Early onset primary immunodeficiency, characterised by eczema, viral skin infections, eosinophilia.
Later allergy, autoimmunity and malignancy.
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Food allergy diagnosis
Getting it right is important because otherwise people (child but also the rest of the family) end up anxious and scared of foods, cut out different foods, spend out on expensive alternatives, and risk nutritional/growth problems as well as aversion in the child.
In young infants, avoiding foods unnecessarily makes it more likely that you will become allergic in the future (“iatrogenic food allergy”). This is especially true with atopy and sensitisation – one series of 11 patients sensitized to cow milk found that all developed true cow milk allergy after a median time of avoidance of 2.3 years (with no significant improvement in their atopic dermatitis, which was the initial reason for avoidance). Pronuts study confirmed that multiple nut/sesame allergies was a factor of age – “secondary spread”. Similarly, in the Learning Early About Peanut (LEAP) study, it was precisely the infants sensitized to peanuts who were more likely to benefit from early introduction.
Having unproven food allergies also causes huge problems for schools and nurseries, and may lead to the public becoming sceptical of true allergy, with potentially disastrous consequences.
Getting it right can identify other potential allergies; it can help estimate risk of anaphylaxis; it can help with predicting whether the allergy is going to go away or not.
Allergy focused history
EATERS method –
- Exposure – did they actually eat it!? Or was there clear skin contact? Perhaps from surface contamination?
- Allergen (suspected) – one of the common ones? Although you can be allergic to pretty much anything, it is really rare to have an isolated rare food allergy.
- Timing – type 1 is immediate (within 15 minutes, rarely up to 1hr after) and then settles even without treatment within 24 hours. Rare to fluctuate.
- Environment – home (usually during weaning)? Outside home? Co-factors (infection, medicines, exercise, sleep deprivation) come in here.
- Reproducible – consistent reactions with exposure? May have had before with type 1 allergy but often on trying for the first time, and won’t have had recently. Milk/egg different, of course…
- Symptoms – type 1 vs non type 1. Some overlap of course.
Other issues are age (adolescents with hay fever more likely to develop secondary pollen food syndrome type allergies), alpha-gal allergy can be delayed up to 3 hours; raw vs cooked food sometimes makes a difference; usually you already have eczema and family history of atopy.
Testing
At the end of history taking, you should have be able to assess probability of type 1 allergy. If low, you may wish to proceed straight to challenge (unless reactions sound severe). Otherwise testing may help confirm or refute.
If negative/equivocal on initial skin prick or specific IgE testing, do another test! Skin prick if negative/equivocal IgE, and vice versa.
IgE Component testing may give added information, esp where potential pollen co-sensitisation – best evidence (mostly in US population, however) for Peanut, Hazelnut, Cashew (respectively Ara h 2, Cor a 14, Ana o 3 – other components may give extra information in some cases). Jug r 1 v specific (walnut) but not v sensitive.
Challenge
Challenge will be useful where results still equivocal – viz
- Results positive but never eaten or history inconsistent
- Results positive but possibly co-sensitivity without allergy
- Food in alternative form might be OK eg baked
Martha’s Law
13yr old Martha Mills died in 2021 at King’s College Hospital, London, of sepsis after pancreatic trauma (fell off bike in Snowdonia). The family went to the local minor injuries unit, where they were reassured. She continued to have severe pain however, then vomiting – so they went to the nearest hospital. She was admitted to the ward, then ICU, before being transferred by helicopter to King’s College London (1 of 3 specialist centres for pancreatic injury in the UK).
“We are so lucky to be here”, writes the mum.
She was NG fed, a peritoneal drain inserted. She started mobilising after 2 weeks. Then she developed fever and diarrhoea – started antibiotics. “The consultants swooped in, and were ostentatiously deferred to by the junior doctors.
“They were chatty, assertive, grand.”
Martha then started oozing from drip sites and peritoneal drain site. A scan showed small pericardial effusion. She had a persistent fever – and it was the start of a bank holiday weekend.
