Scarlet fever

Cite this article as:
Tessa Davis. Scarlet fever, Don't Forget the Bubbles, 2018. Available at:
https://doi.org/10.31440/DFTB.15511

The start of 2018 has seen UK hospitals receiving an alert from Public Health England about the rise in cases of suspected scarlet fever. What is the extent of the problem and how good are we at actually diagnosing scarlet fever?

DFTB go to South Africa

Cite this article as:
Tagg, A. DFTB go to South Africa, Don't Forget the Bubbles, 2018. Available at:
https://dontforgetthebubbles.com/__trashed/

When Annet Alenyo Ngabirano spoke about Ubuntu at DasSMACC one of things everyone wanted to know was how could they help. There was talking about donating to Supadel or supporting African academics and writers . Another suggestion was for those of us who ware lucky enough to get a study budget to go to Africa and share our knowledge and experience. When Kat Evans came to Brisbane last year to speak at DFTB17 she was very excited about her upcoming secret project.

Arjun Rao: Sepsis at DFTB17

Cite this article as:
Team DFTB. Arjun Rao: Sepsis at DFTB17, Don't Forget the Bubbles, 2018. Available at:
https://doi.org/10.31440/DFTB.15246

This talk was recorded live on the second day at DFTB17 in Brisbane. If you missed out in 2017 then why not book your leave for 2018 now. Tickets are on sale for the pre-conference workshops as well as the conference itself at www.dftb18.com.

A Difficult Beast

Cite this article as:
Alyssa Courtney. A Difficult Beast, Don't Forget the Bubbles, 2017. Available at:
https://doi.org/10.31440/DFTB.12455

A 14 year old girl was brought in by her family with lethargy, agitation, and confusion. Her past medical history includes depression and a recent viral upper respiratory tract infection.

 

Bottom Line:

  • Encephalitis is caused by inflammation of the brain and is difficult to diagnose and manage.
  • Up to 50% of patients experience short-term deficits with 20% experiencing severe sequelae.
  • Overall 10% mortality.
  • Discover the risk factors and first-line investigations!
  • Unique Australian aetiologies: Hendra virus, Australian bat lyssavirus, Murray Valley encephalitis virus and West Nile virus (Kunjin virus) infection.
  • Immune-mediated encephalitides are increasingly noted, these potentially respond to immunomodulatory treatments and have association with underlying tumours.
  • Definitive aetiology may not be identified for 30%–40% of patients with encephalitis.
  • Recovery from encephalitis reaches a plateau at approximately 6–12 months.
  • Rehabilitation assessment (medical and non-medical) should be considered, especially in those with neurological or neuropsychological deficits at discharge.

 

Epidemiology:

  • Annual Australian hospitalisation rate 5.2/100 000.
  • Case fatality rate is estimated to be 4.6%.
  • The highest admission rates are observed in males, and those aged less than 9 or over 60 years of age.

 

How do we diagnose it?

Diagnostic Criteria

Major Criterion (required):
 Patients presenting to medical attention with altered mental status – defined as decreased or altered level of consciousness, or lethargy or personality change – lasting ≥24h.

Minor Criteria (2 for possible encephalitis; ≥3 for probable or confirmed encephalitis):

  • Documented fever ≥38°C (100.4°F) within the 72h before or after presentation.
  • Generalised or partial seizures not fully attributable to a pre-existing seizure disorder.
  • New onset of focal neurologic findings.
  • CSF WBC count ≥5/mm3.
  • Abnormality of brain parenchyma on neuroimaging suggestive of encephalitis that is either new from prior studies or appears acute in onset.
  • Abnormality on electroencephalography that is consistent with encephalitis and not attributable to another cause.

AND

Exclusion of encephalopathy caused by trauma, metabolic disturbance, tumour, alcohol abuse, sepsis and other non-infectious causes.

Important Differential Diagnoses

Consider:

  • Meningitis without parenchymal involvement: bacterial, viral, other (e.g. TB, cryptococcus).
  • Cerebral abscess (below) and other forms of intra-cranial suppuration.

  • Infection associated encephalopathy (e.g. septic encephalopathy, acute nectrotising encephalopathy (ANE)).
  • Vascular disease: ischaemic/haemorrhagic cerebro-vascular accident (CVA), cerebral vasculitides (e.g. systemic lupus erythematosus).
  • Hypertensive encephalopathy including posterior reversible encephalopathy syndrome (PRES).
  • Neoplastic: primary CNS or malignancies.
  • Toxin induced encephalopathy: alcohol, illicit drugs, other drugs (especially neuroleptics, cyclosporin).
  • Metabolic encephalopathy: hepatic, renal, hypoglycaemia, hyponatraemia, hypocalcaemia, thiamine deficiency, Wilson disease.
  • Neurodegenerative: Creutzfeld-Jacob disease (other prion disease), neuroacanthocytosis.
  • Demyelinating disease: multiple sclerosis (MS), neuromyelitis optica (NMO or Devic disease).
  • Endocrine: Hashimoto’s encephalopathy/steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT), Addisonian crisis.
  • Psychiatric: psychosis, catatonia.
  • Seizure disorder.
  • Traumatic brain injury.

 

Causes:

Viruses are the most commonly identified agent in all settings.

Immune-mediated aetiologies are increasingly recognised in up to one third of cases, and are important because they are often treatable.

