Monkeypox

=mpox (considered less stigmatising?).

Emerging infection particularly in men who have sex with men. Reached the UK in 2022.

New variant (Clade I) has high mortality, started in Central Africa (Congo, Central African Republic, Burundi, Uganda), now in Kenya, first case now reported in Europe (Sweden, 2024). Grade 3 human pathogen (along with Yersinia pestis, O157, TB, anthrax…).

Viral haemorrhagic fever found in these areas too, of course…

Incubation period is 5-21 days. High risk would be household contact, mucosal (with bodily fluids) or broken skin, inhalation without PPE if cleaning room or changing bedding. Medium risk would be intact skin with bodily fluids or face to face within 1m considered medium risk – do not need to isolate but should be offered post-exposure prophylaxis. See PHS matrix.

Besides blistering rash, can cause fever, sore throat, lymphadenopathy, myalgia.

Swab blistering lesion, or if none then throat. MSS (molecular sampling solution, as used for flu etc) ideally otherwise extra transport precautions required. Mark sample “suspected HCID”, notify lab in advance – needs to arrive for 9am!!!

Cases are asked to self isolate at home.

PPE – as for viral haemorrhagic fever. https://learn.nes.nhs.scot/58193/high-consequence-infectious-diseases-hcid

Post-exposure prophylaxis with MVA-BN vaccine (Imvanex®) offered within 14 days. Pregnant and children under 5 considered at risk.

Smallpox vaccine was considered effective.

Mental health emergency

Firstly, is there a suicide risk?

Then, consider mental health needs. Is there an alcohol or drug issue?

Are they known to social work? Are there any child protection issues for the young person? Their siblings or other family members? If young person is over 16 then consider Adult Protection measures (Scotland Act 2007).

A proper mental health assessment requires that they are physically well enough (consider intoxication, sedation, pain etc). Consider competency (which can be impaired temporarily by physical illness).

Consider:

  • Violent/aggressive behaviour – needs risk assessment and management
  • Evidence of learning disability
  • Any existing care plans or coping mechanisms?
  • Psychosis? ie delusions, hallucinations
  • Unusually withdrawn/quiet is a red flag.

Chronic Variable Immunodeficiency

=CVID. Another terribly named condition.

Usually presents in adulthood but about 20% in childhood. Typically recurrent infections of ears, sinuses, lungs – usual bugs, not funny ones.

Bronchiectasis may develop. In some cases granulomas develop.

Lymphadenopathy +/- splenomegaly is sometimes a feature.

Autoimmunity is an important feature – low red cells or platelets, thyroid disease.

Enteropathy and arthritis can be seen.

Diagnosis

Low IgG, usually IgA, sometimes IgM. Functional antibodies (to pneumococcus, tetanus, Hib) low.

Treatment

Immunoglobulin replacement – IVIG or subcut immunoglobulin, regularly.

Prophylactic antibiotics in some cases. Screen for infections esp chronic GI.

Bias

In research, many studies are non-randomized, so risk of bias.

Newcastle-Ottawa scale is one attempt to assess bias formally – judged on:

  • the selection of the study groups;
  • the comparability of the groups;
  • the ascertainment of either the exposure or outcome of interest for case-control or cohort studies respectively. 

So things like cohort not being representative; control group coming from different population; measurement being rather subjective; duration of follow up – all increase risk of bias.

Publication bias

Suspicious if small study with big effect!

Vasovagal syncope

=Faints, “whitey”.  Insufficient blood to the brain, due to immature or hyporesponsive autonomic nervous system control of cardiac output and peripheral vascular tone.

Similar symptoms with POTS but with obvious drop in blood pressure.

Symptoms of light headedness, feeling hot/clammy, dizziness, black spots in vision or tunnel vision (pre-syncopal), potentially followed by collapse, which can be remembered in most cases. Can be brief posturing or clonus due to hypoxia, but only for a few seconds, which freaks everyone out as it looks like an epileptic seizure.

Recovery is fast, within seconds or a minute, once circulation to brain improves – requires that person is left lying on ground and not propped up!

They look pale, feel hot, but once on ground go clammy/sweaty. Heart rate and blood pressure typically low.

