What schools need to know to support learners with hypermobility and Ehlers-Danlos syndrome

with Jane Green     

Ehlers Danlos syndrome comes in many guises and as such, it often takes a long time for people with this multi-symptomatic condition to be diagnosed. I should know: I was 46 when the random pains, dizziness and fatigue I'd always had, blew up into enduring disability. My two (adult) children also have it to varying degrees and, as an advisor for the Ehlers Danlos Society, my knowledge of the condition really helped when it came to helping their school understand too.

EDS has many sub-types, some of which are extremely rare, but it is hEDS, (hypermobile type) and the related Joint Hypermobility syndrome, that are far more common. There also seems to be a correlation with neurodiversity.

Jane Green is chair/founder of the award-winning SussexEDS (SEDS) support group and a self-advocacy autism group. She is disabled, autistic and a carer.  She has developed a new school toolkit to help teachers support children with hypermobility/Ehlers Danlos syndrome and is here to tell us ore about it

How the new School Toolkit for EDS and hypermobility can help. 

Ehlers Danlos syndrome (EDS) and Joint Hypermobility syndrome (JHS) are heritable disorders that affect the connective tissues found throughout the body. People with these conditions have defective connective tissues, but everyone is differently affected and as yet, there is no genetic test for this type of EDS.

We know that EDS is thought to affect at least one in every 5000 people and JHS is more common for children and young people. These syndromes are often misunderstood and probably much more common than this statistic shows, as they're easily missed or misdiagnosed. There are very few EDS studies on children which may be because of the unique complexity of the disorders and uncommon representation of symptoms, leading to incorrect assumptions that of a psychosomatic origin. 

This also means very little is known about the impact this has on children and adolescents, their attendance and attainment in schools. They are often labelled as non-attenders for anxiety reasons. The issues are often layered under other diagnoses such as developmental coordination disorders (DCD), dyspraxia, or neurodevelopmental conditions (autism), sometimes ADHD or emotional anxiety, a leading cause in Emotionally Based School Avoidance (EBSA) and non-attendance.  

Double-jointed clues

Often, ideas of being 'double-jointed' are the initial image of hypermobility, but it has a much broader impact on children's physical, emotional and psychological development. Children might be able to extend their limbs much further than normal.  They might often dislocate or sublux, (half dislocating but goes back in), or sprain without a previous accident. They might not even recognise it has happened, or that they are in pain.   This is often found in neurodivergent young people who have challenges with their senses particularly the inner senses, interoception (how their body is feeling) and proprioception (the knowledge of where they are in space - seeming clumsy). Also, alexyithema (inability to identify and describe their emotions) can play a part in not recognising the pain.

Other less-visible symptoms include: 

• Extreme tiredness
• Reflux
• Heartburn
• Stomach pain and spasms not linked to anxiety
• Gastrointestinal issues
• Vascular issues 
• Muscle tightness
• Dizziness/clumsiness
• Temperature issues
• Neurological issues
• Pain acute, persistent
• ANS Autonomic dysfunction -palpitations/anxiety

Drops in blood pressure can cause ‘brain fog’.  Other difficulties are:

• Finger grip issues
• A poor swallow reflex
• Reflux
• Gastroesophageal reflux disease (GERD)
• Fainting (PoTS is closely related)
• Clumsiness
• Headaches/migraine

Headache and migraine pain is thought to be due to chronic neck strain, jaw pain and issues to do with the nervous system. Possibly dehydration due to poor blood flow. Children may also experience a range of toileting issues, motor coordination difficulties, allergies from eczema to anaphylaxis, extreme tiredness, anxiety and occurrences of dizziness or fainting. Such experiences were often labelled or diagnosed as 'dyspraxia' now (DCD) and 'fidgety' from ADHD or autism (Jelsma 2013).

However, when I was a SEND teacher, this didn't seem a reasonable explanation for all I observed. According to Kaplan et al (2001) 'overlapping difficulties in two or more developmental and educational domains implies that discrete diagnosis of a single disorder is often not appropriate.'

Difficulty finding treatments

Parents/carers can struggle to access regular physio, occupational, and speech therapy either during or outside school hours. This can also lead to anxiety and challenges, even before Covid. It isn't uncommon for wounds, bruising, dislocations, fractures, and sprains to occur without any preceding incident, which can affect school attendance. Reasonable adjustments to timetables are not made, leading to injuries, extreme tiredness or anxiety, as well as low self-confidence. 

The ongoing challenges of accessing and organising medical appointments, and of managing a wide range of symptoms, may mean families feel they have to home educate, as their child's needs are not met in school. Occasionally, parents/carers are considered part of  'the problem' (with allegations of deliberate harm/ FII), due to a lack of understanding that symptomatic hypermobility such as EDS are multi-systemic disorders. Until these syndromes are widely recognised, children will continue to be misunderstood and may not attain or achieve their true potential in education. 

The School Toolkit for hypermobility

I've worked in all phases of education as an autism lead. My passion was to help education staff to know about hypermobility issues and the co-occurrences with neurodevelopmental differences. So I'm delighted to have been able to create and develop this school toolkit for EDS and JHS.

