Tag Archives: adult PS

Pyloric stenosis can be for adults too

Infant pyloric stenosis (“PS”) is not well-known in the general community.  Many of those who have been affected by it had never heard of it before.  But today far, far fewer people know anything about the adult form of PS.  As I will explain below, this is (in large part) a measure of the tremendous progress that medical science has been made in this area.

The stories of adult PSers vary even more than those about the infant form of the condition.  Some may find that hard to believe but it’s true!

When did problems start?
What happened?
What did the doctor say and do?
Did it work?
What happened then?
What choices did you face?
How are you doing now?

Think about it:  for adults there are many more variables than a baby might have: diet, wellness, lifestyle, age and stage of life…

However, the basic cause of all PS seems to be the same: high gastric acidity.  And one of the symptoms is usually the same: vomiting that is often severe and possibly (especially in infants) life-threatening.  But unlike babies with PS, adults usually find that with the loss of weight and wellness come pain, bloating, food intolerances, and reflux.  These are usually not a problem for the hungry but (at first) happy infant PS spitter.

In the infant disease, the high gastric acid level over-stimulates the pylorus (the circular muscle valve at the stomach’s exit), causing it to thicken, toughen and choke the muscle’s ability to relax and pass food.

Adult PS is also caused by high acidity, and often also by a virus, Helicobacter pylori, which has the nasty habit of stimulating acid secretion.  The acid erodes the stomach lining and creates conditions which enable the virus to trigger the eruption of gastric ulcers, which then scar as they heal, so thickening the walls of the stomach and pylorus, ultimately narrowing and blocking the stomach outlet.  If left untreated these ulcers can also give rise to stomach cancer.

These differences do not affect the name of the condition: “pyloric stenosis” means a “narrowing” of the pylorus (which means “gate”).  The term “hypertrophic” means “enlargement” and is only used of the infant form.  The effect of PS, whether in its infant or adult form, is also the same: the narrowing of the stomach’s exit ring muscle, causing vomiting and a reduction of food throughput, and thus starvation.

The difference in the development of the two forms of PS explains why the relatively simple “fixes” for infant PS (medication or surgery to relax the pyloric ring) do not relieve the adult form.  The PS baby’s problem is the thickening of the pyloric passage’s muscular outer layers, whereas the adult’s scarring, narrowing and blockage occur inside the pylorus or at the antrum, the tapered (narrowing) part of the stomach closest to the pylorus.

Adult PSers suffer from a range of symptoms including vomiting, reflux, pain, lack of appetite, and inability to maintain body weight.  The adult patients’ general practice doctors and GI specialists will try various treatments, starting with drugs to kill the virus infection and reduce acidity, and then relaxant medication and repeated stretching (“dilation”) to widen the pylorus.  Dilation is often repeated several times but is hardly ever successful as a long-term remedy. Sooner or later the patient usually decides to continue to one or more of a short list of surgical remedies, all of which are more severe and often less effective than the rather simple pyloromyotomy which is the usual surgery of choice for infants.

Pyloromyotomy

The simplest surgical remedy is pyloroplasty, a technique that was modified to become the pyloromyotomy which has usually remedied infant PS since Ramstedt’s accidental discovery in 1912.  Ramstedt discovered that it was not necessary to stitch the cut pylorus after he’d split it to relieve the enlarged muscle. He left the gaping pylorus wound to heal by itself in time.

[Select an image to enlarge it if you wish.]

Pyloroplasty

Pyloroplasty

But very sometimes the infant pylorus will not stay open for various reasons, and then a surgeon may return to the pyloroplasty: here the pylorus is split down to the mucosa or inner lining lengthwise (as in a pyloromyotomy); in a pyloroplasty the split muscle is then stitched closed across the pylorus, thus forcing it to stay open.  But because the adult pylorus is often scarred (thickened and hardened) by ulceration, it is often not in a fit condition to be modified.

Gastroenterostomy

The second option is commonly adopted: gastroenterostomy is a bypass of the pylorus by joining the duodenum to the stomach.  This removes the pyloric “gate” between the two which understandably has an effect on digestion.  Dietary changes and smaller, more frequent meals are necessary and often the “dumping syndrome” becomes part of daily living, as the body struggles to maintain a regular and appropriate source of energy.  Gastroparesis is a fairly common problem after gastric surgery: damage and interference cause the nerves and muscles of the region to stop working as they should.

So adult PS is more complex in its causes, symptoms, and available treatments.  And all these several treatment options are far from assured of success, as our gastric passage is easily unsettled and has a mind of its own: it is part of a complex network of different and linked organs and chemical input and processes.  Moreover, like any worker our abdominal organs can protest against being handled with less than good skill by working more slowly or sometimes a complete stop-work.  Medication and especially surgery can be quite successful or can result in unwanted and significant continuing physical side-effects.  Some “survivors” are pleased with the results of their choice, and others find they have to “adjust”, sometimes struggling to do so.

“Major adjustment” is thankfully something that is needed by only a very small minority of infant PS survivors.

