Wednesday, 12 April 2017

Henoch Shonlein Purpura - Who, What, Where and When?

In Paediatric Emergency Medicine in the UK, it is often the case that a referral requires me to choose between more than one specialty.  For example cuts on the face (the ones that require suturing under general anaesthetic) could be referred to either plastic surgeons or maxillofacial surgeons.  Hand fractures are sometimes looked after by the orhopaedic surgeons (note use of their Sunday name) and sometimes by plastics.  Now that I think about it, the overlap almost always involves plastic surgery.

The decision about who to refer a patient to will depend on various factors.  These include resources, availability and the particulars of the case.  Matching the child and the illness (or injury) to the right person to follow the case up is part of the art of Primary Care.  After much thought and consideration, I've decided to refer children with Henoch Schonlein Purpura (HSP) to the very best.  I refer them to their General Practitioner.  
(Ankle bracelet not included.  See in store for more details)

The reason for this is that General Practice does two things better than any other specialty.  General Practice excels at avoiding the harm done by unnecessary tests and treatments, and (in my opinion) no other specialty in medicine is so good at providing the continuity needed to monitor a condition.  This allows the patient to receive appropriate care while at the same time having an experienced clinician involved who will intervene if needed.  For uncomplicated HSP, that is exactly what is needed.

HSP is the most common vasculitis in children.  Nobody really knows why children get it but the number one suspect is of course 'recent infection'.  These are always getting the blame for mysterious childhood illnesses.  If you ask me, I think it is institutional infectionism.

HSP is best known for causing a purpuric rash on the buttocks and lower limbs,  While this is the typical rash, atypical rashes are pretty common.  The thing that is most consistent about the HSP rash is the following triad:

HSP is a clinical diagnosis.  It is usually identifiable through a typical presentation and because other possible causes of non-blanching rash can be ruled out clinically.  Sepsis, leukaemia and immune thrombocytopaenia are some of the possibilities that should be considered if the presentation is not that of typical HSP, or if the child is unwell, or anaemic or has splenomegally (etc. etc.).

It is also worth mentioning that not all children have the courtesy to present with their symptoms in the correct order.  Everyone know that the rash should come first.  Some children choose to ignore this and complain of aches and pains before the rash appears.  Also, before the rash becomes purpuric it can start out as non-specific erythema or even look a little urticarial.

I am often asked if blood tests are needed for a child when diagnosing HSP.  My answer is this:  
For example, I don't do CRPs to diagnose sepsis.  I use a CRP once I have diagnosed sepsis because that will help in the ongoing management.  In injury, I use X-rays where it is probable that the X-ray will alter my management.  I don't use CXR to diagnose pneumonia.  I use CXR if I suspect complicated or atypical pneumonia.  In HSP diagnosis, tests are really for uncertainty in those cases where the presentation is ambiguous or atypical.  If the presentation is unambiguously HSP, no diagnostic tests are needed.  There are tests to be done but those are screening for complications.

Most cases of HSP follow a benign course.  The rash persists for several weeks and there many children experience aches and pains in their legs and abdomen.  However, symptoms other than the rash will typically settle in the first week or couple of weeks.  Things don't always go this way though.  There are four possible ways for the course of HSP to be problematic.

Problem 1) Extreme symptoms.

Pain is a funny thing and in some cases of HSP, despite a lack of significant complications, some children and young people get severe pain (despite good doses of simple analgesia) in their legs or abdomen.  These children should be referred acutely to be seen by a paediatrician.  This is both to enable management of the pain and to look for more significant complications.

Problem 2) Non-renal complications of HSP

I've said that leg and abdominal pains are fairly common symptoms of HSP.  It is also true that everything in paediatrics has an evil twin.  In the case of leg pains, it is possible for HSP to cause a significant polyarthritis.  If a child has swollen joints, the pain is severe or they develop a significant limp - refer.

Similarly, if a child with HSP shows signs of severe abdominal pain, consider the possibility that they have developed one of the most significant complications of HSP - intussusception.  Other signs might include pale episodes and blood or 'redcurrant jelly' in the stools.  With or without these other signs, assessment by a paediatric surgeon is urgently needed if abdominal pain becomes acutely severe.

Almost anything that can go wrong can go wrong in HSP.  Involvement of other organs than kidneys, skin, bowel and joints is rare.  Atypical symptoms such as headache or chest pain should be taken very seriously and the child should be referred acutely.

At some point, all of these factors will lead to a decision about where the child is managed.  I think that the decision usually looks like this:
There is nothing mandatory about referring a clear cut case of HSP at first presentation.  However, most GPs that I know would prefer an initial assessment in secondary care, even if the child is extremely well and has no symptoms with their non-blanching rash.

Problem 3) Renal complications

While aches and rashes are what cause children and parents to lose sleep over HSP, it is the possibility of renal complications that usually worries the clinician.   A significant chunk of children who have HSP will develop a mild nephritis.  A smaller number go onto get a significant nephritis and a small number develop severe, kidney-threatening nephropathy.  Don't worry too much about the numbers as they don't really help in a case series of one.  What is true is that it is very likely that the child in front of you will either never develop a renal problem, and if urine dip flags something up, it is most likely to be a transient mild haematuria or proteinuria.  In order to identify those cases of silent but significant nephritis, monitoring is needed for all cases of HSP.