It's aimed at schools across the UK and includes an overview of what EDS and JHS are, a diagram of common symptoms, and some of the most common ways these conditions can impact pupils' functional and academic participation. It also includes advice on reasonable adjustments and how to support hypermobile children such as moving around safely in school, reading, writing and studying, physical education, toileting, wearing school uniform, school trips and exams. It is a free resource.

The EDS and JHS school toolkit is available at http://theschooltoolkit.org/. It was funded by Ehlers-Danlos Support UK with funders D’Oyly Care Charitable Trust and The Peter Harrison Foundation with collaboration from HMSA.

About Jane Green

Jane Green MA Ed. Adv.Dip.Ed.(Child dev.), PGCE QTS, BSc (HONS)Psych. Jane is a professional educationalist and champions hypermobility EDS, JHS and autism/ND.  She was previously a SEND teacher, LA autism advisory teacher, Lead designer/trainer for AET, lead educationalist consultant for NAS/AET, across UK and London Boroughs. She is currently the first autistic Trustee at SCIE, CSWS and an autism group. Policy lead for training medical would be medical doctors. NIHR theme adviser, Strategic Oversight group CoChair of the OMMT and  Chair/founder of the award-winning SussexEDS (SEDS) and a self-advocacy autism group. She is disabled, autistic and a carer.

Refs: 

• Adib, N., Davies, K., Grahame, R., Woo, P., & Murray K.J. (2005) Joint hypermobility syndrome in childhood. A not so benign multisystem disorder? Rheumatology, 44 (6), pp. 744–750,
• Baeza-Velasco, C., Cohen, D., Hamonet, C., Vlamynck, E., Diaz, L., Cravero, C., & Guinchat, V. (2018). Autism, Joint Hypermobility-Related Disorders and Pain. Frontiers in psychiatry, 9, 656.
• Clark, C. J., Carr, E. C.J. and Breen, A. C., 2011. Exploring joint hypermobility syndrome, developmental coordination disorder and pain.In: 2nd European Sensory Integration Conference, 27-29 May 2011, Algarve, Portugal. (Unpublished)
• https://edpsy.org.uk/blog/2020/raising-awareness-of-pupils-with-ehlers-danlos-syndromes-hypermobility-spectrum-disorders/Green, J accessed July 2020.
• Green J in NASEN extracted copy https://www.sussexeds.com/post/understanding-hypermobility-disorders-syndromes-in-schools
• Eccles, J., Iodice, V., Dowell, N, et al. (2014). Joint hypermobility and autonomic hyperactivity: relevance to neurodevelopmental disorders. Journal of Neurology, Neurosurgery & Psychiatry, 85:e3.
• Eccles,  J (2020) https://www.medrxiv.org/content/10.1101/2020.09.14.20194118v1.article-infoAccessed 25.9.20
• https://link.springer.com/chapter/10.1007%2F978-981-10-1750-6_45
• Jelsma, L. D., Geuze, R. H., Klerks, M. H., Niemeijer, A. S., & Smits-Engelsman, B. C. (2013). The relationship between joint mobility and motor performance in children with and without the diagnosis of developmental coordination disorder.  BMC pediatrics, 13, 35. https://doi.org/10.1186/1471-2431-13-35
• Kaplan et al 2001 in C, John-Adubasim. (2019). Dyslexia, Neurodevelopmental Conditions and Comobidity: A Rule Rather than an Exception. Archives in Neurology & Neuroscience. 4. 10.33552/ANN.2019.04.000585.
• https://bnpa.org.uk/wp-content/uploads/2021/02/BNPA-datablitz-posters-Georgia-Savage-BNPA-poster.pdf

Also read:

• What’s in a name: Ehlers-Danlos Syndrome gets an important makeover
• Ten Top tips for when your child is diagnosed with a rare disease
• #RareDisease Day 15: Rare with a twist makes a complex medical mystery
• A 16 year quest to diagnose our rare bendy daughter
• The power of pacing: Managing energy with a rare disease or chronic illness
• Targeted next-generation sequencing makes new molecular diagnoses and expands genotype-phenotype relationship in Ehlers-Danlos syndrome
• Psychiatric disorders in Ehlers–Danlos syndrome are frequent, diverse and strongly associated with pain
• How to help manage your children’s pain

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Tania Tirraoro

Founder, Co-Director at Special Needs Jungle

Founder of Special Needs Jungle. (She/Her) Parent of two young adults with autism. New grandma. Tania, who is autistic, is a leading SEND advisor, working with the Special Education Consortium, Ofsted, and Whole School SEND. She is also an Advisory Board member of the Royal Holloway, University of London Centre of Gene and Cell Therapy and an Editorial Board member of Springer Nature Gene Therapy journal.
Tania is an experienced broadcast and print journalist & author. She also runs a PR, web & social media consultancy, SocialOro Media.
Tania is a rare disease & chronic pain patient advocate with Ehlers Danlos syndrome.

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