Onset
On Wednesday, August 20, 2014, I woke up after a night of interrupted and low quality sleep. I had a lot on my mind. Without getting into details, there were issues that were causing me a lot of stress. I had a meeting set for 11:30 am to discuss one of those stressful things.
I did what I do about every other day: I drank a meal replacement shake, grabbed a Gatorade G2, and headed to the gym around 9:00 am. I got on the Precor elliptical machine, set it to a resistance of 11, used it for 30 minutes, dropped the resistance to 10, and used it for another 30 minutes. After a 5 minute cool-down, I headed home. I noticed that I hadn't been thirsty enough to drink any of the G2, so I put it on the passenger seat. I felt great -- my legs were a bit tired, but I had no pain or soreness. Yet.
After I cleaned up and got changed, I headed to the appointment. I met my wife, Dana, at around 11:00 a.m. I noticed a tiny twinge in my upper legs, but I wrote that off to, well, who knows. It seemed like nothing. By 11:30, as I headed to the meeting I noticed that I was walking with a slight stiffness. As I sat through the 11:30 am meeting, I noticed my thighs hurting more and more. After a while, I started to rub my muscles. However, I remained seated the whole time.
What I didn't realize was that my leg muscles were breaking down. The hallmark characteristic of CPT2 deficiency is that once the body runs out of carbohydrates to burn, it tries to switch to fatty acids, but it is missing a key enzyme to do that efficiently. So the muscles begin to burn themselves up for energy. When you stop to think what this means in terms of cognition, there is a red flag: If the muscles are being damaged by hypoglycemia, the brain is also experiencing hypoglycemia. In me, this sometimes manifests as being indecisive during an episode. That is bad news, of course, because this is a complex condition requiring rapid, well considered and decisive action to deal with an episode.
When I stood up around 12:20 pm, all of the damage that I did not feel sitting down with my muscles relaxed came rushing forward. Breakdown that makes a relaxed muscle ache a little makes a flexing muscle hurt like crazy. I found myself walking very stiffly as I made my way to the car. At this point, the choices were (a) go home, drink a gallon of Gatorade, and hope for the best, or (b) go to the hospital. This was my worst attack in years, and I had read enough accounts on the CPT2 Google Group to know that when others have an attack this serious, they go to the ER. So I headed to the nearest emergency room, located at St. Agnes Medical Center (where my dad worked as a radiologist for decades prior to his passing). En route, I drank another meal replacement drink and a bottle of Gatorade G2. I called my brother (who also has CPT2) and my mom while on the way to let them know what was up.
Getting to the ER
I parked as close as I could, I opened the car door, and --- nothing. I couldn't lift my legs enough to get them out of the car. It is hard to explain what massive rhabdomyolysis feels like. For me, the closest analogy is to imagine that somebody has placed hundreds of very thin, two foot long metal needles through your muscles, a needle every few millimeters or so. When sitting still, the needles are slightly painful but totally manageable. When walking, though, the needles are ripping through your muscles with every contraction.
I took a deep breath, steeled myself against the pain, and used both arms to lift one leg, then the other, over the threshold of the car door. Next task: Standing up. I held the door frame, balanced on my feet, and took a horrible, painful step. I couldn't bend my legs at the knees. I remember that my mother described me as walking "like a tin soldier" when I had a bad fever as a child (presumably one of my first CPT2 episodes), and I was sure walking like a tin soldier that day. I walked the 200 feet or so between the parked car and the emergency room door, taking about 15 minutes to get there. Slowest and most painful walk ever.
Without question, I should have just called for an ambulance. I think I came within seconds of collapsing on the pavement.
I made it through the ER doors, caught the eye of the security guard, and said I was going to fall and I needed a wheelchair. He quickly got the wheelchair, and I was quickly done standing for the next eight hours. I vividly remember the strangest detail about that chair: The foot rests were at slightly different heights. As a result, one foot could rest on the footrest while the other hovered a few centimeters above it. Just the effort of balancing my foot above the footrest was excruciating. I slunk down in the chair, angled myself so both feet were resting against the footrest, and took a deep breath as both of my leg muscles went off duty for a while.
