Book review

Love, Learning Disabilities and Pockets of Brilliance by Sara Ryan

Cite this article as:
Liz Herrieven. Love, Learning Disabilities and Pockets of Brilliance by Sara Ryan, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.33746

I’d been looking forward to reading this book and it absolutely did not disappoint. Professor Ryan is a social scientist whose son, Connor, had autism, learning disabilities and epilepsy. He died in 2013 whilst an inpatient at a specialist NHS assessment and treatment centre. Her campaign for justice, #JusticeforLB (Connor was known as Laughing Boy), raised awareness and eyebrows around the inequalities and injustices faced by people with learning disability or autism.

Cover of book

“…It is back to parenting without a map and with some of the (few) signposts kicked over in the ditch or pointing the wrong way.”

This book describes some of the issues faced by people with a learning disability (LD) or autism and their families, from birth to end-of-life. It describes the horrendous moments in history that are still all too familiar to us as lived experience today. Yet, it also describes some of the very meaningful and wonderful acts that can make such a positive difference. Some magnificent quotes from people with LD and their families remind the reader that this is not an abstract text, but an account of real lives. The good, the bad, the heartbreaking and the brilliant are shown, along with some great myth-busting.

Some of the pockets of brilliance may surprise readers. They are fairly mundane or, at least, expected as a good standard of care. The fact that parent-carers describe them as brilliant speaks volumes about the inequalities we face on a daily basis. One example is particularly moving – a speech and language therapist writres a letter to parents with a comment detailing what a pleasure it was to work with their son.

“Out-of-hours home visits, alternatives to the main waiting room, positioning on the consultation list, additional time, talking carefully through what was going to happen, all contributed to positive encounters.”

Ryan challenges readers to consider a viewpoint a little different to the one commonly taken – disability as a social construct, with services set up to mould individuals to fit societal expectations, rather than communities adjusting to accommodate all individuals whatever their needs. A focus on deficit fails to highlight people’s abilities. Society suffers from ‘inclusion phobia‘ and is disablist, often due to ignorance. The first step to overcoming this, she suggests, is recognising that we are all human.

“Parents are constantly trying to prevent the downgrading of children to bundles of burden and toil rather than fully fledged little people.”

She writes with authority, vulnerability and humour. This book made me smile, nod in recognition and, at times, cry. As Mum to a young lady with Down syndrome and autism there was much that resonated – both good experiences and bad. As a clinician, there was also much for me to learn and build into my practice. I particularly liked the “What can you do?” sections, and would have liked more of these although, to be fair, the whole book acts as a guide to professionals in health and social care, whether they regularly work with people with LD or autism, or not. There are many stark truths in this book, which professionals would do well to acknowledge. We need to do better and Ryan not only explains why, but also gives us tools to start on the “how”.

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.

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.

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/

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.

Following bronchiolitis guidelines

Cite this article as:
Ben Lawton. Following bronchiolitis guidelines, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.32798

In 2016 our friends at PREDICT produced a robust, evidence-based guideline for the management of bronchiolitis. They assembled a diverse team of experts, decided on the key questions we ask ourselves when managing babies with bronchiolitis and then did a deep dive of the literature to provide answers to those questions. You can read the guideline here, or the DFTB summary here but the key messages will be familiar to regular readers of DFTB. The list of things that do not help babies under 12 months with bronchiolitis includes salbutamol, chest x-rays, antibiotics, nebulised adrenaline and steroids. In the real world, however, these ineffective treatments continue to be used – so what can we do about that? 

The authors of a new PREDICT study released in JAMA Pediatrics on 12 April 2021 sought to demonstrate whether a group of interventions they developed using theories of behaviour change would be effective in reducing the number of ineffective interventions given to bronchiolitic babies. 

Haskell L, Tavender EJ, Wilson CL, et al. Effectiveness of Targeted Interventions on Treatment of Infants With Bronchiolitis: A Randomized Clinical Trial. JAMA Pediatr. Published online April 12, 2021. doi:10.1001/jamapediatrics.2021.0295

Who did they study? 

This was an international multicentre cluster randomised controlled trial (RCT) involving 26 hospitals in Australia and New Zealand. It is described as a “cluster” RCT as randomisation was by hospital rather than by patient. The randomisation was a bit complicated. It was stratified to make sure secondary and tertiary hospitals from each country were represented in each group. Baseline data was collected from 8003 patient records from the three bronchiolitis seasons prior to the start of the intervention period. A further 3727 charts analysed from the season in which the intervention took place. The data from the three prior seasons were used to ensure baseline similarity between groups and to establish patterns of practice change that were already occurring. In short, this was a big study that ensured representation of both specialist children’s hospitals and mixed general hospitals. 

What did they do? 

Hospitals randomised to the intervention group received a package of interventions based on the Theoretical Domains Framework (TDF), developed following an earlier qualitative study that investigated why we do what we do when managing bronchiolitis infants. The TDF is one of the most commonly used frameworks in implementation science and is considered particularly good at identifying interventions to address barriers and facilitators that influence behaviour change. The package included:

  • Appointing clinical leads from medical and nursing streams in both emergency departments and inpatient paediatric units.
  • The study team meeting with those clinical leads to explore the local practice and any anticipated barriers to change.
  • A one day train-the trainer workshop to ensure clinical leads were comfortable using the educational materials provided to train local staff.
  • An education pack including a PowerPoint with scripted messages specifically designed to promote change, a clinician training video, evidence fact sheets, promotional materials and parent/caregiver information sheets.
  • Monthly audits of the first 20 bronchiolitis patients with the results shared and compared to the best performing hospital.

What about the control group?

Hospitals randomised to the control group were just left to their own devices for the year of the intervention period. They had access to the guidelines and were welcome to share that information as they would in any other circumstances. The intervention package was made available to control hospitals in the season following the study period. 

What did they show? 

The primary outcome was the proportion of infants who complied with all five of the Australasian Bronchiolitis Guideline recommendations known to have no benefit (chest x-ray, salbutamol, steroids, adrenaline, antibiotics). There was an 85.1% compliance rate in the intervention group compared to a 73% compliance rate in the control group. In other words, in hospitals that were part of the intervention group, an average of 85.1% of kids received care in line with the guidelines, compared to only 73% receiving guideline compliant care in control hospitals. This was a significant difference.

Secondary outcomes showed improvement was consistent in both the ED and inpatient phases of care. Unsurprisingly, there was no difference in hospital length of stay or admission rates to ICU. 

The DFTB verdict

On the surface this is a robust, well designed study showing that if we put some thought and some resources into supporting our colleagues in doing the right thing then babies with bronchiolitis will get better care in our hospitals. They won’t leave hospital any quicker and they won’t have a lesser chance of needing ICU but they will be exposed to fewer interventions that will not do them any good and may do them some harm. Dig a little deeper though and the big messages in this paper go way beyond the management of bronchiolitis. The implementation science based interventions used in this study can be adapted to anything, and though they have been shown to be effective in getting us to do the right thing here, we haven’t shown that their efficiency has been optimised yet. Great breakthroughs in novel medical science are exciting but there are huge improvements in care to be gained through getting the best care that we do know about to every patient every time. This paper should serve as fuel for the fires lighting implementation science’s journey from the shadows to the centre stage of improvement in clinical care. 

From the authors

The study’s senior author, Prof Stuart Dalziel gave DFTB the following take: 

“The key finding is that we can do better. By using targeted interventions, based on established behaviour change theories and developed from work looking at why clinicians manage patients with bronchiolitis the way they do, we can improve the management of patients with bronchiolitis such that it is more consistent with evidence based guidelines.

In the field of implementation science (IS) and knowledge translation (KT) a 14% improvement in care is a large change.

Changing clinician behaviour is complicated, this is especially so for de-implementation of medical interventions. Many factors influence clinician behaviour and it is thus perhaps naïve to think that a single intervention can cause a significant change to behaviour. For a number of decades the majority of clinical guidelines for bronchiolitis have emphasised that chest x-ray, antibiotics, epinephrine, corticosteroids and salbutamol are low-value care and not evidence based. Yet despite this consistent messaging from guidelines the use of these interventions has remained considerably higher than what it should be. While the interventions delivered in our study were not unique (site based clinical leads, stake holder meetings, train-the-trainer workshops, targeted clinical education, educational material, and audit and feedback) they were specifically developed, using an established framework for behavioural change, following a qualitative study that determined why clinicians managed bronchiolitis they way they do. This prior study, addressing the barriers and enablers to evidence based care, and the subsequent step wise approach to developing the targeted interventions that we used was critical in achieving the change in clinician behaviour observed in our randomised controlled trial”.

The study’s lead author, Libby Haskell, stated:

“Bronchiolitis is the most common reason for children less than one year of age to be admitted to hospital. We can improve the care of these infants, such that they are receiving less low-value care. In order to de-implement low-value care we need to first understand barriers and enablers of care, and then develop targeted interventions, built on robust behavioural change models, to address these. This approach can be used to improve care for other high volume conditions where we see considerable clinical variation in care and with clearly established clinical guidelines on appropriate management.”

Let us know what you think in the comments below 

Period Problems: Menorrhagia

Cite this article as:
Tara George. Period Problems: Menorrhagia, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.32371

Period problems in teens are common. They can cause significant disruption and distress to adolescents and their parents but rarely have a significant or medically worrying underlying cause. In this first of a series of articles I’ll try to provide a logical and systematic approach to assessing and managing period problems in teenagers (recognizing that this may often be able to be extrapolated to adults too for those of you who do not only see children).

Eloise is 14. She attends with her father complaining she is tired all the time.  When she saw her GP last, they arranged some blood tests – a FBC, haematinics, TFTs and coeliac screen.  The notes from the previous consultation are very sparse. It appears that mood (normal) and bowel habit (also normal) were discussed. Eloise’s dad had mentioned she eats a broad range of foods and is not vegetarian or vegan and she eats red meat 2-3 times a week. Her periods were not brought up by the last doctor. One of her aunts has coeliac disease is noted and that is why the GP had organised bloods. Eloise has come in today for her results.

