Hypermobility: Advice for Referrers

Hypermobility refers to an increased range of movement in multiple joints and is very common in the paediatric population. Hypermobility is a normal finding and many individuals with hypermobility do not experience any significant difficulties. It is important that the condition is not over-medicalised.

Hypermobility can describe a wide range of children with flexible joints. Some children may benefit from their hypermobility and are able to use their flexibility to excel in areas such as gymnastics, dance, swimming etc. Most children with hypermobility do not need support with everyday activities.

However, some children may experience difficulties and symptoms which are understood to be related to being hypermobile. They may experience significant pain, joints may dislocate and difficulties can occur when the body has become weak and deconditioned. Symptoms may impact on day-to-day functioning.

Some patients with hypermobility have questions regarding a possible diagnosis of Ehlers Danlos Syndrome (EDS). Criteria has been published (2017 EDS international classification) to help clarify this: hEDS Dx Criteria Checklist. The significant risks associated with other forms of EDS can mistakenly be assumed to also apply to children with exclusively hypermobile symptoms, causing unnecessary distress for families.

Hypermobility has been observed to be particularly common in the neurodivergent population. This highlights the importance of considering the wider picture when reviewing patients with hypermobility.

Please include the following points from history in the referral letter

Joint and/or muscle aches and pains
Joint stiffness
Joint swelling
Recurrent joint dislocation
Systemic symptoms - cardiac/GI/skin/urinary/eyes
Fatigue and poor sleep
Impact on function – self-care, exercise, school attendance, handwriting

Please include the following points from the clinical examination in the referral letter

MSK examination findings
Skin elasticity
Abnormal scarring
Marfanoid features
Full systemic examination
Height/weight, BP

Referral Pathway

Asymptomatic

Reassurance and advice by referrer

Issue patient information leaflet (see attached reference list)

No requirement for onward referral to specialist centre.

Mild Pain with Minimal Functional Limitation

A child who falls into this category is likely to benefit from assessment/education/re-assurance by a paediatric physiotherapist to promote self-management of their joints and encourage an active lifestyle.

Depending on which joint is affected they may benefit from seeing a podiatrist/orthotist

Moderate Pain with Functional Limitation

Presenting symptoms:

Children in this group may present with 1 or more of the following symptoms.

Joint pain
Recurrent dislocation
Reduced muscle power
Reduced endurance/fatigue
Specific functional limitation impacting on abilities at home/school
Reduced fine/gross motor co-ordination (including those who come under the umbrella term of DCD – Developmental Co-ordination Disorder)

Onward Referral pathways:

Paediatric Physiotherapy – for those children with 1 or more of the above symptoms but who are still managing to live a full lifestyle with the exception of sporting pursuits.
Orthotics/Podiatry – as above but primarily for children with ankle/foot problems
Paediatric Occupational Therapy – for those children that have specific difficulties impacting on functional tasks at home/school e.g. handwriting, self-care tool use.
Consultant Paediatrician – for those children whose symptoms are impacting on their quality of life over and above an inability to engage in sports activities, i.e. poor school attendance, avoidance/inability to partake in any physical activity, poor systemic health. Children with functional problems and pain may be referred to a Paediatric Rheumatologist if necessary.

Non-Inflammatory Hereditary Disorders of Connective Tissue

Presenting symptoms:

Children may be more severely affected with:

Recurrent dislocation
Reduced muscle power
Reduced endurance/fatigue
Moderate/severe functional limitation – more dependent on others and increased impact on abilities at home and school
Systemic features i.e. cardiac/GI/skin/urinary/eyes/skeletal etc.

