Category Archives: Medical treatment

Networking after pyloric stenosis (3): Diagnosis

Parents of babies afflicted by pyloric stenosis (“PS”) are routinely assured that after surgery their little treasure will be “a different (and hugely improved) baby”.  Often this is immediately true: instead of a baby vomiting him- or herself to death, PS babies often thrive and quickly reach the top of their percentile range.

But of course this is so only if the operation was –

  • without complications (like infection or a hernia), and
  • successful in opening up the pyloric passage (which it usually but certainly not always is), and
  • if the baby is not left with years of GERD (reflux) related problems, a rather common outcome.

Many with a personal experience of PS, whether as parents or survivors, do have ongoing problems –

  • during the immediate recovery period,
  • during the child’s first years and sometimes continuing lifelong, and/or
  • in adulthood.
  • These people often resent that the medical advice they received
  • denied these possibilities,
  • left them with false expectations that” all would be well”, or
  • skirted over the future  possibilities embedded in a list of “to be mentioned” items the parents had to acknowledge and sign before surgery.

And when problems did arise (sometimes within days, sometimes after some weeks or years), the typically rather obvious link with abdominal surgery was denied despite several research reports to the contrary.

Diagn palp.jpgAmong those who report having had a bad time with PS however, no group is as numerous or angry as those who experienced a diagnosis debacle, and in this post we listen to just a few of these parents, again from the several PS Groups on Facebook.

A L was born about 1985 and had a son in 2009
I was born with PS and my second child, a son, William was diagnosed with it at 4 weeks.  Luckily, having had it myself, I was well aware of the odds of my child having it, especially a son.  The pediatrician as well as the ER wasn’t extremely willing to diagnose PS until I advised them that I had also had it.  Prior to that, I got quite a bit of attitude and snubs…
It is very disturbing to me reading others’ posts that something that is so easily rectified is so easily dismissed.

E D, born 1984
It feels so nice to know I’m not the only person to have had this condition!  Even now doctors, midwives etc don’t know what PS is: awareness needs to be raised!  I was one of the unlucky ones and had my operation in 1984.  My mum had to fight with the doctors as they wouldn’t diagnose me with PS as I was a girl!  I had my operation at 3 months when my weight was lower than my birth weight – thankfully I’m here to tell the story!  Have had two little boys and thankfully neither of them have had it!

arrogant doc5L G – son born 2014
My son had PS at 5 weeks.  The doc said it was reflux, even though the health visitor said it was PS – because he was born at 36 weeks he said he couldn’t have it that young.

M G – son born 2010
My son was diagnosed at 5½ weeks old with PS in 2010.  It took doctors 5 days and numerous times of me arguing till I was sent to a new hospital to find out what he had.  My son almost died but had his surgery and soon bounced back.  I am so thankful he is better.  But I wish hospitals and doctors would check babies closer for this kind of condition instead of it being fobbed off as reflux all the time.

C L – son born 2014
My son had PS and had his operation at 5½ weeks old after me refusing to leave the doctors as they just kept prescribing gaviscon for reflux.  He had key-hole surgery and was instantly better!
Our surgeon told us our son had been left that long his body had used all its fats and was about to start using up its muscle.  Thank God he was seen when he was.

M K – daughter born 2013
self-harmMy daughter, now 10 months old, had her surgery at 5 weeks old, was misdiagnosed by 5 different doctors over a 5 day period and had 2 negative upper GIs… she weighed 6 lbs at birth and at 5 weeks old was 4lbs 10oz after no doctor would test her for PS.  We almost lost her from dehydration before I could find one that would… all because she was my second born daughter and they said [that the] odds were PS was not the cause for her projectile vomiting!
She is now experiencing bad reactions to milk.  Took her to the doctor and they had no explanation other than possible allergy symptoms from sensitive stomach MAYBE related to her having pyloric stenosis as a newborn.  But they have no clue.  I thought it was all behind us, as those few weeks back in May were the worst I’ve ever experienced.

D M – son born 2014
My son is 16 weeks old and had surgery for PS at 4 weeks after 3 days in a row of me going to the doctor, 3 days in hospital and then still saying it was a virus!!!  Long story short he had his op but still has some ongoing issues.  I have joined other groups and it is clear to see a lot more research needs to be done for PS.  The whole “normally happens in first born sons and everything returns to normal after 48 hours” is just not true in all cases!!!

F M son 2014
My poor boy has not been able to feed for over a week due to “reflux”, the hospital said.  I had doubts and have had him here 3 times (currently back in as we speak).  Finally after him losing 9 oz in 3 days, they have listened to me which I tried to get them to do the first time I brought him in – which was a week today.  I have said all along I thought he has pyloric stenosis but they brushed it off as reflux.  Brought him back today as no improvement with gaviscon and ranitidine.  They did an ultra sound and what does he have?  Pyloric stenosis like I said 1 million times.  My poor boy has been ill and sick after every feed, massive amounts and then made constipated with gaviscon and it’s not even f***ing reflux.
I am so angry it took 5 minutes to diagnose today and should have been done last week but they fobbed me off.  Now having to go to another hospital so he can have surgery a s a p to have the issue fixed.  Can’t believe they let it go on so long without listening to me and checking for this earlier, my poor boy has been through hell because they messed up and he has been made worse because they didn’t believe me.  Upset, angry, and relieved we finally have it confirmed.
Ladies, if you think something is wrong don’t give up, and keep pushing them… if I hadn’t we would have been sent away [for another week] and he would then have been treated for cow’s milk allergy, and God knows what could have happened because he hasn’t been getting anything from his feeds.
Can’t believe they wouldn’t listen to me and I knew what was wrong.

