Medical Mystery - Esophageal Motility

I am a 37yr old female and have a very rare condition involving chronic and severly acute spasm "attacks" invloving my lower esophagus, diaphram and stomach. I have been seen by over 30 specialists and have had a full work up at the Mayo Clinic. After suffering with this for 12 years now I am finally seeking help from a pain management specialist (this was the only recomendation the Mayo Clinic could give me). I have tried every anti-spasmatic available including nitro with no relief. I have been taking Darvocet preventitively and 10mg of valium before meals. I have chronic pain from the time I wake up in the morning and then have acute pain after meals. This problem seems to be all food related. While on this current medication I can only manage to eat basic carbs (crackers, bland pasta, some cheeses)I have been a vegitarian for 25 years but if I eat any vegetables, spices, restaurant food, eggs, sauces,cereals, herbs, etc... I have an acute "attack" which lasts from 4 to 12 hours which resembles passing a kidney stone or labor. It takes 20 to 70mg of valium throughout the duration of the spasms to keep me out of the ER. The problem has now become one of malnutrition. I have had over 25 dental surgeries for bone infections (definitively diagnosed from malnutrition) and many other problems due to the lack of nutrients (constant illness, skin problems, etc.). I can't take any type of vitamin, I've tried every kind avaliable (including sublingual & sprays) I can't even drink water because of the minerals it contains. I have no energy and need to drink 6 to 12 sodas a day for energy (no the soda doesn't bother me at all). All my GI tests have been normal (including pancreas) except for a motility test under light sedation that couldn't be completed because my esophagus clampped down so hard on the wire half way down that I had to be put under full sedation to remove it. I had my gall bladder removed 6 years ago and had no relief. I occasionally have taken Levo Dromoran for the acute attacks which does controll the pain somewhat. My DR.'s now feel I need to be put on narcotics on a regular basis so that I will be able to get some nutrition. They say it is the last resort but it is better than "dynig a slow death" (their words) not to mention having no quality of life. I can't take antibiotics without narcotics & even then usually only amoxicillin. There probably is no medication that I haven't tried and the description of my diet is literal! I have tried for 12 years not to go the heavy duty narcotic route but I seem to have no where else to turn or the DR.'s feel I will end up with a problem that can't be cured. I am terrified of addiction but I guess it's better than rotting away and getting a pneumonia that can't be treated or some other horrible condition. I must ad "stomach" problems have run all through my father's side of the family but I have gotten the worst of it. HAVE YOU EVER HEARD OF ANYTHING LIKE THIS? Any advice PLEASE!!! Thank you

[Kevin Pho, MD]

This is certainly a difficult case.  If you have already been evaluated by 30 specialists as well as the Mayo Clinic, it is unlikely that I would have anything to add.

You may want to consider being evaluated by a competent pain specialist.  That way, you can be on a narcotic regimen with close follow-up to ensure you do not have the side effects.  I agree that nutrition is absolutely a priority.  You may want to inquire about the feasibility of a feeding tube or intermittent episodes of TPN (i.e. nutrition via the veins) to help supplement your nutrition.  A discussion about alternative routes of nutrition may be considered with your primary care physician or nutritionist.

Followup with your personal physician is essential.

This answer is not intended as and does not substitute for medical advice - the information presented is for patient education only. Please see your personal physician for further evaluation of your individual case.

Thanks,
Kevin, M.D.

[krisg]

Have you ever been tested for bile duct stones?? MRCP or ERCP??
They are quite severe in the pain category.  Worse than labor for me.

[mysticalmermaid]

Kristin-
Thank you so much for reading through my whole saga! The answer to your questions are yes, yes and yes. I have been tested for all three. My situation seems to be a very rare autonomic nervous system problem. Although your comments about those tests were entirely appropriate because the Mayo Clinic thought of them too! Again thank you for responding and best of luck to you, I hope you situation is under control!

[nurse12hr]

Why on earth aren't they simply putting in a feeding tube?  If your malnutrition is so severe, it sounds as though that would be a temporary (hopefully) solution to building you back up while they continue to search for answers.  

At least it would bypass your esophageal spasms.  It can even be directly implanted in the stomach if they are not able to pass a tube down your esophagus. If this is not feasible, TPN as Dr. Kevin said, would give you the nutrition you need without involving your GI tract at all!  Better than living on narcotics and dying of malnutrition!

Really, I am surprised that someone in such bad shape hasn't been put on TPN by all those Mayo doctors.  It is done all the time.  I used to do home IV therapy, we had PLENTY of patients on TPN at home.  

Excessive soda drinking can lead to bone loss.

Good luck.

[mysticalmermaid]

Thank you all for your input. I truly appreciate you taking the time! Just wanted to let you know I have an appointment with the head of the pain management clinic at Thomas Jefferson University Hosp. PA tomorrow. Also I will be seeing a nutritionist and the head of the motility clinic for a check in next week also at Jeff. I live in NJ and don't want to have to go all the way back to MN for a 3rd time. I know this condition sounds almost unbelievable and I am bery grateful to those of you who have responded taking me seriously. I will let you know when we come to a workable solution. Best of health to all of you in 1994!

[krisg]

Glad you found a good doc.  I went to the Univ. of PA to seek expertise care.  I travelled from TN too. It was worth every mile.  I have friends and family in the PA<NJ area which also helped.  Good Luck.

[SheraF]

Hi Mystical Mermaid,

I live in NJ too, and I am having a lot of the same symptoms that you are having.  Most doctors think that I am imagining things, because there is really nothing showing up in bloodwork and some major testing.  I DO have Fibromyalgia, GERD, and a hiatal hernia though, but they don't know why I am having all these spasms with the stomach, intestines, gall bladder and pancreas.  It all seems to be a mystery to them.  I am seeing a doctor who works with the Myofascial Pain Syndrom (syndrome) (I have that too), and hoping to get some answers.  Usually all the doctors tell me that it's just the Fibromyalgia, but I know there is more going on than just that.  I was wondering if I have MS. No doctor has taken into consideration of my other symptoms which include loss of memory, the shakes, the headaches, and the nausea, and so much more.  Have you gone out to see that new doctor?  I hope you did well, and I wish you well.  God Bless.
Take Care,
Shera

[Applegeek]

I have heard that they are trying Botox injections into the esophagus for reduce spasms...have they talked about that?

[Ann44]

A related discussion, Did you see the TV Medical Mystery last fall was started.