Scared_DoctorDespite the dismissive words and comforting promises of pediatric surgeons, infant PSers also run a risk of a short list of abdominal and other complaints, some of them after their early surgery and then possibly also in later life.  Remember that the pyloromyotomy does not deal with the baby’s high gastric acidity.  One of the long-term risks is reflux and developing gastric ulcers – and as a result the adult form of PS.  Deja-vu!

But there’s also good news.  Today, thanks to effective and modern surgery, deaths from infant PS are almost nil.  And adults will find that with antibiotic treatment, H. pylori infection is usually quite easily dealt with, and so gastric ulcers and related surgery peaked in the 1970s and are now far less common.

Some personal observations are indicative despite being anecdotal rather than based on a careful study.  Adult gastric ulcers and PS used to be quite common but the only person I have ever known with it in 70+ years was one of my uncles – possibly a family linkage there?

Contrast this with the 8 or so infant PS patients and survivors I have known of or met.

The reader may discover similar figures!

Another measure is Facebook: following its PS Groups for 10 or so years I have logged 1,020 infant PS survivors but “just” 56 adults struggling with the condition of adulthood (several of tham after having survived infant PS).

The medical journal The Gut published an article in 2011 titled, The scars of time: the disappearance of surgery for pyloric stenosis – referring to the virtual disappearance of the adult form of PS, usually caused by peptic ulcer disease.

If medical science had made similar progress in reducing the incidence of infant PS, many parents and survivors would really party!

For readers who have stories or questions about adult (or infant) PS and its treatment and who use Facebook, I can recommend its “closed” Pyloric Stenosis Support Group which includes more than 50 members who have experienced and have posted (in great variety) about their adult form of PS.  (Any Facebook subscriber can find a “closed” Facebook Group, but it cannot be opened and read only by those who have joined that Group.)

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Pyloric stenosis in its adult forms (2)

My life’s work as a church pastor has made me aware of many of the common medical conditions, as I have supported people with all kinds of cancers, many heart, lung and “lower abdominal” conditions… ah, I don’t want to bore you with the long list!

Troublesome and also serious medical conditions often occur without warning and they can totally surprise the sufferer.  When I needed two kinds of eye surgery some years ago, I already knew a lot about the cause, the procedure, and the likely outcome.  My father had already charted my path, as had several people I pastored. 112

But most non-medical people have not had not worked with as many sick people as I have, and many health problems don’t have a clear family / genetic factor.  For these folk especially it can be very frustrating to be dealing with something life-affecting they know nothing about; quite likely they’d never heard of their condition before.

Adult pyloric stenosis (“PS”) is often like that: it is far rarer than the infant form (which itself is not exactly everyday), and it’s not always obviously linked with the family’s genes or the patient’s lifestyle.

A 1998 medical report deals with such a case.  For the details, please use the link to find and read the full article.  All three medical journal reports mentioned in this post are commendably available free and in full on the web! Points of general interest in this first article include –

  • It concerns a (then) 47 year old Caucasian woman, a rare thing as PS in both its infant and adult forms affects about 4 times as many males as females.
  • It seems this PS case was regarded as “primary” – without an obvious cause.  Several symptoms started only some 2 – 3 months before her surgery, and although she had suffered from dyspepsia and occasional vomitting she had none of the common precursors to adult PS: infant PS, adhesions, a gastric ulcer or cancer (which would have made this a “secondary PS”).
  • Examinations and imaging before her surgery showed little abnormality apart from reduced stomach emptying, but post-op pathology showed abnormal cell and muscle structure in the pyloric region.
  • The distal gastrectomy surgery involved removing the lower part of the stomach and the pylorus and attaching the stomach to the duodenum.
  • The report tells us there were no continuing symptoms, although it is not hard to find less concise and straightforward comments from other patients elsewhere!  This patient had severe surgery that should be expected to change digestive functioning.

Several other matters are also worth mentioning –

  • It is mentioned at several points that the cause of primary adult PS is little understood, and it is claimed that there are no statistics on how frequently it occurs.
  • Several surgical techniques are used to manage adult PS, all of which are more radical and affect digestion far more than Ramstedt’s operation which is the usual procedure used to remedy infant PS.

In light of this, I was somewhat intrigued to discover that a 1965 article (also freely available in full) contradicted several of the most significant assertions made by the previous report!  Specifically, Dr Milo W Keynes claimed that –

  • “Primary”, “uncomplicated” or “simple” PS in adults seems to have a genetic base.
  • It is far more common than is often claimed – but adult PS patients and those with a “simple” PS are less likely to seek treatment than the parents of a very sick baby.
  • “Secondary” PS occurs more often than the “primary” form, although reports of the incidence vary greatly from 10:1 to 3:1.
  • Many of the patients had struggled with symptoms for many years (e.g., 12 years), and sometimes from childhood.
  • There is a long list of symptoms, and these vary greatly.
  • Most patients were older, but the documented cases ranged from age 14 to 85.
  • Adult PS occurs in about 2 males to every female – different from the infant form.
  • It is claimed that “simple” PS can usually be traced to the patient’s infancy.
  • It is also claimed that it can be managed without major surgery: by the same muscle-splitting Ramstedt operation as done with babies.
  • However, at the end of the article it is stated that the preferred management of adult PS is by one of the much more severe surgical techniques, so that underlying disease such as a cancer or an ulcer can be dealt with if necessary and because the Ramstedt procedure does not always relieve the obstruction.  This leaves the patient and surgeon with a choice between gastro-duodenostomy, gastroenterostomy, gastro-jejunostomy, partial gastrectomy and pyloroplasty.

