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Noninvasive Ventilation in Neuromuscular Disease
Author(s)
Sreelatha Naik, MD, FCCP
Kelly Lobrutto, CRNP

Click to view more from Pulmonology Data Trends 2024. 

References
  1. Gong Y, Sankari A. Noninvasive ventilation. StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2024.Updated December 11, 2022. Accessed June 19, 2024. https://www.ncbi.nlm.nih.gov/books/NBK578188/
  2. Khan A, Frazer-Green L, Amin R, et al. Respiratory management of patients with neuromuscular weakness: an American College of Chest Physicians clinical practice guideline and expert panel report. Chest. 2023;164(2):394-413. doi:10.1016/j.chest.2023.03.011
  3. Taran S, McCredie VA, Goligher EC. Noninvasive and invasive mechanical ventilation for neurologic disorders. Handb Clin Neurol. 2022;189:361-386. doi:10.1016/B978-0-323-91532-8.00015-X
  4. Rao F, Garuti G, Vitacca M, et al; for the UILDM Respiratory Group. Management of respiratory complications and rehabilitation in individuals with muscular dystrophies: 1st Consensus Conference report from UILDM - Italian Muscular Dystrophy Association (Milan, January 25-26, 2019). Acta Myol. 2021;40(1):8-42. doi:10.36185/2532-1900-045
  5. Respiratory assist devices. Centers for Medicare & Medicaid Services. Revised January 1, 2024. Accessed June 19, 2024. https://www.cms.gov/ medicare-coverage-database/view/lcd.aspx?lcdid=33800
  6. What you need to know about the Philips PAP device recalls. American College of Chest Physicians. February 1, 2024. Accessed June 19, 2024. https://www.chestnet.org/Newsroom/CHEST-News/2021/07/What-YouNeed-to-Know-About-the-Philips-PAP-Device-Recall
  7. Orr JE, Chen K, Vaida F, et al. Effectiveness of long-term noninvasive ventilation measured by remote monitoring in neuromuscular disease. ERJ Open Res. 2023;9(5):00163-2023. doi:10.1183/23120541.00163-2023
  8. Kapur VK, Auckley DH, Chowdhuri S, et al. Clinical practice guideline for diagnostic testing for adult obstructive sleep apnea: an American Academy of Sleep Medicine clinical practice guideline. J Clin Sleep Med. 2017;13(3):479-504. doi:10.5664/jcsm.6506
  9. Phillips Respironics. Trilogy Evo Clinical Manual. 2019
  10. ResMed. Astral Series Clinical Guide. 2018
  11. Breas. Vivo 45 LS User Manual. 2023
  12. Lowenstein Medical. Luisa Life Support Ventilation. 
  13. Ventec Life Systems. VOCSN Clinical and Technical Manual. 2019
Author and Disclosure Information

Sreelatha Naik, MD
Assistant Professor of Medicine
Program Director, Sleep Medicine Fellowship, Divison of Pulmonary, Critical Care and Sleep Medicine
Geisinger Commonwealth School of Medicine
Divison Chief
Geisinger Northeast Region
Divison of Pulmonary, Critical Care and Sleep Medicine
Geisinger Health System
Scranton, PA

Sreelatha Naik, MD, has disclosed the following relevant financial relationships:
Serve(d) as a speaker or a member of a speakers bureau for: 2 talks for ResMed

Kelly LoBrutto, CRNP
Nurse Practitioner
Pulmonary Medicine
Geisinger Health System
Wilkes Barre, PA

Kelly LoBrutto, MSN, has disclosed no relevant financial relationships.

Publications
MDedge Internal Medicine
CHEST Physician
Topics
Pulmonology
Mixed Topics
Author(s)
Sreelatha Naik, MD, FCCP
Kelly Lobrutto, CRNP
Author(s)
Sreelatha Naik, MD, FCCP
Kelly Lobrutto, CRNP
Author and Disclosure Information

Sreelatha Naik, MD
Assistant Professor of Medicine
Program Director, Sleep Medicine Fellowship, Divison of Pulmonary, Critical Care and Sleep Medicine
Geisinger Commonwealth School of Medicine
Divison Chief
Geisinger Northeast Region
Divison of Pulmonary, Critical Care and Sleep Medicine
Geisinger Health System
Scranton, PA

Sreelatha Naik, MD, has disclosed the following relevant financial relationships:
Serve(d) as a speaker or a member of a speakers bureau for: 2 talks for ResMed

Kelly LoBrutto, CRNP
Nurse Practitioner
Pulmonary Medicine
Geisinger Health System
Wilkes Barre, PA

Kelly LoBrutto, MSN, has disclosed no relevant financial relationships.

