Elaboration of a Pronostic Score of Changes on Wheelchair's Seating System (BPIP-Score MNM)

Assistance Publique - Hôpitaux de Paris

Status

Enrolling

Conditions

Neuromuscular Diseases

Treatments

Other: usual care

Study type

Observational

Funder types

Other

Identifiers

NCT05785546

APHP210996

2023-A00831-44 (Registry Identifier)

Details and patient eligibility

About

Development and validation study of a prognostic score - study of prognostic performance by a prospective longitudinal multicenter cohort spread over 8 centers: 4 for the development cohort and 4 for the validation cohort).

Full description

Progressive muscle weakness in neuromuscular diseases leads to motor disabilities leading to permanentfull-time wheelchair use. This is the case for infantile spinal muscular atrophy type II (SMA II), where people are dependent on a permanent sitting position from early childhood, at 2 years of age, or for Duchenne muscular dystrophy, when they lose their ability to walk around 10 years without corticosteroid therapy or around 13 years with treatment. These wheelchair users, weakened by the disease, are subject to musculotendinous retractions, osteo-articular stiffness, spinal deformities and pain. In addition, they use the wheelchair on a daily basis, on average 12 hours a day, whose permanent sitting position also conditions the performance of achieving life habits.

The analysis of the static and dynamic seated postural installation in the wheelchair makes it possible to avoid harmful consequences on the health and quality of life of users on a daily basis. When performed early enough, regularly and optimally, it also contributes to the prevention of complications.

In France, these assessments are carried out by occupational therapists, accompanied by doctors of Physical Medicine and Rehabilitation.

The weakness of the literature in this area and the absence of recommendations do not allow the user or any health professional to identify the need and the level of urgency to benefit from a dedicated consultation.

Faced with this observation, the development of a prognostic score for a change in positioning in the wheelchair, based on a prospective multicenter cohort and its validation on an external prospective cohort, will allow any healthcare professional to guide the user to a necessary positioning consultation. The hypothesis is that this score has discriminating qualities and that it is calibrated to predict a change in the seat of the RF and therefore the need to reassess the seated postural installation.

Enrollment

368 estimated patients

Sex

All

Ages

18+ years old

Volunteers

No Healthy Volunteers

Inclusion criteria

Male or female over the age of 18
Daily wheelchair user (more than 4 hours per day)
Having a neuromuscular disease such as Duchenne Muscular Dystrophy or Infantile Spinal Muscular Atrophy type II
Accompanied by an identified medical equipment provider, able to respond to the recommendations of the clinical team
Patient informed and having signed consent.

Exclusion criteria

Person who already has a seat change or FR prescription at the time of inclusion
Having undergone surgery less than 1 year ago or a fracture
Pregnant or breastfeeding women
Patient under guardianship or curatorship.