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.