The Need for Respiratory Guidelines

Due to the multisystemic nature of myotonic dystrophy type 1 (DM1), the studies and rigorous evidence needed to drive the creation of an evidence-based guideline for the respiratory clinical care of myotonic dystrophy patients were not available. In order to improve and standardize care for DM1, 12 leading DM1 pulmonologists in Western Europe, the UK, Canada, and the US joined together to create the Consensus-based Care Recommendations for Pulmonologists Treating Adults with Myotonic Dystrophy Type 1. The project was organized and supported by MDF.

To promote the development of pulmonary care guidelines for myotonic dystrophy, MDF solicited the input of care experts and organized the drafting of these recommendations. A group of neuromuscular experts in DM1 including pulmonologists, respiratory physiotherapists and sleep specialists created the Consensus-based Care Recommendations for Pulmonologists Treating Adults with Myotonic Dystrophy Type 1 through an iterative process. All authors contributed content to create the first draft and leading author Valeria Sansone, M.D., Ph.D. was in charge of managing further editing cycles. The process began in 2017 and concluded in April 2020, with the article published on Respiration’s website on 4/17/20 and in print in Volume 99, Number 4.

Share this with your Pulmonologist

In patients with DM1, respiratory muscle weakness frequently evolves, leading to respiratory failure as the main cause of death, followed by cardiac complications. These guidelines provide a more detailed outline on the diagnostic and management protocols which can guide pulmonologists who may not have experience with DM1 or who are not part of a neuromuscular multidisciplinary clinic. The 7-page document provides detailed respiratory care recommendations for the management of patients living with DM1, including respiratory testing and management at baseline and during follow-up visits. The resulting recommendations are intended to improve respiratory care for the most vulnerable of DM1 patients and lower the risk of respiratory complications and mortality by providing pulmonologists with practical indications on which tests and when to perform them, adapting the general respiratory knowledge to specific issues related DM. The guidelines represent an exciting step in the respiratory treatment and management of DM1 and MDF is thrilled to support this vital educational effort.

During the COVID-19 Pandemic, please be sure to share the Respiratory Care Recommendations for Myotonic Dystrophy Patients During the COVID-19 Pandemic with your doctor as well!

Resources

You can access the full article online via Respiration and Karger Publishers at https://www.karger.com/Article/Abstract/505634.

Click here to download a copy of the Consensus-Based Care Recommendations for Pulmonologists Treating Adults with Myotonic Dystrophy Type 1!

If you would like a hard copy of the article or the guidelines mailed to you, please contact us at info@myotonic.org with your mailing address.