Exactly how much monitoring is needed is slightly unclear.  Advice is based on clinical experience, common sense and a bit of logic.  If you put those things together, you might come up with this:
This is followup strategy is based on the well child, who has no significant haematuria or proteinuria at any point.

What this aims to do is detect renal involvement early, while avoiding any OCD type behaviour (such as daily urine dipsticks).  The length of follow-up is debatable but is based on a cohort study that showed that no child with HSP developed renal involvement after 6 months.  Roughly half the guidelines from big centres recommend follow up to 6 months and half say 12 months.

There are those that advocate longer followup (including lifelong annual reviews) for all cases.  However, I suspect that there is a significant effect of bias driving that.  If a child has had a complicated case of HSP, this may well be warranted, but if the course of the illness was benign I doubt that they will develop late complications.  The Nottingham renal unit recommends lifelong monitoring if everything else has been normal but there was at least one occasion where the urine dip showed ++ or more of protein. (1) This stratification allows for the possibility of nephropathy in selected cases rather than ongoing screening for all.

The need for a blood pressure check after the first couple of visits is also debatable (thus the *).  By continuing to check BP after it has been normal on two occasions, are we saying that the child could have had renal involvement severe enough to cause hypertension, yet this never showed up as a positive dipstick?  This seems very unlikely to me and I have never found a paediatrician who has seen such a thing happen.  Despite this, all guidelines and review articles that I have seen recommend that BP is checked at every visit.

Problem 4 - Prolonged or relapsing course of illness

This brings me neatly onto the final thing that might cause a clinician to feel that the child needs more investigation and management than can be given in Primary Care.  In some cases, the course of HSP does not follow the typical one of a few weeks of rash and a few days of other symptoms.  The symptoms might continue for months, or relapse after initial remission.  A mild relapse is actually quite a common occurrence in HSP and worth warning the family about.  If a relapse is severe or prolonged I would suggest that these children also need to be referred.  The paediatrician will need to review the diagnosis (in case the reason for an atypical course is that the diagnosis is wrong) and consider whether to investigate e.g. for an autoimmune cause of the rash.


Q. Does the child need blood tests at presentation?

A. Only if there is diagnostic ambiguity or if the urine dip/ BP is abnormal.

Q. Do steroids prevent complications?

A. No, but they may have a role in treating complications of HSP. (2) Other interventions that have found to be unhelpful include antiplatelet treatment or immunosupression.

Q. What counts as hypertension in a child?

A. This is difficult question to give a straight answer to.  It's not a lack of people coming up with definitions that is the issue.  It's more that we don't know much about what levels of hypertension cause what effect in the long term.  Most guidelines go for something like systolic BP >95th centile on three occasions.  It is important to use the correct size of BP cuff for the size of the child and to use an appropriate reference range. (3)

Q. So should I be checking BP at every visit even if the urine has been normal (no more than a trace of blood and no more than + protein) on every test?

A. If you didn't, you would be going against all the guidelines in existence.  I still haven't had a good explanation as to why it is really necessary, but nor have I convinced others that it is unnecessary, so for now you should keep on doing it.

So in summary, managing HSP is relatively straightforward.  After initial assessment, most can be monitored by their GP.  Some will need to stay under the care of a paediatrician and a small number will need to be referred on, usually to a nephrologist.  For the uncomplicated cases, there is no absolute certainty about often or how long to screen for renal involvement, so guidelines have to be mostly based on pragmatic expert opinion.

So, who, what, where and when?  Me, you and maybe them doing as much nothing as possible until we are sure that everything is OK.  Simple really.

Edward Snelson

I would like to express my deepest gratitude to the young person who consented to have photos of her HSP rash used for this purpose.  Thank you.

  1. Henoch-Schönlein Purpura (HSP) Guideline, Nottingham Children's Hospital
  2. W Chartapisak et al., Prevention and treatment of renal disease in Henoch-Schönlein purpura: a systematic review, ADC, Feb 2009
  3. Blood Pressure Levels for Boys by Age and Height Percentile, National Heart, Lung and Blood Institute

Tuesday, 28 March 2017

The Random Goldfish - When confirmation bias met affective bias

In the previous post, I explored how confirmation bias can lead us to believe that something is causing a problem when really it isn't.  Although sometimes unexpected, this kind of news is not unwelcome.  Finding out that fever doesn't really cause febrile convulsions is a surprise to many, but usually a good one.  Telling people that their treatments don't work is much less popular.

Anyone who has been a clinician for a reasonable amount of time knows what it feels like to share good and bad news with someone.  When I am teaching about paediatrics in primary care, it sometimes feels like I am saying that very few treatments actually work.  This feels having something taken away form us.  The real headline is that children make themselves better in the vast majority of clinical scenarios.  It is our job as clinicians to do as much nothing as possible while looking for opportunities to give effective treatments.

