Transcript Size: 1 Mo.) The Ehlers-Danlos Syndrome (EDS). Bird meeting

Ehlers-Danlos Syndrome
EDS
Hôtel-Dieu de Paris
Bird meeting
ASED 06 09 2012
Claude Hamonet*, Monique
Vienne **
*Department of Physical Medicine and
Rehabilitation (Pr. S. Poiraudeau, Dr. JY
Maigne), Hôtel-Dieu Hospital, Paris
**IMAP, Nice.
With support of Department of Public health
and Epidemiology (Pr. Ravaud), Hôtel-Dieu
Hospital Paris.
Grant : Fondation de France.
Welcome Dr. Bird, you safe the life
of our patients or you enhance
their quality of life
André Grossiord
The pionner of French
Rehabilitation. Garches.
First description : a law student. By
Edward EHLERS, a Dermatologist,
Danish Society of
Dermatology
Copenhaguen
12 15 1900.
Published in german
language
What is EDS today?
A genetic disorder of connective tissue
(collagen especially) autosomal
dominant.
Without genetic test, the diagnosis is
clinical
Which is not a "rare disease" but a
frequent and an syndrome currently
confused with another diagnosis…
Most important signs for diagnosis
(personnal serie of 586 patients Beighton
scored 4/9 or more)
PAINS (98%): (joints, muscles, belly,
chest, skin, head,..) 71% very
intensives
ASTHENIA (97%) 69% very intensive
PROPRIOCEPTIVES disorders (85%)
HYPERMOBILITY (100%)
HEMORRHAGES (92%)
FRAGILITY OF THE SKIN (86,6%)
Other family case(s) (95%).
Respiratory symptoms in
EDS (586 patients)
Pseudo Asthma : 65%
Dyspnea : 66%
Dysphonia : 53%
Bronchitis : 56%
Strentchening of skin
Popular but inconstant
Beighton test : the thumb sign
KNEES Recurvatum
Ecchymosis
« EDS FOOT » (Flat forward, Hollow
in the middle, normal behind).
Pathophysiological hypothesis
(proprioception)
No (or bad) emission of signals
from sensors rom too soft
tissues.
Proprioceptive garments
Cereplas.
Rigid trunk orthesis
Special seat adapted for a
young boy with EDS
Light rest hand orthesis
(Néokraft)
Functionnal hand splint
Murphy finger orthesis
Lower limb orthesis with hip
device to stop hip luxation
Lower limbs orthesis with
elastic artificial muscles (Made
by JC Chignon, Orthesist)
She is walking,
She was in a Wheelchair !
The discovery of Dr. Bird
1980, F.M. BIRD invented
« Percussive intrapulmonary
Ventilation »,
with internal percussion of
pulmonary structures by
IMPULSATOR
Effects of Impulsator
Improve breathing status
and the new discover that (+
02) is also in EDS a
treatment
against fatigue
and migraines !
A chidren using percussionnaire
+ oxygen at home
Carnitine is also
used with success
against the fatigue.
Conclusion
«
To believe in
rehabilitation is to
believe in humanity »
Howard Rusk (New-York)