Abstract
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.
References
1.
Gregoretti
C
, Pisani
L
, Cortegiani
A
, Ranieri
VM
. Noninvasive ventilation in critically ill patients
. Crit Care Clin
. 2015
Jul
;31
(3
):435
–57
.
[PubMed]
0749-07042.
Cortegiani
A
, Russotto
V
, Antonelli
M
, Azoulay
E
, Carlucci
A
, Conti
G
, et al. Ten important articles on noninvasive ventilation in critically ill patients and insights for the future: A report of expert opinions
. BMC Anesthesiol
. 2017
Sep
;17
(1
):122
.
[PubMed]
1471-22533.
Racca
F
, Mongini
T
, Wolfler
A
, Vianello
A
, Cutrera
R
, Del Sorbo
L
, et al. Recommendations for anesthesia and perioperative management of patients with neuromuscular disorders
. Minerva Anestesiol
. 2013
Apr
;79
(4
):419
–33
.
[PubMed]
1827-15964.
Mehta
S
, Hill
NS
. Noninvasive ventilation
. Am J Respir Crit Care Med
. 2001
Feb
;163
(2
):540
–77
.
[PubMed]
1073-449X5.
Windisch
W
; Quality of life in home mechanical ventilation study group
. Impact of home mechanical ventilation on health-related quality of life
. Eur Respir J
. 2008
Nov
;32
(5
):1328
–36
.
[PubMed]
0903-19366.
Veale
D
, Gonzalez-Bermejo
J
, Borel
JC
, Rida
Z
, Pontier
S
, Muir
JF
, et al.; groupe CasaVNI
. [Initiation of long-term non-invasive ventilation at home: current practices and expected issues. Surveys from the CasaVNI working party]
. Rev Mal Respir
. 2010
Nov
;27
(9
):1022
–9
.
[PubMed]
0761-84257.
Nasiłowski
J
, Wachulski
M
, Trznadel
W
, Andrzejewski
W
, Migdał
M
, Drozd
W
, et al. The evolution of home mechanical ventilation in poland between 2000 and 2010
. Respir Care
. 2015
Apr
;60
(4
):577
–85
.
[PubMed]
0020-13248.
Lloyd-Owen
SJ
, Donaldson
GC
, Ambrosino
N
, Escarabill
J
, Farre
R
, Fauroux
B
, et al. Patterns of home mechanical ventilation use in Europe: results from the Eurovent survey
. Eur Respir J
. 2005
Jun
;25
(6
):1025
–31
.
[PubMed]
0903-19369.
MacIntyre
EJ
, Asadi
L
, Mckim
DA
, Bagshaw
SM
. Clinical Outcomes Associated with Home Mechanical Ventilation: A Systematic Review
. Can Respir J
. 2016
;2016
:6547180
.
[PubMed]
1198-224110.
Guideline
N.
Motor neurone disease: assessment and management [NG42]2016
.11.
Andersen
PM
, Abrahams
S
, Borasio
GD
, de Carvalho
M
, Chio
A
, Van Damme
P
, et al.; EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis
. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)—revised report of an EFNS task force
. Eur J Neurol
. 2012
Mar
;19
(3
):360
–75
.
[PubMed]
1351-510112.
McKim
DA
, Road
J
, Avendano
M
, Abdool
S
, Cote
F
, Duguid
N
, et al.; Canadian Thoracic Society Home Mechanical Ventilation Committee
. Home mechanical ventilation: a Canadian Thoracic Society clinical practice guideline
. Can Respir J
. 2011
Jul-Aug
;18
(4
):197
–215
.
[PubMed]
1198-224113.
Clinical indications for noninvasive positive pressure ventilation in chronic respiratory failure due to restrictive lung disease, COPD, and nocturnal hypoventilation—a consensus conference report
. Chest
. 1999
Aug
;116
(2
):521
–34
.
[PubMed]
0012-369214.
Boentert
M
, Glatz
C
, Helmle
C
, Okegwo
A
, Young
P
. Prevalence of sleep apnoea and capnographic detection of nocturnal hypoventilation in amyotrophic lateral sclerosis
. J Neurol Neurosurg Psychiatry
. 2018
Apr
;89
(4
):418
–24
.
[PubMed]
0022-305015.
Carratù
P
, Cassano
A
, Gadaleta
F
, Tedone
M
, Dongiovanni
S
, Fanfulla
F
, et al. Association between low sniff nasal-inspiratory pressure (SNIP) and sleep disordered breathing in amyotrophic lateral sclerosis: preliminary results
. Amyotroph Lateral Scler
. 2011
Nov
;12
(6
):458
–63
.
[PubMed]
1748-296816.
