Craniocervical instability (CCI), Severe Bilateral Jugular Vein Compression, IIH and a CSF Leak....HUH?

Well what a ride this one turned out to be!

Started with some self-care. What everyone has been banging on about for past few years now. I found the perfect Osteo literally a 2 minute drive from my house, could get there early for a 9am and then off to work. She understood EDs, she understood me. She was willing to work with SIEDS as she understood the importance of my exercise (which they banned), this were great until the weren't!

We got to my neck. She felt the intense knotted muscle on the right side of my cervial spine, near the base of my skull. She set to work on it after consulting a mentor of her. Crano-sacral drainage they call it. She said it didnt feel right, that things were slow to drain, that my skull almost felt uneven. Took her word for it and tried to relax the best I could. It was a weird feeling. I ended up quite fatigued on the days she would literally drain my head.

Then came the migraines. The aura's were intense and they lasted so long I seemed to go out of one and into another. No headache really, just issues with my eyes and loss of feeling in my extremeties. I also found it really hard to talk. It was affecting work, getting the kids sorted. So I booked an appointment with my GP afer my osteo said she would do a support letter for the referral. We started with Muskoskeletal, both of which declined me outright. Then we tried the only Osteo-Spine surgeon I liked the look of. He met with me a couple of months down the track. Was lovely and had an EDS patient like me 10 years before and referred her to US to Fraser Henderson, the world renowned expert on EDS and CCI. I knew who he was as ive looked as loads of his You Tube lectures. I knew it was out of reach so said, what next? he said go back to your 'community' and find out who everyone else is seeing. I shot him an email and never heard back, I tried the Centeno-Shultz clinic in US and they needed DMX (digital motion xray) which from my knowledge doesnt exist here in NZ. I knew who to ask...

I went home and emailed my specialist hypermobility physio and she said she has worked with a surgeon at the conference last year in Brisbane. I emailed him straight away within a few days I got a reply with a patient registration form and a 5 page questionnaire. I filled out the form, had my Ortho Surgeon as referrer but also attached my GP and Osteo referral letters they had provided. The questionnaire was actually the most thorough I have had to date, i answered it twice and the outcome was interesting. I had never thought of my symptoms as MAJOR or MINOR before, most of the focus being on my cognitive state, memory issues, head and neck pain and dizziness/vertigo. That combo of symptoms are making it impossible to drive, think, focus, remember and I'm dealing with significant overwhelm from light, noise and lack of sleep.

The appointment was booked but had to wait a couple of months for a Zoom appt. Didnt realise that I had to get prior approval from health insurance so financed that myself and I'm glad I did as the report I got after first appt was worth it.

He asked my NZ surgeon to refer me for a MRI of neck and brain. The results came back before my appointment, they said "normal findings" but that was absolutley not the case.

Appointment came around, I was borderline for the Grabb-Oakes score for CCI but I was supine, which told us nothing and the only other was of truely knowing was a shitty upright MRI in Melbourne, Australia, otherwise I would have to be US bound to get the right imagining done. That was not what he wanted to talk to me about though, it was the other two things that were incidental findings. My Empty Cella Syndrome (which turned out to be partial) and possible Internal Jugular Vein Compression and my possible CSF leak. WHAT?

He sent me for more scans, another nearly two hours in an MRI machine, again having to take all my piercings out. I also had a CT Venogram.

I waited about a month for the follow-up appointment. Waited on Zoom for about 2 hours and the call lasted about 10 minutes. He wanted to take my case to an MDT with two colleagues, one is an Intervential Radiologist, the other an ENT surgeon doing some of the only Jugular Vein surgery in Australia.

Two MDT meetings and nearly 4 Zoom appointments later I had a plan for surgery. It is a really good support letter and hope it will be enough to help me secure the funding. I have Meningeal diverticulum in all three areas of my spine (Cervical, Thoracic and Lumbar). 3 largest in my Thoracic are potentially leaking, causing cyclic pressure issues, compounded by Bilateral Severe Internal Jugular Vein compression. Problem is no-one here agrees! This month I go for for a 3rd NZ opinion, this time its a recommended surgeon by my GP. Even before seeing me he wants to do an ICP procedure to confirm my intermittent IIH / CSF leaks / ICPressure. This is invasive and not recommended by my Australian Surgeon. I cannot get to Sydney for surgery unless someone backs me for the HCTP. What to do??