J S
arrogant doc4I had pylorics, so did my son and 16 other family members; unfortunately one did pass away but that was back in the 50’s.  And doctors are still saying it’s not hereditary.  lol  I think our family has proved them somewhat wrong. lol
Total fools!  The thing is, it was on my side and my partner’s side, as I said, 16 members, but [my son was] still diagnosed with gastro reflux for 3 months even with this history.  Total joke!
16 members across the family had PS, and we saw the symptoms straight away.  This stems over 55 years: my son is the most recent case and I was the 10th member but female, so not diagnosed as soon as usual.

L S – son born in 2007
My little boy had pyloric stenosis.  He was diagnosed at five weeks after a hideous time of not being taken seriously by the doctors.  I went back for the third time and refused to move unless they saw us again and self-diagnosed.  Finally they took me seriously.  Literally moments after his op he was a different baby.
He’s seven and a half.  Very pleased, yes.  It’s taken this long to talk about it though.

E T – son born 2014
My son is 2½ month old and 2 weeks ago we noticed small changes in his behavior.  Not as many poopy diapers, acting colicky, constantly hungry like [he was in] one really big growth spurt.  He was never a spit up baby, but a week ago he started projectile vomiting.  We went to his pediatrician twice, only to be told he had a very nasty virus.  We were told to give him clear fluids for 24 hours and the virus should work itself out of his system.  He then started vomiting blood… lots of blood-filled vomit.  We went to an after-hours clinic and they sent us to the hospital because he was dehydrated.  We went to the hospital, they did a blood panel, x-ray, administered fluids via IV, and sent us home with zofran.  The next morning he was still vomiting blood so we went back to the hospital and they took an MRI and discovered he had PS and referred us to a children’s hospital for the operation to fix it.  During the operation I cried, but I cried more after the operation since he had to wait 8 hours before he could eat anything and could only have ½ ounce and was crying uncontrollably because of hunger pains.  My heart hurt for him.  He is doing much better, being 4 days after his surgery!

Doctor woman makes a warningS Y – self born in 1985 (& father 1953)
I was born in Dec 1985 and by the second day I was projectile vomiting every time I was fed, and sometimes after my parents thought I was done I would go again for another round.  My parents both knew that something was wrong with me, my dad remembered his parents telling him what happened with him (they thought he had PS but it turned out to be a tumor bouncing up and down in his stomach)… My parents went to my pediatrician and he said that there was nothing to worry about – all babies throw up.  Then they went through 3 other doctors and they got the same story and a few even told them that the likelihood that I had PS was slim to none, this went on until I was 15 days old and my mom and dad decided to take me back to the hospital I was born at, went into the ER and found the doctor that saved my life.  He was a pediatric emergency surgeon.  I was in surgery within a few hours of being admitted so that they could get all the tests and get me prepped for surgery.  By this time I had lost 1 lb, which was huge considering I was only 6 lb 5 oz.  I was released just in time for my very first Christmas.
After my parents recuperated from my ordeal they talked to that surgeon and found out that if they were to choose to have another child that child most likely would have the same thing I had.  They talked and couldn’t go through it again.
My dad had his surgery in 1953…

The awful stories above would be understandable if PS were a rare condition.  But it is not.  The incidence varies a bit, but in developed countries ranges between 2 and 5 in every 1000.  This means most of us would know several people who carry a PS story.

It must also be recognised that PS quite often (but far from always) takes a week or more to become “full blown” and able to be clearly diagnosed by touch, x-ray and ultrasound scan.  And the health system as well as parents would not take kindly to an unnecessary surgical operation on a baby.

Yet the message is clear from the above stories and hundreds like it on the web.  It is also a simple message that should not be impossible to learn and remember, especially by the highest IQ endowed people in society.

  • Arrogant shirt1Too many parents get “attitude” from their GP and pediatrician: patronising condescension and dismissiveness, even when the parents have done their homework, have PS in their family or personal genes, and find their baby’s weight loss has become serious.
  • One would hope it is true that today’s trainee doctors are being taught more about people skills.
  • Many doctors seem to wait far too long before ordering tests, resulting in too many PS babies being near death and possibly damaged for life by hunger and dehydration.
  • From the countless available stories it seems few doctors advise seeking a second opinion or refer a baby to somebody more knowledgeable.
  • One would hope that those who are humble and self-aware do one of the above – with the result of no traumatised and angry parents writing to a forum site!

This blog has several posts on the treatment of PS by medication with atropine or Ranitidine, a non-invasive option that is 100% safe for PS babies born full-term and older than 2 weeks and is standard practice in several developed (but non-English speaking) countries.  Find these posts using the “Categories” box at the top right.

Parents who strongly suspect their newborn has PS have very good reason to insist on their doctor giving them respect, time, and clear explanations of their advice.  It may be helpful for them to take a supportive person along to the consultation.

