Open Discussion on Primary Esophageal Alchalasia

Ty for sharing. Just a suggestion. Please have a manometry before you have a Myotomy. You need to know, the surgeon needs to know what he's dealing with. And please get a second and third opinion. It doesn't sound right that they want to do a Myotomy on you without a manometry. You have to take charge of your health and beat down walls before you allow anyone to cut on you. I wish you well but I pray you will take my advice. You may end up with worse problems if you don't.

Hello maya, he's right about you needing more tests and unfortunately doctors across America have discovered this disease , finally and many are trying to learn to do POEM procedures and as a result they are getting very quick to cut. I had it all, felt like food was stuck in my throats all the time and every meal had severe chest pains because the muscle wouldn't open to let food into the stomach until it had to and the would push very hard to open and I didn't know if I was having a heart attack or not. So they removed it last June and oh my god have I suffered with a rush of acid which has done tissue damage the entire length of my esophagus and into my vocal cords. Not even talking about the terrible burning that would last about 2 hrs and it would wake me up after only sleeping for an hour or so. Before you ask yes, I sleep almost sitting up and I've had 7 back surgeries for an injury 10 years ago and sleeping the way I do is putting a strain that will most likely cause another surgery. To get back on track , see more than one doctor , you owe it to yourself . I recently had a brand new procedure on my esophagus called a plication with a overstitch where during a Endoscopy they pull up your stomach wall and wrap it to your esophagus and then stitch it in place. To recreate the muscle they just removed. I've been on every medication before and still take 4 meds a day and at first I thought this procedure was a failure but after letting it heal a little I have only had an issue twice and nowhere near as bad as before. I was told I am only the second person to have this done and at first I was angry as I thought I should have been told before the procedure but I'm getting on in age and I figure if they can really improve someone's life using this method and you look around and young people are getting this serious disease I felt like I just wanted to help. Ask many questions about diet, time frames, besides the test mentioned they should also do a Bravo PH Test where they attach a small capsule to the wall of your esophagus and you wear a monitor for two days and push buttons but it tells them how much acid is getting past the point where they placed it and from the monitor and your notes can tell what foods and body positions it happens more frequently. Any questions just yell out. We are here all the time. Sometimes if you like you can state where you are from and maybe get a great referral. Good luck and hope you feel better very soon

1maya and Robert:

1maya242 Robert is right. I would indeed insist on a Ph test be done. I use the word "insist" because many Drs want do them or a manometry. But out of the two, you definitely need a manometry done. For some reason I feel that they don't have your best interest at heart. Or, either lacking in info about Alchalasia. Hun, you MUST get a second, even a third opinion before you allow anyone to cut on you. Surgery is not that clear cut. It can bring about another entire boat load of problems for you. Even if you have to fly to another city/state, do it. Make sure you see only the BEST! This is a life altering anatomical change they are doing on you and you must take control of your own health and educate yourself to ask important questions. If you think your PCP or anyone else is going to do a better job at it than you, you would be mistaken. This is a rare and rather peculiar disease that effects everyone differently. Whatever you read from others experiences don't think for a moment that yours will mirror theirs. Like I said, we all react differently to the same thing. I just can't stress enough the importance of getting second and third opinions from both GI specialists and surgeons a like.

Robert I'm so sorry you are having all these problems. I know acid reflux is horrible. As an RN I also know that the gastric acid within our stomach can burn a hole through carpet. So I can only imagine what it's done to your soft tissue within the esophagus and your trachea and vocal

Cords. I'm so fortunate not to have any of this. I can eat all I want. I've gained 15 pounds because when I eat I keep eating because the food goes into a second stomach created by all the dilatations I've had to my esophagus. So, my stomach doesn't signal the brain telling it that it's full. So I keep on eating. My esophagus can hold three large meals if not more. And it takes up to 48 hours to squeeze through the LES into the stomach. I can take a pain pill and not feel it for hours. Sometimes many will collect in the esophagus and go down at the same time making me high as hell. Which I don't mind. Lol.