In the ER
I waited patiently for the many minutes it took to get to the triage nurse. I explained very clearly what was going on: "I have a genetic disorder called CPT2 deficiency. My body does not metabolize fatty acids correctly. I am experiencing massive rhabdomyolysis. I haven't urinated since it started, but I guarantee you that I have very dark urine filled with myoglobin. I'm also sure I have dangerously high CPK levels. I need to get IV fluids and glucose immediately. I am at risk of kidney failure."
Although the nurse did not roll his eyes, he might as well have. He put me down at the bottom of the list. People with colds, sprains, and other minor ailments arrived after me and went in before me. Did the nurse think I was faking? Just because my disease doesn't involve bleeding or dripping bodily fluids, this does not mean that it isn't dangerous. Life threatening, actually. I was pretty clear about that. I still couldn't shake the feeling that I would have been seen sooner if I gave myself a deep scratch on my arm and showed the nurse that. This was dangerously bad triage.
My wife was watching our children, so my mother came to wait with me in the ER. It was my first time ever in a wheelchair, and it was odd sitting across from her in a wheelchair. My mom knows about my condition, so she asked whether they had water available (lots of fluids are necessary to minimize the risk to the kidneys). They only had a water fountain, and one that I couldn't use from a wheel chair. This was bad, getting worse. I kept telling myself "any minute now, you'll get fluids, you'll be fine." I was wrong. It wasn't until 3-1/2 hours later that I was seen.
After 3-1/2 hours, I was taken back to what can best be described as an inner waiting room. I waited there for a while before being taken back to the emergency department. At this point, I went from feeling ignored and devalued to feeling like I was the most important patient they had. They took my vitals, got my history, and really listened. The nursing staff behind the ER doors was as good as the triage had been bad.
At the time I was seen, as a later test would reveal, my CPK level had jumped to well over the maximum 20,000 level that the test is capable of measuring (normal is 4 to 161). I'll never know what the result would have been if I had been taken back and put on IV fluids immediately, but I suspect I wouldn't have spent nearly as long in the hospital.
Since I'm mentioning my CPK results, at the risk of spoiling the ending, here are the CPK test results from this episode. The first was from a blood draw done at 4:30 pm on Wednesday and measured >20,000 (the test maxes out at 20,000). It wasn't until Saturday morning that I was even down into the measurable range.
Finally! Fluids, a Doctor and Crackers
I was finally taken to a semi-private area and interviewed by a nurse. I again explained what CPT2 is, what my symptoms were, the renal risk, and what treatment is normally given in this situation. I was still in a good deal of muscle pain, and I was offered morphine. I had never been hospitalized for a CPT2-related episode, and I figured if I had morphine, I would not be able to drive home after they finished giving me IV fluids. The seriousness of this episode was not yet fully settling in.
The nurse agreed that I would likely be getting large amounts of liquid via IV, and raised the possibility of a "Foley Catheter". A Foley is basically a tube with an inflatable tip. It is threaded up the urethra, and once the tip makes it into the bladder, the tip is inflated to secure it within the bladder. There is a small risk of infection, but according to the nurse there was a relative certainty that it would hurt going in. "For women it isn't so bad, but it is sometimes really painful going in for men." Now I'm a big fan of making things easier, but having a balloon shoved up my penis and into my bladder seemed like a lot of pain just to save a few walks to the bathroom. As it turned out, "a few walks" was more like once every 20 minutes for the first 24 hours, so if there is a next time, I think I'd say yes to the Foley, but only if I had first said yes to morphine. This scenario alone is enough to make me want to avoid this ever happening again.
I had not moved in my seat since getting into the wheelchair hours earlier, but when I was wheeled into an emergency room bay with a bed, I knew the time had come. I wasn't looking forward to this. Not in the least. I slowly stood up and marched, stiff-legged, to the bed (gurney?) where I flopped down. A very nice and efficient nurse told me I was going to be given IV fluids (not a surprise here, since this is what I told the triage nurse I needed hours and hours before). It needed to be a relatively large bore IV since they were planning to put in at least 1,000 cc of fluid per hour. For a large IV, they needed large veins, so they went for the veins mid-way up my arm.
The nurse tried to find a good vein in my right arm, but my right arm has shy veins. I am right handed, so I also did not want an IV in my right arm. I pointed out the excellent vein in my left arm (the one every blood draw I've had for decades has come from), and within a minute I had an IV. Before they started pushing fluids, they drew blood out of the IV tube (one less needle poke -- awesome!). I again asked them to make sure to run my CPK levels. They then hung saline (with added sodium bicarbonate) and I was off on my 1,000 cc per hour bed-bathroom commuter adventure.