Understanding infant pyloric stenosis (Conclusions)

In the previous few posts I have overviewed and “translated” in some detail the major and professional medical section of a small book recently published by Dr Ian M Rogers and myself, with the descriptive title, The consequence and cause of pyloric stenosis of infancy: Two personal stories.

The reason for this series of posts?

At least some of the survivors of infant pyloric stenosis (“PS”) and their parents will be interested to know and understand this condition better, as we keep being told that “we still don’t know what causes it”.

Let’s face it, “knowledge is power”, and how many parents don’t wish they knew more about PS when they are suddenly and horribly faced with it?  What causes this?  What can and should I do?  How do I look after my sick baby when I feel the doctor fobs me off? Can my beautiful baby avoid surgery for this? What are the possible short- and long-term side effects?

Although I have never had a child with PS, I had it as a baby and wish I had known what I know now much, much earlier.  In the past this was virtually impossible, but the power of the web has changed this completely.

True, there are still some areas that are not fully understood, but it’s also true that drawing on a lifetime’s work with PS, the retired professor Ian Rogers is able to give us a clear and (it seems to me) compelling explanation of what does in fact cause PS.  The fact that he has written his part of the book using a lot of medical language must not keep his knowledgeable and compelling explanations and rich insights from those of us most affected by PS!

In this the last post of this series, I look at the last three of 6 questions which are often regarded as summing up the mysteries of PS, but which can in fact be answered in the light of Ian Rogers’ discussion of the discoveries that together explain PS.

4   Why is PS more frequent in the first born?

first-time-mom2First-born babies are cared for by cared for by first-time mothers.  PS babies are hungry and vigorous, ravenously hungry (at first), and unusually, the condition does not leave them feeling nauseous but immediately voraciously hungry for more.  Understandably but tragically, this gives the stomach and the acid-caused work-enlarged pyloric muscle no rest; an experienced mother will be more inclined to give feeding a break.

Mention is made of a 1962 article by Dr N M Jacoby, who compared two groups of 100 babies treated medically and surgically for PS, with one baby (only) of each group dying.  Of the medically treated children, Jacoby stresses the importance of (1) relative under-feeding, (2) the drug involved (atropine) being carefully used in relation to body weight, and (3) regular stomach washouts.  Two of these cautions can be linked to the hyper-acidity theory.

It is often mentioned that PS occurs far less frequently in under-developed countries: this also fits the theory, although more factors may be involved.

5   Why does pyloromyotomy, and not gastro-enterostomy, cause the tumour to disappear?

There are reports about 52 year old patients whose pylorus was found to be still enlarged after a gastro-enterostomy surgery in infancy to overcome PS; after pyloromyotomy the pylorus returns to its normal condition after several weeks.

Unlike gastro-enterostomy, pyloromyotomy disables the muscle ring and widens the opening temporarily, which breaks the cycle of its becoming enlarged by overwork.  Gastro-enterostomy was used before pyloromyotomy was discovered and is still used occasionally: it by-passes the pylorus which removes the immediate feeding problem but the pylorus continues to be over-stimulated by an over-acidic stomach.

6   Why does PS present at around 3-4 weeks of age?

Dr Rogers mentions two possible reasons.

It could take some weeks for higher than usual acidity to enlarge the pylorus sufficiently to make a clear diagnosis of PS possible.  But if this were so, we could expect other problems caused by hyper-acidity to arise at this time – and they don’t.

More likely is that the switching mechanism between alkalinity and acidity is more immature than usual during the first weeks.

In evolutionary terms, the benefits of the normal high acidity in a baby during the first weeks (incl. guarding against microbial attack as mentioned earlier) outweigh the negative of a few babies with hyper-acidity having problems!

Other lines of enquiry

1   Genetics

genetics1Genetic studies have shown that more than one gene is involved in PS, and that the condition is multifactorial: it can be caused by any one or more of several factors.  This is confirmed by studies of identical twins who are more likely to share PS than non-identical twins or siblings but far from always.

It has been found that PS infants tend to have higher birth weight, and (anecdotally) they may also become more athletic!

2   Chemistry of the pylorus

Prof. Rogers gives three reasons why the reports of chemical abnormalities of the pyloric muscle (the presence of growth factors and the absence of other chemicals) are theoretically attractive but do not stand up to scrutiny.

3   Infection

The analysis of swabs taken from the nose and throat of babies have shown no abnormality in PS babies.

A previous post has mentioned links between hyperacidity in PS babies and in adults infected with a stomach bug, Helicobacter pylorus, which can trigger gastro-duodenal ulcers.  But several studies of PS infants have not discovered any H. pylorus infection.

Dr Rogers remarks how strange it is that none of these studies links the hyperacidity triggered by a H pylorus infection with the hyperacidity that is part of PS.  Strange indeed!

Conclusion

Dr Rogers sums up:

Constitutional hyperacidity coupled with developmental hyperacidity begets pyloric contractions which begets work hypertrophy which begets IHPS.  IHPS begets further hyperacidity, and so on.
Maternal anxiety in the novice mother means that the hungry but vomiting baby is frequently fed with more pyloric contractions and more work hypertrophy – and a bigger tumour.

Wryly, he adds that it seems there is nothing new under the sun, and that “we are almost back where we started”.  In 1921 Dr J Thompson already proposed that the pylorus spasming and overdeveloping through over-work were the cause of PS, and still earlier, in 1903 Dr W Freund had suggested that excess hydrochloric acidity was a key factor.

