In March 2019, an American ENT surgeon published a paper. For me – it was life-changing. It was the first to describe retrograde cricopharyngeus dysfunction, or R-CPD – colloquially known as the inability to burp.
Have you ever burped?
My entire life I have never been able to burp. I can count on one hand the number of times that gas has spontaneously escaped upwards from my oesophagus, and all of these times have been whilst I was laughing heartily.
“What do you mean you can’t burp?”, often accompanied by a smirk, or outright laughter when I reveal my secret to others. “Just drink a some coke / lemonade / dandelion and burdock” (my favourite suggestion, followed by a gift of the stuff from a work colleague).
But it never works. It just makes it worse. The more air goes in, the more air gets trapped.
I never thought it was too much of an issue until I started drinking beer in my teenage years. One teenage birthday my friends and I ‘borrowed’ a crate of beer from a friend’s Dad, and along with a delicious cake we camped in the woods. Great times. Except that later into the evening it felt like a giant balloon had been inflated in my oesophagus and it hurt to swallow and eat for days.
The same thing happened every time I drank beer. When I was about 19, I drunkenly discovered that I could stick my fingers down my throat to initiate my gag reflex and “vomit air”. Oh the relief! During a night out this became part of my routine. After about the third beer I would disappear off to the bathroom, and produce noises that once prompted a friend to run to my aid as he thought he had heard “moving furniture dragging along the floor”. I would feel much better, and the nights festivities could continue.
Now, in my more mature years I am effectively a non-drinker, but certain activities and foods will still cause problems. My favourite activity, cycling, is a particular culprit. After riding a bike for a few hours hours, with all the associated heavy breathing, swallowing, and eating and drinking in the saddle, I soon end up with (I imagine) litres of air inside me, incapacitating chest pain, and an abdomen that might pass for at least 6 months gestation.
It all comes out eventually of course. I am a legendary farter.
Symptoms occur daily. Usually in the morning I feel great – after a long night of flatulence – and my apparent pregnancy resolves every night. But as soon as I eat or drink the problems begin.
Symptoms vary from comical gurgling noises in the neck, through to gastroesophageal reflux, chest and abdominal pain and marked abdominal bloating. Drinking anything carbonated (especially beer) is a decidedly unpleasant experience, and after most meals I feel a sensation of fullness that it seems others do not.
“It’s not a thing”
I first consulted my GP about the problem aged 21. “That’s not a thing I’ve heard of” I was told. I was instead diagnosed with reflux. PPIs made no discernible difference. I had a scope but it was normal. I commented about my inability to burp to the gastroenterologist but it wasn’t a symptom that registered much interest or interpretation.
I pondered if it was all in my head. I gave up drinking, and made several other lifestyle modifications with limited success. Life went on. But I became more and more convinced that my symptoms were really due to trapped air.
I looked through the literature, but all I could find was a single case study from 1987, and certainly no cure.
- This case study provides some useful insight into the possible pathophysiology of the condition. The patient in this study underwent oesophageal manometry, and it was noted there was a seemingly endless cycle of an air bubble being released by the stomach, travelling up the oesophagus, and then reaching the upper oesophageal sphincter (the cricopharyngeal muscle) which – under normal circumstances relaxes under the presence of air in the oesophagus – but which in this condition stays firmly closed. The air bubble would then be pushed back down the oesophagus with normal peristalsis. Upon the air bolus arriving at the stomach, the cycle would repeat.
- As a sufferer I can attest that this is exactly how it feels – like a big bubble of air in constantly moving up and down the oesophagus and trying (but failing) to get out
- Much higher pressures were noted within the cricopharyngeus muscle – confirming its inability to relax under the normal stimulus.
- Patients with R-CPD have also been noted to have a “saggy” or “baggy” dilated oesophagus on scope – presumably due to years of over-distension.
In my mid-twenties I took up cycling, and I started Emergency Medicine training. And this is where it seemed to become more of a problem. Now symptoms were severe every day. Over the course of about 2 years, I lost 10kgs (from an already Marfanoid body habitus) and my BMI was 19. I just didn’t want to eat because the symptoms afterwards were too unpleasant. One day a consultant at work pulled me to one side to check I was OK. I blurted out that I had such incapacitating GI symptoms, presumably exacerbated by my shift and eating patterns, and that reflux treatment had not made any difference. On the spot my colleague referred me to a gastroenterologist.
I had another scope, and oesophageal pH study. And then they referred me to another gastroenterologist. But still nobody had an answer.
In the end, I gave up shift work to be able to better control my diet. It worked – mostly – but the cycling was still a problem. I had to give up career aspirations for Emergency Medicine too but my old hospital were understanding and I continue there as non-trainee working day shifts part time, around my GP training.
Occasionally reports would pop-up in the popular press about others with the condition. Around this time I came across a reddit group – r/NoBurp (tagline “The Struggle is Real”) – and things started to pick up pace. Here there were hundreds, indeed perhaps even thousands of fellow sufferers!
There was certainly a pattern of symptoms, but the disability seemed variable, from minor annoyance to intermittently totally incapacitating.
It seemed it “was a thing” after all. Some of those in the group were seeing an ENT surgeon in the US who was performing Botox procedures to anecdotally good effect. I even called him up and spoke to him and had a quote for the procedure. But I didn’t fancy flying half way around the world for a possibly psychosomatic disorder.
Others in the reddit group, usually through laborious trial and error had managed to “teach” themselves to burp – typically with a routine of neck-strengthening exercises, neck massage, aspiration-inducing swallowing exercises and in several cases physically punching themselves in the neck!