 

Selected paediatric immune-mediated encephalitides:

ADEM

Acute disseminated encephalomyelitis is an inflammatory, multi-focal, demyelinating condition of the central nervous system. It presents with encephalopathy and multi-focal neurological deficits. Mean age 5–8 years old, slight male predominance. Diagnosis by MRI. Corticosteroids are the established first-line therapy, with other immune-modulatory therapies used in refractory cases.

Acute haemorrhagic leuco-encephalopathy (AHLE) is a rare, hyper-acute form of ADEM that overlaps with cerebral vasculitis.

 

Anti-NMDAR

Anti-N-methyl-D-aspartate receptor encephalitis has been shown to be one of the principal causes of encephalitis. It typically presents with psychiatric symptoms, seizures, memory loss and mutism. The syndrome evolves to include movement disorders, dysautonomia and sometimes hypoventilation. MRI is most often normal. It is diagnosed by identifying CSF or serum antibodies against the NR1 subunit of the NMDA receptor.

Anti-VGKC

Anti-voltage-gated potassium channel-complex encephalitis includes a broad clinical spectrum. In children it presents as temporal lobe focal seizures, status epilepticus and encephalopathy (behavioural disturbance, hallucinations) and cognitive decline.

 

Check out these vital risk factors!

  • Neonate (<4 weeks): HSV-2, CMV, toxoplasmosis, T. pallidum (syphilis), L. monocytogenes, enteroviruses parechovirus.
  • Infant/Child: HSV, VZV, enteroviruses, HHV6/7, M. pneumoniae, EBV, parechovirus, Bartonella sp., ADEM.
  • Female: anti-NMDAR.
  • Immunocompromised patient: HHV6, CMV, EBV, measles, VZV, LCMV, toxoplasma, cryptococcus, JCV, BKV, Bartonella sp.
  • Tropical Australia: JEV, dengue, MVEV, KUNV, B.pseudomallei.
  • Travel history
    • Asia: JEV, dengue, malaria, MTB, Nipah, Angiostrongylus cantonensis.
    • Pacific: JEV, dengue, malaria, MTB, Angiostrongylus cantonensis.
    • North America: WNV, LACV, SLEV, CTFV, EEEV, neuroborreliosis, Rickettsia rickettsia (RMSF), ehrlichiosis (HME), anaplasmosis (HGA), babesiosis, coccidiomycosis.
    • South America: WNV, VEEV, dengue, MTB, trypanosomiasis (Chagas).
    • Europe: TBEV, TOSV, neuroborreliosis, anaplasmosis (HGA).
    • Africa: malaria, trypanosomiasis, MTB.
  • Animal exposure
    • Monkeys: herpes B, rabies.
    • Bats: rabies, ABLV.
    • Dogs and other canids outside Australia: rabies.
    • Cats: Bartonella hensellae.
    • Horse: Hendra, KUNV.
    • Rodents: LCMV, leptospirosis.
    • Snails/other moluscs: Angiostrongylus cantonensis.
    • Swine: Nipah.
    • Mosquito or Tick bite history.
    • Arboviruses: MVEV, KUNV, JEV, dengue in Australia by region.
    • Rickettsiae: Rickettsia typhi, R. australis, R.honei, Orientalis tsutsugamushi in Australia by region.
    • Other: neuroborreliosis, ehrlichiosis (HME), anaplasmosis (HGA).
  • Recreational
    • Sexually transmitted: HIV.
    • Fresh water: leptospirosis, Naegleria fowleri.
    • Soil/mud: Balamuthia mandrillis.
  • Occupational
    • Animal husbandry, farming: C. burnetii (Q fever), leptospirosis.
    • Abbatoir workers: C. burnetii (Q fever).
  • Unvaccinated: measles, mumps, rubella, VZV.

 

Examination:

Level of consciousness, subtle seizure activity, meningism, abnormal movements (e.g. chorea, parkinsonism), weakness, sensory loss and cranial nerve involvement (including deafness and anosmia).

Features of raised intracranial pressure or autonomic dysfunction. Mental status examination should be recorded. A rash or other skin lesions, respiratory or gastrointestinal signs may give clues to the aetiology.

 

First-line Investigations:

CSF: Opening pressure, microscopy, gram stain and bacterial culture, cell count and type. Biochemistry: protein, glucose. PCR: HSV, enterovirus, VZV. Antibodies: oligoclonal bands, VZV IgG Antigen: cryptococcal Ag.

Serum Serology: HIV, flavivirus, M. pneumoniae, EBV

Respiratory PCR testing for enterovirus, influenza A and B, adenovirus

Faeces PCR or antigen testing for enterovirus, adenovirus, rotavirus (child); enterovirus culture/typing

Skin swabs (where lesions present) PCR testing for HSV 1/2, VZV, enterovirus

Neuroimaging MRI (sequences to include: T1,T2, FLAIR, DWI, gradient-echo, gadolinium contrast)

EEG

 

Directed Management

HSV: Minimum 14 days intravenous acyclovir for immunocompetent patients and 21 days for immunocompromised patients. Consider repeat lumbar puncture for CSF HSV PCR at planned completion of treatment especially in immunocompromised and children.

VZV: Consider 7–14 days intravenous acyclovir with or without corticosteroids in consultation with an infectious diseases specialist.

Enterovirus: Intravenous immunoglobulin if hypogamma-globulinaemic. Intravenous immunoglobulin is used widely in Asia for enterovirus.

CMV/HHV6: Reduce immunosuppression and consider ganciclovir and/or foscarnet in consultation with infectious diseases specialist.

