My journey with peripheral polyneuropathy has probably lasted my lifetime. It made itself known along the way but by 2009 its whisper was clearly audible, by 2011 it was shouting, by 2012 it was screaming at me. But what on earth had caused this autonomic then sensory, then by 2013 even the start of motor neuropathies?
I was born to an alcoholic who had attempted (failed) abortion twice with Quinine so peripheral nerve damage right from the start may well have been probable. By six months old I had recurrent infections (later diagnosed as primary immune deficiencies) and a number of viruses that could have caused it. I may have inherited salt wasting from my father and certainly did inherit a collagen disorder from my mother. Did I have symptoms of autonomic, sensory or motor neuropathies in early childhood? Autonomic, certainly, sensory, on and off, motor, maybe, episodically at different times throughout my life.
The closest we came to understanding why my polyneuropathy got to the level its now at was the theory that my collagen disorder – Ehlers Danlos Syndrome – had played a part; that collagen protects myelin which protects peripheral nerves and mine is degenerating. Another specialist suggested my autonomic neuropathy had developed as a result of chemo. But being immune deficient all my life, I wondered if this also played a part, particularly as I’d had Chicken Pox, EBV and Herpes Simplex 1 (cold sore virus), all of which have been linked to peripheral neuropathies. And what of salt craving and my dramatic improvements when on high sodium intake? I knew chemo had tipped the balance and that Tomoxifen was implicated. But how to make sense of the puzzle, the fruit salad?
I could have accepted the word ‘idiopathic’ but maybe that’s just a word people have when they cannot find the ONE cause. But what if that’s because there actually were multiple, cumulative causes?
For me, peripheral neuropathy was sensory, autonomic and finally motor neuropathies. It meant an ongoing fluctuating circus of stabbing and shooting pains in my toes and feet then up the back of my scalp with a scalp that would then hurt to touch, 10 months of pain so intense in my feet it made me cry to walk and hands that I’d wake to find immobile and clawed and stiff, surging pain running up my arms, tingling and numbness in my hands and feet, buzzing running up and down my legs, lips and tongue that lost sensation then turned burning with a permanent sensation of having eaten chillies and everything tasting like salt. It meant vertigo, nausea, temperature dysregulation, inability to sweat, urinary retention and frequency, nursing constipation, tachycardia, arrhythmia, hypopneas, bradypnea, central apnea (even during the day and requiring a ventilation machine at night), blood pressure and circulatory chaos, headache, somnolence. It meant first one leg collapsing, then the other then both, falling up steps, legs going to jelly then sudden surging heaviness making it extremely hard to even lift my feet. It meant waking to find I was like Tin Man struggling to straighten my arms or lift my arm up at the shoulder, getting up from sitting and walking like a 90 year old until I got going. I had times I couldn’t cope, other times I did what I could to wing it, keep myself involved with life, keep seeing my personhood, keep finding my humor, keep adapting. I couldn’t return to my old life so I started one that I could manage. I went to U3A as probably their youngest member, joined classes then became a tutor teaching art and then drama. It wasn’t just my body I had to manage, it was the impact of this circus on my identity and mood. I needed to keep celebrating life even if my body was haywire.
From the Oncologist diagnosing autonomic dysfunction during chemo, the sleep specialist diagnosing Mixed Apnea, to the cardiologist diagnosing Orthostatic Hypotention, to the Ehlers Danlos Specialist diagnosing EDS, to the rheumatologist diagnosing inflammatory tenosynovitis, to the Neurologist diagnosis small fibre neuropathy, slowly the answers came together. The final verdict went something like this…
Collagen protects the peripheral nerves, in an essential part of immune regulation and is an essential part of forming connectivity in the brain which related to information processing. As a person with the collagen disorder of Ehlers Danlos Syndrome I may already have had some level of altered neuro-cognitive development, immune dysfunction and autonomic dysfunction all associated with this collagen disorder.
Estrogen replenishes collagen. As women get into their forties collagen decreases. Chemo damages collagen and results in menopause. Following chemo I was put on estrogen blockers. This kept me in menopause and reduced my ability to replenish damaged collagen. Part of my immune dysfunction is immune deficiencies and food intolerances.
The immune deficiencies mean I struggle to fight off certain bugs, including HSV1 which can cause neuropathy. I’m also gluten intolerant and had returned to gluten in 2010 when the neuropathy began to diversify and worsen.