Lechtzin
N
, Scott
Y
, Busse
AM
, Clawson
LL
, Kimball
R
, Wiener
CM
. Early use of non-invasive ventilation prolongs survival in subjects with ALS
. Amyotroph Lateral Scler
. 2007
Jun
;8
(3
):185
–8
.
[PubMed]
1748-296817.
Vitacca
M
, Montini
A
, Lunetta
C
, Banfi
P
, Bertella
E
, De Mattia
E
, et al.; ALS RESPILOM Study Group
. Impact of an early respiratory care programme with non-invasive ventilation adaptation in patients with amyotrophic lateral sclerosis
. Eur J Neurol
. 2018
Mar
;25
(3
):556
–e33
.
[PubMed]
1351-510118.
Birnkrant
DJ
, Bushby
K
, Bann
CM
, Alman
BA
, Apkon
SD
, Blackwell
A
, et al.; DMD Care Considerations Working Group
. Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management
. Lancet Neurol
. 2018
Apr
;17
(4
):347
–61
.
[PubMed]
1474-442219.
Hull
J
, Aniapravan
R
, Chan
E
, Chatwin
M
, Forton
J
, Gallagher
J
, et al. British Thoracic Society guideline for respiratory management of children with neuromuscular weakness
. Thorax
. 2012
Jul
;67
Suppl 1
:i1
–40
.
[PubMed]
0040-637620.
Bradley
WG
, Anderson
F
, Bromberg
M
, Gutmann
L
, Harati
Y
, Ross
M
, et al.; ALS CARE Study Group
; The American Academy of Neurology
. Current management of ALS: comparison of the ALS CARE Database and the AAN Practice Parameter
. Neurology
. 2001
Aug
;57
(3
):500
–4
.
[PubMed]
0028-387821.
Bourke
SC
, Gibson
GJ
. Non-invasive ventilation in ALS: current practice and future role
. Amyotroph Lateral Scler Other Motor Neuron Disord
. 2004
Jun
;5
(2
):67
–71
.
[PubMed]
1466-082222.
Vry
J
, Gramsch
K
, Rodger
S
, Thompson
R
, Steffensen
BF
, Rahbek
J
, et al. European Cross-Sectional Survey of Current Care Practices for Duchenne Muscular Dystrophy Reveals Regional and Age-Dependent Differences
. J Neuromuscul Dis
. 2016
Nov
;3
(4
):517
–27
.
[PubMed]
2214-359923.
Andersen
PM
, Kuzma-Kozakiewicz
M
, Keller
J
, Aho-Oezhan
HE
, Ciecwierska
K
, Szejko
N
, et al. Therapeutic decisions in ALS patients: cross-cultural differences and clinical implications
. J Neurol
. 2018
Jul
;265
(7
):1600
–6
.
[PubMed]
0340-535424.
Nava
S
, Navalesi
P
, Gregoretti
C
. Interfaces and humidification for noninvasive mechanical ventilation
. Respir Care
. 2009
Jan
;54
(1
):71
–84
.
[PubMed]
0020-132425.
Racca
F
, Appendini
L
, Berta
G
, Barberis
L
, Vittone
F
, Gregoretti
C
, et al. Helmet ventilation for acute respiratory failure and nasal skin breakdown in neuromuscular disorders
. Anesth Analg
. 2009
Jul
;109
(1
):164
–7
.
[PubMed]
0003-299926.
Mj
T
, editor. Principles and Practice of Mechanical Ventilation: McGraw-Hill Education / Medical
. 2013
. pp. 683
–97
.27.
Mehta
S
, McCool
FD
, Hill
NS
. Leak compensation in positive pressure ventilators: a lung model study
. Eur Respir J
. 2001
Feb
;17
(2
):259
–67
.
[PubMed]
0903-193628.
Storre
JH
, Bohm
P
, Dreher
M
, Windisch
W
. Clinical impact of leak compensation during non-invasive ventilation
. Respir Med
. 2009
Oct
;103
(10
):1477
–83
.
[PubMed]
0954-611129.
Gregoretti
CN
, Ghannadian
S
, Carlucci
A
, Pelosi
P
. Choosing a ventilator for home mechanical ventilation
. Breathe (Sheff)
. 2013
;9
(5
):394
–409
. 1810-683830.
Toussaint
M
, Chatwin
M
, Soudon
P
. Mechanical ventilation in Duchenne patients with chronic respiratory insufficiency: clinical implications of 20 years published experience
. Chron Respir Dis
. 2007
;4
(3
):167
–77
.
[PubMed]
1479-972331.
Toussaint
M
, Soudon
P
, Kinnear
W
. Effect of non-invasive ventilation on respiratory muscle loading and endurance in patients with Duchenne muscular dystrophy
. Thorax
. 2008
May
;63
(5
):430
–4
.
[PubMed]
0040-637632.
