End stage autonomic polyneuropathy

Hi @cheyne, I can't imagine how difficult it is to deal with your symptoms and then having your doctors not helping you find a treatment or management strategy. If I'm understanding correctly it sounds much like the autonomic polyneuropathy has you dealing with an extreme case of gastroparesis. Mayo Clinic has some information here including treatments under study (gastric electrical stimulation) that might provide some help - https://www.mayoclinic.org/diseases-conditions/gastroparesis/diagnosis-treatment/drc-20355792.

The above Mayo link mentioned this as a surgical treatment that sounds similar to the J-tube:
"Some people with gastroparesis may be unable to have any food or liquids. Then healthcare professionals may suggest that a feeding tube, called a jejunostomy tube, be placed in the small intestine. Another choice is a gastric venting tube to help relieve pressure from gastric contents."

Hello Cheyne,
I don't have medical advice, but I would like to pray for you.
Dear Lord please help Cheyne through this painful disease, guide her doctor's to give her the right treatments for her condition. Lord wrap your loving arms around her filling her with your peace so she knows you're with her and you will never leave her alone. In Jesus Name I Pray 🙏

cheyne, if the nerves of the small intestine are affect with neuropathy your body would be unable to digest and the j tube would be dangerous. Are you familiar with the Nasojejunal (NJ) tube. Have you been given a trial with this nasal tube. Your neurologist may understand by other means that nerves of the small intestine are unable to process food.
I'm sorry you are in this situation.
https://www.sciencedirect.com/science/article/abs/pii/S1566070220301521
If this article looks useful to you, I can send you the full content.

Hi all,
What is happening with advanced autonomic polyneuropathy is the upper stomach is paralysed due to the nerves not being able to make the muscles move. This bottle necks the food and can not pulverise the food into the sloppy paste that is required to pass into the lower intestine where it is absorbed into the body. As a result the stomach can't expand and accept more food as the insructions are not sent to the brain and the instruction for the muscles to work don't exist any more. It make little difference be it food or fluid I'm constained to volume which is at the moment equal to 4 cups of fluid and a small hand full of solid food per day. It is deminishing as time transpires. Currently the nerves in the throat are under threat and falling to the same fate. So the resulting time bomb from this digestion problem is that my BP and diabetes T2 spike directly proportional to the fullness of the upper stomach. I run into the 28mmol/l glucose reading regularly and constantly over 20mmol/l by early evening with BP raising to 225/140. If I don't eat to get my glucose under control it will not go below 15.5mmol/l in the mornings as the body is panicing and dumping the required chemicals to raise the glucose to attack what it perceves as a threat. These spkies and the lack of nutrients are speeding up the decline of the meylin coating on the nerves which is compounding the problem. Now as the brain sees trouble it grabs all the nutrients it can starving the body of what it need to work. While the brain is doing this it also stops the breathing in an attempt to save itself. Now the interesting piece. The cortex gets involved which is our very basic instinct to survival and kick starts the breathing restoring life functions. So that is what is happening with the stomach component.
What I thought was vertigo happens to be autonomic polyneuropathy affecting my limbs, so there is no sensation in the feet up to the knees and not a lot in the hands either. So what this does is delete the ability for the brain to calculate where the feet are and what they are doing, no feed back. Combine this with an hour of nutrients per day which is used up in the first hour of each day and the legs get wobbly and don't have the strength to carry me. I can lock the knees and stand but walking is a different matter. You may notice spelling and grammer mistakes. This is because I was a poor speller to start with , but the finger do not feel pressure so I'm never sure if I have pressed the keys hard enouhg and when I do the brain is racing miles ahead and I transpose letter. I struggle to spell the simplest of words as I can rmember what the look like momentarily. Now onto the eyes. They are slow to adjust to light or dark making night driving difficult and something I try not to have to do. I have always worked in relative darkness without windows for years. This atleast has given me the ability to find my way around with a very good memory of my surroundings, Night or day as when I look away when traveling i loose the ability to know where horizontal is and wonder.
Lastly as a result of the delamination of the meylin coating I have become super sensitive to medications. This means I am untreatable for diabetes and BP and a few other thing as well. With medication being caught in the food lock it can't get to where it needs to be to be effective. So the paradox is medication given to sort the stomach can't get to the lower stomach to be effective and if it does by chance it can work becase the nerves it is supposed to activate can't activate because the meylin has gone and does not work so again the muscles can work. This is the part I can't get through to the "specialists"
As I have found all information stops at the begging of where I'm at. There are no medical document available in the public arena for viewing. Not even with Mayo.
From what I have read people in my state of health are usually bed ridden. For some unknown reason I'm up, about, talking and doing what ever I can. Wether it is my stubborn determination to deal with this disease or something else I don't know. What I do know is I'm not knocking it just grasping it and going with what ever it deals me.
Now the J tube. It is located to the side of the belly button approximately and enters the lower stomach where the fluid pumped in can be absorbed into the body. It by passes the troat down to the bottom of the upper stomach. It needs to be calculated to suit each person and generally takes 7 to 12 hours per day to ingest.
This why I'm pushing as hard as I can for the J tube. It will buy me time but not stop the progression of this disease.
With out the tube life isn't going to be long. With the tube life will be a little easier to cope with but will only be extended. Eventually this disease will kill me, there is no getting around that. It is here to stay. Because the autonomic polyneuropathy is so advanced I may only see until Xmas, we don't know. But the next stage is I will loose the ability to walk and stand and then the organs will come under attack at which time game over.
The concesus is I shouldn't have made it this far, but as I a stuborn fighter and have a huge will power with an indepth knowledge of what my body will take, so I push the boundries every day trying to defeat what I know I can't. I think it is the challenge that although ruff it keeps me focused and fighting for every moment I can get.
This is a 14 year journey so far and not once have thought why me. I tend to think there are people worse off than me, what have I got to complain about. My new neighbour is riddled with cancer and has not long to go, I should worry. I know what death is like, tried that once, drowned, and was resusitated, watched my mother die with the body shutting down to protect the brain until even it could not stop the process and had my kidneys shut down on me for 18 hours and survived that with Campylobacter, given hours to live but survived that only to contract the autonomic polneuropathy and start this journey. The body tries to fight the campylobacter bugs but can't differentiate between the bugs and the meylin sheeting in the process destroying the meylin and starting the journey. Anybody that has a serious stomach infection like long covid and others this is what you can expect if you are unlucky.
For those who don't know the autonomic nerve is the conduit that transmitts the messages between the muscles and brain to make things happen with out our having to think to do whatever the instruction is.
That is the full picture. How long my piece of string is I don't know but I can say with some accuracy it is definitely getting tighter. Until it breaks I won't give up pushing.
Just had word I'm to endure a colonoscope on the 25th. That should confirm what I'm saying to the specialists. If it dosen't then I have something very wrong inside me. I will do it without sedative so I get to see what the specalists team see.
Sorry for the length but half a story means little.
Cheers.