Blood results showing iron deficiency anaemia

Iron deficiency anaemia (IDA) is common in young women. Paediatricians may be much more comfortable assessing dietary intake and encouraging iron supplementation or increasing iron in the diet than they are in talking about periods. 20-30% of all cases of IDA are caused by menorrhagia. Both NICE and the British Society of Gastroenterology advocate a trial of iron for menstruating females with iron deficiency, as long as coeliac disease has been ruled out and there are no red flags for cancer. Prescribing iron and advising Eloise to “eat more steak” isn’t going to address WHY she might have IDA. This could mean that she ends up on long term iron supplements unnecessarily. If she has menorrhagia significant enough to cause anaemia, it is likely to be having an impact on her education and her social life.

Approaching the subject is probably easier than you think, remembering if you are embarrassed the patient may well think there’s something to be embarrassed about”.

So let’s talk about periods….

First a little bit of nomenclature revision.

Menorrhagia – heavy periods

Dysmenorrhoea – painful periods

Oligomenorrhoea – scanty/sparse/irregular periods

Amenorrhoea – absence of periods (primary: failure to attain menarche by the age of 15 with the development of normal secondary sexual characteristics or failure to attain menarche by 13 with no development of secondary sexual characteristics. Secondary: cessation of menstruation for 3-6 months in someone who has previously had regular periods)

Intermenstrual bleeding (sometimes called metrorrhagia) – irregular and unscheduled bleeding including unexpected bleeding between periods

Menarche – the onset of menstruation (the last stage of female puberty)

The symptoms of problematic periods are not always found in isolation. Menorrhagia and dysmenorrhoea are very common and frequently coexist. It is not uncommon for periods to be irregular, painful and heavy especially in the first few months after menarche. In the UK, the average age of menarche is 12.9 years. The average girl will be in Year 8 at secondary school when she starts her periods. Most women will menstruate every 28 days, though irregular and prolonged cycles are common in early menstrual life.

The average period lasts for 2-7 days and on average 80ml of blood will be lost during the period. In developed countries a number of sanitary products are available. The majority of girls are likely to start off with disposable sanitary towels, though environmental concerns mean period pants and washable pads are gaining popularity. Tampons are often the easiest option for girls who do a lot of sport, especially swimming, and can be used from the onset of menstruation. Menstrual cups have a much greater capacity but can be tricky to get the hang of especially for young teenagers.

Absorbency of different products

Absorbency of sanitary products for menorrhagia

What to ask in a history of menorrhagia

Start with an open question (recognising that lots of teenagers are much more comfortable with closed questions and giving specific answers): 

Tell me about your periods…

If you need to be more specific:

  • On average, how long do your periods last for?
  • How often do your periods happen?
  • Do you think they are heavy?
  • Does the bleeding change over the course of the period?
  • How often do you have to change your sanitary protection?
  • What sort of sanitary products do you use? (Pads or towels? Tampons? Period pants? Other?)
  • When did you start your periods?
  • Do you leak though your tampons/pads? If so, how often?
  • Do you pass clots? If so, how big are they?
  • How often do you need to change your pads/tampon at night?
  • Do you have to change your sheets/pyjamas?
  • Can you manage your period at school? How often do you need to leave lessons to change your sanitary product? Do you ever stay home from school because the bleeding is too heavy?
  • Are there activities you enjoy that you’ve had to stop doing because of your periods?

Eloise looks embarrassed and keeps looking at her dad. He is staring firmly at the floor looking as if he wishes it would open up and swallow him. You ask her if she would prefer to talk to you without her dad there and she nods. He takes his newspaper to the waiting area and you reassure him you’ll come and find him in a few minutes. 

Eloise tells you she started her periods at 11. They last 5-6 days on average and she has one around every 30-32 days. She uses tampons backed up with period pants as she often leaks. She uses SuperPlus tampons and on the first couple of days needs to change them every 45 minutes or so. This can be very difficult at school. She passes clots the size of grapes for a day or so each month. She has to set an alarm at night to wake her to change her protection every 2 hours but can end up with bloodstained sheets. She has stopped gymnastics and now only swims socially but not competitively. She was dropped from the squad because she wasn’t comfortable training when she had her period – the other girls had laughed when she had leaked during training. Worse still, when at a gala with lots of other teams, blood poured down her leg and she had been jeered by the crowd. She thinks her periods are heavy (heavier than all her friends) but her mum has told her this is normal and to stop making a fuss.

Whilst there is no truly objective “test” for menorrhagia, with this history and the marked iron deficiency anaemia, it is pretty straightforward to assume Eloise has menorrhagia. This is likely to be the cause for her IDA as well as affecting her sport participation, her sleep and her schooling. She had normal thyroid function tests (TFTs) as part of her tiredness workup (though it is worth noting that NICE do not recommend checking TFTs routinely in cases of simple menorrhagia). You might want to ask about other bleeding history like epistaxis, bleeding after dental extraction, family history and to consider testing for von Willebrand’s disease. NICE recommend this is for patients who have had menorrhagia from the start of their menstrual life. Most cases of menorrhagia at this age are, however, idiopathic.

Other factors to consider in your assessment

It is so important that Eloise feels listened to and heard. Her perspective is vital for compliance with any plan you make. You’ve already asked her if she thinks her periods are heavy. Now is a good time to continue to explore her ICE (“ideas, concerns and expectations”) by finding out how worried she is about her periods, whether she thinks they are a problem and if she has any ideas for what might be available to fix the problem.

Family history and past medical history are relevant here too in terms of management options as you might well want to consider the combined pill or tranexamic acid both of which are contraindicated if there is a first degree relative family history of venous thromboembolism or a known prothombotic mutation and the COCP is contraindicated if she has focal migraine. It is important to take into account the thoughts and feelings of Eloise’s parent as well during this assessment but remembering that at aged 14 she is likely to have capacity to make decisions some about her own care and be fully involved in the process.

Management of menorrhagia

The NICE guidelines on heavy menstrual bleeding contain a useful interactive flowchart for managing menorrhagia. The first line according to NICE is a levnorgestrel IUS (e.g. Mirena) but this is not always going to be the best tolerated or most suitable in a young teenager. Pragmatically in teenagers we are much more likely to opt for the second line options of tranexamic acid +/- NSAID or the combined pill.

Tranexamic acid (TXA) may be familiar to people who work in haematology or with major trauma patients as an antifibrinolytic. It is licensed for menorrhagia management to be taken as 1g three times daily for up to four days starting on the first day of the period. There are few contraindications but it cannot be taken if there is a history of VTE and should be used with caution if the patient is on the COCP because both increase thrombotic effect. TXA will reduce menstrual blood loss by up to 50%.

NSAIDs for managing menorrhagia often causes confusion as surely they make people bleed don’t they? It’s worth going back to basic pharmacophysiology and revising how NSAIDs act on prostaglandins.  NSAIDs are cyclo-oxygenase inhibitors and cyclo-oxygenase is the enzyme involved in production of prostaglandins. In menorrhagia most women will have increased levels of prostaglandins which, as you might remember, are powerful vasodilators. The local effect of prostaglandin on endometrial blood vessels causes increased bleeding. By reducing the level of prostaglandins using oral NSAIDs the blood loss volume will be reduced by up to 40%. NSAIDs will also have a significant effect on dysmenorrhoea which will frequently coexist with menorrhagia.

The COCP is frequently prescribed for menorrhagia. It is important to be familiar with the UKMEC guidelines when prescribing the COCP. Whilst the licensed regimen for COCP is to take for 21 days with a seven day break, the RCOG FSRH and most menorrhagia guidelines recommend using extended or tailored regimens. This allows for shorter pill free intervals and reduced numbers of bleeding days. Tailored regimens are associated with less frequent bleeds, and in many cases a reduced number of bleeding days.  Satisfaction with tailored regimens is high. 

Eloise seems delighted that you think her periods might not be something she simply has to “put up with”. As she isn’t sure about her family history you call dad back in and he confirms that he knows of no family history of clotting or bleeding disorders. Eloise has had several dental extractions for orthodontic work and has never bled much after these and has never had epistaxis. Eloise has never had a migraine. Her blood pressure and BMI are normal and after discussions of options you prescribe her the levest COCP using an extended tricyling regimen with a five day break after 63 pills to minimise the number of bleeds she experiences and the volume. You also prescribe oral iron and arrange a repeat haemoglobin and ferritin in 3 months, with follow up consultation in four months time.

Selected references

Heavy menstrual bleeding: assessment and management (2018, updated 2020) NICE guideline NG88

Goddard, A.F., James, M.W., McIntyre, A.S. and Scott, B.B., 2011. Guidelines for the management of iron deficiency anaemia. Gut60(10), pp.1309-1316.

Lethaby A, Augood C, Duckitt K. Nonsteroidal anti-inflammatory drugs for heavy menstrual bleeding. Cochrane Database Syst Rev. 2000;(2):CD000400. 

Nash, Z., Thwaites, A. and Davies, M., 2020. Tailored regimens for combined hormonal contraceptives. BMJ368.

Who’s who in children’s services?

Cite this article as:
Tara George. Who’s who in children’s services?, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.31749

One problem we often encounter working with children and young people, especially those with complex health or social care needs, is trying to work out who the myriad of professionals referred to actually are. More importantly, who is actually involved and who should be involved? In the context of safeguarding the concept of the “Team Around the Family” or “TAF” is key in building connections and providing stability and safety for vulnerable youngsters.

A really important point to remember is that many children and young people don’t live in a traditional nuclear family unit consisting of a mother, father and siblings.  As part of an assessment, it is vital to ask about who is at home but not to make any assumptions about how household members are related (or not). Don’t assume every child lives with their parents or that they have a female and a male parent.

Interagency work is full of buzzwords which may well feel alien to doctors new to paediatrics or to the NHS. The aim of this article is to provide you with a sense of “who’s who?” with a glossary of some relevant and important terms. The author of this article is a GP working in the UK and the terms used here are relevant in the UK though there are almost certainly equivalent professionals internationally.

