Another Rhabdo Attack

[Kira]

Well, I am writing on the computer in the waiting room at the ER (fancy, huh?). I think I have another rhabdo; the symptoms are pretty typical for me and I have been puking for two days so it is a usual trigger. The only question is really how high my CPK is and if it will require an admission or just an overnight stay in the ER to get my D10. I hate the hospital. This will be rhabdo #9 and/or admission #18, all in the last two years.

[BrokenBladder]

Kira I'm so sorry!! Please keep us posted on what's going on!! My thoughts and prayers are with you.

[lobelsteve]

Unsure of your current doses.
If not here, you may need to increase to:

800mg per day CoQ10
3 tabs 3x daily of the 330mg L-carnitine

Get CoQ10 and carnitine levels now- we need to see the levels at the time of an attack.

If you have to spend a day in the hospital, so be it. Get you kidneys happy, stay out of ATN. When you are discharged:

Call Bruce Cohen at Cleveland Clinic Dept of Neurology, he is the best in your region. http://www.clevelandclinic.org/staff/getstaff.asp?StaffId=1129

If you can make it to Atlanta, John Shoffner is the best in the world.
http://www.choa.org/default.aspx?id=1566

WIth cardiac involvement- concerned about a cardiomyopathy due to a mitochondrial disease. Those darn little powerhouses are having a brown out....again. Did the muscle biopsy results come back yet?

PM me for more info if needed.

Steve

[waggytalk]

that sucks.

at least they let you use the computer and interenet. last time i was in they said i couldnt take it in with me. bah.


but really hope you start feeling better.

[BrokenBladder]

Kira sounds like LobelSteve has some help for you, thank goodness. I just wanted you to know I'm thinking about you and waiting to see another post from you to let us know how you're doing. Take Care.

[lobelsteve]

I've mobilized my mitochondrial specialist.... the info is rolling in.

ABSTRACT: Mitochondrial disorders share common cellular consequences:
(1) decreased ATP production; (2) increased reliance on alternative
anaerobic energy sources; and (3) increased production of reactive oxygen
species. The purpose of the present study was to determine the effect of a
combination therapy (creatine monohydrate, coenzyme Q10, and lipoic acid
to target the above-mentioned cellular consequences) on several outcome
variables using a randomized, double-blind, placebo-controlled, crossover
study design in patients with mitochondrial cytopathies. Three patients had
mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes
(MELAS), four had mitochondrial DNA deletions (three patients with chronic
progressive external ophthalmoplegia and one with Kearns–Sayre syndrome),
and nine had a variety of other mitochondrial diseases not falling
into the two former groups. The combination therapy resulted in lower
resting plasma lactate and urinary 8-isoprostanes, as well as attenuation of
the decline in peak ankle dorsiflexion strength in all patient groups, whereas
higher fat-free mass was observed only in the MELAS group. Together,
these results suggest that combination therapies targeting multiple final
common pathways of mitochondrial dysfunction favorably influence surrogate
markers of cellular energy dysfunction. Future studies with larger
sample sizes in relatively homogeneous groups will be required to determine
whether such combination therapies influence function and quality of life.
Muscle Nerve 35: 235–242, 2007

PM for the article- I can get you the PMID if you have institutional access for your school.

I also was sent a listserv excerpt from the metabolic - inborn errors of metabolism discussing the various mitochondrial cocktails being used by the folks who do ALL the research (worldwide).

All docs love the NINDS patients, as long as they are not their patients. Out of my league. It's important to know what you know, what you don't know, and what you can figure out before doing undue harm.

[Mark N]

Sorry to hear you are having another attack. Get better and let us know how you are doing.

[Tbackpain1]

Kira,

You do what you need to do to make sure you stay well. Dr. Lobel has given very good info for you, make sure you pick his brain :) In the meantime, get the D10, and feel better soon.

<hugs>

Theresa

[Bobbi]

Please let us know when you can how you are feeling, Kira.

[ErinENj]

Kira, I'm so sorry to hear that you're having a rough go of it! And that's really neat that you're on your computer in the hospital! My local hospital just did this major upgrade, built 2 new wings (including the most amazing surgical suite!! When I have my next surgery, I want to be there! They're hotel rooms. Almost all are private, they're HUGE, they've got nice wood cabinetry, a sofa, a comfy chair, a flat screen tv, dvd player and VCR, sink besides the one in the bathroom, refridgerator... yeah. it's really nice! My aunt was in there and I was so amazingly jealous!!) but they don't have any online access. It makes no sense to me whatsoever that they would put all that work into it, put in this great ER (which is huge, but the last time I was there for my appendix scare, I ended up in the hallway because there wasn't enough room. But noone else was in the hallway...hmmm) and everything, but no internet. I think they have it in the new cancer center, but nowhere else.