Rabies or ABLV: Consider Milwaukee protocol in consultation with infectious diseases specialist.

ADEM: Methylprednisolone 30 mg/kg daily in children up to1000 mg (adult daily dose) for 3–5 days in consultation with a neurologist. Second-line treatments in consultation with a neurologist. Ab-mediated: Immunosuppressive therapy in consultation with a neurologist. Investigation for underlying tumour and removal (where indicated). Ongoing tumour surveillance.

 

Prognosis:

  • Overall mortality of encephalitis is approximately 10%.
  • Up to 50% of patients experience short-term deficits with 20% experiencing severe sequelae; long-term outcome is poorly characterised, and neuro-cognitive sequelae likely underestimated.
  • Adverse prognostic features: depression of consciousness at presentation is the main adverse prognostic feature. Poor outcome has also been associated with refractory status epilepticus, intensive care unit admission, focal neurologic signs, abnormal MRI findings, extremes of age and immune compromise, a diagnosis of HSV in adults, and JEV or mycoplasma pneumoniae in children, or delay in the initiation of directed therapy.

Recovery from encephalitis reaches a plateau at approximately 6–12 months. Rehabilitation assessment (medical and non-medical) should be considered, especially in those with neurological or neuropsychological deficits at discharge.

 

In Summary:

Encephalitis presents a complex challenge, it requires the performance of a detailed clinical assessment, consultation, and judicious investigation. Unnecessary delays must be avoided, and it is essential to institute empiric therapies appropriately and provide high-quality supportive management. Optimal application of current knowledge is likely to improve diagnosis; however, even with an extensive diagnostic work-up, definitive aetiology may not be identified for 30%–40% of patients with encephalitis.

 

References

Britton PN, Eastwood K, Brew B, Nagree Y, and Jones CA. Consensus guidelines for the investigation and management of encephalitis. Med J Aust 2015; 202 (11): 576-577. || doi: 10.5694/mja14.01042

Kawasaki’s Disease

Cite this article as:
Alyssa Courtney. Kawasaki’s Disease, Don't Forget the Bubbles, 2017. Available at:
https://doi.org/10.31440/DFTB.12282
A four-year-old Japanese boy was brought into Emergency with 5 days of fevers, non-exudative bilaterally injected sclerae, erythematous pharynx and irritability.

Wondering about the possibility of Kawasaki Disease, I turned to check the 2017 update of the American Heart Association Scientific Statement, focusing on considering a diagnosis of Incomplete Kawasaki Disease.

 

Bottom line:

  • Unchanged diagnostic criteria of complete Kawasaki Disease (KD)
  • Refined algorithm for evaluation of suspected incomplete KD (15-20% of cases)
  • Recommended ECHO at diagnosis, and repeated at 1-2 weeks and 4-6 weeks after treatment
  • Unchanged acute management– Intravenous immunoglobulin (IVIG) single dose 2g/kg over 10-12 hours. Ideally prior to day 10. Some countries continue to use high dose aspirin for varying durations.
  • Additional therapeutic options are outlined for the 10-20% with persistent or recurrent fever
  • New model of KD vasculopathy

 

What is Kawasaki Disease?

An acute, self-limited febrile illness of unknown cause, predominantly in children <5 years. It is the most common cause of acquired heart disease in developed countries. Without pathognomonic tests, we need to detect it clinically!

 

Epidemiology

  • Most common in Japan (where it was first described) with an annual incidence of 264.8 per 100 000 children in 2012. The estimated incidence in North America is 25 cases per 100 000 children <5 years of age per year. Australia has one of the lowest reported rates (3.7 per 100 000 <5 years of age), equivalent to 50–60 cases Australia-wide per year. It is likely that the current Australian incidence is higher.
  • Highest relative risk is in Asian children, especially of Japanese ancestry
  • The ratio of males to females is 1.5:1
  • Predominantly affects children 6 months to 4 years
  • Predisposing factors have been reported inconsistently
  • In Japan, the recurrence rate is 3%, and the relative risk in siblings is ten-fold higher

 

What is the aetiology?

We have no idea why…. BUT the resultant systemic inflammation leads to associated clinical findings: liver (hepatitis), lung (interstitial pneumonitis), gastrointestinal tract (abdominal pain, vomiting, diarrhoea, gallbladder hydrops), meninges (aseptic meningitis, irritability), heart (myocarditis, pericarditis, valvulitis), urinary tract (pyuria), pancreas (pancreatitis), and lymph nodes (lymphadenopathy).

A new model of Kawasaki disease vasculopathy involves three processes impacting muscular arteries. The first is a necrotising arteritis, followed by subacute/chronic vasculitis. The final process is luminal myofibroblastic proliferation.

 

How do we diagnose it in Australia?

Diagnostic Criteria

Fever for 5 days or more (typically high spiking (>39°C to 40°C) and remittent)

Plus 4/5 of:

  • polymorphous rash (usually within 5 days of fever onset)
  • bilateral (non-purulent) conjunctival injection (usually begins shortly after fever onset and often spares the limbus, an avascular zone around the iris)
  • mucous membrane changes e.g. reddened or dry cracked lips, strawberry tongue, diffuse redness of oral or pharyngeal mucosa (oral ulcers and pharyngeal exudates are not consistent with KD)
  • peripheral changes, e.g. erythema of the palms or soles, oedema of the hands or feet, and in convalescence desquamation
  • cervical lymphadenopathy (> 15 mm diameter, usually unilateral, single, non-purulent and painful in the anterior cervical chain)

AND exclusion of diseases with a similar presentation

  • Staphylococcal infection (e.g. scalded skin syndrome, toxic shock syndrome)
  • Streptococcal infection (e.g. scarlet fever, toxic shock-like syndrome not just isolation from throat)
  • Measles
  • Viral exanthems
  • Steven’s Johnson syndrome
  • Drug reactions
  • Juvenile rheumatoid arthritis.

