A Whole New World - Honesty in Paediatrics

Paediatrics is a specialty where lying about a diagnosis is normal practice.  It's not because we're bad people.  When you think about the challenges of diagnosis in children combined with the expectation of a diagnosis, it is completely unsurprising.  The adult accompanying the child would like a diagnosis (please and thank you) and the clinician would very much like to give one (you're welcome).

While that all seems very reasonable, in child health it often isn't entirely truthful.  It is one of the mantras of medicine that the diagnosis is going to come from history and examination in most cases.  Hurrah for clinical diagnoses.  In paediatrics, the history is often from a third party and will have an inevitable element of bias.   The examination will also contain more uncertainties more of the time.  You have to accept a significant lack of information when interpreting examination finding in children.

The result of this is that clinical diagnosis is more challenging in paediatrics.  Here's the paradox: clinical diagnosis is the default position in child health.  Why?  Because we don't want to do tests on children or give them treatments "in case" unless these investigations or therapies are very likely to benefit the child.

This week, something big happened and it didn't even hit the news.  The General Medical Council released some new and updated guidance: "Guidance on professional standards and ethics for doctors Decision making and consent."  While much of the content is old news, there is a new emphasis on honesty when there is diagnostic uncertainty that is hugely relevant to paediatric practice, thanks to the fact that uncertainty is where we work.

So, when are we lying to our patients or the adults that accompany them?  The truth is that there is a spectrum of how far what we tell people lies from the truth.  What we should probably do in the light of the new GMC guidance is to re-evaluate our approach to a variety of clinical presentations and ask, "Should I change what I say about this?"

You could argue that nothing is certain in medicine, so what are the thresholds of uncertainty that decide when we should be honest in this way?  That's a fair comment.  We need to apply some measure here - enter the certometer.

The certometer takes the things that we are already using in our diagnostic approach and gives us an idea of how truthful it is to give that diagnosis.  Last week, I asked the medical Twitter world for a few suggestions of diagnoses that we could feed into the Certometer and this seems like a good time to give this contraption a go.

First up is an intriguing suggestion:  Diagnosis - Viral illness.

In my experience this diagnosis is usually given to children with a fever and signs or symptoms of upper respiratory tract infection without signs or symptoms of a more specific diagnosis.

Let's imagine a common scenario then: a 2yr old previously healthy child with a fever for 2 days.  They have a runny nose but no cough.  They have no respiratory abnormality.  Pharynx and both tympanic membranes are inflamed.

The pre-test probability of this being a viral illness is high. It's a child with a fever so the probability that the illness is viral is around 90%.

Positive predictors of a viral cause do exist and include wheeze and urticarial rash in children. This child has none of these things.

Good negative predictors of a diagnosis of viral illness in this sort of case would be some signs of suppurative complications such as mastoiditis.  We haven't seen any signs to suggest this.

So having looked for something specific that truly discriminates and found none, what you are left with is your pre-test probability, dialled down slightly by virtue of the absence of signs of another diagnosis.  In other words, all we have truly achieved is to rule out complications.  Since complications are rare, we're essentially no more certain this is a virus than before we started.

Calling it a viral illness implies that we've added some certainty to the underlying cause that in reality, we haven't.  In fact, by calling it "a virus" we have admitted that there are no specific finding identifying a particular viral illness.

What we have done is far more important.  We have looked for signs of complications and more serious infection (sepsis, meningitis etc) and found none.  What we can say with honesty and certainty is that this is an uncomplicated upper respiratory tract infection.

To emphasise the point about how often the lack of specific signs and symptoms is the norm in paediatrics, I'll give a couple of examples of common, clinical diagnoses that are usually made with enough certainty to be considered completely honest.

• Croup
• Chickenpox
• Febrile convulsion
• Vasovagal syncope

Yep, that's pretty much it.  Most other common problems are really labels given with real uncertainty due to the lack of specific signs or symptoms with good positive or negative predictive value.

Here are a few other examples of diagnoses that are commonly given in what is in reality a great deal of uncertainty that this problem is causing the symptoms or signs.