Now, pardon my ignorance, but what is rhabdo? I've never heard of that before and I tried looking it up on webmd with no success. Sometimes, that place is no reference at all!

I hope that you're feeling better asap and home, and comfy! Keep us posted!!

[Kira]

Hey guys... thanks so much...

I didn't have to get admitted, just spent the night in the observation part of the ER, got a few liters of D10... labs started looking better and I was able to keep down a can of soda and some crackers, so they let me go. My CPK was not super-high, and once we saw that it was trending down, they let me go... though I am supposed to go back if I can't keep enough food/fluids down. Kidneys are okay, too. Knock on wood, but my kidneys have been okay so far in general, other than the Renal Tubular Acidosis (which is from my underlying disorder, not the rhabdo).

We think that the puking is from something metabolic, because the D10 fixes it. So I guess that says two things:

1) The port is really important so I can do the D10 at home early on.

2) When I first start getting nauseated/pukey, that is the time that I need to MAKE myself keep down sugar/fluids, even if it is hard, because that is the only thing that is going to stop it.

I am going to get my metabolic guy to set up the port thing since my primary deferred to him. If he isn't comfortable with a port yet, I am going to ask about a trial with a PICC line and see if that cuts down on these episodes. I have NO venous access left and so it really wouldn't be possible for me to just start a normal IV on myself at home.

Dr Steve, thanks for all of the info. My CoQ10 and carnitine doses still need tweaking... I just finally started on them last month, after the biopsy, because we wanted to make sure that the biopsy reflected my actual CoQ10 and carnitine levels, not the supplements.

Dr Cohen doesn't take any new adult mito patients anymore. He has written some really good info on mito, though, that is on the United Mitochondrial Disorders Foundation website (www.umdf.org (http://www.umdf.org)).

I do see Dr Shoffner... he did my biopsy at the end of December and I am seeing him again in spring to discuss results. I am coming at the end of April for a CDC conference (for work) and am trying to make the appointment for the same trip so I don't have to pay for airfare & hotel twice.

I have a good metabolic doc here in town, too, but he only has a few mito patients, so it is nice to have one of the gurus on board too.

Shoffner thinks that it is fatty acid oxidation dysfunction... he is not sure if it is a primary fatty acid oxidation defect or if it is fatty acid oxidation dysfunction secondary to mito. However, my pattern of inheritance looks most like mito, and other organ systems being affected also makes it look more like mito.

They sent my muscle, blood, and urine for a LOT of stuff in Atlanta... enzymatic, genetics, ox phos (mito), CoQ10 and carnitine in the muscle and blood... I have had the carnitine labs drawn with attacks in the past, as well as organic acid profiles, basic metabolic screens, etc. The carnitine profile has actually been okay.

This has gotten way too long... but I wanted to say thanks to you guys for being so supportive.

(And yes, I think it's cool that they have computers in the waiting room at the ER. They just built a new ER in June and it is really nice, lots of little touches that make it more comfortable. There is a section off to the side with four computers and two phones, and you can use them while you wait. I wasn't using my computer, I was using those... but it is a good way to kill time.)

[Bluey]

It was interesting reading about your condition as I've never heard of it before.
Sorry you have to have it though!
Hope you are feeling better and can enjoy the Spring that is on it's way.
I always look forward to the first early tulips and primroses.
All my best wishes to you, Bluey

[Kira]

Okay... so looked back and wanted to answer specific questions that people asked:

Erin,

Rhabdo is short for rhabdomyolysis (might find better info if you google that). Basically, it is when a bunch of muscle tissue dies. There are a lot of things that can cause it, but when I get it, it is due to an energy crisis in my cells. I have a broken enzyme somewhere in the process of fatty acid metabolism, we think (waiting on muscle biopsy results to confirm). Normally, when people are not eating, they burn fat from their fat cells as an energy source. My body can't do that very well, so when I need extra energy, I don't have it. It is a supply & demand issue... either I can't supply enough energy (from carbohydrates/sugar) from not eating or not eating the right things, or I am demanding more energy than my cells can produce (from exercise/exertion, fever, infection, hot weather, etc). When that happens and my body can't keep up with its energy needs, the cells start dying. For me, it affects mostly my muscles, but for other people it affects other parts of the body.

