Development of a Functional Assessment Scale for Ambulatory Boys with Duchenne Muscular Dystrophy

Authors

  • Elaine Scott,

    Corresponding author
    • Muscular Dystrophy Campaign, London, UK
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  • Michelle Eagle,

    1. Institute of Human Genetics, Newcastle, UK
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  • Anna Mayhew,

    1. Institute of Human Genetics, Newcastle, UK
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  • Jenny Freeman,

    1. University of Sheffield, Sheffield, UK
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  • Marion Main,

    1. Great Ormond Street Hospital, London, UK
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  • Jennie Sheehan,

    1. Evelina Children's Hospital, London, UK
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  • Adnan Manzur,

    1. Dubowitz Neuromuscular Centre, Institute of Child Health, UCL, London, UK
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  • Francesco Muntoni,

    1. Dubowitz Neuromuscular Centre, Institute of Child Health, UCL, London, UK
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  • The North Star Clinical Network for Paediatric Neuromuscular Disease

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    • The North Star Clinical Network for Paediatric Neuromuscular Disease: Collaborators (53): Manzur A.Y., Muntoni F., Robb S., Main M., Kemp J. (Great Ormond Street Hospital, London), Scott E. (Muscular Dystrophy Campaign, London), Bushby K., Straub V., A. Sarkozy, E. Strehle, R. Venkateswaran, Eagle E., Mayhew A. (Institute of Human Genetics, Newcastle), Roper H., McMurchie H., Grace A. (Heartlands Hospital, Birmingham), Spinty S., Peachey G., Shillington S. (Alder Hey Children's Hospital, Liverpool), Quinlivan R., Groves L. (Robert Jones and Agnes Hunt Royal Orthopaedic Hospital, Oswestry), Wraige E., Jungbluth H., Sheehan J., Spahr R. (Evalina Children's Hospital, London), Hughes I., Bateman E., Cammiss C. (Royal Manchester Children's Hospital), Childs A.M., Pallant L., Psyden K. (Leeds General Infirmary), Baxter P. (Sheffield Children's Hospital), Naismith K., Keddie A. (King's Cross Hospital, Dundee), Horrocks I., McWilliam R., Di Marco M. (Yorkhill Children's Hospital, Glasgow), Hartley L., Sheen B., Fenton-May J. (University Hospital Wales, Cardiff), Jardine P., Majumdar A., Jenkins L. (Frenchay Hospital, Bristol), Chow G., Miah A. (Queen's Medical Centre University Hospital, Nottingham), de Goede C. (Preston Royal Hospital), Thomas N., Geary M., Keslake K. (Southampton General Hospital), White C., Greenfield K. (Morriston Hospital, Swansea), MacAuley S. (Royal Belfast Hospital for Sick Children), Baxter A., Yirrell Y., Longman C. (Royal Hospital for Sick Children, Western General Hospital, Edinburgh).

Elaine Scott, MPhil, MCSP, c/o Muscular Dystrophy Campaign, 61 Southwark Street, London SE1 0HL, UK.

Email: elaines@muscular-dystrophy.org

Abstract

Background and Purpose

The aims of this study were to develop a clinical assessment scale to measure functional ability in ambulant boys with Duchenne muscular dystrophy and to determine the reliability of the scale in multiple centres in the UK.

Methods

Focus groups and workshops were held with experienced paediatric neuromuscular physiotherapists to determine scale content. A manual was prepared with accompanying videos, and training sessions were conducted. A total of 17 physiotherapists from participating centres used the videos to determine inter-rater reliability. Five determined the intra-rater reliability.

Results

Strength of agreement for these groups based on total subject scores was very good (0.95 and ≥0.93 for consistency and absolute agreement, respectively). Test–retest ability was high, with perfect agreement between occasions for all but two items of the scale.

Conclusions

Our study indicates that the North Star Ambulatory Assessment is practical and reliable. It takes only 10 minutes to perform and incorporates both universally used timed tests as well as levels of activities, which allow assessment of high-functioning boys with Duchenne muscular dystrophy. Copyright © 2011 John Wiley & Sons, Ltd.

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