Fontanelle paediatric education

Many of us see children with joint pain, some of whom are incredibly bendy. But have they "got hypermobility" or even EDS? Join Dr Alice Leahy and Marisa McMillan in their wide ranging discussion about EDS, hypermobility syndrome, arthritis, factors affecting how people perceive pain, and possible reasons why EDS has become more prominent on social media recently.

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Pearls include:

The Beighton Score: not very useful in the local paediatric population and is not validated.

---- Ehlers Danlos Syndrome (EDS) is a collection of collagen disorders

13 subtyes of EDS, 12 of which have known genetic mutations and histopathological changes, the

13th - neither genetic nor histopathological changes, otherwise known as hypermobility EDS. ----The 3 types worth remembering are:

☆Vascular type EDS - very rare - usually present to genetics depts rather than to rheumatology department - FH vascular rupture, Distinctive facial characteristics.

☆☆Classical type EDS. Also rare. Extremely hypermobile. Excessively stretchy skin. Can pull skin out from forearm 6cm (upper range of normal 2cm)

Autosomal dominant. Often very bruised with plentiful scarring on body.

☆☆☆Hypermobility type EDS: Beighton score of >6 pre-pubescent children; >4 in pubescent children

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Joint pain is not a good indicator of inflammatory arthritis. ♡♡♡JIA usually presents with very little pain. Arthritis will present with joint swelling.

If arthritis is suspected, request an ultrasound. A normal USS will exclude inflammatory disease. Blood tests are not helpful in diagnosing arthritis - neither are X-rays.

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Useful Resources:

•••The 2017 international classification of the Ehlers–Danlos syndromes (Malfait et al)

https://doi.org/10.1002/ajmg.c.31552

••••The RCPCH position statement on establishing a correct diagnosis of Ehlers Danlos Syndrome hypermobility type (hEDS) in children and adolescents 

https://www.rcpch.ac.uk/resources/establishing-correct-diagnosis-ehlers-danlos-syndrome-hypermobility-type-heds-children#footnote5_xb0pjtb

•••Head First by Alistair Santhouse Head First: A Psychiatrist's Stories of Mind and Body https://www.amazon.co.uk/dp/1838950311/ref=cm_sw_r_apan_glt_fabc_6KB3WZ7BY8XBW3WEYA8Q

•••It's All in Your Head by Suzanne O'Sullivan

It's All in Your Head: Stories from the Frontline of Psychosomatic Illness https://www.amazon.co.uk/dp/0099597853/ref=cm_sw_r_apan_glt_fabc_M77GAQV12ES834PWHCTT

A look inside the world of adolescent medicine and the benefits of the HEEADSSS tool with Dr David James, who explains to Marisa McMillan how we can be opportunistic in reaching the 10-22 year olds presenting to our ED departments. Check out the must-have HEEADSSS app on Google Play/Apple or http://app.heeadsss.uk Review on itunes 🌟🌟🌟🌟🌟 if you like what you hear and get in touch if you want to get involved. Spread the word and share the knowledge!

Low appetite? Tired? Tummy ache? Perhaps your child is constipated. Let Dr Mark Tighe guide you through the essentials of constipation - what it is, what sets it off, and most importantly, what you can do about it. Go to eric.org.uk for excellent resources. Please review 🌟🌟🌟🌟🌟Meet us online @fontanellepod - we'd love to hear from you.

Dr Antoinette McAulay, General Paediatrician in Poole talks to Dr Marisa McMillan, Wessex Paediatric Trainee, guest hosting on Fontanelle - Thank you Marisa and Antoinette! Refer to www.bsped.org.uk (British Society for Paediatric Endocrinology and Diabetes) for up to date guidelines. Get in touch if you would like to get involved in any of the work of the Fontanelle team by writing to me, Caroline Storey at [email protected]

