Understanding hypermobility in children and young adults 

Estimated reading time: 9 minutesIt is important to remember that most children are hypermobile – this does not mean they have a syndrome or connective tissue disorder. Hypermobility can be asymptomatic, and does not mean joints are unstable. Children under the age of six will not be assessed for any hypermobility syndrome as it is a normal part of life and hypermobility can decrease as they age. If however, after this age problems persist, it is worth investigating further. 

I never considered I was bendy as a child – I could do a full back bend but was never able to do the splits. I wasn’t signing up for gymnastics for sure. But I had trouble writing, holding a pencil, terrible fatigue after school, and coordination issues at school games – I was never picked for netball or tennis! I really lacked upper body strength – I always came last in throwing activities at school.

Back then, I don’t believe there was very much information about hypermobility in children. Now of course we have The SchoolToolkit which is an amazing resource for parents and teachers.

If your child continues to experience hypermobility as they age, and experiences pain as well as multisystemic symptoms like fatigue, headaches, digestive issues, and bladder problems (and more explained below), it is worth consulting with a pediatrician or rheumatologist.

An introduction to joint hypermobility

Hypermobility itself simply refers to an increased range of motion in the joints. This can be acquired affecting one joint or widespread impacting the whole body. It can also be asymptomatic or symptomatic with joint instability, pain, and more.

Sometimes, hypermobility can be a sign of a connective tissue disorder that impacts the structure or function of the connective tissue (1).  Hypermobility syndromes occur when joint hypermobility is a key feature of a combination of symptoms impacting many systems of the body including the gastrointestinal, urogenital, neurological, autonomic, and cardiovascular systems (2).

Some common terms used to describe more generalized or systemic hypermobility conditions include:

• Generalized joint hypermobility (GJH): when the Beighton score is greater than 6/9. It is usually inherited but can be acquired related to inflammatory joint or musculoskeletal disorders (3).
• Hypermobility Spectrum Disorder (HSD): Beighton score of greater than 5/9 in post-pubertal people and greater than 6/9 in people who have not entered puberty. There is GJH but also joint pain, poor proprioception, soft tissue damage, injury, recurrent or chronic pain, fatigue, headaches, GI problems autonomic dysfunction and more (4).
• Ehlers-Danlos syndrome (EDS): There are 13 types of EDS. EDS is clinically characterized by skin hyperextensibility, joint hypermobility, subluxation, dislocation, tissue fragility, vascular fragility (5). Hypermobile EDS (hEDS) is the most common form of Ehlers-Danlos syndrome and currently lacks genetic testing so a diagnosis is clinical.

The following information refers to HSD/hEDS which, as mentioned to not currently have genetic markers approved for diagnostics. A geneticist can help rule out other (Classical EDS) and rare forms of EDS (like Vascular EDS) or other connective tissue disorders.

According to The Pediatric Working Group and members of the hEDS/HSD Working Group of the International Consortium on EDS and HSD, the 2017 diagnostic criteria may be used with biologically mature adolescents, but children do not often have a stable presentation as they grow (6).

As mentioned above, hypermobility is very common in children. Hypermobility decreases with age in childhood normally, and some of the skin features associated with EDS like stretch marks and scarring may have not yet developed (6).

Instead, they propose a more “fluid” diagnosis as children with GJH grow (6).

• If they grow out of their they are considered typical
• If symptoms improve but they remain hypermobile, they can be considered asymptomatic GJH (generalized joint hypermobility).
• If they present new symptoms and GJH they are considered pediatric generalized HSD (pgHSD) which will allow them to get current treatment and support.

The goal of this framework was to identify those with hypermobility as a physical trait while keeping in mind that is relatively common, however if musculoskeletal issues related to hypermobility may mature into a clearer diagnosis of EDS.

You can read more and access the diagnostic framework from The Ehlers-Danlos Society.

Symptomatic hypermobility in children: reported symptoms

There is a myth, which is damaging and sadly still out there in the medical world, that you can be too young to have pain or chronic pain. Children and parents are often dismissed and assumptions are made that the child is attention-seeking or trying to get out of school.

Sadly, I have heard these stories from my clients. Pain does not have an age limit. If we know pain is a major feature of hypermobility syndromes, this must be taken into consideration in our younger patients. Early intervention can help prevent injuries, manage pain, and improve the overall quality of life in children.

