DFTB in EMA #4 – Spoonful of sugar: Improving the palatability of emergency department visits for children and their families

Cite this article as:
Andrew Tagg. DFTB in EMA #4 – Spoonful of sugar: Improving the palatability of emergency department visits for children and their families, Don't Forget the Bubbles, 2016. Available at:
https://doi.org/10.31440/DFTB.8049

Whilst it has been available online for some time now the fourth EMA article from the DFTB team has just hit doormats across Australia.

“Pain, fever, fatigue and fear can all add to anxiety and distress for unwell children and their families, as well as making assessment of their clinical state even more difficult. This article aims to describe some ways of helping the medicine go down for your paediatric emergency patients.”

Click here for the link to the full article – ‘Spoonful of sugar’

Reference:

Lawton, B., Davis, T., Goldstein, H., and Tagg, A. (2015) Spoonful of sugar: Improving the palatability of emergency department visits for children and their families. Emergency Medicine Australasia, 27:504506. doi: 10.1111/1742-6723.12506.

Spina bifida – what is spina bifida?

Cite this article as:
Lydia Garside. Spina bifida – what is spina bifida?, Don't Forget the Bubbles, 2015. Available at:
https://doi.org/10.31440/DFTB.6758

A couple come to see you following their 18 week ultrasound. They have been told that the ultrasound showed that their baby has spina bifida. The family wish to discuss the situation with a paediatrician.

How common is spina bifida?

The incidence at birth <1 in 200.

The number of cases of spina bifida is declining. This is likely due to earlier ultrasound diagnosis and the increasing use of folic acid supplements by women of child-bearing age.

80% are now diagnosed antenatally and many are terminated in the antenatal period. Blood screening for raised alpha fetoprotein is helpful if positive.

What is spina bifida?

Spina bifida is a congenital lesion of the spinal cord which results in some part of the spinal cord and meninges being exposed.

Spinal lesions are classified into:

  • Anencephaly – the natural history is 67% stillborn, 33% neonatal death.
  • Encephalocele – the natural history is 23% stillborn, 43% neonatal death. Many of the survivors have a significant disability – spastic quadriplegia, epilepsy, hydrocephalus, and intellectual disability.
  • Spina bifida.

..and where is the lesion?

Most spinal defects are in the lower lumbar to sacral region, but they can occur at any level. Most are located posteriorly.

Spina-bifida

Greater than 97% of cases of spina bifida are associated with Chiari II malformation in the brain. This causes displacement of the cerebellum, fourth ventricle and medulla through the cisterna magna resulting in a banana shaped cerebellum. There is also concavity of the frontal bones resulting in lemon shaped skull and ventriculomegaly.

The more severe lesions can be seen as early as 11-12 weeks, but most are diagnosed at the routine fetal anatomy scan at 18-20 weeks

How can we predict functional outcome?

Ascertainment of the level of the spinal lesion is the most important predictor of functional outcome. MRI may be used to aid in this – but a thorough high resolution US examination is more effective at determining the level of the lesion.

There can be associated deformities of the lower limbs such as club feet and hip dislocation. Most neural tube defects are isolated malformations, but up to 15% have an association with other abnormalities (VSD, renal, IUGR), and 3% have an association with chromosomal abnormalities.

A lipomyelomeningocele is the mildest form of spina bifida – usually there is no Chiari malformation. Mobility issues are harder to predict in these cases though, and they may be progressive. Patients often have incontinence/bowel issues.

The main questions to ask when considering the likely functional outcome are…

  • Is this spina bifida?
  • Where is the lesion? How big? What type?
  • Is there a Chiari malformation?
  • Is there hydrocephalus?
  • Is there bony deformity?
  • Are there leg movements?
  • Any other anomalies?

You can check out the rest of the series here:-

Communication: Drug error

Cite this article as:
Tessa Davis. Communication: Drug error, Don't Forget the Bubbles, 2014. Available at:
https://doi.org/10.31440/DFTB.4896

Scenario: you are the register on the General Paediatric Ward. As you are reviewing patients, you realise that one of your patients, 9 month old Kayleigh, has been prescribed and given the wrong dose of paracetamol. She received one dose that was 5 x the recommended dose.

You need to speak to the family and let them know – how do you approach this?

What is autism?

Cite this article as:
Tessa Davis. What is autism?, Don't Forget the Bubbles, 2014. Available at:
https://doi.org/10.31440/DFTB.5235

As paediatricians we may not be required to diagnose autism, but we should be able to understand and recognise the main features.

There are two main diagnostic areas under the new DSM V criteria

A. Persistent deficits in social communication and social interaction

B. Restricted, repetitive patterns of behaviour, interests, or activities

For a diagnosis of Autism Spectrum Disorder (ASD) children need to have A. and at least two of the criteria in section B.

