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... 

Bowel MRI with contrast / breathing protocols

Well, wasn't expecting that!

It seems to just get funnier by the minute. It was just a routine appointment to discuss my results of breath testing but my Gastroenterologist decided to do a physical exam as part of my appointment. He described my abdominal pain as 'diffuse' and listened to my GI sounds with a stethoscope., it seems he might of heard an abdominal bruit and had an idea? It wasn't till I spotted he had referred to it in the clinical notes after the MRI that I realised it might actually be true!

I might have a MALS. Median Arcuate Ligament Syndrome, otherwise known as Celiac Artery Compression Syndrome, Celiac Axis Syndrome or Dunbar Syndrome.

I had heard of this condition before as a few of my EDS group have battled severe cases and had a rough journey leading to major surgeries. Wasn't expecting to read this in the outcomes of my MRI though. I did wonder why I was doinf breathing protocols however.

Apparent narrowing of proximal coeliac trunk raises the possibility of median acute ligament syndrome, however accurate assessment is limited due to spatial resolution and non-dedicated sequence. If required, ultrasound can be considered to further evaluate.

So Tuesday im off for a Doppler Ultrasound with more breathing protocols to test the pressure of blood flow and presumably check the blood flow to the organs that may be affected which are a portion of my stomach, my spleen and my liver. I do also hope to confirm if my transverse colon is in fact in my lower abdomen which apparently is called a U-shape. This would explain the very low pain I get a few hours after eating. I'm sure all will be revealed once they have the whole picture. I cant for the life of my think what other scans they could do that they haven't already but apparently there are a few.

Update to come with results from my Ultrasound. Hoping that its conclusive and I don't have to spend more time waiting for a result. Even so, the only fix is surgery, laparoscopic or open... and finding a specialised vascular surgeon, so really i'm hoping its a no, but prepared for a yes. There is really nothing else that could possible top an artery compression for me but suppose its early days yet.

IMO, Fructose Malbsorption and Digestive Testing

I've spent the last month or so waiting to see a Gastroenterologist for his once a month clinic. He reported on my breath tests which he met with me about this week.

So it wasn't SIBO in the end. I'm feeling pretty thankful about that but he has confirmed that I do in fact have Intestinal Methanogean Overgrowth. 

My results also showed that I malabsorb Fructose. This has been the biggest shock as all this time I thought it was intolerance not malabsorption. Turns out im also not intolerant to Lactose, which is hilarious to me, that this whole time fruit and vegetables have been the enemy! Going vegan was the worst thing I could have done as all that coconut was doing me in. Pretty annoyed as the list of Fodmap, low acid and low fructose is getting smaller by the minute. The elemental drinks might be here to stay and replacing a few more meals once I see the dietitian again. 

The Gastroenterologist was concerned regarding my low weight. What? Hang on a minute! I've gained 6 kgs since I finished my breath testing. 45kgs was low... this is normal. I will have to watch that I don't shed too much now I have the energy to do a bit more exercise and housework. Now that spring is here and my garden is needing me Im definitely burning more calories than Im consuming, its a constant balancing act. Im just thankful that Im finding out now as I could of battled on for a while not knowing.

Outcomes of meeting? We talked about slow transit causing the IMO or IMO causing the slow transit? So he has referred me for a Colonic Transit Test. This is thus far the most interesting test so far! I have to swallow little bits of metal that will show up on an xray later on. He is positive the test will come back normal as most of his EDS patients have normal tests, there is very few that come back with Gastroparesis or Slow Transit confirmed. These are both rare and mine seems to be more of hopefully a curable IBS-C than a Functional Constipation. He also ordered a small bowel MRI as my anatomy is somewhat complicated, so ruling out there is any obstruction there too. Once we know for sure I can decide if I take the $1k worth of IMO antibiotics (56 over a 14 day period), a combination of Rifaximin and Neomycin. 

