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Faye Hawkins case

Clinical governance, Ethics, General paediatrics, Generic

A consultant paediatrician who received a formal warning from the GMC for missing a case of fatal appendicitis.

Found that she failed to consider possible underlying serious cause for fever, and that lethargy and mildly elevated heart rate are “red flags”. But actually, they are not – common and poorly predictive, and not in NICE Fever in under 5s table. Patient was 5 already, anyway. Did not flag on Sepsis tool.

Also found that she failed to examine again or look for other possible red flags when she discharged Elspeth from hospital; failed to adequately advise parents on how frequently they should monitor her temperature and pain symptoms; and failed to record the advice given in the notes.

British association for general paediatricians complained that the pressure of the acute unit were not taken into account, but GMC response was that tribunal (which is independent from GMC) took this into account, although they could not agree on what standards for a “reasonably competent clinician” could be applied!

Allergen families

Allergy, General paediatrics, Immunology

Hundreds of different allergens have been identified, and can be classified by similarities in structure/genetics, usually based on the plants being related to each other in evolutionary terms.

This is useful because cross reactivity is more common, the more closely different proteins are related. Not all are allergenic however, and not all cross react.

You can also predict how heat stable these proteins are by how cross linked their structure is. Linear proteins are more easily disrupted by heat, so the allergy is likely to only be an issue with raw (or frozen from raw).

Most allergens belong to one of a small number of groups:

  • PR-10 eg Bet v 1, Ara h 8, Cor a 1, Pru p 1, Mal d 1. Heat labile, homologous. Most pollen food syndrome cases (birch pollen).
  • Profilin eg Bet v 2, Cor a 2. About 20% of pollen food syndrome cases. More common in Southern Europe – birch and oak too, but also olive tree, London plane, grasses (eg Phl p12), ragweed. More common in high grass pollen intensity areas? Citrus, tomato, banana, melon/watermelon (but different from latex-fruit syndrome). Can be associated with severe food reactions (not citrus so much but the others).
  • Prolamin – includes nonspecific lipid transfer proteins (nsLTP) which are heat stable but very cross reactive, and are found in fruit, vegetables, nuts, legumes, seeds and cereals. Best known is Pru p 3, which is a good surrogate marker for any nsLTP sensitisation, even if peach (prunus persica) isn’t a known issue. Ara h 9, Cor a 8, Jug r3, Mal d 3. Mugwort related pollen food syndrome is usually due to an LTP (Art v 4 with Api g 4 of celery and Dau c 4 of carrot, else Foe v 5 (fennel), Sin a 3/4 of mustard). Severe reactions possible.
  • Cupin – includes legumins. Heat stable. Ara h 1 and 3, lentil, cor a 9/11 (hazelnut), some soya.
  • Thaumatin – named after W African shrub! Various fruit including apple, kiwi, plus cedar pollen.
  • 2S albumin eg Ara h 6.

Glasgow lab offers only hz, peanut, peach/cherry, egg (Gal d 1), alpha galactose and bee/wasp. Dundee offers those plus milk and cashew.

Private testing available for Bos d 8 (milk casein) and soya.

Pubertal staging

Clinical, Common, Endocrinology, General paediatrics

Tanner stages – verbal descriptions but images helpful esp for self assessment.

Pubic Hair Scale (both males and females)

  • Stage 1: No hair
  • Stage 2: Downy hair
  • Stage 3: Scant terminal hair
  • Stage 4: Terminal hair that fills the entire triangle overlying the pubic region
  • Stage 5: Terminal hair that extends beyond the inguinal crease onto the thigh

Female Breast Development Scale

  • Stage 1: No glandular breast tissue palpable 
  • Stage 2: Breast bud palpable under the areola (1st pubertal sign in females)
  • Stage 3: Breast tissue palpable outside areola; no areolar development
  • Stage 4: Areola elevated above the contour of the breast, forming a “double scoop” appearance
  • Stage 5: Areolar mound recedes into single breast contour with areolar hyperpigmentation, papillae development, and nipple protrusion

For males you then have testicular volume, measured by orchidometer (between £26 and £208):

  • 4 ml (1.8cm long by formula below) is first pubertal sign
  • Adult is >20 ml (or >3 cm long)

Cadbury’s Teasers and Truffles (from Celebrations box) are 8ml, equivalent to 50th centile at age 13.

If you only have a ruler, use maximum width in millimetres and the formula: (W-1.5)3 x 0.88, where ss is double scrotal skin thickness (for Tanner stages 1, 2, and 3).

Medically unexplained symptoms

Clinical, Common, General paediatrics, Mental health, OPD, Psychology

“Persistent physical symptoms” preferred term? Chronic pain overlaps.

“Functional disorder” is used for various gastro and neurological problems, preferred by some adults, but needs explained!

Chronic fatigue syndrome and PANDAS are health disorders that appear to have a physical/scientific cause but disputed.

Health anxiety, malingering, or factitious illness, different from a psychological point of view.

Bleed the symptoms dry! [John Stone, Glasgow].

