By Gail Balfour, Muscular Dystrophy Canada
People affected by neuromuscular disorders today are living longer than ever before – which is very positive news. But the challenge of these disorders becoming more of a chronic disease state is that while many of these people are living longer into adulthood, they continue to face major quality-of-life challenges.
One of the key things having a negative impact on the quality of life in these individuals is their respiratory health. Although neuromuscular disorders do not impair the lungs directly, they often affect the muscles involved in breathing, coughing and swallowing.
Empowering both patients and their healthcare professionals with the tools and knowledge to help them live better and lengthen the time between emergency room visits is crucial, says Dr. Janice Richman-Eisenstat, Neuropulmonary Rehabilitation Medicine and Palliative Respiratory Care Specialist, Associate Clinical Professor with the Pulmonary and Neurology Divisions of the Department of Medicine at the University of Alberta.
“Imagine if you are coughing and choking all the time,” she said. “I’m sure I don’t need to tell you that people don’t really have fun when they have to go to the hospital. You are being looked after by doctors that usually are not informed about neuropulmonary conditions and they may not know how to help people cough or clear their airway.”
Educating patients and caregivers on tips and tools that they can use at home, such as swallowing techniques, breath stacking and lung volume recruitment devices, is vital, she said.
Four years ago, she and her colleagues started the first multidisciplinary neuromuscular clinic for adults in North America. Although a common model in pediatrics, the holistic multidisciplinary approach is still rare to find in adult care. This approach allows people to see different specialists, ranging from a dietician to a rehabilitation medicine specialist, in the same place on the same day.
“More and more, chronic illness is about working with a team. This is the model we need to aspire to in chronic disease management,” she said. “I learn from my colleagues, they learn from me and I feel that together we are moving the field forward in improving patient care and patient support – to live as best as possible in their homes and in their communities.”
Looking toward the future, while we search for cures, Dr. Richman-Eisenstat stresses the importance of framing research and care strategies around ways in which to make an immediate and positive impact in patients’ lives.
“There is always something that can be done. Maybe we cannot achieve goal one, but maybe we can reframe goal one into an achievable goal two. And if we can’t achieve that, maybe we can reframe it further,” she said. “We have to take all approaches to help our patients live as best as possible today as well as tomorrow.”
The quest for improved quality of life for these patients is a research priority for Muscular Dystrophy Canada – which is why we continue to award seed grants into respiratory health research areas. Four new grants were awarded this year:
- A grant awarded to a team led by Dr. Reshma Amin at the Hospital for Sick Children in Toronto looks at assessing tests that predict the respiratory risks of air travel for those with Duchenne Muscular Dystrophy. Because of respiratory muscle weakness, these individuals are at risk of decompensation due to respiratory muscle fatigue, elevated carbon dioxide levels and even respiratory failure in the low air pressure environment of airplane flight cabins.
- A grant awarded to a team led by Dr. Marta Kaminska at the McGill University Health Centre in Montreal investigates the validation of home portable monitoring for diagnosis of sleep-disordered breathing in patients with neuromuscular disorders. Home portable monitoring is a potentially viable alternative for those who might otherwise be unable to undergo in-laboratory sleep studies.
- A grant awarded to a team led by Dr. Hans Katzberg at the University Health Network in Toronto seeks to evaluate factors associated with nocturnal hypoventilation in patients with myasthenia gravis, and help identify patients in neuromuscular clinics who would benefit from interventions.
- A grant awarded to a team lead by Dr. Louise Rose at the Sunnybrook Research Institute in Toronto seeks to describe current practice related to monitoring cough effectiveness and clinician recommendations for airway clearance strategies.
Note, the above article was republished and is copyrighted from Research in the Works, a Muscular Dystrophy Canada annual publication. For more information, visit www.muscle.ca/respiratory.
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