Implementation of duchenne muscular dystrophy care considerations

Jennifer G. Andrews, Kristin Conway, Christina Westfield, Christina Trout, F. John Meaney, Katherine Mathews, Emma Ciafaloni, Christopher M Cunniff, Deborah J. Fox, Dennis Matthews, Shree Pandya

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

BACKGROUND: Duchenne muscular dystrophy (DMD) is an X-linked disorder characterized by progressive muscle weakness and multisystem involvement. Recent advances in management of individuals with DMD have prolonged survival. Lack of standardized care spurred an international collaboration to develop consensus-based care considerations for diagnosis and management. In this study, we evaluate adherence to considerations at selected sites. METHODS: We collaborated with the Muscular Dystrophy Surveillance, Tracking, and Research Network. Our sample included males with DMD and Becker muscular dystrophy <21 years as of December 31, 2010, with 1 health care encounter on or after January 1, 2012. We collected data from medical records on encounters occurring January 1, 2012, through December 31, 2014. Adherence was determined when frequency of visits or assessments were at or above recommendations for selected care considerations. RESULTS: Our analytic sample included 299 individuals, 7% of whom (20/299) were classified as childhood-onset Becker muscular dystrophy. Adherence for neuromuscular and respiratory clinician visits was 65% for the cohort; neuromuscular assessments and corticosteroid side effect monitoring measures ranged from 16% to 68%. Adherence was 83% for forced vital capacity and ≤58% for other respiratory diagnostics. Cardiologist assessments and echocardiograms were found for at least 84%. Transition planning for education or health care was documented for 31% of eligible males. CONCLUSIONS: Medical records data were used to identify areas in which practice aligns with the care considerations. However, there remains inconsistency across domains and insufficiency in critical areas. More research is needed to explain this variability and identify reliable methods to measure outcomes.

Original languageEnglish (US)
Article numbere20174006
JournalPediatrics
Volume142
Issue number1
DOIs
StatePublished - Jul 1 2018
Externally publishedYes

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Duchenne Muscular Dystrophy
Medical Records
Delivery of Health Care
Muscular Dystrophies
Muscle Weakness
Vital Capacity
Research
Adrenal Cortex Hormones
Outcome Assessment (Health Care)
Education

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Andrews, J. G., Conway, K., Westfield, C., Trout, C., Meaney, F. J., Mathews, K., ... Pandya, S. (2018). Implementation of duchenne muscular dystrophy care considerations. Pediatrics, 142(1), [e20174006]. https://doi.org/10.1542/peds.2017-4006

Implementation of duchenne muscular dystrophy care considerations. / Andrews, Jennifer G.; Conway, Kristin; Westfield, Christina; Trout, Christina; Meaney, F. John; Mathews, Katherine; Ciafaloni, Emma; Cunniff, Christopher M; Fox, Deborah J.; Matthews, Dennis; Pandya, Shree.

In: Pediatrics, Vol. 142, No. 1, e20174006, 01.07.2018.

Research output: Contribution to journalArticle

Andrews, JG, Conway, K, Westfield, C, Trout, C, Meaney, FJ, Mathews, K, Ciafaloni, E, Cunniff, CM, Fox, DJ, Matthews, D & Pandya, S 2018, 'Implementation of duchenne muscular dystrophy care considerations', Pediatrics, vol. 142, no. 1, e20174006. https://doi.org/10.1542/peds.2017-4006
Andrews JG, Conway K, Westfield C, Trout C, Meaney FJ, Mathews K et al. Implementation of duchenne muscular dystrophy care considerations. Pediatrics. 2018 Jul 1;142(1). e20174006. https://doi.org/10.1542/peds.2017-4006
Andrews, Jennifer G. ; Conway, Kristin ; Westfield, Christina ; Trout, Christina ; Meaney, F. John ; Mathews, Katherine ; Ciafaloni, Emma ; Cunniff, Christopher M ; Fox, Deborah J. ; Matthews, Dennis ; Pandya, Shree. / Implementation of duchenne muscular dystrophy care considerations. In: Pediatrics. 2018 ; Vol. 142, No. 1.
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