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Skeletal muscle channelopathy (Na or Ca) management

Discussion in 'Other Health News and Research' started by pattismith, Nov 24, 2017.

  1. pattismith

    pattismith Senior Member

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    What can we learn from management of Hypokaliemic Periodic Paralysis?
    This may be useful to ME patients with low/borderline plasma potassium levels and muscles weakness

    Here an interesting link with some extracts :


    "Ion channels are tiny pores (openings) that allow ions (molecules) of sodium and potassium to move through the cell membrane. For the muscles to work properly sodium and potassium ions must be kept in the correct ratio inside and outside the cell. When the level of potassium in the blood falls these ion channels fail to regulate the flow of ions properly. The ratio of sodium and potassium inside and outside the cell become unbalanced. The muscle responds less when asked to move, which is felt as weakness. If the imbalance becomes pronounced the muscle quits responding at all, i.e. is paralysed. This person with HypoKPP is very sensitive to drops in serum potassium. Some patients with HypoKPP may become paralyzed while their potassium levels remain within "normal" limits."

    "However the level of potassium always falls. Sweets or starchy foods trigger attacks because these foods cause potassium to move into the muscle cell, and lower the level of potassium in the blood. Potassium (by mouth or by an IV line) makes the attack go away."

    "Many HypoKPP attacks are triggered by food. Food triggers include sweet or starchy foods like candy, cakes, pie and other desserts, soft drinks which are sweetened with sugar, fruit juices, bread, cereal products, rice, potatoes, and pasta. Foods like these are digested very quickly and raise blood sugar rapidly. The pancreas responds to this rapid rise in blood sugar by producing a lot of insulin. Insulin drives potassium from the blood into the muscle cell, which triggers weakness. Salty foods, like potato chips or pickles, are also a trigger for many HypoKPP patients.

    Getting too hungry, or eating a large meal (especially if you are very hungry) triggers episodes in many patients. Other common triggers include unusual activity or exercise - usually the day before the attack, but sitting still for too long may also trigger attacks.

    Patients learn that pacing their activities is absolutely vital. The activity level should be kept close to the same each day. Sleep is a strong trigger. Many patients wake up paralyzed in the morning or after a nap. Getting too cold (or too hot) makes some patients weak. Humid weather or a rapid change in the weather (like a storm) can trigger attacks in some patients."

    "Some medications may cause attacks. These include muscle relaxants and beta-blockers, some tranquilizers, pain killers, antihistamines, the puffers used to treat asthma attacks, some antibiotics and cough syrups. The eye drops used to dilate the pupil during eye exams have been reported to cause paralysis. Epinephrine or adrenaline , a drug routinely added to local anesthetics, should be absolutely avoided by patients with HypoKPP. Many patients report paralysis attacks occurring in the dentist's chair or following dental work, or after having a wound stitched or a mole removed. Over the counter drugs can be dangerous and should be avoided if at all possible. If it’s necessary to take something it's wise to try 1/4 of the recommended dose to make sure it doesn’t cause weakness."


    "What medications are prescribed for HypoKPP?

    "The carbonic anhydrase inhibitor 'Diamox' (acetazolomide) is often prescribed for HypoKPP patients. This medication helps keep the potassium from getting out of balance in the first place, by affecting the mechanism that moves potassium from the blood into the cell. ...."

    "Diuretics which cause the kidneys to 'spare' (or conserve) potassium are also used to treat HypoKPP. Dyrenium and Aldactone (spironolactone) are in this family. .....Patients who are taking diuretics which cause the kidneys to conserve potassium should consult with their doctors before taking potassium supplements."

    "Most people with HypoKPP still require potassium, even on diuretic therapy, but it should be carefully monitored.
    Those on carbonic anhydrase inhibitors generally need to take some potassium to replace what is lost due to therapy itself.
    Most patients with HypoKPP use potassium to abort developing episodes. Patients differ in their reactions to different forms of potassium but, as a rule, most people find that the effervescent potassium citrate or bicarbonate tablets which dissolve in water are the most effective and easiest on the stomach.
    Potassium chloride tablets are slow to dissolve and are hard on most people's stomachs, though some forms of potassium chloride capsules have coated granules of potassium which dissolve in the intestine and are much easier on the digestive system. If potassium chloride tablets are required these are preferred by many patients."
     