Recognising this can help children be better prepared and supported at school. Children may struggle with activities like standing or sitting too long at school, often being told off for fidgeting. With a correct diagnosis, modifications can be made to the environment to make the child’s school experience more pleasant and successful. For example, rest breaks, modified physical education classes, and ergonomic desk set ups.

Often it can be hard to connect all of the symptoms together – but the connective tissue is the most abundant tissue in the body (2). Here are some symptoms that are reported to be common in children with hypermobility syndromes (2, 5, 6):

• Joint instability: joint sprains, subluxations, dislocations
• Musculoskeletal pain
  • A study on children (8-17 years) diagnosed with hEDS, had subjects rate their pain with a validated numeric scale. On average they rated 5.53 which indicated moderate pain on average. This pain was also widespread based on the widespread pain index (7).
  • A review assessed several studies in both EDS and JHS and found pain in children was commonly reported in the back, neck, shoulders, wrists, hips, knees, feet, and small joints (5).
• Gastrointestinal symptoms like nausea, diarrhea, and constipation
• Headaches
• Fatigue (atypical levels for children)
• Orthostatic intolerance (Dysautonomia): symptoms with standing
• Allergies
• Urinary incontinence
• Anxiety

Tofts, et al. (The Pediatric Working Group guidelines paper) also cite numerous studies that report comorbidities commonly found in children and adolescents with hEDS/HSD including developmental coordination disorder, ADHD, Autism/ASD/ASC , heavy menstrual bleeding and moderate to severe pain with menstruation, and adolescent idiopathic scoliosis. Parents also tend to report delayed motor milestones that impact daily activities of life and functioning in school (2).

The Spider Questionnaire has been validated for use in adolescents aged 13-18 (and more recently in adults up to age 65). This is a tool used to assess the impact of the various comorbidities often present in people with HSD/hEDS. The ultimate benefit of this assessment is helping direct and prioritize care (8).

How common is hypermobility?

There is a lot of variation in reporting of how common hypermobility is. This is probably due to the variation in the presentation of hypermobility which can change with gender, ethnicity, previous injuries, and strength training (9).

• In children, GJH has been reported to effect 34% of children and adolescents globally based on a systematic review and meta-analysis of 20 research studies (10).
• One report found the prevalence of HSD/hEDS to be 10 cases per 5000 people in the UK (11).
• Ewer, et al. found that in children there is a great variation in reports from 4%-65%, and they say the variation can be due to age, ethnicity, sex assigned at birth, and what criteria were used for the diagnosis.

Despite being a potentially common condition is thought to be underdiagnosed (12).

The benefit of early diagnosis

In children, diagnosis of hypermobility syndrome can be more difficult because joint hypermobility is a normal trait in children. It can be hard to distinguish normal hypermobility from an underlying connective tissue disorder (6). While symptoms from EDS can occur as early in life, people are not often diagnosed until later in life – some being diagnosed in adolescence or early adulthood. This leads to the reports of frequent delays in receiving care (5).

While it can be challenging, early diagnosis can be very beneficial. Some doctors do not want to ‘label’ children but this approach can prevent an appropriate management plan from being put in place that includes exercise therapy, physiotherapy, and occupational therapy. Chronic pain has been cited as the most common symptom in children and pain management should be included early on as a management strategy (13).

Early diagnosis could help with injury prevention – start working on exercises to support the joints. Appropriate strategies could also help prevent chronic pain syndromes and fear of movement. If a child continually sprains an ankle, for example, the impact on that child’s movement patterns and participation in activities could be affected.

Managing pain and pacing activity

Learning to pace at a young age is a valuable tool – I wish I had learned about it sooner. Balancing activity and rest to prevent overexertion physically and mentally can be a valuable tool to manage energy levels and reduce pain.

With this technique, children could learn to recognize the signs of overexertion and give themselves permission to rest without feeling guilty or bad about it. Pacing can also help us avoid flare ups – thereby avoiding the boom and bust cycle of exercise that can be experienced by everyone.

Importantly, I believe it helps develop self-management skills. Recognsing how our body feels, we can understand our limits, rather than pushing through pain and fatigue. It builds that all important body awareness from an early age. It can also help avoid things like repetitive strain injuries – maybe too much writing at school for example – if we teach children to recognize when to stop and take a break, it could prevent injuries.