I asked them if they were going to give me glucose as well, but the nurse declined, instead offering me crackers and juice. I know that most of the literature about CPT2 recommends IV glucose, but I was so tired, in so much pain, and so grateful to be getting something -- anything -- with carbs that I didn't argue further. By the way, when your body is in partial metabolic failure due to lack of carbs, anything with carbs is the best tasting thing ever. At the moment, those saltines tasted better than any dessert I'd ever eaten. And the boxed reconstituted orange juice? Better than any liquid I'd ever encountered.
At this point, my mom had gone home to watch my children, and my wife was at my side. My blood pressure was about 15% above normal. I was hooked up to a machine that took my blood pressure at regular intervals. The first time it read my blood pressure after my wife arrived, I stared into her eyes the whole time. My blood pressure came down, with the most normal read since the event began. Intrigued, the next time it read my blood pressure, I looked away from her and my blood pressure returned to the abnormally high level. I repeated this a few more times. Wow, my wife was able to bring down my blood pressure. Scientific proof of the power of love, perhaps.
Soon enough I was ready to pee. I had not urinated since before the whole episode began, and I was quite worried about how it would look. Of course, my worry that my kidneys had shut down was simultaneously reduced because I was producing urine. I gingerly and painfully stood up and shuffled to the bathroom, IV pole in tow. I locked the door, grabbed a sample cup, and peed. It was dark, quite brown/red (although the lab later called it "yellow" so maybe I was seeing what I expected to see), and cloudy.
I don't know if it will help others, but just in case, here are the abnormal portions of the analysis of those first blood and urine tests:
Blood:
WBC Count: 18.9 thou/mcL (normal is 4.5-11)
Neutrophil Absolute: 16.53 thou/mcL (normal 2.6-8.2)
RDW: 15.5% (normal 12%-15%)
Lymphocyte percent: 7.41% (normal 20%-43%)
Neutrophil percent: 87.44% (normal 51%-74%)
Osmolality Calculation: 273 (normal 278-305)
Creatine Kinase Total: > >20,000 IU/L (normal 4 - 161)
Urine:
Amorphous Sediment Urine: MOD (normal: None)
Bacteria Urine: OCC (normal: None)
Blood Urine: LG (normal: NEG) (note: This is probably a false positive from a dip stick test, since myoglobin and hemoglobin will both trigger a positive test for iron, which I'm guessing is what the dip stick tested for)
Protein Urine: 100 mg/dL (normal: NEG)
Clarity Urine: Cloudy (normal: Clear)
With the test results in, the doctor came to talk with me. "You know your CPK is really high?" I thought "which is exactly what I told your triage nurse before he put me at the bottom of the list," but instead of saying that, I said "yes, doctor, that's the way it goes with CPT2." "No," he said, "I mean really high, like higher than our test can measure. In excess of 20,000, probably 200 or more times normal." Surprisingly, this did not surprise me. I've read enough about this disorder to know that those kinds of numbers aren't shocking. The next thing he said was shocking though: "So we're going to have to admit you until your numbers come down to a safe area. We need to push a lot of fluids through you to keep your kidneys from failing." "I have three daughters and a wife," I said, "you're the doctor, just tell me what I need to do to get out of this alive and healthy and I'll do it."
I guess I could have had that morphine after all. I wasn't going home any time soon. I was looking forward to getting into a nice quiet room, closing my eyes, and letting the magic of IV fluids wash my blood clean of the nephrotoxic CPK.
How Did I Get To This Point?
With the blood and urine tests confirming massive rhabdomyolysis, I took some time to think about how I ended up with CPT2-induced muscle breakdown. An article by counsyl.com describes the triggers that I've experienced fairly well:
"These attacks can be brought on by exercise, exposure to cold, stress, general anesthesia, sleep deprivation, or long periods of time without eating." While I have received general anesthesia without becoming symptomatic, each of the other triggers has, at various times, caused me to go into CPT2-related rhabdomyolysis. I have never been sure whether dehydration is a trigger or just an aggravating factor, but I treat it as if it is a trigger. Better safe than sorry.
There is an additional trigger, as described in an article on nextprot.org: "These symptoms are triggered by prolonged exercise, fasting or viral infection."
