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.

 

Lap. Surgery and Recovery

My follow-up with my new specialist was probably the most informative appointment I have had in a long time. He was my 15 specialist and had only just met me but had taken the time to study my file and really look at my case.

Not only did he understand my conditions he had a plan for anything he thought might adventuate and had a plan. We talked at length about the cyst, it's location, the risks and what might happen if he found anything in there he didnt like the look of.

Surgery was booked that following week once I got prior approval sorted and filled in all my paperwork. 17th July was the day, first day back at school for the girls and a really busy week at work, but we made it work.

I had to go off all my supplements two weeks before so decided also to go nil-by-mouth also for those two weeks to assist my body with its pains levels leading up to the surgery. Best thing I ever did as was ready and comfortable on the day. I only really shed about 500-700g which is good for me. I had weighed in at a whopping 51.6Kgs at pre-op appoinment... which was a win!

I arrived with DH to what looked like a hotel suite. It was a lovely room with and ensuite, with all I needed, and a great view as it was a nice sunny day in the middle of winter. I got settled and DH went and got a few trashy mags so we would have a distraction for the hour wait for the surgeon, anaethestist and the nurses to turn up to cannulise me and do my obs/covid test.

After chatting with the surgeon again re the plan, I gowned up, stockings on and jumped into bed. The anaethestist had his chat and then we were ready to go. Fraser walked me to the lift and then off to theatre we went. 

It is always a surreal feeling climbing from the gurney onto the operating table, there are several people hovering about chatting with you (this time about my tattoo), when the other half are very serious and getting ready to put you under. Seds in my arm, mask on, night night.

Woke up to a very busy recovery ward, they avoided giving me tramadol this time and think that really helped as i sleepily ate my iceblock and struggled to keep my eyes open. It wasn't long till I was heading up to my room again and DH was waiting for me. He had heard the news before I. Just a cyst he thinks, was dangling there ready to be removed and was easy-peasy. No sign of Endo at this stage that he had to deal with. Appendix in normal place. Couldnt of been better news really.

I was soo sleepy I managed to ask DH about 3 times what he had for lunch even though I knew the answer. We visited for a while and they got me comfortable and medicated and dosed while DH left to go sort kids. 

Dinner time came and went for the other patients on the ward. I waited until my drip bag had finished before asking for one of my nutritional drinks, just to spread out my fluids as getting up for a loo stop was not top of my list for then... but eventually managed to get up on my own and go, which is a first, normally I would have waited hours to attempt. I put on my nightie and fresh knickers and boy did that feel good.

I watched a little telly to wake myself up a bit then my Mum came to visit. I was lovely just to sit and chat with her, we don't often get the time. I knew it must be getting on a bit so we vid-called the girls to say goodnight and re-assure them I was ok. I've been using this as a learning experience for them as sometimes you have to do things that are hard, but you are stronger than you think. Hope it gives them some resilence when it comes to their own health journeys one day.

The night was long, interrupted and painful. You always look forward to daytime, especially when you know your family will be there soon, its lonely in a hospital room when you are feeling poorly. 

It went so well it could of been a day surgery but due to my complex history they like to keep me in overnight. Being home is defintely harder to recover with constant jobs to do and children needing you. Blood pressure still a bit low but keeping up my fluids. Pain level mostly under control but happy to have some pain if it means not taking Tramadol. It's hard for me to rest, but I'm just doing my best.

Follow up with surgeon in 4 weeks time, pathology report still to come and photos to receive of the operation. I will post when I have more information about outcomes.

Next week I'm meeting with my OB/GYN to discuss my dilated ovarian vein and my stuck ovary, see what the long term plan is for that. That will be a whole other blog post...

To be continued...

Peritoneal Inclusion Cysts & Liver MRIs??

Just when you thought things could not get any weirder then came the cyst.

Its pretty common for every scan I have to find something else and its pretty common for anyone of my age group to have random finds on their scans, but when one follows another and they become known, then we have to make decisions and deal with things.

So, there is a cystic type structure adjacent and just behind my liver which appears to be a benign cyst but they are not entirely sure. Its gradually growing in size, only a bout 2-3mm in 3 months but that is enough to be curious about what it is and why its there. They believe its a Peritoneal inclusion cyst either caused my my Endometriosis, my previously existing Ectopic Ovary or scar tissue from my surgery.

Who would of though removing what could turn into something sinister would cause something that might turn into something sinister.

So Im off for a Liver MRI with contrast on Tuesday coming to find out if we can get to it or its staying put. While the thought of someone pushing my liver around to disect and drain my cyst and then hoping for the best scares the crap out of me I now know thats the reason for some of my rib and back pain after pushing on it sent me into a tail-spin. I wonder what else they will find on this adventure?

Watch this space for the next installment....