The future

Dr Rogers urges that his theory is “perfectly testable”.

  • It is well known that PS babies’ serious loss of acid through their vomiting must be remedied before surgery, as their alkalosis (excess alkaline in the body) causes dangerous hazards during and after the operation.
  • ranitidine01Adults similarly affected by vomiting and acid loss are quickly and effectively treated with drugs to reduce the excessive release of acid.
  • Successful medical treatment of babies with PS is regularly reported: intravenous atropine is used to rapidly reduce the size of the pyloric tumour while the malnutrition and chemical imbalance are corrected.
  • Surgical treatment is now more prompt with a drug (cimetidine) that rapidly corrects alkalosis.  The author mentions an as yet unpublished report that this drug when given to mild cases made surgery unnecessary in 16 of 17 cases.
  • Another powerful acid blocking drug, Ranitidine, has also proved to be very effective in avoiding surgery when PS is recognized early; it should also be useful when surgery is not safe or accessible.  Dr Rogers then briefly outlines several other avenues of treating PS safely and medically.

He concludes his comments on considering non-surgical treatment by adding:

Such a pre-operative strategy with babies with IHPS is long overdue. It should not come as a surprise if we find that such temporary treatment promotes a lasting cure. 

Understanding infant pyloric stenosis (3)

Infant pyloric stenosis (“PS”) is a condition that may affect babies during their first months, and it is regarded as the most common life-threatening affliction of early infancy, affecting between 3 and 5 babies in every 1,000 in “Western” countries.  It will usually starve the infant to death unless treated, either by surgery or medically.

ponderStrangely, we are usually told that the cause of this malady is unknown.  But is it really?

In the previous 4 posts I have summarised the main points made by Dr Ian M Rogers in a recently published small book, The consequence and cause of pyloric stenosis of infancy: Two personal stories.  Ian is a retired surgeon and professor of pediatric surgery, and his book enlarges on his argument in several medical articles which are based on a lifetime of observation, research and experience with PS.

Dr Rogers believes that the cause of PS is not a mystery, and that surgery is often not needed to remedy it.

This post continues a series and overviews the first three of Ian Rogers’ six conclusions, all based on the discoveries and facts which he has presented.  I have outlined Dr Rogers’ book in lay terms to make it accessible and understandable for non-medically trained parents, PS survivors and others interested in this subject.

Having set out the information which I have covered (selectively) in the previous posts, Ian Rogers comments that in 1970 his career put a halt to his work on PS, but that he returned to it in the 1990s, when several basic questions called for answers he believed could be given.

1   What makes some babies develop IHPS when normal babies do not?

Without entering into the more complex areas of the medical science that Dr Ian visits and must be considered to answer this question more fully, “the bottom line” is that inherited hyperacidity would be an answer to this question that fits what we know, although some details remain to be discovered.

Dr Rogers mentions several other known facts that add support to this answer, even explaining why some babies develop PS well before the usual 3 weeks to 3 months “window”, and the well-known link between PS and “O” blood.  Of course heredity as a key factor also explains the frequency of PS in certain families.

Babies with normal gastric acid secretion do not overwork the pylorus despite the raised levels of the hormone gastrin during the first post-natal weeks.

2   Why do male babies predominate?

Male dominance1The 4-5:1 male dominance of PS runs parallel to the incidence of gastric ulcers, well known to be linked with hyperacidity, and males having a more active parietal cell mass (PCM), the acid secreting part of the stomach.

Male babies have also been shown to secrete almost twice as much gastric acid as girls during their first 10 days.

3   Why self-cure with the passage of time?

Release of the hormone gastrin is at very high levels after birth, normally peaks at between 10 and 17 days, and then falls to the equivalent of adult levels at 3 – 4 months.  In normal babies raised acid levels continue for about 4 weeks, thus protecting the baby from microbe attack.  But in PS infants, while gastrin release starts to fall, acidity continues to rise and the sensing that normally allows the stomach to switch between acidity and alkalinity is overwhelmed.

Pyloromyotomy (the operation to remedy PS) disables the pyloric ring, immediately blocking its overwork and allowing normal peristalsis and acidity and alkalinity to return.

The traditional medical treatment of PS in babies and adults was based on the belief that the muscle was not hypertrophied (enlarged through overwork) but spasming, and cutting the vagus nerve and administering the anti-spasmodic drug atropine, combined with regular stomach washouts were used to reduce acidity and spasms.

Effectively, these measures (together with the relief of dehydration and the restoration of healthy blood chemistry) allow babies to survive until they are old enough to outlive the natural raised acidity levels of the first post-natal weeks.

Next post –
Dr Ian Rogers answers three other “obvious” questions about the character and “unknown” cause of PS.

Understanding infant pyloric stenosis (2)

This post is the 4th in a straight series in which I have overviewed the section of a book in which the retired Scottish professor of paediatrics, Dr Ian Rogers, recounts what he has learnt from a lifetime of the observation and study of infant pyloric stenosis (“IPS”).

How digestion works

Dr Rogers describes the process of digestion that occurs in the stomach.  He describes the stomach as having two parts, each with a distinct structure and function, and the stomach’s three stage role in digestion.  During these stages the pylorus contracts and relaxes, early to allow fine and easily processed food to pass, after which the stomach continues grinding and breaking down the coarser food to chyme (or pulp) before the pylorus allows it to pass.  He concludes that the contraction of the pyloric ring muscle is associated with feeding.

