Poster II How to prevent and treat Ehlers Danlos syndrome A medico social emergency A tewnty years experience Hamonet. Manicourt .pdf
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Syndromes Ghent (Belgium) September 26–29, 2018
How to prevent and treat Ehlers-Danlos syndrome?
A medico-social emergency. A twenty years
Claude Hamonet* ** MD, PhD, Daniel Manicourt***, MD, PhD
*Department of medicine, University Paris-East-Creteil (UPEC), 8 rue du Général
Sarail 94010 Créteil France. **Ehlers-Danlos Clinics, Prevention and Health Center,
29 bis rue d'Astorg 75008 Paris. *** Rheumatology Department, Universitary Hospital
Introduction: state of arts and arguments.
If it is true that, for the past twenty years, a renewed interest in this disease has
been observed, then the second meeting of Ghent is one of its expressions. It
consequences. Today Ehlers-Danlos is never diagnosed despite its frequency
(or because of it? By trivialization?) And, above all, even if the diagnosis is
made or suspected, no proper treatment is offered. At most the patient is
directed towards pain centers that aggravate the symptoms by treating them
with psychiatric medications (especially antidepressants) or with opioids that
have very negative effects on these patients. In other cases, the patient is
advised to see a psychiatrist or a physiotherapist who, by ignorance of the
syndrome, will more often than not, worsen the patient's state. We must add
diagnosis errors with unfortunate consequences: multiple sclerosis, Crohn's
endometriosis ... Two iatrogenic complications are particularly serious:
psychiatrization and orthopedic surgery, mainly cervical, which is very rarely
justified. During the two last decades, we implemented a treatment protocol with
proven efficacy in over 5,000 patients. We have to improve proprioceptive defects
that account for many symptoms. EDS patients are also at increased risks for
complications because of their weakened connective tissues.
The "typical" medical prescription for a patient with an
1-Screening for complications: Look for gallstones (frequent and dangerous) as
well as for aneurysms (RMI): cerebral or the aorta and its branches (brachiocephalic
2-Contraindications: morphine, corticosteroids, anticoagulants, pregabalin, high
doses antidepressants, neuroleptics, cervical manipulations, endoscopy, intraarticular injections (except capsulitis in shoulder dystrophy), lumbar or arterial
punctures (except epidural), orthopedic surgery (except for very rare exceptions).
3-Prefer a diet low in gluten. Eat salty food
4-Maintain physical activity for an hour a day minimum (Dr. Amoretti);
Kinesitherapy isometric and proprioceptive; psychomotor skills; occupational therapy;
Tai Chi; Walk, jog, skate, swimming, dance, cycle, ride a horse, take up archery... No
sport is contraindicated, the provoked pains are. Have fun!
5- Strengthen proprioception and reduce pain to increase functional
independence: mattresses, memory foam seat or back cushions, plantar
ortheses, lumbar belts, shoulder scarf, knee, elbow, wrist, hand and fingers
ortheses, specially developed for EDS compression garments, K-taping, elastic
bands after sprains to avoid immobilization favoring dysproprioception. Walk
ortheses (pelvis, hips, knees, ankle), molded seats (children), rigid lower limbs
orthosis (anti-pain and correction of retractions of the lower limbs) for children
especially. Buccal gutters. Prisms correcting diplopia.
«Three points» belt » Garments E.Vlaminck orthopedist
Walk orthoses. By Rami Haidar, Orthesist
Molded seats Rami Haidar
The fragile EDS joint: Shoulder. Using of K Taping ant Montreal scarf
6-Oxygenation of tissues (brain and muscles) Intermittent oxygen therapy, several
times a day. 1-5 liter/min. Very active on fatigue, headaches, shortness of breath
7-Local treatment of pains. Local injections of anesthetics such as
lidocaine/procaïne for tendinous and muscular pain points. Lidocaine patches on
the skin or gel on the skin and especially on the mucous membranes: the gums and
the vagina for dyspareunia. Transcutaneous delivery of anti-inflammatory drugs,
TENS, heat treatment, cryotherapy local or general at very low temperatures,
massage, stretching, pressotherapy for lymphedema,
8- Pain treatments through parenteral way and others: Nefopam (20mg, 6 per
day maximum), a powerful painkiller well-adapted to EDS. Baclofen (10 à 30 mg
par jour). Anti-inflammatory drugs with gastric protection. L-Carnitine. Relaxation.
Hypnosis. MEOPA for luxation reduction or dental care. Cannabinoids, not yet
authorized in France, have positive effects.
9-Dystonia (57%) on pain and movement control. Low doses of L-Dopa (125 mg
in the morning and 62.5 mg in the evening) or another anti-dystonic drug. Botulinum
toxin for localized dystonic crises (limbs, face or bladder).
10-Other symptomatic treatments. Vitamin D 3,000 IU a day, Vitamin C, 1 g. per
day. Melatonin (insomnias) 4 to 8 mg. Solifenacin (pollakiuria). self-catheterization
(urinary retention), esophageal anti-reflux treatment (twice a day), Biprosolol (in
Fludrocortisone in case of severe hypotension and DHEA for intense tiredness,
Speech therapy is very useful for swallowing disorders, dysphonia and cognitive
rehabilitation (memory, attention concentration) according to the methods used for
brain injury. Antihistamines if there are manifestations, especially cutaneous, of mast
cell Activation Syndrome. Actions on the intestinal microbiota.
11-Anxiety is almost constant (Bulbena) A psychological support is very useful
especially in teenagers. A low-dose intake of venlafaxine (Bulbena) may be
helpful. Dyslexia is common and, in addition to EDS treatment, requires
psychomotor therapists, occupational therapists and speech therapists, in
conjunction with the school. Hyperactivity may require Methylphenidate or
Ritalin which often has very positive effects. Autistic behavior is often
observed and raises the question of the link between Ehlers-Danlos and
Autism / Asperger for which EDS treatments have beneficial behavioral effects.
8-Technical aids for functional and situational autonomy. Walker, Wheel chair,
electric wheelchair, assistance dog, adaptation of the environment and... human
1- Hamonet Cl., Laouar R., Vienne M., Brissot R., Bernard J.-C., Comberg A.,
Vêtements compressifs et syndrome d’Ehlers-Danlos. Étude multicentrique et
prospective sur 49 personnes du handicap avec le Handitest. Journal de
réadaptation médicale, no 4 2010; 30:pp 184–191.
2- Audrey Sultan, Christophe Chesneau, Pierre Denise, Stéphane Besnard, Boris
Bienvenue, Emma G. Dupuy, Pascale Leconte, Elodie Vlamynck, EhlersDanlos Syndrome, Hypermobility Type: Impact of Somatosensory Orthoses on
Postural Control (A Pilot Study). Front. Hum. Neurosci., 08 June 2017 |
3- C. Hamonet, M. Vienne, C. Leroux, M.P. Letinaud, B. Dehecq, A. Metlaine, I.
Brock, F.M. Bird, Manifestations respiratoires dans le syndrome d’EhlersDanlos (SED), Nouveaux apports thérapeutiques, Journal de Réadaptation
médcicale, 2016 ;36 :56-61
Thanks to Doctor:Lucette Ducret for her efficient help