• Infant reflux disease
• Cow's milk protein allergy (non-IgE)
• Asthma
• in the under 5 yr old child
• where the diagnosis is based on chronic cough without wheeze
• Mesenteric Adenitis
• Hypermobility

Then there's a whole new level of diagnostic uncertainty.  At the beginning I used colic as an example.   Let's try feeding a classic colic presentation into the Certometer.  You see a three week old baby whose only symptom is "crying all the time".  The pregnancy and birth were uncomplicated.  The baby examines normally and is thriving.  They are feeding well and passing urine and stools normally.

What is the pre-test probability that this is colic?  Unfortunately there's no good answer to that because it's not an actual disease.  There is no pathology or treatment.   Colic is simply a label to be given to crying infants that have no pathology.  If you try to put this through the Certometer, you will break it because you can't have any certainty of something that doesn't exist.

It is often argued with colic that the label is therapeutic.  The new GMC guidance should give us an opportunity to re-evaluate that approach.  What would be wrong with telling the parent of the infant described above that their baby is normal and healthy?  That would be honest and potentially just as therapeutic.  We could then use the time that we might have spent (explaining a condition that doesn't exist) on being supportive and encouraging to the parent.  The crying excessively phase does settle and in the meantime, it's all about making sure that it doesn't break the parent.

Here are a couple of other examples of diagnostic labels in children that are without evidence for any disease process.  Neither of these has ever had any pathology associated or been shown to respond to any treatment:

• Growing pains
• Non-specific abdominal pain

Is it time to embrace the idea of greater honesty when we diagnose and explain symptoms in children?  I certainly find that an explanation without a diagnosis is entirely acceptable to families when it comes to a situation where in the past I might have given a non-diagnosis.  Changing that practice is relatively straightforward.  You simply stop saying the thing.

For the situations where we are dealing with an actual diagnosis but there is significant uncertainty, we've got a few options.  The infant with crying and regurgitation of feeds is a good example.  Perhaps we should be stricter about starting off with a label of "possible GORD"?  Perhaps we should go further and start with "Feeding symptoms under observation and follow-up."  Increasingly, I don't give a diagnosis.  Instead I tell the parents that (in the absence of red flags such as fatering growth) "crying and regurgitation can be normal, it can be early symptoms of reflux disease and it can be rarer problems such as allergy.  We don't want to give unnecessary treatments to babies but we also want to treat problems when it's going to help.  This is how we're going to try to get the right balance between those two things..."  

It's a whole new world being honest about our uncertainty but it does work and it works like this:

Edward Snelson
@sailordoctor

Disclaimer: I am not certain about any of that, or that you or I exist.

Reference

General Medical Council UK, Guidance on professional standards and ethics for doctors Decision making and consent, 9 November 2020

Too much choice - What milk do you need to give a baby with a feeding problem?

To paraphrase one of my favourite comedians (John Finnemore) - Once upon a time there was too little choice.  Then around 1980, there was just the right amount of choice.  Now, there is way too much choice.  In paediatrics, this is perhaps more true for special milks than any other treatment decision we make.

Have you been to a supermarket recently to buy some milk?  Just trying to find what you want when you know what you want can be overwhelming.  Should it be 0% fat?  1%? 2%? Organic? Cow's milk? Goat's milk? Filtered? I could go on but you get the idea.  There is just too much choice.

The same is true when trying to decide which milk to use* for a baby with a feeding problem.  There are so many possibilities that it can be quite confusing.  Don't worry though - all the choice is an illusion.  There are only a few real differences which can easily be explained.  The best way is probably by going through the problems rather than the milks.  So here we go.

*Note that this only applies to choice of formula in formula fed infants.  While breastfed babies less commonly develop problems such as milk allergy, the solution is not to change them to a formula feed.  It should be possible to continue breatfeeding no matter what the problem is.

Colic

What is it?  Nobody knows. No cause has been found to really explain why some children cry a lot of the first few months of life.

Which special milk helps this condition?  There is no good medical evidence from RCTs published in peer reviewed journals that I know of to support the use of any special milk.  The keystones of managing colic are to rule out other pathology and to give a good explanation of the condition to the parents.  Ultimately, the only thing that really works to resolve colic is the passage of time.