This raises the question: how would this process work in a baby?  Dr Rogers points out that –

  • Milk in the stomach raises alkalinity which triggers the secretion of gastrin and acid.  This in turn activates the pylorus, and if acid secretion is higher or feeding is in greater quantity and/or more frequent than usual, then the pyloric muscle is overworked and over-develops.
    IHPS causation - I Rogers
  • Artificially narrowing the pylorus in rats stimulates the development of the stomach’s acid secreting lining, thereby increasing even more the stimulation of the pylorus.
  • Studies have shown that gastrin-induced hyper-acidity is indeed a symptom of IPS.
  • In 1976 Dr J A Dodge reported that he had generated PS in 28% of 84 puppies after injecting 20 bitches with a synthetic form of gastrin; it is known that gastrin crosses a dog’s placenta and stimulates acid secretion.  Still more puppies developed PS when they were injected after birth.

Dr Rogers concludes from this that the pyloric muscle is clearly the culprit.

  • Gastroenterostomy03Disable the pylorus by splitting the muscle and its enlargement soon disappears; bypass it (which the gastroenterostomy operation does) and the enlargement remains.
  • This also shows that although the gastric hyperacidity may be hereditary, the cause of IPS is not a hereditary tendency for the pylorus to be enlarged.
  • The only cause of pylorus enlargement that has been found is repeated contraction.
  • Pathological study has found no abnormality in the tumour tissue.
  • The erythromycin phenomenon also confirms this conclusion.  Erythromycin is a macrolide antibiotic widely used to treat bacterial infections, but when taken during pregnancy or by a newborn it has been found to cause a 7-fold increase in the incidence of IPS.  This group of antibiotics works like the hormone motilin which increases stomach activity and contractions of the pylorus when the stomach should be empty after it has done its work: an empty duodenum (as with IPS) releases motilin.  Some details of the function of motilin are not yet fully understood.

Other indicators – clinical aspects

  • Diagn palp.jpgIPS is normally recognized by a doctor using the standard test meal for a baby who is vomiting profusely without bile in the vomitus:  gentle palpation (feeling while stroking) will reveal the swollen pylorus (the “pyloric olive”) in some 80% of cases, and peristaltic waves can be seen moving from left to right.
  • Adults with a duodenal ulcer caused by hyper-acidity can be treated by ranitidine (or in the past by atropine sulphate), a drug that blocks the release of acid, thus raising the alkalinity of the stomach contents.  This suggests the possibility of also managing IPS by using a drug that reduces the secretion of acid in the digestion process.
  • As mentioned, a baby who inherits a high gastric acidity added to the raised acidity levels normal in early infancy can develop uncontrolled hyperacidity which will over-work and over-develop the pylorus.  A first-time and understandably anxious mother who continually re-feeds an obviously hungry baby will amplify what happens.
  • A decline in both IPS and sudden infant death syndrome (SIDS) in babies who sleep on their back has recently been reported in Sweden.  The back sleeping position results in feeds moving with gravity, making gastric emptying easier and faster: this would also seem to confirm hyperacidity as the cause of IPS.

In the next post, we follow Dr Rogers as he applies the information in these four posts to the classic characteristics of PS in babies.

Can pyloric stenosis be a mild case?

How long is a piece of string?  The variety of ways infant pyloric stenosis (“PS”) can raise its horrible head is like answering that hoary question.

Almost two years ago I posted an attempt to answer this question,  It is not only a post still well worth reading if you are asking yourself this question.  The post had several responses which underline how frustrating and kaleidoscopic it is to answer several of the questions about PS.  Let me run through the main parts to my answer again here.

Pyloric Stenosis or pylorospasm?

Whether there is a significant difference between Pyloric Stenosis and Pylorospasm has been argued at least since the early years of the 20th century and it is still debated in medical circles.  Those favouring the distinction describe Pylorospasm as a spasming of the pylorus triggered by the nerves and resulting in the delayed emptying of the stomach – but nothing much more .  It is agreed that true PS involves the thickening and hardening of the muscle band of the pyloric ring, and when advanced enough this would not let any food pass into the duodenum, usually with fatal results.

However, others have preferred to see Pylorospasm as the beginning or a milder form of PS.  There is a difference, but it may only be clear from an ultrasound study, as this article explains and illustrates.  Another useful article may be read (although only in summary) here.

From my simple and probably simplistic description it should be clear that Pylorospasm is not serious enough to warrant surgical and perhaps not even medical intervention, and that a full PS will be fatal unless there is some form of intervention.

Baby sick2 (Medium)To add to the confusion, PS is often termed “Pylorospasm”.  I well remember that in my native Netherlands the condition I had was called “pyloruspasmus” even though it was clearly a full-grade PS!  I find that the same confusing interchangeability continues in at least several different languages today.

The bottom line is: some misbehaviour of the pylorus is mild enough to not warrant surgery.  Not all projectile vomiting is deadly, so doctors will sometimes await further developments.  This is not to be confused with the fact that many doctors are accused of belittling parents, not taking time for careful diagnostic questions and tests, and brushing off parents with talk of reflux – all this when the baby has lost significant weight and condition, and even when the parents mention the family’s history of PS.

