At the end of my rope with nausea, vomiting, and malnutrition.

I would ask to be put on a drip to get nutrients into you.  I wouldn't worry about not getting a diagnosis; worry will make you feel worse. Wait to see what your specialist says in November.  With the number of conditions you have, any one of them could be the cause of your current symptoms.  EDS can cause gut problems on its own.

I don't remember if I mentioned, and I can't find it in my post, but I also have very frequent heartburn, and plenty of colon/intestinal and bladder issues we're still trying to work out. (Even when I'm in the hospital for weeks or months and on constant fluids, I only urinate once every 40-60+ hours and don't feel like I have to at all until it's an emergency. I've tried going on a schedule but nothing happens if I don't feel like I have to go. Bowel movements are the same; once every 7-10 days no matter how much or little I'm eating, odorless, often mucus-y, often clogs the frickin toilet, always breaks skin coming out and a few times recently there's been blood that's browner than the bright red torn-anus blood. Tried fiberous foods and supplements a while ago and all I learned was that any amount of extra fiber (or just particularly fibrous foods) gives me explosive diarrhea all day. But we've come to agree that the constipation is NOT causing the nausea/vomiting as this started two years after the nausea and vomiting did and things that did temporarily improve the intestinal issues did not result in any help with my stomach, so that's been a source of frustration as well. I was a very infrequent urinator all my life though, though it was always once a day, not once every 2-3 days. (My mom has brought up that she once asked me in first grade when I started a new school "how are the bathrooms?" and I replied "I don't know; I don't go during the day." And she replied "but you don't go in the morning before school either.." to which I replied confidently, "Because I go at night before bed!" And her response was "you go ONCE!?" and that's when I learned that that's not normal.....but we never looked into it because of money. And I drank two bottles of water at school or more every day, and drank in the morning, and with every meal at home, so back then it definitely was not dehydration. One of the many mysteries of my childhood that makes a lot more sense now than they did back then. 

I don't know if any of that is relevant but figured it wouldn't hurt to include. 

(Also my breath and for some reason my tears always smell like sulfur, so idk what's up with that. It made my old GI insist the issue is gastroparesis, which everyone is telling me now seems most likely especially because of my EDS and possible mitochondrial disease, but the old GES was fine. Maybe now it would be different but if I throw up what I eat how will we be able to test for it. 

OH and contrast tests are a no-go because of a shellfish allergy so please keep that in mind. (I'm allergic to so many things and intolerant of so much besides; it really limits what I can even try if eating itself weren't an issue. And then the mast cell disorder doubles the problem. Nothing can be easy, it seems.) 

Horrible condition! OMG.

Many things you stated are linked to each other, hEDS sucks greatly when it manifests in other organs, not 'only' joints.

Since also blood vessels are easier compressed, everything just lax, ligaments inside not holding as they should,

was 

SMAS and MALS looked at thoroughly and excluded?

For MALS the best way is a doppler ultrasound of the celiac artery, in expiration supine/standing and inspiration. Don't worry, my daughter couldn't stand either, she just sat fully upright, (yet was not fully supine due to positional induced vomiting). Yet....you need a doc, who knows what he is doing. 

We are in Australia and pretty 'stuffed'.

Technicians -who do not even note the physical position of 'not fully supine' (supine has the higher pressure = velocities, if that was the case)-forwarding the ultrasound pics, this is not good enough for MALS diagnosis. (My daughter had high velocities supine expiratory, above the 200cm/sec cut off, but since oh dear no kink could be seen, dismissed as 'most likely insignificant'. thanks. This teenager has no arteriosclerosis to explain high velocities..... We will fly to Europe.....if we can somehow.)

Sometimes it is not the blood vessel that is suppressed (and causes vomiting and nausea), it is the same diaphragm  ligament cutting into the coeliac plexus nerves that can cause havoc.

I am also there now to find a pain specialist or whoever to trial a celiac plexus nerve block, this is diagnostic if it helped symptoms or not. (quite a thing to do, they go from 'back spine' to front stomach area with needle, but one is desperate)

For MALS and SMAS an MRI with sagittal plane would be great (yet we had an 'abdominal' MRI, but no sagittal plane as the question was 'abdominal pain' and they just look for tumours, volvulus, the big obvious....so even it came back 'normal', there might be something up. Always depends what indication was.

On our list is also a head MRI, to see the posterior fossa, where the vomiting center lies. (braces yet don't allow that harmless imaging yet)

My girl also suffers abdominal continuous cramping pain in one spot (appendix is gone and no ovarian cysts), colonic inertia and has some hyper mobility (some call it hEDS, others refuse to call it that way since only 5/9 Beighton score) and there is nothing that seems to help.

Her colon is also far too long. Visibly far too long, 'elongated, tortuous'.

We have looked at a full faecal microbic assessment and found nothing interesting. Yet we add Clostridium butyricum (as no eubacteria were found, which produce butyrate = anti inflammatory) and Lacto LGG and boulardii to our daily regime.