Dr Phil Parslow, training programme director for ST4-8 in Wessex Deanery guides us through the whole appraisal process, with plenty of advice and wisdom to light your way! Amazing nuggets on how to write an effective reflection. ***************************************************🌟🌟🌟🌟🌟🌟🌟🌟🌟🌟🌟🌟🌟🌟🌟🌟🌟🌟 Fontanelle is growing and has now reached 50 countries with over 5000 listens. Many thanks for all your support. If you would like to help Fontanelle or get involved in any way, please get in touch! Thanks 🌟🌟🌟🌟🌟

Dr Mich Lajeunesse consultant in paediatric allergy takes us through using the EATERS method to take an allergy focused clinical history and help diagnose food allergies. EATERS stands for Exposure, Allergen, Timing, Environment, Reproducibility, Symptoms. Based on a 15 Minute Consultation in E&P edition of ADC (http://dx.doi.org/10.1136/archdischild-2018-316397) If your interest is piqued Mich also directs us to the RCPCH Paediatric Allergy Training study days.~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~ Other news: Fontanelle is nearly 1!🎂🎈To celebrate our first birthday we're giving away a pair of Sony headphones so the winner can listen to educational podcasts on their way to work. For a chance to win, go to Twitter, find me @fontanellepod and retweet the pinned tweet. The winner will be announced at the PIER conference 2019. Tickets still available. Come and find me there and say hello! Thanks so much for listening! 🌟🎂❤🍭🍬

Dr Amy Mitchell takes us through a real case looking at two oncological emergencies: hyperleucocytosis and tumour lysis syndrome. With reference to the PIER guidelines at piernetwork.org.uk Children's Cancer and Leukaemia Group cclg.org.uk stjude.org If you found it useful, leave a review on iTunes 🌟🌟🌟🌟🌟 and thank you for listening👏🏻👏🏻👏🏻 @fontanellepod

Inconsolable crying. Is it CMPA? Transient lactose intolerance? GORD? Infantile colic?

The majority of babies cry for 2-3 hours a day, and that's completely normal. It's just how babies express themselves!

Aude Cholet, dietician who designed the Hampshire Infant Feeding Guidelines, joins me on this episode of Fontanelle to discuss the treatable causes of excessive crying, which include CMPA, transient lactose intolerance and GORD.

If red flags and treatable causes have been excluded, in a baby under 3 months who is crying for more than 3 hours a day, more than 3 days a week for longer than 3 weeks, you're looking at infantile colic.

Infantile colic has a huge effect on bonding and attachment. It is the most common reason for stopping breast feeding as well as being the leading cause of shaken baby syndrome.

for more information and links go to www.fontanellepod.com

www.what0-18.nhs.uk

https://cks.nice.org.uk/colic-infantile#!topicSummary

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Thank you for listening!

A musical anti-episode of Fontanelle. We're all multi-faceted human beings after all!

More clinical stuff coming soon x x x

Sepsis can be deadly, but difficult to spot. Sepsis is life threatening organ dysfunction characterised by altered physiology. Capilliary leak leads to tachycardia, tachypnoea, reduced oxygen saturations, poor peripheral perfusion, reduced urine output and altered level of consciousness. Sepsis evolves quickly, but is easily confused with common viral infections, which is why we have sepsis screening tools, alerting clinicians to the possibility that this child could be septic. See http://www.sort.nhs.uk/Guidelines/Guidelines-diseases.aspx for the PIER tool developed and used by Thames Valley and Wessex, as well as SORT sepsis management pathway. For Paediatric Sepsis 6 see https://adc.bmj.com/content/99/Suppl_1/A93.2 For parent safetynetting sheets on fever, refer to www.what0-18.nhs.uk . Also worth looking at the NICE Guidelines on both Sepsis and Fever in Under 5s. This podcast explores the recognition and management of sepsis, as well as the difficult job of balancing the need to treat sepsis aggressively with trying to combat the rising use of broad spectrum antibiotics. How can we get better at managing sepsis whilst not overtreating simple viral infections? Please share and leave a review on iTunes 🌟🌟🌟🌟🌟 Find me on Twitter @Fontanellepod . Enjoy the podcast!