I had three of the triggers, possibly as many as five, that morning:
Exercise: I did my normal, regular amount of exercise: 60 minutes on a Precor elliptical machine.
Stress: While the details aren't important to this accounting, I was experiencing substantial stress that morning.
Sleep Deprivation: I did not sleep well at all the night before the attack. A combination of plain-old-insomnia and stress contributed to a night with just a few hours of sleep.
Viral Infection: Although I did not feel sick prior to onset of symptoms, my blood work showed elevated white cell counts that may have indicated infection (or could have been related to the stress of the muscle breakdown, or a combination). This is a trigger that may have been present.
Long Periods of Time Without Eating: I did eat that morning (only 180 or so calories though), and I did have snacks with carbs the night before, so this factor was not really present in clear form. However, I had been slightly reducing my calorie intake overall in an effort to lose weight. So I'll count this as a possible, partial triggering factor.
Heading Upstairs
Waiting in the ER for a hospital room to open up is a strange thing. You cannot avoid overhearing things you'd rather not know about. Like the woman in the bay next to me struggling to understanding the doctor's discussion of how her ectopic pregnancy required emergency surgery and could also cause infertility. Or the woman who was there with her boyfriend who had been in a car accident, and who reached near-panic when she came back from the bathroom to find his bed empty (he was whisked away to get a CT Scan while she was gone). Or the woman who was about to be transferred from Fresno to Stanford, and was endlessly awaiting an ambulance to become available. By extension, I know others were wondering about me, having heard snippets about brown cloudy urine, paralyzing leg pain, and admission because of the levels of something (CK) that were hundreds of times above normal. We were all going through something substantial and important at the same time, in the same place but well isolated from each other all the same.
"Is my room ready yet?" No sooner had I asked the nurse this than I realized how odd the question sounded to me. I've asked that same thing dozens of times, but always about hotel rooms. I got the same answer I often hear at hotels -- "not yet, we'll let you know." Just like waiting for a hotel. But with more IV fluids and less luggage.
A few minutes later I was told that my room was ready. A young man showed up with a wheel chair and quickly whisked me through what I would swear had been designed as a maze. Soon enough we were at the elevator, up to the second floor, and heading to a room. A nice, empty room. Sure, there was a space for a second bed, but it was empty. This was going to be a comfortable place to recover. Or not.
Bad Roommate
I was on the "pee every 20 minutes" program, but at least with no roommate there was no impediment to my ability to do the "pee ritual" -- i.e. unplug my IV from the wall (it had a powered device that controlled flow rate), shuffle (relatively) quickly to the restroom, pee, and reverse the process. I knew rest was important, so after settling in, I quickly closed my eyes and tried to sleep. After a bit of a struggle to sleep followed by Ambien, I dozed off. About an hour after that (so we're now talking around midnight or so), I woke to all kinds of commotion in the room. Curtains being pulled, loud voices, and then the sound of a bed being wheeled in. A roommate had arrived.
I felt a little impaired in going to the bathroom. Perhaps impaired is too strong a description -- self-conscious is better. I was going every 20 minutes, I worried I was annoying my roommate, and frankly I felt a little weird about how much I was peeing. It wasn't a big deal, though. I thought things would be o.k.
Morning came with my 4 a.m. blood draw. After that I was mostly awake, although I tried to sleep. Around 7 a.m. I gave up, but out of courtesy for my roommate, I left the window shades down (I had the "window bed").
Soon breakfast arrived. I loved it. I realized that the hospital food was objectively not great, but I was so starved for nutrition and carbs that everything tasted great. Right after breakfast, my roommate's doctor arrived. Despite my efforts to mind my own business, a one millimeter thick curtain does little to block sound. I learned that he was admitted after surgery for a kidney stone. I was also a little jealous -- my doctor visited just once a day, usually around dinner time.
I also learned that he was to recover quickly. That first day he was listening to television loudly and taking phone calls. He also had a visit from a friend wearing a breathing mask. Yes, his very sick friend who was coughing and sniffling, but who at least had the sense to cover up. So now, every trip to the restroom meant trying not to trip over his friend's feet.
