By Sabrina Wells

Movement is an essential part of a child’s every day. From playing on the playground to brushing their teeth, they are continuously coordinating their body to perform precise movements to achieve desired outcomes. The ability to think about a movement and perform it is something known as praxis or motor planning. This skill can become even more difficult when a child also experiences joint hypermobility. Joint hypermobility is when joints move beyond their typical range of motion. Joints are held together by connective tissue, which is made up of collagen, a strong but flexible protein. Children who experience hypermobility will have collagen that is stretchier and weaker, causing joints to be unstable and move beyond their typical limits (Nicholson et al., 2022). Many children have some form of joint hypermobility, but for some this can greatly impact their motor planning and performance. This means they may need to give greater attention to their joint flexibility to minimise risk of injury. It may also mean that everyday movements are more challenging to coordinate. There is growing evidence to support a strong relationship between neurodevelopmental disorders and joint hypermobility (Donaghy et al., 2023; Kindgren et al., 2021). A study investigating the prevalence of joint hypermobility in children with Autism Spectrum Disorder (ASD) by Romeo et al. (2023) found that 73% of children in the cohort aged over 5 years had co-occurring joint hypermobility.
Hypermobility Spectrum Disorder (HSD) is a disorder that can affect children in many different ways including which parts of their body experience hypermobility (Nicholson et al., 2022). For some it may be only in their hands for others it may include their hips, shoulders and knees. Some children may have genetic conditions such as Ehlers-Danlos Syndrome or Marfan Syndrome which require specific medical intervention. If you suspect your child has HSD this can be assessed by your occupational therapist through completion of a Beighton Scale, then further investigated by your Paediatrician for a formal diagnosis.

Symptoms

Children with joint hypermobility syndrome can experience symptoms such as:

  • pain and stiffness in their joints and muscles
  • muscle weakness
  • delay in gross motor skills
  • increased incidence of sprains
  • fatigue more easily than their peers
  • digestive problems like reflux and constipation
  • problems with their bowel or bladder
  • anxiety and reduced self-esteem

Pain, fatigue and reduced self-esteem are often reported as being the most impacting symptoms on children’s participation in everyday occupations (Clark et al., 2024). Children may experience pains and aches which often result from muscles compensating for joint instability and consequently fatiguing (Jeong et al., 2023). At times pain medicine such as paracetamol or ibuprofen can help, but it’s important to get to the root cause of the pain and build the muscle supporting the hypermobile joints.

Fatigue is another symptom that can have a sudden impact on children with HSD, going from all out in team sports to needing days to recover due to the inflammation and muscle tension on the body (Clark et al., 2024). This is a phenomenon known as the ‘boom and bust’ cycle. Managing and reducing the impact of fatigue so children can continue to participate in everyday activities is frequently misunderstood for those with HSD.

The symptoms of HSD can affect a child’s ability to perform and participate in everyday activities. Difficulties with handwriting, playing sport and completing self-care tasks such a dressing can have a profound impact on a child’s self-esteem (Blajwajs et al., 2023; Baets et al., 2022). It is pivotal as parents to continue to encourage your child to build their skills and resilience. It may take some more time and effort, but they can master the skills needed to confidently participate alongside their peers. Having a supportive and encouraging environment around them builds positive self-esteem (Song et al., 2023).

Impact on Function

Symptoms such as pain and fatigue are commonly reported by children in our clinic can impact on their engagement in many vital childhood occupations such as play, sports, dressing, handwriting and meal preparation (Tofts et al., 2023). Let’s look a bit deeper into how HSD impacts these occupations.

  • Play: HSD can delay a child’s motor development and cause floppy movements. Ensuring that play is at an achievable and enjoyable level for the child will support their engagement. Some children with HSD can become disengaged in play due to their symptoms, supporting these children to continue to engage in play will facilitate positive outcomes into adolescence and adulthood.
  • Sports: Due to joints being weaker and having greater range of motion, engaging in high impact sports can pose greater risk of injury for children with HSD. These children may appear clumsier, fall over frequently and have difficulties coordinating body movements. Protective strategies such as wearing joint supports, adequately warming up and cooling down the body alongside monitoring pain and fatigue levels can support children to continue engagement in preferred sports.
  • Dressing: Doing up zips, buttons and laces takes intricate finger work and shoulder stabilisation. For children with HSD coordinating their planned movements may take more practice and time than their peers. Being patient and persistent towards mastering these skills will give these children the self-esteem and resilience needed.
  • Handwriting: For some children with HSD, they may experience reduced control of their pencil strokes and difficulty maintaining grip on the pencil shaft. These children may find it easier to use a thicker pencil style or specialised grips to relieve the pressure on their fingers and thumb.
    Meal Preparation: Like holding a pencil, manipulating and coordinating cutlery to chop foods is a commonly difficulty task for children with HSD. Due to reduced strength in their hands and fingers they may become quickly fatigued when engaging in meal preparation or find cutlery is slipping out of their hands. Practicing appropriate grip and using modified utensils may support in building their performance and confidence in meal preparation.

