Impact of an early respiratory care programme with non‐invasive ventilation adaptation in patients with amyotrophic lateral sclerosis

Vitacca, Michele; Montini, Alessandra de Ávila; Lunetta, Christian; Banfi, Paolo; Bertella, Enrica; Mattia, Elisa De; Lizio, Andrea; Volpato, Eleonora; Lax, Agata; Morini, Roberta; Paneroni, Mara

doi:10.1111/ene.13547

Cited by 69 publications

(69 citation statements)

References 28 publications

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“…[9][10][11] It has also been shown that NIV might also delay respiratory decline. 13,14 In this study, we found an overall median survival of 9.6 months, which is in line with previous publications. 13,14 In this study, we found an overall median survival of 9.6 months, which is in line with previous publications.…”

Section: Ventilation Parameterssupporting

confidence: 93%

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Non-invasive ventilation and hypercapnia-associated symptoms in amyotrophic lateral sclerosis

2018

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Objectives During the course of amyotrophic lateral sclerosis (ALS), progressive weakness of respiratory muscles leads to chronic hypercapnia which causes various symptoms like sleep disturbances, daytime fatigue, and depression. Non‐invasive ventilation (NIV) improves survival and quality of life, but little is known about its effect on these specific symptoms, in particular during the later course of disease. Our aim was to evaluate the short‐ and long‐term effects of NIV on hypercapnia‐associated symptoms in ALS. Material and Methods We prospectively analyzed sleep disturbance, daytime fatigue, and depression using standardized scales (Pittsburgh sleep quality index [PSQI], stanford sleepiness scale [SSS], Beck depression inventory [BDI], and Clinical hypoventilation score [CHS]) in 58 patients with NIV. Follow‐up was done every 3 months up to a maximum of 24 months. Results We found significant improvements of all outcome parameters except BDI within the first three months after NIV initiation. The median PSQI improved from 6.5 (95% CI: 5.0‐8.5) to 6.0 (95% CI: 4.5‐7.0; P = 0.042), the SSS from 3.0 (95% CI: 2.0‐4.0) to 2.0 (95% CI: 2.0‐3.0; P = 0.004), and the CHS from 22.0 (95% CI: 19.5‐25.0) to 18.0 points (95% CI: 12.0‐23.5; P = 0.013). Patients with bulbar and spinal onset were not significantly different, and positive effects were long‐lasting. Conclusions Our data show that NIV improves hypercapnia‐associated symptoms within the first 3 months after initiation in spinal as well as bulbar patients, and that beneficial effects are long‐lasting.

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Section: Ventilation Parameterssupporting

confidence: 93%

“…>80% might benefit from NIV. 13,14 In this study, we found an overall median survival of 9.6 months, which is in line with previous publications. 15 We did not find a signif- bulbar-onset patients.…”

Section: Recommendations) Even Patients With Forced Vital Capacity (Fvc)supporting

confidence: 93%

Non-invasive ventilation and hypercapnia-associated symptoms in amyotrophic lateral sclerosis

2018

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“…However, several arguments suggest that early ventilation could reduce the respiratory decline in ALS and decrease respiratory work . A large retrospective study and three small series have demonstrated an improvement in survival for patients initiating NIV with VC > 80% predicted, although this effect was not confirmed when sham NIV was used in the control group . In practice, patients with only minimal respiratory symptoms, essentially presenting features of motor disability, are unlikely to continue long‐term NIV if the therapy is initiated prior to exhibiting hypoventilation.…”

Section: Criteria For Initiation Of Nivmentioning

confidence: 99%

NIV in amyotrophic lateral sclerosis: The ‘when’ and ‘how’ of the matter

2019

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Non-invasive ventilation (NIV) has become an essential part of the treatment of amyotrophic lateral sclerosis (ALS) since 2006. NIV very significantly improves survival, quality of life and cognitive performances. The initial NIV settings are simple, but progression of the disease, ventilator dependence and upper airway involvement sometimes make long-term adjustment of NIV more difficult, with a major impact on survival. Unique data concerning the long-term adjustment of NIV in ALS show that correction of leaks, management of obstructive apnoea and adaptation to the patient's degree of ventilator dependence improve the prognosis. Non-ventilatory factors also impact the efficacy of NIV and various solutions have been described and must be applied, including cough assist techniques, control of excess salivation and renutrition. NIV in ALS has been considerably improved as a result of application of all of these measures, avoiding the need for tracheostomy in the very great majority of cases. More advanced use of NIV also requires pulmonologists to master the associated end-of-life palliative care, as well as the modalities of discontinuing ventilation when it becomes unreasonable. Respirology (2019) 24, 521-530 522 C Morelot-Panzini et al. ALS, amyotrophic lateral sclerosis; EPAP, expiratory positive airway pressure; I/E ratio, inspiratory time/expiratory time ratio; NIV, non-invasive ventilation; RR, respiratory rate; Ti, inspiratory time.Respirology (2019) 24, 521-530

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“…A recent retrospective study demonstrated that in a group of ALS patients initiated on NIV very early (i.e., with FVC ≥80%) and for at least 4 h/day or 120 h/month, the survival from symptom onset was significantly longer and the crude death rate was significantly lower compared to those patients initiated to NIV later (i.e., with FVC < 80%) [16, 17]. …”

Section: Rationale and Timing For Niv Applicationmentioning

confidence: 99%

“…Several studies have clearly shown that NIV treatment can prolong survival of NMD patients who accept and tolerate NIV compared to those who do not accept, tolerate or use it [17, 33, 110-115]. …”

Section: Ethical Dilemmasmentioning

confidence: 99%

Long-Term Ventilation in Neuromuscular Patients: Review of Concerns, Beliefs, and Ethical Dilemmas

Crimi

Pierucci²,

Carlucci

et al. 2019

Respiration

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Background: Noninvasive mechanical ventilation (NIV) is an effective treatment in patients with neuromuscular diseases (NMD) to improve symptoms, quality of life, and survival. Summary: NIV should be used early in the course of respiratory muscle involvement in NMD patients and its requirements may increase over time. Therefore, training on technical equipment at home and advice on problem solving are warranted. Remote monitoring of ventilator parameters using built-in ventilator software is recommended. Telemedicine may be helpful in reducing hospital admissions. Anticipatory planning and palliative care should be carried out to lessen the burden of care, to maintain or withdraw from NIV, and to guarantee the most respectful management in the last days of NMD patients’ life. Key Message: Long-term NIV is effective but challenging in NMD patients. Efforts should be made by health care providers in arranging a planned transition to home and end-of-life discussions for ventilator-assisted individuals and their families.

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Impact of an early respiratory care programme with non‐invasive ventilation adaptation in patients with amyotrophic lateral sclerosis

Abstract: Very early NIV prescription prolongs the free time from diagnosis to death in NB ALS patients whilst tracheotomy reduces the mortality risk in all patients.

Cited by 69 publications

References 28 publications

Non-invasive ventilation and hypercapnia-associated symptoms in amyotrophic lateral sclerosis

Non-invasive ventilation and hypercapnia-associated symptoms in amyotrophic lateral sclerosis

NIV in amyotrophic lateral sclerosis: The ‘when’ and ‘how’ of the matter

Long-Term Ventilation in Neuromuscular Patients: Review of Concerns, Beliefs, and Ethical Dilemmas

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