Trickily, these children may have a concurrent viral infection, often adenovirus. Adenovirus is more likely with exudative pharyngitis and conjunctivitis and positive PCR assay. Kawasaki disease is more likely with erythema/swelling of hands and feet, a strawberry tongue, and a desquamating groin rash.

Inflammation and crusting of a recent Bacille-Calmette-Guérin (BCG) injection site may occur.

Consider an alternative diagnosis to Kawasaki Disease if there is exudative conjunctivitis, exudative pharyngitis, ulcerative intraoral lesions, bullous or vesicular rash, generalized adenopathy, or splenomegaly.

 

What is Incomplete Kawasaki Disease?

Scarily, this is so easily missed. They make up 15-20% of all cases!!
Patients with incomplete KD, particularly those <6 months of age and older children, may experience significant delays in diagnosis and these children are at high risk of developing coronary artery abnormalities.

Consider KD if:

  • Infants <6 months old with prolonged fever and irritability
  • Infants with prolonged fever and unexplained aseptic meningitis
  • Infants or children with prolonged fever and unexplained or culture-negative shock
  • Infants or children with prolonged fever and cervical lymphadenitis unresponsive to antibiotic therapy
  • Infants or children with prolonged fever and retropharyngeal oroparapharyngeal phlegmon unresponsive to antibiotic therapy

Evaluation of suspected incomplete Kawasaki disease (via McCrindle BW et al. 2017)

What can we investigate?

As suggested by RCH Melbourne guidelines, all patients should have

  • ASOT / Anti DNAase B
  • Echocardiography (at least twice: at initial presentation and, if negative, again at 6 – 8 weeks).
  • Platelet count (marked thrombocytosis common in the second week of illness)
  • Consider Mycoplasma

In addition, findings can provide support when considering Incomplete Kawasaki Disease – refer to the above algorithm.

Evolution of laboratory findings via Tremoulet et al.

  • KD is unlikely if ESR, CRP, and platelet count are normal after day 7 of illness.
  • Low WBC and lymphocyte predominance suggests an alternative diagnosis
  • Leukocytosis is typical in the acute stage, with granulocyte predominance
  • Normocytic, normochromic anaemia is common during inflammation
  • CRP and ESR elevation is nearly universal, CRP normalizes more quickly with inflammation resolution. ESR is elevated by IVIG therapy.
  • Minimally elevated ESR in the setting of severe clinical disease should prompt investigation for disseminated intravascular coagulation.
  • Thrombocytosis is a characteristic feature that generally doesn’t occur until the second week, peaking in the third week, normalizing by 4 to 6 weeks
  • Thrombocytopenia can be a sign of disseminated intravascular coagulation and is a risk factor for the development of coronary artery abnormalities
  • Mild to moderate elevations in serum transaminases or gammaglutamyl transpeptidase occur in 40% to 60% of patients, and mild hyperbilirubinemia occurs in ≈10%.
  • Hypoalbuminaemia is common and associated with more severe and more prolonged acute disease
  • Urinalysis may show pyuria in up to 80% of children, non-specific for KD
  • In children who undergo lumbar puncture, ≈30% demonstrate pleocytosis with a mononuclear cell predominance, normal glucose levels, and generally normal protein levels

 

Pitfalls

Fever and pyuria in an infant or young child may be diagnosed as a urinary tract infection, with subsequent development of rash, red eyes, and red lips attributed to an antibiotic reaction. Irritability and a culture-negative pleocytosis of the cerebrospinal fluid in an infant with prolonged fever suggestive of aseptic meningitis (or if antibiotics have been given, partially treated meningitis) may cause a diagnosis of KD to be overlooked. Cervical lymphadenitis as the primary clinical manifestation can be misdiagnosed as having bacterial adenitis. Gastrointestinal symptoms are considered for surgical causes, other physical findings of KD can be overlooked.

 

What is the treatment?

We’re aiming to prevent important coronary artery abnormalities. Timely (as soon as possible, ideally within 10 days) IVIG treatment reduces the incidence of coronary artery aneurysms (defined from absolute luminal dimensions) from 25% to 4%. Studies with additional therapies to IVIG have not substantially reduced this residual risk of 4%. Adverse effects are rare but include Coomb’s positive haemolytic anaemia and aseptic meningitis. The measles, mumps, and varicella vaccine should be deferred for 11 months unless at high risk (seek advice, may need repeat vaccination). If the diagnosis is delayed, IVIG should still be given (after the tenth day of illness) IF there is presence of fever, or continued elevation of ESR or CRP>3, indicating ongoing inflammation. Aspirin is used with the theoretical rationale of reducing coronary artery aneurysms (although there is no well-established evidence for this). In Australia, a dose of 3-5mg/kg daily from diagnosis until cardiology review at 6 weeks is routine. The newly released statement advises the administration of moderate to high-dose (80–100 mg/kg/day) aspirin is reasonable until the patient is afebrile. Patients should receive a seasonal influenza vaccination.