One question that I am often asked is, "If these treatments don't work, why do people use them?" Good question.  I tend to assume that my sample cohort of co-workers is representative of clinicians and I work with good, conscientious people who want to give children the best treatment possible. Since I don't believe that clinicians are generally unintelligent, malicious or lazy, I will take a risk and say that there must be powerful forces at work if any of us are giving ineffective medicines to children.  The problem that leads us all to use ineffective treatments is our very desire to make children better and parents happy.

If you want something really badly and do something in an attempt to make it come about, when the thing happens, we are likely to believe that this was cause and effect.  This approach would work in a fixed environment,  For example, consider a person trying to solve a puzzle (like a Rubik's Cube).  The puzzle isn't going to solve itself, so if the person tries many different strategies and then something works, they have solved the puzzle.  This assumes that the puzzle has not been solved by chance, which in this case is extremely unlikely.

Now consider someone with a different problem.  He wants a picture of his goldfish next to the castle in the goldfish bowl.  He tries shining a light to get the goldfish to move into position.  He tries tapping the glass, placing some food and tries making waves in the tank.  Whatever he does just before the goldfish moves into position must have done the trick right?  Wrong.

Well, much of paediatrics is like that.  Childhood symptoms often fluctuate or resolve.  We want our treatments to work.  We want to make children better and parents to be happy.  These factors are the perfect ingredients for us to wrongly believe that what we did worked.  Sometimes though, the goldfish just moves.  Because it's a goldfish.

Enough about goldfish. Let's use a really common example of how confirmation bias leads us and our patients to believe in an effect that is not real.

If I tell someone that antibiotics will cure a child's throat infection within a week, you can imagine how that sounds plausible.  Then, the initial belief in my statement will be reinforced when the child does indeed get better.  The true believer does not consider that the alternative is just as plausible - that all (uncomplicated) throat infections get better with time.  You know that guy who still has the viral sore throat he caught when he was two years old?  No?  Neither do I.

Paediatrics is a branch of medicine where most illnesses will resolve with time and many symptoms that could be attributed to a treatable cause.  But we and the parents both want the problem to be treatable.  The problem is, you're too nice.  You want to help and you want everyone to leave happy. This is called affective bias (the thing that reinforces confirmation bias). The end result is that we can easily believe that we are treating a problem, when in fact it gets better on its own.

Now, bias gets a bad name in medicine, but I would like to defend bias.  Clinicians could never learn or make decisions without bias.  We would be permanently uncertain and unable to choose. Without confirmation bias, we would never notice a pattern.  Without affective bias we would never take the parents seriously or care about the child.

Bias is good.  There, I've said it.

Bias is your friend, but friends can be fickle.  The thing about friends is that despite their faults, they're still your friends.  It is good to know what to expect from them.  That way, you don't feel surprised when they do the thing that they always do.  In the case of bias, your friend wants to mislead you and get you do do things you shouldn't do.

So, what are the best examples of the confirmation bias of presumed effect in paediatrics?  I've already mentioned antibiotics for upper respiratory tract infection.  There are many more, but lets just look at one in detail as an example:

Confirmation bias (presumed effect) - 
Example number 2: Treatments for gastro-oesophageal reflux disease in infants
If there was ever a paediatric condition that fitted the brief for this subject, it is feeding problems in babies.  The symptoms that babies present with are so often simply withing normal limits for infancy.   Did you know that straining is a thing that 1 in 6 babies do and that it is called dyschezia, not constipation?  If you add regurgitation of milk (aka 'reflux'), colic and other common gastrointestinal complaints, most babies have some sort of symptom that we could treat if we chose to during the first few months of life.  With a few exceptions, these are normal for being a baby, and will resolve in time.

The cardinal sign of a self resolving problem is that there are treatments available without good evidence of efficacy.  (Cough medicines for children are a good example of this.)  By way of contrast, there are very few treatment strategies for the management of pneumonia.  I'm guessing that you probably use antibiotics.

The evidence for the available treatments for reflux disease is not good.  In many cases the evidence is that they have little effect.  Rather than posting several dozen references, I am simply going to signpost you to the NICE guideline for Gastro-oesophageal reflux disease in children and young people. (1)  In the full document there is an extensive literature review which makes for interesting reading.  The bottom line is that the evidence is usually lacking.  The evidence that we do have form research points toward little or no effect for alginates, H2 agonists and PPIs.  By their own admission, much of the advice in the guideline depends on the experience of the experts involved in the guideline writing process.

Of course the problem is that there are infants with genuine pathology.  These children often begin their visits to healthcare professionals with non-specific presentations which easily fit the bill for what is 'normal for infancy'.  If we are honest with ourselves, the niggling doubt that the child might have a significant problem is one of the factors that pushes us in the direction of pulling out our prescription pads.  After all, it won't look as bad when the child turns out to have a problem later if we were busy trying treatments instead of reassuring the parent that these symptoms are usually part of normal infancy.

Avoiding unnecessary treatments is gold standard care.  Alginates are the most frequently used medications for reflux symptoms but in my experience this treatment runs a high risk of causing constipation.  This is far from ideal if you are trying to make life easier for baby and parent alike.   Motility drugs have repeatedly been associated with dangerous cardiac side effects and PPIs have been shown to increase the risk of respiratory tract infection. (2)  The take home message from this is that, although medication is an option, we need to be sure that a treatment is really likely to be better than watchful waiting.

