All paediatricians are complicit in delivering a racist healthcare service

Cite this article as:
Zeshan Qureshi and Anna Rose. All paediatricians are complicit in delivering a racist healthcare service, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.33938

We’re supposed to be the nice ones. The friendly, fun, caring and supportive speciality – right? We’re the ones who have teddies on our stethoscopes and know all the words to Disney songs. In the hospital, we’d like to think we’re the Good Guys – but maybe we’re not when it comes to race. 

The COVID-19 pandemic has been a monumental challenge to the NHS and has, undoubtedly, showcased the everyday heroism of our staff. It has also thrown a sharp light onto the ongoing racial inequalities in our society and healthcare systems. Racial disparities in the pandemic have been widely documented – and make for sobering reading. Analysis of national hospital data suggests that people of black and other minority backgrounds are up to twice as likely to die from the virus, as compared to white Britons – with some groups, such as black African-born men living in Britain, having an even higher risk [1]. Despite this, we have little doubt that the major impetus for the unprecedented emergency measures, national lockdowns, and political obsession was not the deaths of the poor, the ethnic minorities, or those in low and middle-income countries – but the perceived threat to wealthy, predominantly white, Westerners.

In an eerie parallel to the racial inequalities highlighted by the pandemic, the past year has also seen racial tensions in the USA reach boiling point. Following several high-profile incidents of police brutality, there was an eruption of social unrest and protest in America and around the world. The systemic disregard of black lives is not just written in blood on American pavements. It is written into the systems that surround us in our everyday working lives. As a speciality, and in the NHS as a whole, we must confront these engrained systemic inequalities, if we are to provide truly equitable care to all of our patients. 

In this blog series, we will examine how clinical outcomes for common paediatric conditions are worse for children from minority ethnic backgrounds. Stillbirth, low birth weight and preterm birth are all more common in minority groups as compared to white northern European populations [2,3,4 ]. Outcomes for common chronic conditions, such as asthma and type 2 diabetes, are also worse for children within minority groups [5,6]. This could be because care received by children with chronic conditions is worse. Non-white children with renal failure are less likely to pre-emptively receive a renal transplant, for example [7]. There are also complex social and environmental roots to these adverse health outcomes – such as increased poverty in non-White groups [8] — and we will try to investigate these issues in more detail. 

We will also explore how paediatrics has normalized white Northern European genetics, physiology and behaviour, leading to biased clinical decision making. Normalization of one ethnic group has lead to the classification of other normal values (in both the laboratory and social sense) as pathological or inappropriate. In other words – you are only normal if you are white and normal. Neutrophil counts are often lower in black babies [9]. Rather than reporting ethnically normal ranges babies often end up having multiple blood tests due to a lack of awareness of the variation. Parents get told that their neutrophil count is low, but it’s acceptable for a black baby (rather than categorically stating that their count is normal). Worst still, it might be classified as a disease – benign ethnic neutropenia – despite not being associated with increased morbidity or mortality.

Within medical education, we are guilty of peddling irrelevant and outdated racial and religious stereotypes. These hold little educational value, but risk enforcing dangerous bias within our future doctors. Any paediatrician would be able to tell you about the association between Tay-Sachs disease and Ashkenazi heritage, or sickle cell disease and sub-Saharan Black Africans. Such associations are often over-simplified and over-emphasized, to the point of creating a disease-ridden caricature, particularly in exam questions. Most of these stereotyped conditions are very rare, and over-emphasis during medical school risks blinkering us to more common diagnoses. We’ll explore how racial bias is ingrained in medical education in the UK, and try to come up with some ideas on how we can improve MedEd to be more diverse and inclusive in the future. 

There also seems to be a disproportionate concern that those from Muslim backgrounds might be consanguineous, and that we need to ask about this even when it is not relevant to the presenting complaint. Conversely, when genetic testing is being sent off, a detailed family tree needs to be drawn. It should include details of any consanguinity – yet it seems that a white family is less likely to be asked. As first or second cousin marriages are no longer a social norm in the UK, they have become defined by pathological associations with genetic conditions, such as inborn errors of metabolism. And whilst there are, of course, differences in the prevalence of disease alleles in different populations, and an increased risk of recessive disorders in families with intergenerational consanguinity, it does not automatically follow that a child from a Muslim background has a recessive disorder, or that a white British child does not. The same considerations need to be given to other cultural practices that might be different to the social norms of Northern and Western Europe. Putting children on a vegetarian diet is often classed as a ‘restrictive diet’ – despite the fact that it is only restrictive based on traditional Western standards – and might, in fact, hold health benefits [10]. 

Finally, in our series, we will examine how systemic racism within the health service tolerates – and sometimes even facilitates –  the unacceptable behaviours demonstrated by some parents. One thing that sets paediatrics apart from adult medicine is that patients are almost never seen alone, and a parent is often required to deliver care. This can present a dilemma to staff when confronted with a racist parent. Any punishment directed towards the parents might directly harm their child. We will explore how guidelines should be developed to help clinicians handle racist parents, whilst minimizing the effect on the clinical care of our patients. 

It can be painful for us – as individuals and as a speciality – to consider that we might be complicit in a racist system that ultimately leads to poorer health outcomes for some children. Just because something is painful, does not mean we shouldn’t do it. We hope that you’ll join us for this series of short articles, as we try to explore how we can begin to move from a white-centric healthcare system to a child-centred one.

James Baldwin quote on racism

Selected references

1) The IFS Deaton Review. Are some ethnic groups more vulnerable to COVID-19 than others? 

2) Gardosi J, et al. (2013). Maternal and fetal risk factors for stillbirth: population-based study. BMJ 346:f108.

3) Kelly Y, et al. (2008). Why does birthweight vary among ethnic groups in the UK? Findings from the Millenium Cohort Study. Journal of Public Health, 31:131–137.

4) Aveyard P, et al (2002). The risk of preterm delivery in women of different ethnic groups. British Journal of Obstetrics and Gynaecology 109:894-899.

5) Asthma UK (2018) On the Edge: How inequality affects people with asthma. Available at www.asthma.org.uk

6) RCPCH (2020) State of Child Health: Diabetes. Available at www.rcpch.ac.uk

7) Plumb LA et al. (2021) Associations between Deprivation, Geographic Location, and Access to Pediatric Kidney Care in the United Kingdom. CJASN. 16:194-203.

8) Office for National Statistics (2020) Child poverty and education outcomes by ethnicity. Available at www.ons.gov.uk.

9) Haddy TB, Rana SR, Castro O. (1999) Benign ethnic neutropenia: what is a normal absolute neutrophil count? J Lab Clin Med. 133:15-22.

10) Kalhoff H. et al (2021) Vegetarian Diets in Children—Some Thoughts on Restricted Diets and Allergy. International Journal of Clinical Medicine. 12:43-60.

Scurvy header image

Scurvy

Cite this article as:
Julia Hall and Abigail Lazenbury. Scurvy, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.33473

A three-year-old boy with speech and language delay presents to the Emergency Department with a unilateral atraumatic limp. He is initially managed as per the ‘Limping Child Pathway’. At his follow up review, he is now non-ambulatory, resistant to examination and holds his legs in a flexed position.

He has an extensive array of normal investigations including basic blood tests, pelvis and bilateral leg x-rays and abdominal ultrasound. He subsequently has an MRI which shows florid symmetrical increased signalling in his long bones with a wide non-specific differential. He develops a small patch of petechiae on his ear.
Reviewing his history, it appears that his diet is extremely restrictive and made up of only lactose-free milk and cheesy biscuits.

Could this be scurvy?

Definition

Scurvy, or vitamin C deficiency, is a disease of malnutrition. It is one of the oldest nutritional deficiencies identified, and the source of one the world’s first randomized control trials. In 1753 James Lind published A Treatise of the Scurvy. Like a lot of research, this paper was widely ignored. It wasn’t until about 10 years later, when sailors got to try sauerkraut, wort, or syrup of oranges and lemons (all contain vitamin C) did the higher ups at the Admiralty begin to take notice.

Rime of the Ancient Mariner - all the sailors died of scurvy
Gustave Doré’s illustration from Coleridge’s Rime of the Ancient Mariner

Scurvy is rare in higher income countries and therefore often forgotten as a potential differential diagnosis.

Pathophysiology

Vitamin C, also known as ascorbic acid, is a water-soluble essential vitamin. It is found in citrus fruits, peppers, potatoes and broccoli. In babies, vitamin C is provided in the breast milk. Vitamin C has important roles in the body including wound healing, bone, cartilage and blood vessel maintenance and helps with the absorption of iron. It is also involved in fatty acid transport, neurotransmitter synthesis, prostaglandin metabolism and nitric oxide synthesis. 

Dietary doses of up to 100mg/day can be absorbed in the distal small intestine. It is renally excreted and therefore the kidneys are important in regulating vitamin C concentration in the blood. The World Health Organisation recommended daily allowance for infants and children is 25mg per day.

The clinical manifestations of scurvy result from disordered pathways that utilise vitamin C, such collagen and connective tissue synthesis. Symptoms can occur after just one to three months of inadequate vitamin C intake. 

Signs and symptoms 

Early manifestations of scurvy are often non-specific. Consider the infant with fatigue, anorexia, weight loss and low grade fever – these are all early signs of scurvy but scurvy was unlikely to have made it into your differentials list. Petechiae can also be present – you probably ran an FBC and CRP. But petechiae, follicular hyperkeratosis and perifollicular haemorrhage are all cutaneous manifestations of scurvy, while gingival signs include swelling, bleeding and loss of teeth.

Radiographic findings in scurvy
Case courtesy of Dr Matt Skalski, Radiopaedia.org. From the case rID: 19946

And consider the limping child. Arthralgia, limb and joint swellings, limp, inability to weight-bear are often the presenting features of children being brought to medical attention. Although rare, scurvy should be considered in the differential of the limping child once the more common causes have been ruled out.

Risk Factors 

In children of the developed world, risk factors for developing scurvy are severe dietary restriction of fruit and vegetables. These can be secondary to autism, developmental delay and psychiatric disorders, as well as the extremely fussy child.

Diagnosis

Scurvy is a clinical diagnosis based on presentation of typical signs and symptoms alongside a dietary history of restrictive vitamin C intake for at least 1-3 months. Most laboratories cannot process ascorbic acid levels. If they can be measured then a level less than 11 umol/L would be considered deficient.

The diagnosis is, in the main, a clinical one. When a signs and symptoms respond to dietary changes or supplementation with Vitamin C then you know you are on the right track.

Classic bony signs on imaging include: periosteal oedema, sub-periosteal collections and sub-periosteal haematomas, lucent bands through long bone metaphyses, osteopenia and widening of the distal extremity of the femur. 

Management

  • Vitamin C supplementation orally for children with 100-300mg for one month or until full recovery.
  • All children under five years of age are recommended to take a multivitamin (unless formula feeding as this is already fortified). 
  • Referral to a dietician for dietary education is imperative. 
  • Resolution of symptoms can start within 24 hours but may require a few weeks of treatment to fully resolve.

Two months later, the child was seen in paediatric outpatients running and jumping in the consulting room. They subsequently had an appointment with the dieticians who discussed dietary changes with the family and ensured that all calorie and nutrient requirements were being met. The child continued to have a restricted diet and will therefore need a multivitamin supplement long term. 

Scurvy is a preventable, easily treatable disease which due to its non-specific symptoms is often misdiagnosed or carries a delay in diagnosis, with patients presenting to healthcare professionals on multiple occasions. A good nutritional history is key to diagnosis. 