In a 1965 paper by Eliot Slater, more than half of patients diagnosed as having “hysteria” later turned out to have “organic” disease – but John Stone’s study of adult neurology referrals found very few who turned out to have an occult disease.

Louise Stone in Australia has done a lot of work in primary care. She identifies negative feelings and a lack of diagnostic language and frameworks as barriers to managing these patients effectively. The negative feelings (such as frustration, shame and helplessness), are shared between doctors and patients…

Managing your own feelings and frustrations, and finding ways of understanding and managing the therapeutic relationship important.

Let family feel validated for all concerns – at least in the first instance. Helps develop a therapeutic alliance.

Commit to the patient, which includes advocacy and support.

Family response to symptoms?

Explore beliefs, specific worries (eg cancer). May then allow broadening out to more general worries. 

Manage uncertainty – including managing the need for a disease name! Not having a predictable outcome is hard.

Harm minimisation. Shift from diagnosis to coping with ongoing symptoms.

Good to offer a tentative preamble to difficult conversations! “This is something we as doctors have to deal with every day – signs and symptoms that are very real, with a real impact on a child/family, but where physical examination and investigations do not offer any clues to what the underlying problem might be…”

Paed psychology if issues mostly seem related to child and this is a new problem; CAMHS if new problem adding to existing child/parent issues.  

Can be rewarding in the long term!

[Louise Stone, Aust Fam Physician 2013 Jul;42(7):501-2]

Atopy

General paediatrics, Immunology

Funny old word – means “without place” in Greek, which refers to the fact people didn’t know what was going on with these kids – not the mechanism, not a particular infection, not a specific organ or system.

Some clues from genetic mutations – JAK1 mutations are a cause of severe atopy and eosinophilia. Involved in IL-4, IL-13, and IFN signaling. Long-term ruxolitinib (kinase inhibitor) treatment of 2 children carrying the JAK1GOF (p.A634D) variant improved their growth, eosinophilia, and clinical features of allergic inflammation.

The idea of the immune system being about defence is wrong! Tolerance is everywhere! B and T regulatory cells keep everything under control.

Microbiome in atopic conditions different, and differences can precede disease.

Neonatal gut microbiome predicts sensitisation (3 groups). Main differences are bifidobacteria and enterobacteria. Differences are related to maternal diet, delivery method, feeding method, antibiotic exposure etc.  Cumulative effect.  Still apparent at age 3.

Breast milk contains 1/3 oligosaccharides that are not digestible by baby!  Designed for microbiome!? Some genetic variations. Oligosaccharide composition associated with later sensitization.

Short chain fatty acids eg butyrate associated with sensitization – yogurt! Component of soluble fibre! Affect T reg cells.

Morganella morgani produces histamine!  Higher levels in asthma, esp higher risk.

[Liam O’Mahony – Cork, prev Davos]

Karelia study – biodiverse environments eg forest and agricultural had greater diversity of skin microbiota cf urban, shore.  Inversely related to atopy, and IL10 expression. [Finland now has national programme for allergy prevention]

Raynauds etc

General paediatrics, Rheumatology

Raynaud’s syndrome is where digits turn cold, white and numb transiently, usually triggered by the cold but more particularly by cold, wet weather. Can be painful and disabling.

Severe with ischaemia (ulcers, atrophy) likely to be part of a broader rheumatological syndrome.

Prevention mostly. Smoking and cocaine likely to worsen. Moisturise dry skin!

Avoid injury while numb!

Differentiate from acrocyanosis (sluggish peripheral circulation, asymptomatic, common in young children), vibration white finger, perniosis (chilblains), cervical rib, subclavian steal.

Investigations are for underlying causes, namely connective tissue disorder (especially scleroderma in adults), vasculitis, malignancy. So if unusually severe and/or atypical:

  • Full blood count
  • ANA, rheumatoid factor, antiphospholipid antibodies
  • Complement levels
  • Protein electrophoresis
  • Cold agglutinins and, in children, cryoglobulins. You don’t expect distal pallor, just blueness.

Nifedipine has most evidence to support use, other calcium channel blockers could be considered. Main side effect is low blood pressure.

Some evidence for diltiazem. Other options losartan, GTN patch, fluoxetine, alpha blockers.

For severe, sildenafil and atorvastatin! No evidence for aspirin but makes sense…

Medical Professionalism

Ethics, Generic, Patients

“We teach good communication skills because we accept not everyone has them. But we also need to teach professionalism… We’re happier to challenge poor clinical skills, or to point out a gap in knowledge, than to have a conversation about behaviour or attitudes.”

“We expect learners to improve and progress.  So, by implication, they’re not perfect professionals: they can make mistakes.  It gives people permission to say, “That doesn’t look professional”.”

“We need to work on accepting  constructive feedback for unprofessional behaviours… We want a positive culture, where we teach people to speak up to promote professionalism.”

[Sheona MacLeod, BMJ 2020;368:m768]

Easier to spot when people are being unprofessional than to teach professionalism!

Which makes me think the issue is conflicting values/priorities rather than not knowing what is “good medical practice”.

