I may well have been known as “Fountain Fred” within 10 days of my birth. Or, because I was actually born in the Netherlands, “Free de fontein“. I had pyloric stenosis (“PS”) almost from birth: not just the “wet burps”, “baby puking” or “a bit of reflux” that all babies have.
This blogsite is about a weird condition that causes several tiny bubs in every 1,000 to vomit violently and uncontrollably, how this condition and infant surgery have affected me and others, and how PS in babies can be recognized more promptly and treated better than they and their parents so often are.
If you’ve experienced any of this, please consider sharing your story too, below my story here or on the “About” page, under one of this Blog’s posts, or on one of Facebook’s busy PS Group pages. Any comment or contact is most welcome: it may well be therapeutic for the reader and will certainly add to the wider awareness of this horrible, all-too-common but hardly known and often poorly diagnosed condition.
On this “page” I give an overview of what PS looks like and how it’s treated, as well as something of my story. Click on an image if it’s too small for you to get its message. Many of these images are taken from the web and have been used in my posts.
This is what pyloric stenosis looks like (the image is from a medical video on YouTube) . . .
Ewww… yuck, Fred!
PS is popularly dubbed “projectile vomitting”: it is so forceful that milk shoots out, often in an arc and for 1, 2, or even 3 metres (and some report, “even 30 feet” (that’s almost 10 m) and “even to the ceiling”! Small wonder this affects the parents so powerfully that they instinctively know: This is not normal “baby sick”.
In 1945, even grainy b/w photos were hardly affordable to my War-weary parents… and in those times people didn’t talk about ugly things, let alone capture them on camera, so the web image of another hapless baby above here has to remind me that PS is a truly scary condition that can end a baby’s life within days.
I was born in a small “city” (with just a few thousand inhabitants but city status) in the northern Dutch province of Friesland. As happened there in the 1940s, my proud father paid somebody to announce my birth to all the town notables… my parents were so proud and pleased.
It is hard to imagine how devastated my parents must have felt when the violent vomiting that is PS started a few days later. Soon it was clear that I was vomiting myself to exhaustion and death when only a week old. The violence of PS puking can cause bleeding in the stomach, create a hernia, and damage the young and fast-developing brain.
This set of photos is not me either, but tells me that it would have caused great interest and consternation when my stomach performed like this… This is another clear sign of PS: you can see the stomach muscles trying to push food (waves of peristalsis) through the overgrown and blocked pyloric valve at the end of the stomach.
“Pylorus” is Greek for “gate”, and in PS, the stomach’s exit gate ring-muscle (A) becomes thickened (B), narrowing (‘stenosing”) and then often blocking the stomach’s exit passage. The cause is too much acid in the passage over-stimulating the muscle; all this is still only partially understood, but has been carefully explained in articles and a small book by the retired Scottish Prof. Ian Rogers. His research has shown that the hormone gastrin which stimulates acid release into the stomach is the prime cause of PS. The list of possible culprits that increase gastrin release is lengthy: several genes are involved in about 20% of PS cases, but other common perpetrators have been shown to include erithromycin (a frequently prescribed antibiotic), maternal stress and anxiety, milk chemistry, over-feeding, formula feeding, sleep position, smoking, and other factors.
Recent reports implicate bottle feeding as a risk factor, and a formula’s chemistry is different from breast milk. Some earlier reports have claimed that bottle feeding reduces the risk of PS, perhaps because it made quantity easier to control. Some stats say older mothers are at greater risk of having a PS baby, others claim to have found a youthful first birth is more at risk, and yet others say maternal age is not a factor. Some reports have prematurity as a risk factor and others not. In other words, some risk factors are clearly established and others are still debated, even after several hundred years of PS being recognized.
Again, not me! I am thankful that most babies don’t get to this awful degree of weight loss, hunger and dehydration. Just look at those arms and the neck. How I wish my Mum and Dad had taken some more photos, journalled, or just talked about what happened to me back in 1945.
