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Respiratory Care in FSHD. Lee Guion MA, RRT, FAARC FSHD Family Day Conference July 15, 2017. Respiratory Care in FSHD: Overview. American Academy of Neurology Guidelines (2015) Incidence of respiratory insufficiency in FSHD Assessment of lung function (recommendations)
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Respiratory Care in FSHD Lee Guion MA, RRT, FAARC FSHD Family Day Conference July 15, 2017
Respiratory Care in FSHD: Overview • American Academy of Neurology Guidelines (2015) • Incidence of respiratory insufficiency in FSHD • Assessment of lung function (recommendations) • Treatment/management options (2015 AAN) • Chronic nocturnal respiratory insufficiency • Noninvasive bi-level positive pressure ventilation (NIV) • Acute respiratory failure (2017 ERS/ATS) • Noninvasive bi-level positive pressure ventilation (NIV) • Strategies for lung health (2017 LRG) • 7 Steps to healthy lungs
Respiratory Care in FSHD • American Academy of Neurology Guidelines (2015) Systematic review of medical literature focused exclusively on FSHD by panel of clinicians with FSHD expertise following AAN process (quality of research) • Respiratory abnormalities • Decreased lung function • Daytime symptoms of nocturnal hypoventilation • Frequency of respiratory insufficiency • 1.25% - 13% • Severity also difficult to estimate
Respiratory Care in FSHD • American Academy of Neurology Guidelines (2015) Respiratory insufficiency associated with: • Scoliosis or kyphoscoliosis • Obesity with abdominal distension • Severe proximal weakness • Wheelchair dependence • Co-morbidities: chronic pulmonary or cardiac disease • Respiratory muscle weakness (diaphragm)
Respiratory Care in FSHD • American Academy of Neurology Guidelines (2015) • Pulmonary assessment • Baseline spirometry on ALL patients • Monitor routinely if abnormal or with • Spinal abnormalities (scoliosis/kyphoscoliosis) • Decreased mobility or inability to ambulate • Lung or heart disease • Reported daytime symptoms of sleep disordered breathing
Respiratory Care in FSHD • American Academy of Neurology Guidelines (2015) • Sleep disordered breathing (SDB) • Causes • Weakened breathing muscles shallow breathing increased carbon dioxide/decreased oxygen fragmented sleep decreased REM sleep • Symptoms • Awakening unrefreshed • Feeling sleepy during the day and needing naps • Frequent nocturnal arousals • Morning headaches
Respiratory Care in FSHD • American Academy of Neurology Guidelines (2015) • Recommendations for respiratory insufficiency • (FVC <60% of predicted) or • reported symptoms of SDB • Referral to pulmonologist or sleep medicine specialist • Treatment with noninvasive positive pressure ventilation (NIV) • at night • to rest lung muscles during the day if needed
Respiratory Care in FSHD • Recommendations for acute hypercarbic (CO2) or hypoxemic (O2) respiratory failure • Noninvasive ventilation (NIV) • (European Respiratory Society/American Thoracic Society Guidelines 2017) • Recommendations for extubation and preventing reintubation • Noninvasive ventilation (NIV) • (American Thoracic Society/American College of Chest Physicians Guidelines 2017)
Respiratory Care in FSHD • Lee’s 7 steps to lung health STEP 1 • Attend a multidisciplinary muscular dystrophy clinic • Consistent, coordinated, integrated, compassionate care • One stop shop • Neurologist, RT, PT, OT, SW, representatives from support organizations (MDA) • Research participation opportunities
Respiratory Care in FSHD STEP 2 • Vaccinations • Pneumococcal polysaccharide pneumonia vaccine (PPSV23 – Pneumovax) • Pneumococcal conjugate vaccine (PCV13 – Prevnar) • Annual influenza vaccine (you and family members) • Avoid infective agents (hand washing, distance, masks) • Practice good oral hygiene. Get recommendations for adaptations from OT if brushing/flossing is difficult
Respiratory Care in FSHD STEP 3 • Treat symptoms of upper respiratory tract infection and seasonal rhinitis (to help reduce chances of lower airway infection) • Learn about early detection of lower respiratory tract infection (fever, malaise, prolonged lethargy) • Seek medical attention early and do not delay
Respiratory Care in FSHD STEP 4 • Good hydration • Maintain healthy electrolyte balance • Positive and negative ion exchange (largely sodium and chloride) will assist with normal mucus production, natural mucosal reabsorption and removal of lower respiratory tract secretions
Respiratory Care in FSHD STEP 5 • Maximize nutrition • Malnutrition increases risk of infection (immunosuppression) • Increased work of breathing/increased respiratory rate = more calories/energy consumed by breathing • Nutritional supplements if recommended • Smaller, more frequent meals • Consult nutritionist
Respiratory Care in FSHD STEP 6 • Movement and Safe Exercise • Stretching (reduces stiffness, improves circulation) • Moderation (do not overwork muscles, rest weak muscles) • Coordinate exercise/movement with breath work • Benefits • Increases blood neutrophil counts and • Helps maintain lymphocyte counts to improve immune function and response to infection • Improves gas exchange (oxygen/carbon dioxide) • Psychological/emotional benefits
Respiratory Care in FSHD STEP 7 • Get a good night’s sleep • Quantity (aim for 8 to 9 hours) • Quality (restorative sleep) • Awakening refreshed • Ability to be alert throughout the day • Address barriers to sleep • Shallow breathing and drops in oxygen • Pain or discomfort • Difficulty repositioning • Worry and intrusive thoughts
Respiratory Care in FSHD Thank You! guionlr@gmail.com Neurology Evidence-based guideline summary: Evaluation, diagnosis, and management of facioscapulohumeral muscular dystrophy: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology and the Practice Issues Review Panel of the American Association of Neuromuscular & Electrodiagnostic Medicine Rabi Tawil, John T. Kissel, Chad Heatwole, et al. Neurology 2015;85;357-364