ehlers-danlos syndrome eds hôtel-dieu de paris bird meeting ased 06 09 2012
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Ehlers-Danlos SyndromeEhlers-Danlos SyndromeEDSEDS
Hôtel-Dieu de ParisHôtel-Dieu de Paris
Bird meetingBird meeting
ASED 06 09 2012ASED 06 09 2012
Claude Hamonet*, Monique Claude Hamonet*, Monique Vienne **Vienne **
*Department of Physical Medicine and *Department of Physical Medicine and RehabilitationRehabilitation (Pr. S. Poiraudeau, Dr. JY (Pr. S. Poiraudeau, Dr. JY Maigne), Hôtel-Dieu Hospital, ParisMaigne), Hôtel-Dieu Hospital, Paris
**IMAP, Nice.**IMAP, Nice.
With support of Department of Public health With support of Department of Public health and Epidemiology (Pr. Ravaud), Hôtel-Dieu and Epidemiology (Pr. Ravaud), Hôtel-Dieu Hospital Paris.Hospital Paris.
Grant : Fondation de France.Grant : Fondation de France.
Welcome Dr. Bird, you safe the life of our patients or you enhance their quality of life
André GrossiordThe pionner of French Rehabilitation. Garches.
First description : a law studentFirst description : a law student.. By By Edward EHLERSEdward EHLERS, a Dermatologist,, a Dermatologist,
Danish Society of Danish Society of Dermatology Dermatology CopenhaguenCopenhaguen 1212 15 190015 1900. . Published in Published in german german languagelanguage
What is EDS today?What is EDS today?
AA genetic disorder genetic disorder of connective tissueof connective tissue (collagen especially) autosomal (collagen especially) autosomal dominant.dominant.
Without Without genetic testgenetic test, the , the diagnosis is diagnosis is clinical clinical
Which Which is not a "is not a "rare diseaserare disease"" but a but a frequent and anfrequent and an syndrome syndrome currently currently confused with another diagnosis…confused with another diagnosis…
Most important signs Most important signs for diagnosis for diagnosis (personnal serie of (personnal serie of 586 patients586 patients Beighton Beighton scored 4/9 or more)scored 4/9 or more)
PAINSPAINS (98%)(98%): : (joints, muscles, belly, (joints, muscles, belly, chest, skin, head,..) chest, skin, head,..) 71% very 71% very intensives intensives
ASTHENIAASTHENIA (97%(97%) ) 69% very 69% very intensiveintensive
PROPRIOCEPTIVESPROPRIOCEPTIVES disordersdisorders (85%)(85%)HYPERMOBILITYHYPERMOBILITY (100%)(100%)HEMORRHAGESHEMORRHAGES (92%)(92%)
FRAGILITY OF THE SKINFRAGILITY OF THE SKIN (86,6%)(86,6%)OtherOther family case(s) family case(s) (95%)(95%)..
Respiratory symptoms Respiratory symptoms in EDS (586 patients)in EDS (586 patients)
Pseudo Asthma : Pseudo Asthma : 65%65%
Dyspnea : Dyspnea : 66%66%
Dysphonia : Dysphonia : 53%53%
Bronchitis : Bronchitis : 56%56%
Strentchening of skin Strentchening of skin Popular but inconstant Popular but inconstant
Beighton test : the Beighton test : the thumb signthumb sign
KNEES Recurvatum
EcchymosisEcchymosis
« « EDS FOOT » EDS FOOT » ((Flat forward, Hollow Flat forward, Hollow
in the middle, normal behindin the middle, normal behind).).
Pathophysiological Pathophysiological hypothesis hypothesis (proprioception)(proprioception)
No (or bad) emission of signals No (or bad) emission of signals from sensors rom too soft from sensors rom too soft tissues.tissues.
Proprioceptive garments Proprioceptive garments Cereplas.Cereplas.
Rigid trunk orthesis Rigid trunk orthesis
Special seat adapted for a Special seat adapted for a young boy with EDSyoung boy with EDS
Light rest hand orthesis Light rest hand orthesis (Néokraft)(Néokraft)
Functionnal hand splintFunctionnal hand splint
Murphy finger orthesisMurphy finger orthesis
Lower limb orthesis with Lower limb orthesis with hip device to stop hip hip device to stop hip luxation luxation
Lower limbs orthesis with Lower limbs orthesis with elastic artificial muscles (elastic artificial muscles (Made Made by JC Chignon, Orthesist)by JC Chignon, Orthesist)
She is walking,She is walking,She was in a Wheelchair !She was in a Wheelchair !
The discovery of Dr. BirdThe discovery of Dr. Bird
1980, F.M. BIRD invented1980, F.M. BIRD invented
« « Percussive intrapulmonary Percussive intrapulmonary Ventilation Ventilation »,»,
with internal percussion of with internal percussion of pulmonary structures by pulmonary structures by IMPULSATORIMPULSATOR
Effects of ImpulsatorEffects of Impulsator
Improve breathing statusImprove breathing statusand the new discover that (+ and the new discover that (+ 02) is also in EDS a 02) is also in EDS a treatmenttreatment
against fatigueagainst fatigueand migraines !and migraines !
A chidren using A chidren using percussionnairepercussionnaire + oxygen at + oxygen at homehome
CarnitineCarnitine is also is also used with successused with success
against the fatigue.against the fatigue.
Conclusion
« To believe in rehabilitation is to believe in humanity »
Howard Rusk (New-York)