So dress codes become an issue when someone’s need to express their individuality or fit in with their peer group clashes with public perceptions of what a health care professional should look like.

Or someone complaining about their work place on social media is wanting to assert their independent spirit even as an employee, perhaps also their right to self expression and to attract “likes”.

And professionalism is clearly performative. We can swear all we want in our heads, but to swear out loud (in most cases) would be considered inappropriate. Which also potentially makes class an issue.

Expectations (of the public but also our peers) change over time. Consider suits, white coats, ties, scrubs. Consider also #medbikini twitter controversy – an article by men describes social media posts of women doctors in bikinis as potentially unprofessional.

Rosenthal 2011 – humanism and professionalism student module included blogging about clerkship experiences, debriefing after significant events, and discussing journal articles, fiction, and film. Main focus however was on empathy.

Scott Oliver and Kathleen Collins described differences in attitudes between medical school students which appeared to suggest a hidden curriculum. Students with more knowledge focus failed to identify potential issues of confidentiality, ethics or trust. Students who had not explored such issues struggled to know how to approach such issues even if they did recognise them.

Definition is probably best medical practice but also the duties and responsibilities of being an employee. High level morals/values (as in GMC good medical practice) are uncontroversial but also hard to then produce policies from (and which can then be defended in court when issues arise).

Clearly some personalities can be more playful, or disagreeable, which are not necessarily negative (cf engaging, whistleblowing) so perhaps more about defining the outer limits rather than homogenizing behaviour.

Surveillance capitalism; social dilemma documentary (mental health declines with higher use; disinformation campaigns; extremism encouraged by algorithms)

Social media is performative (Erving Goffman – the presentation of self (name of his book), life as theatre (metaphorically- although some say actual)). 

MedTwitter – now X of course 

Human face vs personality cult and influencers

Social media hygiene. 

Modelling in absence of “official” voices. 

Self curation of brand. Bordieu’s social capital. 

How to teach?

Learn through active reflection on work based learning (cf how artificial PBL etc are).  Think about values, how they shape communication.  Modelling of democratic values.  Appreciation of complexity of communication.

[ Alan Bleakley, Peninsula medical school – Homer as evidence of honour/shame/face directed behaviours, cf feminine, guilt directed behaviours etc]

“We teach good communication skills because we accept not everyone has them. But we also need to teach professionalism… We’re happier to challenge poor clinical skills, or to point out a gap in knowledge, than to have a conversation about behaviour or attitudes.”

“We expect learners to improve and progress.  So, by implication, they’re not perfect professionals: they can make mistakes.  It gives people permission to say, “That doesn’t look professional”.”

“We need to work on accepting  constructive feedback for unprofessional behaviours… We want a positive culture, where we teach people to speak up to promote professionalism.”

[Sheona MacLeod, BMJ 2020;368:m768]

Keratosis pilaris

Common, Dermatology, General paediatrics

Common (familial) – rough, bumpy skin on upper arms, usually asymptomatic but cosmetically not great. Follicular plugging, basically.

Can get quite inflamed in some people.

Exfoliating, moisturizing may help.

Sometimes goes with inflamed cheeks – Keratosis pilaris rubra faciei, so typical KP of the arms (often perifollicular redness), but can lead to hair loss and atrophy of eyebrows and scalp. 

Urea containing (Balneum, Hydromol Intensive, Eucerin intensive) creams or salicylic acid (in zinc oxide) paste may help. Topical steroids can be tried if very red but risky for face, given tendency to atrophy anyway.

Sesame allergy

Allergy, General paediatrics

An emerging food allergy over the last few decades in the UK.

Sometimes isolated, but 25% of peanut allergic are also sesame allergic.

Black, white, red varieties are found – in allergy terms identical.

Other co-sensitivities are pine nut (!), brazil nut and macadamia [Helen Brough, JACI 2020].

Sesame often used in bakery products, also in Far Eastern (gomashio, furikake are sprinkled over Japanese food) and Middle eastern food. Typical white seeds are obvious (and stick to everything, which makes cross contamination a big problem) but black sesame seeds found in Japanese cooking, and tahini (sesame paste, used in hummus and dressings), are not recognisably sesame seeds at all.

Sesame oil is generally unrefined, which is to say that it is likely to contain significant amounts of sesame protein and therefore trigger reactions. With many other kinds of oil, this isn’t the case because they are refined and lack proteins.

Evidence exists that ingested whole seeds can pass through digestive tract of allergic person without causing a reaction – which can confuse diagnosis and/or suggest tolerance when it isn’t. Or delayed rupture of seed case may cause delayed but severe reactions (90 mins plus after ingestion)

Some sesame allergic appear to be sensitized to oleosins, which are not water soluble so are not found in standard skin prick and IgE test solutions, potentially giving a false negative result. In 2020 study from Israel, SPT only 33% sensitive, cf 86% (although specificity also drops to 50%). So recommended that you test with both commercial solution and shop bought tahini – if never eaten/reacted, they recommend avoiding, especially if eczema and/or other food allergies.