Another article in the same medical journal issue as the previous one reported on a study of 100 adult PS patients, all of whom had their diagnosis confirmed at surgery.  All but 3 of these were found to have a cancer or peptic ulcer(s): of these 3, one had Hodgkin’s disease, leaving the other two with a “simple” adult hypertrophic (enlarged) PS. Details in this report which are likely to be of interest to the reader of this blog include –

  • Although the symptoms of adult PS vary greatly, the core symptoms are common and quite unmistakable: vomiting containing food eaten a day or two earlier, loss of appetite, weight loss, and gastric splash (an audible sloshing sound in the stomach when rocked more than 3 hours after a meal).
  • A duodenal ulcer can cause obstruction without vomiting.

These two reviews of informative articles conclude a series of three on adult PS.

Interested readers might use the “Categories” box (top right of the “page”) to find other posts on this subject. DiscussionForum

Facebook members might join one or more of the largest PS Groups on this social web forum, as some 12 adult PS sufferers have shared something of their varied and common experience of this condition.  Several of these Groups are “closed”: all can be found via the Facebook Search box, but to encourage candid sharing only those who join can see their contents.

Pyloric stenosis in its adult forms (1)

Back in the 1950s I once overheard my parents talking about my oldest (and long deceased) uncle Fred having a stomach ulcer and an operation to deal with this.  I well remember being transfixed and “all ears”, but having been stonewalled so often, I dared not ask my parents the question on my mind.  At this time I would have been about 10 years old, and I had worked out that my prominent and hated belly scar was from an operation I’d had as a baby as I’d developed a blocked stomach (pyloric stenosis, “PS”).  I was keen to know whether my uncle Fred would be sporting a scar just like his young namesake.

When I got to see my uncle in swim-mode some 20 years later I was disappointed: his scar was small and almost invisible, far less disfiguring than mine.  Compared with infant surgery in the 1940s, abdominal surgery on adults and 20 years later was quite tidy.

Gastric ulcer01What I’ve discovered only recently is that infant PS and adult gastric ulcers and PS are often related.  All three conditions and several others have been linked with a high output of gastric acid and this is often a family trait.  So my connection with my namesake uncle (and hero) likely went further than our family realised!

The previous post sets out some of the important similarities and differences between the infant and adult forms of PS.  This post will outline two medical articles on adult PS.

The first report was published in 2010 and deals with the case of a 71 year old male who was diagnosed with PS.  The interested reader is encouraged to use the link to read the full article which has been kindly made available to the public.

In brief, it notes that adult PS is found in two forms.  It is often caused by other conditions: a long history of a mild form of the early version, an earlier stomach problem such as a gastric ulcer, or cancer.  Sometimes, as in this case, no underlying disease was found.  This 71 year old was unaware of any such factors, yet tests and surgery proved this man did indeed have a PS.  His symptoms of “abdominal distension, nausea, and vomiting” had been noticed for (just) 3 months.

It is noted that both forms of PS occur far more often in males than in females, and that they often recur in certain families.

The aim of this report is to be applauded: to raise the awareness of both forms of PS within the medical community, and especially the various ways it can appear in adults.

For those like myself and (I expect) most of our readers, the article’s value is also in its acknowledgement that the infant and adult forms of PS are related, and that the infant form does not necessarily end with a simple surgery.  Far from it, as many of us have well come to know!

The second report can thankfully also be read in full by those interested in more detail.  It outlines the very large decline in the number of adult PS sufferers needing surgery.  I have already posted about this.

downward_graph_smallThis study was undertaken by a York (UK) body gathering data on peptic ulcer disease, and based on the statistics from the 3 kinds of stomach surgery done on 4,178 adults and how often adult PS was found during these procedures.  The study ran from 1929 to 1997 and included people born between 1889 and 1959.  It found that the age at surgery fell during these years, that PS was found in 17% of the earliest patients, and just 3% in the last group, and that gastric surgery rates fell markedly (although not uniformly) during the almost 70 years under the lens.

These results show very clearly the effectiveness of modern medical treatment for stomach conditions and introduced in 1977; this includes acid-suppressing medication which reduces the formation of gastric ulcers and resultant scarring of the stomach wall and pylorus.  However, the reduction of PS numbers was even greater than the falling numbers of stomach surgery, suggesting that a number of factors are at work, including diet, medication, and the management of upper bowel disease.

Once again, the great majority of readers will be less interested in the medical messages of the report than in what it might mean for interested lay people.  What do these figures mean for you and me whose history includes PS?