Many cases of PS do not go to the operating room

Google “I had” together with “pyloric stenosis” and you will find articles, forum comments and posts that support the above statement.

There are many mentions of babies with what was clearly PS and whose condition was responsibly judged not to be severe enough to justify surgery.  But these people in later life continued to battle with PS-like symptoms for many years – or lifelong.  Some eventually sought surgery (usually more major than an infant pyloromyotomy) and some did as best they could by managing their food intake and distressing symptoms.

Pyloric stenosis can continue to “grumble” whether or not there has been surgery

As I have stated many times, many doctors promise parents that a safe, simple and quick operation will solve all their baby’s PS problems and that there are most unlikely to be any after-effects.

Surgery can be very successful in treating PS but it can also lead to short-term or lifelong symptoms which seem to be similar to some extent to mild PS: reflux and IBS, sensitivity to gagging and vomiting, overweight or chronic under-weight, etc.

Mild PS which is not dealt with by surgery is sometimes not treated at all or is treated with medication such as Atropine sulphate or Ranitidine.  Either of these two courses may also lead to lifelong and troublesome gastric misbehaviour.

It seems beyond the ability of medical people to know whether a particular set of borderline or mixed symptoms should be diagnosed as PS, mild PS, pylorospasm, or something else.  It also seems to be impossible to predict the future course of any case of PS, whichever way it has been treated or not treated.

A thorough and substantial research project or two would be so valuable to obtain a clearer picture of the consequences of various levels and treatments of this rather common condition in babies.

Medication can treat many pyloric stenosis cases successfully

In most “Western” countries, the medical treatment of PS with Atropine sulphate or Ranitidine has usually been given no or scant consideration.

These two drugs relax muscles and allow the pylorus to relax, reducing its swelling and allowing it to open and shut without surgical intervention, but because of their effect on all muscles they must be introduced over several days and under careful medical supervision.  This course of treatment has been used for many years by pediatric specialists in many countries, and a recent Japanese project found its use succeeded in avoiding surgery in almost every case in the trial.

Parents who could or should consider medical treatment for their baby must 1) know the symptoms of PS well, 2) have gathered strong evidence of their baby’s condition and perhaps have had previous experience with this malady, 3) have a baby who is not close to death, and 4) are willing to learn how to use the medication.  They may also need a supportive person to stand by their side as they may need to insist on being heard and helped by their doctor.  It must be realised that the safe use of medication requires early diagnosis, a baby who is not frail or newborn, and a baby whose condition allows the several days necessary for the drug to take effect.

Interested parents should use the “Categories” search box to the right of this page to find and read posts like this one on “the hushed up alternative to PS surgery.

Pyloric stenosis surgery’s possible long-term effects

One of the things that so often angers me as a survivor of infant pyloric stenosis (“PS”) is a key assurance that doctors so often give the parents of these babies (as reported by them) and so common on information websites –

Superman MD1“PS and the surgery for this condition have no long-term effects.”

If only it were true!

While this is promise may be true for the majority of sufferers (and please note: “may be true”), it is at least a gross generalisation.

The facts are –

  1. Web forums such as Patient UK and Topix, several blogs devoted to this and related subjects (see the Blogroll to the right), and online social pages such as Facebook include people’s accounts of unhappy experiences.
  2. In 17 years of researching this subject area online, I have not found even one substantial medical research report on the long-term effects of PS and the pyloromyotomy (PS surgery).  There have been several small and narrow studies based (say) on 10-15 years of following-up those having surgery in a particular hospital, but many of the problems people have are much more long-term and yet seem to be linked with the condition and/or operation.
  3. It is significant that my 2011 post on this topic is the 6th most read on this site.  A total of over 50,000 visits hardly represents the majority of the world’s PS people – but it’s not a trivial sample either.  More than half of my 160 posts to date have given significant attention to this subject: interested readers can find them (and their titles) by clicking on the “long-term effects” tag to the right.  These 86 posts include some written about my own struggle with the long-term effects of PS and the operation I had in 1945.

A post I wrote recently surveyed much of this subject, under several sub-headings.  This post will restrict itself to a somewhat more personal “take” of how others and I have been affected by PS.

Adhesions

As mentioned in recent posts, post-operative adhesions may affect seriously only a small proportion of those who have had abdominal (and other) surgery. I have corresponded with survivors who are afflicted with adhesions and I have found their stories heart-rending.  Everyone develops adhesions after PS surgery, but if they trouble you, the pain, complications, untreatability, side effects and uncertainties are almost always never-ending.

So why does the medical profession dismiss the documented and significant risk of adhesions as “a minor risk” when in most cases PS surgery can be avoided?  Why aren’t parents empowered to understand and work through the facts and make their own choice?  Why do information sites and powerful medical staff so often and/or effectively present surgery as the only real choice?

Post-traumatic stress disorder

PTSD is another area of known risk that is shrugged off by the medical establishment (including its training, practice, policy, research, publications, administration, etc).

PI know and am grateful that much more is known and done about PTSD today than in even the fairly recent past.  But knowledge does not nearly always translate into knowledge-based practice, especially when any degree of self-interest is involved.