I resigned myself to having a tolerable, if somewhat annoying, roommate. It wasn't too horrible.... yet. That night I took a sleeping pill and slept tolerably, perhaps five or six hours. I remember waking up and wondering why he had been visited only by his sick friend, and not by family. I would learn soon enough: When 5 pm rolled around, it was apparently time for him to throw a party. Seriously. There were perhaps ten people visiting him at once, including an infant and several young children. They were eating chips and other party foods -- loudly. Good thing I didn't have a stomach issue, since the impromptu buffet smells would have surely been trouble. Oh, and they were playing music with no headphones. So I was rooming with "Party Guy".
I complained to the nurse and I was told that there was nothing they could do about it, but when visiting hours were over at 9 p.m., she would clear out the room. So now I was facing several hours of stressful noise and activity, and all of the sudden my shy bladder got concerned. I was wearing a hospital gown and little else. There were at least two female children visiting him (for hours....). Regardless of how stupid or inconsiderate it was to have little kids spending an extended visiting period in a shared room, I have daughters of my own, and there was no way I was going to saunter, half naked with my butt hanging out of my hospital gown, right past the little kids.
So I did what I really shouldn't have done -- held it in. Knowing no help was forthcoming from the nursing staff until after visiting hours, I decided to try to hold my urine for the several hours until the visiting children left. Of course, I was being pumped full of fluid (at this point, 200 cc per hour) and I was drinking prodigious amounts of water and Gatorade G2, so this was not a very well considered or successful plan. After about an hour, I faced a choice: Parade half naked past little kids (wasn't ever going to happen), pee the bed (also a bad plan), or use the "male urinal" container that the nurse had placed near my bed.
I made sure the curtain between my bed and the roommate/picnic/party area was closed (or as closed as it would get). I turned away from the curtain, lifted the blanket to cover the "action area", and peed in the urinal. As an aside, I now know that my bladder can hold around 500 cc's of liquid before I am in pain. Yes, the urinal has "amount" markings. To my surprise, when the nurse came in, she measured the amount of liquid in the urinal, emptied it, and told me that I was supposed to have been using the urinal the whole time so that they could measure the amount of urine I was producing. That's the kind of information that would have been helpful to share right away with the patient, but better late than never.
Finally, 9 p.m. came and the visitors left. Awesome! I could finally get to sleep. I was given a sleeping pill and reached that semi-sleep state where you feel so close to sleep that you don't want to risk your near-sleep state in order to complain about things. This was terrible timing, since around 9:30 new visitors arrived for Party Guy. Loud, new visitors. At least they were adults. In my half-sleep state, I kept wondering "do I wake all the way up to complain, or do I just hope they go away soon". In yet another tour-de-force of bad timing, a little after 10 pm I finally decided I needed to get these people out of my room. I sat up, called the nurse, and just before she entered the room, the visitors told Party Guy that they had to get going. Great. So now I was awake again.
Worries About Lack of Sleep and Stress
This was around the time I started to worry seriously about a relapse caused by two of the big triggers: Lack of sleep and stress. Thanks to the roommate situation, I was experiencing both. Big time. I let the doctor and nurses know about my concern that sharing a room might lead to a relapse. Although the literature on CPT2 is pretty clear that these are legitimate concerns, I wasn't sure it was being taken seriously enough. I do know that I wasn't yet moved into a private room.
Worse Roommate
The morning after my roommate's party, his doctor came in and told him he was ready to be discharged. Funny thing, I was ready for him to be discharged too. Party Guy called his sick friend, who quickly arrived again wearing a face mask. By mid-afternoon, I had no roommate. It felt like a weight had been lifted off my chest. The last thing I wanted was to risk a relapse over inadequate sleep. I pulled down the window shades and napped. I relaxed. I concentrated on feeling better. All was well with the world.
It wasn't to last, however. I was about to be introduced to The Yeller. Later that afternoon I was napping when, like a bad rerun, I again woke to all kinds of commotion in the room. Curtains being pulled, loud -- very loud -- voices, and then the sound of a bed being wheeled in. A new roommate had arrived. He was getting blood by IV, and the nurses buzzed about him for a couple of hours. In my wish-I-was-still-napping-daze, I overheard more than I wanted to know. The poor guy was perhaps 75 or 80 years old. His house was around 4,000 square feet, all converted for handicapped access when his now-deceased wife got Alzheimer's, and now occupied by him alone. He has a caregiver who helps him during the day. Unfortunately for him, he fell out of bed the night before and was unable to reach a phone. He remained on the floor, bleeding, until the caregiver arrived the next morning.