Author and Disclosure Information

Sreelatha Naik, MD
Assistant Professor of Medicine
Program Director, Sleep Medicine Fellowship, Divison of Pulmonary, Critical Care and Sleep Medicine
Geisinger Commonwealth School of Medicine
Divison Chief
Geisinger Northeast Region
Divison of Pulmonary, Critical Care and Sleep Medicine
Geisinger Health System
Scranton, PA

Sreelatha Naik, MD, has disclosed the following relevant financial relationships:
Serve(d) as a speaker or a member of a speakers bureau for: 2 talks for ResMed

Kelly LoBrutto, CRNP
Nurse Practitioner
Pulmonary Medicine
Geisinger Health System
Wilkes Barre, PA

Kelly LoBrutto, MSN, has disclosed no relevant financial relationships.

Click to view more from Pulmonology Data Trends 2024. 

Click to view more from Pulmonology Data Trends 2024. 

References
  1. Gong Y, Sankari A. Noninvasive ventilation. StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2024.Updated December 11, 2022. Accessed June 19, 2024. https://www.ncbi.nlm.nih.gov/books/NBK578188/
  2. Khan A, Frazer-Green L, Amin R, et al. Respiratory management of patients with neuromuscular weakness: an American College of Chest Physicians clinical practice guideline and expert panel report. Chest. 2023;164(2):394-413. doi:10.1016/j.chest.2023.03.011
  3. Taran S, McCredie VA, Goligher EC. Noninvasive and invasive mechanical ventilation for neurologic disorders. Handb Clin Neurol. 2022;189:361-386. doi:10.1016/B978-0-323-91532-8.00015-X
  4. Rao F, Garuti G, Vitacca M, et al; for the UILDM Respiratory Group. Management of respiratory complications and rehabilitation in individuals with muscular dystrophies: 1st Consensus Conference report from UILDM - Italian Muscular Dystrophy Association (Milan, January 25-26, 2019). Acta Myol. 2021;40(1):8-42. doi:10.36185/2532-1900-045
  5. Respiratory assist devices. Centers for Medicare & Medicaid Services. Revised January 1, 2024. Accessed June 19, 2024. https://www.cms.gov/ medicare-coverage-database/view/lcd.aspx?lcdid=33800
  6. What you need to know about the Philips PAP device recalls. American College of Chest Physicians. February 1, 2024. Accessed June 19, 2024. https://www.chestnet.org/Newsroom/CHEST-News/2021/07/What-YouNeed-to-Know-About-the-Philips-PAP-Device-Recall
  7. Orr JE, Chen K, Vaida F, et al. Effectiveness of long-term noninvasive ventilation measured by remote monitoring in neuromuscular disease. ERJ Open Res. 2023;9(5):00163-2023. doi:10.1183/23120541.00163-2023
  8. Kapur VK, Auckley DH, Chowdhuri S, et al. Clinical practice guideline for diagnostic testing for adult obstructive sleep apnea: an American Academy of Sleep Medicine clinical practice guideline. J Clin Sleep Med. 2017;13(3):479-504. doi:10.5664/jcsm.6506
  9. Phillips Respironics. Trilogy Evo Clinical Manual. 2019
  10. ResMed. Astral Series Clinical Guide. 2018
  11. Breas. Vivo 45 LS User Manual. 2023
  12. Lowenstein Medical. Luisa Life Support Ventilation. 
  13. Ventec Life Systems. VOCSN Clinical and Technical Manual. 2019
References
  1. Gong Y, Sankari A. Noninvasive ventilation. StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2024.Updated December 11, 2022. Accessed June 19, 2024. https://www.ncbi.nlm.nih.gov/books/NBK578188/
  2. Khan A, Frazer-Green L, Amin R, et al. Respiratory management of patients with neuromuscular weakness: an American College of Chest Physicians clinical practice guideline and expert panel report. Chest. 2023;164(2):394-413. doi:10.1016/j.chest.2023.03.011
  3. Taran S, McCredie VA, Goligher EC. Noninvasive and invasive mechanical ventilation for neurologic disorders. Handb Clin Neurol. 2022;189:361-386. doi:10.1016/B978-0-323-91532-8.00015-X
