A new perspective

Yesterday I read the most fascinating post by Jen Brea, author of the wonderful film Unrest.  For those that don’t know, Jen developed M.E. after a meningeal-type viral infection 8 years ago and has been severely affected ever since.  She also has dysautonomia/POTS (as many people with M.E. do), Mast Cell Activation Disorder (as many people with M.E. do) and has all the hallmark symptoms of hypermobile Ehlers-Danlos Syndrome only without the hypermobility (we hear this more and more, ie that people can appear to have connective tissue disease without having bendy joints!).

After being very ill for several years, Jen started not breathing when she lay down.  To cut a long story short, she turned out to have cervical cranial instability (CCI) and tethered cord syndrome, both of which are among several neurological and spinal problems seen in hEDS.  Jen admits she’s lucky enough to have access to one of a handful of specialists in the world who treats these diseases, and has the resources to fund surgery which she successfully had a few months ago.  She writes about the remarkable transformation the surgery has had on her health and in particular that she is now in remission from M.E.  That’s right, in remission from M.E. so severe she has been largely bedridden for 8 years!  She also says her POTS has vanished and her MCAD is getting better all the time.

Yet she had classic M.E.  No symptoms whatsoever before coming down with the meningeal-type viral infection back in 2011.  So how on earth can a viral infection, which is the precipitating factor in most cases of M.E., cause mechanical issues like CCI and tethered cord?  I’m no expert, but the obvious link is that the viral infection affects mast cells (which I’ve been saying for eons), which then weakens/affects connective tissue (as mast cells live in connective tissue) and this weakened connective tissue then doesn’t hold the spine in place properly.   Of course, weakened connective tissue can also affect veins (leading to POTS) and all the other symptoms we see in traditional hEDS including things like gastroparesis and chronic pain.  Having our spinal cord compressed would also account for brain fog, pins and needles, muscle twitching, vertigo and many of the other symptoms found in M.E.

So it looks like there may be two issues going on.  There are people, like myself, who were born with hEDS.  This may pre-dispose us to developing M.E. due to the issues having faulty connective tissue has on our mast cells, which are at some stage in our lives triggered by a viral infection.

There are also people who don’t have hEDS, but who develop a viral infection which affects mast cells, which in turn affects connective tissue.

So, one is the chicken and one is the egg but the end result is the same.

I have no doubt I have cervical cranial instability.  For a start my head feels like a bowling ball sat on top of my neck and is much of the reason I have to spend 17 hours of every day in bed – I simply can’t hold my head up all day.  Plus, if I’m sat upright and til my head backwards even a little way the room starts to spin, I get severe buzzy pins and needles throughout my entire nervous system, I develop palpitations, my brain feels like it’s cramping and just feel really, really, weird.  I have also suffered from lower back pain since I was 11 years old, which wasn’t cured following surgery for rare congenital spinal stenosis.  I can only assume I also have tethered cord or some other hEDS related spinal issue.  Not that I’m going to attempt surgery for either, as I don’t have the money or support, and can’t tolerate the drugs or analgesics used in surgery even if I did.

It’s all food for thought.  I’ve long suspected faulty mast cells to be at the heart of everything so, rather than going through the drastic step of surgery for CCI and other spinal issues, surely if we can inhibit mast cells that would stop the connective tissue from being weakened in the first place?  At least for people not born with hEDS.

For those who have genetically weakened connective tissue the issue is more complex, but even so if we can inhibit mast cells would that not theoretically make our connective tissue less unstable?

I don’t know if any of my thoughts on the mechanisms involved in M.E. are on the money – I’m so dizzy and have such severe brain fog today it’s a wonder I’m functioning at all 😉  I’m sure my less brain-fogged and more intelligent readers will share their own theories.

I’m so thrilled for Jen though.  She’s been to hell and back, yet despite that managed to make the best film I’ve ever seen on M.E. and promote it world wide.  She deserves to be well and I hope her recovery goes from strength to strength.

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14 thoughts on “A new perspective

  1. Autisticzebra

    The more I think about these issues, the more convinced I am that none of us are actually born with HEDS but rather that it’s activated in some of us quite early. That HEDS isn’t actually an EDS at all but rather a symptom of MCAS. And that’s why they haven’t yet found the genes for it.
    I’m trying to get my thoughts straight on it and when I do I’ll write a blog post.

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    1. Jak Post author

      I’m in two minds. My Mum has hEDS and I’m convinced my brother either has Marfans or hEDS, so for us there definitely appears to be a genetic link x

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      1. Autisticzebra

        You misunderstand me. It’s definitely genetic but rather than being a fault in the collagen making genes, I’m thinking it’s a fault in mast cell genes leading to mast cells attacking our collagen.

