Schools need to be more aware of Ehlers-Danlos syndromes (EDS) and hypermobility spectrum disorders (HSD) to better support pupils diagnosed with the conditions, argues Jane Green.
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 that 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, it can be too weak or crumbly to support the building. And if the cement is weak, that is likely to affect other parts of the building, eg, the electrics and waterworks.
There are around 14 types of EDS; most common is hypermobile EDS (hEDS) and this 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 affect 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 children have smooth, velvety skin, or very thin skin that is liable to significant bruising without trauma. Other symptoms are less visible, such as:
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 syndrome (PoTS – this is where there is an abnormal increase in heart rate after standing up), both of which can trigger experiences of anxiety.
The anxiety link
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 may be in a fight-or-flight state, hypervigilant to sensations, injury or pain. Pupils with PoTS seem to be more sensitive to the chemical norepinephrine, which can cause symptoms such as anxiety.
In addition, the parasympathetic nervous system, responsible for the "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 or disability 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 comfortably
Clumsiness
Fainting
EDS and other HSDs still seem poorly understood within education. Many pupils are home educated as parents/carers can struggle to organise the frequent physio, occupational and speech therapies 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 owing to a lack of awareness and knowledge that EDS/HSD are heritable multisystemic disorders.
Until these syndromes are widely recognised, pupils will continue to be misunderstood and not attain or achieve in education.
Jane Green is a former national education lead at the National Autistic Society (NAS) and advisory teacher for autism in a local authority. She now volunteers for hypermobility charities.
This article originally appeared on edpsy.org.uk and is republished here with its kind permission and the permission of the author Jane Green References
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