Fever usually resolves within 36 hours after IVIG infusion has been completed; if not, the patient is considered to have resistance to IVIG. 10-20% of patients will not respond to the single IVIG treatment dose. There is minimal data to support therapeutic agents for the child with IVIG resistance. Repeating the IVIG dose, 3 days of high-dose pulsed steroids, or 2-3 weeks of tapering prednisolone are all options. There are lower levels of evidence for infliximab and cyclosporine.

 

Coronary artery abnormalities

An angiographic study of 1100 patients showed coronary artery lesions in 24%, with aneurysms in 8% and a number of patients with stenoses and occlusions. Valvular regurgitation is usually mild to moderate in severity and resolves prior to follow-up. MR can occur after the acute stage from myocardial ischaemia. Patients after KD have been shown to have functional and anatomic abnormalities of the aorta with unknown long-term implications. Myocarditis is common during the acute illness but complete resolution is expected. Risk stratification for long-term management is based primarily on maximal coronary artery luminal dimensions, normalized as Z scores, and is calibrated to both past and current involvement. Patients with aneurysms require life-long and uninterrupted cardiology follow-up.

 

What is the prognosis?

  1. The case fatality rate is <0.1% in Japan, virtually all from cardiac sequelae.
  2. Peak mortality occurs 15 to 45 days after onset of fever, during which time well-established coronary artery vasculitis occurs concomitantly with marked elevation of the platelet count and a hypercoagulable state
  3. Coronary artery aneurysms from KD account for 5% of acute coronary syndromes (ACS) in adults <40 years of age

 

In Summary:

  • Be aware of the diagnostic criteria of complete Kawasaki Disease (KD)
  • Highest relative risk in Asian children, especially Japanese ancestry
  • Always consider incomplete KD (15-20% of cases) and refer to the algorithm if concerns, there are pitfalls!
  • Liaise with cardiology regarding an ECHO
  • Unchanged acute management– Intravenous immunoglobulin (IVIG) single dose 2g/kg over 10-12 hours. Ideally prior to day 10. Some countries continue to use high dose aspirin for varying durations.
  • Additional therapeutic options are outlined for the 10-20% with persistent or recurrent fever, minimal evidence for these
  • New model of KD vasculopathy but we are still in the dark regarding aetiology
  • Coronary artery aneurysms from KD account for approximately 5% of acute coronary syndromes (ACS) in adults <40 years of age

 

 Selected references

McCrindle BW, Rowley AH, Newburger JW, et al. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. Circulation2017;Mar 29

Yim D, Curtis N, Cheung M, Burgner D. Update on Kawasaki disease: Epidemiology, aetiology and pathogenesis. Journal of Paediatrics and Child Health 49 (2013) 704–708

Clinical Practice Guidelines, Royal Children’s Hospital, Melbourne

Kim DS, Kawasaki Disease. Yonsei Medical Journal. 47 (2006) (6): 759–72. PMC 2687814 PMID 17191303. doi:10.3349/ymj.2006.47.6.759

Yim D, Curtis N, Cheung M, Burgner D. An update on Kawasaki disease II: Clinical features, diagnosis, treatment and outcomes. Journal of Paediatrics and Child Health 49 (2013) 614–623

Tremoulet AH, Jain S, Chandrasekar D, Sun X, Sato Y, Burns JC. Evolution of laboratory values in patients with Kawasaki disease. Pediatr Infect Dis J. 2011;30:1022–1026

Dengler LD, Capparelli EV, Bastian JF, Bradley DJ, Glode MP, Santa S, Newburger JW, Baker AL, Matsubara T, Burns JC. Cerebrospinal fluid profile in patients with acute Kawasaki disease. Pediatr Infect Dis J. 1998;17:478–481

 

Quadrivalent Meningococcal Vaccination: W(135)hat’s all the fuss about?

Cite this article as:
Phoebe Williams. Quadrivalent Meningococcal Vaccination: W(135)hat’s all the fuss about?, Don't Forget the Bubbles, 2017. Available at:
https://doi.org/10.31440/DFTB.12309

I’ll never forget the throbbing, pounding headache that heralded the onset of meningococcal meningitis and septicaemia as a 14 year old girl; the complete overwhelm of the horrifying physical sensation that is sepsis. Within two hours I was unconscious – but thanks to a vocal family member advocating their concern for how sick I was, a NETS retrieval to a tertiary unit was swiftly arranged where I was diagnosed with serotype C strain meningococcal disease. I was incredibly fortunate to receive excellent care and survive unscathed.

A gentle nudge…

Cite this article as:
Tessa Davis. A gentle nudge…, Don't Forget the Bubbles, 2017. Available at:
https://doi.org/10.31440/DFTB.11516

Children have a right to receive healthcare regardless of the decisions made by their parents. This week, the RACP released a further statement citing refusal to treat patients for non-vaccination as ‘unethical coercion’. Whilst the majority of paediatricians are clear on the benefits of vaccinations, parents can choose not to vaccinate their children. Under the No Jab No Pay policy, this choice can lose them their right to Child Care Rebate. However, it should not lose them their right to receive healthcare. A poll by the Royal Children’s Hospital in Melbourne found that 17% of children who were not fully vaccinated had been refused healthcare by a provider. But what does ‘refused’ actually mean? And does the RACP’s statement leave us confused rather than providing clarity?

 

What was the poll?

The Australian Child Health Poll team at RCH is led by Dr Andrea Rhodes. It runs a quarterly poll on contemporary child health issues by conducting national surveys across Australia. The latest poll, released earlier this week, looked at vaccination perspectives of Australia families.