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        1. melody

          Autisticzebra, I requested access to your blog, in case it’s not clear who that is. I would like to read your post on this when you write it!

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  2. melody

    Jak, what you say sounds here so right on, it rings like a bell in my head. I am wondering about genetically weakened connective tissue and what that might really mean–does it take different paths depending on environment and triggering events? I’m bendy, my Mom was bendy and diagnosed with schizophrenia, which the more I know, the more I’m convinced she had a lot of the same problems I do, though thank God, I’m doing pretty well considering. My middle sister isn’t bendy in the same way, but she has had an awful time, Bell’s Palsy three times, probably Sjogren’s, and the usual asthma, joint problems, and allergies. As a teenager I had ??–acck, the name just exploded away–oh, yeah, mono, the internet is useful!–which maybe set me up for later problems???
    So I’ve been reading about connective tissue (basically the building blocks of our entire bodies and connective tissues’ rather startling properties, piezoelectric semi-conductor, etc.), straining my pretty limited knowledge of physiology, but the more I read, the more fascinating it becomes–as mast cells are one of the main constituents of plasma-based connective tissues or blood, and apparently, unlike other immune cells, hang out in the brain.
    Thanks for this post, it’s definitely engaged my interest!

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    1. Jak Post author

      It’s weird how these issues affect family members differently. My Mum definitely has both MCAD and hEDS, yet isn’t affected anywhere near as badly as I am physically but has worse mental health issues than me (mast cells definitely affect mood and are implicated in autism). But then, I wasn’t badly affected until I was in my 40s – why not? Are my peri-menopausal hormones entirely to blame for my decline or is there something else at play? Why, when my M.E. started to improve, did my hEDS and MCAD explode? So many questions! Jak x

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  3. Jan Groh

    I’m also of two minds about whether hEDS (and HSD) is a genetic collagen defect like all the other truly rare forms of EDS. (I will go to my grave saying hEDS is still not rare even though that’s what the white coats insist on calling it arbitrarily – no one really knows. And that HSD is just a related phenotype based possibly on a different hormone panel or?? who knows. I’ve gone from globally bendy to stiff myself just with age. Did I not have hEDS before? Was I “cured” of it by aging? I don’t think so. Any more than Pluto stopped being a full planet in 2006. We just changed our minds about what to label it, right?)

    Anyway, I do think mast cells play a role in messing up our extra-cellular matrix, which in turn exposes our mast cells more. But I find it interesting that I was “born this way” with clear signs since birth, but that just weren’t recognized until 7 years ago. (I had to fall completely apart from head to toe and stop walking to get diagnosed with anything but depression finally. Nope, depression didn’t split my SI joint or take my right leg out of its socket, no!)

    In any case, I rather favor the RCCX hypothesis for why we have no answer to hEDS/HSD, and why we vary so much (some super bendy, lifelong, some not). I’m also not entirely convinced the CCI etc are the entire answer for ME, or for all cases. I just can’t help thinking there has to be some additional immune system component seeing how very sick people get when it flares. The RCCX hypothesis (deep rabbit hole) for anyone not yet familiar:

    http://rccxandillness.com

    But I’m very happy for Jen and anyone else who got or gets better from treating their Chiari or CCI etc. (read: squished central nervous system). I hope others can benefit from this experience. I’m thrilled for her remission. And pray we can continue helping others achieve their own too. (Says one who feels she was misdiagnosed with CFIDS 30 years ago, whence my domain name: OTWIST.com as in: Oh That’s Why I’m So Tired!). Best wishes to all.

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    1. Jak Post author

      Totally agree Jan that hEDS isn’t rare. Since I was diagnosed 8 years ago I now see it everywhere! You can tell a hEDS person just by looking at them in many cases.

      My brother isn’t bendy at all now (he’s 54) despite being hypermobile as a kid/teenager. I’m very stiff but still bendy. My Mum is still bendy at 79 but not stiff! So we’re all affected differently.

      Like you I was born with both hEDS and MCAD, as was my Mum. Yet I had to diagnose myself at 42 with hEDS and my MCAD didn’t got bonkers til I was 44. Why not til then? And why has my Mum or brother never had a huge flare up/crisis like I had?

      I hadn’t come across the RCCX theory so thanks for sharing. Sadly with my brain issues it’s all a bit too technical for me but I got the gist.