Universal services (sometimes called Tier 1 services) are those services that are provided to or are routinely available to, all children, young people and their families.

Targeted services (often called Tier 2) for children and families beginning to experience – or at risk of – difficulties, for example, school counselling, parenting programmes, support for teenage parents and so on. 

Specialist services are for children and families with multiple and complex needs. They are usually referred to in two tiers:


Tier 3 services such as intensive family support, specialist child and adolescent mental health services, and services for children with disabilities. 


Tier 4 specialist services for children and families with severe and complex needs, including child protection services, inpatient child and adolescent mental health services.

In England, the process of commissioning of services is a complex one and beyond the scope of this article. Some services which appear to be “health” are in fact commissioned by local councils rather than by the CCG. Whilst it is good practice for health, education and social care to work closely together, the links are often not as effective as we would like them to be.

The following professionals are likely to be ones which anyone working with children and young people need to know about. Some of the people in this list are well known to all. Others might be new to you. If I’ve missed any off, please let me know, especially if you are reading this as a fellow professional who I’ve forgotten.

Universal services

Community Health Universal Professionals

GP Practice– everyone living in the UK is entitled to be registered with an NHS GP practice.  A number of people who work within the surgery are part of Universal Services for every child.  The UK GMS contract is a complicated subject well beyond the scope of this article but if you are interested in finding out more, the website of your local LMC (local medical committee) would be an excellent place to start. Every GP surgery will have a safeguarding lead GP and almost every locality will have a named safeguarding GP.

GP– following medical school and foundation training, junior doctors can be appointed to a three year GP speciality training programme. To gain a CCT to allow practice independently as a GP doctors must pass the MRCGP examination as well as three years of workplace based assessments. Many GPs will have specific area of interest like child health, gynaecology, musculoskeletal care but all GPs are qualified to assess and treat children. GPs are the community first point of contact for illnesses, developmental concerns and can refer to secondary care if necessary. 

Practice Nurse– Practice Nurses undertake a diploma in primary care nursing following their undergraduate nursing degree. They see all children for their immunisations and are involved in managing children with long term conditions such as asthma. In most practices, nurses will be involved in contraceptive services provision for young people as well as adults. Nurses are regulated by the Nursing and Midwifery Council (NMC)

Midwife – Midwives are registered health professionals and the majority are educated to degree level.  They have a statutory duty to children and families up to 10 days postnatally. They take the newborn blood spot test (“heel prick” formerly called the Guthrie test) at day five. Midwives are regulated by the Nursing and Midwifery Council (NMC)

Health Visitor – Health visitors are registered nurses/midwives who have additional training in community public health nursing. Health Visitors take over from the midwife at day 10 though most will have arranged to meet the family during the last few weeks of pregnancy.  They are the first point of contact for families with questions around feeding and development. Health Visitors are regulated by the Nursing and Midwifery Council (NMC)

School Nurse – School nurses are qualified and registered nurses many of whom have chosen to gain additional experience, training and qualifications to become specialist community public health nurses (SCPHN – SN). School nurses work across education and health, providing a link between school, home and the community. They work with families and young people from five to nineteen and are usually linked to a school or group of schools. School nurses are usually the first point of contact for supporting teachers and children in school with minor health or developmental problems.  In most areas they run clinics for primary nocturnal enuresis. School Nurses are regulated by the Nursing and Midwifery Council (NMC)

Optometrist – Previously known as opticians, optometrists are trained to examine the eyes to detect defects in vision, signs of injury, ocular diseases or abnormality. They assess eye health, offer clinical advice, prescribe spectacles or contact lenses and refer patients for further treatment, when necessary. Optometrists study at university for at least three years and are registered with the General Optical Council.  Children should start seeing an optician regularly from around aged 4 – their parents need to register them with an optometrist for NHS care. Eye assessments and glasses are free of charge for children in the UK. Children have a one-off vision screening run by the school health service during their reception class year.

Dentist – Dentists train for five years at University and then have two supervised years of practice. They are regulated by the General Dental Council. Children are entitled to free dental check-ups on the NHS and in most areas are offered an appointment twice a year from aged 2. Parents need actively to register their child with a dentist.

Education Universal Services

School – Statutory school age in the UK is the term after a child’s fifth birthday, though in England and Wales children become eligible for a full-time reception class place in the September following their fourth birthday.  Most families send their children to a pre-school or school nursery from aged 3 when they get 30 hours a week in term time of funded early years education. It is compulsory to be in education or training until aged 18, though “post 16 education” may be vocational/apprenticeship based rather than academic.

In the UK we have state schools which are open to all children free of charge although there are rules around distance and catchment areas. Private schools  (confusingly sometimes referred to as public schools) are fee paying schools which usually have entrance examinations and academic requirements to remain in the school. There are also special schools which provide tailored specific education for children and young people with significant special educational needs who would not be able to access education within a standard state school.  All state schools must have a designated safeguarding lead who is usually the head or deputy head. There must also be a lead for anti-bullying and a lead for looked after children who may be the same person as the safeguarding lead, or the SENDCO or another senior leader.

Teacher – Teachers in the UK are all graduate professionals. They may work in a primary (4-11 years) or a secondary school (11 years upwards).  Teachers are regulated by the General Teaching Council. Teachers spend many hours outside the classroom at evenings and weekends working on education and pastoral duties. 

Teaching Assistant (TA) – Teaching assistants work closely with teachers to support the delivery of education. They are qualified to at least an NVQ 3 but many are qualified teachers who chose to work in a lower paid but less demanding role. A TA may have a specific role working with children with special needs, or may be a general classroom TA.

SENDCO– All state schools must have a SENDCO (special educational needs and disability coordinator). They are almost always an experienced teacher who takes on this role in addition to their classroom duties. In Scotland the term SENDCO is not used, instead they have a principal teaching for additional support for learning.

More Intensive Services

Secondary Care Children’s Services

Child Health teams are often based together in hospital locations though many members may work out in the community too.

Paediatricians are specialist doctors who work exclusively with children and young people (usually up to the age of 18 though this may vary in different places). Paediatricians may be general paediatricians who had a wide-ranging interest and expertise or may have subspecialised into for example paediatric nephrology, cardiology, rheumatology etc. All paediatric departments will have a named doctor for safeguarding children and usually a named nurse for safeguarding too.  In larger hospitals there will be specialist doctors in Paediatric Emergency Medicine working in the emergency department, whereas in smaller District General Hospitals all doctors in the ED will be expected to assess and treat children acutely. Paediatric Surgeons look after children with surgical problems but again in a DGH it may well be that general surgeons operate on paediatric cases, with younger children transferred to specialist paediatric surgical units.

Paediatric Specialist Nurses are qualified nurses with additional qualifications and skills in specific disease areas. It is common to have specialist paediatric nurses looking after a caseload of children with long term conditions e.g. children’s epilepsy specialist nurses, children’s diabetes nurse, Cystic Fibrosis Specialist Nurse. Specialist Nurses may also have additional qualifications allowing them to prescribe. 

Community Paediatricians see children for a wide variety of reasons. It may be to assess general medical problems, specific developmental problems (such as ADHD or autism), learning difficulties (if a medical or neurodevelopmental cause is suspected), complex disabilities, or sensory impairments such as visual difficulties or hearing loss. Community Paediatricians do not generally deal with one-off, short-term or acute illnesses. They generally offer long-term support, co-ordination of services and management on a continuous basis. Much of the work of a community paediatrician is of a statutory nature, carried out under the Children Act, the Education Act and adoption regulations. The team has responsibility for preparing medical advice for education health care plans. The team also sees children who are being adopted or are in foster care

Community Paediatric Nurses generally work with children with long-term conditions; children with disabilities and complex conditions including those requiring continuing care and neonates; children with life-limiting and life-threatening illness, including those requiring palliative and end-of-life care.  Commonly they may be involved with tracheostomy care, complex wound management, home ventilation.  Some Paediatric Specialist Nurses are based within children’s community nursing teams rather than at a hospital base, this varies by area.

Play Specialists use play as a therapeutic tool to help children understand their illness and treatment. They lead play activities with children and young people who are in hospital or attending a hospital or clinic. Play Specialists are an invaluable part of the paediatric department when it comes to helping children cope with and understand painful or distressing procedures such as blood tests. They are qualified to NVQ Level 3.

Physiotherapists help people affected by injury, illness or disability through movement and exercise, manual therapy, education and advice. They can assess gait problems, help disabled children with mobility problems and help children with cystic fibrosis with chest physio. Physiotherapists are degree qualified professionals who may choose and area of specialisation such as chest physio, neurophysio etc.  Physiotherapists are registered with and regulated by the Health and Care Professions Council (HCPC)

Occupational Therapists (OT) provide intervention, support and/or advice to children and young people who are having difficulty joining in with the activities they need and want to do every day e.g. dressing, using cutlery, completing jigsaws, riding a bike, writing.  These difficulties may be due to poor gross and fine motor co-ordination, poor core stability, poor motor planning skills, visual perceptual difficulties or sensory difficulties.  OTs may offer advice and information to nurseries and schools to develop the staff’s knowledge of some of the difficulties the children/young people may have and how they can support them to join in these environments. OTs are degree qualified professionals. They are registered with and regulated by the Health and Care Professions Council (HCPC).

Speech and Language Therapists (SALT) support children and young people with speech, language, communication, oromotor and feeding problems. Speech and Language Therapists are degree qualified professionals. They are registered with and regulated by the Health and Care Professions Council (HCPC).

Paediatric Dieticians help babies, children and teenagers to eat and drink well.  They support children and their families where nutrition and special diets can be part of their treatment, including allergies, restrictive eating and cancer.  They have a key role in supporting families with children with allergies and those children who are fed via PEG, PEJ or other enteral means. Dieticians are degree qualified professionals. They are registered with and regulated by the Health and Care Professions Council (HCPC).

