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....

Facing a world without food

I have never felt better than what I have when I stopped eating altogether. I never wanted food to be the enemy but its starting to feel that way.

I was shocked to think all the time I have been trying to navigate my way through the fodmap diet and unaware of my fructose malabsorption and my IMO I was unknowingly contributing to my world of pain. 

Surviving only on E028s and Hydralytes Ive been very surprised at how great I feel, how much energy I have and I am sleeping better. I could of done this years ago. The biggest concern we had was maintaining weight but my regular consumption of plant-based chocolate which is my stress-eating go to, has meant that even thought it sets of my GERD its helped me to stay above 50 kgs even while my drinks ran out and I waited for my next dietician appointment.

My dietician is the most caring, compassionate and smart person I have ever met and even though I have seen a few, she is indeed my favourite. I be understood is everything. She understands my intense restriction, she understands my dear of eating and she understands why I need to keep things like chocolate and coffee in my life... as they can't take it all just yet. If my nodules get worse and my osephageous affected, it might be time, but not just yet.

We have decided to carry on as we are until we get more answers about the rest of my new discoveries. She upped my E028s to 3-4 a day and now im going to try little snacks of my soluble foods like eggs, rice, bananas etc. Only if I feel like eating solids otherwise Im solely on a liquid diet as I enjoy other drinks like Kombucha and Soda & Lime. I even found a 0% alcohol beer that didnt send me off the deep end... so if I want to go out, I can feel that little bit more normal.

Whilst it doesnt feel normal for the family, its working for me. I have to keep an eye on my kidney whilst I loose too many fluids and esophageous for now eating all my no-no foods but its sounding pretty good that Im not having to be tube fed, so I need to lay off the chocolate and coffee and find a better way of dealing with my stress in the meantime. 

A future with Vascular Compression Syndromes...

I met with my 15th specialist. This time a Vascular Surgeon that specialises in transplant surgery. 

Can you even believe that, that is 5 more specialists than 1-a-year for the past 10 years of this blog, and my journey.

He was a very nice man that seemed to find my results a bit challenging as even though he has probably seen it all, he probably hasn't seen it all happening to the one person. He called me 'complex', as they all do. I've decided that is my next tattoo.

We straight away got into my reason for meeting with him today and that was to discuss the possibility I have a specific compression called MALS. It turns out I do not. I have what he thinks is abnormal stroma (connective tissue holding my organs in place) and blames my Ehlers-Danlos Syndrome for growing that way. He believes that its a stenosis that will continue to possibly occlude eventually and there is no way of fixing it. We have to just watch out for aneurym and see what my body does in the way of bridging or redirecting, and if Im symptomatic at all once it happens. I have never in my life disliked a 'wait and see' more but when he said operating on me would be a mess and more than likely make it worse or me more symptomatic, I agreed that a wait and see approach was a much safer option. I brought up the sonagraphers opinions on my left ovarian vein and my pelvic congestion syndrome and he immediately went to my CT / MRI with contrast that I have had previously done and there he pointed out a clear indication of Nutcraker Syndrome and said thats most likely causing my varicosies in my pelvis. Again, watch for symptoms. As I more than likely have the trificta we will watch out for addition signs in my legs of May-Thurner Syndrome and that will probably be diagnosed in due course. I will book a separate appointment about that at a later date.

So there you have it. Is it the answer to my GI pain? He doesn't think so. Is it a contributor to my other symptoms? Pain, posture, circulation, breathing, POTS? We will see. Most worried really about the NCS affecting the artery of my only kidney so following up on that one in late July with my OB/GYN and possibily with my Uroligist. Lets hope I dont add a Nephrologist to that list.

I visited my GP to go through the results and she was a little surprised but not surprised at the same time, this has been as much of a journey for her. We decided without a diagnosis of MALS, we would call it 'celiac artery stenosis'... and list them as VCS on my surgery or medical forms moving forward.

"VCS - Vascular compression syndromes are a group of conditions that occur when a person's blood vessels are under abnormal pressure, limiting the size of the blood vessel and the amount of blood that flows through it."