Consultant went home after morning round. Martha’s mum raised her concern about septic shock but was told “it’s just a normal infection”. She was told not to look up things on the internet – “you’ll only worry yourself”.
When she developed low BP and tachycardia, then widespread rash, it was diagnosed as a drug reaction.
““Trust the doctors – they know what they’re doing,” said the nurses.
The consultant was contacted to discuss worsening PEWS – did not come in – no change in management was made. The consultant phoned PICU (routine) but gave limited info – advised against review “to avoid parental anxiety”. The night shift junior did not review. Martha was drinking copiously.
At 0545 she had a seizure, at which point people started arriving and things seemed to happen – she was moved to PICU, intubated and then moved to Great Ormond Street hospital for ECMO. She died 4 days before her birthday.
Issues
- Mum is editor at Guardian newspaper
- Nothing to do with insufficient resources, overstretched doctors/nurses, or cuts, or a health service under strain
- Consultants dismissive and arrogant
- Juniors “performing” competence
- No one expressed concern, even if they had it
- Lack of note keeping
- Lack of consultant presence at weekend
- All doctors mentioned at inquest were men
Mum’s advice to parents
- Our trust in doctors should have limits. Plenty of clinicians prone to arrogance and complacency.
- However indebted you feel to the NHS, don’t be afraid to challenge decisions if you have good reason to.
- Remember most of the doctors in hospitals are just [sic] training. Don’t be afraid to ask how long a clinician has been qualified. Junior doctors are often green and trying to stay composed to impress their superiors.
- Make sure, if you can, that a single consultant has overall responsibility: we all know that if you’re answerable for something, you try harder.
- Google like crazy.
Aftermath
September 2023 Riya Harani dies from invasive Group A streptococcus and influenza B. Seen in hospital the day before. Junior doctor diagnosed a virus and discharged her with advice to take over the counter painkillers and info re: management of sore throat. Consultant not involved. At inquest, coroner says ““I think it highly likely that if it had been open to Riya’s family to seek a second opinion at that point, they would have done so without hesitation.”
UK Minister for health has said they will progress with the right to urgent second opinions across the health service.
Second Opinions
Right to second opinion already in Good Medical Practice: “In providing clinical care, you must respect the patient’s right to seek a second opinion”. But not traditionally associated with acute care and no detail otherwise.
Seeking a second opinion is more common in:
- women, middle-age patients,
- more educated patients, higher income or socioeconomic status,
- chronic conditions,
- living in central urban areas.
Motivation is to seek to gain more information, or reassurance. Potential major impact on patient outcomes in up to 58% of cases.
Solution
Condition Help (Pittsburgh, 2000s), Call 4 concern (Royal Berkshire) – hospital hotline to call rapid response team to bedside. Ryan’s rule (Queensland) is state-wide number for review of medical care.
But evidence of benefit sparse. Tends to be pain management and communication breakdown rather than acute deterioration. 18% of patients generated nearly half of all calls to Condition Help (in 41.4% of cases, a change in care was made).
International Society for Rapid Response systems includes family trigger system as one measure of effectiveness.
“The recurring problems of hierarchy, arrogance and poor culture have not been tackled despite decades of effort… It is not the job of patients and families to wait around for healthcare providers to sort out their culture.” [(Helen Haskell, BMJ 2023)]
Such systems do not address problems of overcrowded wards, lack of beds, delayed assessments, poor nurse:patient ratios etc… Perhaps don’t appreciate informal senior discussions that happen all the time. Potential for delays in appropriate treatment if process of getting second opinion interferes with management?
“I’d like to imagine a world in which Martha was transferred to intensive care in time and her life was saved.In this parallel universe, I talk endlessly about the doctors and nurses who helped her. I go on a fundraising walk for the hospital.
Bright and determined girl as she was, Martha aces all her exams, goes to university, has a career and children.
She visits us at weekends and we recall those distant weeks when she was in hospital.”
Mrs Mills
Medically unexplained symptoms
Royal College of Psychiatrists and Paediatric Mental Health Association guidance on MUS – https://www.rcpsych.ac.uk/mental-health/problems-disorders/medically-unexplained-symptoms
Perplexing presentations (PP) – term is used to describe the presence of alerting signs when the actual state of the child’s physical or mental health is not yet clear and there is no perceived risk of immediate serious harm to the child’s physical health or life.