Orthotists provide devices for children and young people to be worn externally (orthoses) such as splints, insoles, spinal braces, lycra garments and specialised footwear to promote best posture and enable the best and most effective mobility. Orthotists are degree qualified professionals. They are registered with and regulated by the Health and Care Professions Council (HCPC).

Child and Adolescent Mental Health Services (CAMHS) comprise Psychiatrists (mental health doctors) and allied workers such as nurses, OTs, psychiatric social workers to look after children with mental health conditions. CAMHS services have historically been severely underfunded and there is little consistency across the UK in terms of what provision is made or what is commissioned from these overworked services. Inpatient CAMHS are provided at a Tier 4 superregional level and so young people needing specialised inpatient care may well end up many miles from their home. 

Social Care Services

Social Workers are degree qualified professionals who work with children and families and often specialise in a specific field of work – such as support for children and families or working with children with physical disability or mental health related needs. They work with social networks, families or communities, as well as individuals, and help develop supportive relationships.  The aim of social worker involvement is to empower families to achieve better outcomes at a “child in need” or “child protection” level. Social Workers in children and family services, may work with ‘looked-after children’, young offenders, children who have experienced or are at risk of abuse, children with health and mental health needs – and with their families. This may include helping families experiencing difficulties to resolve their problems. They may work specifically to assess and intervene where there are child protection concerns within a family or from elsewhere. Others may also manage the adoption and fostering processes, and support children with a disability. Most social care services will also employ OTs, support workers and other allied professionals within their teams and liaise closely with children’s services.

A brief note on Looked After Children (LAC)

This is a very important and much misunderstood area in terms of who’s who and how to address and refer to people who are a day to day part of a child’s life.  This is only a tiny snapshot, a much fuller article is mid production and when published will be referenced here.  


Looked After Child is a child who has been in the care of their local authority for more than 24 hours.  The term is synonymous with the term “in care” though this nomenclature has fallen out of current use.  
Foster Carers are paid by the local authority to look after children in care. Foster carers undergo rigorous training and being a foster carer is at least a full time job.  Foster placements may be for respite (especially for children with significant additional needs) now referred to as “short breaks”, short term or longer term.  Parental Responsibility for a LAC in foster care who have a care order is usually held by the local authority, possibly in conjunction with the birth parents.  A child in receipt of short breaks may be a LAC but their parents retain parental responsibility. A LAC will always have their own social worker and foster carers usually have their own social worker as well.


A Special Guardianship Order (SGO) is an order in England and Wales appointing one or more people over the age of 18 years to be a child’s permanent guardian. The order gives the special guardian parental responsibility for the child. The order is a private law order, which is made where a child cannot return to a parent but does not need to be kept in foster care or be adopted. The Scottish equivalent is a legal guardian.

Justice System Professionals

Probation Officers/Youth Case Workers support offenders on their release from prisons and young offenders institutions in England and Wales. They work closely with offenders, liaising with employers and educational services to try to ensure rehabilitation of young people who have committed criminal acts. In Scotland this role is carried out by social workers.

Youth Justice Workers work with young offenders who are in custody and on community sentences to ensure transition and rehabilitation when they are released from custody.

Family Case Workers work with children and young people whose parents are in custody.

How to interpret Rinne and Weber’s tests

Cite this article as:
Chiadika Uzor. How to interpret Rinne and Weber’s tests, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.30856

Hearing loss in children may be due to a broad range of pathology. Early detection and management are essential for proper language and psychosocial development of children. Clinicians need to be able to to identify potential reversible causes and rule out more sinister conditions.

There are three main types of hearing loss: sensorineural, conductive and mixed. The former, sensorineural, although generally uncommon in children, is the main cause of permanent hearing loss in the paediatric setting. It results from a disturbance of the auditory pathway involving the cochlea of the inner ear, through to the brainstem. Conductive hearing loss typically occurs due to a disruption in the transmission of sound at the level of the external or middle ear. The most common cause of conductive hearing loss in children is otitis media with effusion, otherwise known as glue ear.

This article provides a clinical approach to assessing a child with hearing difficulty using Weber’s and Rinne’s tests and a guide to interpreting the examination findings.

But first, Sarah’s Story….

Sarah is 9-year-old girl who has just begun Grade 4. She is very excited to be back at school after many months of limited contact with friends amid the pandemic. Over the summer, her mum, Michelle, notices that Sarah has become more ‘absent’ and doesn’t seem to be hearing anything she tells her. This is concerning and out of the norm for Sarah who is usually very witty and quick to respond. Mum has noticed that the volume of the TV is higher and many times she has ignored the doorbell ringing (according to mum it’s impossible to miss the doorbell!). Following her attendance at the Parent-Teacher’s meeting where complaints are made about Sarah’s inattentiveness in class, consequently leading to her grades dropping,she decides she has to do something. Sarah has undergone numerous treatments for ear infections in the past with oral antibiotics and topical treatments as well. Michelle and Sarah got to their GP the following day. Otoscopy shows a very narrow external auditory canal, dermatitis and a milky discolouration of the external ear. The GP is concerned and feels that he has exhausted every treatment option and decides to refer for review in the Emergency Department. Sarah is generally well and has no other past medical or drug history. There is no known family history of deafness and Sarah does not swim.

Anatomy of the ear as it relates to hearing tests

The history and physical exam

The presentation of hearing loss differs depending on the age of the child. Auditory anomalies, in newborns, are exclusively picked up via universal screening programmes. In older children, the presentation is usually similar to Sarah’s story. They may have behavioural changes, delayed language skills or listen to the TV at a louder volume than everyone else. These are often picked up by their teachers. It is important to ascertain whether there are other associated otological symptoms including otalgia, otorrhoea, vertigo or tinnitus. A thorough history should be obtained. This includes asking about other neurological symptoms, background medical history (including a thorough medication history), and possible precipitating events such as recent viral infection, trauma, or commencement of a new medication.

The tuning fork tests

Weber and Rinne tests are reliable and useful tools for assessing hearing loss in older, verbal children. They help distinguish between conductive and sensorineural hearing loss and so are more useful in patients with unilateral hearing difficulty. Children with mixed, or bilateral hearing loss, should be referred to an ENT specialist for a more formal pure tone audiometry assessment. It doesn’t matter which test you perform first. These tests should, however, be performed with a full cranial nerve and neurological exam.

How do I do Rinne’s test?

Before you begin the exam, it is important to explain to the child what you are about to do to in way they can understand. Try to put them at ease and make sure they are sitting comfortably, ideally in a silent room. Ask about pain especially over the mastoid.

Rinne’s test aims to compare air conduction with bone conduction. In a normal situation, air conduction is greater than bone conduction.

  • Begin by striking a 512 Hz tuning fork against your knee or elbow.
  • Place the base against the patient’s mastoid process (for those who like to watch it on a video, check one out here)
  • Allow it to stay there for 2-3 seconds to allow them to appreciate the intensity of the sound then promptly raise the fork off the mastoid process and place the vibrating tips about 1cm from their external auditory meatus
  • Leave it there for a few seconds before taking the tuning fork away from their ear
  • Ask the child whether the sound was louder at the beginning (when it was held against their mastoid) or whether it became louder (when it was held in front of their ear).

How do I interpret Rinne’s test?

In a normal ear, air conduction is greater than bone conduction. Air transmits sound to the cochlea more efficiently than bone. A patient who hears the tuning fork loudest when it is placed 1cm from the external auditory meatus has a positive Rinne’s test and a person who hears loudest when placed over the mastoid process has a negative Rinne’s test. The challenge in making these interpretations is in the case of a false positive Rinne’s test where there is unilateral sensorineural hearing loss. This is where Weber’s test is most useful in providing further clues.

How do I do Weber’s test?

  • As in Rinne’s test, begin by striking the 512 Hz tuning fork against your knee or elbow
  • Then, place the base of the fork in the midline, high on the patient’s forehead
  • Ask whether they hear the sound in the midline or if the sound lateralises to either the affected or good ear.
  • If the child is unclear, then ask if they hear the sound everywhere – be careful not to ask in a leading manner.
How to interpret Weber's and Rinne's test

How do I interpret Weber’s?

If a patient has a unilateral sensorineural hearing loss, the sound will lateralise – move to the good ear. If a patient has unilateral conductive hearing loss, the tuning fork sound would be heard loudest in the affected ear. Where hearing loss is bilateral and symmetrical of either type, Weber’s test would be normal. 

The table below summarises the outcomes of Rinne’s and Weber tests

How to interpret Weber and Rinne's test
AC = air conduction; BC = bone conduction

Differential diagnosis

Sarah presents to the Emergency Department and mum narrates the story to the attending doctor. Her right ear appears to be the troubling ear and she is experiencing mild pain on that side. The doctor proceeds to perform a focused ENT exam. He begins with otoscopy and notes that there is unilateral, right-sided narrowing of the external auditory canal secondary to oedema The overlying skin is milk coloured, resembling candida infection. Her left ear is impacted with cerumen but otherwise looks normal. Using a 512 Hz tuning fork, he performs Weber and Rinne’s tests. Rinne’s test demonstrated unilateral right-sided negative result i.e. bone conduction is greater than air conduction in the right ear. There is lateralisation of the tuning fork sound to the left ear on Weber’s: Sarah has reduced hearing in her right ear due to conductive hearing loss likely secondary to fungal otitis externa. The rest of her ENT exam is normal, and she is neurologically intact. The doctor explains to mum that Sarah most likely has a fungal infection of her outer ear and will require a trial of anti-fungal ear drop for 2 weeks. He also prescribes a short course of topical steroid ear drops to reduce the swelling and recommends oil drops for the impacted left ear, telling Michelle to return to her GP if Sarah has further issues with her hearing in the future. He reassures mum that a formal auditory test is not required at this time until the treatment is completed.