I pray that the overstitch works for you. Give it more time and eat things non acidic causing. I think sodas would be good for you because of the acid base balance. But I could be wrong. I was trying to remember all the science I had to take but forgot it all. I would suggest gaviscon though. And I'm sure you're own PPI's at large doses. But, you have to be careful with them because they do cause renal problems after prolong use. Or can I may add. Research Gatric Acid and Ph Balance in The Stomach. You may find some info there to help tolerate or lessen the acid, or to balance it out. I'm glad you are sleeping sitting up. I have to or I will aspirate big time. And my aspiration is severe and it feels like I'm going to die when it happens. When your airway is 100% occluded the only thing on your mind is clearing it however you can and taking that one breath. I've had to do self heimlick menuever on me by falling forward on the floor to dislodge food and fluid. I'm honestly scared I'm going to choke to death one night when I can't clear it. I have severe chest pain. Last night I had to take nitro tablets for it. It's a crushing pressure pain that radiates to my jaw and teeth. Often it's hard to tell if it's esophageal spasams or heart anomalies. When in serious doubt I go to the ER immediately. Many have died assuming it was their stomach/esophagus. Please don't chance yours if your symptoms deviate from the norm you've come to know. I can't stress the importance of that. We don't want anything to happen to you.

DWJRN

I'm sorry if I didn't mention that they recently did an endoscopy, manometry, and barium swallow to make sure about my diagnosis which was originally given to me 16 years ago so the surgeon will have all the pictures and information. I do not have any peristalsis in my esophagus. They said that my esophagus is dilated and my LES is somewhat open, but I suspect that it spasms at times, and that's why nothing, not even liquid will go in. I suppose I could get a second opinion but I don't think it will be different. At first I was hesitant about the surgery, but now I really want it. I will, however, ask about the balloon procedure again.

Thank you both for your suggestions.

Maya, I'm glad that they did all of those tests. You can see why I would have been worried and didn't think you were getting the best care. A pneumatic dilatation may be an option if you're a candidate for it. I'm not because I have stage three spastic Alchalasia. I did have the botox but it only worked for 6 days then I was back at aspirating in my sleep. I know that you trust your surgeon, I do as well. But, I still got 3 opinions. Not everyone was in agreement. I would still highly suggest you at least see another surgeon before you allow them to do your Myotomy. It may be that you need a partial Fundoplication as well or either would benefit best with a POEM. I'm having a POEM and not the Dor Fundoplication the surgeon wanted to do. It just makes more sense for me. Just know that just because your surgeon excels at and known to do one particular Fundoplication, that doesn't mean that it's best for you. A surgeon is going to push what he does best. That's why I would get three opinions from three different GI specialists and see what they propose. I personally think that they know more about which procedure or fundo you need moreso than the surgeon. I saw three GI docs and two different surgeons. Be safe and not sorry. I would sit down with another GI doc and ask him if he were you, what type of surgery would you want your daughter to have. But, that's all up to you. A less invasive procedure may last 6 months to two years. You may not have to have a Myotomy right off. That way Drs will know more about the treatment when or if you need surgery.

DJRN

Hello, Maya--so sorry that this disorder is making life so difficult for you.  I am not swallowing the way I should, either, lately and so I have at least some idea of your discomfort.  Ever since I read this post by you a few days ago [and simultaneously became aware of achalasia] I have been reading papers on the disorder in medical journals.  One can do that online.  I am quite involved in DNA studies and so the last paragraph of your message especially intrigued me.  The papers dated back to 2010.  On the one hand, it is said that achalasia is "rarely inherited" and is idiopathic, meaning it arises spontaneously, but those papers I viewed seemed to me to be gradually creeping toward a genetic component.  Now, we are all genetically susceptible to some disorders, some of us to multiple ones.  After all, we have the genes of two parents contributing.  I have described my family's obvious weakness when it comes to GI problems in the IBS forum, but that doesn't necessarily show up in a genetic profile that one receives when one has a DNA test.  However, mine indicated I am definitely genetically susceptible to Crohn's disease and my sister has suffered from that since her early teens.  So far I have escaped, they say, but what has been diagnosed as IBS for me may be the start of something else,. 