So more updates to come once I navigate this new area of my anatomy. I'm now part of another rare disorder group and many of them are part of my EDS community but we each have our own battles and stories, so meeting with some soon to find out how they are getting on.

Onwards and upwards.

My E028 experience

I'm not sure when I decided to give it a go but once I started it was hard to stop. I decided to reduce and replace what little meals I was having with my E028 elemental drinks as I wasn't feeling like x2 a day when I was eating breakfast and dinner. It was a slippery slope to not eating at all.

It was recently figured out that my system has such a delayed transit that I literally feel like I'm digesting a meal for 48 hours. That means if I was to eat a large serving, like going out for dinner and ordering a normal main meal, its likely I wont feel like eating for a few days. I believe that is almost why I don't feel hunger. I was eating quite a high fibre breakfast that by the time I was ready to pass it my poor system was exhausting from shoveling it through my system and to be honest sometimes it was pretty brutal when i finally got to the end of the tunnel. Not much digesting happens which also isn't helpful or comfortable.

So when my truck full of Elementals arrived and I figured out I had nowhere really to store a 3 month supply, I decided to up my 2 to 3 a day and replace breakfast, lunch and dinner with them. In between I would continue to drink my normal and I've been adding 1-2 Hydralite ice blocks into the mix which helps with my POTS symptoms a lot.

First few days of getting used to feeling the hunger was ok as I had been on a restrictive diet before. I had also been stress eating Oat Milk chocolate in the evenings which had become a bit of a bad habit... but that didn't stop my body going from one extreme to the other, so what used to be chronic constipation was now chronic diarrhea. That wasn't really a problem as I work from home but did kind of limit the time I spent out of the house. I did almost feel like it was triggered by the consumption of the drinks, which seemed similar to Dumping Syndrome so just put it down to my body getting used to the drinks and that I just needed to ride it out.

It had affected my POTs, my energy levels, my weight and now was affecting my bowels. I persevered though knowing that it would be worth getting through the week and I never thought once I hit the end of week one that I would be feeling so damn good! That was the problem as you become addicted to that feeling, the one of restricting, as you have no pain, no reflux (well apart from that caused by the drinks and the chocolate of course).

Eating my first meal in a few weeks was when I tested positive for Covid this past week. I ate a small bowel of cereal, had my drink for lunch and then had brocolli, carrot and rice for dinner. Within a few hours I was already in pain and being my first 48hrs of Covid was hard to figure out if the chest pain and back pain I was experiencing was food or covid related? It takes me 4 days to 'process' a meal so its now Saturday after 2 days back on my drinks and I've passed a few more solid stools but biggest thing ive noticed is the reduction in stomach cramps... a more settled sleep and fatigue levels are no worse.

Where does this leave me with eating? Don't get me wrong, I REALLY miss food, healthy food...and like I said I'm still eating here and there with a bit (sometimes too much) chocolate but that cant continue due to the sugar content, which I've heard can be a significant trigger for Dumping.

My latest blood tests said everything looks perfect apart from extremely high B12, so Ive taken out my morning B-Complex for now to give that time to drop and see if that was contributing to my spike. It may have caused the loose bowel motions, which in turn has seen my kidney function EGFR drop from 89-68. The doc thinks its the loss of the water in my system so just need to keep up my fluids and look for kidney damage signs.

I see the surgeon one week Monday so should have some answers about what next for my vascular system. Its only a 30 min appointment so Im expecting him to either allocate a few more tests my way or try and dismiss my symptoms and tell me its no major and to just carry on.

Carry on restricting? carry on losing weight? It was scary getting down to 45kgs but I felt so good? Its a slippery slope I've slid down before and I know I'll slide down again, and I'm worried this time I would let it consume me. To be honest it's a constant daily battle that no-one quite understands... unless they understand of course. I'm thankful to have a few of those people to talk to if needed as its a really lonely journey... but there is always a positive, a light. Mine is I feel good enough to play with the kids, to exercise, to stay awake long enough to get my jobs done... to have the energy to work a 40 hour week.