Selected references on interpreting Weber’s and Rinne’s

Dimitrov L, Gossman WG. Pediatric Hearing Loss. [Updated 2020 Nov 19]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK538285/

http://www.oxfordmedicaleducation.com/clinical-examinations/tuning-fork-rinnes-webers-test/

https://www.rch.org.au/clinicalguide/guideline_index/Acute_otitis_media/

https://www.youtube.com/watch?v=cZYJL9Jg-3k

https://www.uptodate.com/contents/image/print?imageKey=PC%2F74212&topicKey=PC%2F15359&source=see_link


 [dh1]Andy, I don’t think this image has a Creative Commons license. Do you want to create a new one? Or use a wiki one? There are some free to use ones without labels if you want to use Montserrat here https://www.google.com/search?q=ear%20anatomy&tbm=isch&tbs=il:cl&hl=en&sa=X&ved=0CAAQ1vwEahcKEwiY2fWm4s3sAhUAAAAAHQAAAAAQAg&biw=1190&bih=714#imgrc=HraZbFesh1dWIM

 [dh2]This is definitely not free to use. Do you want to make a new one Andy?

Emergency Contraception for teenagers

Cite this article as:
Tara George. Emergency Contraception for teenagers, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.31958

Olivia presents to the children’s emergency department at 3:40 one Monday afternoon still in her school uniform and with her friend Annabel. Annabel does all the talking to start with and tells the receptionist her friend has “a gynae problem”. She is somewhat assertive with the triage nurse arguing that Olivia needs to be seen by a doctor, in a private room and not in a cubicle with curtains.  They disclose that they are both 15 years old.  When you arrive in the room, Olivia tells you she’s come to A&E to get “the morning after pill”.

Requests for emergency contraception (EC) are a common reason for presentating to GPs, sexual health services and the emergency department. As a children’s doctor it can be a bit of a shock to the system to recognise that adolescents are just as likely as adults to need emergency contraception. There is often a learning gap in the paediatric medicine curriculum. In the UK, around 7% of all women of childbearing age have used emergency contraception in the past 12 months. 10-20% of sexually active teenagers acknowledge they do not always use condoms when having sex.  A request for emergency contraception in a teenager signals that contraceptive and sexual health needs are not being met.

What do you need to cover in the history?

  • Why does she think she needs emergency contraception?
  • When did the unprotected sex (UPSI) occur? (i.e. how long ago)
  • Where is she in her cycle? (i.e when was her last menstrual period (LMP)?)
  • Is her cycle regular and predictable?
  • How many other episodes of UPSI have there been this cycle?
  • Who did she have sex with?
  • Was it consensual? Do you need to consider Sexual Assault Referral Centre (SARC) or forensic medicine input? STI risk? Childhood Sexual Exploitation risk.
  • Was he a regular partner? (STI risk especially)
  • Remember she is 15 – the Fraser Guidelines apply here – does she have capacity to consent to sex? And to the treatment she is seeking? The NSPCC have a brilliant summary of Gillick Competence and Fraser Guidelines.
  • What contraception, if any, does she normally use?
  • Does she have a preference for the form of emergency contraception?
  • What is her weight? And BMI?
  • Is she on any regular medication which might affect the efficacy of oral emergency contraception?
  • What plans has she got for ongoing contraception?

Olivia tells you she had sex on Saturday night at around 2am.  She was staying over at her boyfriend’s house  Her boyfriend, Jack, and she have been in a relationship for about a year. He told her this morning that he thinks the condom might have split.  Olivia says she is normally careful to use condoms so feels pretty embarrassed about this situation. You ask a bit more about Jack.  Annabel tells you Jack is her twin brother. They are all in the same year at school and have known each other for years.  Olivia says her LMP was 10 days ago, her periods are really regular – every 28 days.  This is the only time since her LMP that she has had sex.  Jack is her only sexual partner ever and she is pretty sure he is hers too. Her BMI is 19 and she has no past medical history and is on no medication.

Now it’s time for a revision session on menstrual physiology*

*and on the life span of eggs and sperm without a good understanding of which, providing emergency contraception is really confusing

In a 28 day cycle the first day of the period is always referred to as day 1.  Ovulation then occurs around day 14 of a 28/7 cycle.  If the ovum is not fertilised within 12-24 hours, menstruation will occur 14 days later. The first part of the cycle is called the follicular phase and the second part the luteal phase.  The luteal phase is fixed in length in all women, if the cycle length varies the follicular phase may be longer or shorter than 14 days but following ovulation, if the ovum does not meet a sperm and implant, menstruation will always follow 14 days later

Sperm released into the female genital tract can live for upto 120 hours meaning if unprotected sexual intercourse (UPSI) has occurred any time from 6 days before the earliest possible calculated ovulation to 24 hours after ovulation there is a risk of pregnancy. If an ovum is fertilized by a sperm, implantation will occur 5-6 days after ovulation.

There’s a brilliant diagram in the BJFM article linked in the references section which shows dates, phases, risks and times of action really effectively.

These dates and timelines are crucial in establishing pregnancy risk and in supplying emergency contraception.  If ovulation has already occurred, a method which acts by postponing ovulation will be ineffective.  A method which works by preventing implantation cannot be used after the earliest possible implantation because it is unlikely to be effective but more importantly because it could be considered to be providing a termination of pregnancy which is only legal in specific situations.

What are the options for emergency contraception for Olivia?

There are 3 options licensed for emergency contraception in the UK.  The Copper IUCD, oral levonorgestrel (Levonelle) and Ulipristal (Ella-One)

The flowcharts and decision-making algorithms from the RCOG FFPRHC provide a really clear guide to prescribing.

The Copper IUCD is considered the gold ctandard first choice for EC because it:

  • Is the only method which is effective post-ovulation.
  • Is unaffected by body weight.
  • Is unaffected by enzyme-inducing drugs.
  • Provides ongoing contraception if required.
  • Has a failure rate of only 0.09% when used for emergency contraception.
  • Can cover multiple episodes of UPSI provided early in cycle.

Any copper IUCD can be used for emergency contraception though the “gold standard” for ongoing use is a device with a minimum of 375mm of copper, for example the T-safe 380a.  It is probably worth emphasizing here that this is not a Mirena IUS which cannot be used for emergency contraception.  A copper IUCD for emergency contraception can be fitted any time up to 5 days after the earliest possible ovulation in a cycle (so up to day 19 in a 28 day cycle). Fitting a copper IUCD for emergency contraception can cover several episodes of unprotected sex earlier in the cycle provided it is now before day 19 of a 28 day regular cycle. If a patient has had a single episode of unprotected sex after day 19 a copper IUCD can still be fitted.

It is unlikely there is a service within your emergency department for fitting copper IUCDs. This means that if a copper IUCD is the chosen option you are going to have to arrange for Olivia to attend a contraception and sexual health clinic or her GP if they have a coil fitting enhanced service. She’s on day 10 of her cycle so this is possible as she doesn’t “need” it until day 19 but are you sure she will go there? Not having had children is NOT a contraindication to having a copper IUCD fitted but it can be extremely uncomfortable and she may need a cervical block or gas and air to tolerate the procedure.

Oral EC  in the form of Levonorgestrel and Ulipristal both work by delaying ovulation. This is an important piece of information to bear in mind because if your patient has already ovulated oral emergency contraception is not going to work. It is also worth being aware of for those patients who believe life begins at fertilisation and who may be ethically or religiously opposed to preventing implantation via the use of a copper IUCD but who would find the postponement of ovulation (as per other hormonal contraceptive measures also) acceptable.  The flowchart in the FFPRHC guidelines is really useful to consult every time you have a case of this sort.  

Ulipristal (Ella-One) is second-line after a copper IUCD for the majority of situations when emergency contraception is needed. It does have a number of interactions, most importantly with enzyme inducers and progestogens. It is contraindicated in asthma if the patient is taking oral steroids.  It is important to note that if a patient has taken a progestogen within the preceding 5 days ulipristal will not work. It is not suitable in the case of “missed pills” requiring emergency contraception and it is not possible to “quickstart” POP/COCP/depo progestogen/Nexplanon if you give ulipristal.

Levnorgestrel is the only emergency contraception available both over-the-counter from pharmacies and on prescription in the UK.  It should really only be used if the risk of pregnancy is low and copper IUCD and Ella-One are both not suitable/not available. It is given as a 1.5g stat dose (though this should be doubled to 3g if she weighs over 70kg or has a BMI over 26 and cannot have Ella-One). If levonorgestrel is given the clinician can (and if reviewing the FFPRHC gold standard and NICE CKS advice, should) “quickstart” alternative contraception immediately with condoms until the next period.  The patient should do a pregnancy test three weeks after use if she has not had a normal period.  If your patient is on an enzyme inducer and declines IUCD, levonorgestrel is unlicensed but is the only other option as she cannot use ulipristal.

What else do I need to consider?

Any teenager who has had unprotected sex is at risk of STIs as well as pregnancy. There is no role for doing swabs urgently as they will not pick up STIs contracted as a result of this episode of unprotected sex.  Your patient should be signposted to a Contraception and Sexual Health (CASH) Clinic or her GP surgery for swabs in 3 weeks. You may want to write some free-text on your discharge letter to ensure the surgery are aware of this, and if the department isn’t too busy you might even want to ring her GP surgery and book her in for an appointment with the practice nurse for swabs.

Every teenager presenting needing emergency contraception has unmet ongoing contraceptive needs.  Whilst provision of such contraception may well be outside the scope of your role in the Emergency Department, basic counselling about ongoing contraception with signposting to CASH/GP and some relevant patient information leaflets such as those from www.fpa.org.uk should be within the scope of all clinicians.

If you have any concerns about a young person’s sexual activity and think there is any risk of sexual exploitation, abuse or inability to consent to treatment or to the sexual activity they have disclosed you need to raise this with the safeguarding lead in your department before you let her leave

You supply Olivia with ullipristal and some written counselling information about reliable contraception. You phone her GP surgery and get her booked in for a telephone appointment with a GP to discuss contraception a few days later and an appointment with the practice nurse for triple swabs for an STI screen in three weeks. Olivia rings her mum whilst in A&E and tells her what has happened. Her mum comes to pick her up and is engaged with the suggestion for ongoing follow up and contraception.