Selected references on scurvy

Agarwal A, Shaharyar A, Kumar A et al. Scurvy in pediatric age group- A disease often forgotten? Journal of clinical orthopaedics and trauma. 2015; 6(2): 101-7 https://doi:10.1016/j.jcot.2014.12.003

Alqanatish JT, Alqahtani F, Alsewairi WM, Al-Kenaizan S. Childhood Scurvy: an unusual case of refusal to walk in a child. Pediatric Rheumatology 2015; 13(1): 23 https://doi:10.1186/s12969-015-0020-1 

Chalouhi C, Nicolas N, Vegas N et al. Scurvy: A New Old Cause of Skeletal Pain in Young Children. Frontiers in Pediatrics. 2020; 8:8 doi:10.3389/fped.2020.00008 

De Ioris MA, Geremia C, Diamanti A et al. Risks of inadequate nutrition in disabled children: four cases of scurvy. Archives of Disease in Childhood. 2016; 101(9): 871  https://doi:10.1136/archdischild-2016-310911, https://doi:10.1136/archdischild-2016-310911

Kitcharoensakkul M, Schulz CG, Kassel R et al. Scurvy revealed by difficulty walking: three cases in young children. Journal of Clinical Rheumatology: practical report on rheumatic and musculoskeletal disease. 2014; 20(4): 224-228 https://doi: 10.1097/RHU.0000000000000101 

Noble JM, Mandel A, Patterson MC, Scurvy and rickets masked by chronic neurologic illness. Pediatrics. 2007; 119(3): e783-90 https://doi:10.1542/peds.2006-107

Pazirandeh S, Burns D. Overview of water-soluble vitamins. In: D, Seres, L, Kunins eds. UpToDate. 2020. Waltham, UpToDate [ Accessed 28th February 2021 ] Available from https://www.uptodate.com/contents/overview-of-water-solube-vitamins

Ratanachy EK, Sukswai P, Jeerathanyasakun Y, Wngtapradit L. Scurvy in pediatric patients: a review of 28 cases. Journal of the Medical Association of Thailand. 2003; 86(3): S734-S740

Weinstein M, Babyn P, Zlotkin S. An orange a day keeps the doctor away: scurvy in the year 2000. Pediatrics. 2001; 108 (3): E55. https://doi:10.1542/peds.108.3.e55

World Health Organization. Scurvy and its prevention and control in major emergencies [online]. World Health Organisation, 1999 [Viewed 28th February 2021]. Available from: https://apps.who.int/iris/bitstream/handle/10665/66962/WHO_NHD_99.11.pdf?ua=1

51st Bubblewrap header

The 51st Bubble Wrap

Cite this article as:
Currie, V. The 51st Bubble Wrap, Don't Forget the Bubbles, 2021. Available at:
https://dontforgetthebubbles.com/the-51st-bubble-wrap/

With millions upon millions of journal articles being published every year it is impossible to keep up.  Every month we ask some of our friends from PERUKI (Paediatric Emergency Research in UK and Ireland) to point out something that has caught their eye.

Article 1: Anti-coagulation in CRRT

Chen Z, Wang H, Wu Z, Jin M, Chen Y, Li J, Wei Q, Tao S, Zeng Q. Continuous Renal-Replacement Therapy in Critically Ill Children: Practice Changes and Association With Outcome. Pediatr Crit Care Med. 2021 May 7. doi: 10.1097/PCC.0000000000002751. Epub ahead of print. PMID: 33965990.

What’s it about? 

This paper focuses on the paediatric patient outcomes for anticoagulant therapies used in continuous renal replacement therapy (CRRT) (heparin versus citrate).

The authors conducted a retrospective single centre audit over a 10-year period from 2010 to 2019 (289 patients included in the analysis) in Guangzhou, China. The median age of the patients was 4 years of age with a median fluid overload (FO) percentage at the initiation of the CRRT of 5.4%, and overall mortality of 46.3%.

Interestingly in 2015, the CRRT anticoagulant therapy practice was changed from heparin to citrate. There was no direct head-to-head comparison of the anticoagulants and in the paper 8 out of 10 patients received citrate anticoagulation. This paper assessed if the change in practice of anticoagulant had an impact on patient mortality.

The most common indications for CRRT in the paper were cytokine clearance 40%, acute kidney injury 23% and metabolic disease at 22%. In the paper, the heparin cohort had a higher mortality of 65% versus 47% in the citrate cohort. Interestingly if CRRT was initiated later (greater than 24 hours) then patients had a higher proportion of receiving vasoactive medication support, required longer mechanical ventilation days and had a greater fluid overload percentage.

Some limitations are that this is a single centre study, and the way in which they determined CRRT initiation time was time from admission to PICU to CRRT initiation time- which is not the usual way of evaluating early vs late CRRT.

Why does it matter? 

CRRT provides life-saving kidney support and assists with removal of electrolytes, fluid, and toxins from the body. Citrate anticoagulation in CRRT has offered patients an alternative to heparin. Heparin has the risk of heparin induced thrombocytopenia.

The emergence of citrate anticoagulant in CRRT over the last decade has offered promise for patients in this high-risk group and decreased mortality.

Clinically Relevant Bottom Line:

Paediatric patients presenting to hospital who require CRRT have a high risk of mortality. This paper concluded that a lower mortality with CRRT was associated with fluid overload % < 10%, CRRT initiation in less than 24 hours, and the use of citrate anticoagulant.

Reviewed by: Michele Cree

Article 2: It’s not always COVID

Snelson E, Roland D, Munro APS Throat and ear infections in children: URTI in the time of COVID-19Archives of Disease in Childhood – Education and Practice 2021;106:172-174.

What’s it about? 

This piece, prompted by restrictions presented by the COVID19 pandemic, discusses an alternative approach to the management of upper respiratory tract infections (URTI – tonsillitis and otitis media) in the UK.

The reduction in rheumatic fever incidence and vaccination against Haemophilus and Pneumococcus has resulted in a reduction in invasive URTI. The primary complications of URTIs are now mostly limited to peritonsilar abscess formation or mastoiditis. The incidence of both of these is not reduced significantly by antibiotic use (NNT >4000) and remain rare in children. The duration of tonsillitis symptoms following antibiotic treatment is only 18hours less, on average, and only if treatment is initiated before day 3 of symptom onset. In addition to this, antibiotics are demonstrated to cause many side effects including GI disturbance, allergy, and increased incidence of atopy and autoimmune conditions later in childhood.

The UK (NICE) guidance for otitis media was clarified in 2019. It now provides a graded approach of observation, delayed prescribing or same-day prescribing depending on the presence of systemic features, complications or in patients <2 years of age with bilateral AOM. Similarly, sore throat presentations are risk-stratified using FeverPAIN or CENTOR scoring – the former has not been validated in patients <3yrs and the latter was derived in an adult population.

Due to these guidelines frequently using the term ‘consider’ before suggested actions, the variation in clinical practice is not a surprise. This results in frustration for both clinicians in doubt and families expecting prescriptions..

Why does it matter? 

There is growing evidence that antibiotic use in URTI does not reduce symptom duration, and that the incidence of URTI associate complications is declining. This unfortunately has not detached clinicians’ hopes of reducing symptoms as a key factor in decision making.

COVID19 had a dramatic impact on clinical practice with the rise of virtual consultation and changes in health-seeking behaviour following government advice.

The Royal Children’s Hospital of Melbourne implemented a binary approach to reduce decision fatigue and improve antimicrobial guardianship. In this model, after the exclusion of sepsis and complications of URTI, AND if the patient is not classified as high risk (e.g. immunocompromised) then antibiotics are not immediately prescribed.

Clinically Relevant Bottom Line:

The proposed binary approach to decision making regarding antibiotic provision in URTI aims to reduce decision fatigue, unify the approach of clinicians, aid antimicrobial stewardship and efforts to inform health seeking behaviours.

For a round- up of the most up to date COVID literature in paediatrics why not visit https://dontforgetthebubbles.com/evidence-summary-paediatric-covid-19-literature/

Reviewed by: Joshua Tulley

Article 3: A dipstick for diarrhoea?

Cotter JM, Thomas J, Birkholz M, et al. Clinical Impact of a Diagnostic Gastrointestinal Panel in Children. Pediatrics. 2021;147(5):e2020036954

What’s it all about?

This was a multicentre cross sectional-study of paediatric patients, in Colorado, who had stool testing from 2013 to 2017. The study looked at two time periods: 24 months before the introduction of rapid multiplex polymerase chain reaction gastrointestinal panel (GIP) and 24 months after the introduction of GIP. The GIP detects 22 enteric pathogens, including bacteria, viruses, and parasites with an aim to provide antibiotic susceptibilities. Stool cultures were additionally performed when the GIP was positive for Shigella or Salmonella.

The authors compared test use, pathogen detection and time to results. Among children who were hospitalized, they compared length of stay (LOS), ancillary testing including labs and imaging studies, time to treatment, and hospital charges.

After GIP was introduced, there was an increase in stool testing of 21%. There was a higher percentage of positive results (40% vs 11%). Among the 1,986 hospitalized children, only 3% received antimicrobials to treat bacteria/parasitic causes for diarrhoea. There was a significant decrease in time to result (4 vs 31 hours) and time to treatment (11 vs 35 hours). There was also a significant decrease in LOS by 2 days (3.1 vs 5.1 days). However, there was no statistical difference in LOS, ancillary testing, or charges in the overall population.

I would have liked to see whether there was a difference in rate of associated complications or adverse events in the pre and post GIP era among hospitalized children with community-acquired diarrhoea. This study was also conducted within a single healthcare system and in an ideal world should be investigated at other institutions.

Why does it matter?

Children get diarrhoea… a lot. Common culprits include viruses, bacteria, parasites and the occassional non-infectious aetiology. GIP allows for the testing of multiple organisms with faster turnaround of results, but it is also expensive.  We need to be judicious about which patients should undergo testing with GIP and determine whether results will impact clinical care.

The Bottom Line:

GIP may allow for faster turnaround of results, faster time to treatment, and decrease in LOS for a small subset of patients. Although the GIP yielded positive results in 4 out of 10 patients, most positive tests were due to viruses and 6 out of ten of GIP results were negative. Given that most cases of diarrhoea are self-limiting and can be managed with supportive care, the routine use of GIP does not seem cost-effective. Consider GIP (if available in your institution) if the pre-test probability for an actionable and treatable aetiology is high.

For more information on gastroenteritis why not visit Angharad Griffiths’ post at https://dontforgetthebubbles.com/gastroenteritis/

Reviewed by: Dennis Ren

Article 4: Oxygen in African children with pneumonia

Maitland K, Kiguli S, Olupot-Olupot P et al. Randomized controlled trial of oxygen therapy and high-flow nasal therapy in African children with pneumonia. Intensive Care Medicine 2021;47(5):566-576

What’s it about?

This multicentre African study investigated which delivery method of oxygen support, children (age 28 days to 12 years) admitted with confirmed pneumonia (deemed severely hypoxic (Sp02 80-91%) or hypoxic (Sp02< 92%)) would need to decrease mortality. The trial was named COAST (The Children’s Oxygen Administration Strategies Trial). Those in the severely hypoxic arm (n=388) were randomised to high flow nasal therapy (HFNT) (n=194) or low flow oxygen (LFO) (n=194). Patients in the hypoxic arm (n=1454) were randomised to HFNT(n=363), LFO(n=364) or permissive hypoxia (n=727). HFNT was delivered by AIRVO2– initiated at room air and LFO was oxygen delivered by nasal cannula or standard masks. Ethical approval was sought.

Primary endpoints were mortality at 48 hours and up to day 28 post-randomisation. The entire trial was prematurely stopped due to a campaign in Uganda that deemed permissive hypoxia unethical.  Of the data collected, in the severely hypoxic group, 48 hours mortality was better using HFNT 9.3% vs 13.4% for LFO, demonstrating a 40% risk reduction in the HFNT arm.  For the hypoxic group, there was similar mortality at 48 hours across all arms (1.1% for HFNT, 2.5% for LFO and 1.4% for permissive hypoxia). Similar patterns were demonstrated at 28 days mortality. However, interpretation of the statistical significance of these results was severely limited by the inability to adequately power the study, due to early trial termination.

(For more information on High Flow see Padmanabhan Ramnarayan’s post on High Flow- When and How available at: https://dontforgetthebubbles.com/high-flow-therapy-when-and-how/)  

(In addition to this great learning module on Pneumonia by Ellis Collins and Michelle Alisio: https://dontforgetthebubbles.com/pneumonia-module/)

Why does it matter?

In developing countries, the supply and demand of oxygen in hospitals can be financially and logistically challenging. Studies that explore the rationalisation of oxygen therapies that are clinically safe, as well as reduce cost burden are beneficial. This is also relevant, in recent times with the increased demand for oxygen therapy in the COVID-19 pandemic.

Although oxygen therapy is deemed a standard pneumonia treatment, there is evolving evidence of oxygen toxicity with over-use, thus needs more careful consideration.

The bottom line

The use of HFNT vs LFO vs permissive hypoxia in children admitted to hospital with pneumonia in low resource settings still remains unclear.  Although this study does not provide definitive data to inform treatment guidelines, it supports the need for future, adequately powered studies of a similar design to explore this further. Until then, clinicians should be guided by the World Health Organisation (WHO) ‘Hospital Care for Children’ guidance for best practice.

Reviewed by: Melanie Ranaweera

Article 5: Splenic injury? Off you go

Leah Plumblee et al, J Trauma Acute Care Surg.Isolated low-grade solid organ injuries in children following blunt abdominal trauma: Is it time to consider discharge from the emergency department?  2020;89: 887–893 DOI: 10.1097/TA.0000000000002899 PMID: 32769952

What’s it about?

