RECRUITINGINTERVENTIONAL

Rehabilitation in Muscular Dystrophies From the Hospital Facility to the Home: Pilot Project [RIMUDI]

Rehabilitation in Muscular Dystrophies From the Hospital Facility to the Home: Pilot Project

About This Trial

Until twenty years ago physical exercise in muscular dystrophies was considered harmful to the muscle cells, inducing an acceleration of cell necrosis. In fact, it is now certain and validated that an active lifestyle and the practice of controlled and regular physical activity are to be considered therapeutic in neuromuscular pathologies with the aim of optimizing muscular and cardio-respiratory function and preventing atrophy In particular, it seems that the optimal care is extensive and can be carried out in a safe and controlled manner even at home. It is well documented that exercise has beneficial effects on muscle with increased strength and muscular endurance.

Who May Be Eligible (Plain English)

Who May Qualify:- definite genetic diagnosis of Limb Girdle Muscular Dystrophy: LGMD2A/R1(calpain 3 deficiency), LGMD2B/R2(dysferlin deficiency), LGMD2I/R9 (FKRP), LGMD2L /R11(ANOCTAMIN 5) or Facio Scapulo Humeral Dystrophy (FSHD) or Becker muscular dystrophy (BMD); - strength values at the level of the main antigravity muscles \> or equal to 3, according to the Medical Research Council (MRC) scale; - independent walking in a protected (internal) environment, even with assistance; - patients must have performed at least 1 clinical-functional evaluation at our facility in the year preceding the start of the trial. - They must express compliance with joining the project and must not be followed from a physiotherapeutic point of view elsewhere during the 6 months of participation in the project. Who Should NOT Join This Trial: - dilated or ischemic heart disease with Left Ventricle Ejection Fraction \<50%; - chronic respiratory failure with Forced Vital Capacity \< 40% predicted, nocturnal oxygen desaturation - \> 5% of nocturnal time spent with peripheral oxygen saturation levels \< 90). Always talk to your doctor about whether this trial is right for you.

Original Eligibility Criteria

View original clinical language

Inclusion Criteria:- definite genetic diagnosis of Limb Girdle Muscular Dystrophy: LGMD2A/R1(calpain 3 deficiency), LGMD2B/R2(dysferlin deficiency), LGMD2I/R9 (FKRP), LGMD2L /R11(ANOCTAMIN 5) or Facio Scapulo Humeral Dystrophy (FSHD) or Becker muscular dystrophy (BMD); * strength values at the level of the main antigravity muscles \> or equal to 3, according to the Medical Research Council (MRC) scale; * independent walking in a protected (internal) environment, even with assistance; * patients must have performed at least 1 clinical-functional evaluation at our facility in the year preceding the start of the trial. * They must express compliance with joining the project and must not be followed from a physiotherapeutic point of view elsewhere during the 6 months of participation in the project. Exclusion Criteria: * dilated or ischemic heart disease with Left Ventricle Ejection Fraction \<50%; * chronic respiratory failure with Forced Vital Capacity \< 40% predicted, nocturnal oxygen desaturation - \> 5% of nocturnal time spent with peripheral oxygen saturation levels \< 90).

Treatments Being Tested

OTHER

tele-rehabilitation system with virtual reality Home Kit - Virtual Reality Rehabilitation System (VRRS KHYMEIA)

A personalized exercise plan following macro-areas of objectives: * maintenance of muscle lengths with selective and/or prolonged static stretching exercises; * maintenance/improvement of muscle trophism with selective and/or postural and/or functional muscle recruitment exercises at moderate intensity (approximately 65%-70% of the maximum); * maintenance of respiratory muscle weakness with thoracic and/or diaphragmatic breathing exercises combined with muscle exercise; * endurance training with walking, cycle ergometer (to be used with both the upper and lower limbs) and treadmill at moderate intensity (approximately 65%-70% of the maximum). Home treatment will mostly take place with the KHYMEIA tele-rehabilitation system.

Locations (1)

Scientific Institute IRCCS Eugenio Medea

Bosisio Parini, Lecco, Italy