I've pretty much searched every possible corner of the net and looked for books on how to improve my diet from here and its not looking good. It's just a waiting game now. Update to come with MRI results.

When my normal test results weren't in fact normal

Endoscopy #2. 

It was touch and go that week as the whole house fell crook with a coughing virus and I thought surely being off all my pills (was asked to stop all supplements prior so have literally been off them since my breath testing (Vit B Complex, Vit C, Magnesium). I took a few sneaky Vit Cs and hoped for the best. The day came and I was snot and cough free so off I went.

It was later in the day so I decided to bus there. Thought if I was going to be laid up for the afternoon I ought to get some exercise. I masked up and got through town in good time and arrived at the clinic 15 mins early.

They took me fairly promptly and I did my pre-procedure stuff, got changed, toilet stop and waited... what seemed like a while, until I was called in. Nurses were still getting set up and I answered a few questions. They said "you were here not long ago?". Correct! They had found something which looked a bit sinister in my distal esophagus and I was there for another biopsy to see if we could figure it all out.

The nurse had trouble finding a vein in my arm and after sticking the needle into my nerve decided to give up and wait for my specialist to insert a cannula in my hand. They commented on the colour of my hands and again said "your veins are so delicate". Of course they are! ive been food and fluid free for 12 hours... dehydration not helpful. I explained my circulation issue and reassured them my hands were ok, they were just cold which makes them go purple. 

My specialist arrived and asked how I had been? I said doing ok as I had been to dietician for a chat, we had a plan and I was slowly working on getting some weight back on. I advised him that my throat was pretty ok after the last biopsies and that I was looking forward to an update of where we were at. 

After a very painful few minutes he had a cannula in my hand and we were off. Little did I know that later than cannula would be snagged and its removal caused a blown vein which took a good week to sort itself out.

Meds on board and throat spray in I drifted off and remember less about this one... and woke up being taken to recovery. There I felt really slow. I could hear the machine and new my bradycardia was playing up and that my blood pressure was low as even lying down I was dizzy. On my last reading before I mobilised I asked her what it was sitting at and it was about 91/62 which is low but pretty standard for me. I took my time getting dressed and only fell over once, luckily I was close to the ground trying to get on my converse boots.

Once next door and my throat had woken up I was served a vegetarian dairy and gluten free sandwich and to my delight a Kombucha, even my favourite flavour. The nurse came and read my results which was the ulcer had mostly healed, things looked healthier and he took more biopsies of the nodules. He was happy with what he saw, so that in itself was good news for me.

Its been a about 1 week since the procedure and to my surprise my results came in via GP. It seems that my distal esophagus had sustained injury from what appears to be gastro-oesophageal-reflux. I had a sneaky suspicion that was the cause even though my surgeon had ruled it out in the first instance.

So, where to from here? Looks like i'm back to the dietitian to think of a new plan and probably scripted more Elemental Drinks as the list of foods I can consume is growing smaller by the minute. I never thought there would come a time when I would not be allowed chocolate, soda or coffee. 

I see the new Gastroenterologist in 4 weeks time to go over my results from my breath tests and to talk about motility. I think that's definitely going to be an issue relating to my EDS and also now by most recent reflux situation. I've already started to make improvements to my diet (cutting out the stress eating, the chocolate, x1 coffee a day and really trying hard to stick to the Low FODMAP, low Fructose diet now it seems I need to work on the Reflux stuff too. Crazy though as I was hunting high and low but I found a book! The Low Fodmap Acid Reflux diet, so Im kind of feeling like there is light at the end of the tunnel. My E028 (elemental supplementation) seems to be going ok, the problem is I actually prefer the drinks to eating, I was having them for breakfast and lunch but finding i was still hungry after tea so mixing it up starting tomorrow and going back on my low fodmap cereal early morning and supplementing lunch and maybe an after tea, or second one in the afternoon... i'll see where I need it the most. 

Lots of reading, lots of research... and lots of waiting. So the story goes...