Avatar universal

Have we finally hit the wall? No more solutions?

I'm new to the group, and this is my first post. I'm a member of several dysautonomia-related FB pages, and it always seems my 21 year old daughter's case is a compilation of everyone's symptoms times ten. We have plenty of diagnoses, but are out of treatments, and it seems there's nothing more to do but watch her slide downhill. SO, I'm hoping someone on the site has something new to add to give us hope.

Diagnoses: Chronic refractory migraine, POTS, severe orthostatic hypotension, central pain syndrome, Ehlers-Danlos Syndrome, non-specific dysautonomia, cyclic vomiting. Now daughter has a jtube for enteral feeding and a port for fluids because she cannot take anything by mouth without vomiting. She has been on every medication everyone else has tried for this. Cardiac doc took her off florinef because of the side effects, but that sent her right to the ER. She cannot even feed on the jtube without excruciating pain, even with Fentanyl and anti-nausea drugs. So, her weight is dropping, dropping, dropping. She goes days or weeks without any nutrition. Her GI cannot offer her anything more except TPN in a hospital setting (IV feeding). Her pain doc keeps upping her Fentanyl but cannot keep up with the pain.

We are having her evaluated for Chiari I malformation (common with EDS) to see if she has this, and if spinal/cranial surgery will even help.

We have considered going to Vanderbilt or Cleveland Clinic or some dysautonomia center (already been to Mayo in MN--no help), but my honest opinion is that nobody really knows how to treat dysautonomia. They can run a million tests and diagnose, but beyond that all treatment is palliative. Especially when it comes to gastroparesis. Am I wrong? Am I missing something?

My daughter is in the ER all the time and we see her life dwindling away. Any guidance or ideas? Her cardiologist even wants to try a new device that acts like acupuncture on her earlobe. That's how desperate we are.

Thanks for listening, all.

13 Responses
4489852 tn?1375753951
First off I want to say welcome to the group! I am so sorry that your daughter is having so many issues! I am 20 years old, so similar in age to her, but not nearly as severe. My current diagnoses includes Chiari 1 Malformation, Ehlers-Danlos Syndrome, POTS, severe orthostatic hypotension, generalized dysautonomia, and IBS. We suspect gastroparesis as well as possible tethered spinal cord syndrome and cervio-cranial instability.

Am I correct in saying that the nutritional issues and GI issues are the main concern right now? I know there a couple of members who have feeding tubes so hopefully they will chime in on that issue because I do not have much knowledge in that area. I am much more knowledgeable with regards to the neurological side of her issues. Who are you seeing for her Chiari testing? It is essential to make sure that you see a Chiari specialist. If you go over to the Chiari forum they have a list of doctors that members have seen and liked. Since her symptoms are so severe I would consider maybe going to The Chiari Institute in New York. Regardless of where you go make sure you get a FULL spinal MRI: cervical, lumbar, and thoracic, along with a CINE MRI to check on the flow of CF fluid. Rule of tethered spinal cord syndrome as well as this may contribute to her digestive issues. If she has a syrinx (pocket of fluid in the spinal column) this may also cause GI issues. Check for cervio-cranial instability too since this is more common with EDS. Cranial pressure issues should also be ruled out as well as having a full brain MRI done if you have not done so already.

Good luck, and again I am sorry I cannot be more help. I cannot stress enough how important it is to get a Chiari doctor that truly specializes in Chiari and its related disorders. If you don’t it is incredibly frustrating to get the help she truly deserves.

Which FB pages are you a part of that you like?

Sara Jane
612876 tn?1355514495
Hi and welcome to our community!

I responded to your PM, but wanted to add a thought here. Pain with tube feeds is not uncommon! In my experience, there are several things to try. How many formulas has she been through? I'm guessing they already tried to troubleshoot by moving to a semi-elemental or elemental formula? My pain on regular formula was intolerable, even with morphine. Second, does she have a g/j or a separate g-tube so she can vent her stomach? Intermittently or continually venting the stomach via Farrell or urinary leg bag can make a HUGE difference in pain, as well as reduce vomiting. Third, have they tried her on bentyl and/or Levsin for the crampy pain that can accompany feeds? Fourth, what pro-motility agent do they have her on? What about acid and/or bile reducing medication?

Also, I've found that I cannot function without simethicone to treat gas from formula (taken three times daily as infant drops via tube). I also use a tens unit on my back during feeds, as I find I get back spasms from sitting upright while on the feeding pump.

If they haven't changed her tube since the pain began, that's another possibility. Sometimes people just get a bad placement in the jejunum and it's painful until the tube is changed.
Avatar universal
Thank you Heiferly and Sara_Jane19 for your speedy and incredibly helpful comments. Good heavens, you both have provide more good information targeted to what my daughter's been thru than all the docs put together.

First, in regards to Chiari, I really am hoping to get MRI studies done here in TX, then traveling to a Chiari center for further eval if warranted. I'll ask for the ones mentioned. Is it important to get an upright MRI (vs lying down)?  Everything I read re: Chiari surgery is frightening. 30% have repeat problems. Dr. Durrani facing 120+ fraud lawsuits for Chiari surgeries. Eek. Any thoughts on TCI vs Cleveland Clinic or another center?

Next, Heiferly thanks for the PM. I'll read that next! Our GI knows ZILCH about jtube feeding. How'd you get such good info? In the hospital, when they first did jtube surgery, they only tried a couple if formulas. Neither worked well. The one she's on is "predigested." Not sure if that's what "elemental" means.