  • yay1People who have had infant PS are less likely to have serious long-term effects than they might have expected some 40 years ago or earlier, thanks to better diet options and medication.
    The fact that today we can read much more about the long-term problems experienced after infant PS is a reflection of our access to the media, not of rising numbers.
  • People who have had infant PS are ever less likely to need further surgery for ongoing problems including a renewed stomach blockage. We need to be aware that for some of us these problems are significant, testing, and often interminable.
    The report states what many have found (as I have read), that surgical responses to such problems are not only severe but also likely to be ineffective in the longer term.
  • Peptic ulcer disease continues today but it now very rarely results in PS.

So – generally good news for the PS-aware community!  My next post will review several other medical articles on adult PS.

Pyloric stenosis: its infant and adult forms

This blogsite has mentioned several times that pyloric stenosis (“PS”) afflicts adults as well as infants.

Pain01Recently I devoted a post to the adult form, passing on the stories of several people who had related these on one of the Facebook PS Groups’ pages.  By using the “Categories” box at the top right of this site, interested readers can locate several posts that deal with adult PS.

The stories of adult PSers vary even more than those about the infant form of the condition.  However, the basic cause is the same: high gastric acidity.

In the infant disease, the high gastric acid level over-stimulates the pylorus (the circular muscle valve at the stomach’s exit, causing it to thicken, toughen and choke the muscle’s ability to relax and pass food. Peptic ulcer3

Adult PS is also caused by high acidity and often a virus, Helicobacter pylori. The acid erodes the stomach lining and creates conditions which enable the virus to trigger the eruption of gastric ulcers, which then scar the stomach and pylorus, ultimately narrowing and blocking the stomach outlet.  These ulcers can also give rise to cancer.

These differences do not affect the name of the condition: “pyloric stenosis” means “narrowing” of the pylorus (which means “gate”).  The term “hypertrophic” means “enlargement” and is only used of the infant form.  The effect of PS is also the same: the blockage of the narrow exit valve, starvation and vomiting.

The difference in the development of the two forms of PS explains why the relatively simple “fixes” for infant PS (medication or surgery to relax the pyloric ring) do not relieve the adult form of the condition.  The PS baby’s problem is the thickening of the pyloric passage’s muscular outer, whereas the adult’s blockage occurs inside the pylorus or at the antrum, the tapered part of the stomach closest to the pylorus.

Scared_DoctorThe main post mentioned above reflects the range of symptoms that adult PSers may suffer and the various treatments which are offered by GI specialists: unhappily adult PS is more complex in its causes, symptoms and available treatment.  All treatment options are far from assured of success and they tend to have unwanted and significant physical side-effects, far more often so than the great majority of infant PS survivors report.

Infant PSers have a much higher risk of a list of abdominal complaints after their early surgery and in later life.  One of the risks is of developing gastric ulcers – and the other form of PS.  Deja-vu!

But today, thanks to effective antibiotic treatment, H. pylori infection is usually quite easily dealt with, and so gastric ulcers are now much less common than they were in my younger years.

The next post will continue the stories of adults with PS.

Networking after pyloric stenosis (6): adult forms of PS

Pyloric stenosis (“PS”) is far more common in babies than in adults, and its cause is also easier to diagnose – despite many stories of doctors being far too slow to suspect let alone test for it.

In babies PS occurs in between 2 and 5 times in every 1,000 live births.  Adult PS is a well-recognized and documented condition, but (perhaps because there are two forms of it making it harder to diagnose) there don’t seem to be any significant (i.e., large study) statistics on its frequency.  I read in one report that there are only 200 reports of it in the English language, but this gives no helpful indication of anything much!

Two forms

  • Primary PS in adults is quite rare and most similar to PS in babies: it is marked by a thickened pyloric muscle narrowing or even blocking the stomach outlet, causing nausea, pain, vomiting and food retention with stomach enlargement. This condition is caused by hyperacidity which is caused by raised levels of gastrin, a blood hormone that triggers the release of gastric acid.  Why this should occur later in life is not well understood; it is suspected that higher levels of gastrin may have existed through life but for some reason start to cause problems only at a later age.
  • Secondary PS is also caused by higher acidity in the gastric passage, but in these cases the acidity helps give rise to stomach or duodenal ulcers and/or cancers. These or the scarring caused by healed ulcers then narrow the pylorus, causing similar symptoms.

This background will help explain why the diagnosis for adult PS is often a challenge to make and why the choice of treatment will also vary.

Both forms are linked with infant PS in that their symptoms are similar, they are caused by high acidity, there may be an obvious hereditary link, and they occur about four times more often in males.

My search of medical articles on this subject produced a 2010 report of primary adult PS in a 71 year old man that is well worth reading for the information it gives and on which I have drawn here.

This medical report is instructive, but the personal experience of sufferers of adult PS will also be valuable to readers here.  Once again in this series I also want to draw on the stories of some of these people as reported to the PS Groups on Facebook.