In Australia we plan to spend 4x as much on the centenary of a World War I conflict (Gallipoli) as we’ll spend over the same 4 year  period on the support and rehabilitation of our military who have returned home from recent scenes of conflict with PTSD.  In some countries and circles, male and female circumcision are still routinely or commonly practised, both with and without analgesia (pain control).

Even when parents follow this practice for religious or family tradition reasons, it is inexcusable to dismiss the use of pain management.  To many outside the USA, it seems there may well be a link between the prominence of emotional and physical violence in US society and the prevalence of male circumcision.  Who can know how much depression, home and public violence, suicide and other signs of dysfunction are the result of PTSD recognized or unrecognized as caused by infant surgery?

It is not hard to trace the link between my PS surgery in 1945 and the relatively mild PTSD that has dogged me for much of my life.  Most infant surgery past and present causes a lot of trauma:
– the condition itself and
– the all-too-frequent effects of tardy diagnosis,
– the desperate needle-sticking and perhaps a cut-down to establish an IV line,
– awake intubation,
– separation from parents,
– pain and
– hospitalization.
To this we must add the relayed effect of what most parents tell us was their most traumatic experience ever.

Until the late 20th century, most of these factors were magnified: especially the maternal separation and hospitalization lasted for weeks and sometimes months.  To this we must add: awake surgery with a paralysing drug and intubation, often without even local pain relief and the “comfort” of a sugar cube laced with alcohol.

All this would not matter if we could still maintain today that “babies do not really feel and certainly don’t remember pain” – as many did until the 1990s.  But in 1987 Dr Anand proved that to be nothing more than wishful thinking, and much has changed since then – but far from everything that needs to change.

Again: how can doctors today maintain that the surgical remedy for PS is free of long-term effects?  Why aren’t the issues around this condition and the surgery considered responsibly and parents given the missing facts?

My personal story

ponderNot for a moment have I ever thought that everybody who has had PS and/or infant surgery will have struggled as I have.  For many years I dealt with my troubles alone, but with the coming of the internet I have been able to link up with and learn from others with the same past – and all kinds of stories of their personal journeys.

Like some and unlike others I grew up in a secret world of –

  • self-obsession (I’m sure I wore out a few mirrors and certainly wasted loads of time),
  • self-injuring (I learnt a lot and am thankful I did myself no lasting harm), and
  • searching libraries for anything to explain my scar and what caused it.
  • I discovered that I was fearful of especially doctors but also of anyone I regarded as in authority, and became passive-aggressive, internalising my anger.
  • Although I have functioned quite well in my work and relationships I am also known as the sole reserved, introverted reclusive in my immediate family of seven and even my extended family.
  • I love the water but at the pool and beach I never felt able to relax and enjoy myself except actually in the water.  For many years when out of the water I would keep my arms tightly folded to hide my scar from curious eyes and tug my shorts or swimmers up to my chest, forever annoying my mother who kept reminding me that this looked ridiculous – which I didn’t really care about as much as…
  • I lied, denied and pleaded ignorance when people did ask me what “that” was on my belly or what my scar was from.  I avoided phys-ed classes at school, sports and overnight camps which involved changing or showering in public, and my face blushed and heart raced whenever I heard any of a short but telling list of words.

way-forward-signIn one of my earliest posts I wrote at greater length about how I feel PS and my operation have affected my life. Whilst I have learnt that I’m not typical it’s also been reassuring to discover that I am not unique and a freak.  This is just one of the reason I urge parents considering surgery:

“Spare the knife and don’t spoil your child!”

And to any PS survivor “with issues” I say:

“Shit happens, but don’t let this spoil your life!”

When pyloric stenosis is hard to diagnose

Scared_DoctorA 1975 article on the difficulty of diagnosing some cases of infant pyloric stenosis (“PS”) underlined the sympathy I have for medical doctors: these important professionals have to be across thousands of symptoms and medical conditions, on good days and less easy ones, and also across the latest findings and journal reports… and up to dealing with upset or just plain difficult people, and with their receptionists and colleagues.  Is it any wonder that we, the general public, tell and retell many GP and MD stories that are not all that flash?

Mum w baby01This blogsite includes several posts about this, warning parents of sick children to –
1) do their own homework,
2) be your child’s best and well-informed advocate,
3) take somebody supportive if needed to what may be a difficult consultation,
4) stand your ground if it is solid, and
5) if all else fails, get a second opinion, go to the hospital Emergency Department and find another doctor.

Far too often I have quoted or commented on the stories of some parents of PS babies are outrageously out of order.  See the “Topic Categories” box (upper right of this page) to find some of the stories of parents and PS patients (at the list’s bottom).

Having mentioned the sad and bad stories again, I have also written about the complexity of diagnosing some cases of PS (see “Pyloric Stenosis – Diagnosis” in the Topic Categories).  The 1975 report I mentioned above deals with four unusual but not abnormal PS cases, and this report has told me more than it actually says.  Click the link above to read the entire and (to me at least) interesting 1975 report which I will overview and comment on here.

It is stated of 3 of the 4 babies that their PS showed itself quite clearly within two weeks of their birth.  All 4 were eventually operated on – but not until well after the normal “window” of “3 days to 3 months” for the majority diagnosis and treatment of PS.  As I understood the report, the youngest was almost 4 months and the oldest 9½ months at surgery.  What happened?