The NICE guidelines focus on consideration of how the infant is affected - severe distress or red flags (faltering growth, feed refusal etc.).  It is also important to consider other possible diagnoses such as UTI.

I said that there were quite a few problems that have a similar story.  Going through all of them in detail would take a long time, but here is a list of some of the treatments that lend themselves to this combined bias effect:
The evidence for all of these treatments in children is that they work rarely (antibiotics for URTI, inhalers for cough alone, suspected CMPA based on colic alone) or never (cough syrups, simethicone or lactase for colic in babies).  All of these clinical scenarios share a common theme- the likelihood that the symptom will resolve in time.

So lets come back to bias.  Confirmation bias will cause us to believe that a treatment is effective while affective bias will make us want to give something even when there is little or no benefit. "But earlier, you said that bias is good.  You said that bias is my friend!" you might well say.  I stand by that.  As long as you know how you expect your friends to behave, any misbehavior can be managed and they can still be your friends.

For confirmation bias, we need to have good evidence to justify treatment that is used for symptoms when the natural course is resolution with time.  For example, I don't need evidence to back up my belief that morphine works for pain when a child has a broken leg.  If the child feels less pain afterwards, it has nothing to do with a goldfish effect.  Conversely, I should want evidence that a treatment is effective for any of the symptoms listed above.

For affective bias, we need to harness our desire to be nice to parents and children.  That means not wasting their time with ineffective treatments or worse still causing new problems. Sometimes, doing nothing is the nicest thing that you can do.  Managing to treat where appropriate and avoid unnecessary treatment is the holy grail of paediatrics.  Ultimately, the child is the patient and we need to only give them medication that is more likely to help than harm.  At least, that's what we should be trying to do despite our biases.

Edward Snelson
Amateur Medical Errorist

Disclaimer - I am too biased to be taken seriously, even by myself.

  1. NG1 - Guideline for Gastro-oesophageal reflux disease in children and young people, NICE
  2. Orenstein et al., Multicenter, double-blind, randomized, placebo-controlled trial assessing the efficacy and safety of proton pump inhibitor lansoprazole in infants with symptoms of gastroesophageal reflux disease, J Pediatr. 2009 Apr;154(4):514-520

Friday, 3 March 2017

Why has no one told me this before? Confirmation bias - It lies to you. It lies to everyone. What has it been telling you about children? (Part 1)

Recently, after I had explained why something was a medical myth, a colleague in Primary Care looked at me and with genuine exasperation said, "Why has no one told me this before?"

Good question.  The answer to this is complicated.  A lot of the time there is a big 'Emperor's New Clothes' factor.  Declaring a myth to be untrue requires someone to burst the bubble and it is not always the case that someone is listening or that anyone wants to change their belief.

Myths start for various reasons but only persist if they are fed.  For example, very few people actually believe in the existence of fairies.   Seeing a fairy or hearing from someone who claimed to see one might change that.   People do however believe that mice like cheese without any good evidence to support this.  Where does this belief come from?  Surely we can't all have taken Tom and Jerry cartoons at face value?  Since there was never any reason to doubt what we were told we continued to believe it. Well, it turns out that, whatever the basis for the belief, it is wrong when tested scientifically (Yes, this has been researched and published!).

The best ingredients for a myth are plausibility and confirmation.  Take the old chestnut about not being allowed to use adrenaline with anaesthetic in fingers as an example.  This myth originated when lidocaine and adrenaline were commonly mixed with various things to aid anaesthesia and asepsis.  The mix often contained cocaine, procaine and boric acid.  When skin necrosis developed in fingers, the cause was not isolated, but the idea that a vasoconstrictor (adrenaline) was the cause was credible.  In fact, the other ingredients were probably to blame.  Thus a myth  has persisted for roughly a century was created by a plausible theory and repeated episodes which seemed to confirm this theory. (1)

Confirmation bias comes in several forms.  It affects how we search for, interpret and retain information.  They have been responsible for misleading us about quite a few things in paediatics.  There are so many, in fact, that it would be ambitious to put them all in a single post.  Instead I will divide them roughly into two groups - those where we have been misled about cause and those where we are misled about effect.

  • We tend to consider what the cause of something is when we witness an event.  
  • We concentrate on an effect when we think we can influence events.

For now, I am going to run through some examples of presumed cause.  Lets start with the things that you may have been told are caused by something else, but probably are not.  It works like this:

Of course sometimes, the presumed cause is real.  We have confirmation bias for a reason and in most cases it is teaching us, not lying to us.  Assumption has a bad name for itself, but is a necessary part of how we work and learn.
(No disrespect to Mrs. Sullivan, who taught me that to assume makes and ass of you and me.  Mrs. Sullivan was an English teacher and the spelling mnemonic is valid even if the statement is completely wrong in the context of exploratory learning.)

In certain circumstances, the reality is very different from our assumptions.  This is usually due to a factor that is not as obvious as the two that we have associated.