By early evening he got settled, and his family was soon brought in. This is when I learned that he was deaf in one ear. His family would yell to him, the nurse would yell to him, and he would yell back. He also seemed to have some cognitive deficits -- for example, he clearly did not understand that answering "just leave it be" to the question "what do you want us to do if your heart stops beating" meant "let me die". His daughter quickly rephrased the question for him and he quickly changed his answer. No "no not resuscitate" order after all.
By the way, how are shared hospital rooms in any way compatible with the doctor-patient privilege or, for that matter, HIPPA? If you are trying to sleep, but failing, and a conversation is happening in the room, it is essentially impossible to tune it out. As you can guess from this blog, I'm not embarrassed by or secretive about my genetic CPT2 condition, but what about people who are sensitive? You can imagine the guy who had colon surgery to take care of the "million to one shot, doc" isn't exactly anxious to discuss things in a crowded room.
Around 9:00 p.m., The Yeller was wheeled out to x-ray. I called the nurse in. "I would love to get to sleep right now. I don't want to relapse and lack of sleep is a trigger. Do you think I should take a sleeping pill now, or wait until he's back from x-ray so the commotion doesn't wake me up?" The nurse answered honestly -- it can take hours in x-ray, but it is unpredictable. In my shoes, she would take the sleeping pill now and hope for the best.
I agreed, and around 10:00 p.m. she gave me a sleeping pill. Perhaps 20 minutes later I was just falling asleep when The Yeller was returned from x-ray. This time he had lost his hearing aid somewhere in radiology. So now everybody attending to him was screaming to be heard. Then his phone started ringing. Now The Yeller was screaming into his phone in between screaming to family and nurses (and hearing them scream back). I wasn't going to sleep at all in this situation.
After 30 minutes of yelling about his missing hearing aid to the nurse and family members, and being yelled back to, he finally noticed the hearing aid on his nightstand. Great! Finally a tiny bit of quiet.... or not. He put the hearing aid in only to find it was broken.
I called the nurse in and begged her to find me a single room. I didn't want to relapse and I was experiencing serious stress and lack of sleep. I was also starting to feel twinges of muscle weakness in my arms. The nurse told me she completely understood and would take care of it. Within a few minutes, it was resolved, but not in the way I had hoped: The Yeller was wheeled off to a different room, and I was told that I would soon be given a new roommate. After Party Guy and The Yeller, I figured it couldn't get any worse. I closed my eyes and tried to sleep.
I started to shiver. My body temperature was about 2 degrees below normal. I asked for blankets. I was told that my "un-fever" was nothing to worry about, but I knew better. Cold is a trigger for CPT2 attacks, and while a slightly below normal temperature might not be a big deal for most people, for somebody recovering from a CPT2 attack, it isn't a good thing. I got as comfortable as I could and tried to get back to sleep. I had just fallen asleep when I again woke to the now-familiar commotion in the room. Curtains being pulled, loud voices, and then the sound of a bed being wheeled in.
Worst Roommate Ever
What could possibly be worse the Party Guy or The Yeller? Methadone Guy. Naked except for underpants. Sweating profusely. Arguing with the nurse over whether his most recent dose of methadone was enough. You know, your ideal roommate.
A bit later the nurse came in and noticed I was still awake. I asked for yet more blankets, and she told me that the room was 68 degrees, and she would bring it up a few degrees. Methadone Guy objected. He was, after all, sweating profusely, presumably related to a sudden lack of heroin. Now my muscle twinges spread to my legs and back and began to feel all-to-familiar as the early warning signs of a CPT2 attack. It is well worth noting that cold environments are capable of causing (or contributing to) muscle breakdown in CPT2 patients. So having to share a room with somebody who objected to raising the temperature was enough, by itself, to impair my recovery or even cause relapse.
When Methadone Guy's doctor came in the next morning, she was quite discreet, speaking in appropriately quiet tones. I was thankfully able to put the television speaker to my ear and drown out learning the cause of his sorrow. I did learn a little about what happened to him as a result of his very loud telephone conversations. In one conversation, he recounted how an acquaintance embezzled from his father. In another, there was a discussion that vaguely seemed to be about a robbery. In another, a discussion about drugs. In another, I learned that he expected to be in the hospital for another six weeks as he is on long-term intravenous antibiotics to treat an infection. I'm guessing it was from a dirty heroin needle, but who knows.
