Ehlers-Danlos Syndrome Hypermobility Type
Conditions
Keywords
Ehlers-Danlos syndrome, hypermobility, Compression garment, pain, isokinetic evaluation
Brief summary
The Ehlers-Danlos syndrome (EDS) is a rare genetic disease caused by an anomaly of the connective tissue. This syndrome has different forms. Hypermobility type is the most common. It is characterized by generalized joint hypermobility, associated with a chronic pain syndrome. Pains appear as the most frequent demonstration of this syndrome, the worst lived and the most crippling. Faced with insufficient effect of drug treatment, other alternatives have been proposed to relieve these patients. Compression garments have been developed. They appear as medical devices targeted specifically for patients with hypermobility EDS type: in the symptomatic treatment of joint pain, in stabilizing joints by proprioceptive effect and of muscle globulization, and in the functional improvement. The VETCOSED study is a quasi-experimental study of type Before / After, monocentric, open. The study is realized in the Centre Médico-Chirurgical et de Réadaptation des Massues - Croix Rouge Française de Lyon, in association with the Pôle Information Médicale Evaluation Recherche des Hospices Civils de Lyon. This study concerns about 40 patients with EDS hypermobility type, which will be followed for 8 weeks: 4 weeks during which they will wear a compression custom-made garment (short-sleeved vest CICATREX SED®), and 4 weeks during which they do not carry this garment. The main objective is to demonstrate the effectiveness of compression garments in the EDS hypermobility type, especially in terms of symptomatic improvement joint pain. The main outcomes expected of this study are: * an improvement of the power and the peaks of couple of muscles rotators of shoulders. * a decrease of the pains and the defects of articular stability of the shoulder * an improvement of the quality of life and the functional independence of these patients
Interventions
DEVICEshort-sleeved vest CICATREX SED®)
Each patient wears a compression custom-made garment (short-sleeved vest CICATREX SED®) according to the medical prescription during the first 4 weeks (BEFORE phase). Then each patient should not wear the compression garment for the next 4 weeks (AFTER phase).
Sponsors
Hospices Civils de Lyon
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
SUPPORTIVE_CARE
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Ehlers-Danlos syndrome hypermobility type confirmed the diagnosis of a geneticist * with an instability of one or both shoulders, painful or considered invalidating by the patient (recurrent dislocation or subluxation : at least once / month or at least 12 times / year) * having a joint hypermobility syndrome (abnormal mobility and in a non-physiological axis), with a Beighton score of greater than or equal to 5 of 9 * age greater than or equal to 18 years * size greater than or equal to 1 m 40 * sufficient understanding of the French language in order to respond to self-report questionnaires
Exclusion criteria
* hyperalgic episode in progress * unstable cardiac disease * previous history of shoulder surgery * severe skin lesions, * pregnancy in progress
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Power (in watts) of the external rotators of the shoulder of the dominant arm measured by concentric isokinetic evaluation at a constant angular velocity of 180 °/s | 2 months follow-up | The evaluations will be conducted 3 times: * at the inclusion (D0) * at the end of the BEFORE phase (4 weeks with the compression garment: D0 + 28) * at the end of the AFTER phase (4 weeks without the compression garment: D0 + 56) For each measurement, evaluations will be made with then without the compression garment. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Level of pain expressed in the shoulders of patients | D0,D28,D56 | Evaluation of the level of pain each month by the physician investigator on Visual Analogue Scale : * at the inclusion (D0) * at the end of the BEFORE phase (4 weeks with the compression garment: D0 + 28) * at the end of the AFTER phase (4 weeks without the compression garment: D0 + 56) |
| Score of quality of life measured by the medical outcomes study Short-Form General Health Survey (SF-36 scale) | D0,D28,D56 | The score of quality of life is measured by self-administered questionnaire (SF-36 scale) at each follow-up visit: * at the inclusion (D0) * at the end of the BEFORE phase (4 weeks with the compression garment: D0 + 28) * at the end of the AFTER phase (4 weeks without the compression garment: D0 + 56) |
| Score of satisfaction measured by the Quebec User Evaluation of Satisfaction with assistive Technology (QUEST) | D0,D28,D56 | The score of satisfaction is measured by self-administered questionnaire (QUEST) at each follow-up visit: * at the inclusion (D0) * at the end of the BEFORE phase (4 weeks with the compression garment: D0 + 28) * at the end of the AFTER phase (4 weeks without the compression garment: D0 + 56) |
| Functional independence score measured by Health Assessment Questionnaire (HAQ) | D0,D28,D56 | The score of functional independence is measured by self-administered questionnaire (HAQ) at each follow-up visit: * at the inclusion (D0) * at the end of the BEFORE phase (4 weeks with the compression garment: D0 + 28) * at the end of the AFTER phase (4 weeks without the compression garment: D0 + 56) |
Countries
France
Outcome results
None listed