Q mark1All four babies showed inconsistent and unusual symptoms of PS –

  • their vomiting was unusual, sometimes subsiding or intermittent,
  • although all failed to thrive and gain weight normally, they never fell below their birth weight;
  • gastric peristalsis (muscle movement across the upper belly after feeding) was often weak or absent and didn’t cause the usual cramping, pain or distress;
  • the swollen pylorus could not always be felt (“palpated”) and did not always show clearly on x-rays.

ponderOn the other hand –

  • their vomit was bile free, showing their stomachs were blocked,
  • changing or thickening their milk or formula and keeping them upright after feeding did nothing to remedy their condition, and
  • medication with antispasmodics to rule out pylorospasm (in which the pylorus spasms but does not thicken and block the passage *) and confirm gastric obstruction brought no relief.

Yes, my complaint about doctors’ poor attitude and diagnoses has been often and clearly stated on this blog.  But in the light of this report it should be clear that –

1                    When there is a family history of PS, when parents are experienced, when they show they have done their homework, and when several of the symptoms of PS are clearly present, a wise doctor will agree to immediate further tests, with good reason (see below) although this is something that is commonly resisted.

2                    When there are some symptoms suggesting PS but not enough evidence for a diagnosis of PS or another condition it would also seem sensible to run further tests.

3                    When no clear diagnosis of PS is possible it is necessary to eliminate other abdominal conditions including pylorospasm from the doctor’s considerations.  This may be done by changing the feeding regime (as mentioned above), trying an anti-spasmodic medication, conducting further tests, and/or by waiting for a week or so and then if necessary repeating some of the tests.

4                    Waiting will be difficult for anxious parents of a small baby who is losing weight and condition.  A good doctor will understand this and convey empathy as well as working through what such parents may need to watch out for and do in the interim if necessary.

Father compassion01e5                    Again, if the doctor is not supportive and seems negligent and especially when the baby is clearly losing condition (losing significant weight, not soiling diapers / nappies and not urinating, appears drowsy and is sleeping excessively) it is high time to bypass the family doctor or paediatrician and insist on the local hospital taking responsibility – and urgently.

What eventually did help the four babies in the report was the comparison of their earlier with more recent x-rays: there are several typical radiological (x-ray) signs of PS, all of which are not always evident or clear from one series of images, but do become so by comparisons.

What did I learn?

1                    The report shows clearly that when it is not possible to diagnose PS with confidence (and what parent, patient or doctor wants unnecessary surgery or medication?) a careful process of tests, management and observation will clarify the real problem.

2                    PS manifests itself and can develop in a variety of ways.  Although most cases arise along textbook lines, there are occasionally exceptions to the “rules”.  Case 3 (a 7 month old girl) is the clearest example of this: her case should be read in full by interested readers.  Her PS was evident at 10 days but seemed insufficiently developed at her first pyloromyotomy at the age 7 months and she needed a second operation at 9½ months when a fully developed PS was found.

3                    It will be clear that these four cases were different from other groups of PS sufferers, including the “late onset” infants who first show PS symptoms after age 3 months, and the mild PS cases who are not considered as needing surgery, perhaps treated with medication, and sometimes continue to live with the signs of mild PS, to the extent that some of them seek a pyloromyotomy later in their childhood, teenage or adult years.  There are numerous stories from and about such PS sufferers on the web.

4                    Once again there was a loud silence in this 1975 report about the medical treatment alternative for PS babies.  This blog has often urged that medical management of PS be tried before surgery is even considered.  The “Topic Categories” box (top right of page, “Pyloric stenosis – Medical treatment”) will give the links.  Medical treatment of PS is of course slower to take effect than surgery, but it controls the condition in 70-90% of cases (depending on which report you read), makes good use of the time lag that is necessary to eliminate the possible diagnosis of pylorospasm, can always be set aside for surgery when necessary, and is non-life-threatening and non-traumatic for both baby and parents.

M820/00925                    Parents can learn from this 1975 report and (I trust) my post about it.  It underlines the need for parents to be as well-informed as they can be: to be their baby’s best possible advocate, they will know about and understand the symptoms of PS, will ensure that their medical consultants are exercising their duty of care, and will be willing to “change horses” if necessary.
This may sound presumptuous and “a big ask” of distressed parents, but as I have explained, it is far from impossible, especially with the help of a suitable supportive family member or friend.
Realize that the life of your little one may be at stake.
Today, medication, anesthesia and infant surgery for PS are almost always safe.  But from what appears on the web, the negligent delay, diagnosis and treatment of PS are the most frequent killers of PS infants.

*  What is the difference between pyloric stenosis and pylorospasm?
Narrowing of the pyloric canal is a common finding in infants with chronic vomiting, and it is most often due to pylorospasm.  In such cases, there is no thickening and hardening (hypertrophy) of the pyloric passage, causing it to narrow (“stenose”), and the spasms (contractions) of the pyloric muscle can be treated by modifying the feeding technique and/or with an anti-spasmodic drug.
When the vomiting persists, a question arises as to whether it is due to pyloric stenosis.  Distinguishing between the two conditions is difficult but important, for while spasm can be treated medically, true stenosis (physical narrowing) may require a carefully managed course of medication or surgical intervention.
Some believe that surgery is the only remedy for true pyloric stenosis and that all cases that respond to medication only had pylorospasm; this has been rejected in the light of studies of diagnosis and treatment.