BNL  (born about 1990)
Did anyone get diagnosed with pyloric stenosis as an adult?  Mine is not completely closed apparently but that’s what the surgeon who did my upper GI said I have.  He prescribed Prilosec 40mg twice daily.  Does this really fix it?  Just really worried.
I would like to introduce myself and give you my story.  My name is B and my diagnosis of pyloric stenosis was just made yesterday.  It all started when I was about 15 years old.  I used to get intense abdominal pains on my right side after running in gym class.  Over the next 10 years I had gallbladder surgery in which they found nothing wrong with my gallbladder, numerous pain medications in which I’ve had bad reactions to, was put on antidepressants and being told the pain was all in my head just to have terrible reaction to that that impacted my life negatively.
It was not until I was given an upper GI yesterday to show that I had pyloric stenosis, however the doctor was able to fit the probe through and look at my duodenum however was told that I still had pyloric stenosis because it was so tight.  I have endured so many years of pain and nausea that it was depressing.  I have a 2 year old son and 1 year old boy girl twins in which my boy twin has a rare genetic disorder that causes seizures.  I have a busy stressful but amazing life that the pain makes impossible to keep up with.  I look forward to hearing your stories and also getting and giving support from everyone.  I very much appreciate being part of this group and don’t feel so alone anymore.  I have included a pic from my endoscopy to better explain what I mean sorry if it’s TMI but I have gotten a lot of questions lately about this.  Again thank you!  Any advice is appreciated!…
Please help!  I am desperate!  Is there anyone here or knows someone with pyloric stenosis?  I am being yanked back and forth being told that I do, then don’t have pyloric stenosis.  Since I posted last my symptoms are getting worse.  I am nauseous all the time, severe right upper abdominal pain that is just below my ribs and I can literally feel a ballooning feeling there, I am losing weight and am feeling very tired and fatigued all day.  Apparently my esophagus is inflamed, my stomach is hard, biopsies came back normal, nothing on CT scan except for some most likely benign cysts on my liver and some fluid in my cul-du-sac.  Sorry if this is tmi, but I am at my wits’ end.  Was sent home on Prilosec 40 mg twice daily, they refuse to give any pain meds but tramadol which I refused now because I can no longer handle the side effects.  I called my surgeon’s office and told them I need a straight answer and treatment because I can’t handle this.  They gave me some “katafate” for my chronic ulcers although I was told I didn’t have one on endoscope.  Please any advice is appreciated.  I have three kids at home, one with special needs, and I can’t function like this.  Thank you so much and sorry for such a long post.  Below is my endoscope pics again if anyone can give their input.  Thank you!…
My surgeon confirmed that I do have pyloric stenosis and wants me to see him sooner than planned since my symptoms are persisting despite being on Prilosec 40mg twice daily and carafate four times daily.  (2014)

MM  (born 1991)
I’m a 23 year old female currently undergoing treatment for adult pyloric stenosis.  I was wondering if anyone on here was the same or if the cases on here are for infant PS, as I’m finding it difficult to obtain information on the adult version as it seems extremely rare compared to that in babies.  Any information or help would be appreciated, thanks…
Yeah, I think I’m like the 1 in a million person who has it (but classic symptoms)…
I have so far had two balloon dilatations via upper endoscopy.  My surgeon doesn’t want to do keyhole surgery f it can be helped.  The problem is they don’t know what’s causing my PS,  I have extreme ulceration of the duodenum which isn’t improving with certain PPIs like omeprazole.  So he’s looking at other things like dissolvable stents etc., but yeah, they can do keyhole surgery as they do in babies.  Rather than the older method of the larger scar.  It’s a waiting game with me, haha! …
I’m just glad to get a doctor who listens to my symptoms.  He’s been so great.  The keyhole surgery performs the same surgery as with the large scars but with technology advancements they don’t need to slice anymore.  But it should be the same surgery performed…
TB wrote in response to this:  … I was diagnosed with adult p s about 10 years ago.  And there really isn’t a lot of information out there for adults with PS.  I’ve had a balloon dilation probably about 15 times.  My doctor doesn’t want to do the keyhole surgery either because it is very dangerous on adults.  I get them done every 3 to 6 months with the dilation and that seems to keep things under control…

Other adult PS experiences including these have been shared on other web forum sites –

SG (born about 1979)
hi there im a 32 year old female with pyloric stenosis. i know this is very uncommon in adults and finding it hard to find any information. i have no family history of this and had no signs of it as a child. i got diagnosed with the condition about 4 years ago. i had severe abdominal pain and could not keep solids or liquids down. this lead to kidney problems from been dehydrated. it took over a year to find out what was wrong and during the process the doctors also found crohns disease and endometreosis (no family history of these conditions either). my bowel, uterus, and pelvic wall are all fused together so symptoms of all my conditions are hard to define as they all affect each other. i have had 2 balloon dilations on my stomach to stretch the opening. i would like to know if any one has information on whether it is common with no family history or common with the other conditions. also if anyone knows any useful advice on this condition which may help me.  (posted on Healthboards 2011)

NN (age not given)
I decided to start a blog so I can update people, raise awareness, and just get out my story when I’m having a rough day.  I have had many people messaging me to ask what has been going on in the past month making me end up in the hospital and on so much medication so this is an easy way to answer everyone :).  Right now I’m just living in the present moment and taking each day at a time.  I don’t know what tomorrow will bring or when I will start feeling better, but just have to try and be positive and get through it.  Here is my story…
My story

