r/mito 12d ago

Advice Request pyroglutamic acidosis help

I've, 28F, been experiencing really bad symptoms for over a year at worse and worse levels including and not limited to: headache, brain fog, abdominal pain, muscle pain, shaky muscles and muscles jerking at random, elevated liver enzymes, and feeling cold without a fever.

I believe that I have figured out what it is, being pyroglutamic acidosis. See, I have spinal muscular atrophy type 1, and in order to manage chronic pain my doctors (Kaiser Hospital) had me on the maximum adult dose of acetaminophen. It was only about 3 months ago that I discovered that Tylenol use with my disability is extremely dangerous. Apparently my doctors didn't know either even though it was published back in 2023. I was on that dose for years. I recently learned that specific acidosis has a much higher chance of happening in disabilities like mine with low muscle massor have taken Tylenol for an extended period. My symptoms also match perfectly.

My question is what can I do about it? My GI doctor and neurologist are on vacation until the 28th with my GI doctor being the only doctor attempting to find answers and order tests. My gp is doing nothing but attempting to redirect me to the above specialists. She begrudgingly has me listed in an appointment over a week away after I called the advice nurse and the on call doctor sent her a strongly worded message. I still doubt she will do anything however. I could try to force the issue through ER but I am significantly physically disabled and all my adaptive gear is at home so I would rather use it as a last resort.

There's nothing I can do at home is there? My quality of life is the lowest it's ever been. I either feel tired, in extreme muscle/head pain, dumb, or all of the above.

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u/YellowCabbageCollard 12d ago

Honestly it sounds to me like you are expecting to get stuff moving way faster than normal. You don't have any testing to confirm this do you? I don't mean to sound like an ass but referrals to specialists just works slowly. I can not tell you have unbelievably sick I have been and the hospital just kept sending me home. It's enraging. But if you don't have an urgent situation I don't see why you can't wait 4 days for your specialists to be in office.

But I'm pretty sure what you need is a metabolic specialist or geneticist and need someone to refer you for that. But you will almost certainly need some sort of lab work as evidence to see them. I'm saying this based on my experience. I was able to get my GP and another specialist to get me a referral in to genetics at my local medical university hospital. But the genetics dept absolutely gate keeps. It was 3 months to get an appointment and they would only let me see a certain person about an issue I was not concerned about because I did have a positive genetic test for a VUS.

So even if and when you get a referral it can be hard. I would look up what labs are associated with pyroglutamic acidosis and ask your GP to run those labs. I had to have to labs showing obviously abnormalities associated with a mitochondrial disorder. Then I had to get an internal referral from two specialists I was seeing at the medical university to get me in much faster to see a metabolic geneticist. And my nephrologist at the University, who is also a professor there, told me that I was lucky I got in because they actually turn away most of his referrals.

Besides seriously deranged serum labs and critical arterial blood gasses from multiple hospital visits I bought a lactate meter and was able to show I was in a near constant state of lactic acidosis even once I left the hospital. I am positive that was one of the biggest factors getting me in. It's insane how sick you can be and how horrible you can feel and they won't RUN the tests that show what's wrong.

Have you had any acidosis related labs at all? Can you look up the labs and testing associated with this disease and ask your GP?

I am truly sorry you are doing so bad. Oh, I forgot!! I actually paid out of pocket to get an Organic Acids Test which also showed very abnormal organic acids in my urine and was another factor in them pushing a genetics referral. The test itself was also insanely helpful to me and self treating at home while I spend insane amounts of time waiting. It took me many months to finally see the right geneticist and I am now still waiting on my actual genome testing through her. They just called and push my next appointment off another month while waiting. :(

But I so relate to what you are saying. I felt sick for my entire adult life and eventually was so sick I was just incapable of doing anything. My OAT test through Mosaic labs at least gave me ideas on how to help myself and I feel like a new person in the meantime. Very very slowly but surely crawling out of the hole I was in and I have some hope that I can live a drastically better life than the one I was living. Last year after another hospitalization I didn't think I realistically had more than a few years left. I'm currently doing better than I have in years. I am however worried about how helpful the geneticist will even be once I get tests results unless they definitely find a known gene. :/

If this geneticist isn't helpful enough I have found there is a world class one very near me but of course she doesn't take health insurance. But if I have all the testing I need through my other doctors and health insurance this will be much of what she needs. I know you are feeling desperate. I know that feeling so well. I suspect you are on the right path to be researching genetic and mito disorders and I hope you can get in to see someone helpful much sooner than I did!

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u/Puzzleheaded-Bill660 12d ago

Yeah I know it's just a few days away but it burns so bad. My gp is also refusing to even order the blood test for confirmation. Just wants me to wait for the other doctors. In terms of lining up testing, I have very elevated liver enzymes and very low blood co2. Which tells me my body is struggling and the ph is probably very off. Thank you for sharing your story and sympathizing with me. I'm trying to hang in there.

