hardest on Digger.


He was able, with help from a Home Bound Tutor, to finish junior high, but high school proved to be too much for him.  Digger and his mommy had tapped out all of the resources available to them by the end of his sophomore year.  He tried his hardest to go to school the traditional way, but it just wasn't going to happen without accommodation.  He dropped out of school in the middle of his junior year and took his GED.

As Digger emerges from puberty, he's getting better control over his disease and sometimes goes weeks between attacks.  When he does have an attack, they are now less severe and don't last nearly as long.  

Four weeks ago, Digger took a big step.  He enrolled in college and has completed his first class.  The ability to attend class at night will help him to succeed.  Even if he has an attack in the morning, he's usually recovered by late afternoon and will be able to continue to attend night classes.


What about his sister, Ashee-butt, and his brother, Monster?  They both exhibit mild symptoms of the disease, very mild symptoms.  So mild, that they rarely, if ever, interfere with daily living.


There's no cure for HypoKPP, but there is the ability to live a normal life (once out of puberty).  And sometimes, that's the closest someone can get to a Happily Ever After.