      I’m not convinced that people with these conditions are gifted or high achievers etc. I’m always suspicious that these kinds of people are the ones who are intelligent enough to find each other and become prevalent voices, or who offer themselves for research. Many of my ME friends are intelligent and driven but that’s why I’m drawn to them – like seeks like. And I think brain issues like autism and bipolar etc. are just becoming more widely accepted and diagnosed, so are bound to pop up more frequently in any patient population than they used to. Ditto with the gay and transgender community. Obviously that’s why we need large scale studies of a wide patient population, to see how prevalent various traits actually are.

      All totally fascinating though!

      Liked by 1 person

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    2. Jan Groh

      Oh, this is interesting… it looks like this has been proposed before, hat tip a FB friend who shared this with me: https://www.massmecfs.org/more-resources-for-differential-diagnosis/172-cfidsfm-and-chiari-malformation-surgery

      And I agree, there should not be a rush to surgery without proof of true need for it. I.e, this shouldn’t be an easy bar to meet. (I can’t imagine it is, unless surgeons are being unscrupulous and operating just for money.) But I also know how desperate people can be.

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  4. Lyuba

    Jak, you have stopped me in my tracks with your post on Jen Brea. It totally makes sense in mine and my children’s case. I am now taking a massive doses of Ketotifen, Chromoglicate, Ranitidine and Fexofenadine and my MCAS symptoms and ME symptoms have improved dramatically.I am able to function. Some days I am nearly a normal person. But. Life is still hard. The need to pacing and being careful is still there.
    We all have hypermobility and the advice we get (like most) is exercise etc. Nobody ever tries to connect the dots. Interestingly, Dr L. Afrin in one of his videos did say that it is possible that mast cells might be causing hypermobility. It stuck with me. For years I’ve had weird neurological symptoms like at one point I couldn’t type. My fingers weren’t doing what I wanted them to and pressing all the wrong keys. I couldn’t even type my surname. I had to do it with one finger. Then I have started noticing that I couldn’t understand the logical connection between things. My lower back would go numb if I leant forward for a bit, I would get horrendous pressure headaches when driving (which I believe was due to sitting in a certain position and was relieved after lying down). My vision would go blurred for hours at the time. Then the tingling in my extremities and circulation problems where my feet would literally go black. The reaction to stress became ridiculous. I would feel “explosion” in my stomach and arms (possibly of adrenaline) when driving and many others… But very interestingly I have noticed that my neck feels unstable plus when I turn my head from side to side I can hear a sound of bones rubbing together. An issue with possible cranio-cervical instability fits the bill completely. It is obviously still a question of what is causing it, but the mast cell degranulation would of course exacerbate the inflammation. Could it be possible that an issue with cerebral fluid is causing systemic mast cell inflammation? In any case, the connection between the ME, MCAS and hypermobility is unquestionable at least in a portion of sufferers. I dream of a day when a miracle happens and a surgery (like in Jen’s case) could solve it all. To think how much we all are costing the NHS is ridiculous.

    I would like to propose for the ME charity to meet and talk with MCAS charity and find a way of a joint way forward. I have recently listened to a Radio 5 show that talked about the families falsely accused of Munchausen Syndrome and the hell they have been through, some had their children taken away! I have been in a similar situation with my children and was lucky that my son was eventually diagnosed with severe Crohn’s (which is now miraculously under control with no immunosuppression, but only Cromoglicate… surprise, surprise). The one issue that was constantly brought up was HEDS. There are people who provide support for these families and who campaign for a national database that would point out that certain undiagnosed conditions like HEDS put families through hell and waste huge public resources through involvement of social workers and endless fruitless visits to specialists who know nothing of HEDS. These families (and I believe there is over a thousand that are known to seek help from this charity) and the specialists treating them could be another group of people suffering from ME, MCAS and hypermobility. This group of people need to be reached out to by the ME and MCAS charities. Only together we can make a bif enough impact and a difference.

    My next step is to try and get the right type of scans and if not the treatment, then at least a diagnosis of possible cranio-cervical instability or Chiari 1. I would also like to assist the meeting of ME (which I know you are on the board of) and MCAS charities to consider a joint way forward. Can you help, Jak?
    Lyuba

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    1. Jak Post author

      Sadly I’m no longer connected to the ME charity Lyuba – it all became too much for me when both my parents became ill so I had to give it up.

      Dr Shepherd at the ME Association is aware of the link for some patients between MCAD, hEDS and ME and is in contact with the charity Ehlers-Danlos Support UK.

      You could always reach out to Mast Cell Action and ask them to get in touch with the MEA? https://www.mastcellaction.org/

      I’m so pleased you and your family are finally getting the help you all need, although I know what a huge struggle it’s been for you x

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