  2. Gingergrrl

    Gingergrrl Senior Member

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    @pattismith Was there a link to the calcium channelopathy part or only potassium? I read through it and think I missed that part. I belong to a group re: the CA+ autoantibody but there is shockingly very little info out there! Thanks in advance. (I saw CA in your thread title so I was very curious)!
     
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  3. pattismith

    pattismith Senior Member

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    I know it seems strange, but calcium and sodium channelopathies induce sensitivization to potassium fluctuations.

    This means that skeletal muscle symptoms are the result of these potassium variations.

    I was very long to understand this, because the official name of the genetic disease is "Hypokalemic Periodic Paralysis" (HPP), but actually the affected channels are not potassium channels as we would think, the affected channels are Calcium and Sodium channels.


    Here a wikipedia extract:


    "Genetics
    Mutations in the following genes can cause hypokalemic periodic paralysis:

    HOKPP1 170400 CACNA1S (a voltage-gated calcium channel Cav1.1 found in the transverse tubules of skeletal muscle cells) 1q32

    HOKPP2 613345 SCN4A (a voltage-gated sodium channel Nav1.4 found at the neuromuscular junction) 17q23.1-q25.3 "

    You will find more complete medical articles about this condition here:

    https://emedicine.medscape.com/article/1171678-overview
     
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  4. pattismith

    pattismith Senior Member

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    From this paper, I took a list of drugs that can induce hypokalemia (these drugs can trigger an episode of wealness in this HPP). It is strange to notice that acetazolamide is used to treat HPP and can induce hypokalemia at the same time. I guess a patient need more potassium supplementation when taking it...

    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4357351/table/t2-ptj4003185/

    @Gingergrrl , what can you tell us about autoimmune calcium channelopathy? Does it affect skeletal muscles?
    Is there any worsening with blood Potassium variations? (note that it can be either decreasing or increasing potassium blood level that can trigger problems, as Hyperkalemic Periodic Paralysis also exists)
     
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  5. Gingergrrl

    Gingergrrl Senior Member

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    Patti, honestly I cannot tell you much and I have tried to study it in as much depth as my brain can understand! I believe there are four types of CA+ channels (N, L, R and P/Q), unless I am forgetting one, and that I have the autoantibody that attacks the N-type. They are considered paraneoplastic autoantibodies even if no cancer is ever found. The N-type correlates most commonly with LEMS or Small Cell Lung Cancer (SCLC) and requires ongoing cancer checks. But often, no cancer is ever found like in my case.

    I was diagnosed by the PAVAL Panel w/blood sent to the Mayo Clinic in early 2016. I know both the N and P/Q types are associated with LEMS which involves major muscle weakness. I was having muscle and breathing weakness that was progressing even though I was negative for LEMS on an EMG and NCT in 2016. I also have POTS, which made the situation more complex, and my doctor believes is due to other autoantibodies that I have in addition to the CA+ auto-antibody. The first thing to significantly improve my muscle and breathing weakness was high dose IVIG and then it plateau'd until I did Rituximab which led to additional improvements. I have never experienced any kind of paralysis.
     
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  6. pattismith

    pattismith Senior Member

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    Patients with HPP experience mostly weakness, so it may be very similar to your symptoms, although I don't know were are located the Ca channels affected by your antibodies, I guess symptoms will depend on their precise localisation.
    Do you know your potassium plasma level?
     
  7. Gingergrrl

    Gingergrrl Senior Member

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    Patti, I have no idea where the CA+ channels are located and assumed they are spread throughout the body?

    Also, the triggers in your article above do not make me worse. I feel better with no food or periods of fasting and I also have no problems with sugar or salt. I do well w/pain pills, beta blocker, and antihistamines. I do NOT do well though w/Epi or anything stimulating.

    My potassium runs low and I take a prescription supplement Klor Con that is 20 MEQ per night.
     
