What is the toolkit?
A free resource for school staff to help support pupils with joint hypermobility syndrome (JHS) or the Ehlers-Danlos syndromes (EDS) with leading content and research from Jane Green in partnership with EDS UK and HMSA.
What are hypermobility, JHS and EDS?
How Joint hypermobility syndrome (JHS) and the Ehlers-Danlos syndromes (EDS) affect pupils at school...
Hypermobility is quite common in the general population, particularly in children and is not usually a problem on its own. Sometimes it is associated with long-term, widespread pain and other problems. This is called symptomatic hypermobility and can be a sign of joint hypermobility syndrome (JHS) or connective tissue disorders such as Ehlers-Danlos syndrome (EDS).
There are different types of EDS, each with distinct body-wide symptoms which can be difficult to recognise and can vary widely from person to person.
JHS and EDS may cause problems for pupils at school and these are likely to occur before a diagnosis has been given.
Some people may be able to extend their joints further than is usual – this is known as being hypermobile or double-jointed. Hypermobility is quite common in the general population, especially in children and, on its own, is not necessarily a problem. However, hypermobility can be associated with long-term, widespread pain, frequent dislocations, damage to the tissues surrounding joints and fatigue. When hypermobility comes with these problems it is called symptomatic hypermobility. People who are hypermobile may also struggle to sense the position of their joints (this is called having poor proprioception). This can make them appear clumsy and can lead to falls and other injuries.
The Ehlers-Danlos syndromes (EDS)
EDS are a group of genetic (heritable) conditions affecting the connective tissue which is found throughout the body. Connective tissue holds joints and internal organs in place and is an important structural component of skin, blood vessels and other tissues. In EDS, a certain kind of connective tissue (collagen) is fragile and stretchy. This can cause symptoms throughout the body. The different types of EDS are quite distinct from each other. While some signs and symptoms are specific to certain types, there are some (such as symptomatic hypermobility) that are common to most types of EDS. Vascular EDS (vEDS) is different to the other types of EDS and can lead to sudden, life-threatening events.
Joint hypermobility syndrome, closely related to EDS
Joint hypermobility syndrome (JHS) is a condition involving symptomatic hypermobility. It is thought to be closely related to some types of EDS. It is more common for children to be defined as having symptomatic hypermobility or JHS than EDS. People with JHS can display similar symptoms to people with some types of EDS. The differences between the two are not important from a school’s perspective and we suggest pupils with symptomatic hypermobility, JHS and EDS be treated and managed in the same way except for individual differences.
Effects throughout the body
JHS and EDS are complex syndromes affecting many systems of the body at once. Despite this, they are often invisible. In addition to problems associated with hypermobility, those affected may also have skin which damages easily, bruises and is slow to heal, or alternatively can be thick and velvety. Different symptoms and their severity vary considerably from person to person and are unique, even in the same type of EDS and within the same family. Some other conditions seem to be more common in people with JHS and EDS compared to the general population but we can’t say for certain that they are linked.
Individuals with JHS or some types of EDS can experience symptoms related to the abnormal function of the autonomic (involuntary or automatic) nervous system. The term autonomic dysfunction is used. It is not yet clear how JHS or EDS and autonomic dysfunction are linked. In some people with autonomic dysfunction, symptoms come on when they are upright and are mostly relieved by lying down. This is called orthostatic intolerance or postural orthostatic intolerance (PoTS). Symptoms of PoTS include palpitations (a sensation of your heart pounding in your chest), light headedness or dizziness, sweating, fainting, fatigue and headaches. They can also affect the gut (where nausea, diarrhoea, constipation, bloating, abdominal pain and vomiting can be experienced), concentration and vision (excessive glare, blurred or tunnel vision).
There is evidence of an association between hypermobility and anxiety but the relationship is not yet fully understood.
Common symptoms of JHS and EDS
Symptoms of JHS and EDS are widespread and can vary greatly from child to child. The diagram below shows the symptoms of the most common type of EDS, called hypermobile EDS (hEDS). There are additional distinctive symptoms for the rarer types of EDS.
How do JHS and EDS affect pupils at school?
Symptoms can vary widely between pupils
Fatigue caused by the conditions may manifest as slowness which gradually gets worse throughout the day or bouts of extreme tiredness causing the pupil to fall asleep
Particularly severe symptoms may affect a pupils attendance, as will the frequent medical appointments which often come with having the conditions.
Symptoms can vary widely between pupils with JHS and EDS, even in those from within the same family. Similarly, the same pupil may experience huge variation in how their condition affects them day by day and over their time at school.
Pupils with JHS or EDS may present as being distracted, fidgety and uncooperative in class. This could be caused by a number of factors including pain, the need to move for joints to be comfortable and/or to stop muscle spasms, difficulty writing, tiredness or anxiety.
Tiredness caused by JHS or EDS may present as slowness in the body and in thinking, which gradually gets worse throughout the day. Sometimes bouts of extreme tiredness might cause the pupil to fall asleep. Pupils who have postural tachycardia syndrome (PoTS), which can be associated with JHS and EDS, may experience worse tiredness in the morning but this may also be prolonged after a PoTS flare.
Pupils who are in pain may experience this as a general persistent pain or as muscle spasms or sensory pain. It may appear the pupil is able to attain set targets at times but at other times they may appear ‘brain fogged’ – difficulty thinking or concentrating.
Pupils may not be able to keep up with their peers in the playground, may be less able to participate in social activities outside of school and may experience anxiety making maintaining friendships challenging. They may need extra emotional understanding and support.
Particularly severe symptoms may affect a pupil’s attendance, as will the frequent medical appointments which often come with having the conditions. Most pupils with JHS or EDS want to fully participate in school life but find it too challenging physically and mentally in the school environment.
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