Strategies from an OT

Occupational therapists are equipped to support children in managing the symptoms of HSD, through individualised and targeted strategies so that they can positively engage in their daily occupations (Palmer et al., 2021). Some frequently used strategies here at Stepping Stones are:

  • Joint Protection: Pain can quickly build if children are not protecting their joints during everyday tasks. At times, this can lead children to become avoidant of certain occupations, such as tying shoelaces, writing, doing up zips, the list goes on. By using joint protection strategies this minimises discomfort and builds confidence. We often recommend avoiding tight grasp, using both hands when possible, maintaining good posture, avoiding staying in one position for prolonged times, and use of larger & stronger muscles and joints where possible.
  • Pacing: To minimise fatigue, pacing strategies are often explored with the child. This consists of planning activities carefully throughout the day or week to keep the intensity low-moderate and having short rest breaks when required. It’s a fine balance of using muscles regularly to build strength and not resting for so long that the muscles become deconditioned which leads to further pain and discomfort.
  • Strengthening & stretching: Building strong muscles to support joint stability is key for children with HSD. Completing stretching warm up and cool downs when engaging in physical activities is important to minimise pain and aches. Engaging in low impact sports such as gymnastics, pilates, swimming, and bike riding are great for strengthening muscles whilst reducing potential flares in pain.
  • Education: We provide coaching and support to children in how to appropriately care for their body and joints alongside supporting parents in identifying necessary changes to the child’s day to day to support their HSD. This may be identifying poor posture, appropriate grip and manual handling along with assistance with identifying impacts of other symptoms.
  • Occupation Modification: Children who experience severe pain or fatigue may benefit from occupation modification. This means that how the child completes handwriting or dressing may need to be modified. This can be through the use of assistive devices (such as buttons hooks or slant boards) or by learning a different way of completing the task (such as different method of tying shoelaces or using an adaptive pencil grip).
  • Further Resources:
    Joint hypermobility – information for children and young people | CUH
    Joint hypermobility factsheet | The Sydney Children’s Hospitals Network (nsw.gov.au)
    What is HSD? – The Ehlers Danlos Society (ehlers-danlos.com)

References
Song, J.Z., Luong, D., Feldman, E.C.H., Tran, S., Perrier, L., Eubanks., K., Bayley, M., Kastner, M., Slepian, M., & Munce, S. (2023). Psychological interventions for individuals with Ehlers-Danlos syndrome and hypermobility spectrum disorder: a scoping review. Orphanet J Rare Dis, 18, 254. https://doi.org/10.1186/s13023-023-02799-y
Clark, N.L., Kainth, G.S., Johnson, M., Rangan, A., Kottam, L., & Swainston, K. (2024). Psychological interventions to improve pain, fatigue, anxiety, depression, and quality of life in children and adults with hypermobility spectrum disorders and Ehlers-Danlos syndrome: a systematic review. Rheumatol Int, 44, 41–55. https://doi.org/10.1007/s00296-023-05503-2
Jeong, H. J., Engel, J. M., Wilwert, O., Muriello, M., Basel, D., & Slavens, B. A. (2023). Pain Characteristics and Symptom Management in Children with Hypermobile Ehlers–Danlos Syndrome and Hypermobility Spectrum Disorder. Physical & Occupational Therapy In Pediatrics, 43(5), 630–643. https://doi.org/10.1080/01942638.2022.2163601
Hornsby, Elizabeth (2023). Insights into the assessment and management of children with generalised joint hypermobility and secondary musculoskeletal pain. PhD Thesis, School of Health and Rehabilitation Sciences, The University of Queensland. https://doi.org/10.14264/a751bfe
Blajwajs, L., Williams, J., Timmons, W., & Sproule, J. (2023). Hypermobility prevalence, measurements, and outcomes in childhood, adolescence, and emerging adulthood: a systematic review. Rheumatol Int, 43, 1423–1444. https://doi.org/10.1007/s00296-023-05338-x
Palmer, S., Davey, I., Oliver, L., Preece, A., Sowerby, L., & House, S. (2021). The effectiveness of conservative interventions for the management of syndromic hypermobility: a systematic literature review. Clin Rheumatol, 40, 1113–1129. https://doi.org/10.1007/s10067-020-05284-0
Baets, S., Temmerman, M., Calders, P., Malfiat, F., Hove, G., Venderstraeten, G., Wandele, I., & Velde, D. (2022). The impact of hypermobile “Ehlers-Danlos Syndrome” and hypermobile spectrum disorder on interpersonal interactions and relationships. Frontiers Rehabilitation Science, 3. https://doi.org/10.3389/fresc.2022.832806
Kindgren, E., Quiñones Perez, A., & Knez, R. (2021). Prevalence of ADHD and Autism Spectrum Disorder in Children with Hypermobility Spectrum Disorders or Hypermobile Ehlers-Danlos Syndrome: A Retrospective Study. Neuropsychiatric Disease and Treatment, 17, 379–388. https://doi.org/10.2147/NDT.S290494
Tofts, L.J., Simmonds, J., Schwartz, S., Richheimer, R., O’Connor, C., Elias, E., Engelbert, R., Cleary, K., Tinkle, B., Kline, A., Hakim, A., van Rossum., M., & Racey, V. (2023). Pediatric joint hypermobility: a diagnostic framework and narrative review. Orphanet J Rare Dis, 18, 104. https://doi.org/10.1186/s13023-023-02717-2

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