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  8. Nine lives

    Nine lives

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    Very interesting! Within last few years, my salt issue seems to be getting worse. My potassium is in normal range.

    I found a useful clinical write up here: Hypokalemic periodic paralysis - an owner’s manual http://simulconsult.com/resources/hypopp.html

    Triggers

    Carbohydrate meals. I need small, higher protein meals during day especially lunchtime otherwise need to lie down/tiredness. Large carb meals make me feel strange (not a blood sugar issue as fasting blood sugar around 80 rising to 105-125 an hour after eating).

    Sugar/Candy. Stopped from teenage years onward. Only use low glycemic carbs for mood stability.

    Salt. I cannot salt food and if I eat salty food I use ionic potassium to control arrhythmia/tiredness.

    Epinephrine. Cannot use at dentist - racing heart.

    Exercise - PEM

    Cold - avoid as much as possible and I live in warm place

    Better with alcohol - but not too much (night waking/ not being able to breathe)

    EMF - electric shocks from holding phone. No wifi at night etc Feel ghastly in cell phone shops/Apple store..

    Feeling knocked out after sleep occasionally (too much progesterone).

    Sub-group susceptible to heart rhythm abnormalities talked about in the article seems to be me. But my muscle weakness is more upper body than lower (but I do get leg cramps fairly often).

    I definitely need to look into this more...
     
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  9. pattismith

    pattismith Senior Member

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    I too cut off sugar/candy 6 years ago because I felt very bad after taking it.

    Most people think it is a consequence of hypoglycemia, but it may be the result of induced hypokalemia as well (or combined)...

    When we have muscle problems, I guess we need to keep our blood potassium level far from the extrem values (min 3.6 mmol/l and maxi 5.2 mmol/l) and the more stable as possible. Level at 4.2-4.6 mmol/l should be the safest...

    I supposed it may be also important for other muscles than skeletal (Intestine, heart...) and nervous system as well.:thumbsup:
     
  10. Gingergrrl

    Gingergrrl Senior Member

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    Thanks for posting, Patti, and I had been curious about PPK (or KKP?), I have seen different acronyms but assume it is the same illness? Thanks for laying out the symptoms and they do not match for me and my CA+ autoantibody is definitely different (even though I do have low Potassium on tests when I do not supplement it).

    I have no problem w/carbs but am gluten free for autoimmunity.

    I have no problem w/sugar.

    Salt is extremely beneficial to me for both POTS and muscle weakness/pain and I used to take salt stick tablets throughout the day (although this is no longer necessary as I have improved).

    I have complete alcohol intolerance for almost 5 yrs, I suspect b/c it is a vasodilator and I already have low BP and b/c of the high histamine content in alcohol.

    I have no problem w/cell phones, wifi, or EMF.
     
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  11. kangaSue

    kangaSue Senior Member

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    Brisbane, Australia
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  12. Gingergrrl

    Gingergrrl Senior Member

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    I know you have explained this to me before, KS, and I know what "VGCC" is but I cannot remember what "cav 1.1 means"?! I know that I do not have the potassium periodic paralysis disorder (not sure of the exact formal name) but was interested in the CA+ channel connection. It sounds like you are saying the connection is the L-type vs. the N-type auto-antibodies (even though I forgot what "cav" means). Is this correct?
     
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  13. aquariusgirl

    aquariusgirl Senior Member

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    @Gingergirl can you recommend a brand of salt stick tablets?
     
  14. Gingergrrl

    Gingergrrl Senior Member

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    Yes and the brand I buy is actually called "salt stick" and here is the link:

    http://saltstick.com/product/saltstick-caps/

    I buy the blue bottle which is caffeine free (vs. the green bottle contains caffeine which I do not want).
     
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  15. pattismith

    pattismith Senior Member

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    The wikipedia link seems a bit simple, but it helps to understand the differences between calcium channels, especially between the N type you have problem with and the L type (HypokalemiaPP)

    "High-voltage-gated calcium channels include the neural N-type channel blocked by ω-conotoxinGVIA, the R-type channel (R stands for Resistant to the other blockers and toxins, except SNX-482) involved in poorly defined processes in the brain, the closely related P/Q-type channel blocked by ω-agatoxins, and the dihydropyridine-sensitive L-type channels responsible for excitation-contraction coupling of skeletal, smooth, and cardiac muscle and for hormone secretion in endocrine cells."