What constitutes a persistent defect in social communication and social interaction?

This can be deficits in social-emotional reciprocity. A child with autism has difficulty engaging in normal conversation. That’s not to say that child wouldn’t be able to answer direct questions – some children can do this very well – but the normal flow of conversation isn’t the same. That might be that they don’t share things in the same way as other people and don’t feel the need to tell you how they are feeling or respond to social interaction in the same way as others.

It can also be deficits in nonverbal communication behaviours used for social interaction. This can show itself as unusual body language or difficulty with maintaining eye contact. Again, autism is a spectrum disorder so it’s not as black and white as eye contact or no eye contact. It’s the quality of the eye contact – is it meaningful? Is it fleeting? Is it appropriate? Does the child smile at you or are they smiling at a toy they like? Body language deficits might also be difficulty using gestures to explain and communicate – such as pointing or waving.

And finally it could be deficits in developing, maintaining and understanding relationships. This issue is around the nature of play and interaction with peers. The child may not engage in interactive or imaginative play. They may enjoy playing but it has to be on their own terms and the child may not be bothered if another child doesn’t like the game or wants to play something else.

What are the criteria for showing a restricted, repetitive pattern of behaviour, interests, or activities?

For the diagnostic criteria for ASD, a child also has to have at least two of:

Stereotyped or repetitive motor movements, use of objects or speech. Children with ASD can demonstrate echolalia or use odd or repetitive phrases. They might have certain movements like hand-flapping; or they demonstrate repetitive behaviours like lining all their action figures up in a row.

Insistence on sameness, inflexible adherence to routines, or ritualised patterns of verbal or nonverbal behaviour. Some children can show very rigid thinking where they are unwilling to budge on their viewpoint regardless of what they parents or peers are telling them. Sometimes a routine can be very strict and any deviation from this can cause distress. This may be that they have to eat from the same plate and cup and have the same mat to eat their breakfast off every day.

Highly restricted, fixated interests that are abnormal in intensity or focus. The classic is a child who loves spinning objects – wheels, hoops, anything that turns. The child might be fascinated by watching the wheels spin endlessly. Or, the child may have a specific toys that they love, for example trucks – where they are obsessed by trucks and cannot focus on other activities.

Hyper- or hyporeactivity to sensory input. Children with ASD can have an unusual interest in sensory objects – lights, sounds or textures. Sometimes this can be a positive experience – they may love feeling soft objects against their skin or be fascinated by toys with lights. Or it can cause distress – not liking particular food textures, or becoming very upset at the feel of sand on their hands or feet.

What does it mean to have ASD?

Autism simply means that the way the child thinks and sees the world is different to other people. Although it’s a spectrum disorder we don’t grade severity.

A child with autism may have poor language skills; or they may have particular difficulty with rigid thinking and routines; or they may have a particular problem with a fixation on one type of toy; or they may have difficult making friendships or relating to their family members.

Autism means something different for each child and they may be very severe in one area but mild in another.

So, how it is best managed?

Management of autism is geared towards helping the child lead a life that is meaningful and happy for them.

Any therapies will be geared around supporting the child and family in managing the aspects of autism that cause the child the most difficulty.

Speech Therapy, Occupational Therapy, ABI, behavioural intervention, positive parenting and support groups can all help; but the most useful therapy will be very much tailored to that child’s needs.

What was the fuss about the DSM V?

In May 2013, the DSM IV was replaced and there were new diagnostic criteria. The main changes are:

Asperger syndrome is no longer a diagnosis. Asperger syndrome, PDD-NOS, childhood disintegrative disorder and autistic disorder have all been unified to ASD.

For diagnosis, symptoms can be in the present or reported historically. And, any symptoms should be taken in the context of an underlying genetic, neurological condition, or intellectual disability.

There is also a new Social and Communication Disorder which has the social and communication elements of ASD but without the rigid thinking and repetetive patterns.

People previously diagnosed using DSM IV criteria can keep the diagnosis and do not need to be rediagnosed. Any funding they had previously will not change.

N.B. For toddlers, we are allowed to use the DSM IV criteria as toddlers with autism are unlikely to show the rigid thinking and routine dependence that older children do, and this is a requirement for diagnosis in the DSM V.

Understanding autism is not easy for paediatric trainees or parents. Getting a handle on what it means for the child and the child’s way of thinking helps parents to support the child and helps paediatricians to recommend therapies.

The over-arching aim is to recognise what autism means to that individual child and how help them manage their way of thinking to maximise their enjoyment and ability to lead an independent life.

 

Other Resources

Autism Speaks

DSM-V