Cf Factitious or Induced Illness (FII, prev Munchausen by Proxy) –
- Mostly associated with the mother
- extreme end of spectrum of abnormal parenting behaviours, from ‘mild’ erroneous belief or overanxiety at one end, through manipulative fabrication to ‘severe’ imminently dangerous induction of illness at the other end.
- Danger that effect on the child underestimated.
- Even milder abnormal parental behaviours creates difficult relationships between family and health professionals
Features of FII
- Unwitnessed, or at least, not independently observed
- Poor response to treatment
- Unexplained impairment esp school but also social
- New symptoms, and multiple opinions sought; yet frequent WNB
- Changes of school/clinician, complaints and demands. Home schooled often – which also makes it harder to get independent observations
But these features are only to raise suspicion, not diagnostic! RCPCH has guidance.
Is there a risk of harm? If in doubt, discuss early with safeguarding team, rather than trying to decide alone on whether to involve social services.
Offer a “current consensus opinion” together with at least one other professional, and ideally input from education/nursery! Continue to monitor until things improving. No right to access parental health records however –would need explicit consent.
Chronology needs to include basis of previous diagnoses, and justification
Admission for observation – some special issues:
- What should nursing staff be looking for?
- Is 24/7 supervision required or possible? Can they leave the ward?
- Where do you keep notes?
Second opinions – for specific issues, and ONLY TO HELP YOU manage the child. And necessary to provide background of concerns.
Discussing with family –
- Bringing father, and child’s own views, into the picture helpful. Child often defers to mother in these cases [presumably as their views are ignored]
- reassure the parents that there is no current health concern, but emphasise that the team can/will still support and manage any condition the child has
Truancy and going missing as risk factors for sexual exploitation. Experience of conflict, threat and violence – so dealing with these as protective, along with access to safe, stable accommodation, increasing awareness of rights
Acutely – toxicology, parental responsibility, local sexual assault referral centre (SARC – but don’t wait on them), specialist police (good if hesitant). emergency contraception, post-exposure prophylaxis (PEP) incl hepatitis B vaccination. Advice from the sexual offences examiner (SOE) or forensic medical examiner (FME). Document if unable to get consent.
Forensics – urine samples, toilet tissue used during the admission, clothing at the time of presentation, underwear and any sanitary wear. Option for self referral for forensic exam (13+yrs) where police are not required.
General paediatrician may be required to get involved with sexual offences examiner, with view to paediatric history, consent issues, referring to social services and/or psychological support
Police protection (which can involve transport to secure location) does not confer right to consent to treatment – needs emergency protection order.
Up to 18 in some respects – Children’s act 1989 defines child as under 18.
Neglect
Type of abuse.
Neglect is not always easily recognisable in children and young people with disabilities – demonstrate emotional distress in different ways; for example, through repetitive or challenging behaviours and self-injurious or self-harming behaviours.
Usually involve neglect across not just health issues but other domains eg hygiene, stimulation, appropriate clothing, protection from harm.
If in doubt, the graded care profile (GCP) tool can help to assess the severity of neglect.
Child protection
Abusive head trauma is the most common cause of traumatic death in infants and can cause severe neurodevelopmental delay, hearing and speech problems, impaired vision and blindness in surviving infants.
Severe faltering growth is associated with neglect. During the first 2 years, a child’s brain grows rapidly, and even moderate nutritional deprivation during this period of rapid brain growth and differentiation can lead to adverse neurodevelopmental outcomes.
Neglect is associated with developmental delay, particularly delays in expressive language and imaginative play.
Several studies have reported associations between child maltreatment and ischaemic heart disease, chronic lung disease and liver disease in adulthood.
Those who have been maltreated as children are more likely to engage in smoking, drinking alcohol and engaging in risky behaviour which can give rise to physical health problems.
Child abuse is also strongly associated with obesity in later life.