It is a thing
Finally, in early 2019 the ENT surgeon in the US – Dr Robert Bastian – published his paper about the condition and gave it a name – retrograde cricopharyngeal dysfunction or R-CPD for short. Now I felt I had the proof I needed to find a fellow medical professional who also thought “it was a thing”. I called and emailed some local ENT surgeons. Only one provided any useful insight. He hadn’t heard of it and didn’t seem to think it was a proper thing and spent a lot of time talking to me about the treatment of reflux. But, he did know a neurologist 300 miles away who did cricoypharyngeal Botox injections. So off I went to my GP, printed copy of the R-CPD study in hand, and was duly referred to see the neurologist. He agreed that it might be a thing and I might have it, and after some more investigations (including a CT of my chest due to my “Marfanoid body habitus” which led to a fruitless wild goose chase for a dilated aortic root – phew!) he finally went ahead with the Botox.
But he was cautious. It was a low dose – only 10 units (compared to 50 units used in the published study), he used a transcutaneous approach which doesn’t require anaesthetic, but it is also less accurate – and it didn’t work. However (and if you’re still with me at this point, I’ll buy you a celebratory carbonated beverage) during the course of investigations I had been referred to another ENT surgeon, and they really did think it was a thing. They had even discussed R-CPD with colleagues at a conference!
I was booked in for the procedure. It typically requires a general anaesthetic, and despite having administered hundreds of these in my career I was still nervous. But it went ahead without a hitch. The procedure took about 30 minutes. The following morning at about 5am I was loudly awakened and startled by my own burps! Over the next few days huge uncontrollable releases of air came from my oesophagus. Oh the relief! I was a proud member of the burping human race.
But it was still far too early to tell if I was cured – the Botox can wear off after a few months, but it seems that most patients are able to “learn” how to burp and retain the ability indefinitely, even once the Botox is long gone.
Unfortunately for me, it wore off. It worked really well for about a month, with then gradually decreasing effects for the following 6 weeks or so. My final burp was about 10 weeks after my procedure. I felt so good, and was able to eat and drink anything I liked, and cycle to my heart’s content without worrying about chest pain or reflux. I was very keen for a repeat procedure.
So, after a delay due to the coronavirus outbreak, I had a second procedure – 8 months after the first. This time a larger dose of Botox was used. The effects were even more pronounced that the first. I had more control, and I didn’t have to “push” so hard.
Now – 4 months later the Botox has likely worn off, but the effects don’t show any signs of reducing – and I am starting to consider that I might be permanently cured. There is one minor drawback however – my wife for some reason does not seem as thrilled as I am with all this burping!
I now feel it is my duty to spread the word – particularly amongst the medical profession, so that fellow sufferers will no longer have to wait years, or be laughed at, casually dismissed or otherwise demeaned for their symptoms.
I think there are at least three things that can be learnt from this whole experience:
- The inability to burp, seemingly caused by R-CPD is really “a thing”. It can vary from a minor inconvenience to a serious disability, and any patient with symptoms suggestive of the disorder should be referred to a sympathetic ENT surgeon (a list of which is kindly crowd-sourced in the /NoBurp group). I suspect that in a few years this list will have grown exponentially. Although officially described as “rare” or “unknown incidence” – I know of at least 6 others in my social circle, and I suspect it is quite common, although minor for most.
- This condition seems to have been effectively “crowdfunded” by the r/noburp reddit group. Without a powerful patient lobby of thousands of patients sharing their experiences, I doubt any ENT surgeon would have been able to collect enough patients for a study, and it may have remained “undescribed” indefinitely. I wonder how many other undiscovered disorders whose symptoms we label as unimportant or psychosomatic remain to be discovered. I have certainly been made to feel by friends and colleagues alike that it is “all in my head”. What is potentially quite exciting about this disorder is that (total conjecture from me here) I believe it may explain a proportion of cases of IBS
- When doctors hear symptoms that don’t seem to fit, or don’t make sense, it is important for us not to be dismissive
What strikes me when reading others’ experience of r/NoBurp, is how badly the medical profession has been reflected. It is an extremely common experience to be told that it is “not a real thing”, or “it doesn’t exist” or even “it’s probably psychosomatic”.
These kind of attitudes are damaging to the individual doctor-patient relationship, and to the profession as a whole. I appreciate the complexities of teasing out unusual biological symptoms from those of a psychological origin. I deliberately refrain from using the word “real” symptoms, because I believe that psychosomatic symptoms are just as “real” to the patient, they just have a different origin.
I think that most of the time, these responses are meant with the best intentions. A misjudged attempt to reassure that patient. “Don’t worry about this, it can’t be important because I’ve never heard of it [and neither have any of my colleagues].”
But, we must strive to find the path of understanding that leads to validation of the patient’s symptoms and appropriate investigation. It’s true that most of the time that the doctor hasn’t heard of the particular combination of symptoms, it probably “isn’t a real thing”, but that doesn’t mean that it’s not important to the patient or their long-term health. Just having someone say they don’t know what it is, but they will look for something serious and try to find someone who might know what it is, would go a long way to relieving diagnostic anxiety and building rapport.
I have written an almostadoctor encyclopaedia article about R-CPD if you would like to know a bit more about its clinical features, diagnosis and treatment.
My enormous gratitude to Dr Robert Bastian MD for his quite literally pioneering work in this field. I would also like to mention Mr Yakubu Kagama, an ENT surgeon in the UK, who has been mentioned in the popular press and widely on r/noburp as performing the Botox procedure in the UK.
Huge thanks also go to my surgeon in Sydney (Australia) – whose details are available upon request for any patients locally wishing to seek treatment.
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tl;dr – reddit speak for “Too long, Didn’t read” usually interpreted as “Summary”.