There are several examples of this below.  The one that often surprises many people is finding out that it is a fallacy that fever causes febrile convulsions.  I know, right?  I mean it's in the name and everything!  It makes sense that fever causes febrile convulsions since a child develops a fever and then has a convulsion.  We even see a correlation between febrile convulsion and fever that comes on particularly quickly (or so we think).

The only problem is that the evidence goes against this being true.  When children are treated for their fever, it seems that they have the same number of fits.  (2) So what is the cause of the fits?  Probably badness.  Badness is the stuff that infections make which causes the fever, the flu symptoms and all that.  You know, chemicals and stuff.  So even when we treat the symptoms of the infection, badness still causes the seizure to occur.  We can't get rid of viral badness.  In most cases we just make children feel better until they make themselves well.

How does this change our practice?  When I found this out, it completely changed my approach to children who had suffered a febrile seizure.  I no longer worry that treatment needs to be focused on the fever rather than the child's wellness.  Most importantly, I now tell parents that the seizure was not preventable.  Often, the parent blames themselves for failing to treat the fever adequately.  They need to know that this convulsion was not their fault.

Next up is the apparent epidemic of allergy to amoxicillin.
We have to work this one backwards from the evidence.  Approximately 95% of children who have a label of amoxicillin allergy have no allergy when tested or challenged. (3) The explanation for this poor correlation is that children of a certain age frequntly develop a rash (which is often urticarial) while ill with a virus.  Viral and bacterial infections are difficult to tell apart, so it is not uncommon for a child to be given antibiotics while unwell with a viral illness.  When a culprit is sought for the rash, the antibiotics may be blamed, though the reality was that the virus caused it.

Finding this out completely changed my practice.  By careful case selection, I take every opportunity to undiagnose penicillin allergy.

Next up: another much maligned medicine - Ibuprofen.   Ibuprofen is often avoided in children who have history of wheeze.  I suspect that this is one of the biggest cases of (wrongly) presumed cause currently in paediatric practice.  You may have been told that ibuprofen causes wheeze or that ibuprofen should be avoided in children with a history of wheeze.  Well, it turns out that this is another myth that has persists despite being disproved.

Once again, the association in space and time of the medicine and the symptoms leads to a very rational fear that it is the ibuprofen causing the wheeze.  When large groups are studied, it seems that Ibuprofen may even be protective against wheeze. (4)  I'll just leave that one with you for a minute...

So after that bomb shell, something a little more palatable but still interesting.  Growing pains are not caused by (wait for it........) growing.  In fact no one knows what causes children to have growing pains.
Feel free to file this under 'how does that change my practice?'  I just think that it is interesting that we feel the need to have an explanation for a symptom which has no known cause and no effective treatment - a bit like colic really!

Next up is something a bit more meaty.  Based on the sessions that I do for GPs here and there, I would approximate that roughly three quarters of primary care clinicians are aware that there is a concern about using ibuprofen for children with chickenpox.  I also know that the basis for this concern is poorly understood.

The truth is that this concern was raised based on a cluster of cases of children who developed severe complications of secondary infection about 20 years ago (5).  No causal link has ever been convincingly shown and the fact that huge numbers of children continue to have ibuprofen in this context makes me think that more robust evidence would have emerged if there was genuine cause and effect.

Invasive streptococcal infection during varicella infection is something that all clinicians should know about.  It is also true that most children who have chickenpox are not very unwell and so paracetamol should be all that is needed.

So why does this matter?  It matters when someone is blamed for something based on poor evidence.  So, let's be clear.  The Emperor appears to be naked, but if anyone else can see that he's got clothes on, I am prepared to be convinced.

Edward Snelson
Non-steroidal guardian of the year 2014-2016

Disclaimer- I would never use any of the treatments listed above.  For many years now I have only used fairy magic to treat my patients and any prescribed medication is a pretence.  No one can prove to me that fairies don't exist. 

  1. Bradon et al, Do Not Use Epinephrine in Digital Blocks: Myth or Truth?, Plastic & Reconstructive Surgery, February 2001
  2. A Sahib El-Radhi, W Barry, Do antipyretics prevent febrile convulsions?, ADC, Volume 88, Issue 7, 2003
  3. Caubet JC et al., The role of penicillin in benign skin rashes in childhood: a prospective study based on drug rechallenge, J Allergy Clin Immunol. 2011 Jan;127(1):218-22.
  4. Kanabar et al., A review of ibuprofen and acetaminophen use in febrile children and the occurrence of asthma-related symptoms, Clinical Therapeutics, Volume 29, Issue 12, December 2007, Pages 2716-2723
  5. Zerr DM. et al., A case-control study of necrotizing fasciitis during primary varicella, Pediatrics. 1999 Apr;103(4 Pt 1):783-90.

Thursday, 9 February 2017

Dates for your diary – Constipation in Children

If (like me) you are still sorting out your 2017 diary, I have some important dates for you to add.  They all relate to that child who you are about to see with constipation.

Constipation is very common in children over the age of one year old, being prevalent in around 20% of children.  It is responsible for many presentations to GPs, Emergency Departments and other unscheduled care providers.  Sometimes the family knows that constipation is the problem but often they are perplexed by symptoms that they do not necessarily associate with constipation.