?The latest paper that I read is from 2016.  While, again, it repeats "rarely inherited", it rather confusingly discusses "genetically susceptible" and I would really have to wonder just what distinction is being made there.  I quote:  "The likely etiology includes neuronal degeneration, viral infection, genetic inheritance, and autoimmune disease. Current evidence suggests that the initial insult to the esophagus, most likely a viral infection or other environmental factor, causes inflammation in the myenteric plexus and leads to an autoimmune response in genetically susceptible individuals, with subsequent chronic destruction of inhibitory myenteric ganglion cells resulting in the clinical syndrome of idiopathic achalasia."

??Here is the paper:

https://www.nature.com/articles/srep31549

?Some of it is clear enough and the rest leads to instant brain freeze, although I am used to such papers.  This is the circle it goes goes around in:  It is still a rare disorder because the genes that make one susceptible are not often found in the population to pass on--like Crohn's disease.  Crohn's is the result of an autoimmune response and likewise achalasia.  But the paper indicates no great belief in the "spontanaity" of achalasia, suggesting something must trigger it.  That's contained in my above quote.  I realize all this is less than cold comfort, meaning none at all.  I am merely pointing out the road the scientific community seems to me to be taking in attempting to understand achalasia.  As far as the genetic inheritance component is concerned--I doubt that can be wrong.  Everything that we are is programmed in our genes.

 

Just to be clear, "idiopathic" does not include achalasia where opioids are suspected as a contributing factor.

This is true. But, they don't know for certain they are a direct link to the cause of Alchalasia. Only blind studies to suggest there is a correlation between the two. Which I think there is.

I am not disagreeing as such, but I think the experience in the USA may be quite different from the UK, especially in the NHS.   The surgery for achalasia in the UK is undertaken by surgeons who are typically dealing with quite a lot of cancer surgery, and there is a strong ethos of working in Multi Disciplinary Teams for the cancer cases, and that spills over to the benign work.   So the chances of having one particular course of action strongly pursued by an individual do become much reduced in the collegiate discussions.   Which is not to say that there are not strongly expressed preferences about surgical options, of course, and differences of professional opinion both amongst gastroenterologists, and between some gastroenterologists and some surgeons.    The NHS being a publuic health service does bring its own benefits and issues;  the private health sector in the UK is smaller.

Over the past generation the GI surgery has migrated to a very much smaller number of centres, and there is quite a lot of pressure to reduce further so that a higher level of expertise is maintained.

​The issue of the manometry showing details of the condition is absolutely right.

Thank you Alan for the kind comment. I understand where you are coming from. Medicine and multidisciplinary approaches due seem to differ. I just know that many surgeons in the US are either Thoracic or Bariatric surgeons. And for the most, each of these surgeons have adapted to a specific way or approach to treat Alchalasia. ESP when it comes to the type of Fundoplication they choose to do.

Regards,

DJRN

Good Morning, I had my appointment yesterday with my gastro and I received multiple items of bad news.

Let me ask you, when I did my 4th Bravo test for him he told me my acid levels were the highest he had ever seen or heard of. I was told a normal reading for a person with reflux is between 30-40. They then showed me mine, which were 116+ . I was told I had multiple pockets of severe scar tissue from acid and that there is no movement anywhere along my esophagus. I was told I could get Barrett's but that would be from the acid and possible because it's more common in white men middle aged. Also was told by the looks of things and because I have already had squamous cell cancer in another body part he thinks my Achalasia is so severe that it almost certain. I was told today that the plication with an overstitch is not a permanent fix and needs to be done like once every year or two, which I was never told. I was right I am the 2nd person he's done this procedure on with Achalasia and there's a doctor in California that has done 7. So, there's 9 of us walking talking lab rats. Now they want me to do another Nravo ph test which I feel is bs cause it will only benefit him, as per if my acid numbers went down just a little viva success. They also want a Barium swallow study , I already know it's all gravity and lastly the want me to eat a radio active breakfast to track it. I really thought they were going to call security cause at one point I told him he was a very lucky man that I was behind a walker. Lol shitty day ! Hope you feel better