Gastro-oesophageal reflux disease (GORD)

What is it?  It is normal for babies to reflux/ regurgitate milk.  GORD is the term used to label reflux associated with significant symptoms such as marked and persistent distress.  The amount of milk that the infant brings back is not what separates GORD from physiological reflux.  If a baby has several large regurgitations a day but is minimally affected, this is not considered to be a disease. (1)

The first thing to do is to make sure that the infant is not overfeeding.  This is a surprisingly common scenario that is reasonably simple to manage.

If you suspect that the distress and vomiting is related to overfeeding, try reducing feeds to 150ml/kg/day under the age of three months and to 120ml/kg/day if over the age of three months.  Those targets are a rough guide and overfeeding is only likely to be the issue if the volume of feed is well over those guideline amounts.

Which special milk helps GORD? Most infants with GORD are symptomatic due to reflux of milk, rather than having a problem with acid reflux.  This is almost certainly why the evidence for routine use of acid suppression treatment (PPIs and H2 blockers) suggests little or no effect.

For infant GORD without red flags the mainstay of treatment is to avoid over-feeding, and to use a thickener in a standard feed.  Alginate preparations should be used as second line treatment since they have a high risk of causing constipation.  There are reflux milks on the market which are essentially standard milks with thickener already blended in.  These are not usually prescribed.

Lactose Intolerance

What is it?  Inadequate production of the enzyme lactase in the bowel means that lactose (the sugar in milk) is left undigested.  Bacteria then ferment this sugar and produce noxious chemicals which inflame the bowel, further diminishing the ability to produce lactase.

Lactose intolerance is often secondary to an episode of viral gastroenteritis, in which case it will resolve (and more quickly so with the right milk).  Primary lactose intolerance is actually very rare according to the epidemiologists.  There is an inexplicably large difference between the epidemiology of lactose intolerance, and the frequency with which it is diagnosed by parents and clinicians.

Possible reasons for the overdiagnosis of lactose intolerance include:

• Lack of specific symptoms - normal infant crying and colic might be labelled as lactose intolerance
• Confusion with Cow's Milk Protein Allergy, which is more common.
• Confirmation bias - Colic and reflux symptoms both have a tendency to resolve in time.  If the resolution of symptoms happens to occur after a change in milk, this will give the wrong impression that the special milk caused the improvement in symptoms.

Which special milk helps this condition?  If an infant genuinely has lactose intolerance then a lactose free milk will result in a dramatic resolution of symptoms, usually within days.  There are numerous lactose free formulas which are commercially available.  Soy milk is also lactose free but is not recommended for infant boys as the effect of phytoestrogens is unknown.

Lactose free milk does have the potential to cause more dental caries than standard milk, so there is good reason to avoid the overdiagnosis of lactose intolerance.


Cow's Milk Protein Allergy (CMPA)

What is it?  Well, confusingly, there are two types of CMPA.  IgE type CMPA is what you might call a classic allergy.  With this immediate type of response to cow's milk protein, typical symptoms include wheeze, urticaria, swelling etc.  Usually, it is quite obvious that the child is having an allergic reaction, leaving the main question to be what the allergen was.

In most cases, infants usually have a non-IgE CMPA.  This is sometimes referred to as milk intolerance, which causes it to get confused with lactose intolerance.  I prefer to avoid that term to avoid this misunderstanding.  Non-IgE CMPA is predominantly an enteritis, without systemic features.  As such the symptoms are vague and difficult to distinguish from other conditions such as GORD and colic.

Clues that an infant may have non-IgE CMPA include

• Onset of symptoms at an age which is atypical for GORD (e.g. over 6 months old)
• Symptoms that may originally have suggested GORD but fail to respond to treatment or progress despite treatment
• Lower GI symptoms
• Significant eczema.  The relationship between eczema and CMPA is complicated.  Eczema is quite common and colic is also common.  As a result, it would be foolish to say that eczema + unsettled child = CMPA.  However, there are many infants whose eczema and unsettled behaviour seemed to resolve as soon as they were treated as CMPA.

Which special milk helps this condition?  The best way to stop an an allergic reaction is to remove the allergen.  In order to reduce the allergenicity of the formula, CMPA milks have the proteins broken down to varying degrees.  While there are partially hydrolysed feeds available, an extensively hydrolysed feed (EHF) is recommended for a child with CMPA.  There are many EHFs available.