I have lived my whole life with a nauseous stomach and constant vomiting.  When I was three my parents said I would grab my stomach and cry.  No pediatric gastroenterologist or pediatrician could figure out what was wrong.  They all just attributed my stomach problems to stress and eventually abdominal migraines.  And that I just happened to get the “stomach flu” more than anyone else.  I thought feeling really full after eating a small amount was normal. The older I got I started having horrible side pains on top of the nausea. My mom and I went to the pediatrician when I was 19 and after having tests to rule out appendicitis, told me to try some yoga. I was appalled…was I really that stressed to cause this many health problems? I had vomited every week of my life almost… on road trips, my first communion, from eating too much (or rich of foods), in stores, and on field trips.  I threw up during a final my freshman year of college and was back to finish it right after. I spent my life in emergency rooms and urgent cares for iv fluids and nausea medicine.
Finally, I had enough and needed an answer or at least give it another try.  I found a gastroenterology clinic in Lone Tree and just happened to get Dr. Patt as my GI.  I remember my mom telling me to not get my hopes up when we had my appointment during winter break of my sophomore year of college.  But Dr. Patt surprised us and has been such an amazing doctor and even inspiration since then.  I got a blood test for celiac disease (gluten intolerance)… negative.  I had a gastric emptying study (GES) which came back abnormal.  For the GES I had to eat oatmeal with radioactive substance and take pictures of my stomach every so often.  Dr. Patt also did an endoscopy (boy was I nervous!) and saw my pyloric sphincter (muscle between my stomach and small intestine) was so small and tight.  Pushing the endoscope through made it bleed… he had never seen such a case.  Of course I had to be a rarity!  My diagnosis was adult pyloric stenosis and gastroparesis.  Pyloric stenosis is almost always only seen in infants and requires emergency surgery because the pyloric sphincter is completely closed leaving the food in the stomach resulting in vomiting.  My pyloric sphincter was big enough just to let a tiny bit of food through but not nearly enough.  Gastroparesis literally means paralyzed stomach and the stomach does not move correctly to get food through.  This is why I was always so full and vomiting so frequently.
I tried reglan (a stomach motility medication) which made me worse because my stomach was trying to empty against an almost closed opening.  Dr. Patt injected botox in the summer of 2008 in my pyloric sphincter with the endoscope to relax the pyloric sphincter muscle.  Wow it made such a difference!!  I was able to eat and live normally for a few months.  But of course I started getting worse again.  Botox was injected once again in January of 2009, but no such luck that time around.  The last option was surgery to widen the pyloric sphincter.  I went to a general surgeon who had never heard of pyloric stenosis in adults.  I later learned there is only one other reported case of primary adult pyloric stenosis so no wonder no doctor had heard of it!  I had pyloromyotomy surgery which is what they do in infants.  The surgeon cut some of the layers of my muscle.  He made his incision in my abdomen and was shocked at what he saw.  My pyloric sphincter had grown so much scar tissue and was extremely inflamed from food trying to push through my whole life.  My liver and gallbladder had attached itself to the pyloric sphincter with scar tissue… scary I was living like that!  This inflammation was causing me side pain and low-grade fevers.  He described cutting the layers as trying to cut a wad of rubber bands.  He also had to cut off the liver and gallbladder.  My stomach got punctured in the process and I woke up in the hospital with an NG tube (to vent my stomach) and on a liquid diet.  I had many complications with the surgery… ileus (intestines stop working after abdominal surgery) and a gross infection.  It would all be worth it though in the end I kept telling myself.  I would be cured I thought… little did I know…  (posted 2011 as a blog)

NN (age not given, source not recorded) –
I was diagnosed with this in the beginning of February after it was revealed in an endoscopy exam.
I had the same problem in April 2008 after complaining of abdominal pain. The doctor at that time was able to dilate the pylorus using a balloon.
In November I started getting abominal pain again after eating, bloating, vomiting if I ate too much (3 meals in a day), feeling of fullness, growling and gurgling noises at all times, etc.
The endoscopy showed the pylorus was now so closed that the doctor said he was afraid to dilate it due to risk of perforation. He said that 1 or 2 dilations is ok but when it keeps shutting the only option is surgery, especially since I’m young.
I saw a surgeon and he said that he would not perform a pyloroplasty (cutting the pylorus and widening it) because he believed the area is now scar tissue. This condition is found mostly in babies and it’s rare for someone my age to get it. As a baby the tissue is still soft and will heal well, but at my age he said there is a risk for the cut to not heal properly, and then I would be dealing with a ton of other problems.
He wants to do a gastroduodenostomy and create a connection between the stomach and the small intestine to bypass the pylorus. I’ve read about the complications of this procedure and they scare the shit out of me, mainly the dumping syndrome that arises from undigested food entering the small intestine as well as the possibility that bile from the gallbladder may enter the stomach and cause irritation.
Does anyone know if the pyloroplasty can be done at my age? The complications for that are much less problematic, no dumping syndrome, no bile leakage, and it is the preferred procedure for babies.
I would much rather get a pyloroplasty done but the surgeon I spoke with does not want to take that route. As of now, if I eat really small meals, avoid thick, tough breads or foods, and make sure I chew my food, I still get a little bit of pain but I am not vomiting and I can get through the day. I would rather hold out to see if the pyloroplasty is a viable option than jump right in and get the gastroduodenostomy.
Also, if you know a forum where I could get some really good advice, I’d appreciate it. I’ve read articles regarding this procedure (yay for college library access!) and the pyloroplasty has been performed on adults in the past with success, so that is a light of hope for me.