There are some hEDS supplement regimes and some find them helpful (we didn't).

We have started 'electrostimulation' therapy for colon stimulation as even a too long colon would be no problem....if it worked (had nuclear transit study, all far too slow)

(4 electrodes diagonally placed, 45minutes in a certain setting program 4 on that TENS machine) with physiotherapist, who works together with a GI doc. It should tell in ..... 3 months if it worked.

Other GI docs have given up. "Celiac ruled out, diets tried, sugar breath tests negative, scopes done, appendix out. What do you want? Just keep Movicol up."

My girl has gastroparesis proven though (milk and egg).

I am sick of diary free, gluten free, grain free....nothing helped.

A leaky gut could cause a lot of intolerances. (We seem to have none, fructose and the likes no problem, also in colon biopsies those responding enzymes were all really there in abundance)

So is there a way to address the gut's health? With probiotics and the likes.

We have to be low fibre due to gastroparesis, but others don't have to and can add 'probiotics'. ? 

And oh wonder, one does not get certain colon motility drugs, why not?

Did you ever get any?

I plea and plea and nope, never any prucalopride, linaclotide and the likes tried.

I will not use hydragogues like Senna, bisacodyl and Aloe Vera on a daily basis. They can damage the colon nerves in long term. 

We do use magnesium citrate and Movicol, but it doesn't cut it.

and "lucky" you get the Fentanyl patches, we were told to have to stop opioids after few months (that were given after a year of intense pain and missing school) soon and her abdominal pain is so bad without, stopping due to 'effect on brain' or 'getting used to it'....but there seems no other option.

We can only choose between cholera or the plague.

I tell that to everyone who says 'omg, your teenager is taking tapendadol'. Yes, and so thankful for it, would actually need it twice a day, not only once, and not because of being used to it, because from the start only covering 10 hours and would have needed more from the start. Sleep become very much impossible for longer than 20min and never relaxing.

And yes, one didn't get the side effects of the oh so slowing down opioids before taking them.

Can't stand that argument anymore either. We know if something got worse and yet, there are compensatory anti-opioid constipation effect drugs out there.

We also take low dose Amitryptiline as an abdominal pain helper (yet, TCA do tend to rather constipate yet again)

We were recently told to take anti-depressants (SSRI) Fluoxetine, apparently doc thinks it can do something for motility (?), but it is not proven for that off label really if you try to find studies. The placebo just the same.

In general SSRI could hang more on diarrhoea side and TCA more on constipation side.

But it is too little evidence to use it for that indication.

Also with Amitryptiline and Ondansetrone (my girl takes 2x8mg per day, highly expensive, but can't stand nausea otherwise), this is another serotonin effect with SSRI and could get too much.

....

No one gives TPN quickly (the vein nutrition), I asked for it often, when nausea vomiting were so bad, that she went on retching after ridding of food and couldn't tolerate any sugar drinks anymore. One has to be passing out before they get it at the ER.....I am so over the ER, that we simply stay at home even she reeks of ketone, try to give her frozen icy poles and electrolyte sticks and water simply to survive the worst days even an into vein hydration, not even nutrition, would be best. So we are back to freezing electrolyte liquids and put a bit of glucose in drinks, if she can't handle any food. It comes and goes in waves re severeness.

Currently can handle cottage cheese! (actually was THE life saver), milk, apple puree.

But she tries everything as her appetite is not bad currently...and it comes simply back up.

We also ordered a full nutritional drink, via UK.

It's like tube food, but just not administered via tube.

I asked my GI here if he could allow it (as that company does not sell it in Australia without doc permission, duh), and he said 'just keep veggies and chicken meat up'. Hugh? Not getting it, are you, she lost 10kg (20pounds) in this year, she can't 'just' eat.

So we self help where we can

and I can't wait to press all the dismissive docs a final diagnosis into their dismissive faces.

'just needs more physio'. Have you heard that before?

Ah btw, there is a good hEDS physiological therapy book and need to work with physio together apparently.

Living Life to the Fullest with Ehlers-Danlos Syndrome.

I doubt it will do anything for colonic inertia or nausea...... but for joints.?

Was NJ tube ever tried or suggested? (prior GJ through skin) An NG makes no sense. Don't let them put a tube into stomach, that rebells, it needs to lie in duodenum or jejunum. We don't need those useless trials when vomiting is an issue already.

All all the best,

and yes, we found a lot of info and doc suggestions via other social media. You might want to read and ask away there, too.

 

oh dear, 'moderated', might not get through.

Send you pm.

BTW MTHFR depends which mutation (location) and if hetero- or homozygous mutation at that location.

If you had a

MTHFR A1298C heterozygot (only one, not both bases different) mutation,

it would be very common (40% of us) and usually no problem in VitB household.

Please look into low stomach acid. My gastritis, acid reflux, and many food sensitivities stems from low stomach acid, not high stomach acid. I hope this helps.