Improving Access and Guideline Adherence in Pulmonary Care in Patients With Duchenne Muscular Dystrophy

Duchenne muscular dystrophy (DMD) is a devastating, progressive neuromuscular disease that results in cardiopulmonary failure and death. In 2018, the DMD Care Considerations guidelines were updated to improve the multidisciplinary approach to care and promote early respiratory management. We sought...

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Bibliographic Details
Published in:Respiratory care 2022-03, Vol.67 (3), p.347-352
Main Authors: Kaslow, Jacob A, Soslow, Jonathan H, Burnette, William B, Raucci, Frank J, Hills, Tracy J, Ibach, Michaela G, Hebblethwaite, Rita C, Arps, Kara M, Sokolow, Andrew G
Format: Article
Language:English
Subjects:
Complications and side effects
Diagnosis
Duchenne muscular dystrophy
Evaluation
Guideline Adherence
Humans
Methods
Muscular Dystrophy, Duchenne - complications
Muscular Dystrophy, Duchenne - therapy
Original Research
Polysomnography
Practice guidelines (Medicine)
Respiratory Function Tests
Respiratory therapy
Retrospective Studies
Citations: Items that cite this one
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Description
Summary:Duchenne muscular dystrophy (DMD) is a devastating, progressive neuromuscular disease that results in cardiopulmonary failure and death. In 2018, the DMD Care Considerations guidelines were updated to improve the multidisciplinary approach to care and promote early respiratory management. We sought to evaluate the impact of a multidisciplinary clinic on access to pulmonary care and adherence to respiratory care guidelines. Utilizing retrospective data, we assessed for pulmonary care between 2016-2019 and congruence with guidelines from March 2018-February 2019. Using a standardized visit protocol, subjects were monitored for adherence to pulmonary function testing (PFT) and polysomnography (PSG) recommendations. Of the 84 subjects with DMD, only 51.2% had prior pulmonary involvement, and approximately one-third were seen in the year prior to clinic onset. Only 23% of subjects with a pulmonary referral completed this visit. After clinic initiation, the average age of a subject's first pulmonary contact decreased from 11.8 y to 7.9 y ( < .001), and 45% of the 77 unique clinic subjects had no previous pulmonary encounter. Adherence to PFT guidelines increased in both ambulatory (8.7% to 86.1%) and non-ambulatory subjects (25.9% to 90.1%). Approximately 79% of subjects seen in clinic either completed or had an order for PSG in the last 12 months. Development of a multispecialty clinic expanded access to pulmonary care and evaluation in subjects with DMD. Continued care in this clinic will allow a better understanding of barriers to access and the opportunity to monitor long-term pulmonary health.
ISSN:0020-1324
1943-3654
DOI:10.4187/respcare.09502