  4. Rao F, Garuti G, Vitacca M, et al; for the UILDM Respiratory Group. Management of respiratory complications and rehabilitation in individuals with muscular dystrophies: 1st Consensus Conference report from UILDM - Italian Muscular Dystrophy Association (Milan, January 25-26, 2019). Acta Myol. 2021;40(1):8-42. doi:10.36185/2532-1900-045
  5. Respiratory assist devices. Centers for Medicare & Medicaid Services. Revised January 1, 2024. Accessed June 19, 2024. https://www.cms.gov/ medicare-coverage-database/view/lcd.aspx?lcdid=33800
  6. What you need to know about the Philips PAP device recalls. American College of Chest Physicians. February 1, 2024. Accessed June 19, 2024. https://www.chestnet.org/Newsroom/CHEST-News/2021/07/What-YouNeed-to-Know-About-the-Philips-PAP-Device-Recall
  7. Orr JE, Chen K, Vaida F, et al. Effectiveness of long-term noninvasive ventilation measured by remote monitoring in neuromuscular disease. ERJ Open Res. 2023;9(5):00163-2023. doi:10.1183/23120541.00163-2023
  8. Kapur VK, Auckley DH, Chowdhuri S, et al. Clinical practice guideline for diagnostic testing for adult obstructive sleep apnea: an American Academy of Sleep Medicine clinical practice guideline. J Clin Sleep Med. 2017;13(3):479-504. doi:10.5664/jcsm.6506
  9. Phillips Respironics. Trilogy Evo Clinical Manual. 2019
  10. ResMed. Astral Series Clinical Guide. 2018
  11. Breas. Vivo 45 LS User Manual. 2023
  12. Lowenstein Medical. Luisa Life Support Ventilation. 
  13. Ventec Life Systems. VOCSN Clinical and Technical Manual. 2019
Publications
MDedge Internal Medicine
CHEST Physician
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MDedge Internal Medicine
CHEST Physician
Topics
Pulmonology
Mixed Topics
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Noninvasive Ventilation in Neuromuscular Disease
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Noninvasive Ventilation in Neuromuscular Disease
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Noninvasive ventilation (NIV) delivers oxygen into the lungs via positive pressure without the need for endotracheal intubation and is typically used in COPD, obesity hypoventilation syndrome, and neuromuscular disease (NMD).1 Clinicians are used to recognizing pulmonary diseases that require ventilation, but NMDs—in which early intervention is critical due to its effect on respiration—are often overlooked. Emerging data show that patients with lung function even at 80% may benefit from early NIV in the long term.2 NMDs that benefit from NIV include amyotrophic lateral sclerosis (ALS), myasthenia gravis, and muscular dystrophies.2-4

New CHEST guidelines for NMD respiratory management provide guidance on the timing of pulmonary function testing, when to initiate NIV, and how to manage sleep-disordered breathing.2 Clinicians should be aware of inconsistencies between CHEST and Medicare/insurance reimbursement guidelines.5 For example, current Medicare/insurance guidelines require vital capacity to be < 50% to treat with NIV, whereas CHEST guidelines recommend a threshold of 80% if a patient is symptomatic based on more recent evidence.5

Due largely to increased respiratory fragility during the COVID-19 pandemic, there has been an increased need for NIV and home ventilation (HMV) devices, and the number of available devices has also expanded due to the NIV recall.6,7 These new ventilators each have their own unique features that can optimize to certain conditions and populations and more data is now available to address previously unanswered treatment questions.6 Data on measures, such as mode, observed overall usage, respiratory rates, tidal volumes, and pressures, can now help determine optimal ventilator use and long-term outcomes in NMDs.6

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