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Feasibility, Validation and Application of Digital Tools for the Follow-up of Neuromuscular Patient Mobility in Daily Living

Not Applicable
Recruiting
Conditions
Neuromuscular Diseases
Interventions
Other: 2MWT
Other: MFM32
Other: MyoGrip
Other: QOL-gNMD
Diagnostic Test: Spirometry
Device: Acceleromerty
Other: 10mWT
Other: PUL
Other: NSAA
Other: NSAD
Other: TANS
Other: MyoPinch
Other: MyoQuad
Other: ACTIVLIM
Other: PREM
Other: SF-MPQ
Other: FSS
Other: IPAQ
Other: Rang of motion
Device: Goniometry
Other: Video captured monitoring
Device: Activity monitoring
Registration Number
NCT05798325
Lead Sponsor
Institut de Myologie, France
Brief Summary

The low prevalence of rare diseases hinders the design of clinical studies with sufficient statistical power to demonstrate the efficacy of new drugs. This can only be achieved by setting up international multicentre studies, which is challenging due to a lack of objective, universal outcome measures that generate high-quality, reproducible data. One of the hurdles in attaining universal outcome measures for clinical trials is the difficulty to capture and distinguish ambulatory from non-ambulatory, autonomous and assistive or involuntary movements. This makes a trial assessing the ambulatory phase very challenging at this moment. Excluding many participants from trials and many patients from access to medication.

Integration and validation of the technology in trials, research and patients' lives is essential in overcoming this hurdle. For example, in dystrophinopathies separate outcome measures exist for ambulant and non-ambulant participants, but the relation between these outcome measures or a transitional outcome measure/end point is largely missing.

Following an exhaustive literature review, several tools have been selected to remotely follow various symptoms of neuromuscular patients including weakness, pain, fatigue, cognitive defects, motor impairments (including loss of dexterity, ataxia...), metabolic, respiratory and cardiac troubles, contractures, tremor, falls, hypo or hypersomnia... The toolbox includes common measures for all patients but may include additional measures specific to the patient's symptoms (hence in turn to the patients' disease).

The measurements are designed to not be invasive, intrusive or burdensome for the patient.

DT4RD is going to leverage state-of-the art technology, clinical rating scales and psychometric/data analysis to deliver fit for purpose remote clinical assessments of mobility to ensure maximum patient benefit, specifically:

* Compare face to face clinical data collected in hospital with Patient Generated Data recorded remotely

* Examine how sensors can enhance measurement potentially at home and during clinical visits

* Promote a clear focus on user centered design and the integration of technology

* Use reliability and validity analyses to equate any common measures (those with the same or a similar construct)

* Demonstrate a proof-of-concept model into which different measures can be interchangeable

Detailed Description

Not available

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
40
Inclusion Criteria
  • Aged between 12 and 60 years
  • Patients with a genetically confirmed/molecular-proven neuromuscular or neurometabolic disease*
  • Patients experiencing walking difficulties in the home and at high risk of limiting participation and walking outside of the home.
  • Written informed consent
  • Able to comply with all protocol requirements, including video recording
  • Affiliated to or beneficiary of a social security scheme (for France)
Exclusion Criteria
  • Patients with undefined diagnosis or any diagnosis other than neuromuscular or neurometabolic disease
  • Patient walking 10m in less than 10s
  • Guardianship/trusteeship
  • Pregnant or nursing women
  • Patients having relevant concomitant pathologies that, in the appreciation of the investigator could interfere with protocol compliance
  • Patients not being affiliated with local social security (for France)

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
PrincipalMyoPinch-
PrincipalMFM32-
PrincipalMyoGrip-
PrincipalPUL-
PrincipalQOL-gNMD-
PrincipalAcceleromerty-
Principal2MWT-
PrincipalSpirometry-
PrincipalPREM-
Principal10mWT-
PrincipalMyoQuad-
PrincipalSF-MPQ-
PrincipalFSS-
PrincipalActivity monitoring-
PrincipalIPAQ-
PrincipalRang of motion-
PrincipalNSAA-
PrincipalNSAD-
PrincipalTANS-
PrincipalACTIVLIM-
PrincipalGoniometry-
PrincipalVideo captured monitoring-
Primary Outcome Measures
NameTimeMethod
Global home evaluations' attendanceThrough study completion, an average of 1 year

Proportion of home assessments carried out compared to the number of home assessments planned

Secondary Outcome Measures
NameTimeMethod
Compliance regarding using of the spirometer at homeThrough study completion, an average of 1 year

Analysis of the proportion of valid spirometry data collected at home

Correlation of home and hospital spirometer Forced vital capacity measurementsThrough study completion, an average of 1 year

Comparison of Forced vital capacity data collected during home and hospital spirometer measurements

Compliance regarding using of the LINKS sensors device at homeThrough study completion, an average of 1 year

Analysis of the proportion of usable accelerometry data collected at home

Compliance regarding using of the activity sensor at homeThrough study completion, an average of 1 year

Analysis of the proportion of usable data collected at home using the smartwatch

Correlation of home and hospital spirometer peak expiratory flow measurementsThrough study completion, an average of 1 year

Comparison of Peak expiratory flow data collected during home and hospital spirometer measurements

Compliance with filling out questionnaires at homeThrough study completion, an average of 1 year

Analysis of the proportion of replies to the questionnaires at home

Correlation of home and hospital spirometer forced expiratory volume in 1 second measurementsThrough study completion, an average of 1 year

Comparison of forced expiratory volume in 1 second data collected during home and hospital spirometer measurements

Compliance regarding achievement of video tasks at homeThrough study completion, an average of 1 year

Analysis of the usability of the video tasks performed at home

Trial Locations

Locations (2)

John Walton Muscular Dystrophy Research Centre

🇬🇧

Newcastle Upon Tyne, United Kingdom

Association Institut de Myologie

🇫🇷

Paris, France

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