The distressing web image below also shows the dangerous malnutrition (hunger) and
dehydration which PS sometimes causes before it is diagnosed and managed.
Sunken bones making up the baby’s skull (see right) are seen more commonly and are also a real danger sign. This early starvation can cause lifelong damage, especially to some of the brain’s functions.
Having recently discovered this I have noted that several of my skills are clearly below those of my four siblings…
Parents get very angry and frustrated when doctors stall in deciding on what’s become obvious to them, especially if they themselves had PS or if it’s in the family, and/or after they’ve done their homework. Doctors used to go by the usually clear physical signs of PS and (if known) the family history. Now (judging by countless reports) too many belittle parents, talk about “alternative diagnoses” and wait until costly technology can be brought into play (ultrasound and xray tests etc) – all in the name of “the scientific approach” (although one may suspect that the cashflow of the medical industry may also play a part).
Judging by the many reports online, PS babies all-too-often get to a very distressing and extreme state before they are diagnosed. There are several possible causes of infant vomitting, and it seems many surgeons will not diagnose PS or even order tests until the other possible diagnoses are eliminated.
Because of this, parents of a suspected PS baby are well advised to do some important “homework” to help their doctor decide on the correct diagnosis as quickly as possible.
1 Read what’s on the web or in books about infant PS,
2 Keep records of your bub’s doings, and
3 Use social media to compare notes with parents with PS experience.
4 Have your partner or a close friend support you rather than face a doctor alone.
This will empower parents to if necessary confront their doctor or ER staff with hard facts. All parents can become unnecessarily anxious, but usually a mother knows very well when her baby is losing weight and condition, and that this is serious. There are far too many stories on the web of parents being brushed off with, “Doctor knows best”, “All babies vomit”, or “You’re just a typical over-anxious mother”.
Is it any wonder there are so many stories on the web of parents despairing of their doctor and taking their dying baby to the local hospital’s Emergency Dept? There, to add insult to injury, it seems to be typical that they’re told: “If you had brought your baby in any later, (s)he wouldn’t have made it through the night.”
This workmanlike guy deserves pride of place here: the German Dr Conrad Ramstedt doesn’t look like an eminent surgeon when judged by today’s dress codes, but back in 1911 he discovered and in 1912 (over a century ago now, yay!) he published the simple remedy for projectile vomiting. And … he’d discovered it partly by accident, partly through good observation and thinking. One of my posts is about this (and there are several more about this pioneer’s impact: find them using the Categories or Search box).
I owe my life to Dr Conrad Ramstedt and am deeply grateful to him. Without my early op I wouldn’t be here 70 years later, let alone writing this!
Another and more distinguished looking photo of Dr Ramstedt. He started his medical career in the German Army. It looks like he suffered some collateral damage – mebbee in a sword fight in Cavalry training? “Can I stick to medicine, Colonel?”
This 1950s series shows how the Ramstedt pyloromyotomy for PS was (and often still is) done, by open surgery.
What this doesn’t tell us is that anesthesia for babies was primitive and dangerous until the 1960s, and because of this it was often avoided until the late 1980s, something that has had lasting effects (psychological issues incl. PTSD) on some of us who had infant surgery before the 1990s.
My PS was remedied in the Netherlands in 1945, and at that time (my research suggests) only some babies were given light ether, and that only if somebody with the rare and needed skill to put babies to sleep was available. Judging by most reports of the time from the Netherlands but also Britain and North America, I was more likely tied down, perhaps given some local anesthetic injected around the incision site, and perhaps a sugar cube to keep me quiet (or try to?). As the above illustration shows, the tiny patient was strapped down and wrapped up tightly to keep it warm, to stop it straining, and to help it cope with surgical shock.
Some babies not given a general anesthetic were intubated anyway: they had a breathing tube pushed down their throat (to keep them breathing) and they were then given a paralysing drug to keep them from writhing with the pain of the surgery. Of course my mind doesn’t remember how exactly my operation was managed… but as for my body (“pre-verbal”) memory?
