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Raising awareness of pupils with Ehlers-Danlos syndromes / Hypermobility Spectrum Disorders.

Increasingly Ehlers-Danlos syndromes (EDS) and the closely related Hypermobility Spectrum Disorders (HSD) are being diagnosed in some pupils but very little is known about the impact this has on the pupil, parents/carers and their attainment and attendance in school.

EDS (previously JHS, Joint Hypermobility syndrome) and HSD are heritable disorders that affect the connective tissue found throughout the body. People with these conditions have connective tissue which is defective but each person can present differently, from being asymptomatic to severely disabled. Connective tissues can be thought of as the cement between bricks holding up a house. Some of the cement is in the right proportions, but some is not, therefore too weak or crumbly to support the building. If the cement is weak that is likely to affect other parts of the building e.g. the electrics and waterworks.

There are around 14 types of EDS, most common is hypermobile EDS (hEDS) and has no genetic test. They are thought to be rare disorders but it is difficult to say how rare as sufferers are often not believed and can go undiagnosed.

How hypermobility disorders present

Often ideas of being ‘double jointed’ come up, however the symptoms of EDS/HSD are much broader and can impact on children’s physical, emotional and psychological development. Pupils may extend their limbs much further than average, dislocating or spraining without previous trauma, and they sometimes do not present as acutely in pain. Some have smooth, velvety skin, or very thin skin and liable to significant bruising without trauma. Other symptoms are less visible like:

  • Extreme tiredness
  • Reflux
  • Stomach pain and spasms not linked to anxiety
  • Gastrointestinal issues
  • Vascular issues
  • Neurological issues
  • Dizziness/clumsiness

Drops in blood pressure can cause ‘brain fog’. Pupils can get surges of adrenaline that raise cortisol levels particularly in the co-occurring condition Postural Orthostatic Tachycardia (POTs – this is where there is an abnormal increase in heart rate after standing up) both of which can trigger experiences of anxiety.

Anxiety

There are some studies showing the co-occurrence of EDS/HSDs with allergies, autism, and anxiety (Baeza-Velasco 2018; Casanova, 2018, 2020; Eccles, 2012, 2014, 2015). These present particular challenges as symptoms can flare and be difficult to predict. This unpredictability can lead to anxiety for pupils and parents/carers.

Some students maybe be in a state of ‘fight or flight’, hypervigilant to sensations, injury or pain. Pupils with POTs seem to be more sensitive to the chemical norepinephrine, which can cause symptoms like anxiety. In addition the parasympathetic nervous system, responsible for their ‘relax response’, may not be functioning normally and so symptoms of anxiety are further exacerbated. You can watch this useful video that explores the link between joint hypermobility and anxiety in more depth.

Why this matters for pupils with EDS/HDS

Often, pupils experiencing symptoms have poorer levels of attainment and attendance. Even pupils who do not have Special Educational Needs may be unable to articulate where any pain is coming from. This is because they have always felt it and so think it’s normal or they do not know or understand the internal sensations they are feeling.

In my work as national education lead at the National Autistic Society (NAS) and advisory teacher for autism in a Local Authority, I noticed that pupils with these difficulties often experienced a range of other needs such as toileting issues, motor coordination difficulties, allergies and extreme tiredness. Such difficulties were often labelled or diagnosed as ‘dyspraxia’ though this didn’t seem a good explanation for what I saw in the young people I worked with. Additional difficulties included:

  • Finger grip issues
  • Anxiety
  • Unable to engage in school curriculum
  • Absence from school
  • Unable to sit on chairs comfortable
  • Clumsiness
  • Fainting

EDS and other HSD’s still seem poorly understood within education. Many pupils are home educated as parents/carers can struggle to organise the frequent physio, occupational, and speech therapy out of school hours.

In my experience, reasonable adjustments to timetables are not often made aggravating injuries, extreme tiredness, anxiety and low self-confidence.

Even now with multidisciplinary meetings the dots are sometimes not joined up. Occasionally, parents/carers are positioned as ‘the problem’ due to a lack of awareness and knowledge that EDS/HSD are heritable multi-systemic disorders. Until these syndromes are widely recognised, pupils will continue to be misunderstood and not attain or achieve in education.


References

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 psychiatry9, 656.

Casanova, E. L., Sharp, J. L., Edelson, S. M., Kelly, D. P., & Casanova, M.F. (2018). A Cohort Study Comparing Women with Autism Spectrum Disorder with and without Generalized Joint Hypermobility. Behavioral Sciences8 (3), 35.

Casanova, E.L., Sharp, J.L., Edelson, S.M., Kelly, D.P., Sokhadze, E.M. and Casanova, M.F. (2019). Immune, Autonomic, and Endocrine Dysregulation in Autism and Ehlers-Danlos Syndrome/Hypermobility Spectrum Disorders Versus Unaffected Controls. bioRxiv, p.670661.

Critchley H. D., Garfinkel S. N. (2017). Interoception and emotion. Current Opinion in Psychology, 17, pp. 7-14.

Demmler, J.C., Atkinson, M.D., Reinhold EJ, et al. (2019). Diagnosed prevalence of Ehlers-Danlos syndrome and hypermobility spectrum disorder in Wales, UK: a national electronic cohort study and case–control comparison. BMJ Open, 9.

Eccles, J.A., Beacher, F.D., Gray, M.A., Jones, C.L., Minati, L., Harrison, N.A., & Critchley, H.D. (2012). Brain structure and joint hypermobility: relevance to the expression of psychiatric symptoms. The British journal of psychiatry: the journal of mental science200 (6), pp. 508–509.

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., & Owens, A., Mathias, C., Umeda, S., & Critchley, H. (2015). Neurovisceral phenotypes in the expression of psychiatric symptoms. Frontiers in Neuroscience, 9 (4).

Garfinkel, S. N., & Critchley, H. D. (2013). Interoception, emotion and brain: new insights link internal physiology to social behaviour. Commentary on: “Anterior insular cortex mediates bodily sensibility and social anxiety” by Terasawa et al. (2012). Social cognitive and affective neuroscience, 8 (3), pp. 231-234.

Garfinkel, S. N., Tiley, C., O’Keeffe, S., Harrison, N. A., Seth, A. K., & Critchley, H. D. (2016). Discrepancies between dimensions of interoception in autism: Implications for emotion and anxiety. Biological psychology, 114, pp. 117-126.

Tsakiris, M., & Critchley, H. (2016). Interoception beyond homeostasis: affect, cognition and mental health. Phil. Trans. R. Soc. B, 371.



About Jane Green

Jane passionately advocates for autism and EDS charities (as dx both) plus a Trustee at SCIE, CSWS, SEDS. She was a professional autism educationalist for the Local Authority, NAS and AET plus AHT.She is currently part of the team that develops the EDS/Hypermobility Education Toolkit for Schools.

View all posts by Jane Green



One Comment so far:

  1. Kate says:

    Can I say how wonderful it is to read this. One point I would like to gently bring up, is that often the parent has the same struggles, so advocating and fighting for their child is even more difficult. Thank you for writing this.

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