Self-harm is strongly associated with sexual abuse, although not necessarily with physical abuse and neglect.
Physical abuse and sexual abuse are both associated with a doubling of the suicide risk for young people.
Children who have been physically or sexually abused are also more likely to experience intimate partner violence as adults.
Peter Connelly (also known as ‘Baby P’)
Peter died at the age of 17 months with multiple injuries including rib fractures and a fracture-dislocation of the thoracolumbar spine.
The inquiry into Peter’s death identified numerous opportunities where health and other professionals could have intervened to protect him from harm.
Training requirements
Over a 3-year period, professionals should be able to demonstrate refresher education, training and learning equivalent to:
- a minimum of 8 hours for those requiring Level 3 core knowledge, skills and competencies
- a minimum of 12 to 16 hours for those requiring role-specific additional knowledge, skills and competencies
- multidisciplinary and interagency and delivered internally and externally.
It should include personal reflection and scenario-based discussion, drawing on case studies, child safeguarding practice reviews (local and national) and the lessons learned from research and audit.
Protective factors
- Social support for mothers, safe play areas, at least one secure attachment
- adequate income and good quality housing
- good nutrition
- safe play areas and appropriate learning materials
- good quality day care (developmentally appropriate, skilled staff, attention to non-educational needs, parental engagement)
- academic socialisation (school readiness programmes)
- links with other adults and community networks good antenatal care have been found to promote resilience
Deaths
Child Safeguarding Practice Reviews or CSPRs (previously known as Serious Case Reviews or SCRs).
SIDS = sudden death of an infant under one year of age that remains unexplained after a thorough case investigation, including the performance of a complete autopsy, examination of the death scene and review of the clinical history
Livedo only becomes permanent around 4 hours after death, so if the infant is moved in this time period, and the livido not recorded, this information may be lost. Other marks such as those from strangulation may also fade.
Test should be down before declaration of death!
- amino acids, newborn blood spot test, toxicology and cytogenetics
- CSF
- Urine (from nappy if necessary)
- Skin biopsy for fibroblast culture
For deaths at home/community, senior police and healthcare should decide on whether there should be a visit, how soon (within 24hrs),who should attend. Routine for sudden infant death.
All child deaths in England are reviewed by a child death overview panel (CDOP) established by the local safeguarding children partnership (LSCP).
In the UK, an estimated 1 to 2 children die per week as a result of maltreatment.
National Case Review Repository website – for details of how review should be done
Purpose is establish what lessons are to be learned from a case to safeguard and promote the welfare of children
Wider cultural factors associated with increased risk include:
- attitudes towards parental responsibility
- attitudes towards rights of the child
- attitudes towards violence and crime
Mental health professionals, addiction services and learning disability services working with adults (as well as probation, social care and police) have a duty to consider the welfare of any children dependent on those adults.
Writing Medical Reports
When writing a medical report:
- avoid medical terms wherever possible and use common English
- if medical terms are required, attempt to provide the common English term as well
- conclude by stating whether the injury is consistent with the explanation provided
- if no explanation is provided, outline the most likely explanation (where possible)
ACEs
Abuse and neglect obviously, but also family dysfunction eg divorce/separation, domestic abuse, drug/alcohol misuse.
67% of people have experienced at least one. 10% have experienced 4+
Increased risk of physical ill health – dysregulated stress? Plus unhealthy coping behaviours, of course.
Online abuse
NSPCC (Childline) for family advice. MindEd.org.uk https://www.minded.org.uk/Catalogue/Index?HierarchyId=0_45158_45175_45180&programmeId=45158
Report concerns to Internet watch foundation and CEOP https://www.ceop.police.uk/Safety-Centre/
Trafficking
Expensive clothes/phones?? Tattoos and specific colours can be associated with gangs.
What is the quality of the relationship with the accompanying adult?
Modern Slavery and NSPCC have helplines.
County lines = mobile phone numbers used to take drug orders. Children may be used to transport money or drugs, often across geographical boundaries. Common for properties (typically belonging to vulnerable people) to be used as bases for criminal activity.
Financial security at home can drive attempts to get involved with activities perceived as “profitable”
“Trauma informed care”???
