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
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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.
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Welcome Dr. Bird, you safe the life of our patients or you enhance their quality of life
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André GrossiordThe pionner of French Rehabilitation. Garches.
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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
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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…
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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%)..
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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%
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Strentchening of skin Strentchening of skin Popular but inconstant Popular but inconstant
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Beighton test : the Beighton test : the thumb signthumb sign
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KNEES Recurvatum
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EcchymosisEcchymosis
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« « EDS FOOT » EDS FOOT » ((Flat forward, Hollow Flat forward, Hollow
in the middle, normal behindin the middle, normal behind).).
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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.
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Proprioceptive garments Proprioceptive garments Cereplas.Cereplas.
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Rigid trunk orthesis Rigid trunk orthesis
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Special seat adapted for a Special seat adapted for a young boy with EDSyoung boy with EDS
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Light rest hand orthesis Light rest hand orthesis (Néokraft)(Néokraft)
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Functionnal hand splintFunctionnal hand splint
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Murphy finger orthesisMurphy finger orthesis
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Lower limb orthesis with Lower limb orthesis with hip device to stop hip hip device to stop hip luxation luxation
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Lower limbs orthesis with Lower limbs orthesis with elastic artificial muscles (elastic artificial muscles (Made Made by JC Chignon, Orthesist)by JC Chignon, Orthesist)
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She is walking,She is walking,She was in a Wheelchair !She was in a Wheelchair !
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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
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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 !
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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.
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Conclusion
« To believe in rehabilitation is to believe in humanity »
Howard Rusk (New-York)