Neuromuscular disorders such as amyotrophic lateral sclerosis or muscular dystrophy can pose treatment challenges for clinicians. The diseases are uncommon, and patients have significant difficulties due to their respiratory symptoms. To help clinicians navigate those challenges, CHEST issued the first North American guideline on respiratory management for patients with neuromuscular weakness earlier this year.
“We do not have an established standard of care for patients with neuromuscular disease. In addition, initiating noninvasive ventilation (NIV) is difficult, and having guidelines will help preserve patient access through insurance,” said Akram Khan, MD, MBBS, FCCP, Associate Professor of Pulmonary and Critical Care Medicine at Oregon Health & Science University. “Specialists do a great job, but they are working out of deep clinical experience that very few clinicians have. This guideline will help standardize practice for all of us.”
Dr. Khan will co-chair a panel discussion exploring the clinical implications of the new guidance, Respiratory Management of Patients With Neuromuscular Weakness: CHEST Clinical Practice Guideline, on Sunday from 10:45 am to 11:45 am, in Room 316B of the convention center.
“We are focusing on the practical highlights of the guideline, focusing on pulmonary function evaluation, treatment of sleep-disordered breathing with noninvasive ventilation, and management of secretions that are the mainstay of caring for these patients,” said Session Co-Chair, Peter Gay, MD, MS, FCCP, Professor of Pulmonary Care and Critical Care Medicine at Mayo Clinic College of Medicine and Science. “We are providing a relatively easy road to the respiratory management of a variety of very complex neuromuscular disease processes.”
NIV is a common approach to dealing with respiratory symptoms, Dr. Gay said, but neuromuscular weakness can give rise to nuances that clinicians rarely see in sleep-disordered breathing. Symptoms typically worsen with disease progression, and, in some cases, albeit rarely, patients even require a tracheostomy and mechanical ventilation. That means selecting a device that can be used with both noninvasive and invasive ventilation as the patient’s needs change.
Patients also need help managing secretions and clearing the airway.
“As you get weaker with progressive disease, you don’t have the force and the power to cough, sneeze, clear your throat, or even breathe forcefully to help clear out secretions and keep the lungs from collapsing,” Dr. Khan said. “Patients have an increased risk of infection. The guideline provides guidance on how to better manage secretions and clearance.”
Lisa Wolfe, MD, FCCP, Professor of Pulmonary and Critical Care Medicine and Neurology at Northwestern University Feinberg School of Medicine, will discuss the ins and outs of NIV in patients with neuromuscular disease. She noted that initiating NIV can be a reimbursement challenge, and having a guideline will help preserve access and help set expectations for NIV equipment manufacturers.
David Zielinski, MD, FCCP, Associate Professor of Medicine at McGill University and Director of the Pediatric Asthma Clinic, Montreal Children’s Hospital, Montreal, Canada, will discuss some of the pediatric considerations in managing neuromuscular disease.
Dr. Khan spearheaded the CHEST guideline writing committee, addressing a need he noticed in his own practice.
“I had multiple patients with neuromuscular diseases who have faced respiratory issues where I lacked guidance on what to do next,” he said. “There is not much evidence of best practices for respiratory management in neuromuscular diseases, so we created an expert panel to review the evidence, some of it very good and some of it of very low certainty, to build treatment recommendations.”
Some recommendations are strong, such as NIV for patients with neuromuscular diseases and chronic respiratory failure. Other recommendations are conditional, such as invasive home mechanical ventilation via tracheostomy for patients with advanced end-stage symptoms who fail NIV or do not tolerate it. There are also stepwise treatment recommendations for the management of sialorrhea and multiple breathing and coughing techniques that may benefit some patients.
“This is practical management using state-of-the-art recommendations from CHEST,” Dr. Gay said. “This is an in-depth dive into areas where the evidence is solid and areas where it is less solid and more research is needed.”