References

https://cks.nice.org.uk/topics/contraception-emergency/

https://www.fsrh.org/standards-and-guidance/documents/ceu-clinical-guidance-emergency-contraception-march-2017/

https://www.bjfm.co.uk/emergency-contraception-which-option-when-part-1

https://learning.nspcc.org.uk/child-protection-system/gillick-competence-fraser-guidelines#heading-top

Burack, R., 1999. Teenage sexual behaviour: attitudes towards and declared sexual activity. The British journal of family planning24(4), pp.145-148.

Conversations about constipation

Cite this article as:
Chris Dadnam. Conversations about constipation, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.31554

Like most of you I have to deal with the issues of constipation within the ED or CAU environment and most of the time it’s not the reason the child attends the department! This can then lead to a series of awkward questions and issues that parents may ask that we need to consider in order to provide safe, useful and most of all, worthwhile advice! 

So let’s go through these questions ask issues:

1. My child has a UTI, why are you talking to me about constipation? 

This is something I get asked not only when a UTI is diagnosed but other clinical conditions including; appendicitis, bedwetting, incontinence, urinary retention, obstruction, etc…. it is important that parents understand the implications of constipation, not only from a pain and symptoms point of view but also the complications surrounding it. Indeed, many parents also struggle to understand how their child, who is rolling around in agony, is only suffering from constipation (you can literally feel them questioning your medical acumen). 

During these tough times I always mention two key points. Firstly, the fact that your bowel covers the majority of your abdomen. A build up of wind and solid matter in the bowels can bring about severe griping abdominal pain, when pressing against sensitive nerves. As it covers a lot of your abdomen, when full, it will compress other structures like your child’s bladder leading to urinary infections, incontinence and retention. The second point is that stools are like a toxin your body wants to expel, when it remains in your bowels it can get into small structures like your child’s appendix and cause it to become inflamed and that leads to appendicitis.

Also, the longer the stools sit in the colon the more water is absorbed leading to harder, solid stools. This will cause a blockage and lead to vomiting and obstruction which may require surgery. 

Once parents have a better understanding of these points they’re less likely to roll their eyes at constipation! 

2. How are they constipated, they go every day? 

This in fairness is a good question, one that used to throw me quite a bit in my early paediatric years, but let’s break it down. Constipation is not simply the length of time between going to the toilet, rather it is the build up and insufficient clearance of stools in your bowels. With this in mind, a child can go daily and pass small amounts of stool but still have a backlog of faecal matter in their bowels. Therefore also question the time spent on the toilet, straining and pain during defecation. These are all signs of constipation. If you can, ask your young patients too! 

3. They already drink plenty of fluids

Don’t let this answer fool you, explore the parents’ meaning of fluids. When we are saying it, we mean clear liquids like water and squash (preferably sugar-free), but for the parents, it’s anything from water and tea, to milk and hot chocolates. Now there is a myth that milk makes us constipated but that’s simply not the case….well not entirely. Milk is indeed a liquid and it doesn’t make us constipated but it’s thicker and takes longer to digest (hence why in surgery they stress only clear liquids 4hrs before). Milk fills up the child and so reduces their intake of solid food which will be full of fibre. 

Always remember, parents may say they’ve cut out all the milk, but this may have been substituted for milky teas and hot chocolates, so double check! 

In terms of managing the milk, appreciate the difficulty the family is about to face. Wean the milk down slowly, starting with the bottles in the middle of the day, then the morning bottle and finally the night bottle. Milk shouldn’t be stopped entirely, having a 250-500ml glass of milk daily is perfectly fine. 

4. They eat a really good diet 

Whenever I get this response, I immediately think they haven’t and 99% of the time I’m right……says a lot. 

Again, this is either due to a misconception as to what a good diet is, or they don’t think it’s an important issue so they simply brush it off with this generic statement so that they can get to the medication that will actually help. Another quick way to check is to just ask the child. They normally find it much more difficult to turn a blind eye.

I always try to tackle this in one of two ways:

Tell me what they eat?

– it’s surprising how many children don’t have breakfast or any of the three square meals a day. If they do, just add in tips when you can.

Breakfast; dried fruits in cereal (especially raisins), don’t switch the cereal completely but rather mix in an all bran, so they’re still getting their tasty favourites, but now with added fibre. Toast – if it’s white bread, freeze it; it’ll keep longer and by placing it straight into the toaster means that the strands of carbs, fats and protein are bound together and form fibre.

Lunch; Try to include salad into sandwiches. If the parents say they don’t like salad then how do they expect their children too! Encourage healthy eating in the parents as well, to form positive connotations for their kids.

Dinner; any sauce can hide a multitude of veggies if blended or chopped fine enough – so get them cooking and where possible get them to encourage their kids to join in. If they cook it themselves, they’ll appreciate the food and, for some reason, enjoy it more……probably a labour of love! And it’s a great time to leave out and pick on a bowl of fresh berries or grapes, the more accessible things are the more they get eaten.

Do they eat all their fruit and vegetables?

This again leads to a classic ‘Yes’ response – which falsely reassures a lot of healthcare professionals. In truth, it’s a vague and rather inadequate question to ask. If I told you that my child eats peeled apples and pears, has a glass of orange juice and then eats loads of veggies which I boil until soft… It might make you think twice about the goodness they’re actually getting.  So I always ask – Do they eat the skins of the fruit and vegetables? How do you prepare them? The skin of most fruits and vegetables holds the majority of fibre along with different vitamins and minerals required. In all honesty, if you are peeling apples and pears, all you’re left with is sugar and water, so I tell parents to give their child the peel instead! 

Again with veggies, I tend to suggest for microwaving or steaming as people tend to overcook them when they boil them. They need a crunch as that equals fibre. Root vegetables (potatoes, sweet potatoes, carrots, butternut squash, celeriac, parsnips) – all these lovely fibre rich foods – contain most of the fibre in their skins. I tell parents to roast them, long and slow – they’ll taste better (caramelizes the sugars in the veg/skin) so children will prefer them! 

Be mindful of smoothies and fruit juices. Yes, they can count for 1 of our 5 a day (soon to be 10 a day) but they can have little to no fibre, especially with the models that separate out the pulp. The pulp is fibre!! Try to get them to have whole fruits instead or 1 x 250ml glass of fruit smoothie a day with the pulp. 

5. I have tried all this and it doesn’t work 

Before you dismiss this answer make sure you look over the medical background again (cystic fibrosis, hypothyroid disease, Hirschsprung’s). Ask these all-important questions:

  • When did they have their very first poo? It should be within the first 48 hours. Then double-check it was a good amount – small smears don’t count.
  • Have they had issues with weight gain and prolonged issues with chest problems (in cystic fibrosis, LRTIs tend to happen towards the end of their first year of life).
  • Did they have a Guthrie / heel prick test. Any developmental delay? A large soft spot on their head?

In all of these conditions, the child would have always had an issue with constipation since birth, so don’t miss them. 

Once covered, it’s important to go through what they have tried….. most parents will only have been given a packet of Movicol and told to get on with it. Look at the summary section to see how to structure a constructive management plan.

6. I’m scared they’ll starve so I give them what they want. 

How many of us have been told this with little Jonny sitting there looking larger than life?

In general, throughout the developed world, children are unlikely to starve if their parents are trying to feed them a healthy balanced diet. There are caveats to this:-

  • Autistic children or children with textural issues. 
  • Children with a background of eating disorders (bulimia or anorexia). 

These children will need extra support and input from community and nutritional teams. 

All the other children will always put up a fight (normally a good one!) but then their bodies will give in and want food. This is an important step for parents to understand, especially when the child is too young to go out and get food themselves. Make sure you tell the parents this won’t be a simple task, and the main reason children normally win, is that carers will be busy and won’t have time to tackle this problem. It’s a quick fix to give them something just so that they know they’ve eaten…….then the habit starts. I always tell parents, wait until you have a week off and prepare yourself/ yourselves for a bumpy ride. Have a united front, it’s no good if one parent plays the ‘strict/ bad guy’ whilst the other literally feeds the problem behind their back. Prepare meals and hide away the unhealthy processed snacks (or don’t buy them in the first place) and leave fruit out. Again, get the child involved in cooking, build a healthy connection with food and make it fun. Children will most likely throw tantrums at the start, but remind them that eventually their child’s body will give in and they will come for food, most likely with a grumpy face. 

Just make sure they’re hydrated with clear fluids! And NO milky substitutes. Remind the parents they’re not bad people and this will help fix things in the long run.

7. Movicol doesn’t work and I don’t want it to make their bowels lazy 

This age-old answer…..makes you wonder why we bother using Movicol? More often than not they’ve not prepared it correctly, despite the instructions being on the box. Honestly, the ways parents use Movicol; sprinkle on cereal, mix into snacks or food, add to tea….the list goes on!

Movicol is only effective when it is bound with water. After this, the parent can then mix it with a small amount of any other liquid or flavouring. Make sure they don’t add it to a litre bottle of squash as the child will have to drink the whole lot. Also, this means they don’t need the flavoured versions (which taste vile – remember when they made you try them in medical school!). 

Another myth is that “it’ll make their bowels lazy.” Explain that Movicol is not a stimulant, it is an osmotic diuretic and acts to drive the water you mixed it with into the child’s stool to make them softer and easier to pass. With this in mind, even stimulant laxatives won’t make your bowels lazy. I always say, they can be on it for the rest of their lives, it’ll never make their bowels lazy – that tends to reassure parents.

8. I tried laxatives before and they suddenly had diarrhoea so I stopped using it. 

This answer may again throw you into thinking that the laxative has done a great job in under 72 hours and fixed a months worth of constipation… it sounds too good to be true?? Well, it is. The big problem here is, if clinicians don’t pre-warn the carers what might happen after starting a laxative, it can lead to long term mistrust in both the medication and in our advice.

I always start off by setting the day to start. Aim to start the laxative at the end of the week, a Thursday or Friday, to avoid accidents in the school. They will deter the child from ever trying them again.