In this study, the authors examine the rate of “major” intervention (surgery, angiographic embolization, or blood transfusion) received by patients with grade I-III solid organ injuries from blunt trauma. The authors performed a secondary analysis of data gathered from two previously conducted trials (PECARN Network). Their hypothesis was that patients with isolated grade I-III solid organ injuries would be unlikely to receive interventions, which would indicate that these patients could be managed by simple observation in the ED, followed by discharge instead of the traditional pathways of admission for overnight observation.

A total of 517 patients were assessed of whom 34 underwent a major intervention. None of the patients with isolated grade I-II solid organ injuries and only 2.6% of the isolated grade III injuries received a major intervention. Almost 1 in 10 had multiple low-grade injuries, and this did not increase the risk of intervention.

It should be noted however, that in the original studies, only 3% of these patients were discharged from the ED directly by the treating clinicians, suggesting that there was something pushing the original clinicians to admit most of these cases. The median length of stay was 2 days.

Additionally, it is important to highlight some patients were excluded from the original trial if they had a CT at their original institution before being transferred to a participating centre. This could be an important source of bias, if the original clinician was concerned enough to transfer a low-grade injury, then the likelihood of intervention is probably higher; but we do not know, as these patients were excluded from the original studies.

Why does it matter?

In contrast to adults, the majority of paediatric solid organ injuries are managed conservatively, particularly low-grade injuries from blunt trauma. The authors conclude that it is likely safe to discharge patients with grade I and II injuries without other significant injuries from the ED after a brief period of observation. While I agree it is unlikely that these patients would require what the authors define as “major” interventions. This definition is too narrow and there are a lot of important therapeutic interventions that are reasons to admit patients that have been excluded from the trial by this definition. Additionally, there were no attempts made to assess for other important factors addressed by admission such as follow up, MDT input and patient satisfaction. As a result, I find that I cannot agree with the sentiment expressed by the authors, while they have shown that this may be a safe option, they have provided insufficient evidence to demonstrate that it is an option that we should pursue. The author’s conclusions are unsupported by the evidence provided and represent too big of a leap. We have no idea what other services patients will miss from inpatient services.

Clinically Relevant Bottom Line:

Caution must be taken when discharging patients from the ED who have evidence of solid organ injury based on the conclusions of this study alone.

Reviewed by: Sean Croughan 

If we have missed out on something useful or you think other articles are absolutely worth sharing, please add them in the comments!

That’s it for this month. Many thanks to all of our reviewers who have taken the time to scour the literature so you don’t have to.

All articles reviewed and edited by Vicki Currie

Peripheral IV cannulation

miniMAGICal thinking

Cite this article as:
Amanda Ullman, Tricia Kleidon and Elizabeth Andresen. miniMAGICal thinking, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.33480

18-month-old Byron presents to ED with septic arthritis. You know you need to take cultures, so you think you might pop a peripheral intravenous cannula in his antecubital while you’re there – he’ll need intravenous (IV) antibiotics for sure, maybe some fluids. He’s toddler-level chubby, and not exactly compliant.

Time for a deep breath.

Inserting peripheral intravenous catheters (PIVCs) is a core skill in paediatric acute care. We often make a quick decision to insert a PIVC in order to ensure accurate diagnosis and commence important treatments. We often worry about our capacity to insert the PIVC, especially in children like Byron. The vessels can be hard to visualise and palpate, and putting a young child going through a painful procedure can be stressful for all concerned.

But quick decisions have consequences

We know that PIVCs can result in harm. Multiple insertion attempts, extravasations, and infections are all a risk. They often stop working prior to the completion of treatment. PIVCs last only 48 hours, on average, in children and young people. When they fail, we not only pause treatment, but we frequently have to start the process all over again. According to children (and their families) cannulation is the most stressful part of their healthcare experience.

Think carefully about the IV, before it is inserted.

We have an array of IV devices and a variety of places to insert them. In 2020, the Michigan Appropriateness Guide for Intravenous Catheters (miniMAGIC) was published. Its aim was to improve the safe selection of IVs in children across a range of indications. You can read them (open access) here; or download the app for Google or Apple.

In Queensland (Australia) we have developed our first practice targets to improve IV selection, insertion, and securement based on miniMAGIC, and thus reduce IV associated harm. We are currently rolling them out at Queensland Children’s Hospital (Australia). These are:

Paediatric IVC and miniMAGIC

Improving device selection

Peripheral devices (like PIVC or midline catheters) should only be used for peripherally compatible therapies, outside of an emergency or crisis

To ensure safe administration of ‘at risk’ infusates, we consider:

  • Is this PIVC working? Check for good flow prior to administration
  • Is this PIVC optimally placed? Ideally, it should be away from a joint?
  • Are we giving this medication slowly and with enough dilution? Check the Paediatric Injectable Guidelines (PIG)
  • Is this PIVC site visible? It shouldn’t be coverfed up with crepe bandages

We are proactive in our device planning:  

  • If the child needs >2 days of IV therapy, make sure the PIVC is optimally positioned (e.g., in forearm)
  • If the child needs >5 days of IV therapy, consider a midline catheter

STOP and THINK, Make a plan! If this PIVC stops working, do we need to replace it immediately or are there other options? If this child is going to theatre, can we have the device ‘upgraded’?

Peripherally Inserted Central Catheters (PICCs) should not be inserted for antibiotic administration without discussion with the Infectious Diseases team. We use the minimal lumens necessary for treatment (NO just in case PICC/additional lumens).

Improving device insertion

Inserted them away from joints, where possible, looking for visible AND palpable vessels in the forearm. If there is nothing obvious it is time to turn to the trusty ultrasound machine if you have the skills. Even when we place topical anaesthetics, we pop some on the mid-forearm rather than antecubital fossa.

When faced with a child without palpable or visible vessels, don’t have multiple IV insertion attempts. Instead, refer early to an experienced clinician (+/- USG).

Improving device securement

keep the cannula visible, clean and secure
  • Keep it visible: Do not use crepe bandages; use tubular bandages.
  • Keep it clean: Use sterile products
  • Keep it secure: Use two points of securement

Take a look at Henry’s Twelve tips to placing a well secured PIVC.

For Byron, this means we consider the planned duration of intravenous antibiotics, assess his vessels, and our skills. If practical, Byron would have a PIVC inserted in the forearm (most likely via USG) where we can simultaneously take blood cultures. We would also consider an upgrade to midline or PICC, once the cultures and sensitivities are known, providing a better understanding of the actual duration of therapy and targeted antibiotic therapy.

We are evaluating the project, to see its impact on PIVC related morbidity. Based on the impact of MAGIC on adults, this is likely to be considerable (read here).

Another way to make sticking children with sharp needles less painful for everyone.

Want some top tips on paediatric cannulation? Watch our paediatric cannulation video on the DFTB YouTube channel here.

Defining Learning Disability

Cite this article as:
Liz Herrieven. Defining Learning Disability, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.33287

In all areas of paediatric practice (and beyond), we come into contact with children with a learning disability, learning difficulties or autism. Terminology is important, not only for making sure we understand a child’s diagnosis properly, but also for providing the best possible care. Getting it right can also help gain the trust of parents and carers who will often know far more about appropriate language use than we clinicians. This guide aims to describe the key points to remember – some of them easier to grasp than others.

Learning Disability

Let’s start with learning disability or LD. 2.5% of the children in the UK have a learning disability, yet it’s one of the most commonly confused terms. Probably the most pragmatic and descriptive definition is that used by Mencap, a UK charity supporting people with LD: “A learning disability is a reduced intellectual ability and difficulty with everyday activities – for example household tasks, socialising or managing money – which affects someone for their whole life.” The World Health Organisation keeps it short, although open to discussion and interpretation. For them it’s “a state of arrested or incomplete development of mind.”

Having a learning disability means an individual will not only find learning difficult but also face challenges with retaining, processing, reasoning and deducing information. Some people will find different areas of learning more challenging than others. Children with Down syndrome have a relative strength in visual learning and find learning or remembering auditory information more difficult. Building on strengths can help to balance out some of the more challenging areas. Some people with LD may be able to communicate very well, even if they struggle to understand all of what is communicated to them but many will have an associated speech and language problem.

The term intellectual disability, or ID, is used rather than LD, to signify that the condition affects intellect and is lifelong. This fact is important – the individual will need support, depending on their level of disability, for the whole of their life. There are many causes of LD, all involve the developing brain – genetic or chromosomal conditions, intrauterine infections, perinatal hypoxic brain injury to name but a few. After the brain has developed, such an insult is described as an acquired brain injury.

The level of disability may be mild, moderate or severe, depending on IQ, although this is rarely formally calculated and actually doesn’t really add much. Support and care should be tailored to an individual’s needs rather than their IQ.

The term PMLD is used to describe individuals with Profound and Multiple Learning Disability. These patients may have fairly complex comorbidities alongside severe learning disabilities. They can affect not only their ability to learn and process information, but also their ability to communicate and to be independent.

Learning Difficulty

A learning difficulty is very different to a learning disability, and is far more common. Things like ADHD, dyspraxia or dyslexia are all examples of a learning difficulty. They all make learning more difficult, but don’t affect overall intellect or IQ.

Autism

Autism, or an autistic spectrum condition (ASC), is not itself a learning disability, although about one-third of people with ASC will also have LD. The National Autistic Society (UK) describes autism as “a lifelong developmental disability which affects how people communicate and interact with the world”. There are lots of different elements, each of which may be present to a greater or lesser extent within one individual, so each autistic person is different from the next. The autistic spectrum is not a linear thing, with someone being more or less autistic. “High functioning” or “low functioning” are not particularly appropriate terms either. It’s more helpful to think about how someone’s autistic features affect them. The most common features include social communication difficulties, sensory processing disorder and restrictive or repetitive movements.

Social communication difficulties include challenges in interpreting body language or facial expression, and reading hidden meaning into words or phrases, particularly when metaphors are used. Sensory processing difficulties involve the body misinterpreting sensations. A light touch may be perceived as very painful whilst a deeper touch may be more comforting. Bright lights or certain noises could be very distressing. Restrictive or repetitive movements are often comforting, or theymay distract from upsetting or uncomfortable situations.

ASC was more commonly known as ASD, or autistic spectrum disorder. The move to calling it a condition, instead, is an attempt to remove unnecessary negativity. ASC encompasses many other conditions such as that previously known as Asperger syndrome. This name is no longer preferred – Hans Asperger has a troubling history. It was used to describe people with normal or even high intelligence, coupled with autistic features. Other conditions included under the ASC umbrella include PDD (pervasive developmental disorder) and PDA (pathological demand avoidance).

Person First vs Identity First Language

We use person-first language for many conditions. Someone with asthma is not defined by their asthma but has a whole identity of their own, so they are described as someone with asthma. Someone with Down syndrome may share certain physical features with someone else with Down syndrome, but they have their own identity and character which is very different from that of others with Down syndrome, so they are described as a person with Down syndrome, not a Down’s person or, even worse, a Downs.

Child with learning disability

Many autistic people feel that their autistic features form part of their identity – that they would be a very different person if they did not have autism, so they describe themselves as autistic, rather than a person with autism. You can read more about person first vs identity first language here.

And if you can’t remember what to say when?

Ask! It’s always better to ask someone how they would prefer to be described than to guess. All people. whether they have LD, a learning difficulty or ASC are individuals and will have their own preferences, likes and dislikes. Getting the language right can be a great start, but being honest and open when you’re not sure is a very close second.

The 50th Bubble Wrap

Cite this article as:
Currie, V. The 50th Bubble Wrap, Don't Forget the Bubbles, 2021. Available at:
https://dontforgetthebubbles.com/the-50th-bubble-wrap/

With millions upon millions of journal articles being published every year it is impossible to keep up.  Every month we ask some of our friends from PERUKI (Paediatric Emergency Research in UK and Ireland) to point out something that has caught their eye.

Article 1: Are steroids of any use in pre-school wheeze?

Wallace A, Sinclair O, Shepherd M, et alImpact of oral corticosteroids on respiratory outcomes in acute preschool wheeze: a randomised clinical trialArchives of Disease in Childhood 2021;106:339-344

What’s it about? 

This paper looks at a common problem and one where there is huge variation in practice; should we give oral steroids to pre-school children who present to the emergency department with wheeze?

This was a double-blinded, randomised, placebo-controlled trial based in three hospitals in New Zealand. Children were either allocated 3 days of oral prednisolone or a placebo of similar colour, taste and viscosity (yuk!).  The primary outcome was measured by a change in Preschool Respiratory Assessment Measure (PRAM) score at 24 hours, although several interesting secondary outcomes were also measured.  The authors looked at 24–59 month-olds in order to exclude bronchiolitis cases. 3247 children were identified as being eligible with 477 children making it to the intended to treat analysis.