Typically fast growing teenagers, but can be anyone.  In some, lifelong tendency.  You can suffer significant facial trauma from syncope so it’s not trivial, if recurrent can also be disabling with significant psychological co-morbidity. Usually prolonged standing, or standing up after sitting/lying for a while (especially boys going to the toilet for a pee).  Other common triggers are physical stimuli (reflex syncope) eg painful procedures, esp immunisation (distinguish from anaphylaxis), cannulation.  Some unusual triggers eg hair brushing/combing.

Mostly trivial but potential for injury (eg facial fractures) and can be disabling.

Useful to become aware of triggers, so countermeasures can be tried.  But sometimes no warning, which is difficult.  Worth asking directly how often symptoms happen, as child might not tell anyone.

Other differentials are epilepsy, arrhythmia. Syncope with exercise is a red flag.

Abortive manoeuvres

  • Lower body muscle tensing – abdomen, buttocks, thighs
  • Same, but with leg crossing
  • Whole body tensing!
  • Squatting
  • “Brace” position – sit with head between knees

These should all work, probably worth practicing though.  And should work within seconds.  You hopefully only need to keep doing it for 30-60 seconds. [Krediet, J Appl Physiology 2005 Vol. 99 no. 5, 1697-1703 DOI: 10.1152/japplphysiol.01250.2004]  All of the evidence seems to come from the Netherlands!  Some evidence for isometric arm/hand exercises, but might be incidental abdominal tensing at same time!

Preventive measures

Regular meals/snacks. Avoid big carbohydrate meals though – low GI foods (ie wholegrain/wholemeal) best to avoid big swings in sugar levels. Avoid sugary drinks for the same reason.

Getting hungry is probably bad – have emergency snacks available (oat cakes, nuts, malt loaf, dark chocolate – not high sugar).

Drink plenty – consider increasing to 2-3 litres. Try to drink 1L in the first 2 hours of the day.

Caffeine?

Salt intake may need to be boosted, especially if a “healthy” eater, or losing a lot in sport. Slow salt tablets?

Good cardiovascular fitness.

Could try putting blocks under the foot of the bed to raise it up 2-3 inches! Retrains baroreceptors.

Medication

Desmopressin, to increase circulating blood volume?

Beta blockers? Fludrocortisone? See POTS.

Patient/family info at www.stars.org.uk.

Graphic stories

A creative way of learning/teaching about illness. But biases around being for kids, or frivolous, or simplistic.

But actually the ability to use images, fonts or other text effects means you can express things in ways both delicate and brutal that might require a lot of reading. Visual understanding is often more intuitive. By combining the two you are involving the different parts of the brain that handle language and image processing, and research shows understanding is enhanced.

They teach observational skills – you read but you must also interpret what is implied.

See MediKidz and GraphicMedicine.org.

POTS

POTS (Postural orthostatic tachycardia syndrome) – more common in females. 

Orthostatic tachycardia (NOT hypotension, which suggests vasovagal syncope), dizziness, chest pain, palpitations, headaches, dyspnoea.  Sometimes bluish red discolouration in lower limbs. 

No known cause, can have sudden onset in previously fit individuals.  Associated with Ehlers Danlos (venous return problem?). 

Can be debilitating, associated with chronic pain, sleep problems, GI symptoms.  Can improve over time.  Diagnosis – heart rate increases by 30 beats per minute (bpm) or more (40bpm in those aged 12-19) within 10 minutes of standing, or if it increases to more than 120bpm. “Hyperadrenergic” POTS is where BP actually goes up, rather than down.

Monitor during valsalva manoeuvre to look for autonomic dysfunction.

Increase fluid intake to 2-3L daily. Increase salt intake?

Waist high compression stockings?

Consider treatment with beta blocker, fludrocortisone, desmopressin, clonidine, modafinil, SSRI.

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.
[Mich Erlewyn-Lajeunesse, ADC 2019]

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

  1. Our trust in doctors should have limits. Plenty of clinicians prone to arrogance and complacency.
  2. However indebted you feel to the NHS, don’t be afraid to challenge decisions if you have good reason to.
  3. 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.
  4. 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.
  5. 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 herI 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