Issue 14

Ehlers-Danlos Syndrome [EDS] is a relatively rare inherited disorder of connective tissue resulting from an abnormality of collagen - a body protein. Twelve forms of the syndrome have now been identified with the range of symptoms resulting from both the type of inheritance and actual fault in the collagen.

The fault may lie in all body tissues involving skin, bone and blood vessels or it may affect only a few isolated tissues. Ocular, aural, dental, gastro-intestinal, cardio-vascular, urinary, skeletal and rheumatological effects, either in isolation or combination, can be found. The skin may be fragile, splitting and bruising easily with many paper-thin scars. The joints can be hyper mobile leading to instability and spontaneous dislocation. These symptoms lead to early signs of wear and tear, tiring, fatigue and chronic pain. EDS children can also need longer to achieve mobility and other physical skills due to the need to overcome the effects of hypermobility.

Life with EDS needs to be lived as carefully but as usefully as possible. Prevention of accidents and damage is better than cure as healing can take much longer than normal and repeated dislocation compounds the damage to joints. Days need to be carefully planned to not only reduce fatigue, wear and tear but also allow the child as normal a life as possible. It must be noted that an activity which is possible one day will be impossible the next. The effects of EDS certainly vary from day to day.

Initially the child with EDS may look no different from their peers as their difficulties only become apparent during functioning. Others may be noticeable because of their bruising, curled fingers and toes and bendy limbs. Children who have the rarer, life-threatening, vascular type of EDS will have wide set almond eyes and lobeless ears. It is not uncommon for the parents of EDS children to be investigated for child abuse as their children repeatedly arrive at A & E with split skin and haematomas. "He only crawled across the carpet" does not seem a reasonable cause of multiple stitches. Such cuts and splits can be very slow to heal.

What are the functional effects of EDS as seen in the classroom?

Due to instability in wrist, hand and fingers, writing can often be difficult, especially for extended periods. Carrying bags, books, meal trays etc. around school can be very difficult as extra strain is used to stabilize elbows and shoulders. If a child's fingers are very mobile, playing with toys, especially constructional toys, can be extremely difficult.

All sporting activities may cause stress to muscles and joints, with contact sports bringing the additional risk of damage from knocks with peers. Sadly, EDS children love being involved in sports, often because their hypermobility aids them when young, making them very agile in the gym or on the ballet floor.

The child with Ehiers Danlos Syndrome

All activities of personal care can be difficult for the EDS child as fingers have not got the stability or strength to manage buttons and zips. Handling cutlery can be difficult as the finger putting pressure on the knife just

'gives way'. Even once food is in the mouth the children can report difficulty with chewing and sometimes with swallowing. These activities are often even more difficult for girls after puberty, during menstruation.

Academically, EDS children display the usual range of cognitive ability. This is despite EDS often only being included in sections on learning disability in SEN Paediatric texts! There is a suggestion that dyspraxia, dyslexia and autistic spectrum disorder are to be found co-existing with EDS, but research has not yet clarified any connection. Children with EDS may have exhibited a slower development of articulatory and language skills, but they usually catch up. Some EDS children have difficulty maintaining their voice, finding that shouting is not possible.

Due to the nature of the syndrome children need to miss school for hospital appointments, admissions convalescence from procedures, or just because chronic pain and a flare up of symptoms makes attendance impossible. Disturbed sleep patterns are not uncommon as pain makes night time positioning, and therefore good sleep, difficult. Even when at school children may still tire easily and be subject to the effects of any medication given to alleviate their various symptoms. Although splinting is given to aid stability it can also impede functioning, making activities more difficult.

It goes without saying that a child who needs to overcome any of these difficulties to function may have psychological and emotional issues. However it must be said that, although a good sense of humour is not recognised as a clinical feature of EDS, many children and adults with EDS are inclined to minimise the effects of EDS when asked about it and view their lives with humour.

How to help an EDS child in class

Splinting will both stabilise and protect fragile skin and joints, and encouraging the child to use these is important especially at playtimes and when doing sport, even riding a bicycle. Modifying games and sport and having good supervision from a learning support assistant will help. Swimming can be a good activity for a child with EDS, but lifting head from water and breast stroke can be troublesome. Physiotherapy advice on shoes and other mobility aids may be required.

Writing can be made easier by allowing a modified pencil grip, or even building the pencil up with a grip. Having a sloping desk can also make writing easier. For some EDS children continuous writing is painful so a computer is needed for class work. Occupational therapy advice is needed for adaptations to daily activities to allow the child to perform as independently as possible.

EDS children are usually able to carry out most activities providing they can do shorter periods of sustained physical effort with rest periods in between. They may need longer than usual to finish a task, for example dressing and undressing, or eating. Good planning is needed for anyone with EDS.

A child with EDS can have a mix of special needs during their school day, therefore a Statement will help to ensure they get the help they need throughout their school life and into University. It is also important to be aware of guidance given by parents in the care of the child and what action to be taken if the child has an accident. Wrong handling at these times can greatly compound the effect of any injury.

EDS is a hereditary condition, so the child may well come from a family where he or she is not the only person living with pain and disability. At least one parent will be living with similar difficulties with the additional effects of ageing with EDS.

Further sources of help:

Ehlers-Danlos Support Group. Tel: 01252 690940

Email: This e-mail address is being protected from spam bots, you need JavaScript enabled to view it

www.ehlers-danlos.org

This article was written by Angela Hunter of the Hollybank Trust

This article was scanned directly from the SEN Journal Issue 14

Permission has been given from SEN, the Journal for Special Education,