Pyloromyotomy (splitting the pyloric muscle) has become the favourite operation of many surgeons: it is simple, quick, doesn’t remove anything, and usually immediately and obviously effective. But in the past surgeons were trained to disregard the clear signs of great distress during infant surgery, as only a very few surgeons in large hospitals had the skill to give a baby a safe general anesthetic. “At least we’re saving this baby’s life… the parents will be grateful, and so one day should (s)he!”
Specialist pediatric anesthesia was born only in 1949, and its use grew only very slowly. Meanwhile it was convenient and generally taught and believed that babies don’t remember pain and are not affected by it – until a 1987 study by Dr K J S Anand showed conclusively that this is not so. And so it has only been over the past 25 years that it has become almost standard that under 2 year olds are given a general anesthetic for any surgical procedure, and local pain control for so-called “minor procedures” such as circumcision.
My op was done just like the above drawings show, except that I was cut roughly down my middle rather than just to the right of center as in these drawings. The vertical incision was the most typical of PS surgery until the oblique (angled under the right ribs) and the the transverse (straight across) incisions were promoted (starting with the USA in the 1930s) and became the standard. A small incision around or in the navel was introduced in 1986 and has proved popular and far less disfiguring. Minimal access surgery (MAS) gets access to the pylorus via three puncture wounds and instrument probes (and now sometimes just one conduit through the umbilicus). MAS has developed and grown in use since 1990.
Seeing the drawing above here prompted me to discover the buried scar from the transverse (or cross-cut) under my vertical scar (illustrations 2 & 4). Some surgeons believed (and others rejected this) that two cuts at right angles reduced the risk of the wound rupturing when the baby flexed or cried while healing, which it seems is a hazard with the vertical incisions.
Another internet drawing of what happens in a pyloromyotomy, the medical term for PS surgery. The thickened muscle band around the stomach outlet (left pic) has become 1-2 cm long, whitish, and very hard (typically like cartilage), blocking everything. It is slit and then prised open length-wise down to the mucosa, the inner lining of the passage, but keeping this intact is vital: any leak must be recognized and repaired before the wound’s closure. There are several graphic videos showing a pyloromyotomy on YouTube: the one by Dr Wealthy shows the simplicity of the procedure as well as the care that has to be taken to avoid collateral damage that can quickly lead to death.
As for me… yay! My stressed-out Mother’s milk could get through. A simple but effective fix.
Another photo from the web – this little gash through the overgrown muscle is what saves almost all PS babies today from a slow death by dehydration and starvation. This is the gist of the PS fix – the enlarged pylorus muscle has been split open wide but the lining of the gastric passage is left intact. Bring on the food! Like many PS babies I flourished after my op… just look at the 3rd pikkie below here.
My parents dumped any records of my PS story (grrrr), but I remember them having a weight chart which looked very much like this one, with a steep dip at 10 days (not 2 months) – followed by a rapid rise. Whooppee! My pylorus had learnt a severe lesson: it seems it was so keen to avoid further punishment that it obligingly let my intake pass through very quickly – which allowed me to eat as much as I liked without a weight problem.
A bit more about me. This is one of my first photos (and it’s very basic) – with Mum. I was born just after the War ended; Mum and Dad had endured a 7 year engagement due first to the Depression and then the WW2 German occupation of their country. Then, 10 days after my arrival they had to hand their firstborn over for what by today’s standards was rough and ready surgery to remedy my uncontrollable vomiting. This was followed by a time of quarantined separation in hospital because of the danger of infection (2-3 weeks was the norm): Ma had to travel 32 km (20 miles) to the hospital every day by steam train to deliver some breast milk. Must have been fun times for Mum and Dad. Surgery and hospital care were certainly rugged and rudimentary in the 1940s when compared with today. Is it any wonder they refused to talk about any of this?
But here’s the good news: I survived and flourished. Half a year after my operation (and probably already at half a month) I was looking very healthy, thank you. Many PS babies quickly make up their weight loss and go to the top of their percentile range.