Why does it matter? 

In 2009 Panikar et al found there was no reduction in duration of hospitalization when giving steroids for pre-school wheeze, looking at children from 10 months to 6 years of age. However, in 2018 Foster et al found that giving oral steroids for children aged between 2 and 6 years old reduced their average length of stay from 540 to 370 minutes.

The authors of this paper found no difference in PRAM score at 24 hours between the groups (their primary outcome measure) and also no difference in the number of doses of salbutamol administered, length of stay for those admitted or representation rates within 7 days. A novel finding was that at 24 hours the median PRAM score was 0 for both groups and only one child still had severe disease (PRAM 9-12).  This might be the fact that 218 children were excluded for ‘severe disease’ despite this not being in the exclusion criteria, as well as 175 children excluded for previous life-threatening asthma and 123 excluded for chronic respiratory or cardiac disease.

However, the authors did find that the prednisolone group had less respiratory distress 4 hours after medication administration and reduced requirement for hospital admission, additional corticosteroid or intravenous treatment.

Clinically Relevant Bottom Line:

The results of this study suggest that giving oral steroids for pre-school wheeze is of limited benefit. However it may explain why there is perceived benefit, children clinically respond in the first 4-6 hours even if the overall outcome isn’t altered. It’s not clear from the study how long after arrival steroids were given and this may be an interesting area of further study.

Many centres now give single-dose dexamethasone which concords with the findings of this study in that 3 days of prednisolone may not be necessary given the median PRAM score of 0 at 24 hours. Some clinicians may find the reduced need for intravenous medications or hospital admission sufficient benefit to give a single dose of dexamethasone in the PED and so it is unlikely the debate about which pre-school children should receive steroids has been put to bed.

Reviewed by: James Thyng

Article 2: This is just how I cope

Herrick SS, Hallward L, Duncan LR. “This is just how I cope”: An inductive thematic analysis of eating disorder recovery content created and shared on TikTok using #EDrecovery. International Journal of Eating Disorders. 2021 Apr;54(4):516-26.

What’s it about? 

This Canadian study investigated the impact of eating disorder (ED) recovery videos shared on the social media platform TikTok.

The study completed a thematic analysis of the first150 TikTok posts under the hashtag #EDrecovery (Eating Disorder recovery) in June 2020 (454.5 million views as of June 2020 and around 1,500 posts with this hashtag).

Five themes were identified: ED awareness (N=32), Inpatient story time: “ED unit tings” (N=28), Eating in recovery (N=27), Transformations: “how about a weight gain glow up?” (N=27) and Trendy gallows humour: “let’s confuse people who have a good relationship with food” (N=36).

In around 1 in 5 of the posts creators shared different aspects of recovery to encourage a better understanding of recovery and ED’s. Four subthemes were found: recovery victories, reality of recovery, education and sharing positivity within these raising awareness posts.

Although the #EDrecovery videos raised awareness about eating disorder, some videos contain content which blur the line between ED recovery and pro-ED content and may be harmful to some TikTok users.

Why does it matter? 

Social media channels such as TikTok has a large impact on its user base, the majority of which are children and teenagers. TikTok formed in 2017 is one of the fastest growing mobile apps- with over 2 billion downloads (as of January 2020) and a whopping estimated 80 BILLION active users, the majority being children and young adults.

TikTok can be used to spread ED awareness and build a supportive community for ED recovery users. It is important to explore whether TikTok can be used as a tool to assist in ED recovery.

Clinically Relevant Bottom Line:

A fine line exists between ED recovery and pro-ED content. Some users find #EDrecovery videos helpful, while others may misinterpret the videos as triggering their ED behaviour.

Reviewed by: Jessica Win See Wong

Article 3: Do rapid diagnostic methods improve antibiotic prescribing in bacteraemia?

Faugno, AK., Laidman, AY., Perez Martinez, JD., Campbell, AJ., & Blyth, CC. (2021). Do rapid diagnostic methods improve antibiotic prescribing in paediatric bacteraemia? J Paediatr Child Health, 57(4), 574–580. https://doi.org/10.1111/jpc.15272

What’s it all about?

Rapid diagnostic methods are being developed to identify causative pathogens earlier to optimise early antibiotic therapy for patients with bacteraemia. The authors performed a retrospective study of 255 bacteraemia cases at a tertiary referral hospital in Western Australia. They compared patient outcomes in cohorts receiving antibiotics before and after the implementation of two rapid diagnostic tests: matrix-assisted laser desorption ionisation time-of-flight (MALDI-TOF) and GeneXpert Xpert MRSA/SA.

The median time taken to administer optimal therapy was not significantly different between those undergoing rapid diagnostic testing and those who did not (39.1 vs 44.4hrs, p= 0.66). Similarly, there was no significant difference in hospital length of stay (7 vs 9 days), number of ICU admissions (20 vs 15) or all-cause mortality (1.6 vs 1.6%).

Why does it matter?

It is well-established that timely administration of empirical antibiotic therapy in cases of sepsis can reduce mortality and morbidity. In fact, one-third of paediatric deaths within intensive care units are associated with sepsis or septic shock. What’s more, the prolonged use of broad-spectrum antibiotics and delay in targeting optimal therapy can potentiate antibiotic resistance.

Adult studies have already shown that timely identification of pathogens can improve appropriate antibiotic therapy but there is little known about its effect in paediatric populations who present with a difference clinical and microbiological profile.

The Bottom Line:

The lack of significant difference in this clinical outcome data suggests that there is no beneficial impact of implementing rapid diagnostic testing in paediatric populations with bacteraemia. The authors suggested possible reasons for this, such as the lack of explanations or training in interpreting rapid diagnostic test results for treating doctors, and therefore the need for a real-time programme to support clinical decision making. 

However, it would be interesting to compare this data to other paediatric hospitals through a multi-centre study, given that other regions may have higher rates of antimicrobial resistance and candidaemia which can alter the effectiveness of empiric antibiotic therapy. Moreover, changes to hospital guidelines for empirical therapy may be better captured through prospective studies in the future.

Reviewed by: Ivy Jiang

Article 4: What’s the time, Mr Wolf?

Stellman, R., Redfern, A., Lahri, S., Esterhuizen, T., Cheema, B. (2021) How much time do doctors spend providing care to each child in the ED? A time and motion study. Emergency Medicine Journal, Published Online First: 15 April 2021. doi: 10.1136/emermed-2019-208903

What’s it about?

The authors conducted a cross-sectional observational study over a 5-week period in two EDs – a tertiary centre and a large district hospital, both with separate paediatric ED areas – in the Western Cape of Africa. The “time and motion” methodology was used, where a single trained, independent observer was placed in the ED and observed and timed Doctors’ interaction with patients that met inclusive criteria. Criteria included age (0-13 years), unplanned presentation (not referred  by another source) and had an initial assessment by a qualified medical officer employed by that site. The patient triage category, as per the South African Triage Scale, was also recorded. Primary objective: to measure the total time taken by doctors to assess and manage each of a series of paediatric patients. Secondary objective: whether acuity of patient—as estimated by triage category affected the average time taken, and to compare these averages to the estimated benchmarks used to calculate hospital staffing allocations in the region.

A total of 100 patients were included and the median DTPP (doctor time per patient) for each triage category was as follows:

  • Green (routine; see within 4 hours): 31 minutes (Target set for staffing calculations are: 15 minutes for green)
  • Yellow (urgent; see within 1 hour) 39 minutes (Target 40mins/ patient)
  • Orange (very urgent; see within 10 minutes) 48 minutes (Target 50min/patient)
  • Red (emergency; see immediately) 96 minutes (Target 50min/ patient)

These time frames were compared with the local hospital benchmarks (developed to guide assigning ED staffing) and found that the median timing for patients triaged as green and red were significantly higher (p=0.001 and p=0.002, respectively). 

Why does it matter?

Time often seems warped inside the Emergency Department (ED) – some shifts fly by while others seem to drag on. Sometimes, you see a new patient every 30 minutes, and other times, you spend 3 hours with just one patient! Whilst most ED have audits to monitor Key Performance Indicators (KPI) looking at waiting times, time to treatment, time to admission / time to discharge etc few studies actually look at the (DTPP), which is the time spent assessing and managing a patient. This value depends on a large number of medical and psychosocial variables but if it can be accurately measured, it could be used to determine the right staffing required in a department.

The bottom line

Even with the limitations of this study (small sample size, only a single observer collecting data for a short period of observation per day), the DTPP was found to be significantly higher than the local expected time frame for the highest and lowest triage categories. This study could be repeated at your local ED (with the limitations addressed) and the information could be useful in determining whether your ED is adequately staffed, which would in turn improve a variety of KPIs.

Reviewed by: Tina Abi Abdallah

Article 5: Is the use of excessive non-resuscitation fluid associated with worse outcomes?

Barhight MF, Nelson D, et al.Non-resuscitation fluid in excess of hydration requirements is associated with higher mortality in critically ill children [published online ahead of print, 2021 Mar 17]. Pediatric Research. 2021;1-6.

What’s it about?

This study looks at whether giving excessive non-resuscitation fluid to critically unwell children is associated with a worse clinical outcome.

The authors conducted an observational study in PICUs of two large American hospitals between 2010 and 2018. They reviewed 14,483 patients and calculated their daily fluid balance for the first 3 days of their stay. This included all fluid given that wasn’t prescribed as a ‘bolus’. They then used the cumulative fluid balance at the end of day 3 to work out a percentage fluid overload (FO) which they stratified into <10%, 10-20% and >20%. The primary outcome was in-hospital mortality and the secondary outcome was ventilator-free days at 28 days.

For each FO category, the volume of fluid that was given in excess of the estimated requirement (using the Holliday-Seger method) was calculated. This demonstrated that excess non-resuscitation fluid was given in just over 1 in 2 of the patients and that maintenance fluid and nutrition were the largest contributors to fluid input. The number of patients receiving excess fluid increased with each FO category and equated to an excess of 164ml/kg in the >20% group.

Analysis was performed for each FO strata to look for an association between % FO and mortality, adjusting for confounders such as age and illness severity. This demonstrated an increase in adjusted odds of death when compared to the <10% group of 1.8 times in the 10-20% group and 2.6 times in the >20% group.

The authors also found that for every 10ml/kg of excess fluid given, there was 1.01 times higher odds of death. There was also a 1% decrease in likelihood of having a ventilator free day.

As this is an observational study, it can only demonstrate associations and not causality.

Why does it matter?

In the adult population studies have shown that almost 60% of the fluid administered to patients was in the form of maintenance fluids and “fluid creep” (the combined volume used from medications, electrolytes, and continuous infusions used to keep access lines open).

Even though this study relates to critically unwell children, maintenance fluids are something we prescribe routinely within paediatrics, and this study highlights the need for a carefully considered approach to their use in all children, with frequent readjustment according to fluid balance so as not to cause harm.

Further studies are required to examine whether our current prescribing practices should be modified, but nevertheless, this study should make us think more carefully about how much fluid we are giving to our patients.

Clinically Relevant Bottom Line:

 This study shows that non-resuscitation fluids are frequently given to patients in excess of their hydration requirements and could represent potential iatrogenic harm. 

Reviewed by: Laura Duthie

If we have missed out on something useful or you think other articles are absolutely worth sharing, please add them in the comments!

That’s it for this month. Many thanks to all of our reviewers who have taken the time to scour the literature so you don’t have to.

All articles reviewed and edited by Vicki Currie

Croup

Cite this article as:
Laura Riddick. Croup, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.32637

It’s 0200 hours in the Emergency Department and you hear a seal …

As children have returned to school we have seen more croup through the ED so it’s time to refresh your memories!

What is it?

Viral laryngotracheobronchitis. It is essentially inflammation around the main large breathing structures and caused usually by parainfluenza 1 + 3. Other respiratory viruses including SARS-CoV-2 and RSV may also be involved. This inflammation causes a tell-tale cough and noisy breathing due to the obstruction to flow. There may be signs of increased work of breathing too such as sub-costal recession or a tracheal tug. They are generally quite well and are running around the waiting room!

Who gets it?

A lot of children – roughly 2-3% of all children per year! These kids are usually between six months and four years of age, and occurs at the beginning of autumn, though this spring we are seeing a lot of cases. Children with croup may present with a preceding coryza-like illness and a low-grade fever. This then develops into a barking “seal-like” cough and, for some reason, always seems worse at night. Boys are more commonly affected than girls, and some children seem to get it yearly.

How do we treat it?