PS (born 1984)
I’m 22 yrs old and for the most part I have had a very healthy and happy life until this past January where I started having what I call the “rotten egg burps.”  I went to my primary care dr. and was diagnosed with having ulcer like symptoms and I was put on prevacid.  Shortly after in early march I started throwing up after eating.  To make a long story short I went to the gastro dr. and convinced that I was having ulcer problems he told me throwing up was normal and I got sick every day for three months dropping 30lbs.  I was hospitalized for dehydration after passing out where after several tests it was determined that I had a gastric outlet obstruction.  I had about six endoscopes with dilatations that proved unsuccessful and the burping came back almost instantly.  I decided to have a second opinion done and found that after about six more dilatations I bled internally and the talk of surgery began.  The prognosis was pyloric stenosis which apparently is very common in children but almost unheard of in adults.  The drs. felt that I could possibly have annular pancreas after doing an MRI and scheduled an exploratory surgery that would take care of whatever was going on.  I don’t have annular pancreas.  I have pyloric stenosis and no cause for it.  The first surgeon I talked to said no to surgery unless I had gone to Boston or New York to have studies done on me because I am so rare, and yet the surgeon at the same hospital went ahead and did it anyway.  I had a pyloroplasty and the side effects are dumping syndrome and I guess sometimes anemia.  The procedure could have been done laparoscopically but because they had intended it to be annular pancreas they cut me from under my rib cage to below my belly button.  I don’t mind the scar although it upsets me that further tests were not done to know for sure and lessen the area affected with scars.  I don’t have the burps but I still have an awful taste in my mouth from something that is unexplanable.  I was also told in passing that they detected H-Pylori in me at one time or another but all biopsies were negative for Crohnes and cancer. It’s been two weeks since the surgery and I’m on an emotional roller coaster. I’m angry that I have something this rare that no one knows what to do I feel like I’m the experimental patient and honestly that’s what I am. I don’t really know what I am trying to get out of this post but I guess at this point anything will help.

Are infant pyloric stenosis and gastric ulcers linked?

Some 40 years ago, one of my uncles had surgery for a gastric ulcer.  When he visited us I found that his scar from that operation was not unlike mine from pyloric stenosis (PS) surgery when I was a newborn.  But little did I think there might be much more of a connection.

In the first sections of his part of the book to which I devoted last week’s post, and which Dr Ian Rogers and I have co-written, Ian lays the foundations for understanding the link between infant PS and a duodenal ulcer.  Here is the first part of my summary of Dr Rogers’ comprehensive report – for the medically untrained (and isn’t that most of us reading this?)  And do get a copy of our book if you’re interested in more details.

This is information I want everybody with an interest in PS to have!

Personal history, gastrin and acid

Rogers Ian frcs frcp (Small)When Scottish medical student Ian Rogers graduated and started his work in 1970, duodenal ulcers (DU) were common and dangerous, and related research was prominent.  At that time it had become well-known that the ulcers were caused by over-acidity in the stomach which was triggered by the hormone gastrin, the release of which was in turn caused by food reducing the acidity of the stomach.  When acidity rises, the release of gastrin stops, and thus balance is maintained.

It was also known that DU patients produced more gastrin than usual after a meal (especially when the gastric acid had scarred the stomach’s outlet or pyloric passage), that males produced more gastrin than females, and that 5 male DU patients presented for every one female.  It was found that the vagus nerve (the hunger messenger) also raised gastrin secretion.  In those days the role of Helicobacter pylori (or H. pylori) bacteria and the possibility of antacid treatment were unrecognised, and surgery was the accepted answer to a DU: the vagus nerve was immobilised and/or the lower part of the stomach was removed.

Because the drug atropine blocks the vagus nerve it was used to test whether a vagotomy would be effective in reducing stomach acidity.

All the above classical features of a DU are similar to those of infant pyloric stenosis (PS), including what was already described in 1921 as the PS baby’s “voracious appetite”: being ravenously hungry immediately after vomiting (not normal behaviour after bringing up a meal!).

The important role of the bacterium Helicobacter pylori was not yet recognised in 1970: it has infected 80% of DU patients, creating an alkaline environment in the lower stomach to protect itself from acid and also stimulating acidity.

Ian Rogers lists 11 telling clues about babies who present with PS, and yet the cause of the condition remains (it is claimed by most) unknown today.