One pitfall in the prescribing of milks to children with CMPA is to confuse lactose free milk with EHF.  Another is to accidentally prescribe an amino acid formula, because these are also licenced for use in CMPA.  Amino acid formulas are only used for extreme cases of CMPA.  These feeds are quite unpleasant and rather expensive, so EHFs should be first line unless there is a specific reason to chose an amino acid feed.

Amino acid feeds (AAFs) are generally reserved for use in secondary care.  This type of milk is the ultimate in hypoallergenicity (real word?) but is also very expensive and rather unpleasant to taste.  AAFs are usually resorted to rather than being chosen first line.  Indications include severe IgE type allergy or non-IgE allergy that does not respond to an ECF.

One alternative to use first line is soy milk.  This may or may not be successful as a treatment strategy.  The problem with soy milk is that although it does not contain cow's milk protein, it is not in itself hypoallergenic.  Many infants who have reacted to cow's milk also react to soy milk so you end up no further forward when that happens.  There is some caution regarding the phyto-oestrogens that are found in soy milk.  Although it is an unproven risk, the concern is that these chemicals may have an effect on infants and so it is sometimes recommended that soy milk is not given to boys under the age of six months old.

Parents may also want to try other mammalian milks (e.g goat).  Most dietitians recommend that these are avoided due to the poor nutritional content.  Again, these milks are not hypoallergenic and there is significant risk of further reaction.

The most important part of managing non-IgE CMPA is that the diagnosis is a three part process.  First the diagnosis is suspected.  Then, is the infants symptoms settle with treatment the diagnosis is provisional.  Only when the infant is re-established on a standard formula and the symptoms return is the diagnosis confirmed.

When introducing an ECF, it is worth considering that the infant may object to the taste of the new milk.  Although nowhere near as unpalatable as an AAF, ECFs do have a distinctive taste difference from standard feeds.  A sudden switch can cause problems at feeding times.  One strategy to avoid this is to blend the new milk in over a few days.  If the infant is on a 6oz feed, use 5oz of standard feed mixed with 1oz of ECF on the first day.  On the second day, increase that to 2oz of ECF and 4oz of standard feed.  After a few days, the infant should be fully established on the new ECF feed.

The best way to avoid the problem of too much choice is to simplify things.  Find out a locally available ECF or check your local guideline/ formulary.  That way you only need to know one milk- choice doesn't get more simple than that.

Edward Snelson
Simplologist
@sailordoctor

Disclaimer - I frequently buy the wrong milk.

References:
Gastro-oesophageal reflux disease in children and young people: diagnosis and management
NICE guideline [NG1] January 2015

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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
@sailordoctor

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

References

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

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:

• The tendency for symptoms to resolve with time (e.g. vomiting and crying in infancy)
• In childhood there is usually an illness that can’t be treated which mimics those that should be taken more seriously (e.g. viral urticaria leading to overdiagnosis of antibiotic allergy)

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
Diagnosectomist
@sailordoctor

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

References:

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. Asthma.com, Child Asthma Control Test

Reverse Engineering - Feeding Problems in the Under-One-Year-Old

What's in a name?  When it comes to the medical conditions affecting babies, there is so very much to be confused about.

Whatever your reaction to that, you are probably aware that the terminology used to describe infant feeding problems is confusing and inconsistent.  I have spent a little time recently looking at the relevant websites, guidelines and journal review articles available to professionals on this subject.  So far I have not found two that use exactly the same terminology to categorise the various clinical entities that exist.  I also know from professional experience that understanding varies and even conflicts between clinicians.

Let me give you an example: reflux.  "Reflux" in babies means to many doctors that a child has a problem with bringing up milk that needs treating.  However, bringing milk up is normal for babies, so many encourage the use of the words posseting or regurgitation to describe harmless reflux.  Many academic papers and guidelines use the medically correct term of gastro-oesophageal reflux (GOR) for the harmless type but distinguish this from gastro-oesophageal reflux disease (GORD) when there is a significant problem.  So there we have five different ways to describe just two clinical entities.  Two of those terms might be used interchangeably to describe either entity.

