Long-Term NIV in NMD PatientsSrivari
Long-Term NIV in NMD Patients
Long-Term Ventilation in Neuromuscular Patients: Review of Concerns, Beliefs, and Ethical Dilemmas
Claudia Crimi, Paola Pierucci, Annalisa Carlucci, Andrea Cortegiani. Cesare Gregoretti; Respiration 2019;97:185-196.
Approx. 10-51% of indications for home mechanical ventilation are patients with neuromuscular disorders (NMD). Noninvasive ventilation (NIV) is an accepted and effective ventilation support for NMD patients. In this review timing of NIV initialization and initialization of invasive ventilation are discussed as well as the choice of modes and at-home monitoring, training and ethical aspects of end-of-life use.
NMD patients benefit from preventive early NIV (improving HrQoL, survival, etc.) However, life on NIV is – even though the newest features have simplified use and comfort – challenging. There is little educational support and few home care programs are available. Regular data monitoring provided by the device is recommended, and telemonitoring might be helpful in delaying hospital admission. End-of-life discussions should be held as early as possible in a positive surrounding.
(1) Initialization of NIV
Preventive NIV has shown positive effects in patients with rapidly progressing NMD. Pulmonary function tests (PFT) are recommended for assessing respiratory muscle impairment. Even though the guidelines consider a forced vital capacity (FVC) of <80% of predicted values with symptoms of respiratory impairment to initiate NIV, there are studies suggesting an even earlier initialization, which is associated with benefits for survival. In the case of nocturnal hypoventilation or sleep-disordered breathing (SDB), which is associated with negative outcomes, NIV with a backup rate is recommended.
(2) Modes: Pressure controlled / volume-controlled / target volume / mouth piece
There is no significant difference in volume- or pressure-controlled (PCV/ST/PCV vs. VCV/AVCV) modes. Ti min/max should be set e.g. to prevent prolonged inspiratory phase, which can lead to asynchrony. Volume-target pressure-controlled ventilation maintains adequate volume without unnecessarily high-pressure levels but there is no clear evidence of superiority over conventional settings. Mouthpiece ventilation is beneficial in terms of air stacking. PEEP in NMD patients is rarely needed but is beneficial in coexisting OSA. The synergic combination of NIV and cough assistance can prolong the successful use of NIV.
someone recommends oximetry as the first screening to prioritize patients in need of further nocturnal investigation, such as capnometry or PSG. Remote monitoring via the built-in software in devices provides reliable results. Nurse-led teleassistance programs and telemonitoring are appreciated by patients and can be a promising strategy but further research is needed.
(4) Home Care Management
The preferred location for NMD patients should be at home; it maximizes the quality of life and is less expensive than a hospital setup. Integrated health solutions for chronic disease management (as in France) are needed for focused interventions, there are rarely available yet, though.
(5) Ethical questions
Even though studies show a prolonged survival when using NIV HrQoL might be impaired. 30% of ALS patients can be intolerant to NIV due to various reasons (physical and psychological). End-of-life treatments should be an integrated part of the early treatment plan.