History

In 1921 Dr John Thompson reviewed what was then known and done about PS – the details are most significant but too many to detail here.  Most importantly he (1) identified 3 common levels of PS, the acute, the ordinary and the very mild, and (2) reported on how PS could often be managed medically (and without surgery).

Dr Thompson also mentioned two theories about the cause of PS: (1) that is was caused by an abnormality of the pylorus, and (2) that the pylorus was being stimulated to malfunction.  He favoured the 2nd theory, citing the work of an 18th century anatomist on what causes muscles to hypertrophy (over develop).

Next:  My journey 

PS book coverWhenever I asked my parents about the weird marks on my stomach their answer was limited to “A doctor did that because you were a little bit sick when you were a baby”.  Yikes, I hoped I wouldn’t get “a little bit sick” ever again.  So as a child and ever since, I’ve been in the hunt for a better answer.  But apart from one-paragraph entries about pyloric stenosis in encyclopedias and medical handbooks, there was nothing.  So in recent years I’ve been passing on what I’ve learnt about PS issues by blogging (“Surviving Infant Surgery”).  And when the British professor of pediatric surgery Dr Ian M Rogers invited me to contribute “My Story” to a small book he was publishing about his conclusion after a lifetime of work – that all the symptoms of PS agree with it being caused by over-production by mother and baby of a gastric hormone, the result was: “The consequence and cause of infant pyloric stenosis: two personal stories”.  I hope that this modest book will go viral and make it onto many bookshop, library, and doctors’ shelves.  Interested PSers, parents and others can find out more about the book by googling for the title – and get a copy if you have a spare €24 for a medically priced publication – lol.  Anything I get will go to medical research into the diseases of infancy.

Understanding adult peptic ulcers and infant pyloric stenosis

As a boy I remember my heart churning and racing when I overheard my parents talking about my namesake uncle in the Netherlands having a peptic ulcer and facing surgery for this.

Although my parents would never tell me much about the kanji-like scar on my belly from an infant pyloric stenosis operation and didn’t tell me much about my uncle’s problem, I immediately sensed some kinship.  It was obvious that I had also had a stomach operation… maybe my uncle Fred and I would have similar scars?  Migration to Australia had taken my family half a world away from our Dutch relatives so I assumed I’d never find out.  In the 1950s people didn’t talk about such personal and medical matters, and they certainly didn’t post “selfies” on Facebook.

However, two decades later my uncle visited us in Australia and during a hot summer day’s outing my young family together with my parents and Dutch uncle and aunt enjoyed a swim… and I had confirmation at last!  Despite the usual male body hair I had a glimpse of my uncle’s similar vertical scar.  (It looked tidier and a lot smaller than mine!)  Of course I kept my deeply unloved scar hidden and didn’t dare to talk: I was too choked with shame and other emotions.  Besides this, I was around age 30 and my uncle was 43 years older than me, and I’d come to know him as a friendly but very distinguished man.

I treasured but filed the link away and thought little more about it until 30 years later…

Genes baby1When I started researching PS and related issues on the web in 1997, I soon learnt that sometimes there is a genetic element causing it, and that although a family link is not discovered in the majority of cases, five genetic loci have been identified as having possible links with the development of PS.

I have not heard of anyone else on either side of my family tree having PS and this suggests that there is no clear genetic factor in my story.  There are a few other recognized risk factors which do apply to me: male, firstborn, Caucasian, and maternal stress make a substantial list.

But more recently I discovered a small number of medical journal articles reporting infant PS and peptic ulcers occurring in adulthood in the same families.  Contact me for links if some case stories would be of interest.

Peptic ulcer3And then my web searches yielded a 2007 article written by a retired professor, Dr Ian M Rogers – and since added to by an interview and another article.  Dr Rogers has proposed hyperacidity as the key cause of PS and as the consistently demonstrable link between virtually all that we know about the symptoms and incidence of PS.  His theory also argues that hyperacidity explains why in some families PS can occur in babies and gastric ulcers in adults.

A search of the web will show that Dr Rogers and others have been studying the link between gastric acidity and PS since at least the 1970s and that Dr Rogers’ thinking and knowledge of this have been developing throughout his professional life.  Again, contact me for links if you are interested.

Peptic ulcers of the stomach, pylorus and duodenum are now a very much reduced threat thanks to the discovery that the presence of a bacterium, Helicobacter pylori, was usually needed to develop an ulcer from hyperacidity.  H. pylori is now treated with antibiotics in most or many cases, avoiding the need for a surgical remedy.

This gives me a new line to explore when I reconnect (as planned) with my Dutch relatives in 2014: apart from my uncle’s peptic ulcer is there evidence of hyperacidity or even PS in my Dutch family?

Some readers may be more than interested in building a similar knowledge of their genetic legacy!

The answers to this line of information will help my family and other present and future generations to –

  • better understand the risk profile of their family members,
  • simplify and speed up the diagnosis and management of PS in infants and of hyperacidity and its possible damage in adults, and
  • reduce the likelihood of PS developing to the extent that surgery is considered or recommended for babies, whether actually necessary or not.

The more we know and understand about PS and its related issues, the more we can do to manage it with less cost, upheaval and trauma for baby and parents alike.