I will hopefully have another whole post to explain the above after the next appointment. I don't REALLY want to be booked into ICU for a week and be awake while someone drills a whole in my skull, but if needs must? To ensure I have a better quality of life and live until my 60's dementia free, I will do anything at this point.

Outpatient on the Eating Disorder Service

I have just had my monthly catch-up with my psychiatrist at the Eating Disorder Service. He was in a great mood but had to cut our session short this month as he had an acute case in Nelson he needed to Zoom in for in 25 mins. I felt for their family and wondered if at any stage he thought I was acute? He said on intake he believed I was on the line between compromised choices and critical illness. You see, I felt like I normally do, not unwell at all, but in a very hard way to explain.

Eating is no longer something I enjoy. It is a chore, it causes me no end of grief and it is now constantly a focus, which for some in ED recovery is not helpful you might say.

My diagnosis has caused me no end of grief, with my family, with my bank, with my work and pre-September 2024, it simply didn't exist. That's the weird part, just the wonder why food was my enemy, the thing I avoided the most. Many doctors have tried to get to the bottom of it, and the strang thing was, it as Anorexia Nevosa all along.

Now what? where to from here? My team at the service have been very patient in working out what my triggers were over these past 8 months (part of the SSCM Im receiving as an outpatient), what I can and can't or would or wouldn't eat. I slowly put on a gram here and there for many weeks, and while small gains were main, some regression happened too. The threat of being an inpatient and being tubed and force fed to gain a kg-a-week is what they use to keep you as an outpatient on a service that is constantly overwhelmed by the acutely ill.

He happily wrote me a medical certificate for work (they have banned me from using my sick leave after it became excessive between my appointments and the childrens), but now it means I have to talk to strangers about my ED, am I ready for that? He said I can continue reading his book and was happy I was finding it helpful, The Balanced Brain: The Science of Mental Health. 

Hard part is while reading it I think I will end up back on the exercycle this week trying to finish it which might undo all my 'hard work' as it called it but I'm almost unable to stay awake through a paragraph, let alone a chapter, so needs must.

The content is similar to what I am learning through UC online's Wellbeing series (Pschology of Wellbeing, Lifestyle & Welling and now Nutrition & Wellbeing).Self-regulation appears to be key in recovery, but how do you regulate the overwhelming need to control your intake to feel a sence of normality? More reading and more study will hopefully help with that. I'm obviously partway there? Even though Im still fighting have I given up?

What I have learnt is stress is a massive factor, not to use that as an excuse, as that is one my Psychiatrist won't accept, it is my normal to retain control of possible binge eating from stress, to plan and restrict. Not only does that allow things to remain in my control, it is also because, I feel better empty and want to keep it that way, having what I need to 'survive', I don't care about the 'thrive' part. I just want to be comfortable, to be able to relax. Food robs me of that.

It was talk like that, the maltrution talking, my starved brain he says, that lead to my time on the service and my AN diagnosis in the first place. I'm not sure anyone actually understands my daily struggle, quite like DH and those in this very isolated and lonely space, my invisible illness, my EDS.

Food is my enemy, the instigater of fear, chronic pain, bloating, fatigue. Why? we don't know, a million reasons, we may never get to the bottom of the real why. I just know the more I do it the more I suffer. With Anorexia Nervosa some see the suffering as a visual thing, the gaunt face, the lack of strength, frailty. 

The real suffering comes from trying to figure out how you got there in the first place! Obviously I am quite complex, there is a huge list of reasons, but on reflection this past week, I think I found my trauma point, the part that my family doesn't think exists or everyone has wrong. 

The time I was dumped by someone I was obsessed with, that I thought wanted me for me and all that came with me. No, I came home one day to my posession in a dark, cold concrete garage, told to sleep there and then get out. Why? It appears because I was no longer wanted, I had put on weight, and when given the choice, 'fat' me was not what he wanted after all. Problem is, I wasn't fat? was it my bloating and distention from severe GI issues and undiagnosed food intolerances and connective tissue disorder? Who knows as it appears I have blocked parts of my adolence out. No, it was probably just regular old 16-17 year old me and all that came with me. From memory I called home and pleaded to come back. That is where I believe it may of started? I will do a whole separate post on the 'forgotten me' at some point.

My psychiatrist tried to tell me this week I looked better? When I questioned why I appeared 'better' and if I appeared to have put on weight, he said no, it was different to that and hard to explain. After many years on the service, he said I was 'present', in a way he couldn't explain but was pleased to see. I think he gave himself a pat on the back for that as I appeared to have a nearly 1kg gain since last month (46.4kgs). He asked if I was ok with that? was he expecting me to say no? Of course I said yes, truth or not.