In a telling sequence, the nurse came in and asked him what his pain level was. "Ten. Ten out of ten. It is horrible. I need something. Also I need my methadone. Aren't I due yet?" Wow, sounded like he was in serious pain. The nurse said she would go grab the painkillers and be right back. No sooner had she left the room than his phone rang. Like most conversations, this one apparently led with "how are you," as his first words after "hello" were "no, man, I'm feeling pretty good. It hurts a little, but I'm really doing ok. Much better than before." Dear Nurse: You were had.
When my doctor arrived the next afternoon, I explained what had been happening with the roommates, my situation with Methadone Guy, the fact that lack of sleep and stress can trigger a new CPT2 attack, and finally that I was actually feeling the physiological signs of an impending attack. He put into my chart that I was to have a single room. I figured now that the doctor stated that it was medically necessary, it would be an easy lift. It wasn't.
My CPK that day had finally dipped into measurable territory, 16,078. I was looking forward to going home, and soon. I knew I wasn't going anywhere until my numbers dropped more, but I was hopeful.
I spoke with the unit coordinator (the "PC") and was told that they absolutely had no single rooms available. I was going to be first on the list if one freed up. I again told her that the importance of a single room was in my chart at this point -- I was risking relapse. Nothing she could do, she said.
Now things got weirder. I had a headache, and I knew I couldn't have Advil since it is processed in the kidneys. I asked for Tylenol. Here was the shock: The nurse told me that Tylenol was not on my list of medications, so she couldn't give it without the doctor's approval, and she thought that would take hours, possibly not until morning. So in the "killing a flea with a bazooka" category, she told me that I did have dilaudid on my list of approved medications (a painkiller much more powerful than morphine), and that would take care of my headache. I'd had enough of the headache, and I figured I would kill two birds with one stone. I had her give me the sleeping pill and dilaudid simultaneously. It worked. I got perhaps five hours of good sleep despite Methadone Guy's moans and my own discomfort.
After lunch the next day, I heard some commotion near Methadone Guy. He had visitors. There were a couple of guys and a girl in her late teens or early 20s. The conversation between her and Methadone Guy started off something like "Hey dad, I'm so happy I got out of jail today so I can visit you." They went on to discuss who the best parole officers in Fresno were, how she was going to behave while on parole but after that anything goes, and they then moved into a strategy session about where she could stay where there were no felons or illegal drug users, per the conditions of parole. Apparently, they did not know many such places. Finally, the discussion turned to her time in jail. She showed him photos of some of the people from jail (I didn't know you could go home from jail with photos of your fellow inmates). She told him who wanted her to be her girlfriend ("But I'm straight, so I just said no way"), who she was friendly with, and other characters. Conversation then turned to their friends and the various laws they may have violated.
So my new roommate was Methadone Guy and his sidekick Jail Daughter. This sucked. I could feel the CPT2 relapse happening. "What do you know," I thought, "the literature is right about stress and lack of sleep. Even with no exercise and lots of carbs, I'm having muscle breakdown from my roommate situation."
Mid-afternoon I again called the unit coordinator and begged to be moved. She said she was in a meeting and while they didn't have available beds, she would talk with me after the meeting got out. A few minutes later, my doctor showed up. I explained what was happening and I described what were now unambiguous signs of a CPT2 relapse. He put one hand on my hand and the other on my shoulder and told me I was getting too stressed out. He would take care of it.
A few minutes after the doctor left, the unit coordinator and a couple of helpers arrived to move to me a newly available single room. That doctor is my hero for getting me into a room where I could recover.
To be fair, Methadone Guy was nice to me. He just was so far out of my usual element that it was really stressful. His daughter seemed to be clean after her time in jail, and I deeply hope that his own six weeks of hospitalization will allow him to stop using heroin and go clean.
The Relapse
When I awoke in my private room to my 4 a.m. blood draw poke, I looked around, waited until the technician left and closed the door behind him, and was able to get back to sleep. Awesome. The results were available by mid-morning: My CPK had dropped to 3,476. I had been told I would go home when my results dropped below 3,000, so I was sure that a test later that afternoon would be my ticket to freedom. I did have some worry, however, that my arm, back and leg muscles were suspiciously sore. They seemed to be getting better, but I was worried that they were indicating a secondary CPT2 attack had taken place.