Once we know when to take them, always triple check they’re using them correctly… mix with water first, then add to a small volume of any other liquid for taste.

Finally, but most importantly, the change in stool. Referring to the Bristol stool chart (the only card I carry around!), I explain the child will start with Type1-3 stools. Then, they’ll have what looks like diarrhoea, brown watery smelly stools, but, of course, it’s overflow. Take the time to talk about why this happens; the Movicol is slowly moving through the hard stools, like rain trickling down a wall, in their child’s bowel. The Movicol/water mix will initially run over it but over time their stools start to soften. 

The next step, again important due to risk of pain, is the big logs. And big means big! I’ve had parents say they’ve used shears to cut up these stools in the toilet. This is essentially the wall slowly being emptied out. 

Once this is over, they will finally have the soft mushy stools. The laxative story should not end there! It is important to mention this ‘wall of stool’ has caused the bowels to stretch. This will lead to a build-up of stools again as the child won’t know when they need to go. This is reservoir constipation. It can take months to revert back to normal so I always advise to continue on with the laxatives and reduce (but not stop) the maintenance daily dose down if the child is passing clear watery liquid. Usually, treatment should continue for at least three months to treat reservoir constipation (although in some children it can be longer).

9. They don’t like my cooking. They’re vegetarian now, I’m not. 

It doesn’t have to be vegetarian, of course, this is just what an angry mum said to me once about her daughter. Parents will mention the difficulties of preparing food they’re not used to cooking, I always suggest ‘get your child involved‘. This is the perfect time to do something together (bonus points as well in tackling mental health and isolation. It gets the family talking). The child will appreciate their intent and willingness to give their lifestyle a try, which will build confidence in the relationship as well. They can get a cookbook, go online (it’s all free and easily accessible these days) and adapt their cooking style. Again. this will make their child feel more involved and interested in cooking and eating healthy foods. 

10. They don’t like fruit and vegetables. 

I think we’ve all suffered from hating vegetables and fruit at some point in our lives. I remember hating tomatoes and peppers, so I feel for any parent tackling this problem. 

There are several factors to contend with here:

  • Their child not liking the fruit because it’s unknown to them or feels texturally unsatisfactory.
  • Having access to other more ‘enjoyable’ foods such as biscuits, chocolates and crisps around the house which they can graze on and avoid these unwanted bags of goodness. 
  • Watching older siblings and parents, and copying them.

To this, I normally offer a number of solutions but be mindful that parents will have busy lives around their child’s eating habits so it has to be a conscious effort at a convenient time, like over the weekend or annual leave.  

Firstly, hide unhealthy snacks or simply reduce the amount you buy, what they don’t see they don’t know…. out of sight, out of mind technique.

Secondly, I always tell carers that they and older siblings are role models. If you’re not eating it, why should they? There should be a united front by the parents. 

Finally, get cooking and get your child involved. Any child who cooks will appreciate the food they’ve made and the sense of achievement, even if it doesn’t taste nice, they’ll love it. It’s a great time to chat over a bowl of fresh berries…

My own enthusiasm then tends to kick in. I like to say “Get creative in the kitchen!” I’ve mentioned simple things for example: make flapjacks and throw in lots of dried fruits; raisins, dates, apricots, prunes – all-natural sweetness with skinned nuts & oats. Freeze smoothies with the pulp into ice-lollies. Fruit crumbles with honey and oats… With vegetables, always remember to steam or microwave them, they need the crunch. Again if kids don’t like them…. Chop them up/ blend them and throw them into sauces, pies and mixed into other dishes. If it’s the taste they don’t like, again mixed into gravy or a tasty sauce will fix that problem! 

It is important to mention the importance of a healthy balanced diet. Food is your best medicine. This is can be true for managing many conditions; anaemia , skin problems, poor immunity, nail and hair problems..you get my point. A varied diet holds the key to a lot of management plans, and it’s important to mention this even when the child is on supplements. A classic example is the parent says we’ve fixed the iron problem with iron tablets, but they fail to realise, without vitamin C your body can’t absorb the iron through the small intestine. Therefore, they’ll be questioning why their child remains anaemic in months to come. 

11. They’ve gone back into nappies as they’re scared of using the toilet. 

This is an important issue. It’ll mean the child will probably have problems with incontinence which may be affecting their social life such as staying over at a friends houses. Yet another reason why it needs tackling. 

Always start with asking what happened? More often than not it’s a series of bad habits and untimely events that have led to a regression in the family’s good practice. It happens to the best of us. Reassure everyone and give them the positive reinforcement that they’ve identified a problem that needs to be solved. Then offer the advice below. Take your time with these parents, it would have taken a lot for them to come into an acute setting to seek advice, so try to give them some. 

A framework for managing constipation.

This is ultimately an important topic that you have or, no doubt, will see at some point in your paediatric career. Knowing how to manage it is a core skill. 

I always frame my management in 3 steps: 

1. Diet and fluid intake – take the points from the above questions. Ultimately, the parents control the diet and food at home. They, and older siblings, are the child’s role models so what they eat will influence the child’s diet too. Remind them that food is their family’s best medicine so they need to get it right. Cancel out the milky drinks, cook smart & healthy and don’t forget clear fluids.

2. Toileting – our constipation module covers this but key points; get them into a routine (20 to 30mins after dinner – to sit on the toilet). Make the toilet a fun place with all their toys and gadgets and don’t forget to get them to blow the bubbles. Optimise positioning with knees above bums when sitting, using a footstool. 

3. Medication – ensure parents are giving them correctly – mixing with water first then adding to any other liquid for taste. Make sure this isn’t a full bottle, as they’ll have to drink the lot!. Movicol doesn’t make your bowels lazy. If they’re on a disimpaction regime, think about the volume they’ll be drinking each time. It may be better to split it into thrice daily doses instead. Briefly touch on the sequence of stool changes to reduce misconceptions of overflow and diarrhoea. Lastly start Movicol towards the end of the week, Thursday or Friday to avoid accidents at school. 

End with: 

  • Referencing the ERIC constipation website. It’s a great tool for constipation and bedwetting. It talks to the parent and child, so easy to understand and explain.
  • It will take time for things to fall into place. There is no quick fix. There will be tantrums, sleepless nights and days you’ll want to give in. Hang in there and once you’re sorted you’ll wish you’d done it sooner!

Ethical considerations and decision-making about the resuscitation of very sick children

Cite this article as:
Karen Horridge. Ethical considerations and decision-making about the resuscitation of very sick children, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.30898

You wouldn’t judge a book by its cover, would you?

When thinking about the knotty matter of decision-making about resuscitation of sick children, we need to remember:

Every child’s life matters.

To always listen to the child, their parents and those who know the child best and include them in best-interests decision-making.

To always make decisions within an ethical framework and record the process of decision-making and who was involved.

To ensure pathways are in place to record and make visible best-interests’ decisions about levels of intervention.

So, what can help us with an ethical framework? Our friends at the General Medical Council have laid this out for us, in ‘Treatment and care towards the end of life: good practice in decision-making’.

Covers of GMC guidelines

This helpful document reminds us that:

  • Equalities, capacity and human rights laws reinforce our ethical duty to treat all children, neonates and young people fairly. 
  • We must always listen to child, parents and others close to them. We must take account of their views. Where there are different views, our primary duty of care is to the child.
  • Decisions must always be in a child’s best interests. 
    • We should weigh benefits, burdens and risks of treatment. 
    • We should consider all relevant factors in the decision-making process.
  • Decisions must start from a presumption in favour of prolonging life.

When making decisions, even in a dire emergency, we are reminded to:

  • Take account of up-to-date, authoritative clinical guidance.
  • In case of uncertainty, seek further expert opinions early.
  • Explain, justify and document all factors considered in decision-making.
  • Not rely on personal values when making best interests’ decisions.
    We must be careful not to make judgements based on poorly informed or unfounded assumptions about the impact of disability on the child or young person’s quality of life. 

So, you wouldn’t judge a book by its cover, would you?

Disabled children and young people come in all shapes and sizes. We can’t all be experts in each child, which is why it is so important for us to listen to parents and those who know the child best.

Please do beware of falling down the rabbit hole of preconceived ideas about what a child’s quality of life is like – always ask parents and familiar carers what the child is like when well. They may have photos and videos they can show you of their child having fun and taking part in everyday activities.

Disabled children may appear, sound and behave differently when unwell. This may be because they cannot tell you where it hurts or how they feel. They may present with behaviours that others may see as challenging. They may not tick the expected boxes on the usual screening tools, for example, for sepsis. Their temperatures may be low when you might expect them to be high, they might have high or low heart rates and may not be able to mount the expected increased work of breathing, because their respiratory muscles may be too weak for them to do so. Their control centres may not work as expected.

Hasty decisions based on preconceived ideas can lead to poor outcomes, including premature death. None of us want that.

So, what’s all this about premature death in people with learning disabilities?

If you are ready for a shocking read, then take a look at Death by Indifference, written by Mencap in 2006, but just as relevant today.

If you prefer some e-learning on the subject, take time to work through the Disability Matters session: Equal Access to the Best Health Outcomes Matters. This was co-produced with disabled children, young people, parent carers and other experts.

Then there’s the Confidential Inquiry into Premature Deaths of People with Learning Disabilities report. This report showed that women and men with learning disabilities die 29 and 23 years, respectively, sooner than women and men without learning disabilities.

So what about children? The series of reports on Why Children Die show that more than half of all children who die in England have a pre-existing, life-limiting condition. So, we all need to pay great attention and ensure we achieve the very best outcomes for these children and all children.

The team at Bristol University have built on the work they did for the CIPOLD report and now lead on the Learning Disabilities Mortality Review (LeDeR) programme. This links to the Child Death Review programme and considers the circumstances of every death of anyone with a learning disability at any age. The latest annual report can be found here.