I was surprised as I had not tried. If anything I thought I had restricted, maintained. Kept to my plan. Did it bother me? I think so? It's hard to tell as I came home and had dinner that night, just a small one. Maybe that was my brains way of saying 'you've got this'? We can do 50kgs easy! (my goal to get my life insurance back). I almost felt normal for a moment, normal thoughts, almost happy to have a positive experience. I appears I was wrong...

Regret. Almost instantly. Pain, fatigue, reflux, more pain. Was it worth it? no, will it pass? who knows. Will I do it again this week, probably not, Is that good for me? Probably not, will it stop me, probably not. These are the reasons I can't explain.

So I'm booked in for next month, will AN get a good hold of me again and I'll present with a loss? or will it be a further gain? All I know is that I'm bumbling around in this for the long-haul. It's a delicate balance between maintaining control and losing it all together, but I think I'm winning. I've been told I'm not being treated for severe and enduring, well not yet anyway? That means there is hope lurking around. That's apparently a good thing.

Distraction from thinking about food all the time is key, hard, but the only way through. Learning also helps. Seeking out the right healthcare for the rest of my chronic illness helps. 

Finishing the book may help? I have my course, my CCI workbooks, I have supposedly got this. Again, time will tell.

Being misunderstood...all the time.

It's been a while since I posted last because to be honest, life is just difficult at the moment.

Stress has been a massive factor. It's amazing to me that I don't feel stressed but others can see the toll its taking on my body and even my soul.

Things are harder now that I'm very underweight. I'm struggling to maintain a weight over 45kgs at present and dealing with all the outcomes that come along with it.

The control that I use to maintain some sense of structure, organised chaos has resulted in me restricting to the point of not eating at all as the more I did the better I felt. Gone is the pain that wakes me from my sleep. The chronic fatigue, the brain fog, the constant severe cramps and bloating. My body felt calm, my energy felt up and it was so easy just to drink my nutrition, but little did I know that the E028, even with the added Calogen (hi calorie additive) was not enough to sustain my lifestyle. Things went downhill from there.

I never thought I would find myself in an assessment for an eating disorder but there I was. I don't see what they see and they don't feel what I feel. I was honest, I filled in the form truthfully and sat calmly in the office with the Psychiatrist and medical student and let my self be assessed.

Anorexia Nervosa. I couldn't believe it. I had sworn it was ARFID, but no, based on my long history of restricting, binging (well I called it stress eating) and purging using laxatives, here I was. Mid-aged and so in control I had made myself ill.

It really didnt sink in until I saw that my liver was showing signs of distress as well as my kidneys and my body was starting to digest it's muscle. Ambulance at the bottom of the cliff. Border between comprimised choices and critical illness. Kgs away from inpatient.

Im currently in a very slooooow recovery process. I swear im being treated for AN-SE (severe enduring) but both my dietician and my psychiatrist have told me nothing thus far.

I have been under the service now for 5 months and have managed to climb slowly up a couple of kgs, to start having a regular breakfast and starting to enjoy eating again. Have the ANTS (Automatic Negative Thoughts) gone? No. Am I progressing as fast as they would like? No. Will they kick me off the service for losing weight? Probably. 

The therapy I am under apparently works the best with adults, its weird to be even getting therapy considering I don't feel unwell enough? 

Specialist supportive clinical management (SSCM) we are hoping will be the slowest and best path forward for now. I realise what the issues were, where I have ended up and where I need to be, there are just too many roadblocks.

Stress

Kyphoscoliosis

GI issues

POTS symptoms

MCAS reactions

Vascular Compressions

All of the above may or may not be contributing to my constant adverse affects from eating. What I have learnt is to eat in small amounts, standing up where I can and at times when I feel hungry or in the right mindset. I need to work, look after the kids, organise my life and I can;t do that when I'm overcome with pain and discomfort and can't think through the fatigue.

I have a cardio appointment in a couple of weeks to check out my POTS symptoms, I am seeing an osteo to address my pain. My family have attended an information evening, I'm reading books about recovery. I'm doing what I can to avoid inpatient admission, mechanical refeeding and heading in the right direction. Im starting workbooks soon on long term recovery, I believe I will get there eventually, I struggle to comprehend how I got here in the first place.

