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Treatment and Diagnosis in Children and Adults with JHS/hEDS

haleysnipes


Research in the area of hypermobility disorders has become more prominent within the last few years. A great deal of information has been uncovered about Joint Hypermobility Syndrome (JHS) and Ehlers-Danlos-Syndrome-hypermobility type (hEDS), but along with this new information has come the need to redefine the treatment, diagnosis, classification, and assessment strategies for hypermobility disorders. Things like the Beighton scale that have traditionally been the basis for diagnosing these disorders are not enough anymore. It has become apparent that quality of life factors must be taken into account as well when diagnosing and treating these disorders.


In this post, we will be summarizing and discussing the article, “The evidence-based rationale for physical therapy treatment of children, adolescents, and adults diagnosed with joint hypermobility syndrome/hypermobile Ehlers Danlos syndrome”


(Disclaimer: I am not a medical professional and all information provided in this post is from the article which is available in the “references” section at the bottom of the page. This post is meant only to provide a synopsis of the information covered in the original article.)


Pain and Health Related Quality of Life

For those who struggle with JHS/hEDS, pain is often present. Although it is unknown why some patients experience pain while others do not, it is hypothesized that localized mechanical overload may be the cause. Repetitive mechanical overload on joints increases the risk of repetitive trauma which, over time, can lead to abnormal movement patterns. These abnormal movement patterns remove the strain from affected joints but, in doing so, transfer the strain onto other joints, which leads to further injury over time.


It has recently been discovered that individuals with JHS/hEDS are characterized not only by chronic pain and generalized joint hypermobility (GJH) but also by the presence of generalized hyperalgesia (GHA). Hyperalgesia is an increased sensitivity to pain and the presence of GHA in JHS/hEDS patients may indicate the involvement of the central nervous system in the development of chronic pain. Nervous system involvement in JHS/hEDS may indicate different diagnostic qualities that may be helpful in clinical practice as well as providing insights into genetic factors of JHS/hEDS.


Proprioception, Muscle Strength, and Balance

Activity limitation and decreased muscle strength are both associated with JHS/hEDS patients. Joint proprioception (awareness of the joint and its movement) is associated with these factors and should be considered when designing treatment plans for patients. Furthermore, these factors have also been found to negatively affect balance in patients.


Extra-Articular Features

The involvement of various body systems that consist of collagen suggests a more systemic rather than localized impact in those with JHS/hEDS. That is, because those who have JHS/hEDS have other body systems that are impacted because of their illness, it suggests that JHS/hEDS are a whole body illness rather than just a joint disease.


JHS/hEDS and developmental coordination disorder (DCD) appear to have some overlap which may have something to do with a neurodevelopmental issue in those who have JHS/hEDS. Furthermore, children with DCD have been shown to have more JHS/hEDS symptoms such as dysautonomic symptoms, double jointedness, easy bruising, joint pain, and flat feet compared to other children. Symptoms of Dysautonomia and/or gastrointestinal dysfunction (GID) may begin very early on in childhood.


Psychological and Physical Impacts on Children With JHS/hEDS

Adolescents and children with JHS/hEDS have been seen to have significantly lower emotional functioning according to the Pediatric Quality of Life Inventory. Additionally, symptoms of JHS/hEDS such as poor proprioception and joint hypermobility are associated with poor gait in children with the condition. Along with poor gait, children with JHS/hEDS tend to have worse aerobic fitness, most likely because of a mix of musculoskeletal pain and deconditioning.


Because of all of the issues that come with JHS/hEDS, many children find it difficult to participate in sports or activities with their peers. This lack of ability to participate can cause significant social difficulties for children. Furthermore, it can be difficult to find hobbies and activities that children enjoy that are also safe for JHS/hEDS.


Conclusion

Although much of this article discusses children with JHS/hEDS, the difficulties described are mostly the same for adults as well. Just like children with JHS/hEDS, adults also have chronic pain, generalized hyperalgesia, autonomic dysfunction, and reduced quality of life. Patients of all ages face challenges in finding hobbies and sports that they can safely participate in. Current research suggests that JHS/hEDS is more complicated than simple hypermobility and a more intensive diagnostic and treatment process should be devised.


References

The Evidence‐Based Rationale for Physical ... - Wiley Online Library. https://onlinelibrary.wiley.com/doi/10.1002/ajmg.c.31545.


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