Between 01/07/2016 and 31/12/2019, 516 children aged 4-17 years were notified to the LeDeR programme. Of these children and young people who died, 43% were from Black and Minority Ethnic groups. 46% had profound and multiple disabilities.

Whilst 7% of the deaths reported to the LeDeR programme were of children and young people aged 4-17 years, the death rate overall in the 5-19 years age group in England was 0.3%.

The LeDeR programme 2019 report highlights good and problematic practices that the multidisciplinary team, including people with learning disabilities, identified.

Good practices included:

  • Good care coordination across agencies and specialities
  • Excellent end-of-life care
  • Person-centred care, adjusted as the child or young person’s needs changed

Problematic areas of practice to reflect and learn from included:

  • Delays in responding to signs of illness or investigating illness.
    • This is known as ‘diagnostic overshadowing’, where clinicians may see the disabilities and think all presenting signs and symptoms can be explained by those, rather than undertaking a careful and structured clinical assessment to identify the underlying cause, such as pain from appendicitis, constipation, sepsis etc.
  • Poor quality multidisciplinary team working.
    • The needs of disabled children and young people are usually multifaceted and require a range of expertise to adequately assess and address them all.
  • Poor advanced care planning. 
    • Clinicians can be reluctant to have those difficult conversations with families about the risk of both sudden and unexpected death and also the risk of deterioration and death. We all need to do better on this.
  • Problems with the direct provision of care.

Recommendations from the LeDeR report included:

  • Identification of a key worker to coordinate care and communication for disabled children and young people.
  • Timely advanced care planning embedded in care pathways and clinical practice, responsive to changing needs.
  • Better discharge planning and better community support.
  • Consistent support and communication throughout each child’s life.

So where is the evidence about what parents think about end-of-life decision making? Dr Sarah Mitchell is a GP who is really interested in this and has written a useful paper in BMJ Open on the subject.

Sarah and her team interviewed parents and reported that:

  • Parents have significant knowledge and experiences that influence decision-making process
  • Trusted relationships with healthcare professionals are key to supporting parents making end of life decisions
  • Verbal and non-verbal communication with healthcare professionals impacts on the family experience.
  • Engaging with end of life care decision-making can be emotionally overwhelming, but becomes possible if parents reach a
    ‘place of acceptance’
  • Families perceive benefits to receiving end of life care for their child in a PICU 

With regard to the last statement, preferred place of death is, in my experience, different for each family. Some choose home, others need to know that no stone has been left unturned right to the end, so choose intensive care. Whilst this may not always sit comfortably with paediatric intensive care teams, for some families it is what is needed to bring them peace in their journeys of grief. I have been witness to what I would consider to be ‘good deaths’ in a wide range of settings, including homes, hospices, children’s wards and intensive care units over many years. What matters most and what families remember, are how the child’s needs were identified and addressed at every step and how they are families were kept in the loop about what was happening, including being fully involved in all decision-making.

Smiling boy with disability
Matthew

It’s all well and good listening to my views as an experienced disability paediatrician on the subject, but much better to hear directly from a mother who has been on the advance care planning journey. Here are Kay’s words, describing her and her family’s journey with her son Matthew. If you prefer to hear her speaking directly, please check out the Disability Matters e-learning session Advance Care Planning Matters.

Kay says:

“Matthew had quite severe learning and physical disabilities. He was a very complex child and we used the Emergency Health Care Plan to help plan for the future and to enable us to communicate fully with other health professionals in the healthcare setting. 

Matthew didn’t have the capacity to actually make decisions for himself although he was a very wilful little boy who had very clear likes and dislikes, so he could make decisions for himself that were relevant to his day to day needs like what he wanted to eat, where he wanted to go. He had no formal communication, but as parents and the people who were involved with him we learned to read what he was trying to tell us, and as I say, he was very clear on what he liked and disliked doing but wouldn’t have been able to make the kind of big decisions that as parents we were responsible for making for him.

We did have a very large team involved in Matthew’s care and we discussed the Plan with his consultant paediatrician but she also took into account the views of people like the occupational therapists, physiotherapists, the surgeons who were dealing with him as well as the parents, myself and Matthew’s dad. We were all involved in the discussions around the development of the Plan and what was in his best interests.

Matthew’s Health Care Plan went everywhere with him and it lived in his communication bag on the back of his wheelchair. So it went with him to school, to his respite and to his dad’s house when he was there and also it was with him whenever he needed to go into hospital.

The Emergency Health Care Plan actually protected Matthew’s rights should he ever become seriously unwell. It clearly stated that he needed full resuscitation and any treatment that was available to him and clinicians then could use the plan to make judgments on what treatment would be necessary but he was able then to access a full range of treatment that would be available to any other child in a similar circumstance.

Matthew was very well for the early part of his life but when he turned 11 he had an accident, a quite serious accident and we were able to use the Emergency Healthcare Plan to access full range of medical interventions for him, as a result of that he did end up in intensive care for the first time. After that we did go through a period when he was 12 he had a twisted bowel, so he was literally in and out of intensive care and needed to be resuscitated on quite a few occasions, unfortunately, but his plan enabled us to access all of this medical intervention for him and saved his life in that instance.

Over a period of about a year, Matthew became more and more unwell. He needed regular trips to Intensive Care and regular trips to the hospital. The Plan actually went with him to hospital in Newcastle. It gave us the assurance that during these periods of Matthew being very, very poorly that the doctors in the hospital that weren’t used to dealing with Matthew, the Plan gave them all the information so we didn’t have to go through it every time that he was admitted. They trusted what we were saying because it was backed up by the Plan”.

Matthew’s paediatrician says:
“Matthew developed a lot of new symptoms that were unexplained; his seizures were becoming less controlled and we needed discussions around how we were going to treat these new symptoms as and when they appeared and what needed to be done for them.

So at this point in time, having taken account of the views of both of Matthew’s parents and the whole of the multidisciplinary team, we made a decision in his best interests that, at that time, further intrusive procedures or further intensive care was not going to be helpful or appropriate for him and might cause him further distress. So, together, we changed the wording on Matthew’s Advanced Care Plan at that time to reflect the possibility of him being allowed a natural death when his time came, recognising that we would always be there to manage his symptoms and always be there to support his family”. 

Kay continues:
“Matthew had had a chest infection and was having great difficulty breathing and his dad brought him up to hospital. We called all the family, it was obvious that Matthew was dying at this point. We had all the family called from all corners of the UK to come and say goodbye to him. We sat for hours and hours with him in the hospital ward and he was almost pronounced dead when all of a sudden, he decided it wasn’t quite his time and he took a great big breath and started breathing normally and all his colour came back. His dad described it as, like, “re-booting” his system as he wasn’t on any treatment. He was having no medication or anything and he just decided “No, it’s not my time. I’m going to come back” and we had him for another five weeks after that.

Then one day, approximately five weeks after the “re-booting” incident, Matthew wasn’t very well at all. He was due to see his paediatrician in clinic that day, but I phoned and said that “I don’t think he’s up to actually travelling to hospital,” so the paediatrician agreed to visit at home, so we waited. He’d had a massive seizure. I’d had to give him medication to bring him back from the seizure. When his paediatrician arrived, it became obvious that he was deteriorating very, very quickly and we decided that we were just going to keep him at home and see what the outcome would be, whether he would “re-boot” again or how it would play out this time”.

Matthew’s paediatrician continues:
“So Matthew was really very frail on clinical assessment at this point, so I needed to put in place the right procedures to make sure that his needs were met and the family’s needs were met. He was very peaceful and he was essentially drifting off to sleep. He didn’t have any difficult symptoms at that point that needed any changes in medication or changes in his Plan. What we did at that point is I made arrangements for if Matthew was to slip away in the night, a colleague to be able to come out and support the family and to confirm his death at that point. I also contacted the Coroner, because our Coroner liked to know in advance about any child’s death and if there is a death that is likely to be expected as defined under the Child Death Review Procedures, then our Coroner liked to know in advance. So, we made all of those arrangements and let the family have their special private time together”. 

Kay reflects:
“We spent quite a few hours with Matthew – we called close family this time around. We didn’t get everybody coming from the far end of the country to be with Matthew. So, the time when Matthew did die, it was very, very peaceful. We had some quality time with him. We had a wonderful five weeks planning memories, planting memories for the other children and we spent those few hours reflecting on that and talking and supporting each other through the inevitable outcome of Matthew dying in the early hours of the morning.

We found that, when dealing with the professionals around Matthew’s death, that being able to change the wording of the Emergency Health Care Plan it kind of validated for us that what we wanted … we wanted Matthew to be peaceful and at home when he died, surrounded by his toys and his family. We felt that the Plan, when we read the wording of it, was quite a shock to see that he should be allowed to ‘die with dignity’ but it validated what we were feeling, that it gave us permission to ask that he could die at home where we wanted him to be. It made us feel that we weren’t asking for anything that was out of the ordinary or not possible and felt that we were actually more in control of the situation when the time actually came for Matthew to pass away”. 

Achieving a supporting a child or young person through a good death is an important part of our job, when death is inevitable. We need to ensure we always steer the best possible course through the tricky waters of decision-making, protecting the rights of all children and young people to the best possible outcomes.

So, back to the key messages

  • Every child’s life matters.
  • Always listen to the child, their parents and those who know the child best and include them in best-interests decision-making.
  • Always make decisions within an ethical framework and record the process of decision-making and who was involved.
  • Ensure that pathways are in place to record and make visible best-interests’ decisions about levels of intervention.

If you want to read more about advance care planning, look at:

Horridge KA. Advance Care Planning: practicalities, legalities, complexities and controversies. Arch Dis Child. 2015;100:380-385

If you want to see and hear examples of the conversations that underpin advance care planning, more free e-learning can be found here:

https://councilfordisabledchildren.org.uk/our-work/health-and-wellbeing/practice/emergency-healthcare-plans

To listen to discussions between paediatricians and a parent about signs of sepsis in disabled children, tune in to Episode 4 of the RCPCH sepsis podcast series here.

Thank you for your time and all the best for your advance planning and decision-making.