All I know that I am not the only one, I don't feel alone, I feel supported. 

Why is that not enough though. Why can't I just be all that they need me to be. Just like them. Newsflash. That's never going to happen. The only one that understands that the best is DH. Without him I absolutely know I would not be here doing as well as I can be.

EDS is hard work. But I've got this.

Ambulance Trip, Sinus Bradycardia, Stage 3 Kidney Disease and Neutropenia (again)

So it turned out to be quite the eventful month.

Since seeing my Gastroenterologist and weighing in at a measly 50.5kgs (which he was not happy about) things got busy, I got distracted and somehow my weight plummeted to a concerning 44.5kgs. I noticed myself getting weak and slow which was the first real physical symptoms I had that things had changed. Being underweight for so long I had got used to my stats being a little 'lower and slower' than everyone elses. 

One night things really stood out though. As normal, it had been another busy evening and by the time I was ready to sit down for the night, it was time to take my nightly Amtriptyline as normal about 9-10pm. DH and I normally watch a show before I fall asleep/nod off but that evening I felt a bit peaky, which was weird as I had just had a Hydrolyte Iceblock (electrolytes help with my POTS symptoms). I looked at my fitbit, which I never use as a medical device, and my heart rate was 35 bpms. Shocked by that I got up and moved around, I was very light-headed and it might be my blood sugar? I grabbed a handful of blueberries and ate them to see if that made a difference. It would not go higher than the low 40s-late 40s. I sat down again and thought it must be a blip and tried to distract myself with the show but it was having trouble getting back up to 50s. DH was off to bed and I said to him I was concerned about sleep at this point as that is normally when it drops, so off he went and I got out my pulse-oximeter and tried getting a solid reading. It set off the alarms as could not get a proper reading. Normally with my poor circulation it struggles, but it said my blood-ox ought to be low given my pulse rate. I took my blood pressure and that seemed ok, so called Healthline for advice on how to get it back up. She assured me that the equipment should work fine and to put the pulse-ox back on. Alarms again and read out the details to her. She was concerned at how erratic it was so advised me to go to the ED. As I was not allowed to drive myself and DH had to stay with the kids, they called me a non-urgent ambulance who could at least check me out and see if I needed seeing to. I snuck in to tell DH not to fret but we would be getting a visit.

He heard the reversing up the drive in less than 10 mins, they had come on red lights! In a flurry they came straight in to where I was sitting and got out the trace machine and starting hooking me up. DH was approached to try and figure out my meds, I had my dispenser already packed with a go-bag. All of the things that I learnt from having a poorly dad was be prepared always.

Something wasn't right. My heart rate was considered irregular bouncing from the 30s to 70s, to 120, to 180 and back down. My blood pressure was systolic of 134 which is high for me, and DH looked panicked. He was seeing it bounce all over the place. They asked if it was normal for me? Ummm no. They decided to take me in as they were not allowed to leave me at home with that level of concern. Off we went. On reaching ED it had calmed somewhat on the journey and was just low. I was feeling fatigued. 

There was a wait to be seen by a nurse of about an hour. They did a trace and it seemed to be fine, ie no irregular heartbeats. Bloods taken. I was then left in a cubicle for 6 hours and not seen to. No water, nothing. I couldn't sleep due to being right next to the nurses station. All I could hear was my Bradycardia setting off the monitor every time I got close to sleep.

Then came 6am staff handover and another set of obs. They would avoiding checking on me as they thought I was asleep. As if! I was sore from being on the gurney bed all night so sleep was impossible. They did another 12 lead EKG and again no irregular heart. It was a further wee wait for doctors rounds and they finally questioned me on what had happened and general health questions as my bloods had come back with Leukopenia (WBC of 3) and a low eGFR. When we determined there was no immediate danger or sign of infection I was discharged to see my GP for follow up bloods in a week. Diagnosis for admission was: Asymptomatic Sinus Bradycardia (Sinus meaning normal rhythm. That we already knew but good to have it documented. They suggested I was frail and malnutritioned and should be monitored. 

Follow-up bloods scheduled for a week later, turns out my WBC didn't budge and my eGFR dropped even further and my Creatinine levels were high? Concerned my GP suggested it might be an extremely rare allergic reaction to my new GERD medication and to stop it immediately as she thinks it might of caused Interstitial Nephritis. On repeat bloods my levels went up only just slightly to Stage 4 CKD (where I sit normally with my renal agenesis). This is an ongoing saga however and we wont know for sure for another 5 weeks on my follow-up test.

