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Breathing Issues and Myotonic Dystrophy

A European Workshop Report

Researchers and medical professionals from Europe, Canada and the USA gathered recently for a workshop in Europe. The meeting, entitled Chronic Respiratory Insufficiency in Myotonic Dystrophy: Management and Implications for Research, was presented by the Neuromuscular Omnicomprehensive Center at the University of Milan, Italy.

The conference objectives were to identify the main respiratory issues associated with myotonic dystrophy (DM), and build consensus regarding the best DM respiratory treatments.

In reviewing previously published research, they identified respiratory problems and problems due to breathing issues in myotonic dystrophy type 1 (DM1), including:

  • Weakness in muscles responsible for breathing
  • Low oxygen levels during sleep
  • Complex brain changes interfering with the performance of muscles used for breathing
  • Fatigue
  • Daytime sleepiness

The workshop discussed ways to build awareness of DM respiratory issues and treatment options among patients and healthcare providers, something that has been difficult in the past. Treatments discussed included non-invasive ventilation such as C-PAP, BiPAP or Auto-SV, and use of machines to help patients cough. The professionals agreed that increased awareness of C-PAP and other prescribed ventilation systems, and more consistent use of this equipment by DM patients will ultimately lead to decreased respiratory symptoms and better quality of life.

Experts from around the world described what treatments work best in their clinics, which led to the creation of a standardized set of treatments that should be followed by everyone treating respiratory issues in DM patients. The workshop highlighted the need for people with DM to have better accessibility to neuromuscular clinics with experience in prescribing and using these kinds of treatments, and the need for a standard approach to screening for respiratory symptoms.

Participants also discussed other issues such as management of respiration during surgery, weight control, exercise programs, and the importance of educating people with DM and their caregivers. The participants created a symptom checklist, a list of respiratory lab tests, and list of pros and cons for different types of treatments. A full report on the workshop, including treatment guidelines, will be published in the journal Neuromuscular Disorders later this year.






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