The conditions involving any sort of reaction to milk are even more confusing.  Some try to simplify things by insisting on having just two diagnoses- cow's milk protein allergy or lactose intolerance.  This goes a long way to help clinicians to avoid getting confused between the two, but then many children who are having a reaction to cows milk do not have lactose intolerance, and do not have any evidence of allergy to cow's milk protein either.  For this reason, the term Milk Intolerance is used by some including NHS choices.  Some feel that this helps to distinguish children who have a proven allergy, and so need to be managed as such, from those who do not.  The term milk intolerance does however confuse us again by it's vagueness.  Intolerance to what?  There is now a growing consensus that there is only milk protein allergy or lactose intolerance.

When you can't get started because nothing makes sense, the process that will get you there is called reverse engineering.  Start with the end point and work backwards.  So, lets have a go at this for the infant with feeding problems.  There is one outcome that we are aiming for: a well child.  There are only a few treatment options, so lets start with those and describe the children who should be having that treatment.  All scenarios rely on a thorough history and examination.  One of the most important parts of this is to take a feed history.

Lets start with my favourite outcome and reverse engineer that:

How to get there: reverse engineering option 1 - doing as much nothing as possible

Babies who regurgitate feeds without choking, gagging frequently, faltering growth or being significantly distressed during or after feeds do not require intervention.   It is important to safety-net so that parents know to return if problems such as feed refusal develop.

How to get there: reverse engineering option 2 - reducing the feeds

The baby that vomits and cries a lot may be over-feeding.  This is because, while most babies determine their own requirements, some get stuck in a loop called the feed-cry cycle and need to have their feeds restricted to something more sensible.  Take a feed history in any baby presenting with vomiting and distress and limit the feed to 150ml/kg/day.  If that doesn't work, look at the other options.

How to get there: reverse engineering option 3 - thickeners and alginates

This is where to start with the infant who fits these characteristics:

• Onset of vomiting before the age of three months old
• Gagging, choking or significant distress
• Symptoms are not progressive over a few days and no signs of other illness
• Adequate weight gain and still taking a sensible amount of feed

Thickened milks are the recommended first line of treatment in the NICE guideline for GORD in children.  If thickened feeds do not help or if the baby is breastfed then an infant alginate preparation is a thickener as well as an antacid.

How to get there: reverse engineering option 4 - what am I missing?

This is not really a feeding problem treatment option but an important reminder that we all need to re-think when a child does not improve.  Even if the initial assessment was reassuring, by the second or third visit we should always be asking "what am I missing?"  One thing not to miss is a urinary tract infection.  These low-grade UTIs may be sub-clinical apart from symptoms indistinguishable from GORD.  Other conditions such as pyloric stenosis should be considered if the vomiting escalates over the space of a few days.

How to get there: reverse engineering option 5 - acid suppression

H2 receptor agonists (H2RAs) and proton pump inhibitors (PPIs) are an option for the child who has not responded to option 3 and does not have anything to suggest a hidden diagnosis (option 4).  As long as there is a re-evaluation of the pathway, acid suppression is the next step assuming that the infant does not have sever symptoms or signs of an allergy.

How to get there: reverse engineering option 6 - try an extensively hydrolysed feed (EHF) (or revert to breastfeeding)

There are a few scenarios which might benefit from EHF.  These milks have the proteins broken down so that the feed is very unlikely to trigger an allergic reaction.  

If treatment option 5 has been tried then this is one possible way to go next when there is no response.  In this context the EHF is a trial to see if the child is reacting to the milk with a reaction that only manifests as symptomatic vomiting or distress around feeds.

If an infant who is initially treated with thickeners goes onto develop signs of atopy (such as severe eczema) or fails to improve and has a very strong family history of atopy, EHF can be tried earlier.  Most likely this will be after option 3 in an infant under three months old.

In an infant who presents later in the first year of life, the odds of the problem being a reaction to the milk increase.  In such children, ECF could be tried even without trying options 3 or 4.

The main differences from option 6 are that with option 5 you do not need to refer.  There are a variety of ways to go next.  NICE recommends re-challenging after 2-6 weeks (in this scenario) while local guidelines vary.

How to get there: reverse engineering option 7 – start an extensively hydrolysed feed (EHF) (or revert to breastfeeding) and refer to a specialist

In an infant who has a clear allergic reaction (e.g. develops urticaria after weaning from breast milk) then there is an urgent need to remove cow’s milk protein from the baby’s diet.  This can be done by using EHF or by returning the infant to being fully breastfed.  In these cases the next steps require the input of a specialist so they will need to be referred.  Breastfeeding mothers should exclude milk from their diet until a dietician can fully advise.

