Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2024 Apr 15;79(5):476-485.
doi: 10.1136/thorax-2023-220811.

Development of respiratory care guidelines for Duchenne muscular dystrophy in the UK: key recommendations for clinical practice

Anne-Marie Childs  1 , Catherine Turner  2 , Ronan Astin  3 , Stephen Bianchi  4 , John Bourke  2   5 , Vicki Cunningham  6 , Lisa Edel  7 , Christopher Edwards  8 , Phillippa Farrant  9 , Jane Heraghty  10 , Meredith James  2   5 , Charlotte Massey  11   12 , Ben Messer  13 , Jassi Michel Sodhi  2   5 , Patrick Brian Murphy  14   15 , Marianela Schiava  2   5 , Ajit Thomas  16 , Federica Trucco  17   18 , Michela Guglieri  19   5
Affiliations
Review

Development of respiratory care guidelines for Duchenne muscular dystrophy in the UK: key recommendations for clinical practice

Anne-Marie Childs et al. Thorax. .

Abstract

Significant inconsistencies in respiratory care provision for Duchenne muscular dystrophy (DMD) are reported across different specialist neuromuscular centres in the UK. The absence of robust clinical evidence and expert consensus is a barrier to the implementation of care recommendations in public healthcare systems as is the need to increase awareness of key aspects of care for those living with DMD. Here, we provide evidenced-based and/or consensus-based best practice for the respiratory care of children and adults living with DMD in the UK, both as part of routine care and in an emergency.

Methodology: Initiated by an expert working group of UK-based respiratory physicians (including British Thoracic Society (BTS) representatives), neuromuscular clinicians, physiotherapist and patient representatives, draft guidelines were created based on published evidence, current practice and expert opinion. After wider consultation with UK respiratory teams and neuromuscular services, consensus was achieved on these best practice recommendations for respiratory care in DMD.

Result: The resulting recommendations are presented in the form of a flow chart for assessment and monitoring, with additional guidance and a separate chart setting out key considerations for emergency management. The recommendations have been endorsed by the BTS.

Conclusions: These guidelines provide practical, reasoned recommendations for all those managing day-to-day and acute respiratory care in children and adults with DMD. The hope is that this will support patients and healthcare professionals in accessing high standards of care across the UK.

Keywords: critical care; emergency medicine; referral and consultation; respiratory measurement; respiratory muscles.

PubMed Disclaimer

Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Flow chart summary of respiratory routine standards of care for Duchenne muscular dystrophy (DMD). FVC, forced vital capacity; PCF, peak cough flow; PEF, peak expiratory flow.
Figure 2
Figure 2
Flow chart summary of respiratory care considerations for Duchenne muscular dystrophy (DMD) in an emergency or unplanned situation. CNS, central nervous system; NIV, non-invasive ventilation.
See this image and copyright information in PMC

References

    1. Birnkrant DJ, Bushby K, Bann CM, et al. . Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management. Lancet Neurol 2018;17:347–61. 10.1016/S1474-4422(18)30025-5 - DOI - PMC - PubMed
    1. Hull J, Aniapravan R, Chan E, et al. . British thoracic society guideline for respiratory management of children with neuromuscular weakness. Thorax 2012;67 Suppl 1(Suppl 1):i1–40. 10.1136/thoraxjnl-2012-201964 - DOI - PubMed
    1. Flanigan KM. Duchenne and becker muscular dystrophies. Neurol Clin 2014;32:671–88. 10.1016/j.ncl.2014.05.002 - DOI - PubMed
    1. Aartsma-Rus A, Ginjaar IB, Bushby K. The importance of genetic diagnosis for Duchenne muscular dystrophy. J Med Genet 2016;53:145–51. 10.1136/jmedgenet-2015-103387 - DOI - PMC - PubMed
    1. Ishizaki M, Kobayashi M, Adachi K, et al. . Female dystrophinopathy: review of current literature. Neuromuscul Disord 2018;28:572–81. 10.1016/j.nmd.2018.04.005 - DOI - PubMed

Publication types