Data was collected from 1945 parents (with 3492 children). They were asked questions relating to the vaccination status of their children and attitudes to vaccinations.

 

What were the key findings on refusal of care?

  • 95% of the children were fully up to date with their vaccinations
  • 30% of parents had some concerns about vaccination
  • 9% of parents did not agree that “…it was important to vaccinate their child to protect the community (herd immunity)”
  • Of those not up-to-date, 17% had been refused healthcare (29 children)

 

Why are children not up-to-date with their vaccinations?

24% had delayed some vaccinations due to to minor illnesses, and there were misconceptions about when to delay.  36% thought that vaccines should be delayed in child with a runny nose but no temperature, and 47% thought they should be delayed if the child was on antibiotics. 22% thought vaccines should be postponed due to a local reaction from a previous vaccination.

18% had a preference against vaccinations, and perhaps worryingly 10% thought that vaccinations were linked to autism and a further 30% felt unsure about the link.

22% had delayed because of barriers to education and access. Half of these struggled to attend for vaccination and the other half had questions about vaccinations that they were unable to get answered. This emphasises the importance of our role. This sizeable group needs our input as paediatricians – to offer information, be open to discussion and assist with making vaccinations accessible.

 

What can we take from this?

This is the first poll in Australia providing information about refusal to provide healthcare.

Our role as paediatricians is to provide healthcare for each child regardless of their circumstances and certainly regardless of any actions of their parents. This poll makes it clear that there is a sizeable group of parents with uncertainty about vaccination who are interested in engaging and accessing services. We would be doing them a disservice by refusing to treat their children. And even where parents have made a specific decision not to vaccinate, we still have a duty to provide healthcare for their children. It is only by keeping the channels open that we can support, educate, and engage.

The RACP has released a clear statement on this which reinforces their Immunisation Position statement.

It is inappropriate to refuse to treat unvaccinated children, firstly because it represents unethical coercion and secondly because the children will be further disadvantaged.

Yes, we know that vaccinations are beneficial. And yes, we want to increase vaccination rates and ensure our patients are vaccinated. However, by denying unvaccinated children healthcare we are disadvantaging them even further. The RACP goes as far as to call this ‘unethical coercion’. We need to provide healthcare and not use it as bargaining chip.

However, the concept of refusal may not be clear cut. This poll did not collect data on the circumstances of refusal so we do not know which provider has refused treatment or how that refusal has occurred. It is possible that parents perceive that healthcare professionals are not happy to treat them and classify this as outright refusal.

Research in the US has shown that providers worry that unvaccinated children will pose a risk to young babies in their waiting rooms, or that the unvaccinated child themselves is at risk of catching a serious infection. These are reasonable concerns and therefore steps may need to be taken to minimise these risks. If unvaccinated children need to sit separately, could this constitute refusal in the eyes of parents? If the parents have to engage in an awkward 10 minutes of discussion with the paediatrician about the benefits of vaccination, could this constitute refusal in the eyes of the parents?

So the answer is not as clear cut as the RACP’s statement makes it seem. Of course we should not refuse to treat a child because their parents didn’t vaccinate them. But it is also our role to encourage vaccinations and to engage in discussion about the benefits of vaccination. This engagement may make vaccine-refusers feel uncomfortable and affect their perception of our willingness to provide healthcare. This is not the same as refusal to treat.

 

CVADs – a survival guide

Cite this article as:
Amanda Ullman. CVADs – a survival guide, Don't Forget the Bubbles, 2016. Available at:
https://doi.org/10.31440/DFTB.10530

This guest post is from Amanda Ullman, Tricia Kleidon, Anna Dean and the Paediatric Vascular Assessment and Management Service, Lady Cilento Children’s Hospital, Brisbane.

We all love reliability. Central venous access devices (CVADs) are everywhere – across disciplines and departments, and we just want them to work reliably, without complication.

But every day, somewhere in your hospital, we are problem solving CVADs gone awry. And in many hospitals no specialist vascular access teams are there to help you. 

Being a CVAD survivalist will help you – most likely at 3am. Some situations will need escalating to specialists, but many can be quickly and effectively managed with simple, evidence-based techniques.

 

Needling a totally implanted device (ports)

Some totally implanted devices – or ports – are a pain. This is especially a problem in children with large amounts of adipose tissue, or poorly positioned ports.

But when problem solving this – everyone is different. Ask the child to sit up in the bed (or on their parent), and take off any restrictive clothing (e.g. bras for teenage girls). Use a rolled up towel under their back/shoulder blade and ask the patient to push their chest out, allowing less tissue to cover the port – making it become more prominent.  Make sure you have a firm grip of the port and stretch the skin out over it to reduce the amount of tissue you have to go through – this cannot be overemphasised.  There should be no slack in the skin.  You may need to change your grip a couple of times to get this just right. Also consider your own position when inserting, often placing yourself at the head of the bed is helpful.

 

Post CVAD insertion bleeding

Bleeding after the insertion of CVADs can require frequent dressing changes – not the easiest task on a post-anaesthetic child or toddler.

The application of small drops of medical-grade superglue, or tissue adhesive (cyanoacrylate), directly on the CVAD insertion site results in immediate haemostasis, without resulting in significant skin injury [1, 2]. Most practical on peripherally inserted central catheters (PICCs) and non-tunnelled CVADs, make sure the site is completely dry before applying the glue, try not to stick yourself to the child or the CVAD, and allow the glue to dry completely before covering it with the usual CVAD dressing.