This depends on your assessment of the child. Croup is a self-limiting viral illness and treatment tends to look to short term reduction in the inflammation to improve the work of breathing. Historically clinicians have used Westley scoring system to score croup and assess their severity before giving medication.

Westley Croup scxore
Westley Croup Score

In children who look unwell, it is important to not upset them by avoiding unnecessary interventions such as excessive handling or performing an ENT exam.

Steroids

If the child is able to take the medication, dexamethasone or prednisolone should be given to all cases of croup where any stridor or increased effort in breathing is present.

Dexamethasone appears to be more efficacious than prednisolone. It has an onset of action within 1 hour (30 minutes – 4 hours) and has a half-life of up to 36-72 hours (Schimmer 2005). There has been debate overdosing with doses of 0.15mg/kg, 0.3mg/kg and 0.6mg/kg of dexamethasone. Ultimately, 0.15mg/kg not inferior to 0.6mg/kg. At the time of writing both NICE and the BNFc recommend 0.15mg/kg as the initial dose of dexamethasone. If there are concerns about re-occurrence patients are occasionally sent home with an additional dose to be taken 12 hours later.

Prednisolone tends to be favoured in the primary care setting, at a dose of 1mg/kg with two additional daily doses. There appears to be no significant clinical difference between the two different steroids in terms of the need for additional treatment or length of stay. Dexamethasone was associated with a reduction in re-attendances, which may be due to the shorter half-life of Prednisolone (Gates 2018, Schimmer 2005)

Nebulised budesonide (2mg stat dose) is reserved for children who cannot take the dose. This may be because it was spat ou tor because they are working too hard to breathe. A Cochrane review in 2018 shows that budesonide is not superior to dexamethasone, with Westley Croup scores better in the dexamethasone group at 6 and 12 hours compared to budesonide. A combination of treatment does not appear to lead to additional benefit (Gates 2018)

Adrenaline/epinephrine

In severe cases, when the child has features of severe work of breathing, including significant recession, hypoxia or tiring, nebulised adrenaline has been used (0.4-0.5ml/kg, maximum 5ml of 1:1000). Adrenaline provides short term relief from respiratory distress and can be a bridge to getting steroids on board. The effects are short-acting and wear off after a couple of hours. It can be repeated every 30 minutes, although if you need repeat doses, anaesthetics and senior colleagues should be involved in this patients’ care.

How do we not treat it?

In the olden days parents tried treating croup at home with steam inhalation (not effective). In hospitals, humidified oxygen has also been tried though this has not been proven to be effective either (Moore 2007). Heliox (oxygen and helium combined) has also been looked at as it may improve airflow. The evidence is limited and safety and efficacy remain questionable (More, 2018). There is no evidence that salbutamol works in croup.

They sound better, what’s next?

If they are well and the stridor has resolved, patients can be discharged home with safety-netting advice. The effects of dexamethasone should last as croup itself is usually limited to 2-3 days of symptoms. Parents need to be aware that some symptoms of respiratory distress can return, usually the following night.

Patients may require a prolonged period of observation if:

  • stridor is still present at rest, or there is increased work of breathing
  • the child is very young (<3 months)
  • an adrenaline nebuliser had to be given
  • there is a past history of severe croup
  • there is a history of upper airway problems (i.e. laryngomalacia or subglottic stenosis)
  • concerns about the child returning (i.e. long-distance, social concerns)

When is it not croup?

  • Epiglottitis – a rare condition thanks to the HiB vaccine. A child would present with sudden onset, fever, drooling and looks unwell holding the head back and neck extended. This is a medical emergency and keeping the patient calm is paramount.
  • Tracheitis– thankfully also rare. It presents with the child acutely unwell after a prolonged course similar to Croup.
  • Anaphylaxis/allergy – this may be accompanied with angioedema, rash and wheeze, and requires swift treatment with IM adrenaline
  • Quinsy/retropharyngeal abscess
  • Foreign body – Usually the history would help suggest this, with a sudden onset history in a well-child.

COVID and croup

Most children admitted into hospital are now swabbed for COVID. This can provide a challenge – balancing upsetting the child (and making the upper airway obstruction worse) and performing an invasive swab. It is sensible not to swab the child whilst there is still concern about acute stridor and work of breathing..

There have been some case studies to suggest a small cohort of patients with croup who were SARS-CoV-2 positive are less responsive to the usual treatment (Venn 2020). These cases may need prolonged admission due to lack of response and the need for additional supportive therapy.

Selected references

  1. Al-Mutairi B, Kirk V. Bacterial tracheitis in children: Approach to diagnosis and treatment. Paediatr Child Health. 2004;9(1):25-30. doi:10.1093/pch/9.1.25
  2. Garbutt JM, Conlon B, Sterkel R, et al. The comparative effectiveness of prednisolone and dexamethasone for children with croup: a community-based randomized trial.  Clin Pediatr (Phila). 2013;52(11):1014–1021.
  3. Gates  A, Gates  M, Vandermeer  B, Johnson  C, Hartling  L, Johnson  DW, Klassen  TP. Glucocorticoids for croup in children. Cochrane Database of Systematic Reviews 2018, Issue 8. Art. No.: CD001955. DOI: 10.1002/14651858.CD001955.pub4. Accessed 28 April 2021
  4. Moore M, Little P. Humidified air inhalation for treating croup: a systematic review and meta-analysis.  Fam Pract. 2007;24(4):295–301
  5. Moraa I, Sturman N, McGuire TM, van Driel ML. Heliox for croup in children. Cochrane Database of Systematic Reviews 2018, Issue 10. Art. No.: CD006822. DOI: 10.1002/14651858.CD006822.pub5
  6. Schimmer B P, Parker K L. Adrenocorticotropic hormone: adrenocortical steroids and their synthetic analogs: inhibitors of the synthesis and actions of adrenocortical hormones. Goodman and Gilman’s the pharmacological basis of therapeutics, 9th edition. New York: McGraw‐Hill, 20051459–1485
  7. Smith DK, McDermott AJ, Sullivan JF. Croup: Diagnosis and Management. Am Fam Physician. 2018 May 1;97(9):575-580. PMID: 29763253.
  8. Sparrow A, Geelhoed G. Prednisolone versus dexamethasone in croup: a randomised equivalence trial. Arch Dis Child. 2006;91(7):580-583. doi:10.1136/adc.2005.089516
  9. Venn AMR, Schmidt JM, Mullan PC. A case series of pediatric croup with COVID-19 [published online ahead of print, 2020 Sep 15]. Am J Emerg Med. 2020;S0735-6757(20)30829-9. doi:10.1016/j.ajem.2020.09.034
  10. https://www.rch.org.au/clinicalguide/guideline_index/Croup_Laryngotracheobronchitis/
  11. https://cks.nice.org.uk/topics/croup/

Ultrasound Guided Peripheral Vascular Access

Cite this article as:
Trent Calcutt. Ultrasound Guided Peripheral Vascular Access, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.23253

One of my favourite things in paediatrics is the expanding role of ultrasound guided vascular access.

When I started as a paediatric registrar, I’d just finished an adult ICU term where I’d become spent a majority of time supporting provision of a vascular access service, and as part of this had become a PICC line insertion instructor. Eventually, I got to the point where I dreamt of abstract grey shapes. But then I started a paediatric job in a regional hospital where it seemed that ultrasound was used for vascular access rarely if at all. Initially, I thought there must have been something different about paediatric vascular access that I was unaware of. One day, when looking after a young lady with Rett’s who was known to be difficult to cannulate, I reached for the ultrasound. In the five years since, ultrasound has been a standard part of my practice in achieving vascular access in children, with technique adapted to fit the age of the patient.

Ultrasound-guided vascular access and paediatrics seem like such a natural partnership. The concept of a DIVA (“difficult IV access”) patient is receiving increasing interest and research. Criteria for a DIVA can include prematurity, inability to see or feel a vessel, or an episode of multiple prior attempts. These criteria would be met by a huge number of the kids we care for, in particular toddlers or the previously premature infant. 

Chonky baby arm
Spot the veins

Why is ultrasound not the first-line adjunct in these tricky kids? It’s probably multifactorial, but certainly, ultrasound is more difficult in children than adults. Its utility is varied in the NICU context and for infants under 2.5kg, although can still have a role with a modification to technique. It’s also harder to learn ultrasound in a population who are scared, angry, impatient and poorly tolerant of a prolonged period of needle-through-skin. For these reasons, I think that there is less appeal to replace the familiar (cannulating without an ultrasound), with the unfamiliar (cannulating with an ultrasound). As I’d experienced, this also leads to a culture where ultrasound is infrequently utilized, decreasing the likelihood of implementation by new or more junior staff.

Once the learning investment is made to reach a proficient level of ultrasound competency (about 20 cannulas in adults) the potential benefits are significant. Decreased time spent performing a procedure, decreased number of attempts and subsequent patient trauma, and increased cannula longevity are all achievable.

I’ve spent a lot of time thinking success optimisation in paediatric ultrasound guided cannulation, both during my own development of proficiency and then in an effort to verbalize this skill when educating others. Below are my 5 top tips to enhance your ultrasound-guided cannulation skills:

I’m hoping that some of these words may help avoid some bits of the inevitable trial and error process that comes with learning a new skill.

There is sometimes a general impression of both practical and personal inconvenience in using ultrasound for vascular access. An ultrasound may not be nearby. There is the fear of “looking silly” in front of other people, as turning on, adjusting, and then physically coordinating the use of the ultrasound may be unfamiliar. During the period of establishing proficiency, an approach to decreasing this sense of unfamiliarity is to get in the habit of bringing the ultrasound with you do a cannula. Turn on and optimize the ultrasound to view vessels, and spend a period mapping out candidates for cannulation using your non-cannulating hand. Draw on the patient with a skin pen if you want to keep track of the best sites. Then, discard the ultrasound and cannulate using whatever technique is most familiar to you, but with the added knowledge of vessel location, depth, size, and direction. If this becomes a routine and almost ritualistic process, the mental barrier created by a lack of familiarity with ultrasound settings and holding the transducer should decrease over time. It is a relatively small step from performing vascular mapping to placing a cannula under real-time ultrasound guidance.

The preparation otherwise is quite straightforward. In addition to the set up that you use for all other cannulas, you need the following four things:

  • An ultrasound with a linear array probe (the smaller the footprint and the higher the frequency, the better)
  • Sterile lubricating gel and some form of sterile barrier to cover your probe (this varies institutionally)
  • Cavilon wipe or skin prep (securement devices / dressings / tape doesn’t like to stick to ultrasound gel so will need some encouragement)
  • An extra person (one of your hands is out of action, so you need an additional person to perform the task that your non-dominant hand would normally do; this is typically stabilization of the distal limb)

The ultrasound sits on the opposite side of the bed to the operator, so as to minimize truncal movement in looking from the puncture site to screen. Aside from making sure the correct probe is selected, the only 3 settings you need to know how to adjust are depth (typically as shallow as possible), gain (similar to a ‘brightness’ setting to highlight blood-filled vessels), and a midline marker (for physical-digital landmark referencing).

As alluded to above, pre-scanning is a useful skill even in the absence of cannulating under real-time ultrasound guidance. It’s a good idea to scope out the most appropriate vessels and puncture sites prior to picking up your cannula. Essentially the objective is to place a cannula within a vessel with as few attempts as possible, as quickly as possible, with as little pain as possible, and in a site that will provide the greatest longevity. Characteristics of vessels that tend to correlate with these outcomes are:

  • long and straight stretches
  • vessel 6mm or less below the surface
  • vessels greater than 2mm in diameter
  • vessels that don’t cross a joint (provides freedom of movement and less extravasation)
  • vessels without upstream thrombosis or obstruction

Mid-forearm vessels often meet the above criteria.

The greater length of cannula able to be placed within the vessel can correlate with longevity, however larger cannula diameter may increase the phlebitis and decrease longevity. This requires consideration of the balance between length and diameter of device. Of the commonly available devices, a good balance is a blue cannula (22G). There are several specialised less widely available devices that are longer versions of small diameter cannulae (24G and 22G).

In practical terms, to find these vessels you can start in the antecubital fossa (more familiar area for most of us) and track them down, or plonk down on the forearm and pan circumferentially. Scanning in the short axis / transverse axis / cross-sectional view tends to work best in kids. To assess suitability, translate the probe up and down along a vessel to get an idea of the direction. If it’s running diagonally, rotate your probe until it’s running along the same plane as the vessel to act as a mental reminder of the angle/direction that you need to insert your cannula. Pick the specific spot on the vessel that you’d like to puncture, bearing in mind that you will be puncturing the skin millimetres back from that point. Pick the patch of the vein that is the longest, straightest, shallowest, and biggest. Have a second fallback site planned out elsewhere for if required. Lastly, make sure to track the vein proximally as far as you can to ensure that it doesn’t run into a large thrombosed/occluded/recannalizing patch of vessel.