She just has a jtube, not jgtube. I wonder if simethicone alone would help? That's an easy one to try. She's on fentanyl for pain and marinol. Don't know about bentyl or the other meds. Will have to check and get back to you, but I don't think she's on any motility meds or acid reducers.

We only found one doc within a 4 hr drive who is a motility specialist, and he was a complete jerk who wouldn't treat unless we moved there and offered no ideas over the phone unless I begged.

Who is treating you?

Thanks again!!!
4489852 tn?1375753951
It is not as important to get an upright MRI as it is to get a CINE MRI. Many doctors are concerned with the length of the herniation, but in reality what is important is the amount of CSF that is flowing. If there is a blockage or reduced flow, it does not matter how long the herniation is. This is something most doctors don’t know/believe, so make sure you keep this in mind. Also, if she has a syrinx anywhere in her spine this can be an indication for surgery since the CSF flow is obviously disrupted.

I have never been to either TCI or CC, but I have heard fantastic things about TCI! There is a woman named SelmaS over on the Chiari forum that is incredibly knowledgeable about it, I will have her PM you on that topic. As for the surgery itself, no it is not an easy one. With the EDS it is important to work with a surgeon who is a Chiari specialist. To patch up the dura, the covering of the brain, they will want to use her own tissue as people with EDS are more likely to reject foreign patches. They also need to be careful doing a laminectomy and craniectomy (shaving the first vertebrae down and taking of part of the skull). People with EDS are more prone to cervical-cranial instability. She needs to be evaluated for that because of the underlying connective tissue disorder, and if she does have it, they can do the fusion surgery and the decompression in one surgery in many cases so that she does not have to undergo multiple surgeries. Also, it is recommended to only use steri-strips and glue because many with EDS reject stiches and staples, as I did, which causes pain and may cause a leak. This being said, even with all of these precautions it is still a risky procedure that can have complications. I know the TCI has a lower risk of complications than many. If she does have blocked CSF flow and/or a syrinx this will be the only way to stop the neurological damage from getting worse and reestablish a CSF flow. It will NOT guarantee all her symptoms to go away, but many times at least some symptoms are decreased in severity.

I know it is a lot, but make sure to rule out all conditions before surgery so you get a complete picture of what is happening. Keep us updated and again good luck!

Sara Jane
Avatar universal
Oh, boy. This sounds like a real last resort surgery, Sara Jane. Thanks for the incredibly helpful detail. We will use it during our meeting with the neurosurgeon. Will definitely keep you posted, and my good wishes to you for health and healing!
4489852 tn?1375753951
It is definitely not something to rush into and for sure something only to do with a Chiari specialist on board! Take your time and get multiple opinions :) It was a rough surgery, but in the end it did help me tremendously! Thanks for the well wishes and good luck again!

Sara Jane
Avatar universal
Chiari is a difficult situation for sure.....many patients  (in my experience) continue to have headaches...make sure you have explored the options thoroughly.  It must be frustrating to watch your own child decline.  I have dysautonomia/POTS as a long term consequence of diabetes type I.  For the gastroparesis, my doc put me on erythromycin about six months ago and the nausea has improved tenfold.  There are also gastric pacers being implanted with some success.  Your daughters diagnosis is so complicated.  I too have tried many medications.  The thing that seems to be helping now, are PRN IV fluids to keep my blood pressure relatively normal.  Its is also believed that having normal blood pressures slows down the vision loss I have.  The most important thing of ALL is to have a supportive physician, which I hope you do have.  We are all realizing that this is a relatively new thing for the medical community to deal with.  Have been on a facebook page where we are monitoring our pulse pressure and giving one another support.  prayers and blessings to you all
Avatar universal
Thank you, CaseyToney. My daughter is on IV fluids, too, and has a power port. Otherwise she crashes. Doc took her way down on Fludricortisone (sp?) because of fear of side FX. That caused a crash too. I keep hoping for a period of stability, but the only constant in dysautonomia is imbalance, crises and trips to the ER. I wonder if many folks achieve periods of quietude or remission longer than a few days?
612876 tn?1355514495
I see docs at Ohio state university medical center and Cleveland clinic for GI.
Avatar universal
Please message me--my daughter in same place.  
Avatar universal
If you suspect chiari then have the MRI done both with and without gadolinium so that they can also check for a spinal csf leak. I don't know her symptoms but spinal csf leaks give chiari symptoms plus orthostatic headaches and nausea that after a while tend to be chronic.

Go to csfleak.org

But the first problem must be the weight loss I assume.

Best wishes to your daughter and you!
Avatar universal
I see your post was in 2013.  I hope your daughters issues are all resolved now.  If not, you need to make sure she does not have a CSF LEAK (cranial or Spinal) causing the headaches, hypotension & chronic nausea.  It took 2 years for them to find my daughters leak (caused by a small arachnoid cyst in her lower spine), planning surgical excision at Duke (Dr. Lind Gray for diagnosis & Dr. Allan Friedman neurosurgeon) next month (after 6 failed CSF patches elsewhere) & this will be curative.  I hope you find success like this !
Avatar universal
The truth is that if you come up with a cure for dysautonomia, you will not be on a magazine cover.  You will not have a clinic sponsored by the NIH.  Add to that the fact that the symptoms are diverse, and there may be more than one cause.  As a Ph.D. physicist, i understand that this is not a fertile area to explore.  I you are even tempted to do research on dysautonomia, you will find that it is difficult to get funding.  Fortunately, a brave few will want to work on this problem precisely because it is extremely difficult, and if you do, you are likely to help patients who desperately need you.
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