Other management strategies that may benefit children include (2):

• Working on sleep hygiene
• Managing pain by working with a pediatric pain specialist
• Occupational therapy to find ways to adjust daily activities to meet specific needs
• Physical therapy with an EDS-aware pediatric physical therapist
  • Both PT and OT may be able to help with strategies like bracing, taping, and splinting.
• Starting a practice of mindfulness and self-compassion early in life could be hugely beneficial
• Anxiety is so common in hEDS/G-HSD, especially in adolescents. Working with a Psychologist or mental health provider can be hugely beneficial.
  • Hypermobility is 3 times higher in children with anxiety (8-15 years old) than those without anxiety (14).

Recommended exercises and activities

I have worked with many children and I believe you need to keep the exercises fun and engaging. We are looking to improve joint stability, body awareness, control, and coordination, and build strength, but this can be done in a fun way.

• Fun tools: I like to use balance pads, hedgehogs, soft balls, bands, and tennis balls as part of a movement programme. If we can gamify it, even better for engagement levels.
• Pilates is a great way to build on all of these things.
  • There was a 2020 systematic review that looked at the benefits of Pilates for children (15). They concluded that Pilates did appear to improve flexibility, strength and postural control, and reduce pain in children with musculoskeletal pathology. This was not specifically a study of hypermobile children, but Pilates is a form of movement that can be modified to be suitable for hypermobility. Here is a link to a video with my hypermobile son where we are working on control and balance together.
• If the parents or siblings can get involved in any exercise programme prescribed, it makes it more engaging for the child. In fact, we have many parents in The Zebra Club who do the classes with their children.
• Other activities can include swimming, cycling and hiking, dance, and Tai Chi. But it is important to remember every child is individual with individual needs. Find something that engages them personally.
• Keep movement safe and fun. When we become fearful of movement and stop moving, this can in turn exacerbate pain and fatigue (16).

Importance of proper footwear

Children’s hypermobile feet often have excessive flexibility in the ligaments and joints of the foot and ankle. Ankle issues are very common in hypermobility.  I often work on balancing exercises with children to help build strength and awareness of the foot.  Building a strong base from an early age could be invaluable to future issues as an adult. Spikey ball foot massages to stimulate the feet, heel raises, standing on one leg – all good examples of how to start waking up our feet.

Custom orthotics have been reported to be beneficial in children. In this study, children with GJH, aged 5-18 were given custom orthotics. Based on questionnaires, the children reported significantly reduced lower limb pain, improved health-related quality of life, improved functional capacity, and improved fatigue after wearing orthotics for 1 month. The pain further improved after 3 months (3).

A final note

You know your child best, and you know when something isn’t right. Unfortunately, getting them the care they need isn’t always the easiest. In a survey of parents of children with diagnosed or suspected HSD/hEDS, the understanding of HSD/hEDS among health professionals is one of the most reported negative variables they’ve experienced when it comes to their children’s healthcare (12).

It is heartening that awareness is improving so much and that children are receiving diagnoses earlier than previous generations. It may be helpful to speak with other parents of children with hypermobility, especially when you are managing these conditions in your own body. The Zebra Club has a Prenatal, Parenting, and Beyond subgroup where members who are parents can discuss aspects of both parenting with these conditions, and parenting those who have these conditions.

Below is a video filmed with my son with an idea for a kid-friendly exercise.

FAQ

What disorders are associated with hypermobility?

When hypermobility is symptomatic and is also present with more body-wide symptoms it can be associated with connective tissue disorders like Ehlers-Danlos Syndrome, Hypermobility Spectrum Disorder, Marfan Syndrome, and more.

Is hypermobility a disability in a child?

Hypermobility is not necessarily a disability. In fact, hypermobility itself is quite common in children and can be hard to distinguish from an underlying connective tissue disorder. When there are more system-wide symptoms you may begin to suspect a connective tissue disorder – if you can’t “connect the issues think connective tissues.”  Connective tissue disorders also exist on a spectrum with some being more affected and having more potentially disabling symptoms.

What sports are good for kids with hypermobility?

Find a sport that is engaging and fun for the children. High-impact sports tend to be on the more risky side – as injuries, subluxations, and sprains could occur more in a hypermobile body.  Rugby, American football, and ice hockey could fall into these categories – but staying active is also hugely beneficial.