It’s now April 1951 – and another fuzzy old photo. This one shows not a sign yet of the trauma and manic phobia I developed during the next year, when I came to fear anyone seeing and asking me about the scar down my middle. Mum made her (then) 4 kids cute swimsuits in several sizes like the one I’m wearing; they were of a standard design in those austere post-War times (I’ve seen lots of them on photos from that era). You can see they usually had a neat window – which in my case framed the top part of my belly scar.
Not only women have self-image issues: some of us guys too take years to stop hating things about our nature and our body. My temperament didn’t help; I’m such an earnest and perfectionist retriever/beaver. By age 6 years (as is common) I had become self-conscious and obsessed about the scar I got as a baby from my stomach surgery. Mum then made me a special new swim suit without the window frame but a rather unhip even higher waist than her earlier design which my brother is now wearing. I look very pleased with the new gear, but it only delayed the issues I had to deal with.
I wish now that my parents had helped me to understand and “own” my life-saving story: I didn’t remember any of it of course, but it had clearly started to trouble me – and they certainly noticed that.
Only in recent years have I realized that there is so much reason to be thankful to God, my doctor and my parents that I
– was born in a time and place that allowed me to survive with an imperfect body,
– am reminded daily of the fact that all of us humans struggle in various ways with our brokenness,
– feel proud of being marked with what represents a lifesaver, and
– belong to a worldwide community of people with a PS story to tell or discover.
During my school years I showed all the signs of PTSD, although in those times the word “trauma” was hardly heard. Although it was recognized that many soldiers suffered from “shell shock”, everybody was told that “time heals everything” and “you’d better get over it”.
Sadly, it doesn’t usually work that way. I grew up in a secret world of
– self-obsession (I’m sure I wore out a few mirrors),
– self-injuring (I learnt a lot and am thankful I did myself no great harm),
– re-enacting my operation in a child-like way, 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 authority, and became passive-aggressive, internalising my anger.
From a young age I loved the coast. Swimming has been my favourite form of exercise and relaxation, but at the beach and pool I never felt free to relax and enjoy myself except in the water; here I am (on the right) hugging the flotation device.
Out of the water I would keep my arms tightly folded or tugged my shorts or swimmers up to my chest. Yes, as Mum kept telling me, this looked ridiculous, but I didn’t really care. Although I’m a “hot body”, I would never go without a shirt, even at home or on hot or steamy summer days.
I lied, denied and pleaded ignorance when people asked what “that” was on my front or what my scar was from. I avoided phys ed classes, sports and overnight camps which involved changing or showering in public, and my heart raced when I heard any of a short list of words.
During my late teens and courtship it took me several years (yes, true!) to summon the courage to tell my best friend and future wife about my PS and scar – only to find that they were no big deal to her, and besides, she told me of a niece who had had the same condition and operation – and as I have discovered, a similar scar too.
Even during our honeymoon this pose shows how careful I was about showing my pockmarked belly to the world. It has taken me many years to feel as free, proud and grateful to God and my family and friends as I do now that I survived something that used to kill most babies who had it.
“Scar shame” is feeling embarrassed by the furtive glances and curious questions we get when we PSers aren’t covered up at the beach, gym, or changerooms.
It’s avoided by wearing a shirt or top, and failing that by folding our arms strategically. LOL
“Scar shame” doesn’t worry everyone, but I’ve learnt I am far from alone in having struggled with it for many years. These two web images of PSers make that obvious.
I well remember the horrible hot flushes and racing heart that underlined the state of my inner self whenever I felt “discovered” or “exposed”.
It was several decades before I could “get over it”.
But “get over it” I did!
There is hope for everybody with “scar shame”.
Despite always being a beanpole and now watching my weight, I’ve added a bit of flab in recent years and my scar has become indented, a common look. It’s become clearer that adhesions have also glued it to my innerworks. I’m thankful that this has not caused me any further trouble.
I’ve learnt a great deal about my PS story and how I’ve reacted to it – but almost all from web-based sources and interactions.