When my muscle cells die, a lot of things happen. For one, it hurts. For another, the muscles don't work well, so they get very weak. Also, some stuff gets released from the dying/dead cells: myoglobin, CPK, potassium, acids, etc. Myoglobin is a protein that is toxic to the kidneys, so as the kidneys try to filter it out, it can cause kidney damage. Plus, your pee turns dark brown or black (like cola). You have to get very large volumes of IV fluid to flush it out and try to minimize the kidney damage. CPK is what they measure in the blood as a marker of how much muscle is dead. Potassium is important because if your potassium goes too high it can cause heart arrhythmias and kill you. The acids that get released are important because you can get metabolic acidosis, which also affects how lots of organs function. And also, the dead tissue sucks up calcium, so your calcium level drops the second day. Low calcium can do anything from no symptoms to widespread muscle spasms (tetany) to nerve problems to heart arrhythmias. They can treat it in the short term by giving you more calcium, but it causes long term problems because you get calcifications in your muscles from repeated attacks.

Besides the rare metabolic disorders, other stuff that can cause rhabdomyolysis: statins (lipitor, crestor, etc), extreme exercise (marathons, etc), crush injuries (like being trapped under a building, car accidents, etc), some rare viral syndromes, all of the myopathies (autoimmune ones like dermatomyositis/polymyositis, endocrine ones like those caused by thyroid problems, metabolic ones), complications of anesthesia (malignant hyperthermia), complications of certain antipsychotic meds (neuroleptic malignant syndrome), complications of overdosing on serotonin-related meds (serotonin syndrome), prolonged tonic-clonic seizures, and other stuff. Rhabdomyolysis is not common and really not something any of you guys should worry about unless you get SEVERE muscle pain, muscles that suddenly don't work and are swollen, and cola-colored or black pee. I get it because I have a rare metabolic disorder.

------------------------------------------

Steve,

No cardiomyopathy, according to my cardiologist. I have had a couple of echos and a cardiac MRI. What I have is:
-persistant tachycardia (100-120)
-nonspecific EKG abnormalities ("scooped" ST segments, like you get from being on digitalis... but I'm not on dig)
-mild mitral, tricuspid, and pulmonic regurgitation
-hx of a pericardial effusion for no good reason

But I do have a cardiologist and they follow it with periodic echos and EKGs. So all is good there so far.

For curiousity's sake, here is the other stuff I have:
-Metabolic acidosis: from Renal Tubular Acidosis Type II... mito can cause proximal renal tubule dysfunction, and they think it is mostly because of that. It got a lot worse on Topamax, because it also causes the same pattern of acidosis due to being a weak carbonic anhydrase inhibitor. Going off of topamax helped, and I see a nephrologist for it and take bicarb. Also I get mild lactic acidosis with attacks, so that is additive.

-Neuro stuff: This actually came first, before the rhabdo, even. They did a brief workup for MS and wrote it off as nothing when all of that came back normal. I had paresthesias and weakness at the time. Now I have hyperreflexia, positive Hoffman's and Babinski's, mild/moderate spasticity in legs and forearms. Don't know why... blame it on my underlying disorder. I've had a brain MRI (though it's been a while), LP (part of the MS workup), and four EMGs. I do have a neuromuscular doc. Right now we are just watching it.

-Anemia: with microcytosis, low RDW, and really low ferritin (6; normal 20-150 for females). I take iron for it. We don't know the source of bleeding and they have blamed it on diet and menstruation. The ferritin hasn't gone up even with TID iron so that is kind of weird. But whatever.

-Mood stuff: treatment-resistant depression with some atypical anxiety/irritability stuff that they've tried to call all sorts of things, from atypical bipolar II to atypical anxiety disorder (dx now is just depression). But we found out that it really does follow a pattern... the funky anxiety/irritability stuff happens in the days following an attack and then gets better on its own. Two shrinks, two neuros, and my metabolic doc all think it has to do with the metabolic byproducts/toxins that build up after an attack, as well as possibly the acidosis. So now we try to treat it by taking good care of the metabolic stuff, lots of fluids, treating the acidosis, and just plain reminding myself why I feel that way and that it is going to end in a few days. I have really been through the wringer with this psych stuff, because my first psych doc was a resident and she just kept trying to force it into the DSM-IV and give more and more drugs for it, which made things worse. The thing that worked the best with the meds was to DECREASE the Effexor and to get rid of all of the other meds. My current shrink is excited by the prospect of getting a dx so we can more tightly tweak the dietary stuff (I have a metabolic nutritionist) so that I build up less of the byproducts.

-Allergy: not a mito thing. I have bad environmental allergies as well as chronic idiopathic urticaria.

-GI: this nausea stuff that has been happening a lot lately. Still not sure why it is happening (metabolic? meds? something else?), but it sucks. I have to get a gastric emptying study in the near future; maybe it will show something.