    If you look at the first table, you will find that L type and N type have very different reactions.

    For the N type, they quoted only α1 subunit Cav2.2 (gene CACNA1B).


    A mutation in CACNA1B may help us to understand better how your nervous system may be affected by your auto-antibodies

     
  16. Gingergrrl

    Gingergrrl Senior Member

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    I wish it was simple to me but it is way above my skill level! I get it that there are four types of CA+ channels, and I have an autoantibody that blocks the N-type, (and I assume that the Potassium Paralysis disorder is the L-type which I do not have autoantibodies for and have never had an episode of paralysis). But I don't know what the other terms mean and still can't figure out what "cav" is :bang-head:

    Patti, do you have any of the auto-antibodies or the Potassium Paralysis disorder or are you trying to figure out if it pertains to you?

    Edit: I changed my post above b/c I think there are four types of CA+ Channels and for some reason, I had written five.
     
    Last edited: Nov 26, 2017
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  17. pattismith

    pattismith Senior Member

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    I think but I could be wrong, that cav means "calcium votage" (Nav means "sodium voltage" etc)

    cav2.2 refers to one part (or type) of the subunit alpha1 of the calcium channel complex.

    I don't know if I have hypoPP, but I am now convinced that my brain and my mucles have a kind of sensitization to borderline/low blood potassium. It may be a channelopathy, either genetic or secondary, I don't know...
    I don't know either if there is a test for L type calcium channels auto-antibodies...:lol:

    I don't know much as you see, but I am on PR only for 6 months so I may learn more about it :)
    And with an acurate blood potassium level, I may learn more quickly :lol:
     
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  18. Gingergrrl

    Gingergrrl Senior Member

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    Oh, that makes total sense! :bang-head:

    What symptoms do you personally experience when your blood potassium is low? Do you take a prescription supplement?

    There is b/c I had the test in 2016 as part of the paraneoplastic (PAVAL) Panel at Mayo. It tested for the N-type, L-type, and P/Q-type (but not the R-type for some reason). I was positive on the N-type.

    You know more than you think. I have been on PR since June 2014 and am still learning stuff every day.
     
    Last edited: Nov 26, 2017
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  19. pattismith

    pattismith Senior Member

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    :thumbsup:

    If you want to know more about my story with potassium, I tryed to give symptoms, datas and explanations in this thread.
    I will dig into the Mayo Panel to see if I can find a test in Europe. Thank you for the info.

    (PS: Looking for informations about potassium on PR, I found that fredd had a similar experiment than I did: He felt more confortable with blood potassium not below 4.2 mmol/l)

    Edit 1: I don't think that hibernating mammals are a real model for ME patients, but there is some similarities. Here what I found about calcium channels in hibernating mammals:

    "Compared with cells from non-hibernators, hibernator cells are characterized by downregulation of the activity of Ca channels in the cell membrane, which helps to prevent excessive Ca 2+ entry."

    (this prevent hypothermic Ca overload in cells)

    It may be that ME patients have down-regulated Ca channels from their hypometabolic state...

    Edit 2 : Hydrogen sulfide has the capacity to induce torpor. It is also a Calcium channels modulator:
    In this paper, it is proposed that hydrogen sulfide is inhibitor for L and T types Calcium channels...

    (just to show that auto-antibodies are not the only cause for downregulation of Calcium channels, and that factors at play in ME could do it as well)

    Edit 3: low blood potassium may be real in the hibernation state as you can see on that diagram, where potassium enter cells:

    upload_2017-11-26_13-23-24.png
     
    Last edited: Nov 26, 2017
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  20. aquariusgirl

    aquariusgirl Senior Member

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    All a bit above my head but Judy Mikovits said at a presentation in London that calcium & copper transporters are blocked but they don’t know where.

    I definitely felt better taking supplemental copper fir a while.

    Acumen labs in Devon UK has been looking at intracellular calcium for a while.
     
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