I posted this information in 2017, but I think it’s worth another look. It gives a good overview of what patients with this connective tissue disorder have to deal with.
Below is a collection of PubMed articles discussing 6 (there are many more) different medical problems that people with EDS have to deal with.
Problems with Local Anesthetic
Local anesthetic failure in joint hypermobility syndrome ; Alan J Hakim, Rodney Grahame, Paul Norris, and Colin Hopper; J R Soc Med. 2005 Feb; – full-text PMC article
When taking biopsies to assess skin strength in Ehlers–Danlos syndrome type III (EDS–III), a Danish group noticed that the patients experienced much pain despite conventional local anaesthesia. When asked, all these patients reported previous experience of partial or complete failure of local anaesthesia in dental or obstetric procedures—for which reason some had been dismissed as hysterics.
Pursuing this finding, Arendt-Nielsen et al. compared the effects of local anaesthesia in 8 patients with EDS–III and 8 controls. Although the patients did gain analgesia from intradermal lidocaine the duration of effect was much shorter than in controls. EDS–III (now known as EDS–hypermobility type) is regarded by many authorities as identical to joint hypermobility syndrome (JHS).
We wish to draw attention to the possibility of resistance to local anaesthesia in individuals with this common and under-diagnosed condition.
Joint hypermobility syndrome (JHS)/Ehlers-Danlos syndrome hypermobility type (EDS-HT) is the most common hereditary non-inflammatory disorder of connective tissue, characterized by a wide range of symptoms, mainly joint hyperextensibility and musculoskeletal symptoms.
A majority of patients also experiences gastrointestinal (GI) symptoms.
Furthermore, JHS/EDS-HT has specifically been shown to be highly prevalent in patients with functional GI disorders, such as functional dyspepsia and irritable bowel syndrome
The aim of this review was to examine the nature of GI symptoms and their underlying pathophysiology in JHS/EDS-HT.
Observations summarized in this review may furthermore represent the first step toward the identification of a new pathophysiological basis for a substantial subgroup of patients with functional GI disorders.
[It’s a darn shame that the price of access to medical journals has become ridiculously high. Even universities are forced to cancel subscriptions they’ve carried for decades. Even purchasing just one article to read often costs $40. I’d be happy to pay $1 (and for some even $5) to access a full article]
Nationwide population-based cohort study of psychiatric disorders in individuals with Ehlers–Danlos syndrome or hypermobility syndrome and their siblings
Individuals with EDS and hypermobility syndrome are at increased risks of being diagnosed with psychiatric disorders.
These risk increases may have a genetic and/or early environmental background as suggested by evidence showing that siblings to patients have elevated risks of certain psychiatric disorders.
Problems with Physical Therapy for EDS
EDS-HT/JHS-associated pain correlates with poor quality of life.
While physical therapy is the recommended treatment for EDS-HT/JHS, little is known about therapy-related patient experiences and iatrogenic injuries.
We studied 38 adult EDS-HT/JHS patients, eliciting health-related quality of life (HRQoL) from 28 patients through the RAND SF-36 questionnaire. We also explored physical therapy experiences through focus groups with 13 patients.
Our patients displayed poor HRQoL, with 71% reporting worse health over the past year.
Focus groups identified factors associated with:
- negative past physical therapy experiences,
- iatrogenic joint injuries,
- positive treatment experiences, and
- unmet rehabilitation needs.
- This group of EDS-HT/JHS patients has
- significant decrements in HRQoL and
- many unmet treatment needs,
- as well as arisk for iatrogenic injuries.
We identify several approaches to help meet patients’ needs and improve joint rehabilitation in patients with EDS-HT/JHS
This would be so useful for patients and physical therapists – If only it was affordable
Problems with Bones
Bone involvement in adult patients affected with Ehlers-Danlos syndrome. – PubMed – NCBI ; – Osteoporos Int. 2016 Aug
The Ehlers-Danlos syndrome is characterized by abnormal connective tissue but bone involvement is debated. We found a reduced BMD and bone quality and increased prevalence of asymptomatic vertebral fractures in eugonadal patients with Ehlers-Danlos syndrome. These findings suggest the need of a bone health evaluation in these patients.
The Ehlers-Danlos (EDS) syndrome is characterized by abnormalities of the connective tissue leading to ligamentous laxity and skin and tissue fragility.
We evaluated the
- bone metabolism,
- bone mineral density (BMD) and
- bone quality (measured by trabecular bone score, TBS), and
- the prevalence of vertebral fractures (VFx)
in a group of eugonadal adult EDS patients.
Fifty consecutive Caucasian patients, aged 30-50 years (36 females, 14 males) with classical or hypermobility EDS and 50 age-, gender-, and body mass index (BMI)-matched control subjects were enrolled.
In all subjects’ calcium-phosphorous metabolism, bone turnover, BMD at the lumbar spine (LS) and femur (femoral neck, FN and total femur, FT) and TBS by dual-energy X-ray absorptiometry, and the VFx presence by spine radiograph were assessed.
Patients showed reduced BMD and increased prevalence of morphometric VFx than controls,
while vitamin D levels, calcium-phosphorous metabolism, and bone turnover were comparable.
Fractured EDS patients showed lower TBS values than non-fractured ones, despite comparable BMD.
In EDS patients, the VFx presence was significantly associated with TBS even after adjusting for sex, age, BMD, EDS type, and falls frequency.
EDS patients have reduced BMD and bone quality (as measured by TBS) and increased prevalence of VFx.
Previous studies have reported an increased prevalence of osteoporosis in Ehlers–Danlos syndrome (EDS), but these were limited by a small number of patients and lack of information on fragility fractures.