Unfortunately, there had been a secondary CPT2 attack. Eleven hours later, my CPK was back up to 4,925.
There it was. Proof that stress and lack of sleep (and perhaps cold) do in fact trigger rhabdomyolysis in at least this CPT2 patient.
My Wife and Kids
Since I was in the room for another night, and since I finally had a room of my own, I figured it would be appropriate for my younger girls to visit. My mom brought my oldest daughter a few times already, and those visits were so wonderful and so healing. Of course, it is one thing to bring a teenager into a hospital; quite another to bring a 5 and 7 year old.
The visit went well. My girls shared my hospital dinner (they liked the strawberries, everything else wasn't exactly appetizing to them). They took turns sitting in the hospital bed. My 7 year old, who wants to be an ER doctor, asked endless questions of the staff. That visit, and the visits from my 13 year old earlier, did more to heal me that anything else that happened.
Under 3,000 (Barely) and Discharged
The next morning I woke up well rested. Seriously, I actually got a full night's sleep. It was a-w-e-s-o-m-e. I was relaxed. I barely even woke up for the early morning blood draw. As the sun came up, I woke up. I napped on and off, waiting for my test results to come back. I was probably going home if I dropped below 3,000 -- and sure enough, I was at 2,705. Given the earlier relapse, though, I wasn't positive I would be going home. For that, I had to wait until around 6:30 p.m. when the doctor came to see me.
He discharged me, they whipped me through the paperwork, and before I knew it I was on my way home.
Some Big Lessons
So there is was -- my first ever hospitalization. I am 100% certain that I have had CPK levels above 20,000 before. Perhaps the biggest lesson I learned it that you cannot feel high CPK. Hey, that's important. I'll restate it in italics: You Cannot Feel High CPK. Until this incident, I had always assumed that once my urine returned to a normal color and my muscles felt OK, the incident was over. I was wrong. By the third day in the hospital, I felt so normal that if I didn't know my test results were abnormal, I would have headed to the gym to work out. You literally cannot tell that your CPK is still high. Unless, of course, your kidneys fail.
The urine returns to a normal color when the myoglobin is cleared out. That happens relatively quickly. The CPK clears much more slowly. Going forward, I am going to assume that my CPK levels remain elevated for a full week after all symptoms cease.
I am also going to get my CPK levels tested any time I suspect I have had a CPT2 incident. A few extra blood tests is no big deal; a lifetime on dialysis, that is a big deal.
Some Resources
I did a lot of reading about CPT2 while in the hospital. Here are some of the sites I found:
http://ghr.nlm.nih.gov/gene/CPT2
http://www.ncbi.nlm.nih.gov/books/NBK22581/#A3054
https://www.fodsupport.org/cpt2.htm
http://www.ncbi.nlm.nih.gov/books/NBK1253/
https://www.counsyl.com/services/family-prep-screen/diseases/carnitine-palmitoyltransferase-ii-deficiency/
http://www.nextprot.org/db/entry/NX_P23786/medical
http://www.newbornscreening.info/Parents/fattyaciddisorders/CPT2.html
http://health.mo.gov/living/families/genetics/newbornscreening/pdf/CPT-11.pdf
http://www.hindawi.com/journals/crig/2014/496410/
You absolutely cannot feel a high CPK. My son's CPK was 14,000 when he had no pain at all. At its worst it was 115,000.
ReplyDeletePlease keep in mind that CPK is an indicator of muscle tissue breakdown, not kidney function. It can alert providers that the kidneys may be in trouble but that is not the purpose of the test. BUN and Creatinine levels as well as GFR (glomerular filtration rate) are more indicators of kidney function. The doctors also watch liver function tests ALT and AST. During Michael's first crisis, his ALT and AST were over 3700 each and they are supposed to be under 40.
Please check out www.cpt2kids.blogspot.com if you want to see some of our journey!
So happy to find your blog with all this great CPT 2 info! My 18 year old daughter was just diagnosed one year ago after a 4 day stay in the ICU for Rhabdo she got during her lacrosse team tryout week. She again made a trip to ER last week during the same tryout week only this time lucky that she got released the same day after 2 liters of IV hydration. We are now trying to learn more about living with and managing this disease so THANK YOU for all this great info!
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