Thank goodness for E028's right now, thank goodness my dietician heard me, truly heard me this time...I'm not sure I would be around long without them.

Doppler Flow US, what will happen next?

I rocked up to my Ultrasound not knowing what to expect. I had waited about a week after my Gastro appointment to even book it as it needed to be protocolled due to being a specialist scan. Turns out my sister knows half the radiographers in town so I took a guess and contacted a good friend and yes it was her husband doing the protocolling. I finally got a text and booked in the scan.

It was him doing the scan too! I felt relaxed and in good hands, which you want if you are doing breathing protocols. Not that I get nervous at any of my scans, Ive literally been scanned and tested every-which-way no nothing phases me anymore.

We chatted briefly about why I was here and outcomes of my MRI. He then explained the test and made a start. Straight away he said "...ummm didst take me long to spot the compression... and he turned the screen to show me. Wow that was one thin artery with a bulge at the end.""I can see if without asking you to breath..." he went on to say, so that mad us both think it was permanently damaged? The important part of the test was to measure the PSV (peak systolic velocities) which would tell them the level of compression but they are calling mine stenosis.

After checking my SMA which was the next concern on the list he went on to check my Ovarian Artery after telling him about my pelvic congestion syndrome, and he said it was slightly enlarged, makes totally sense to me. 

Here were his findings.

Findings:
Aorta PSV 99cm/s

There is narrowing/compression/kinking of proximal Coeliac axis in
all phases of respiration diameter 3mm. Distal to compression artery
measured 8.6mm., Post similar dilatation

There were increased PSV through this narrowing in all phases of
respiration.
Inspiration PSV 258cm/s
Expiration PSV 329cm/s

SMA diameter 5mm, PSV 160cm/s.

Conclusion:
Pronounced velocity increases through the proximal coeliac artery
with morphology supporting marked stenosis and the previous MRI
suspicion.

Who could have possibly thought... its been MALS all along?

Well 2023, what an eventful year it is going to be, it wasn't till I posted my current diagnosis list on my new FB community group that I realised the gravity of the situation...

GERD (10 nodules and 1 ulcer in my distal esophagus, scar tissue)
Peritoneal Inclusion Cyst
Adhesions on my left ovary (second time)
Moderately Slow Bowel Transit
Intestinal Methanogean Overgrowth (IMO)
Fructose Malabsorption
Mast Cell Activation Syndrome
Postural Orthostatic Tachycardia Syndrome (POTS)
Acrocyanosis
Hypermobile Ehlers-Danlos Syndrome
Fibromyalgia

This is what I have had diagnosed with prior to that in the 9 years after looking into my health pre-IVF:

IBS-C
Endometriosis (deep infitrating on only ureter)
Renal Agenesis
Unicornuate Uterus / Bicournuate Bicollis (they cant decide which it is till I get a Hysterectomy)
Ectopic Right Streak ovary next to my liver
Pelvic Congestion Syndrome
Hydrosalphinx
Scoliosis
Spondolysisthesis
Sciatica
Occular Migraines
Hypothyroidism
Asthma/Hayfever
Systolic Ejection Murmur (congential)

Ive been under a total of 12 specialists, soon to be 13... now adding Vascular to the list next month. 4 gynecologists, 2 gastroenterologists, 1 colorectal surgeon, 1 ortho/spine surgeon, one Hand & Wrist surgeon, 2 Rheumatologists and 1 Urologist.

None of them thought about MALS (possibly soon to be my next diagnosis) or even picked it up on the 20+ scans/xrays/US ive had.

I emailed my EDS specialist physio and she said I was her 10th client to contact her with a compression syndrome. It's very common with hEDS, one of her clients having all 5 compression syndromes. The others of concern for me at the moment are Nutcracker Syndrome and May-Thurner Syndrome, I show symptoms of these and could easily have been dealing with those all my life. I will post more about those after my appointment with the vascular team.

Next update will be a lengthy one...