In this cross-sectional study, we evaluated the prevalence of radiological vertebral fractures (by quantitative morphometry) and bone mineral density (BMD, at lumbar spine, total hip and femoral neck by dual-energy X-ray absorptiometry) in 52 consecutive patients with EDS and 197 control subjects attending an outpatient bone clinic.
EDS patients were also evaluated for back pain by numeric pain rating scale (NRS- 11).
- Vertebral fractures were significantly more prevalent in EDS as compared to the control subjects (38.5% vs. 5.1%; p b 0.001) without significant differences in BMD at either skeletal sites.
- In EDS patients, the prevalence of vertebral fractures was not significantly (p = 0.72) different between classic and hypermobility types.
- BMD was not significantly different between fractured and non-fractured EDS patients either at lumbar spine (p = 0.14), total hip (p=0.08), or femoral neck (p=0.21).
- Severe back pain(≥7 NRS)was more frequent in EDS patients with vertebral fractures as compared to those without fractures (60% vs. 28%; p=0.04).
In conclusion, this is the first study showing high prevalence of vertebral fractures in a relatively large population of EDS patients.
Vertebral fractures were associated with more severe back pain suggesting a potential involvement of skeletal fragility in determining poor quality of life.
The lack of correlation between vertebral fractures and BMD is consistent with the hypothesis that bone quality may be impaired in EDS.
Problems with Autonomic Nervous System
Dysautonomia and its underlying mechanisms in the hypermobility type of Ehlers-Danlos syndrome. – PubMed – NCBI ; Semin Arthritis Rheum. 2014 Aug
OBJECTIVES: Many non-musculoskeletal complaints in EDS-HT may be related to dysautonomia. This study therefore aims to investigate whether dysautonomia is present and to explore the underlying mechanisms.
METHODS: A total of 39 females with EDS-HT and 35 age-matched controls underwent autonomic function testing
RESULTS: The EDS-HT group showed autonomic deregulation with increased sympathetic activity at rest and reduced sympathetic reactivity to stimuli.
Increased resting activity was indicated by a
- higher LF/HF ratio compared to controls
- decreased reactivity by a greater BP fall during valsalva
- and a smaller initial diastolic BP increase during tilt
Orthostatic intolerance was significantly more prevalent in EDS-HT than controls (74% vs 34%) and was most frequently expressed as postural orthostatic tachycardia.
Lowered QSART responses suggest that sympathetic neurogenic dysfunction is common in patients (p < 0.013), which may explain the dysautonomia in EDS-HT.
Further, connective tissue laxity and vasoactive medication use were identified as important factors in aggravating dysautonomia (p < 0.035).
Dysautonomia consisting of cardiovascular and sudomotor dysfunction is present in EDS-HT.
Neuropathy, connective tissue laxity, and vasoactive medication probably play a role in its development.
This study provides insight into the profile and importance of autonomic symptoms in the hypermobility type (HT) of Ehlers-Danlos syndrome (EDS). The impact of these symptoms is put into perspective by comparing with fibromyalgia (FM) and two other EDS types.
METHODS: Overall, 80 patients with EDS-HT participated, as well as 11 with classical EDS (cEDS), seven with vascular EDS (vEDS), 38 with FM, and 43 healthy controls.
The total autonomic symptom burden was higher in EDS-HT (57.9 ± 21.57) than in controls (11.3 ± 19.22), cEDS (32.3 ± 19.47), and vEDS(29.1 ± 19.18), but comparable to FM (53.8 ± 19.85).
Especially orthostatic and gastrointestinal complaints were prevalent
The importance of autonomic symptoms in EDS-HT was emphasized by the correlation with
- lowered QOL (r = -0.402),
- fatigue (r = 0.304), and
- pain severity (r = 0.370).
Although affective distress and decreased physical activity are often suggested as possible causes for dysautonomia, the ASP did not correlate with the HADS and Baecke score.
By contrast, the correlation of the GHQ (r = 0.298) and PDQ (r = 0.413) with the ASP supports the hypothesis that joint hypermobility and neuropathy may play a role in the development of autonomic symptoms.
CONCLUSION: Autonomic symptoms, especially orthostatic and gastrointestinal complaints, are frequent extraarticular manifestations of EDS-HT and contribute to the disease burden.
Problems Living with EDS
This article presents a large-scale study that reports
- the lived experience of EDS-HT patients,
- the broad range of symptoms that individuals with EDS-HT experience, and
- the impact these symptoms have on daily functioning.
A 237-item online survey, including validated questions regarding pain and depression, was developed.
Four hundred sixty-six (466) adults (90% female, 52% college or higher degree) with a self-reported diagnosis of EDS-HT made in a clinic or hospital were included.
Using self-reports makes this study lose much of its value, but it’s clear that they were able to recruit far more people for much less money in this design.
The data is less than 100% reliable, but some trends are clear:
The most frequently reported symptoms were
- joint pain (99%),
- hypermobility (99%), and
- limb pain (91%).
They also reported a high frequency of other conditions including
- chronic fatigue (82%),
- anxiety (73%),
- depression (69%), and
- fibromyalgia (42%).
Forty-six percent of respondents reported constant pain often described as aching and tiring/exhausting.
Despite multiple interventions and therapies, many individuals (53%) indicated that their diagnosis negatively affected their ability to work or attend school.
Our results show that individuals with EDS-HT can experience a wide array of symptoms and co-morbid conditions.
The degree of constant pain and disability experienced by the majority of EDS-HT respondents is striking and illustrates the impact this disorder has on quality of life as well as the clinical challenges inherent in managing this complex connective tissue disorder.