Obscure angles make things more challenging, in my experience. Right angles and parallel lines are your friends because they assist in mental unburdening and allow you to devote energy to troubleshooting issues. As mentioned above, map the vessel prior to puncture. Part or all of a vein will often wander diagonally along its journey, so approaching from the wrong direction increases the likelihood of punching through the side of the vessel. The centre of the image corresponds to the arrow/marker along the long edge of the probe, so you have a reference point between digital (screen) and physical (skin). Use the ultrasound as a mental reminder of your plane of approach; rotate the probe until the vessel is consistently sitting in the very centre of your image as you plane up and down. In other words, the ultrasound image is perfectly perpendicular to the plane of the vessel.

Speaking of right angles, I prefer to keep the ultrasound at right angles to the surface that you’re scanning. Angling back and forth creates a loss of contact and a distorted image as the ultrasound bounces of structures and does not return to the transducer. This creates a less clear image where vessels artificially look larger. If you need to change your view, translate/glide the probe along the skin, rather than introducing angle. It can be useful to temporarily angle the transducer perpendicular to the shaft of the cannula if you lose sight of it as this will light it up more clearly.

This is a big one. Thinking of your cannulation as a two-phase puncture process is something that I find extremely helpful. Your objective is not to puncture the skin and end up inside the vessel in a single action, and in fact, attempting to do this seems decrease the likelihood of success. 

 

Puncture Phase 1

Puncture 1 is the process from skin puncture to positioning the tip of your cannula on the superficial wall of the vessel. To achieve this, align your probe to achieve a view with the vessel in the centre of the image. Puncture the skin with the cannula a few millimetres distal to the probe. This bit is painful, so do this with a decisive action so that 2-3 mm of the cannula is within the soft tissue. Increase your angle of insertion to 30-45°. Your next objective is to find the tip of the cannula. Moving your non-dominant (ultrasound) hand, translate/slide the probe towards the puncture site until a glimmering white dot becomes apparent in your image. Once you are convinced that you are viewing your cannula, you need to ensure that you are viewing the tip at all times.

The most important thing to remember is the only way to be certain that you are viewing the tip of your cannula is when the glimmering dot disappears when you move the probe 1mm proximally (away). It is frustratingly easy to think that you are viewing your cannula tip when instead you are halfway along the shaft, with the tip out the deep wall of the vessel. Maintain this view via a “walking” approach. For each 1-2mm advancement (step) of the cannula, make an equivalent proximal movement with your ultrasound probe (step). Move the ultrasound away so that you cannot see cannula tip anymore, and then advance the cannula into view. If needed, intermittently stop advancing your cannula and check your tip position as described above. I find advancing at 30-45° until you reach the vessel works well as minimal cannula is wasted on the journey there.

If you find yourself wandering off track, keep the ultrasound focused around the vessel as the centre of your image (as this is your target). Correcting if off centre is slightly counterintuitive. Move your cannulating hand away from the direction that you want to move your cannula tip (ie- moving right will move the tip left). Continue inserting until your cannula tip is sitting at 12 o’clock on top of your vessel. As you reach this point, the tip of the cannula may gently tent the roof of the vessel, turning an “O” shape into a “❤️” shape. This is a good test of correct positioning. Once you’ve reached this point, you’re ready for puncture phase 2!!

 Puncture Phase 2

Puncture 2 is the process of entering the vessel to feeding your cannula fully in. With the tip of your cannula in view and the roof of the vessel tented (❤️), continue incrementally advancing your cannula with tiny movement, walking the ultrasound forward to ensure the tip remains in view (as above). Gently decrease your angle of insertion so that the superficial wall is not tenting towards the deep wall but rather into the potential space of the proximal vessel. Eventually, your tented vessel (❤️) will suddenly encompass the cannula and return to a circular shape (O). This may be associated with a tactile pop. You can check for flashback for additional confirmation of vessel puncture, but I prefer to not take my eyes off the ultrasound screen at this point.

Continue decreasing your angle of insertion to maintain the tip of the cannula in the top 50% of the vessel (keep the sharp bevel away from the deep wall). This may eventually require you be pushing the cannula into the skin, which really requires your assistant to get out of the way. Don’t lose site of your tip! Continue to step forward; cannula then ultrasound. To check whether you are in the vessel and not in soft tissue or dragging on the vessel wall, waggle the tip of the cannula around gently (left, right, up, down). There should be absolutely no distortion of the soft tissue surrounding the vessel; completely free cannula tip movement. I tend to leave the metal stylet in until the plastic catheter is fully inserted to the hub because of greater visibility and added rigidity. This does, however, carry the risk of puncturing the back or sidewall of the vessel if you don’t keep a close eye on your cannula tip. At the very least, ensure 3-4mm of the cannula is inside the vessel lumen prior to gliding the plastic catheter off (to avoid tissuing / tearing the vessel roof). Once this is done, you’ve just successfully place a real-time ultrasound-guided cannula! Well done!

I think it’s reasonable with each healthcare interaction to measure success both in the resolution of issue (beneficence) and in minimization of harm / traumatic experience (non-maleficence). Vascular access is our commonest painful procedure, hence representing a significant potential burden of pain, anxiety, and trauma. Undertaking steps to minimize vascular access attempts, maximize speed/efficiency, and maximize cannula longevity are important considerations in the healthcare interaction. Even if we manage to achieve the elusive goal of a single puncture hospital admission, this still requires a single puncture. 

This discussion is not really directed towards addressing the specifics of analgesia and sedation but suffice to say that time permitting these should be used and optimized readily. A topical anaesthetic is valuable, although in the case of an ultrasound-guided cannula application by the operator is useful in ensuring good placement. Evidence is increasingly suggesting that topical anaesthetic is appropriate in all ages including neonates.

The power of social stories, rehearsal, music therapy, and just general distraction cannot be undervalued. There is a multitude of approaches to this. 

Unfortunately, it is not an uncommon experience to be in a situation where vascular access is required with a degree of clinical urgency. In this circumstance, oral/intranasal/topical medication may have not had time to work, and a specialist in distraction may not be readily available.

In this circumstance, I have found that playing calm and quiet music more useful than positioning a video in front of a child. Maintaining a minimum of people speaking, and using quiet calm voices is valuable. I have had some success using the ultrasound itself as a distraction modality while telling the child a story of the “doughnut that has lost its hole” (vein and cannula tip respectively) as the tip tracks toward the vessel. A variant is the “star that fell from the sky into the lake” (cannula tip and vein respectively). There are many approaches to pain reduction through distraction.

It is my sincere hope that these tips are of some practical and clinical value in your cannulating endeavours. If it makes a difference for a single child, then surely it’s worth it. Good luck!

Communicating clearly

Cite this article as:
Liz Herrieven. Communicating clearly, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.32916

The Joint Royal Colleges Ambulance Liaison Committee (JRCALC) produces guidance for ambulance services across the UK. I was thrilled to be asked to contribute to this in the form of a new chapter on patients with communication difficulties. This post expands on that guidance, which was written to support pre-hospital clinicians in providing the best possible care to their patients who face challenges with communication. This may be due to a wide variety of underlying conditions, including learning disability, autism, hearing loss, dementia and dysphasia.

Communication is vital to all that we do – from the first contact with a patient, through history taking and examination, to initiating treatment and explaining procedures. We have to do our very best to get it right. This is perhaps even more important, and more difficult, in the pre-hospital field, where stress levels are high, the environment can be unpredictable and time is short. Clinicians meeting patients for the first time need to quickly assess the situation and also win trust and gain understanding.

Communication is a two-way thing. It sounds obvious, but it becomes even more important when patients find communication difficult. Not only do we have to try our best to make ourselves understood, but we also have to try our best to understand our patient.

It’s also important to remember that communication and understanding are two very different things. Someone may be able to communicate quite well but understand very little. Conversely, someone may not be able to communicate but may have a very good understanding, including things being said about or around them. Dysphasic or dysarthric patients may appear to be unable to understand when actually their difficulty is in expressing themselves.

So, how can we improve our communication?

Minimise fear and anxiety

Communicating and understanding become more challenging when there is fear and anxiety. The first step is to keep calm and reassure the patient. The specific nature of any communication difficulty needs to be recognised quickly then addressed. Patients with a learning disability may not understand what is happening so careful explanations may help. Some autistic patients may have difficulty interpreting information verbally or non-verbally, or they may have significant sensory processing difficulties which means that loud noises, bright lights and physical touch can be distressing or even painful. Deaf patients may be able to better understand if they can see the clinician’s mouth – difficult with PPE.

Make simple adjustments

Communication might be made easier with simple changes such as speaking slowly and clearly and avoiding jargon. And give time – time for your patient to respond. For some patients, including those with Down syndrome, it can take several seconds to respond – time to receive the auditory information, decode it, understand it, formulate an answer and produce that answer as the right set of noises. We’re all busy so that seven or eight seconds can feel like an age. It’s absolutely worth the wait, though.

Adapt the environment

Can we do anything to make the environment less distracting, quieter, less stimulating? Would it be better to assess the patient in familiar surroundings rather than in the ambulance? Can noisy, scary or flashing equipment be switched off, removed or covered? If the patient has to be moved can they bring something, or someone, familiar with them?

Pay attention to non-verbal communication

Would eye contact help? It often does, but for some autistic patients it can be distressing. Some people respond well to a reassuring touch (I’m a toucher and a hugger) but others find it really uncomfortable – check before extending that hand! Do we need to support our verbal communication with gesture or sign? Pictures or symbols might help to explain what we are saying, but if we don’t have any to hand then pointing to body parts or pieces of equipment can help. We absolutely need to pay attention to our non-verbal communication, body language, posture, facial expression and so on, and also watch for non-verbal cues from our patient. Those who know our patient best might be able to help with this – how would their loved one usually let someone know they were in pain, for example? Pain is often poorly assessed and managed in people with a learning disability (LeDeR – the Learning Disability Mortality Review Programme). We often hear about people having a “high pain threshold” and whilst it’s true that pain is perceived differently by different people, we can’t assume that someone does not feel pain just because they can’t verbalise it.

Play to your patient’s communication strengths

Some patients may have particular strengths and weaknesses when it comes to communication. People with Down syndrome often find it more difficult to understand and remember auditory information, due to a variety of issues including fluctuating hearing impairment and poor short term auditory memory. They may, however, find it much easier to remember and understand information presented in a visual format. Using gesture, sign language (such as Makaton), photos or symbols (such as PECS) may support the verbal information and make things much easier for both the patient and the clinician.

Family and carers can help to identify how best to communicate with the patient but consider other resources too – is there a hospital passport that can give you some clues? These are often used to list medications and past medical history, but their real beauty is in detailing likes, dislikes, behaviours associated with pain, interventions that might be difficult to tolerate, and so on. A care pathway can also give great clinical information and guide management.

Adapt your examination

Your standard examination might need to be altered a little. Give clear warnings before touching the patient, particularly if they have any visual impairment or a sensory processing disorder. Start with those parts of the examination that are less intrusive – watching and observing position, demeanour, breathing pattern, and movements can all give a huge amount of information before you even get your stethoscope out. Distraction might be useful for some patients but for others, including those who may have had previous bad experiences, it might not work. Family and carers may know how best to support your patient through the more distressing parts of the examination and any following interventions.

LeDeR has also found that early warning scores were less likely to be calculated in people with a learning disability, and they were less likely to be acted on if abnormal. There are many likely reasons behind this, including clinicians being reluctant to cause distress to their patients. Things like blood pressure or oxygen saturation measurement can be very uncomfortable, particularly for those who may not understand what is being done or who may have sensory processing difficulties. Those patients still need to be assessed and treated appropriately. If a BP or sats, or any other part of your assessment for that matter, is likely to give important information then it should be done. There may, however, need to be some thought about how best to carry it out. Explanation, communication, visual information, distraction – what will help your patient tolerate the examination?

There is a common misconception that patients with chronic health problems always have an abnormal early warning score, so what’s the point? Any score, normal or abnormal, in a previously healthy patient or not, should be taken in context with the rest of the examination. It can be helpful to know what the patient is like (behaviour, level of alertness, comfort, interaction, early warning score) when well, to help to identify how ill they may be now. Again, family and carers can give vital information about this.

Be attuned to “soft signs”

“Soft signs” can help, too. These are things that family might notice long before health professionals. They are not specific to any particular illness or disease process, but give an indication that the patient isn’t well. For example, someone might be a little paler than usual, not want to get out of bed, not want to finish their favourite meal and not want to watch their favourite TV programme. A family member would know that these things mean their loved one is not themselves, and likely to be unwell. Healthcare professionals can learn a lot by listening out for soft signs.