Probably way more than you wanted to know, but whatever. If you want, for curiosity's sake, I can PM you the H&P from Shoffner. He goes into a LOT of detail and even cites references to back up what he recommends in the "discussion" part.


Oh, the doses of the supplements (still too low, CoQ10 is expensive and I still haven't gotten in to my metabolic guy since the biopsy to get started on the prescription Carnitor, so this is just the OTC stuff and doses are low... held off on supplements from Aug to Jan for the biopsy, so tissue levels were my own instead of from supplements).

Carnitine 500mg po BID
CoQ10 200mg po TID
Creatine monohydrate 725 mg po TID

Antioxidants:
Vitamin C 500mg po TID
Vitamin E 400 IU po BID
Alpha Lipoic Acid 300mg po QAM
Selenium 200 mcg po QHS

B-vitamins:
B-100 po QAM
(the B-100 has 100mg Thiamin, 100mg Riboflavin, 100mg niacinamide, 100mg B6, 400mcg Folic acid, 100mcg B12, 100mcg Biotin, 100mg Pantothenic acid)

Other:
Vitamin D 400 IU BID
Coconut oil (MCT oil) 1 tbsp po BID

[BrokenBladder]

Kira glad to see you back. Well it sounds like all in all you made out pretty good. I wanted to ask you though, how long have you had the nausea and vomiting in general. The reason I ask is I'm on the same medication for the last 6 months, but for some reason starting the end of Jan. I started throwing up almost everyday. There dosen't seem to be a rhyme or reason for it, but it just keeps happening. I've also lost 10 pounds in the last month and half. Most people wouldn't complain but I'm down to 96 pounds!! Too thin for me. Just thought I would pick your brain a bit if you feel up to it.

[Kira]

I have had nausea and vomiting off and on for several years. When I first got sick 6 years ago with mostly neuro symptoms, I was also throwing up a LOT. That gradually got better, though. I mostly get it when I am having other issues, like the muscle/rhabdo stuff, neuro stuff, and/or mood stuff.

It has been a lot worse this past month than it has been for quite some time. I had some problems with it in October but was mostly okay for a few months. Then this January/February I have had four episodes of it, two of which caused rhabdo attacks.

I am wondering if part of it is med-related, because I do get nausea from the high-dose dilaudid I get in the hospital. I do get constipation from the opiates, and maybe this is slowing down my gut motility (the speed food moves through the GI tract) enough that it is making me nauseated? And then once I start throwing up, the fact that I can't keep food down sets off my metabolic-related nausea? Don't know.

What are you on? I use the fentanyl patches and oxycodone, as well as effexor, tetracycline, and prilosec. I have also been taking zofran and phenergan for the nausea and miralax for the constipation. I take a lot of supplements for my metabolic disorder as well.

[BrokenBladder]

I'm on alot of medications for my different illnesses, but here's the list. Elmiron, Atarax, Valium, Oxycontin, Vicoden, and Soma. I've been on all of these same medications for a while and I didn't used to have a problem. One thing that did happen is when I had surgery in the first part of Janurary they told me that I was two pints low on blood and my potassium was low, but two weeks later when the blood work was done again they said everything was normal. Well I don't feel normal and my weight is showing I'm not responding like a normal person. I get so tired of doctors that I just haven't done anything else about it. Maybe I should, but what? I do feel very comfortable with my doctors I'm just tired of living to make yet another appointment to see a doctor.

[mrsdoubtfyre]

I am sorry you are going thru this.

I did a search earlier today on rhabdo...
and found a blog where a gal used this special oral glucose
product successfully...to avoid IV --

http://www.drugstore.com/products/prod.asp?pid=37730&catid=10017&trx=PLST-0-CLOSE-MATCHES&trxp1=10017&trxp2=37730&trxp3=1&trxp4=0&btrx=BUY-PLST-0-CLOSE-MATCHES

This is OTC in USA..

I cannot recall if you have mentioned this before... so I am just posting it now.

[Kira]

Mrs D,

I haven't used the gel before but did try the tablets that diabetics use... dissolved them in my mouth like you do with sublingual meds. I think I will try the gel. Maybe if I can get it to absorb in my mouth I won't have to keep stuff down.

I do have something called Po*****e that is a powder I use to make oral solutions. It is short chains of glucose... shorter than starch, longer than sugar. They use it mostly to add calories to formula for failure-to-thrive babies/kids. The nice thing is that it doesn't taste all sweet like sugar, but it does make the solution a bit thicker and very sticky. The problem is that I have to drink a lot of the stuff. It works when I am trying to ward off an attack triggered by exertion, fever, etc, but doesn't work when I am vomiting.

Thanks for the idea... I will try it while we are trying to get this port business worked out.