I can briefly share what is tracking for me and what I've been told. I've been diagnosed with Sjogren's, polyarthritis and Undifferentiated Connective Tissue disease. I was started on hydroxychloroquine last year. Prior to beginning medicine I started losing weight. Because I was trying to eat healthier with less inflammation I at first attributed it to that. I was slightly underweight to begin with so this was unintentional. As the past year has gone on the weight loss has become dramatic and last January my primary care doctor referred me to a gastroenterologist and also ordered his own tests. I had an endoscopy (which showed mild gastritis) which was consistent with autoimmune disease. There was no real follow-up for a few months but the weight loss continued. I had a barium swallow which came back normal and finally a liquid gastric emptying test which revealed delayed emptying almost three times the normal length of time. This was also followed by a colonoscopy that was normal with the exception of a small polyp. Since then my rheumatologist who is really sharp did small nerve fiber biopsies on my leg which showed SNF damage. I've had edema in my lower legs and other nerve symptoms. I also have had other autonomic dysfunction symptoms for a long time. According to my rheumatologist and information that I've read, this is an area that is particularly impacted but certain autoimmune diseases like Sjogren's. I am currently being treated for the gastroparesis and other SNF related complications. My rheumatologist is trying to go after the neurological source of these by starting me on mycophenolate; the goal is to have me approved for IVIG but I have to start with this med before insurance will agree. The doctor said it's very difficult to find a medication to slow down the attack on the nervous system that doesn't have side effects, but believes that until we do, food replacement is only a stopgap. I am 5'6" and have gone from 127lbs to 96lbs in 11 months. I realize that being diabetic is a different metabolic problem, but wondered what medications are being used to directly slow down the damage being done to your nerves? Has anyone discussed an IVIG medication track?

@fairn
Hi,
In a word, nothing. I'm trying to find a middle road to how much I can eat before the glucose spikes, or how little I can eat to stop the pancreas unloading raising the glucose. Unsuccessfully so far. The major concern is the lack of nutrients I get as this speeds up the demeylination of the nerves, accelerating my decline. What the specialist don't yet understand is that no amount of medication will help the autonomic polyneuropathy. When the meylin which is the sensing part of the nerve tips is destroyed nothing will fix the problem and you can't make the part work when it is effectively dead. Hence no feed back to the brain in either direction. The latest research I read was that a chemical has been found that rebuilds the meylin on nerves. But as it is still being trialed and modified on rats, it will not come to the markets until after I'm gone. But there is hope for MS and dysautonomia patients ahead.
Cheers