Beware diagnostic overshadowing

It’s really important to watch out for diagnostic overshadowing. This happens when a patient has a pre-existing diagnosis, and any new symptoms are assumed to be down to this diagnosis. For example, an autistic person might present as being quite agitated, carrying out repetitive, stereotypical movements and it might be tempting for us to assume that this is all because they have autism. However, if we do that, we may miss the fact that they are in pain or feeling unwell. Again, we have to find out more about what our patient is like when they are well, to know how ill they may be now.

All of this boils down to making reasonable adjustments, which are required by law (Equality Act 2010). We can sum it up with the TEACH mnemonic:

Time: assessing someone with communication difficulties may take more time, but that time is absolutely worth it.

Environment: pick the best environment to assess your patient in. Keep things quiet and calm, remove distractions. Keep things familiar to the patient if you can, or let them have something familiar with them.

Assume: don’t assume anything about understanding – communication aids understanding, but someone who has difficulties with communication may still have very good understanding.

Communication: how can you best communicate with your patient? How can you help them make themselves understood? Would symbols or signs help? Pictures or gesture? Writing things down?

Help: what help does your patient need? What help do you need??

None of the interventions suggested are particularly tricky or difficult, but all have the potential to make a huge difference to our patients. For those working in UK ambulance services, the JRCALC guideline chapter will hopefully help as a prompt. For others, whether pre-hospital or not, I hope this blog helps a little.

https://www.jrcalc.org.uk

https://www.bristol.ac.uk/sps/leder/

Challenges in cannulation

Cite this article as:
Vicki Currie. Challenges in cannulation, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.33103

A look at paediatric cannulation. The good, the bad and the seemingly impossible.

We have all been there – coming onto a busy shift and a child who is well known for having ‘difficult’ vascular access needs a cannula.

It can be a heart sink moment when you realise that the team from the previous shift have already tried and failed. You feel your palms begin to sweat as the nursing staff tell you that access was a huge problem on the last admission. The father of the child tells you that you can have ‘just one go’.

But what really affects the chances of success of getting that tricky cannula in? Are there any modifiable factors that make it easier or harder? And how can we feel more confident in paediatric cannulation?

What is the evidence?

There are several factors that have been shown in the literature to negatively impact the success rates of paediatric cannulation:

  • Use of previous central venous access
  • Obesity
  • Attempts in the hand and lower legs
  • Non-black / non-white race
  • Poor cooperation of the child
  • Lack of confidence prior to the procedure

A recent study by Maduemem et al looked at the ‘Challenges Faced by Non- Consultant Hospital Doctors (NCHDs) in Paediatric Peripheral Intravenous Cannulation in Ireland.’ It aimed to evaluate the level of confidence of NCHD’s and looked to identify the factors that positively or negatively impacted confidence. This is a unique piece of research that is one of the first qualitative studies looking at the level of confidence in doctors in peripheral intravenous cannulation (PIVC).

Maduemem, K., Umana, E., Adedokun, C. et al. Challenges Faced by Non-consultant Hospital Doctors in Paediatric Peripheral Intravenous Cannulation in Ireland. SN Compr. Clin. Med. 2021

The team performed a cross-sectional national survey in 12 hospitals in Ireland using paper-based questionnaires. The survey captured data on the respondents’ clinical demographics (primary speciality, number of years postgraduate experience), clinical experience with PIVC (any paediatric clinical experience, number of children cannulated in preceding three months etc), the level of confidence in paediatric PIVC and potential factors influencing confidence in PIVC.

The primary outcome was the level of confidence in cannulation, measured by a five-point Likert scale assessing the overall level of confidence with ‘agree and strongly agree’ determined as a good level of confidence. Secondary outcomes were self-rated success in PIVC, previous experience and the effect of parental presence during the procedure.

The study had 202 respondents (45% response rate). The median number of years postgraduate experience for SHO level was three years (IQR 2-4) and at registrar level seven years (IQR 5-10.5). Interestingly ALL respondents had carried out paediatric cannulation in the preceding three months with 76% performing the procedure at least 10 times during the three-month time frame.  Despite 89% of respondents rating their performance as at least average, less than half (48%) of respondents reported themselves as feeling confident with the procedure.

Only 29% of respondents were reported as feeling confident in attempting PIVC that had been unsuccessful by a colleague. 37% of the cohort felt anxious when asked to perform PIVC in children, unsurprisingly with NCHD’s below registrar level feeling more anxious than their registrar counterparts.

What was driving this anxiety? More than half of the respondents (56%) stated that nursing staff and parental presence were sources of anxiety with 52% preferring to carry out this procedure without parents present.

Specific phrases that were noted by participants to have an adverse effect on confidence before the procedure were phrases which I’m sure the majority of us have heard before:

So what can we do?

Practice, practice and more practice

The study found that levels of confidence increased with seniority so encouraging junior colleagues and supporting them to perform cannulation is key. Including sessions on simulated patient arms to practice venepuncture may be a useful adjunct for clinicians with limited previous exposure.

Think before we speak

The phrases we use prior to performing a procedure can be powerful – not just the ones we say to ourselves but those we utter to colleagues. Feeding back to colleagues that phrases were unhelpful or signposting to the above study, in a polite way, might be a good way to raise awareness of the impact such phrases can have.

We all have seen the effect a ‘fresh set of eyes’ can have on that difficult cannula. So, if you are the person attempting after a colleague has already had a go, then be confident and try to start from fresh.

What about ultrasound?

Ultrasound guidance as an adjunct to PIVC has been shown to increase the success of the first attempt with good training in the use of ultrasound a big factor in first attempt success.

This is not a mandatory or even optional skill in general paediatric training in the UK. Experience is often gained from placements in PICU, ED or time with anaesthetic colleagues. Courses are becoming more frequent . If you have the opportunity or access to learn this skill from a colleague (paediatric or adult trained) it can be extremely useful.

Vein finders (infra-red lights that magically show veins through the skin) and the cold light that can often be found on the neonatal unit (used to look for evidence of pneumothorax) can be useful adjuncts too.

Are there any scores that can predict if a child’s access is going to be difficult?

The Difficult Intravenous Access (DIVA) prediction score is based upon four variables that are proportionally weighted. The variables are: vein palpability, vein visibility, age (infants score higher) and a history of prematurity. A score > 4 equates to a 50% increase in the likelihood of failure rate with first attempt.

But if a child has a high score, what next? Some difficult access pathways have been proposed with the utilisation of ultrasound, early contact with anaesthetic colleagues to help with access and consideration of midline/ PICC/ CVC in children who are particularly difficult. In practice, highlighting children early who have factors that put them at higher risk of being difficult and early escalation to senior colleagues, limiting attempts and utilisation of some of the steps mentioned can be helpful.

Keep things calm and pain free…

Optimisation of the position of the child and parents can help to not just keep the environment a calmer place but can reduce anxieties all round. The classic ‘bear hug’ position with a parent on a chair and the child chest to chest can provide not only comfort but easy access to limbs.

The use of freeze spray or anaesthetic creams on the area you are going to attempt cannulation can help to reduce pain as well as child and parental anxiety.

The use of distraction techniques can also reduce the child’s perception of pain. Singing, a YouTube video, home video on a smartphone or even bubbles can be easily done whilst attempting cannulation.

And if despite all of this you are still unsuccessful then limit yourself to a maximum number of attempts – usual practice is two to three (two attempts usually for more junior colleagues) before you ask for additional help. This ensures that there are still some veins left for that fresh set of eyes to have a look at. It also gives the child, parent and other staff helping a break from the procedure and means you don’t become super task-focused. In a situation where the child is unwell and access just needs to be attained, this is a different matter, and you will hopefully have multiple people around with lots of sets of eyes.

PIVC in children is tough, it is a skill that takes years to get right and still people who have been doing it for years can have a bad day where they just cannot get that cannula in. Keep practising, keep smiling, think about the words you use in relation to the procedure and how they can affect others and don’t forget the bubbles!

References

Bauman M, Braude D, Crandall C. Ultrasound-guidance vs. standard technique in difficult vascular access patients by ED technicians. Am J Emerg Med. 2009;27(2):135–40.

de Negri DC, Avelar AFM, Andreoni S, et al. Predisposing factors for peripheral intravenous puncture failure in children. Rev Latam Enfermagem. 2012;20(6):1072–80.

Larsen P, Eldridge D, Brinkley J, Newton D, Goff D, Hartzog T, et al. Pediatric peripheral intravenous access: does nursing experience and competence really make a difference? J Infus Nurs. 2010;33(4):226–35.

Maduemem, K., Umana, E., Adedokun, C. et al. Challenges Faced by Non-consultant Hospital Doctors in Paediatric Peripheral Intravenous Cannulation in Ireland. SN Compr. Clin. Med. 2021. https://doi.org/10.1007/s42399-021-00881-9

Nafiu OO, Burke C, Cowan A, et al. Comparing peripheral venous access between obese and normal weight children. Pediatr Anaesthesia. 2010;20:172–6.

Petroski A, Frisch A, Joseph N, Carlson JN. Predictors of difficult pediatric intravenous access in a community emergency department. J Vasc Access. 2015;16(6):521–6.

Sou V, McManus C, Mifflin N, Frost SA, Ale J, Alexandrou E. A clinical pathway for the management of difficult venous access. BMC Nurs. 2017 Nov 17;16:64. doi: 10.1186/s12912-017-0261-z.

Vinograd AM, Chen AE, Woodford AL, Fesnak S, Gaines S, Elci OU, et al. Ultrasonographic guidance to improve first-attempt success in children with predicted difficult intravenous access in the emergency department: a randomized controlled trial. Ann Emerg Med. 2019;74(1):19–27.

Yen K, Riegert A, Gorelick MH. Derivation of the DIVA score: a clinical prediction rule for the identification of children with difficult intravenous access. Pediatr Emerg Care. 2008 Mar;24(3):143-7. doi: 10.1097/PEC.0b013e3181666f32.

Urine dipsticks

Cite this article as:
Laura Riddick. Urine dipsticks, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.32596

This post will cover what’s what on a urine dipstick and clarify what it means when “it lights up like a Christmas tree

It’s 3 am and the 4-year-old with fever has finally produced a urine sample. You dip it and it lights up “positive for everything”. You’re sure it’s positive for infection, but what if the pH is 5.5? What does it mean that there is blood and protein in it?

Leukocytes

Surely white blood cells must mean an infection is present? If you have read the NICE UTI guidelines, so you know that is not necessarily the case.

The dipstick tests for leukocyte esterase. This is an enzyme produced by neutrophils and can be a sign of a urinary tract infection (white cells in urine = pyuria). These neutrophils, however, and the enzyme they produce can also be a sign of infection outside of the body such as vulvovaginitis. They may also be found in the presence of haematuria.

The overall sensitivity for leukocyte esterase is 49 – 79% with a specificity of 79 – 87%. As a result, it can be considered to be suggestive of “possible UTI”, and “probable UTI” if found with a positive nitrite sample (specificity increased to 99%).

What does this mean/bottom line: If positive and history suggestive (i.e. dysuria or fever) consider UTI and send for culture. If negative, then it is quite unlikely that there is an infection.

Nitrites

Nitrites are the breakdown product by gram-negative organisms such as E.coli. They are a more specific test (93-98%) than leukocytes but their sensitivity is lower (47-49%). The sensitivity is particularly poor as the urine needs to sit in the bladder for a while (at least 4 hours) for it to be positive.

What does this mean/bottom line: If it is positive, it is highly suggestive of infection. If it is negative, then does not necessarily rule out infection and needs correlation with leukocytes and presentation

Blood

Blood (haematuria) can be present for many reasons, so it is important to determine if there is microscopic (dipstick only) or macroscopic (visibly bloody). If blood is seen seen with leukocytes and/or nitrites then you should consider the child to have a UTI. If blood is seen with protein, then glomerulonephritis needs to be considered as a cause.

Causes of haematuria

  • Infection
  • Fever
  • Kidney stones
  • Glomerulonephritis
  • Renal tumour
  • Exercise
  • Trauma
  • Menstruation (doesn’t cause haematuria but will show up on dipstick so don’t forget to ask)

Isolated microscopic haematuria is common and only needs investigation if persistent, but make sure a blood pressure is checked as this is an often missed test. If haematuria is persistent it may need further investigation.

Reasons for further investigation

  • Macroscopic haematuria
  • Proteinuria
  • High blood pressure
  • Clinical oedema or features of fluid overload
  • Persisting microscopic haematuria (>2 occasions over 2-4 weeks apart)

Bottom line: If just microscopic haematuria on dipstick without explanation, then request a repeat sample with GP in 2-4 weeks. Don’t forget to check a blood pressure!