With all of this going on, thought it was a good idea to visit the GP re the hospital visit. After a few ob checks and squat and stand BP reading on 90/60 a referral was made on my Gastros request to the Eating Disorder department of our local hospital. She referred me on a semi-urgent basis due to my symptoms, co-morbidities and declining weight possibly being linked. 

Little does she know it's now sitting at 42.6 as of today. Thank god my referral was accepted onto the waiting list as 'Houston, I think we have a problem'.

My next post will be dietician follow-up. She is my saviour xx

Avoidant / Restrictive Food Intake Disorder (ARFID)

The report from my gastroenterologist came in, as you know, I get copies of all my reports so I am well informed before a follow-up or any additional conversations I have with my referrals as I've spent the better part of 10-12 years being referred from one specialist to the next.

There was one part that stuck out to me. After our conversation at the clinic about what I was and had eaten during the year, what my dietician and I came up with as a plan, he was shocked to hear I was on next to no solid food and relying heavily on the E028 nutrition. The conversation then turned to my restricting and any problems it is causing me and might cause in the future. "I feel better when I don't eat" was not something he was expecting. I have reduced pain, better sleep, more energy, less fatigue and reduced IMO/GERD symptoms. There it was written into the report, could it be ARFID? Wow, someone finally said it, could it be an eating disorder? I prefer the term disordered eating to be honest. It was time to start the discussion I suppose, but of course I was reluctant to admit that I might have a DSM-5 Mental Health condition that was causing my symptoms?? Something functional he called it. After much research he was possibly right? How could that be? 

This topic may feature quite heavily in the next few posts, so here is a quick rundown of what ARFID is and how its diagnosed.

Esophageal Dysmotility? Barium Swallow and EAST testing

My GP and I were slightly perplexed as to why eating is such an issue for me at present. It causes me a great deal of discomfort and fatigue, but we are running out of things to explore for answers. I was referred back to my Gastro for further investigations. Part of my EDS diagnosis was to both get an understanding how long term it would affect all of my connective tissues and stroma, so it was key to find out if swallowing had any muscular dysfunction attached. 

There were 3 options, barium swallow, manometry or Acid ph testing.

He said while the barium swallow may not give us much, it would give an indication if further tests were needed.

My gastro ran a basic allergy panel to check for sure if I was allergic to any of the classics like dairy, wheat, egg, fish, shellfish, nuts. All came back normal, so much for the wheat and dairy free testing back in 2006. That started a long journey of trial and error which ended up at FODMAPS, which is where the the restricting began. Go figure.

To be honest the barium swallow x-ray was probably my second worse test to date. The Barium is really thick and gritty and causes instant reflux. The whole test did not take long and I was able to get instant answers from the doctor who performed it. The clincher was whether or not I could swallow the marshmallow after only a few bites. Who knew my fate would come down to a marshmallow. Success! Was happy to hear it all went well and there are no issues with my oesophagus musculature or swallowing.

Results went back to my GP and Gastro. The wait for a reply began. 

Meanwhile I had decided to stick to my liquid diet and try to add a few more options to it, little did I know that would be my undoing.

GYN & Vascular re-visit

Meeting up with my GYN again after a year was interesting this time around as she wasn't expecting the news I had for her when I met with my vascular surgeon. Finally an answer for my pelvic congestion syndrome and more information for us to discuss. Soon we will need to make a decision re a hysterectomy, we both want my uterus out so we can finally confirm if I have Bicornuate Bicollis, but as my AMH is still way high, I'm still pretty far from being in the position to be able to do that without HRT which is scarce to find here in NZ at present.

I discussed with her the possibility and she is really reluctant to remove it I enter menopause properly, I'm in perimenopause due to my ovary removal. Also until we had a greater understanding of what my blood flow would be, so back to the vascular group for answers. It turns out that compensatory flow is a thing and it would find a way round, believe it or not the removal of my uterus would actually improve my pelvic congestion permanently but we won't know until we go there.

It's a tough decision as I obviously want to be rid of any of the problems it gives me, but I also will still have a 'cycle' and all the problems that go along with that, my Adenomyosis would have to get a lot worse to get to the stage of operating, so looks like checking yearly is my reality for now, just another surgeon in a holding pattern until things naturally decline.

As part of my long-term care plan, I want the decision to be based on best outcomes and willing to trust the experts and wait and see... I'm sure around July this year, I will have a re-visit and update on this.