How to get there: reverse engineering option 8 – start a lactose free milk (or add lactase for breast feeding mothers)

In the UK, lactose free milk is usually needed when a child has partially recovered from a gastroenteritis,  The unwellness and vomiting settles but they have persistent and often explosive loose stools.  In these cases, it is likely to be the case that the child will dramatically improve with a trial of lactose free milk.  Once symptoms have been resolved for two weeks, the child can return to normal milk products.

For breast feeding mothers, there is no point excluding lactose from their diet as the breast naturally produces lactose anyway.  One option is to add lactase to the feeds in the form of drops.
Note: EHFs tend to be lactose free (because lactose also causes problems for babies reacting to the proteins in cow's milk) but this is a very expensive option if the problem is purely the lactose. Choose a simple lactose free milk in this scenario.

So, I have managed to describe the various options and the patients who should benefit from them, yet I only mentioned a diagnosis once (in the context of a NICE guideline).  When it comes to feeding problems in the under one year old, the diagnosis matters little and may never be known.  What really matters is to know what has the best chance of success for a given clinical entity and what to do next if that intervention does not work.

Having said all that, if you want to know how the names match up to the scenarios, here's a simplistic table that pretty much does that as well:

Edward Snelson
Paediatrician with sub-specialty training in having a short attention span
@sailordoctor

Disclaimer: I could have just given you the last picture, but what would be the fun in that?

Feed histories in refluxy babies

Over feeding?  Is that really a thing?

One of the questions that I asked most often is “where do I start when treating reflux in babies?”  This assumes two things.  The first assumption is that it is reflux that you are dealing with and second is that you want to treat it.  Each of those assumptions is worth articles of their own, so I may come back to those two issues at a later date.  However, if we are going to start our treatment somewhere, let’s start as always, by doing as much nothing as possible.  The place to start is by taking a feeding history.

I would like to say now that I do not like the term over-feeding as I feel it implies blame if it is heard in a critical way.  I don’t have a better term (yet) and it is commonly used so it will have to do.  Over-feeding is the phenomenon seen in many babies which involves them taking considerably more milk than they need or can keep in their stomachs.  As a result of this their stomachs are distended enough to cause them pain and are likely to have distressing regurgitation.

Why does this happen in some cases?  Because: the understanding between a baby and the person feeding them is that if the baby cries, that might be because they are hungry.  As a result, they are offered a feed.  Babies are fairly reflexive about feeding and so may suckle even when not hungry, leading to overfeeding.  Being overfull, they will find that they are not comfortable.  They need to let someone know about this unsatisfactory situation and so they cry, at which point someone may offer them a feed.  This is called the feed-cry cycle.

The NICE guideline ‘gastro-oesophagealreflux disease (GORD) in children and young people’ (NG1) acknowledges the possibility that a reduction in feeds might help in their recommendations:

In formula-fed infants with frequent regurgitation associated with marked distress, use the following stepped-care approach:

• review the feeding history, then
• reduce the feed volumes only if excessive for the infant's weight, then
• offer a trial of smaller, more frequent feeds (while maintaining an appropriate total daily amount of milk) unless the feeds are already small and frequent, then
• offer a trial of thickened formula (for example, containing rice starch, cornstarch, locust bean gum or carob bean gum). (1)

The devilment is however most definitely in the detail…

If we are going to be scientific about this we need to define our terms.  What is an excessive volume of feed?  Ooh, I know the answer to this one…  Nobody knows.  What we do have is a consensus that approximately 150ml/kg/day is a ‘normal’ amount.  The thing about norms is that they tend to have standard deviations and no one knows what that number is either.  So where does that leave you?

I have read through the detail of the full NICE NG1 guideline in the hopes of finding the answer and there is no centile chart of feed volumes, since one doesn’t exist.  Instead we are left with the responsibility of deciding for ourselves.  My approach is to take the feed history and calculate the volume of formula milk fed thus:

1. No of fluid ounces taken on average per feed x 28 equals mls of milk per feed
2. mls of milk per feed x average daily number of feeds equals daily intake in mls/day
3. Daily intake divided by weight (Kg) gives feed volume by weight in mls/Kg/day

If I believe that the child has GORD and the feed volume is quite a lot more than 150ml/kg/day then I will recommend a trial period of reducing the daily feed volume to 150ml/kg/day and make sure that no one feed is greater than 30ml/kg.  If they get better, it might be by random fluctuation or it might be my intervention.  Either way the child is better.  If they don’t get and stay better, I move on to the next intervention.