 

Lumens that won’t aspirate or flush

Many factors can result in blocked CVAD lumens. A step-by-step approach is necessary…

  1. Don’t over complicate matters! Check for external occlusion due to a kinked line etc.
  2. Change the needleless access device (i.e. the bung) – these frequently dysfunction and result in blockage.
  3. Try small, pulsatile aspirates, and then flushes of normal saline using a 10mL syringe. Never use a syringe smaller than 10mL or be tempted to use excessive force – this can result in catheter breakage. Ask the patient to cough, breathe deeply, or change their position while aspirating and flushing.
  4. Assess for correct placement (chest x-ray, angiography).
  5. Instil a thrombolytic agent, such as Urokinase or Alteplase (unless contraindicated), allow it to dwell for at least 60 minutes, and then aspirate and/or flush with normal saline. This may need to be repeated several times.
  6. In the case of total occlusion where you can’t administer a thrombolytic agent by a simple push – use a 3-way tap and additional empty 10mL syringe.  Turn the 3-way tap off to the thrombolytic agent, pull back on the empty 10mL syringe to create a vacuum.  Turn the tap so it is open to the drug and patient, and the drug should infuse under negative pressure.
  7. If the blockage is a result of drug precipitation or build of lipid products; talk to the pharmacist for an appropriate dissolving agent such as hydrochloric acid or sodium bicarbonate.
  8. Phone a friend, preferably someone who can insert a replacement device.

 

Local CVAD site irritation vs local infection

Identification is of the issue is key.

If there are any signs of inflammation or infection (e.g., raised red area, ooze ) take a swab for microscopy, culture and sensitivity (MC&S).

Early libe sire infection

For uncomplicated exit site infections (i.e., no signs of systemic infection, purulent drainage or positive blood cultures) in long-term CVADs, the Infectious Diseases Society of America recommends the use of topical antimicrobial agents specific to the MC&S results, (i.e. mupirocin ointment for S. aureus infection and ketoconazole or lotrimin ointment for Candida infection). If it is a short-term CVAD (e.g. PICC) take it out and organise a replacement device.

If infection doesn’t seem likely, identify what is causing the skin irritation.

Contact dermatitis

Most frequently it will be the decontaminant (2% CHG in alcohol) that has not been left to dry adequately.  Some patients have a reaction to the fibres in the decontaminant stick. If this is a possibility use bottled (2% CHG in alcohol) with gauze and forceps.  Additionally, consider the use of a barrier film product, provide symptom relief (i.e. itch, pain), and if symptoms persist recommend changing to a less-irritating silicone-based dressing product.

 

Broken lumens

Oh God, it snapped. This happens – a lot it seems.

Broken lumens don’t always result in the end of the entire CVAD, but it does depend on the type of CVAD. Silicone CVADs, such as HickmansTM, have catheter repair kits that sit with skilled clinicians, such as paediatric oncology and haematology. Peripherally inserted central catheters vary by brand and catheter material in their ability to be repaired. This is definitely a phone a friend situation. When repairing a broken catheter, always consider the circumstances in which it fractured and environmental exposure. Blood cultures and prophylactic antibiotics might reduce the risk of subsequent infection.

References

1. Rickard, C.M., et al., A 4-arm randomized controlled pilot trial of innovative solutions for jugular central venous access device securement in 221 cardiac surgical patients. Journal of Critical Care, 2016. 36: p. 35-42.

2. Ullman, A.J., et al., Central venous Access device SeCurement And Dressing Effectiveness (CASCADE) in paediatrics: protocol for pilot randomised controlled trials. BMJ Open, 2016. 6(6).

3. Simcock, L., Managing occlusion in central venous catheters. Nurs Times, 2001. 97(21): p. 36-8.

4. Mermel, et al., Clinical practice guidelines for the diagnosis and management of intravascular catheter-related infection: 2009 update by the Infectious Diseases Society of America. Clin Infect Dis, 2009. 49: p. 1-45.

Big Picture Paediatrics : Adverse Childhood Experiences

Cite this article as:
Henry Goldstein. Big Picture Paediatrics : Adverse Childhood Experiences, Don't Forget the Bubbles, 2016. Available at:
https://doi.org/10.31440/DFTB.10082

So much of paediatrics, and medicine in general, is focussed on small experimental or observational studies. This series of posts takes the wider view; we’re talking here about some of the biggest and longest running studies that help us frame, measure and understand childhood through time and across the world.

Who & what was studied?

Kaiser Permanente is a large Medical Insurer in the USA; they collected data in two waves in the primary care setting with a view to describing the long-term relationship of childhood experiences to important medical and public health problems. The study initially rolled out in 1996 & 1997.

Felitti, VJ, Anda RF, Nordenberg D et al. Relationship of Childhood Abuse and Household Dysfunction to Many of the Leading Causes of Death in Adults : The Adverse Childhood Experiences (ACE) Study. American Journal of Preventive Medicine. 1998:14, 245–258.

The study aimed to assess – both retrospectively and prospectively – the long-term impact of abuse and household dysfunction during childhood on disease risk factors and incidence, quality of life, health care utilization, and mortality for adults.

Here is the actual questionnaire:

Answer yes or no; all ACE questions refer to the respondent’s first 18 years of life.