By far the most common perplexing symptom of constipation is abdominal pain.  This pain can be acute, chronic or both.  It can be severe or mild enough that the child does not articulate their discomfort to the parents.

So, back to the diary: this child who is presenting with what turns out to have constipation has a timeline.  Assuming that they presenting for the first time, now in February 2017, these are the relevant dates:

August 2016

This is the time that the child began to become constipated.  It is often the case that it seems that the problem has been there for a much shorter period.  When the child eventually comes to see you, the parents may have been aware of the abdominal pain for a few days or hours, but it takes a long time to become constipated to the point that symptoms occur.  Think of it like a massive ship.  It takes a long time for one of these to come to a full stop.  Likewise, it takes months for bowels to reach the point that they are loaded and unable to function, which is the point at which they will present to you.

February 2017

The parents will present now because they recognise a problem.  This maybe abdominal pain or perhaps they have seen blood on the toilet paper.  Often, there will be a reluctance to accept that constipation is the cause of these things.  This is for a variety of reasons.

Parents are unlikely to know exactly what a child's bowel habit is.  Asking if the child opens their bowels and if this is normal is unlikely to unmask constipation as the villain here.  Instead it is important to ask specific questions.

  • Does the child strain to pass stool?
  • Is it painful for the child to open their bowels?
  • Is there ever blood on the stools or toilet paper?
  • What do the stools look like?

If they identify type 1, 2 or 3 stool from the Bristol chart, this indicates constipation.  If the child is passing only liquid stools, this may be overflow due to severe constipation.

If the problem is most likely constipation, it is also very probable that it is idiopathic constipation.  In the absence of red flags, it is usually appropriate to treat without any need for investigation.

The best treatment is macrogol laxatives (1) in increasing doses to achieve easy passage of formed stools.  It may be necessary to see this child on a few occasions to get the dose of laxative perfect.  The aim is to have the child passing soft, well formed stools easily.

There is a lot to explain in the first consultation.  Parents and children often disbelieve that constipation may be causing the child's symptoms.  I find that pictures help.

Pictures taken from The Essential Handbook of Common Paediatric Cases and used with permission from BPP learning media. (2)

March 2017

By now the symptoms of idiopathic constipation should be improving or possibly gone.  This is an important visit because there are two essential outcomes for this child.

1 - Do not stop the macrogol laxatives.  It takes a long time to become so constipated that you will go to see a doctor.  By the time children present, they have a stretched, weak and numb bowel.  If you stop their laxative now it is too soon.  NICE recommends 4-6 months of treatment in order to restore the shape, strength and sensation to the colon before the child is weaned off medication.

2 - Reinforce the message about the importance of diet and toileting.  Of course you covered this when you first started the medication, but for ten different reasons, it is unlikely that this message has been fully heard and understood.

August 2017

This may seem like a long time away, but this is when it might be time to reduce and stop the laxatives.  If the child has been taking their macrogol for about six months, then the shape, strength and sensation should have returned.  This gives them the best possible chance of being constipation free.  Diet and lifestyle are just as important at this stage as when they were first diagnosed.  Treating and preventing constipation is a lot of hard work  but the results are worth it.

Edward Snelson
Specialist Spitilomancer

Please note that babies who struggle to pass stool are often  experiencing a normal physiological phenomenon called dyschasia.

  1. Constipation in children and young people: diagnosis and management, NICE CG99
  2. The Essential Clinical Handbook for Common Paediatric Cases: A Practical Guide to Assessing Children in General Practice, the Paediatric Assessment Unit and the Emergency Department, BPP Learning Media

Wednesday, 25 January 2017

The Well Covered Wheezer

Guidelines almost always dedicate themselves to 'what to look out for'- the red fags and risk factors.  Often, we go to a guideline in order to learn about a condition, only to find that we should be afraid, very afraid.  I think that the tendency for the glass-half-empty factor in guidelines is almost certainly due to the understandable desire to err on the side of caution.  When you are writing a guideline, you are very aware that if (when) a patient has a bad outcome, the guideline's recommendations will be critiqued.

However, I don't want my doctor to intentionally err at all. To be honest, the error being on the side of caution is very little consolation.  Unnecessary tests, treatments, referrals and admissions are not what I want.  I want my clinician to be thoughtful and careful, but courageous, not risk averse.   I believe  that calculated risk is a necessary part of practicing medicine well, and so struggle with guidelines that give us the impression that we have to follow a certain pathway even when our instinct tells us that this is not needed.

Of course, I know that many of you will now be thinking, “Guidelines are just guidelines.  You don’t have to follow them.”  While this is of course a true statement, try telling this to a clinician who has been to court regarding an adverse outcome.

I don't want to return to an era bereft of guidelines.  That was no fun at all.  Someone else knew what you were supposed to do, most of the time.  It was your job to guess what was expected of you and then find out afterwards if you were right, rather that to be told beforehand.  However, we need to be aware of the negative effects of guidelines, so that we can protect against these.  (1,2)

I do feel that the guideline era has taken some of what used to be taught and allowed this to drift into mythology.  So, in the interest of history, I thought that I would explore what the guidelines often miss out – the signs that are reassuring.  When marking some assignments for the Primary Care Paediatrics course at Sheffield Hallam University, I was delighted to find the oldest reference that I have ever seen in one of these submissions.