Barrett's Oeophagus (BO) is also called columnar lined oesophagus and should normally be visible on an endoscopy because the cells in the lining of the oesophagus are a darker red colour.   It is heavily associated with prolonged, severe reflux.   The chances of developing adenocarcinoma from uncomplicated BO are low, but they do increase if there is dysplasia, particulalrly high grade dysplasia, which carries a 50% risk of adenocarcinoma in the following 1-8 years.    There are some variations in how BO is defined between the UK and the US so one needs to be a bit careful, and these figures relate to the UK.

?Reflux is very much associated with adenocarcinoma and not squamous cell carcinoma, which does not make your doctor's judgement wrong of course, but the logical progression would be to have biopsies taken and to test for dysplasia and indeed possible cancer.   Talking about cancer at this stage is at best premature, unless they can actually see a tumour there.

?So I think, taking things in priority order, you have to get some tests / biopsies done to check whether you do indeed have cancer.   Then you need an assessment of whether your oesophageal scars will heal.   You need some method of reducing the reflux, which may involve some surgery and this needs to take into account your lack of motility.

?I would say that with prolonged and severe reflux suffered for many years, there is a good chance that you have BO.   There is a procedure called radio frequency ablation that can remove dysplastic BO and thereby give you a reasonable chance of avoiding progression to cancer.

?On the other hand, if your oesophagus is in a very poor condition and there is a high risk of oesophageal cancer developing, you might be better to have it removed.   This is major surgery, and involves pulling up the stomach and joining it up to your throat, but I know a lot of people who have had this done and it is perfeclty possible to enjoy a good quality of life afterwards.   But you do need to be in the hands of an experienced Upper GI surgeon at a specialist centre.

?I mentioned the overstitch method to an experienced Upper GI surgeon in the UK and he raised a sceptical eyebrow but of course doid not know the details of your case, and it was only a very brief conversation.   Another UK surgeon said 'it didn't work'.

I can quite understand them wanting to double check the acid readings, but those numbers do not correspond to the pH scale that I am familiar with, and it may be that I am just unfamiliar with the Bravo methodology.

 

Robert, I really hate that you're having to go through all of this. Damned if you do damned if you don't. The plication overstitch has been done before, but your Dr. has probably, along with another has probably altered it in some way making you one in nine patients to ever have it. It doesn't sound like the usual via overstitch. He should have told you that you had to have tune ups or re-dos every year or so. I would have been very angry.

I wish there was something that I could mention that might help you. Stomach acid will destroy you. It's literally acidic enough that it will burn a hole in a thick piece of carpet. You can imagine what it's doing to delicate, high vascularized sensitive flesh and mucous membranes. They have to find a way to correct it. It will more likely than not do permanent damage to tissue. I just don't know what it is unless they do a full wrap with another Fundoplication. Many people think that these surgical procedures are permanent. Most aren't. Most can be reversed. It may be they reverse what they've done to you thus far and start over again. I'm wondering if a total wrap would stop the acid reflux. Keep us posted. It's always good hearing from you.

DJRN

Come up to Portland Oregon at OHSU my doc Dr.Dolan he has done least 37a year and is very confident on his results.

Thank you again Alan, All I can say say is that I was told there's nothing left to do except to try and reduce the acid . I don't know if I have a overreacting stomach , I've tried to change my diet , the times I eat and my body position when I sleep. I think it's sad that I lost almost 7 years which is when it started getting bad and instead of doctors giving up and trying to find me help , maybe if I had done something earlier things might look better at this point. You are a stranger to me and yet you have helped more than some of the doctors I have seen. Thank you very much. I know sometimes I disagree with you but it's all good. Have a great summertime day

He did mention that a full wrap would be the only thing at this point left to try but he also warned me that in my condition and stated anyone with Achalasia it's very difficult for a surgeon to get it perfect. If it's too tight or to lose your still in trouble and it's not reversible. I'm not angry at my results that's in gods hands but I'm very angry that I wasn't told a lot of facts before jumping into this. I told him that I was angry and he laughed and said so what's the big deal I didn't do anything that can't be undone? I said at 65 I don't like going under general anesthesia for no reason or for that matter giving up my meds for a week prior to or having to stick to a very Strickly diet before and after the procedure. They think it's nothing to put the body through these things but you know better.