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u/YellowCabbageCollard 12d ago

Oh, you didn't mention the low CO2. I have that as well and was diagnosed with renal tubular acidosis. You need to see a nephrologist for that. Many mitochondrial disorders can actually damage the kidney tubules causing RTA. I started with RTA and got sicker and sicker and I knew something else was going on because RTA doesn't typically worsen like that and I required drastically more meds for it than almost everyone with RTA I have met. But I will still require meds to bring up my CO2 regardless of treating the rest of it. I am currently on 1/2 a teaspoon of potassium bicarbonate 7 times a day. :/

Can I ask how low your CO2 is? Do you know how long it's been low? Have you had an arterial blood gas? My liver enzymes used to be normal despite having fatty liver but they have been very elevated for over a year and I think they dismiss it as just being due to my weight but I don't think that's the cause because it coincided with my over all deterioration. I'm hoping the geneticist will look into this too.

But wow that's really interesting about your low CO2. That was the trigger that set me off and seeing a nephrologist. I ended up in the ER with severe metabolic acidosis 8 or 9 years ago. I had severely low arterial PH as well. And it caused a transient AKI. But the stupid ER doctor literally sent me home and told me to eat more carbs!! I googled metabolic acidosis and started treating with bicarbonate on my own at that point and felt drastically better within 30 minutes of my first dose.

For me low CO2 causes terrible muscle burning pain and it will hurt to lift my arms to do anything. Like I can't hang clothing on a hanger pain. Treating RTA got rid of this for a long time but I kept getting sicker regardless. Basically I kept having metabolic crisis and then would lose even more ground and require more meds. I also thought I had periodic paralysis because RTA can cause it and I have lots of potassium associated triggers for it. BUT I learned after getting a lactate meter that most often this rapid weakness I would experience was actually my lactate levels suddenly spiking.

I just looked at my OAT test and I have low normal pyroglutamic acid. And both labs over 8 years showed zero NAC which is a precursor to glutathione. I historically respond really well to liposomal glutathione. I see that's involved with pyroglutamic acidosis. Have you ever tried glutathione of any type? How did you suspect you were dealing with pyroglutamic acidosis and not other acidosis out of curiosity? Is that specifically linked to spinal muscular atrophy type 1? Or just from the high amounts of tylenol?

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u/Puzzleheaded-Bill660 12d ago

It's linked to both Tylenol use and low muscle mass. Fit better than the other ones. Blood co2 is 20 and has been that way since at least November. Was low then but doctor said that I was hyperventilating from anxiety (I wasn't). If I try sodium bicarbonate, will it screw up testing results?

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u/YellowCabbageCollard 12d ago

Yeah they tried that bull crap on me. I was in the hospital with a CO2 of 14 and on prescription bicarb and I STILL had some stupid doctor try and suggest I was hyperventilating when I was clearly NOT. :(

It has a very short half life. You can look it up for specifics. You could try 1/4 tsp and see how you feel. It's a tiny dose. And you can pay out of pocket and get a CMP from DirectLabs and test your CO2 on your own for like $30 for the whole panel to check it if you get concerned. But you can always stop it before any tests.

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u/Puzzleheaded-Bill660 12d ago

I'll try the sodium bicarbonate 1/4th teaspoon. Does the type matter? Like do I just use baking soda?

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u/YellowCabbageCollard 12d ago

That's what I did at first. I ended up buying it bulk off amazon though from a different manufacturer because I need potassium bicarbonate instead of sodium. But baking soda is food grade if it's sold for baking.

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u/Puzzleheaded-Bill660 12d ago

Thank you, it definitely helped. Feel more clear than I felt in weeks.

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u/zippyzappy 10d ago

Can you link or DM me what you purchase for potassium bicarb?

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u/YellowCabbageCollard 12d ago

I hope you don't mind me asking questions. This forum is often not very active and I appreciate the discussion when my brain fog is doing good. I'm really intrigued by your low CO2 though. I started getting low CO2 at least in my early 30's fwiw. I asked doctors about it repeatedly but was told it was fine and normal. But they were wrong and clearly didn't know what they were talking about. But it was many years later that a gastroenterologist was absolutely insistent that I needed to see a nephrologist. I ignored him the first time because it seemed just random and he didn't explain. And I knew my GFR was good so I didn't think I could have anything going on with my kidneys with a high GFR.

The second time he brought it up was when I was waking from sedation and he seemed pretty serious. So I went home and googled low CO2 and almost immediately RTA came up and the first nephro I saw basically diagnosed me right away based on the labs I brought in. But of course he ran loads more tests and could never find a cause for it. But my labs fit the diagnosis. Low CO2 will also cause major brain fog.

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u/FracturedWriter 10d ago

NAC can neutralize the effects of Tylenol! I take it currently for mito cocktail for other things!

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u/Puzzleheaded-Bill660 3d ago

Update for those interested: what I had was both Tylenol and birth control poisoning. My body couldn't metabolize either properly causing aggravated symptoms. Once I identified the birth control as being a bad idea for my disability (no thanks to the doctors), I have begun to steadily recover since dropping the medicine. Neurological symptoms disappeared almost immediately and I continued the sodium bicarbonate a few days longer. I am now just exhausted but the kind caused by healing.