LAAC
Residence orders, or guardianship order (to do with fostering), or long term placements with foster carers, take a child out of care system.
Placement order is for prospective adopter – gives rights
Information about the birth parents can be obtained without consent (for example, if it is not possible to trace a birth parent), if information is anonymised as far as possible to serve the intended purpose. [General Medical Council guidance on confidentiality]
Unaccompanied asylum seekers –
- hearing issues (potentially trauma-related)
- dental and vision issues (never checked)
- haemoglobinopathies (never screened)
- immunisations (uncertain or incomplete)
- malnutrition or growth issues
- worms
- tuberculosis or blood-borne viruses
- female genital mutilation
- issues with emotional health and wellbeing (these may be ongoing if still going through the asylum process)
RCPCH has guidance, as does UASChealth.org and refugee council.
Section 20 agreement is for voluntary fostering – does not give local authority parental responsibility
Foster parents do not usually have parental responsibility.
Attachment affects positive vs negative inner working models.
Survival behaviours – lying, hypervigilance, hoarding/stealing, attention seeking
Screen time
estimates suggest that 11 to 14 year olds in the UK have an average daily screen time of 9 hours – [no idea where this comes from. Self reporting is obviously pretty meaningless. Lots of evidence of screen time going up post COVID esp girls, esp lower educational attainment]
Infant mental health
Feeling safe and experiencing responsive care, able to explore/play.
Rights based participation
UNCRC report June 2023 -Scotland should prioritise non-discrimination, abuse/neglect/sexual exploitation, children “deprived of family environement”, mental health, asylum seeking and refugee/migrant children.
Should develop/strengthen strategies for community based therapeutic mental health programmes for children of all ages, mental health promotion, screening and early intervention.
Benefits to children are not the point – just a human right, under article 12.
Right of individual child but also of groups of children.
Voice is not enough (2007) – Lundy Model:
- Space – to form/express views safely
- Voice – facilitated to express veiws
- Audience – views should be listened to
- Influence – views must be acted upon as appropriate
Space – actively created! Not just reactive. And demonstrably safe. Consider the “seldom heard” child eg talking mats.
Voice – silence can be clear in meaning! Mode of expression? And support to understand options, possibilities. Lundy has published European commission guide to creating child-friendly documents
Subjects that are uncomfortable/awkward in particular – how can they be framed in way that can be discussed openly?
Audience – active listening. Relevant decision makers.
Influence – High expectations are good! But transparent about what is possible. Feedback and follow up.
Framework for feedback:
- What did you agree with?
- What if anything surprised you and why?
- Did you disagree with anything? If so, what and why?
- Has it influenced your views in any way? If so, how?
- What have you decided?
- What is happening next and when?
NI government has evaluation checklist and CYP feedback form.
Talking Mats – Margo
Structured visual communication – real or virtual world. Used for 2020 “Can Scotland be Brave?” report
Jones and Welch 2018 – representation (avoiding adult bias), judgement (viewing children as capable of making informed decisions), validity (even if different from adult views), Impact (how acted on)
What we think is “fine” because it’s what we are used to may not be for kids – eg hospitals/clinics.
Who all is in the room? Why are they there?
Beware leading questions
Before training, only 23% felt confident that CYP views were represented, rose to 89% after. Feedback from children was overwhelmingly “just nice to be listened to”.
Trust in organisations
“Boeing in 2018/9 after the crashes of two 737 Max 8 aircraft was
following a popular playbook:
- First, deny any problem; then
- sow doubt about claims that your products or practices cause harm.
- Once the problem becomes undeniable, endeavor to deflect responsibility for the problem,
- when deflection is no longer tenable, try to minimize or localize the problem eg blame lower-level employees”
Gives other examples of George W. Bush and Abu Ghraib camp (abuse attributed to
“a few American troops”).
Purdue Pharma – in response to public criticism and lawsuits for its irresponsible opioid marketing strategy – tried to be seen as part of the solution rather than the cause of the problem.