I know you haven't been doing well either so thank you for reaching out again . Gotta go doorbell

Oh cowgirl 37 a year, how many total has he himself done? This is a new surgery that doctors like to stick out their chests and bragg.

I'm not sure how many years but he is the professor and teaches also do I'm sure he's good all I can do is try cause the

Have you looked inti you phyloic spincher I got a link tabout it cause I asked. Well it talks about bile acid which could be why the stomach acid pills aren't working. My husband has acid told him Barrets and he take prilsic and it didn't work for him Good luck!

I understand the thought process ,I do. I'm just trying to tell you that they lie like rugs. My last surgery with a doctor who has done almost 500 POEM procedures did not tell me he had never done the procedure he did on me on a patient with Achalasia. Instead he boasted about traveling all over the world to teach other doctors how to do this procedure. Gods honest truth. I only tell you this so you will be careful don't use any doctor just because he's close by, they can ruin your life and not give it a second thought. I know you're in terrible pain I was and still am after 2 surgeries. Use to think I was having a heart attack after meals. I know first hand all about it. I will pray that whoever does your procedure will have great skills in his hands that day for you. Be careful and hope you feel better soon

I have yo travel 100 miles to see doc cause the ones where I live dont know sh*t only lime to stick tubes up your #$$ . selfnvestgated my self after being told I have neoaphty of my esphogus. Thats why I choose them . They teach at that hospital. I'm in gods hands at this point

Both of my surgeons told me Fundoplications partial and full are reversible.

I have a little narrowing of my pyloric sphincter and an ulcer. But not bad enough to cause me symptoms. I only have trouble aspirating for the most and developing bouts of aspiration pneumonia. If I sleep propped up I'm good. I'm looking for a new bed. One that's adjustable and where I can sleep at about 65 degrees. If that works I will not have a Dor Fundoplication or a POEM. IM praying that it prevents it.

Robert, I would shop around for the best surgeon you can find even if you have to make a long road trip or fly. I think if you find one who's done at least a 100 of them you will be goid.

DJ

Wow, I was told 2 days ago just the opposite. I'm getting to a point where I don't believe anything a doctor says to me.

Let me understand, you were told your husband has Barrett's esophagus? All they give him to take is Prilosec? Wow, he must have some serious pain and burning . What dosecand how many a day?

I take 60mg of Dexilant twice a day and it's a once a day drug

Plus I take Pepcid 40mg twice a day. They won't give any more meds as this is past the limit but give no answers. I hope your husband has a good doctor. Good luck

Proton pump inhibitors (PPI) medication can be very helpful for Barrett's Oesophagus patients because they reduce the stomach acid that damages the oesophageal lining and may help with the heartburn pain.

With Barrett's you should have regular monitoring and if dysplasia develops you should consider radio frequency ablation.

Some people do not respond to PPI medication and may need specialist help and possibly anti reflux surgery. With pain, it would depend on the cause of the pain, but for most people any pain from Barrett's would be heartburn-related.

Man I wished my would go away by propping my head up I do at time get 2pillows. I tried to sneak in a Reese's peanut butter cup and NO it didnt get NY 😕 started spazing real bad. I hate I can't eat chocolate!

Robert I dont know how much pain he's in cause he dont do anything to Make it feel better. He dont take his pills he eats bad things as in real hot spicy food and DRINKS alcohol a lot of beer. I know there are a lot of beer drinkers out there bit why drink a fermatining drink and wonder why you gut hurts. Drives me crazy along with my issue of not eating at all till my body has to have it. Even my vidin goes haywire hope to eat soon again. I got real bad spaztic hut!