Sadly, my personal story, what only my parents could have told me, will remain an almost total blank. Their lips remained sealed, sadly to their graves. In their times, medical matters and unpleasant stories were usually not talked about: their mantra was, “the doctor did that because you were a bit sick.” We would talk about this “later”. In the endless mean time, I should “stop acting in such a silly way”. I wish now I’d had it in me to challenge them about this before it was too late! But that was not my way.
I am telling all this in some detail to encourage greater awareness of the many aspects of infant surgery, and not only as I experienced it. With countless parents I want PS to have a much higher profile among medical personnel, and among new parents and their family circle.
I also want every parent of a PSer to be able to do much more today to help their child understand and embrace their story, especially if their growing child struggles to understand and “own” their scar and its story.
This photo from a German medical website is very special to me. The father on the left has almost a clone of my scar, and his son has had a laparoscopic PS operation.
This image was posted on a German website to show the progress PS surgery has made since 1980 when a baby’s belly was still routinely slit open and then sewn up like a football with big black threads… without much thought given to the tiny patient’s looks and feeling in later life.
I have recently learnt that the railway-track-like scar (which I share with the father above here and many other older folk) is the result of a wound being closed with retention sutures. Usually now, such sturdy sutures are buried in the wound, and small staples or small buried stitches and strong adhesive tape hold the outer skin layer together while the wound heals.
In the past wound vulnerable to rupturing were closed with sturdy threads taken through the skin and underlying muscle, which were then drawn tight, tied, and then left to saw through the tender skin for 10 or more days, leaving large and elongated pockmark scars. This was done as a precaution especially when the baby’s condition was weak, to save time (just as critical when it was not considered safe to fully anesthetise the baby, as when the infant was conscious throughout), and to help prevent wound rupture as the baby cried and strained during the first post-op weeks. Midline vertical wounds cut through the blood-poor linea alba and are especially prone to rupture. Today when retention sutures are used, bolsters or tubing (as shown) is used to keep the sutures from cutting through the skin.
Wound rupture is less common today, and retention stitches in a baby are almost always buried in the wound. Stitches closing the skin are usually cleverly buried and leave no visible scars.
Quirkily intriguing to me is that not a few PS scars I have seen, including the German one above and my own, have a little crater somewhere along the incision line: I wonder if a drain was sometimes left in the wound?
In recent years, at least the younger medical world is becoming aware of the importance of doing infant surgery in such ways as will leave the least possible visible damage. Sadly, I have noticed that some “old hands” still ridicule any consideration of cosmetics, and some medical people even promise that the wound will slowly disappear and certainly not grow with the baby. However, there is a growing recognition that infant surgery can leave some (or many perhaps) of the patients vulnerable to various psychological and emotional challenges.
During the past 15 years the surgical repair of PS has often been done via 3 2-4 mm laparoscopic probes. Or, when a surgeon chooses to work through an open incision, the stitches that close the wound are almost always buried to leave a much tidier scar, as this beautifully captured image from the Life Lines blog shows.
Although laparoscopic PS surgery doesn’t leave much damage at first, by school age the probes leave scars that may have become navel-like pits on the abdomen: as someone has commented, “My belly looks as if it’s taken a shrapnel hit”.
Reports from several developed but non-English speaking countries show that over 80% of PS babies will actually respond to medical treatment with the drug atropine or now Ranitidine which relaxes the pylorus muscle until the baby’s growth takes its own care of PS. Although medical drug treatment takes a little coaching of the parents and a course of several weeks, it is safe and non-traumatic for both the parents and the baby… and the surgery option can always be taken up if necessary.
One of my blog’s “messages” is that medical treatment should be the first option offered for all PS babies older than 3 weeks. It will very likely avoid inflicting the trauma of surgery on the parents and their little one. If unsuccessful, surgery remains as a safe second best option. In countries where this is policy, it is just as safe as the surgeons of the Western world’s preference for the scalpel.
Now, welcome to my Blog! Please consider leaving a comment below here, or something of your PS and/or infant surgery story.
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