Non-invasive ventilation effectiveness and the effect of ventilatory mode on survival in ALS patients

Abstract Due to the expanding use of non-invasive ventilation (NIV) in amyotrophic lateral sclerosis (ALS), the question of enteral nutrition is increasingly raised in NIV users ALS patients. Here, we aimed to determine the prognostic factors for survival after gastrostomy placement in routine NIV users, taking into consideration ventilator dependence. Ninety-two routine NIV users ALS patients, who underwent gastrostomy insertion for severe dysphagia and/or weight loss, were included. We used a Cox proportional hazards model to identify factors affecting survival and compared time from gastrostomy to death and 30-day mortality rate between dependent (daily use ≥ 16 h) and non-dependent NIV users. The hazard of death after gastrostomy was significantly affected by 3 factors: age at onset (HR 1.047, p = 0.006), body mass index < 20 kg/m2 at the time of gastrostomy placement (HR 2.012, p = 0.016) and recurrent accumulation of airway secretions (HR 2.614, p = 0.001). Mean time from gastrostomy to death was significantly shorter in the dependent than in the non-dependent NIV users group (133 vs. 250 days, p = 0.04). The 30-day mortality rate was significantly higher in dependent NIV users (21.4% vs. 2.8%, p = 0.03). Pre-operative ventilator dependence and airway secretion accumulation are associated with worse prognosis and should be key decision-making criteria when considering gastrostomy tube placement in NIV users ALS patients.

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Use of nasal pillows in amiotrophic lateral sclerosis (ALS) patients with high non invasive ventilation (NIV) dependence

10.1183/13993003.congress-2018.pa2381 ◽

2018 ◽

Author(s):

Luana Focaraccio ◽

Giulia Rovere ◽

Roberta Di Tria ◽

Renza Ambrosanio ◽

Elisa Clivati ◽

...

Keyword(s):

Invasive Ventilation ◽

Non Invasive ◽

Non Invasive Ventilation ◽

Als Patients ◽

Lateral Sclerosis

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Continuing Non-Invasive Ventilation During Amyotrophic Lateral Sclerosis-Related Hospice Care Is Medically, Administratively, and Financially Feasible

American Journal of Hospice and Palliative Medicine® ◽

10.1177/1049909120982291 ◽

2020 ◽

pp. 104990912098229

Author(s):

Philip J. Choi ◽

Michael Murn ◽

Roberta Turner ◽

Richard Bedlack

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Length Of Stay ◽

Cost Analysis ◽

Rating Scale ◽

Academic Medical Center ◽

Invasive Ventilation ◽

Non Invasive ◽

Non Invasive Ventilation ◽

Als Patients ◽

Lateral Sclerosis

Background: Amyotrophic Lateral Sclerosis (ALS) is a terminal neuromuscular disease with patients dying within 3-5 years of diagnosis. Most patients choose to forego invasive life sustaining measures. Timing of hospice referral can be challenging given the advancement of non-invasive ventilation (NIV) technology. Objective: To describe the characteristics of patients enrolled in hospice from an ALS clinic at 1 academic medical center and to perform a cost analysis for patients who remained on ventilator support. Methods: Retrospective cross-sectional study of patients enrolled in hospice over a 2-year period. Clinical characteristics included ALS Functional Rating Scale Revised (ALSFRS-R) score, Forced Vital Capacity (FVC), use of NIV and mechanical insufflation-exsufflation (MIE), riluzole use, and length of stay in hospice. A cost analysis was performed for patients enrolled in Duke Home Care and Hospice. Results: 85 of 104 patients who died were enrolled in hospice. Median days enrolled in hospice was 84. Patients who continued on NIV had similar hospice length of stay as those on no respiratory support (88 versus 80 days, p = 0.83). Bulbar patients had a trend toward shorter length of stay in hospice than limb onset patients (71 versus 101 days, p = 0.49). Cost analysis showed that hospice maintained a mean net operating revenue of $3234.50 per patient who continued on NIV. Conclusions: Hospice referrals for ALS patients on NIV can be challenging. This study shows that even with continued NIV use, most ALS patients die within the expected 6 months on home hospice, and care remains cost effective for hospice agencies.

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