I have to question DJ-RN, or rather his Surgeon.  I am sorry. Your surgeon clearly feels it is reversible. We were told also that a Fundoplication is not reversible. However, the Heller's Myotomy can require repeating if the muscle grows/overgrows again and CAN be repeated as such and a Fundoplication can be ‘redone’ in that it can be tightened. 

We are fortunate to have one of the British authorities on this subject as my husband’s surgeon. He has performed many of these and this information above is from him. 

I agree with Carol.  

?Sometimes the fundoplication can give problems requiring it to be re-done, and I know somebody who has had this repeated, but I imagine that after a period of time, the fundoplication would adhere to the oesophagus and it would take a very brave surgeon to try and prise it away.   So the idea of the fundoplication being reversible, as distinct from being re-done after loosening through natural means, or tightened, is not one that anybody should rely on.

?It does sound logical that the length of the affected muscles that are cut in order to achieve the myotomy could later be extended, or repeated, depending on the surgeon being able to achieve access.

?I am unqualified in medical matters and these issues really are best dealt with by referring to surgeons experienced in the field who know the patient's particular circumstances.   There are a whole range of variations within an overall condition and it is perfectly understandable that a treatment that suits one person may not be appropriate for another, notwithstanding some differences in schools of thought amongst the surgeons.

Well I don't know what to say other than two different surgeons have told me a Fundoplication can indeed be reversed. To the degree I don't know. And, a GI specialist also told me it can be reversed.

DJ

Alan, I'm only telling you what two prominent surgeons have told me. I don't know anything about the time period they were talking. So as far as prying it away that may be after a point where it couldn't be. I don't make up information as I go. I was merely stating what was told me.

Maybe for a reflux patient but not a Achalasia patient? Maybe a difference? I don't know trying to figure this out as I go and really not doing a good job of it. How are you feeling?

They can also be very damaging to the kidneys. One wouldn't want to be on them unless the benefits outweighed the risks. And with Alchalasia they do. I'm on 40 mg of Prilosec a day. But I do know the potential risk they pose to my kidneys.

DJ

I'm doing quiet well now that I'm sleeping sitting up. I went yesterday and bought a new bed where the feet and head can be raised and lowered. I'm also on the books to have another Botox injection.

DJ

Hope you're feeling better.

I feel the same way Robert. I think they often tell you what you want to hear. I was just repeating what both surgeons told me when they said a Fudo could be reversed. I don't know if it can be or not and don't really need to know. 👍

As Alan has stated, and my first thought with this was that adhesions would immediately create issues. As an RN, you are clearly aware that tissue upon tissue, once stitched in place would create adhesions and this is a natural result.

No wonder that you are cautious of what you are told/what surgery is being suggested to you if both surgeons have told you it is reversible. As an RN, you yourself would realise that this would be problematic to reverse.

We all have individual experiences with individual surgeons and this is why it is crucial to share information so that we can cherry pick facts to enable us to make the best decision based on our set of circumstances. 

I agree. But again, I was just repeating what a GI specialist and two surgeons told me. There are many variables to look at. Theoretically speaking I believe that it is a reversible procedure only after a short amount of time. I understand fine, delicate tissue adhering to the other and how problematic it would be, if not impossible to reverse. But again, I didn't go into it with them about the specifics/time period and all other variables that would play a role in that statement alone. Personally, as a sufferer with this horrific disease and as a nurse, I wouldn't think that much time could pass if they were to try to reverse it, or if it could be reversed at all. I just don't know that much about it. I'm learning like all of you are. Just because I am a Cardio/Thoracic RN by no means makes me anymore knowledgeable on this disease as the next person. I appreciate your comment. It's exactly what came to my mind as well. How long are we talking in order for this to be done? I just wish everyone well and hope that each of us makes the best decisions we possibly can. I'm about to have my second Botox injection and just pray I get as long of relief as possible. You guys are great to talk to and I respect all of your opinions. Again, we've come together as one and have helped each other out tremendously. :-)

DJRN