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Adult North Star Network (ANSN): Consensus Document for Therapists Working with Adults with Duchenne Muscular Dystrophy (DMD) - Therapy Guidelines

Narayan, S; Pietrusz, A; Allen, J; Docherty, K; Emery, N; Ennis, M; Flesher, R; ... ANSN; + view all (2022) Adult North Star Network (ANSN): Consensus Document for Therapists Working with Adults with Duchenne Muscular Dystrophy (DMD) - Therapy Guidelines. Journal of Neuromuscular Diseases , 9 (3) pp. 365-381. 10.3233/JND-210707. Green open access

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Abstract

BACKGROUND The survival of people with Duchenne Muscular Dystrophy (DMD) significantly increased due to improvements in standards of care (SOC) [1]. Consequently, DMD has evolved from a paediatric disease to a severe, chronic, multisystem, adult condition. The published international standards of care advocate specialist multidisciplinary health monitoring through proactive, anticipatory approaches to slow down the effects of the disease and allow advanced, informed decision-making [1–3]. Therapy starts as soon as the diagnosis is made and plays a vital role in symptom management in individuals to improve function, participation and effective quality of life. Therapy interventions for management, differ depending on the setting in which the care is being provided, specifically in terms of the expertise within the teams and resources available within these settings. People with DMD find that when they transition to adult services there is a dearth of expertise and limited access to therapy services. The survey conducted in the UK highlighted substantial differences between the care received by adults and children with the condition [2]. A large proportion of adults with DMD reported increased difficulties with access to professional physiotherapy, particularly at transition from childhood to adulthood. Additionally, having their functional abilities assessed regularly or receiving professional physiotherapy in general were both significantly more difficult to achieve within adult services in the UK. Furthermore, some of the major problems expressed by adults with DMD were mobility and transportation as well as, getting involved in leisure activities and work [3]. Therefore, while pediatric services are predominantly family-centred, after transition the paradigm of patient care changes towards individual-centred with focus on different therapy goals. Those become more tailored to the individuals’ needs, balancing quality of life and management options.This document is aimed at providing guidelines for physiotherapy, occupational therapy and speech and language considerations. The ‘Adult North Star Network’ (ANSN) was founded in 2015 to advance care of adults with DMD living in the UK and to develop a prospective natural history database. There are currently 28 adult centres within the network, caring for at least 700 DMD patients. Transition age is varied depending on services and is generally between the ages of 16 to 18. There is a wide range of severity affecting people with DMD transitioned to adult services, those who are steroid naive will have been permanent wheelchair users for many years and have profound muscle weakness. On the other hand, steroid treated patients will most commonly have good upper limb function, and some maybe ambulant at the time of transition. Additionally the specific type of genetic mutation, compliance to therapy and environmental factors may play a role in disease progression and presentation at transition. The aim of these guidelines is to support therapists working with adults with DMD with little or no experience to assist their clinical practice. Whilst the recommendations can be adopted by other health care systems in the world, we appreciate it will depend on resource availability.

Type: Article
Title: Adult North Star Network (ANSN): Consensus Document for Therapists Working with Adults with Duchenne Muscular Dystrophy (DMD) - Therapy Guidelines
Location: Netherlands
Open access status: An open access version is available from UCL Discovery
DOI: 10.3233/JND-210707
Publisher version: http://doi.org/10.3233/JND-210707
Language: English
Additional information: This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (CC BY-NC 4.0). See: https://creativecommons.org/licenses/by-nc/4.0/
Keywords: ANSN
UCL classification: UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Department of Neuromuscular Diseases
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
URI: https://discovery.ucl.ac.uk/id/eprint/10148162
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