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

NCT06378203 | Recruiting

• 1 other identifier: MEDEA 1013
• Study Type: interventional
• Target: 10
• Locations: 1 country
• Sites: 1

Brief Summary

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.

Conditions

Muscular Dystrophies; Limb Girdle Muscular Dystrophy; Facio-Scapulo-Humeral Dystrophy; Becker Muscular Dystrophy

Keywords

muscular dystrophy; muscular training; muscular exercise; tele monitoring; home rehabilitation

Outcome Measures

Primary Outcomes (4)

• 6 minute walk test (6MWT)

  6 Minute Walk Test (measured in meters- normal range 500-600 meters in 6 minutes)

  6 months : Modification from month 0 (baseline) to month 6

• Motor Function Measure scale (MFM)

  MFM measures Motor function at 3 levels (D1 change position and transfers; D2 axial and proximal motor function; D3 distal motor function), Each i item is scored on a 4-point Likert scale from 0 (cannot initiate the task) to 3 (performs the task fully). Item scores are summed, and the raw score is transformed to an overall total score ranging from 0 (severe functional impairment) to 100 (no functional impairment). rated in % from 0 to 100 and an overall percentage is calculated , in %)

  6 months: Modification from month 0 (baseline) to month 6

• Performance of Upper Limb (PUL)

  Performance of upper limb is a scale specifically designed to measure upper limb function in muscular dystrophy; there is an entry level item ranging from 0 to 6; the PUL scale test the proximal to distal progression of muscle weakness through three levels: high (shoulder domain), mid (elbow domain), and distal (wrist and finger domain); the PUL score (version 1.2) includes 22 items ; the overall scores ranges from 0 to 42

  6 Months: Modification from month 0 (baseline) to month 6

• Time up and go (TUG)

  Recording of the time required to stand up from a chair, walk for 3 meters and going back to the sitting position- normal range \< 10 seconds

  6 months: Modification from month 0 (baseline) to month 6

Secondary Outcomes (2)

• Modification of fatigue and quality of life scales from T0 to T6

  6 months: Modification from month 0 (baseline) to month 6

• Short Form 36 (SF36)

  6 months: Modification from month 0 (baseline) to month 6

Study Arms (1)

Limb GIrdle Muscular Dystrophy

EXPERIMENTAL

Open label study one arm

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

Interventions

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

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.

Limb GIrdle Muscular Dystrophy

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• 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.

You may not qualify if:

• 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).

Sponsors & Collaborators

• IRCCS Eugenio Medea: lead

Study Sites (1)

Scientific Institute IRCCS Eugenio Medea

Bosisio Parini, Lecco, 23842, Italy

RECRUITING

Related Publications (6)

• Preisler N, Orngreen MC. Exercise in muscle disorders: what is our current state? Curr Opin Neurol. 2018 Oct;31(5):610-617. doi: 10.1097/WCO.0000000000000597.

  PMID: 30036194 BACKGROUND

• Veenhuizen Y, Cup EHC, Jonker MA, Voet NBM, van Keulen BJ, Maas DM, Heeren A, Groothuis JT, van Engelen BGM, Geurts ACH. Self-management program improves participation in patients with neuromuscular disease: A randomized controlled trial. Neurology. 2019 Oct 29;93(18):e1720-e1731. doi: 10.1212/WNL.0000000000008393. Epub 2019 Sep 30.

  PMID: 31570565 BACKGROUND

• Siciliano G, Simoncini C, Giannotti S, Zampa V, Angelini C, Ricci G. Muscle exercise in limb girdle muscular dystrophies: pitfall and advantages. Acta Myol. 2015 May;34(1):3-8.

  PMID: 26155063 BACKGROUND

• Eagle M. Report on the muscular dystrophy campaign workshop: exercise in neuromuscular diseases Newcastle, January 2002. Neuromuscul Disord. 2002 Dec;12(10):975-83. doi: 10.1016/s0960-8966(02)00136-0. No abstract available.

  PMID: 12467755 BACKGROUND

• Heutinck L, Jansen M, van den Elzen Y, van der Pijl D, de Groot IJM. Virtual Reality Computer Gaming with Dynamic Arm Support in Boys with Duchenne Muscular Dystrophy. J Neuromuscul Dis. 2018;5(3):359-372. doi: 10.3233/JND-180307.

  PMID: 29991140 BACKGROUND

• Jansen M, de Groot IJ, van Alfen N, Geurts ACh. Physical training in boys with Duchenne Muscular Dystrophy: the protocol of the No Use is Disuse study. BMC Pediatr. 2010 Aug 6;10:55. doi: 10.1186/1471-2431-10-55.

  PMID: 20691042 BACKGROUND

MeSH Terms

Conditions

Muscular Dystrophies; Muscular Dystrophies, Limb-Girdle; Muscular Dystrophy, Facioscapulohumeral; Muscular Dystrophy, Duchenne

Condition Hierarchy (Ancestors)

Muscular Disorders, Atrophic; Muscular Diseases; Musculoskeletal Diseases; Neuromuscular Diseases; Nervous System Diseases; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Genetic Diseases, X-Linked

Central Study Contacts

Maria G D'Angelo

CONTACT

031877111; grazia.dangelo@lanostrafamiglia.it

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NA
• Masking: NONE
• Purpose: TREATMENT
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Model Details: Each patient will carry out a series of evaluation and training sessions (15 sessions) with a physiotherapist for the drafting of a personalized treatment plan which will subsequently be carried out "independently" at domicile. The home therapeutic proposal will include the use of the tele-rehabilitation system with virtual reality Home Kit - Virtual Reality Rehabilitation System (VRRS KHYMEIA) which allows to perform personalized games at home

Study Record Dates

• First Submitted: March 19, 2024
• First Posted: April 22, 2024
• Study Start: December 15, 2023
• Primary Completion: December 15, 2024
• Study Completion: April 15, 2025
• Last Updated: April 22, 2024

Record last verified: 2024-04

Data Sharing

• IPD Sharing: Will not share

Locations

IT (1)