Introduction
The Ehlers-Danlos Syndromes (EDS) are a group of heritable connective tissue disorders characterised by joint hypermobility, tissue fragility and skin extensibility [1]. Hypermobile EDS (hEDS) is the most common subtype, and is associated with pain, activity limitations and participation restrictions [2]. hEDS is diagnosed through clinical examination using the 2017 International Classification [1,3]. Additional consideration must also be given to the comorbidities that exist alongside the main condition due to its multisystemic nature. These include Postural Tachycardia Syndrome (PoTS) [4], Mast Cell Activation Syndrome (MCAS) [5], gastrointestinal issues and bladder and bowel problems [6]. These comorbidities can play an important role when it comes to rehabilitation planning [7]. Comorbidities are associated with worse health outcomes, more complex clinical management, and increased health care costs [8].
This group of patients are poorly recognised and poorly understood, as highlighted in recent studies of healthcare experiences [9, 10, 11]. Physiotherapists play a central role in management, yet many practitioners fail to recognise the complexity of these patients and that inappropriate treatment may be detrimental to their management [7,12]. Existing studies have highlighted the psychological impact of this condition and recommend early detection to assist optimum management [9,13]. Health care practitioners therefore need increased awareness of hEDS.
Dynamic Movement Orthoses (DMOs)® are fabric elastomeric orthotic garments that have strategic reinforcement biomechanical panelling [14] and are a type of Dynamic Elastomeric Fabric Orthoses (DEFO) [15]. Therefore, they may help people with hEDS by exerting a customised paratonic torsional, compressive and supportive effect that could positively influence alignment biomechanics, and neuromuscular activity specific to the individual and their body segments [14]. DEFOs are different to traditional braces which may restrict movement and prevent muscle activity [15]. They can also be individually tailored to the patient’s needs with specific . Case reports have shown their successful use in improving form and/or function in a variety of patients with movement control problems [15,16]. A study of six hEDS patients and six matched controls demonstrated that postural control impairment was partially offset by wearing somatosensory orthoses [17]. Simmonds et al [18] suggested pain, fatigue and fear of injury are common barriers to exercise. Therefore, in the hEDS patient who commonly suffers with pain, impaired proprioception, poor muscle control, increased tissue elasticity [19, 20], and reduced function; DMOs® could be considered as a first line of treatment.
PoTS, a co-morbidity found in EDS [21], is defined as a clinical syndrome lasting at least 6 months that is characterized by: 1) an increase in heart rate ≥30 beats per minute within 5 to 10 min of quiet standing or upright tilt (or ≥40 beats per minute in individuals 12 to 19 years of age); 2) the absence of orthostatic hypotension (>20 mm Hg drop in systolic blood pressure); and 3) frequent symptoms that occur with standing such as light headedness, palpitations, tremulousness, generalized weakness, blurred vision, exercise intolerance, and fatigue [22]. Compression garments have been highlighted as a tool in the management of PoTS [23, 24], therefore the compressive effects of DMOs® might help to reduce symptoms. The management of hEDS must focus on treatment interventions that allow these patients to function, addressing “injury prevention and symptom amelioration rather than a cure” [25].