Trust is based on perceptions of that institution; in contrast, trustworthiness is a quality we attribute. Trying to boost trust without addressing underlying reasons for the loss of trustworthiness are unlikely to succeed, and usually perceived as inauthentic.
You can measure trust (by asking people about their perceptions and beliefs) but not trustworthiness, which is more nebulous.
We tend to talk about trust as being a one dimensional thing but there are probably different kinds of trust – (gives example of a successful financial advisor who has had multiple divorces – you might trust them for financial but not relationship advice). Do they have knowledge, skills, resources (often quite specific) to perform what you have entrusted them to do?
Trustworthiness on the other hand is built around questions of reliability, honesty, and integrity. If you have reliability trust in someone, then you believe that person does (or will do) what they say they do (or
will do).
Along with integrity, there are the values of fidelity, care, and benevolence—relates to putting others’ interests ahead of one’s own. Which raises the question, “whose interests are being privileged?”
So called crisis management experts talk about “optics” – public perception – and respond to it by “public performativity” of trust building in terms of use of language and symbolic actions.
Marks suggests you compare one kind of crisis he calls “opsis,” (ancient Greek word for “appearance” as used by Aristotle for one of his six elements of tragedy, often translated as “spectacle”) with institutional sepsis. “Just as medical sepsis in the human body is a critical condition that endangers life, the loss of an
institution’s integrity and trustworthiness is another form of sepsis—ethical sepsis—that poses an existential threat to the institution. A problem even when the loss of integrity and trustworthiness has not yet come to the attention of the public.
Gives vaccine hesitancy as another example – numerous and varied causes, including misinformation, but note strong ethnic patterns at time of Black Lives Matter campaign and NHS being called “institutionally racist”. Suspicion of corporate interests in public health messages too.
[Jonathan H. Marks, Hastings Centre]Keeping up to date
Years ago it was already pointed out that there is way too much published research for the average doctor to keep up to date. Even to just be aware of all the guidelines that summarize research into best practice means reading hundreds of pages for the conditions that you might only infrequently see.
Of course you don’t always know if the patient in front of you is typical of the condition being discussed – research often excludes complicated cases (or children, or pregnant women).
Even then – “most published research findings are false” [Ioannidis, Plos 2005]. Lots of findings are never confirmed by further studies, and “knowledge” is based on a p value of <0.05. Actually depends on pre-test probability… Are certain things already known? Or is there just a lot of data without established relationships? Even if research design is perfect, bias eg selective reporting or manipulation of analysis. Different studies may use different end points or definitions, which increases the chance findings are false. True findings can be lost in noise or concealed by conflict of interest. Fixed beliefs may be as prejudicial as financial conflict of interest… Expert opinion often differs from outcomes of metanalysis. Small studies and small effects mean any significant result is more likely to be false.
In the model discussed in this study, an underpowered early phase clinical trial that produces a positive finding is likely to be misleading 75% of the time, even before you consider bias. If you are talking about a field where there probably isn’t actually any relationship between the things being studied, then large effects with high significance may just reflect the degree of bias, and should be seen potentially as a warning rather than something exciting!
Authors don’t check primary sources so misconceptions promulgate. Peer review is inefficient, inconsistent and insufficient. Post publication retractions are messy and difficult. See the problem of citations, below.
Systematic reviews are not kept up to date – in fact, they are usually already out of date when published…
Authors of guidelines have a particular duty to ensure rigorous analysis.
The average 10 min consultation will produce at least 1 unanswered question.
[Richard Smith BMJ 2010]
The problem of citations
Citation error rate is estimated at 11-15% in biomedical literature. Propagates mistakes (even academic urban legends eg iron in spinach, due to a misplaced decimal point in a 1930s paper, which I have not verified) and undermines respect for literature review.
Can be non-existent findings, incorrect interpretations of findings, or (20% of errors) chains of errors. Sometimes a hypothesis becomes a fact.
1 surgical study was found to be misquoted by 40% of articles that cited it!
AI can help or make this worse. CONSENSUS.app is AI powered search engine for academics.
Best would be a declaration, that the authors have read the original papers and checked for accuracy and relevance.