Abuse

  • Emotional abuse: A parent, stepparent, or adult living in your home swore at you, insulted you, put you down, or acted in a way that made you afraid that you might be physically hurt.
  • Physical abuse: A parent, stepparent, or adult living in your home pushed, grabbed, slapped, threw something at you, or hit you so hard that you had marks or were injured.
  • Sexual abuse: An adult, relative, family friend, or stranger who was at least 5 years older than you ever touched or fondled your body in a sexual way, made you touch his/her body in a sexual way, attempted to have any type of sexual intercourse with you.

Household Challenges

  • Mother treated violently: Your mother or stepmother was pushed, grabbed, slapped, had something thrown at her, kicked, bitten, hit with a fist, hit with something hard, repeatedly hit for over at least a few minutes, or ever threatened or hurt by a knife or gun by your father (or stepfather) or mother’s boyfriend.
  • Household substance abuse: A household member was a problem drinker or alcoholic or a household member used street drugs.
  • Mental illness in household: A household member was depressed or mentally ill or a household member attempted suicide.
  • Parental separation or divorce: Your parents were ever separated or divorced.
  • Criminal household member: A household member went to prison.

Neglect

  • Emotional neglect: Someone in your family helped you feel important or special, you felt loved, people in your family looked out for each other and felt close to each other, and your family was a source of strength and support.
  • Physical neglect: There was someone to take care of you, protect you, and take you to the doctor if you needed it, you didn’t have enough to eat, your parents were too drunk or too high to take care of you, and you had to wear dirty clothes.

What does this mean?

The ACEs questionnaire accumulates a score from zero to seven based on yes/no responses to the above questions. These results in conjunction with a “Health Appraisal Clinic’s questionnaire” allowed correlation with risk factors such as smoking, severe obesity, physical inactivity, depressed mood, suicide attempts, alcoholism, any drug abuse, sexually transmitted diseases, parental drug abuse and a high lifetime number of sexual partners (>50), as well as the big swingers; mortality and overall morbidity.

The ACE score has been utilised to demonstrate a graded dose-response with more than 40 outcomes. You can see the entire list of publications here.

How good is this dataset?

Although there are almost all of the expected threats to validity from a questionnaire administered to people obtaining health insurance in the USA in the 1990s, the dataset is very good.

Of the 13,494 surveys, there was a 70.5% (9508) response rate, sent a week after standardised medical review. Respondents who did not respond to all questions were excluded from the final analysis. After non-responders and exclusions, a total dataset of 8056 responders was analysed. Alarmingly, more than half of the exclusions were for not answering the question about childhood sexual abuse. This certainly raises some concern for a risk of underreporting, particularly if this was the only question omitted! 

What meaning can be drawn from the results (so far)?

The dataset has lent itself to the associations between adverse childhood experiences and a veritable laundry list of medical, psychiatric pathology as well as social and public health problems.

This is data reports that 1 in 5 were sexually abused, nearly 1 in 4 lived with a “problem drinker or alcoholic” and that around 1 in 6 had a household member who was depressed or mentally ill.

It’s worth remembering that this study paints a picture of the adverse childhood experiences of the older generations in the USA – the mean age of respondents was 56.1 (19-92) years – in a study undertaken just over 20 years ago.

Rather than provide a snapshot of what childhood is like today, this data informs us about the childhood of parents of our patients. This gives us some understanding and frameworks by which to consider expectations of childhood from the parental & societal viewpoint – that most parents hope for a rosier childhood with fewer adverse experiences than their own.

With this in mind, and with a critical eye to some of the correlating outcomes, behaviours such as alcohol & drug abuse, smoking, over-eating, and sexual behaviours might alternatively be viewed as both coping strategies and symptoms of the anxiety, anger and depression that is likely co-morbid with high levels of adverse childhood experiences.

Primary prevention of adverse childhood experiences necessitates change at the societal level; with a focus on improving the quality of family and household environments through the childhood years.

Funding for the original study was combined between Kaiser Permanente (San Diego) and the US Center for Disease Control.

Where next?

The Centre for Disease in Childhood has taken over the study and, since 2009, transformed it into a national program across 32 states of the USA, called “Behavioral Risk Factor Surveillance System” (BRFSS). Data from the 2010 BRFSS has been published and includes more than 50,000 respondents. You can see more about the participating states, future timeline and previous data via the CDC website, here.

References:
Felitti, VJ, Anda RF, Nordenberg D et al. Relationship of Childhood Abuse and Household Dysfunction to Many of the Leading Causes of Death in Adults : The Adverse Childhood Experiences (ACE) Study. American Journal of Preventive Medicine. 1998:14, 245–258. 

Centers for Disease Control and Prevention, National Center for Injury Prevention and Control, Division of Violence Prevention Adverse Childhood Experiences (ACEs)”.U.S. Department of Health & Human Services, Atlanta, USA. Accessed 27 September 2016. https://www.cdc.gov/violenceprevention/acestudy

Centers for Disease Control and Prevention, National Center for Injury Prevention and Control, Division of Violence Prevention. “About Behavioral Risk Factor Surveillance System ACE Data”.U.S. Department of Health & Human Services, Atlanta, USA. Accessed 5 October 2016. https://www.cdc.gov/violenceprevention/acestudy/ace_brfss.html

The not-so Secret Life of Pets

Cite this article as:
Andrew Tagg. The not-so Secret Life of Pets, Don't Forget the Bubbles, 2016. Available at:
https://doi.org/10.31440/DFTB.10118

Everyone knows that Australia is one of the most dangerous countries in the world. It houses deadly spiders, snakes and sea life as well as the deadly drop-bear*. But more children are injured by pets every year than any of the native species.