You might notice that I still haven’t given you a number that would qualify as “excessive for the infant's weight.”  Well spotted.  Please let me know if you find one that is evidence based.  In the meantime I will continue to believe that my number is correct.

Edward Snelson

Senior Spitilomancer

@sailordoctor

Disclaimer: You can't trust my number.  I came up with it by throwing three darts at a dart board.  It was a very good score though.


If you found this helpful you might also like to read:

Why I need GPs to be medical leaders (Easter egg - GORD in babies)

References:

1. NICE guideline ‘gastro-oesophagealreflux disease (GORD) in children and young people’ (NG1) 

Why I need GPs to be medical leaders (Easter egg - GORD in babies)

Please Help Me to Change My Practice

In January 2015 NICE published the first of their newly branded ‘CG’s which happened to be Gastro-oesophageal reflux disease: recognition, diagnosis and management in children and young people.  I don’t know how you decide which NICE guidelines to look at but my filter is based on relevance and the extent to which they might make me cry into my coffee.   This one scored a 10, partly because I see lots of vomiting babies (with accompanying parent – this is usually the one I’m more worried about) and partly because we have known for a long time that the available treatments are inconsistent at best.  In the absence of a guideline it is much easier to work through the various non-pharmacological interventions and then take a stepwise approach to treatment, while hopefully the underlying predisposition to create laundry and sleep deprive your parents gradually resolves in time for me to take all the credit.

The truth is that when I see a child with reflux, things are usually pretty desperate for the family.  They are tired, smell of vomit and feel that every time they see a different doctor or nurse they are told something that contradicts previous advice.  I then find it difficult to admit that the problem with which they present is going to follow a course over which I have little influence.  Certainly I do make a difference where possible.  I explore the way that feeds are being given and often find that the volume of feed is excessive.  Occasionally I discover a previously undiagnosed urinary tract infection and get to feel like a real doctor.  More often there is no easy answer and I reach for my prescription pad to prescribe an alginate. 

The thing is that NICE have now said that the initial treatment should be a feed thickener if the child is formula fed.  That should be a simple thing to change but for me it isn’t.   

From CG1 2015

I remember well how in General Practice I used to do this process change.  One day I would find out something, next I would have a quick chat with my GP colleague and then I would get on and do it.  No major fuss.  How things have changed.  Now that I work in a hospital it takes months to change most things.  Certainly when there is an urgent need we get that turned around much faster.  (I won’t say how long faster is.  It depends.)   However if the change is less urgent it requires consensus, consultation and committees to the Nth degree.

In the interest of balance I should extol the virtues of this more cumbersome approach.  It would be chaos if there was no way of ensuring consistency of practices within the various teams of any hospital.  That consistency only comes if guidelines are agreed and well governed.  Achieving that sometimes feels ungainly but is far better than conflicting practices within the same organisation or changes that are ill thought through and are not universally agreed.

The good news is that in the case of these vomiting children it hasn’t been a problem.  The dilemma that I might have faced was taken away because thankfully the last few children that I have seen with gastro-oesophageal reflux were all being treated with feed thickeners and not an alginate.   Since previously the latter was the norm I can only assume that these GPs have decided to lead the way.  No fuss, no committees and no delay.  How wonderful that GPs are playing to their strengths and getting on with changes that take much longer for Secondary Care to implement.

So thank you for providing some true medical leadership.  For those of us working in hospital, we need you in General Practice to lead the way for us.  I’m sure we’ll catch up eventually.

Edward Snelson

Kiddie Doctor

@sailordoctor #GPpaedsTips

Disclaimer: All disclaimers are nonsense.

1. http://www.nice.org.uk/guidance/NG1/chapter/1-recommendations

More treats from the Easter egg:

1) NICE doesn't want us diagnosing 'silent reflux' so much

2) Some red flags here suggesting that there may be another diagnosis or that the GORD warrants rapid referral