Some examples of non-inflammatory hereditary disorders of connective tissue and some of their systemic features are listed in the table below:

Non-Inflammatory Hereditary Disorder of Connective Tissue	Features
Ehlers Danlos Syndrome	Joint hypermobility, skin elasticity, joint dislocation, abnormal wound healing
Marfan Syndrome	Tall and thin, elongated limbs, joint hypermobility, scoliosis, sternal abnormalities, high arched palate, lens dislocation, cardiovascular involvement, spontaneous pneumothorax
Stickler Syndrome	Characteristic flattened facial appearance, cleft palate, micrognathia, myopia, scoliosis, joint hypermobility, hearing loss
Loeys Dietz Syndrome	Widely spaced eyes, bifid uvula, cleft palate, elongated limbs, abnormal wound healing, sternal abnormalities, joint hypermobility, scoliosis, congenital heart problems

Onward Referral pathways:

For children in this group referral to a Paediatric Rheumatology Clinic would be appropriate. Referral to Clinical Genetics may be warranted if there is a family history. Onward referral to Cardiology or Spinal Surgeons may be appropriate depending on the clinical features.

Following consultation, and dependent on the patient’s needs at the time the patient may receive MDT input from:

Paediatric Physiotherapist
Podiatrist/Orthotist
Paediatric Occupational Therapist
Psychologist
Paediatric Pain team

Suggestions for investigations whilst waiting for outpatient appointment

For the majority of patients presenting with hypermobility bloods will not be necessary, but this will be guided by the clinical picture and can be done if there are other associated signs and symptoms (e.g. excessive pain, fatigue). Please note we would not expect very young children to have bloods done at their GP practice – this can be done with paediatric review.

Suggestions for management whilst waiting for outpatient appointment

Self-management

Explain the importance of maintaining a healthy lifestyle, eating a well-balanced diet, participating in regular gentle exercise, participating fully in school, activities and social events, and getting quality sleep. Sleep Action is a useful resource for information on supporting a good night’s sleep.

A good understanding of pacing activities should be developed by young people, avoiding the “boom and bust” cycle where over-exertion leads to pain and fatigue. A baseline level of activity should be established and maintained and gradually increased. It is well recognised that significant periods of inactivity may exacerbate symptoms.

Writing

Using thicker pens/pencils or grips can help relieve pressure on finger and thumb joints when writing.

Posture

Good posture is important as a slouched posture can cause back pain and lead to weakness in central core muscles.

It is important to use the right style of school bag and to avoid carrying excessively heavy loads as this can lead to back pain. Further information on this is detailed in the parent leaflet below.

Footwear

Children should wear supportive footwear, especially those with knee and foot pain. Shoes should have a firm back of heel and offer arch support. Children with flat feet with foot/ankle pain may benefit from review from orthotics or podiatry for insoles. Further information is detailed in the parent leaflets below.

Useful resources for parents whilst waiting for outpatient appointment

SPARN Advice for Joint Hypermobility in Children:

https://www.nhsggc.org.uk/media/253954/joint-hypermobility.pdf

Parent leaflet - hypermobility:

https://apcp.csp.org.uk/documents/parent-leaflet-hypermobility-hypermobility-spectrum-disorder-2023

Versus Arthritis – hypermobility:

https://www.versusarthritis.org/about-arthritis/conditions/joint-hypermobility/

Parent leaflet – choosing the right school bag:

https://apcp.csp.org.uk/system/files/choosing_the_right_school_bag_2016.pdf

Parent leaflet – choosing footwear for children:

https://apcp.csp.org.uk/system/files/choosing_footwear_for_children_2016.pdf

Parent leaflet – flat feet:

https://apcp.csp.org.uk/system/files/documents/2023-03/Flat%20feet%202023.pdf

Sleep Action:

https://sleepaction.org/

Useful resources for health professionals

British Society of Rheumatology. 2019. Guidance for Management of Symptomatic Hypermobility in Children and Young People – A Guide for Professionals managing Children and Young People with this condition

Malfait, F et al. 2017. The 2017 international classification of the Ehlers–Danlos syndromes. American Journal of Medical Genetics Part C: Seminars in Medical Genetics. Vol. 175. No. 1. 2017.

Csecs, Jenny LL, et al. 2022. Joint hypermobility links neurodivergence to dysautonomia and pain. Frontiers in Psychiatry 12: 786916.

Editorial Information

Last reviewed: 11 December 2024

Next review: 31 December 2027

Author(s): Jackie Keightley, Consultant Paediatrician with an interest in Rheumatology, Royal Hospital for Children, Glasgow

Approved By: RHC Medical Paediatrics