scholarly journals Ehlers-Danlos Syndrome: An Analysis of the Current Treatment Options

2020 ◽  
Vol 4;23 (7;4) ◽  
pp. 429-438
Author(s):  
Bo Song
Keyword(s):  
Physical Therapy ◽  
Occupational Therapy ◽  
Current Treatment ◽  
Treatment Modalities ◽  
Comprehensive Treatment ◽  
Alternative Treatments ◽  
Ehlers Danlos Syndrome ◽  
Care Clinic ◽  
Treatment Methods ◽  
Danlos Syndrome

Background: Ehlers-Danlos syndrome (EDS) is a multifaceted disease that can present with a variety of types of pain. Unfortunately, both the mechanisms and treatments for pain are poorly understood. The proposed treatments for the various musculoskeletal pain syndromes in EDS have had variable success, and it becomes much more imperative to better define and evaluate the current treatment modalities in treating this debilitating disease. Objectives: The purpose of this study was to investigate the currently available treatment modalities for patients with EDS and their efficacies in pain and symptom relief. Study Design: Retrospective cohort study. Setting: Institutional physical medicine and rehabilitation primary care clinic. Methods: All patients were seen between January 2015 and April 2019, in which 98 patients with EDS were identified through retrospective chart review. Institutional review board approval was obtained, and all patients provided written consent to be included in the study. We reviewed various treatment modalities, including complimentary/alternative treatments, opioids/opioid-like medications, nonsteroidal antiinflammatory drugs, physical therapy, occupational therapy, muscle relaxants, neuropathic modulators, steroids, surgery/procedures, and acetaminophen. Treatment methods were extracted from individual patient charts, and efficacy was grouped into 3 categories: improvement, no effect, or worsened symptoms. Results: The most common treatments used were complimentary/alternative treatments (n = 88). Occupational therapy and bracing were the most effective options with 70% of patients reporting improvement. Neuropathic modulators were the least well tolerated with 47% of patients reporting adverse effects. Limitations: Men were a small percentage of the study. Patients were not randomized, and pain score reporting was subjective. Patient data were extracted from a single practice setting. Timing and symptom onset were not measured. Conclusions: There is a relative paucity of published literature regarding the various treatment methods for EDS. Although our study is able to identify positive and negative trends with certain modalities, it is vital to understand that EDS is not a uniform diagnosis among patients, and that a combination of several different treatments usually is needed for optimal symptom control. Further research and investigation are necessary to develop a comprehensive treatment database for this complex condition. Key words: Ehlers-Danlos syndrome, pain, hypermobility, arthralgia, subluxation, genetic, physical therapy, interventional pain

scholarly journals Eosinophilic Gastroenteritis in a Patient with Ehlers-Danlos Syndrome - A Rare Combination

1992 ◽  
Vol 6 (3) ◽  
pp. 129-132
Author(s):  
I Al-Traif ◽  
L Jewell ◽  
ABR Thomson
Keyword(s):  
Weight Loss ◽  
Abdominal Pain ◽  
Elemental Diet ◽  
Eosinophilic Gastroenteritis ◽  
Treatment Modalities ◽  
Ehlers Danlos Syndrome ◽  
Gastrointestinal Manifestations ◽  
Rare Combination ◽  
Danlos Syndrome

A case of eosinophilic gastroenteritis in a patient with Ehlers-Danlos syndrome who presented with abdominal pain, vomiting, diarrhea and profound weight loss is presented. Because of inability to tolerate glucocorticosteroids, symptoms were treated with sodium chromoglycate and an elemental diet given by jejunostomy. The patient did well on this program. The gastrointestinal manifestations of eosinophilic gastroenteritis and Ehlers-Danlos syndrome are reviewed as well as the treatment modalities available for eosinophilic gastroenteritis.

scholarly journals Pelvic Girdle Pain, Hypermobility Spectrum Disorder and Hypermobility-Type Ehlers-Danlos Syndrome: A Narrative Literature Review

2020 ◽  
Vol 9 (12) ◽  
pp. 3992
Author(s):  
Ahmed Ali ◽  
Paul Andrzejowski ◽  
Nikolaos K. Kanakaris ◽  
Peter V. Giannoudis
Keyword(s):  
Pelvic Ring ◽  
Patient Reported Outcome Measures ◽  
Pelvic Girdle ◽  
Spectrum Disorder ◽  
Pelvic Girdle Pain ◽  
Treatment Modalities ◽  
Ehlers Danlos Syndrome ◽  
Ongoing Research ◽  
Patient Reported ◽  
Danlos Syndrome

