Introduction of the meeting

"The International Symposium on the Ehlers-Danlos Syndromes (EDS) is a state-of-the-art meeting in which new research on clinical advances and the molecular and pathogenic mechanisms underlying EDS and related syndromes is discussed. We offer a high quality scientific program with the general theme: “Interaction and signaling: recurrent themes in the molecular mechanisms of EDS.” The meeting brings together an international panel of clinicians, clinical and basic scientists and representatives of patients support groups to foster constructive discussions and multidisciplinary debates that focus on the multiple aspects of the Ehlers-Danlos syndromes and associated pathologies."

Introduction

Though the EDS clinic knows a renewed interest, there is little information about EDS therapy. During the two last decades, we implemented a treatment protocol with proven efficacy in over 5,000 patients. We have to improve the proprioceptive defects that account for many symptoms. EDS patients are also at increased risks for complications because of their weakened connective tissues.

The EDS prescriptions

1-Prevention.

Maintain physical activity for an hour a day; Kinesitherapy isometric and proprioceptive; psychomotor skills; occupational therapy; Tai Chi; Walk, jog, skate, cycle, ride a horse, take up archery. Have fun!

Look for gallstones as well as for cerebral and aortic aneurysms.

Avoid morphine, corticosteroids, anticoagulants, antidepressants, neuroleptics, cervical manipulations, endoscopy, lumbar or arterial punctures, orthopedic surgery.

Refer a diet low in gluten.

2-Proprioception.

Supports engineered with shape retention memory.

Foot orthotics, lumbar belts, kneepads, Orthotics hands, and fingers, compression garments developed for EDS, K-taping, compression stockings, Shoulder Support scarf, elastic bands

Orthotics walk, molded seats, rest orthosis, correction of retractions of the lower limbs

Buccal gutters, prisms correcting diplopia

3-Oxygenation of tissues (brain).

Intermittent oxygen therapy, several times a day. 1-5 liter/min. Very active on fatigue, headaches, shortness of breath.

4-Treatment of pain.

Local injection of anesthetics such as lidocaine for tendinous and muscular pain points, gums, vagina.

Transcutaneous delivery of anesthetics and anti-inflammatory drugs, TENS, heat treatment, cryotherapy at very low temperatures, massage, stretching, pressotherapy for lymphedema

Nefopam, a powerful painkiller well-adapted to EDS

L-Carnitine, Baclofen, anti-inflammatory drugs with gastric protection

5-Dystonia (57%) on pain and Movement Control.

Low doses of L-Dopa (125 mg LP in the morning and 62.5 mg in the evening). Botulinum toxin for localized dystonic crises.

6-Other drugs.

Vitamin D 3,000 IU a day. Melatonin 4 to 8 mg. Solifenacin for pollakiuria. self-catheterization for urinary retention, anti-reflux treatment, and constipation prevention.

7-Anxiety, hyperactivity or possible autistic behavior: Psychological support and psychiatric treatment (Methylphenidate)

8-Supports to Independence program: Walker, electric wheelchair, assistance dog, human assistance, adaptations of the environment.

Conclusion

Our battery of therapeutic products keeps growing with new classes of agents such as anti-MCAS agents, intestinal microbiota, and cannabinoids.

Key-words

Ehlers-Danlos, treatments, prevention, fragility, proprioception, iatrogeny, orthoses, oxygenotherapy, dystony, L-Dopa.