Cassell’s Household Guide to Domestic Medicine (1886) - “…on the minutest air-tubes the cells of the lungs are placed… inflammation of these tubes is one of the most fatal diseases in our climate…The child is quickly bereft of its usual liveliness… the breathing is quick and the nostrils expand more or less… All these symptoms are worse if they occur in delicate children…”(3)

The first thing that struck me about this was that in Victorian Britain, the pathophysiology of bronchiolitis was already known.  The second was that they recognised that scrawny babies with bronchiolitis were the ones to worry about.  This brings me onto the well covered wheezer.
In the days before guidelines, I learned paediatrics by trial and error with a degree of question and answer.

Me: “This child is very wheezy.”
Consultant:  “They’ll be fine.  They’re a fat, happy wheezer.”
Me: “So because they’re a bonnie baby and smiling, they’ll be fine?”
Consultant: “Pretty much.”

I don’t know of any research demonstrating the protective benefits of an extra pound of subcutaneous fat when suffering with bronchiolitis.  What I can tell you is that 20 years on, I’ve never had cause to feel misled by this conversation.  That is partly because I leaned the difference between red flags and those that were more of a blood orange colour.

I would say that it is only the 'significant tachypnoea or recession' which could be negated by other reassuring factors such as being well covered and cheerful.  I also think that this is probably self fulfilling: children with the other red flags are too significantly affected to actually have any reassuring features.

What is also interesting is that, in a straw poll of ten junior doctors, none had heard the term ‘fat happy wheezer.’  This is not down to modernity either.  None of the doctors in question had heard it said that habitus was worth considering when assessing a child with bronchiolitis.  I put this down to an overemphasis on red flags and risk factors, without justice being given to reassuring signs.

So, I am going to appeal to two groups of clinicians to help restore balance to The Force:

Firstly – all you experienced, common sense clinicians, please comment below and let me know what other things are reassuring signs which might be unproven but tell you not to be so worried.

Secondly – all you academics, please research these things and get us the evidence to back up what we all know to be true.  After all, this stuff has been known for 130 years now. It’s time we proved it.

Tuesday, 3 January 2017

Your New Year's Resolution - Undiagnose a Child This Year

If you’re wondering what to do for your New Year’s resolution, don’t give something up or join a gym.  Neither will work out anyway.  This year, do something truly worthwhile - promise yourself that you will undiagnose a child or three.

Paediatrics is particularly prone to the pitfalls of overdiagnosis and overtreatment.  Although this is a problem, the reasons for overdiagnosis are actually good ones:

When there are no good tests available to tell between two possibilities, we sometimes give a therapeutic trial to help answer the question.  That is a strategy which will lead to misdiagnosis if symptoms improve despite our treatment rather than because of it.

With therapeutic trials, it is often best to challenge the assumption that it was the treatment that worked.   The two best examples that I can think of are childhood asthma and cow’s milk protein allergy in infants.

Let me give you a case to illustrate what I mean:

A 3 month old has been treated unsuccessfully for symptoms of gastro-oesophageal reflux disease (GORD).  A clinician suspects non-IgE Cow’s Milk Protein Allergy (CMPA) because first and second line treatment for GORD has been unsuccessful and because they notice that the baby has quite significant eczema.  (Click here to see a guide to diagnosing feeding problems in this age group)  The clinician decides to trial an extensively hydrolysed feed.  Over the next few weeks, the child’s symptoms of being unsettled and bringing back feeds improve considerably.  The eczema is responding to topical treatment.

In this situation, it is easy to assume that the change of milk was what made the difference.  Often, this is simply confirmation bias.  Colic, reflux and other symptoms of infancy have a tendency to self-resolve.  Of course the treatment may have been what worked but at this point in time, we genuinely have no idea.

This is the time to stop the hydrolysed formula and reintroduce a standard formula.  (Only do this for Non-IgE CMPA.  IgE CMPA is the kind that has urticaria and wheeze etc.  The children with this type of allergy need to be referred to an allergoligist.)   If the original symptoms of being unsettled and vomiting lots return in the next couple of weeks, the diagnosis is now more robust.  If the child remains well despite a return to standard formula, you have undiagnosed a thing.  Marvellous.

The second clinical scenario is the 7 year old with a nuisance cough.  The cough has been there for somewhere around 2-3 months.   There are no associated symptoms such as wheeze or altered exercise tolerance, but the cough is waking the family up at night.  The chest is clear on examination.

So, what is the likely diagnosis?  Surprisingly, in research land, coughs like this turn out to be caused by pertussis infection more often than asthma or reflux disease. (1,2)  It seems that although the pertussis vaccination is successful, infection is still relatively common.  Instead of causing a more significant respiratory illness, what we see in vaccinated children is often just the cough that lasts 100 days.  There are other, similarly benign reasons for chronic cough in children.  Also, there are plenty of significant pathological causes of chronic cough that are not asthma.