Pelvic girdle pain (PGP) refers specifically to musculoskeletal pain localised to the pelvic ring and can be present at its anterior and/or posterior aspects. Causes such as trauma, infection and pregnancy have been well-established, while patients with hypermobile joints are at greater risk of developing PGP. Research exploring this association is limited and of varying quality. In the present study we report on the incidence, pathophysiology, diagnostic and treatment modalities for PGP in patients suffering from Hypermobility Spectrum Disorder (HSD) and Hypermobility-Type Ehlers-Danlos Syndrome (hEDS). Recommendations are made for clinical practice by elaborating on screening, diagnosis and management of such patients to provide a holistic approach to their care. It appears that this cohort of patients are at greater risk particularly of mental health issues. Moreover over, they may require a multidisciplinary approach for their management. Ongoing research is still required to expand our understanding of the relationship between PGP, HSD and hEDS by appropriately diagnosing patients using the latest updated terminologies and by conducting randomised control trials to compare outcomes of interventions using standardised patient reported outcome measures.

scholarly journals Recognizing and Effectively Managing Hypermobility-Related Conditions

2019 ◽  
Vol 99 (9) ◽  
pp. 1189-1200 ◽  
Author(s):  
Leslie N Russek ◽  
Patricia Stott ◽  
Jane Simmonds
Keyword(s):  
Physical Therapy ◽  
Holistic Approach ◽  
Joint Hypermobility ◽  
Diagnostic Classification ◽  
Joint Hypermobility Syndrome ◽  
Ehlers Danlos Syndrome ◽  
Diagnostic Categories ◽  
Management Concepts ◽  
Diagnostic Classification System ◽  
Danlos Syndrome

AbstractHypermobility spectrum disorder (HSD) and hypermobile Ehlers-Danlos syndrome (hEDS) can cause widespread or chronic pain, fatigue, and proprioceptive and coordination deficits resulting in functional restrictions. These conditions are common and often unrecognized, and patients are likely to present in physical therapy for musculoskeletal injuries, pain, or coordination deficits. Although physical therapy is considered central to managing these conditions, many patients report pain and iatrogenic injuries due to inappropriate interventions. The diagnostic classification for these conditions was revised in 2017 to supersede previous diagnostic categories of Joint Hypermobility Syndrome and Ehlers-Danlos Syndrome—hypermobility type/type III. It is now known that these conditions affect multiple body systems and not just joints and that patients require a holistic approach. This Perspective article will describe the 2017 diagnostic classification system, clinical presentation, examination, evaluation, and management of patients with HSD/hEDS. Both adult and pediatric cases are presented to illustrate the patient management concepts discussed. This knowledge can lead to more effective management of this patient population.

scholarly journals Are patients with hypermobile Ehlers–Danlos syndrome or hypermobility spectrum disorder so different?

2021 ◽  
Author(s):  
Bérengère Aubry-Rozier ◽  
Adrien Schwitzguebel ◽  
Flore Valerio ◽  
Joelle Tanniger ◽  
Célia Paquier ◽  
...  
Keyword(s):  
Neuropathic Pain ◽  
Physical Therapy ◽  
Severity Score ◽  
Multivariable Analysis ◽  
Real Life ◽  
Bone Fragility ◽  
Spectrum Disorder ◽  
Clinical Severity ◽  
Ehlers Danlos Syndrome ◽  
Danlos Syndrome

AbstractDiagnosing hypermobile Ehlers–Danlos syndrome (hEDS) remains challenging, despite new 2017 criteria. Patients not fulfilling these criteria are considered to have hypermobile spectrum disorder (HSD). Our first aim was to evaluate whether patients hEDS were more severely affected and had higher prevalence of extra-articular manifestations than HSD. Second aim was to compare their outcome after coordinated physical therapy. Patients fulfilling hEDS/HSD criteria were included in this real-life prospective cohort (November 2017/April 2019). They completed a 16-item Clinical Severity Score (CSS-16). We recorded bone involvement, neuropathic pain (DN4) and symptoms of mast cell disorders (MCAS) as extra-articular manifestations. After a standardized initial evaluation (T0), all patients were offered the same coordinated physical therapy, were followed-up at 6 months (T1) and at least 1 year later (T2), and were asked whether or not their condition had subjectively improved at T2. We included 97 patients (61 hEDS, 36 HSD). Median age was 40 (range 18–73); 92.7% were females. Three items from CSS-16 (pain, motricity problems, and bleeding) were significantly more severe with hEDS than HSD. Bone fragility, neuropathic pain and MCAS were equally prevalent. At T2 (20 months [range 18–26]) 54% of patients reported improvement (no difference between groups). On multivariable analysis, only family history of hypermobility predicted (favorable) outcome (p = 0.01). hEDS and HDS patients showed similar disease severity score except for pain, motricity problems and bleeding, and similar spectrum of extra-articular manifestations. Long-term improvement was observed in > 50% of patients in both groups. These results add weight to a clinical pragmatic proposition to consider hEDS/HSD as a single entity that requires the same treatments.

Ehlers-Danlos Syndrome, Kyphoscoliotic Type (Formerly Type VI) as Differential Diagnosis to Neuromuscular Diseases

2016 ◽  
Vol 47 (S 01) ◽  
Author(s):  
M. Schroth ◽  
C. Reihle ◽  
M. Wachowsky ◽  
L. Travan ◽  
M. Buob ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Neuromuscular Diseases ◽  
Ehlers Danlos Syndrome ◽  
Danlos Syndrome
Sign in / Sign up

Export Citation Format

Share Document