Discussion
This report highlights a novel approach to finding a practical solution to facilitate pre-operative physiotherapy prior to hip surgery for DDH in a patient with multiple problems that were presenting as a barrier to exercise engagement. The use of DMO® garments could be considered as an intervention to allow improved function in people with hEDS and PoTs. A garment which encompasses the abdomen and lower body, and exerts a compressive force, has previously been shown to reduce heart rate and improve PoTS symptoms in adult patients [42]. Reduced lower limb proprioception is a common problem in hEDS patients [43] and joint hypermobility has been noted to be a key factor in hip dysplasia [44]; thus, an understanding of these elements can help support this group of patients. This case study builds on Dupuy et al’s pilot study improving postural control with DMOs® in people with hEDS [17]. Fatigue is a common debilitating complaint associated with PoTs [4, 45], and emerging evidence supports the use of compression garments to improve fatigue [46].
We have highlighted the multisystemic nature of hEDS, and the importance of a thorough subjective examination, particularly the past medical history and the need for appropriate multi-systemic questioning [38]. Our case report also emphasises the importance of recognising when timely onward referral is required to suitable medical specialists. hEDS patients have often had a long journey to diagnosis, with published research reporting a mean of 14 years elapsing between the first clinical manifestations and the actual diagnosis [47]. Patients therefore often experience depression and anxiety after years of not being listened to and being discredited [20]. To finally have someone that listens and subsequently acknowledges their multiple issues can often be the first step in their recovery.
In the example of the patient used in this case study, inappropriate treatment led to pain-related fear, fear avoidance and further deconditioning. This is unfortunately common in hEDS patients [48]. Pain-related fear occurs when situations that are related to pain are seen as a main threat [49]. This can be fear of movement, fear of injury or reinjury or fear of pain [50]. Avoidance behaviour is defined as postponing or preventing a potentially aversive situation from occurring [51]. Such behaviour will lead to a diminished quality of life as the patient has difficulties in physical functioning, mood and social functioning [52]. hEDS management overlaps with that of Hypermobile Spectrum Disorder (HSD) [11], so these findings can be useful for HSD patients too. The majority of patients with HSD/hEDS have heightened fear of movement [53] and increased vulnerability to injury [38]. Long-term physical inactivity leads to physical deconditioning [54], including decreased muscle strength and cardio-respiratory fitness. Despite studies identifying kinesiophobia as a possible barrier to exercise [53], the mainstay of treatment for HSD/hEDs is exercise and pain management [41]. Literature conveys that these patients already have higher levels of anxiety [55], which may also have a dysautonomia element with symptoms such as hyperventiliation, nausea and light-headedness which are significantly more common in HSD/hEDS patients [56]. Van Meulenbroek et al [57] found that adolescents with asymptomatic generalised joint hypermobility had the same level of physical functioning compared to non-hypermobile controls. The lower levels of physical functioning observed in adolescents with HSD/hEDS could therefore not only be explained by the presence of generalised joint hypermobility; other issues need to be considered. Within clinical practice, treatment needs to address other factors such as pain, fatigue, multisystemic dysfunction, loss of postural control and pain-related fear [47, 50].
Via the use of the DMOs®, we were able to tackle the key factors on this patient’s problem list and bring about positive changes, enabling them with strategies to overcome barriers to exercise. It is important to note that there were no adverse effects reported by the patient on wearing the DMOs®️. Considering appropriate DMOs®/DEFOs for hEDS/HSD patients could provide the individual with a beneficial level of support. This in turn could decrease pain, improve function and increase confidence in their ability. This confidence might allow them to begin a carefully graduated, patient-specific strengthening programme, thus minimising fear-avoidance and potential injury; and in this specific case report, prepare physically for upcoming major surgery. Not only can the garments be used for the musculoskeletal system, but as this case indicates, they are also potentially beneficial in addressing the multi systemic nature of HSD/hEDS by reducing PoTS symptoms that in this instance was a safety concern with imminent orthopaedic surgery [58]. It was paramount in this case to ensure the patient had a reduced risk of fainting and falling to avoid undue stress to her peri-acetabular osteotomy and the newly forming bony union [40]. This case study demonstrates the need for further robust studies into the use of these orthoses for individual joint problems, multiple joint issues, chronic fatigue and dysautonomia.