Reviewed by Evan Peterson PT, DPT
Mobility over the last several years has been a source of topic when discussing general health and wellness. Many people believe the more mobile a joint is the better off it will be. There are varying degrees to which a joint may be mobile. A hypomobile joint is one in which the bones do not glide or roll as well as one would expect. The opposite is for a joint to be hypermobile. Joint hypermobility is the excess to which a joint has the ability to go through its range. Increased joint play is not always bad, at times it can be advantageous. However, increased mobility may lead to increased risk for injury or be a sign of systemic disease. These diseases are known as Heritable Disorders of Connective Tissue (HDCT). The 2 most common of them is Ehler’s Danlos Syndrome (hEDS) and Hypermobility Spectrum Disorder (HSD).
Where do we see hypermobility?
A variety of factors can influence who gets hypermobility, including age, gender, muscle tone, joint shape, injury, and systemic disease. More often than not, children are more mobile and as they age they become less mobile. Due to the aging affects on joint mobility, scoring tools must be adjusted to determine the severity. Factors such as injury or training can play a factor in the development in mono-articular joint hypermobility. For example, the article notes swimmers tend to have knee extension hypermobility and gymnasts seem to have greater hip hypermobility.
To identify hypermobility, it is important to examine both active a passive range of motion (ROM) in joints. Several screening tools have also been developed to look for generalized hypermobility. The most commonly utilized is the Beighton scale which is a 9-item list. Scores of 6 or more in 5-year olds to skeletal maturity, 5 or more in adults to 50 years of age, and 4 or more when older than 50, is positive. Along with the screening tool, a 5-part questionnaire is typically attached. This is the 5-part hypermobility questionnaire which asks questions such as “Do you consider yourself double jointed” and “Can you now (or could you ever) bend your thumb to touch your forearms?”. Despite this screening tool, it does not completely rule out generalized or other forms of hypermobility. Specifically, the temporomandibular, shoulder, hip, cervical, and ankle, joints should be paid close attention. Other recommended screening tools are the Upper Limb Hypermobility Assessment Tool (ULHAT) and the Lower Limb Assessment Score (LLAS). The LLAS is the only one of the two which has been validated for the pediatric and adult; whereas, the ULHAT has been validated for adults.
Diagnosing Hypermobility Disorders
The most common diagnosis of hypermobility in the past was Joint Hypermobility Disorder; however, most recently hypermobile Ehler’s Danlos Syndrome (hEDS) and Joint hypermobility (JH) are thought of as a spectrum ranging from asymptomatic JH to hEDS. Filling the middle of this spectrum is HSD. The importance of recognizing HSD is to understand that it may appear in a variety of different ways: localized, peripheralized, generalized, or historic. Historic refers to someone who had joint hypermobility in the past but due to an injury lost their ability to demonstrate increased mobility.
The severity of both HSD and hEDS present across a spectrum and can be complex or present with minimal symptoms. Symptoms typically are musculoskeletal pain, fatigue, dysautonomia, gastrointestinal, urogenital, and cognitive symptoms. Central sensitization has also been found to be a common symptom. Other common findings alongside hEDS and HSD are postural tachycardic syndrome (POTS) and Mast Cell Activation Syndrome. The link between hypermobility and these disorders are not fully understand at this time.
Dysfunction in hEDS or HSD typically are related to pain, fatigue, or psychosocial stress. In children it is common to also have developmental coordination disorder which can persist into adulthood. A key aspect of hEDS is that it is a first-degree family diagnosis which can be picked up in the subjective. Due to systemic symptoms, therapist may need to refer out to other specialists for more well-rounded care. When performing physical aspect of assessment pay close attention to end ranges of motion due to increased risk of aggravating pain. It is also recommended to perform tests such as the LLAS or ULHAT. The author suggests tests, such as the navicular drop test, are indicated to further assess mobility of foot and ankle joints. Therapist should pay close attention to neuromuscular control, balance, and proprioception as these have all been shown to be associated with hypermobility disorders. If finding symptoms of dizziness, chest pain or syncope, therapist should take blood pressure and heart rate. Greater than a 30-beat increase in heart rate upon standing in adults is suggestive of POTS; greater than 40 beats in children. When examining children, it is important to address quality of movements as well which may not be observed when utilizing the BOT-2 or Movement ABC.
Evaluation, Diagnosis, Management
For hEDS to be diagnosed 3 criteria must be met. They must have generalized joint hypermobility, systemic manifestations of a connective tissue disorder, and the exclusion of other reasons for the current symptoms. After diagnosis is made, the therapist must address the severity of the disorder in order to establish if a multi-disciplinary approach is required. One of the most important interventions is education. Patients who understand their triggers for exacerbation can more effectively reduce symptoms when flare ups occur. It is also important to understand various strategies to protect joints as well as manage subluxations/dislocations. Other suggestions are adding compression clothing in order to enhance proprioception. Exercise is the mainstay in management as it has been shown to decrease spinal pain, joint pain, and improved neuromuscular control of movements. Often patients with hEDS respond well to strengthening programs but require gradual progressions in order not to aggravate muscle and tendon. In patients with POTS, it is also important to understand their decreased tolerance for exercise and increased fatigue following exercise. Patient’s should be educated on ways to mitigate symptoms such as fist clenching. It is recommended to begin with recumbent exercises and lower extremity tasks to promote venous return.
As is evident by the above presented information patient with hypermobility disorders would benefit greatly from skilled intervention focusing on a variety of factors. It is important to address each of the impairments through exercise and education. It is also important to recognize when a person requires a multi-disciplinary approach for overall improvement in symptom management.
Physical Therapy First
We here at Physical Therapy First are trained to examine a variety of patients who presents with symptoms related to hypermobility. At PTF, therapists are experienced utilizing scales, such as the Beighton scale, and work with patients to create exercise routines appropriate for the individual. We understand the many factors that may present themselves in a person with hEDS and can tailor strategies to decrease pain and improve functionality. If you are someone who was diagnosed with hEDS or feel that you are hypermobile and have increased pain, please do not hesitate to reach out to the therapists at Physical Therapy First.
Simmonds, J. V. (2022). Masterclass: Hypermobility and hypermobility related disorders. Musculoskeletal Science and Practice, 57, 102465. https://doi.org/10.1016/j.msksp.2021.102465