Protein

The body excretes a small amount which is usually not enough to pick up on dipstick.

If the body is “stressed” in illness or infection, it can cause proteinuria, however it is also a sign of inflammation or damage within the kidney and so further history and examination is required.

When there is proteinuria of 2+ or more occurs during illness or a UTI, it can be repeated in a couple of weeks to ensure that it does not persist when the patient is well.

If there is no illness or infection, you would need to consider other causes such as glomerulonephritis and nephrotic syndrome, examine for oedema, and send off a protein:creatinine ratio sample.

Bottom line: small amounts can be seen in illness, but large amounts needs review depending on how the patient is.

Glucose

This is not usually found in the urine, but small amounts can be detected if the patient is unwell, or is on steroids. If there is a large amount of glucose, consider checking a blood glucose to rule out diabetes, and see if there is any other evidence of kidney problems.

Ketones

A by-product from the breakdown of fat when sugar stores cannot be used. These can be seen in patients who have not been eating, vomiting and in DKA. It is always worth checking the blood glucose in these patients, as its absence in hypoglycaemic patients should alert you to a potential metabolic disorder.

Bottom line: Seen during periods of vomiting or not eating. Always check a blood glucose.

Bilirubin

Excessive bilirubin that is not dealt with in the liver is excreted in urine. Thus the presence of bilirubin in the urine can be seen in conjugated hyperbilirubinaemia, and therefore a feature of liver disease. If the urine dipstick measures urobilirubin then this can be seen normally on a dipstick (normal to 1+). A high urobilirubin could suggest haemolytic disease, as it reflects unconjugated bilirubin.

Bottom line: Bilirubin – not normal. Urobilinogen – normal (in small amounts)

Specific Gravity

This measures how dilute your urine is by comparing the solubility if the urine to water. If <1.005 then the urine is very dilute – do they drink a lot of water? If not the kidney may be unable to concentrate the urine, there it would be wise to consider checking a serum sodium and assess the patient for features of diabetes insipidus.

A high specific gravity means the urine is concentrated, and suggests that the patient may be dehydrated. If they do not appear hydrated, then does the patient appear oedematous? This could suggest systemic disease

A list of causes of high specific gravity

Bottom line: compare to the patient’s hydration status

pH

The urine pH varies and is usually slightly acidic. It can be influenced by diet and medication. Usually, alkaline urine is a product of vegetarian diets and medication. It can also be present in UTIs caused by urea splitting organisms, such as Proteus and Pseudomonas. It is seen in renal tubule anomalies or if the patient has metabolic alkalosis. Urinary acidosis is seen with high protein diets and can reflect systemic acidosis (for example, DKA, diarrhoea and vomiting)

Bottom line: Not very useful on its own.

Urine dipticks infographics

Selected references

https://litfl.com/dipstick-urinalysis/

https://patient.info/treatment-medication/urine-dipstick-test

Yates A. Urinalysis: how to interpret results. Nursing Times. 2016 Jun 8;112(2):1-3.

https://geekymedics.com/urinalysis-osce-guide/

https://www.mayoclinic.org/tests-procedures/urinalysis/about/pac-20384907

https://www.nice.org.uk/guidance/cg54/chapter/Recommendations#diagnosis

https://www.clinicalguidelines.scot.nhs.uk/nhsggc-paediatric-clinical-guidelines/nhsggc-guidelines/emergency-medicine/haematuria-management-and-investigation-in-paediatrics/

Fernandes DJ, Jaidev M, Castelino DN. Utility of dipstick test (nitrite and leukocyte esterase) and microscopic analysis of urine when compared to culture in the diagnosis of urinary tract infection in children. Int J Contemp Pediatr 2017;5:156-60

Jeng-Daw Tsai, Chun-Chen Lin, Stephan S. Yang, Diagnosis of pediatric urinary tract infections, Urological Science, Volume 27, Issue 3, 2016, Pages 131-134

Tsai JD, Lin CC, Yang SS. Diagnosis of pediatric urinary tract infections. Urological Science. 2016 Sep 1;27(3):131-4.

Febrile Infection-Related Epilepsy Syndrome (FIRES)

Cite this article as:
Jessica Archibald and Catherine Murphy. Febrile Infection-Related Epilepsy Syndrome (FIRES), Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.32716

An 8-year-old presents to the emergency department following a first seizure episode. They had a witnessed generalised tonic-clonic seizure that morning lasting approximately 60 seconds and remain post-ictal. They have a history of being non-specifically unwell yesterday with subjective fever, lethargy and a mild headache. They have no significant past medical history and no family history of seizures. The examination is unremarkable. Whilst in the emergency department they have a further two self-terminating generalised tonic-clonic seizures.

Febrile Infection-Related Epilepsy Syndrome (FIRES) is a rare epileptic encephalopathy that results in prolonged refractory status epilepticus in previously well patients.

Presenting Features

FIRES typically presents in children between the age of 3 to 15 years, with intractable status epilepticus, 2 to 10 days post a febrile illness. The preceding illness is most commonly an upper respiratory tract infection or gastroenteritis. Fevers may have resolved prior to the onset of the acute phase of the condition.

The acute phase of the illness is characterised by frequent seizures, rapidly progressing to status epilepticus. Although the seizures are initially focal in nature, they may evolve into secondary generalised seizures. The acute phase can be prolonged, lasting from weeks to months. An association with rash, liver derangement and arrhythmia has been noted in the literature. There is no latency period.

The chronic phase is denoted by refractory epilepsy, resulting in seizures that may cluster every 2 to 4 weeks. This is often associated with severe neurological impairment and cognitive decline.

FIRES had previously been thought to only occur in children, and New-Onset Refractory Status Epilepticus (NORSE) only in adults, however this theory has been disproven. Although FIRES is more prevalent in children, it has been known to also occur in adults. As such, FIRES is now considered a subtype of NORSE, characterised by a preceding febrile illness. It has previously been known as Acute Encephalitis with Refractory, Bepetitive Partial Seizures (AERRPS) and Devastating Epilepsy in School-age Children (DESC).

Aetiology

The aetiology of FIRES in unknown and as such the pathophysiology remains unclear.

One theory is that FIRES is a form of severe infectious encephalitis, but as yet no infectious agent has been identified, and the refractory nature of the seizures is atypical of encephalitis. Another hypothesis suggests FIRES is the result of an immune response, however, there is not enough evidence to support this theory.

A case identifying anti-GABA A receptor antibodies in the CSF of a patient who presented with severe refractory status epilepticus associated with a fever led to speculation that the condition may be autoimmune-mediated. Again this has not been proven and the case may have been an exception rather than a rule.

Other theories include genetic associations and potential links with metabolic disease, but as yet a cause has not been identified.

Diagnosis and Differentials

The diagnosis of FIRES is essentially clinical, as FIRES is a cryptogenic illness. The work up is initially general, and focused on the exclusion of other treatable causes, such as infectious or autoimmune encephalitis.

A detailed history will identify the preceding febrile illness, and would be focussed on the identification of risk factors for other causes for the presentation, including exposure to animals, drugs and toxins, recent foreign travel and immunosuppression.

Blood sampling will be used to identify an infectious cause for the presentation, through full blood count, blood cultures and a screen for atypical infective agents. Lumbar puncture should be performed for CSF sampling in order to investigate bacterial, viral, fungal or autoimmune causes. CSF may show a mildly elevated white cell count in those with FIRES.

EEGs may show a generalised slowing, in keeping with an encephalopathic picture, but do little in the way of distinguishing between other causes of seizures. However, they are useful in guiding treatment and identifying non-convulsive seizures.

Initial MRI imaging is often normal, however, follow up imaging has been associated with devastating changes. Early MRI, in the first weeks of the acute illness, has shown swelling of the mesial temporal structures and increased T2 weighted signal. Follow up MRI, greater than 6 months after onset, may be associated with bilateral mesial temporal atrophy and increased T2 weighted signal. It should be noted that MRI may be normal in 50% of cases.

Differentials to consider are Dravet Syndrome, which presents with a febrile illness associated with status epilepticus, though this tends to present within the first year of life. Also Alper’s Disease, which presents with refractory seizures in previously well children, and is often associated with liver disease.

The patient is loaded with levetiracetam (40mg/kg) as per hospital guidelines, and admitted under paediatrics locally. A CT head is unremarkable and bloods show mild LFT derangement with normal inflammatory markers. They are treated empirically with intravenous cefotaxime and aciclovir. Later that afternoon they develop a fever of 38.3.

The GCS fluctuates between 11 to 13 with no full recovery to baseline until later that evening. Following two focal seizures the next afternoon, they are transferred to the local tertiary centre for further investigation and management.

Initial Management

Initial management involves treating the seizure, and more often status epilepticus. Local hospitals have their own guideline for managing status epilepticus but the first line is typically benzodiazepines (lorazepam, diazepam, midazolam, clonazepam). Second-line treatment is standard anti-convulsants (levetiracetam, phenytoin, phenobarbitone, sodium valproate), however, FIRES does not typically respond to these medications even in high doses.


The seizure pattern in FIRES is often resistant to multiple anti-epileptics. Alternative treatment options have to be sought although there is limited evidence as to the optimal treatment.

Long-term Management

There is limited data on the treatment of FIRES, however, they all conclude the seizures are very difficult to manage and often require polytherapy. Some of the alternative treatment options include drug-induced burst-suppression comas, immunotherapy, a ketogenic diet, vagus nerve stimulation, therapeutic hypothermia and intravenous magnesium sulfate. The most commonly used and researched options are discussed below.

Burst suppression coma

Burst suppression coma induction is viewed as standard care for refractory status epilepticus. If first and second-line treatments fail the next option involves high doses of anti-convulsants along with anaesthetic agents, for example, an infusion of midazolam, barbiturates or propofol. Unfortunately when the anti-convulsants are weaned the seizures tend to reoccur. Prolonged burst suppression coma has been associated with a significantly worse cognitive outcome and poorer prognosis.

Immunotherapy

Immunotherapy has been trialled due to the suspected role of inflammation in the pathogenesis of FIRES. High dose steroids, intravenous immunoglobulin and plasmapheresis have all been used. There is limited evidence to suggest a beneficial role in the management of refractory epilepsy. A large-scale Japanese study described 2 out of 12 patients responding to steroids, although there is not enough evidence to support this as a treatment option. Treatment with immunotherapy is often associated with significant side effects

Anakinra is a recombinant and modified human interleukin-1 receptor antagonist protein. Recent evidence has shown it to be an effective and promising treatment option in patients with FIRES, though relapse has been reported after withdrawal. It has been shown to decrease the duration of mechanical ventilation and hospital length of stay, and possibly seizure reduction. Future studies are required to understand the optimum dosing regime and safety of anakinra.

Ketogenic diet

A ketogenic diet is a high fat, adequate protein and low carbohydrate diet aimed at imitating the body’s fasting state. The body, therefore, metabolises fat for energy. The early introduction of the ketogenic diet has shown to be beneficial in the management of FIRES in uncontrolled trials. It has been suggested that the ketogenic diet may have an anti-inflammatory, as well as an anti-convulsant effect. Some reports suggest it may also have a positive effect on long term cognition. Currently, it is one of the only management options shown to be effective. Future controlled studies are needed to prove this efficacy.

Vagus nerve stimulation

Vagus nerve stimulation (VNS) involves the implantation of an electrode that produces intermittent electrical stimulation into the left cervical vagus nerve. Case reports have found benefit from VNS in the cessation of seizures in patients with refractory status epilepticus and NORSE. There is limited evidence of its use in FIRES.

Long term effects

The prognosis of FIRES is poor. The outcome varies with the length of the acute phase with mortality rates up to 30%. Of those patients who survive there is 66-100% chance that they will have long term cognitive impairment due to damage of the frontal and temporal lobe functions. Survivors with a normal cognitive function will present with a spectrum of learning disabilities, behavioural disorders, memory issues and sensory changes. There is a high risk of recurrent status epilepticus. Unfortunately, only a small proportion of survivors will have no neurologic sequelae.

The patient required a lengthy PICU admission where they were managed with a burst suppression coma, ketogenic diet, high dose steroids and intravenous immunoglobulin.

They were later diagnosed with Febrile-Infection Related Epilepsy Syndrome after extensive investigations, including a normal brain MRI and a lumbar puncture which showed a mildly elevated white cell count but was otherwise unremarkable.

They are currently seizure free on a combination of oral phenobarbitone, perampanel and levetiracetam but have some cognitive sequelae.

References

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