Diagnosing ‘cough variant asthma’ is possibly the biggest reason for the current debate about overdiagnosis of asthma in children, fuelled by an article in the BJGP earlier this year. (3)   Many children in the UK are prescribed inhaled steroids for chronic cough symptoms.  If they get better, this is taken as evidence that they had asthma, but there are other possible reasons for this resolution of symptoms.  The evidence suggests that the most likely thing is that the cough has resolved with time rather than with treatment.

This is therefore another opportunity to undiagnose a thing.  As well as stopping inhaled steroids after (Snelson makes up a number quickly…) three months it is probably a good idea to get some sort of objective assessment before, during and after the therapeutic trial.  Peak flows are great if you can get the child to do these well.  In many cases a symptom score (4) is more achievable.  If the only complaint was cough, then a symptom diary is all that is required.

If when you stop the steroids, the child’s cough is still resolved, you have a winner.  Your New Year's resolution is fulfilled.  Of course, once you start, undiagnosing an become a bit addictive.  If you find it becomes a problem, why not join a gym instead?

Edward Snelson

Disclaimer: My New Year's resolution is to get a better disclaimer.

  1. Marchmont et al, Evaluation and Outcome of Young Children With Chronic Cough, Chest Journal, May 2006, Vol 129, No. 5
  2. Wang et al, Whooping cough in school age children presenting with persistent cough in UK primary care after introduction of the preschool pertussis booster vaccination: prospective cohort study, BMJ, 2014;348:g3668
  3. Looijmans-van den Akker et Al, Overdiagnosis of asthma in children in primary care: a retrospective analysis, BJGP, 1 March 2016
  4., Child Asthma Control Test

Monday, 19 December 2016

If you can't decide between turkey and goose... Pertussiolitis and other animals - when a child has noisy breathing

Recently I learned a good way to find the answer to a question.  It happened like this-

On a walk in Sheffield I saw this strange bird:

Unable to find this creature described in a ‘Birds of Britain’ book, I posted the picture to Facebook and commented that (to me) it looked like a cross between a turkey and a goose.  Most other Facebookers were similarly unfamiliar with the species but within a short period of time, I received a response from my niece who declared the bird to be a Muscovy duck.  After quickly confirming this to be true, I asked how she recognised this bird which is not native to the UK.  The response that came back was simply that she had searched the internet for "birds which look like a cross between a turkey and a goose". 


I had the chance to complete this lesson, for myself in a clinical context, shortly afterwards when faced with another unfamiliar animal, this time in the form of a baby with an ambiguous presentation.  The child had developed a cough and feeding difficulties and had now become wheezy.  Preemptively, my diagnostic centres had skipped forward to the disease that I thought I merely needed to confirm: bronchiolitis.   This mental process was interrupted by a cough from the child, and what a cough it was.  It went on and on and on…  At the end of the period of coughing, the child’s face was properly red. The mother informed me that more often than not a spectacular vomit followed these paroxysms of cough.

With the new possibility of whooping cough suggesting itself, I examined the child with a new mission: confirm findings that are consistent with pertussis infection.  I was therefore, properly annoyed to find a wheeze which I felt was more in keeping with bronchiolitis.  Faced with this puzzle and remembering my niece’s methods, I asked the internet and found that, while not a typical feature of pertussis infection, wheeze has been well described in a large number of cases of children with whooping cough. (1)

This case reminded that, as primary care clinicians, we don’t really diagnose infections- we diagnose syndromes.  Bronchiolitis, for example, is not RSV infection.  Bronchiolitis is a syndrome of wheeze, poor feeding and cough which can lead to severe respiratory distress, apnoea and feeding or respiratory failure.  RSV is one possible cause amongst many untreatable viruses.

Similarly, despite what I was once taught, croup is not caused by parainfluenza virus.  Any virus can cause the upper airway swelling that leads to barking cough, possibly stridor and varying degrees of respiratory distress.

Just to keep me on my toes, children seen to present from time to time with features of multiple syndromes.  The most common bedfellows are croup and viral induced wheeze.  When faced with a child who has a barking cough and a wheeze, one initially questions whether the noise is in fact a stridor (and rightly so).  If it is a wheeze, then it is a wheeze.  If the child has both croup and viral induced wheeze, ther is no point trying to limit the diagnosis.  Just get on and treat both.  It occasionally causes a bit of confusion if the child needs admission.  I think that some junior doctors take the referral of a child with the diagnosis of viral wheeze and croup together to be a sign of uncertainty, or perhaps dementia.

I would suggest that perhaps wheeze is not a feature of whooping cough but that it is possible for a baby to have bronchiolitis at the same time as whooping cough, both caused by pertussis infection.  It doesn't really matter though, since the cause of the infection is only of interest if it can be treated, or transmission prevented.

There are so many infectious causes of noisy breathing in children. Here is a simple guide to what’s what and what to do about it:

Many thanks to my niece for teaching me what the internet is for.

Edward Snelson

Disclaimer: I take full credit for inventing the use of evidence based medicine in the consulting room.

  1. Taylor Z.W. et al, Wheezing in children with pertussis associated with delayed pertussis diagnosis, Pediatr Infect Dis J. 2014 Apr;33(4):351-4.
Acknowledgement: This is a slightly different version of a post which I wrote for the Network Locum Blog earlier this year.