I was on B6 but following chemo took a magnesium supplement that also had B6 so my daily total was 40mg. The daily RDA for B6 is 1.5mg with max of 40mg. Excess B6 can cause neuropathy and mine was found excessive.
I had been gluten free from 2001 to 2010 but returned to gluten when I tested negative to an IgA celiac test and biopsy (I’m IgA deficient and had already been GF for 9 years at the time so these results would have been unreliable). But because it was hard to get gluten products that were also casein free, soy free and wheat free I had only low level gluten until 2013 when we found a vast range that fitted these requirements. And with that became such progressive worsening the gluten intolerance contribution to neuropathy finally showed itself in full glory.
This time the neurologist tested not IgA antibodies to gluten by anti-gliadin antibodies. Still negative to celiac. Nevertheless with a strong family history of celiac/gluten intolerance on my father’s side and confirmed IgA deficiency the neurologist still suspected I was gluten intolerant and that gluten was contributing (1 in 500 people are IgA deficient but 1 in 10 people with celiac are IgA deficient). So I went back off gluten, dropped B6 supplementation and increased Lysine.
Within two weeks of going gluten free my ability to sweat returned for the first time in 3 years since 2011. The daytime apnea/bradypnea that had been with me in episodes all my life and had got progressively out of control since 2009 then weekly since 2011 suddenly disappeared. The shooting pains up the back of my head (occipital neuralgia) and burning mouth (burning mouth syndrome) disappeared. The buzzing up and down my legs, falling up steps, the jelly legs, fatigue and surges of sudden heaviness disappeared. The ongoing headache began to dissipate and the nausea left. My elbows and shoulders freed up on waking and I could easily move them again. On standing up I could move without all the ‘rust’. Some of the stiffness was still there and I was still on the ventilation machine for Mixed apnea at night, but after battling it for 3 years I appeared to have finally lost most of the neuropathy.
The neurologist concluded that stiffness and slowness with movement were part of the Ehlers Danlos Syndrome. The night time Mixed apnea remained a mystery.
Then Chris and I went to a friend’s place. The served us snacks: green tea with honey, grapes, almonds, dates – things extremely high in salicylates. I ate these even though I knew I was diagnosed as severely salicylate intolerant in my mid 20s. I figured I didn’t have any acute symptoms at that point – no asthma, hives, migraine – so could wing it. Then next day the neuropathy flared. Burning mouth was back, headache, the stiffness flared, temperature was giving me grief, I needed to keep peeing. I realised it was not only the gluten intolerance that was aggravating the neuropathy, it was the salicylates.
So I spoke to my sleep specialist. Was it really possible that the neuropathies could have been chronic reaction to food intolerances and that because I was looking for acute symptoms I didn’t ‘get it’? Was it really possible I could be on the home stretch here? That if I returned to extremely strict low salicylate that I might get off the anti-inflammatories I’d been on since 2012 and maybe even overcome the night time apnea to no longer need the ventilation machine? He thought this sounded feasible. I asked ‘what happens to people who don’t work it out’? He said ‘sometimes they die’. I asked if the ongoing inflammation had contributed to me getting breast cancer or would make it more likely it would return. He felt it wouldn’t do me any favors to ignore it.
I was already legume free (I’m allergic to soy/peanut and react to other legumes) since 2010, casein and wheat free since diagnosed allergic to these in 1990, gluten free from 2001-2010 after being diagnosed as gluten intolerant and now returned to being GF, low/no sugar due to immune deficiencies and inability to fight candida. I had been low salicylate since 1990 when I was diagnosed through intradermal testing as severely allergic to salicylate/phenol and found to get hives and edema from aspirin. But with salicylates I lapsed a lot depending on who I lived with and how easily they accommodated my diet. But now it was time to seriously return to the dietary wheelchair. There would be no more compromising. I either wanted to get healthy and stay healthy and live with the least level of disability this body could muster, or I didn’t. In spite of being a committed autonomous non-conformist, I decided obedience for the right reasons could be a virtue.
After 10 days returned to extremely low salicylate I came off the anti-inflammatories. The neuropathy appears to be completely at bay with the exception of the ongoing nighttime Mixed Apnea. I have been watching the readings on the ventilation machine. The readings are gradually improving. Who knows, in 60 days I may know if I’m free of the machine and can fall asleep and continue breathing without the machine. What a journey. Wish me luck.
I acknowledge Aboriginal and Torres Strait Islander people as the Traditional Owners of this country throughout Australia, and their connection to land and community.