Toussaint
M
, Steens
M
, Wasteels
G
, Soudon
P
. Diurnal ventilation via mouthpiece: survival in end-stage Duchenne patients
. Eur Respir J
. 2006
Sep
;28
(3
):549
–55
.
[PubMed]
0903-193633.
Bourke
SC
, Tomlinson
M
, Williams
TL
, Bullock
RE
, Shaw
PJ
, Gibson
GJ
. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial
. Lancet Neurol
. 2006
Feb
;5
(2
):140
–7
.
[PubMed]
1474-442234.
Farrero
E
, Prats
E
, Povedano
M
, Martinez-Matos
JA
, Manresa
F
, Escarrabill
J
. Survival in amyotrophic lateral sclerosis with home mechanical ventilation: the impact of systematic respiratory assessment and bulbar involvement
. Chest
. 2005
Jun
;127
(6
):2132
–8
.
[PubMed]
0012-369235.
Aboussouan
LS
, Khan
SU
, Meeker
DP
, Stelmach
K
, Mitsumoto
H
. Effect of noninvasive positive-pressure ventilation on survival in amyotrophic lateral sclerosis
. Ann Intern Med
. 1997
Sep
;127
(6
):450
–3
.
[PubMed]
0003-481936.
Sancho
J
, Servera
E
, Morelot-Panzini
C
, Salachas
F
, Similowski
T
, Gonzalez-Bermejo
J
. Non-invasive ventilation effectiveness and the effect of ventilatory mode on survival in ALS patients
. Amyotroph Lateral Scler Frontotemporal Degener
. 2014
Mar
;15
(1-2
):55
–61
.
[PubMed]
2167-842137.
Georges
M
, Attali
V
, Golmard
JL
, Morélot-Panzini
C
, Crevier-Buchman
L
, Collet
JM
, et al. Reduced survival in patients with ALS with upper airway obstructive events on non-invasive ventilation
. J Neurol Neurosurg Psychiatry
. 2016
Oct
;87
(10
):1045
–50
.
[PubMed]
0022-305038.
Andersen
TM
, Sandnes
A
, Fondenes
O
, Nilsen
RM
, Tysnes
OB
, Heimdal
JH
, et al. Laryngeal Responses to Mechanically Assisted Cough in Progressing Amyotrophic Lateral Sclerosis
. Respir Care
. 2018
May
;63
(5
):538
–49
.
[PubMed]
0020-132439.
Gonzalez-Bermejo
J
, Perrin
C
, Janssens
JP
, Pepin
JL
, Mroue
G
, Léger
P
, et al.; SomnoNIV Group
. Proposal for a systematic analysis of polygraphy or polysomnography for identifying and scoring abnormal events occurring during non-invasive ventilation
. Thorax
. 2012
Jun
;67
(6
):546
–52
.
[PubMed]
0040-637640.
Schönhofer
B
, Sonneborn
M
, Haidl
P
, Böhrer
H
, Köhler
D
. Comparison of two different modes for noninvasive mechanical ventilation in chronic respiratory failure: volume versus pressure controlled device
. Eur Respir J
. 1997
Jan
;10
(1
):184
–91
.
[PubMed]
0903-193641.
Smith
IE
, Shneerson
JM
. Secondary failure of nasal intermittent positive pressure ventilation using the Monnal D: effects of changing ventilator
. Thorax
. 1997
Jan
;52
(1
):89
–91
.
[PubMed]
0040-637642.
Tuggey
JM
, Elliott
MW
. Randomised crossover study of pressure and volume non-invasive ventilation in chest wall deformity
. Thorax
. 2005
Oct
;60
(10
):859
–64
.
[PubMed]
0040-637643.
Fanfulla
F
, Delmastro
M
, Berardinelli
A
, Lupo
ND
, Nava
S
. Effects of different ventilator settings on sleep and inspiratory effort in patients with neuromuscular disease
. Am J Respir Crit Care Med
. 2005
Sep
;172
(5
):619
–24
.
[PubMed]
1073-449X44.
Rabec
C
, Rodenstein
D
, Leger
P
, Rouault
S
, Perrin
C
, Gonzalez-Bermejo
J
; SomnoNIV group
. Ventilator modes and settings during non-invasive ventilation: effects on respiratory events and implications for their identification
. Thorax
. 2011
Feb
;66
(2
):170
–8
.
[PubMed]
0040-637645.
Janssens
JP
, Derivaz
S
, Breitenstein
E
, De Muralt
B
, Fitting
JW
, Chevrolet
JC
, et al. Changing patterns in long-term noninvasive ventilation: a 7-year prospective study in the Geneva Lake area
. Chest
. 2003
Jan
;123
(1
):67
–79
.
[PubMed]
0012-369246.
Lofaso
F
, Prigent
H
, Tiffreau
V
, Menoury
N
, Toussaint
M
, Monnier
AF
, et al.; Association Française Contre les Myopathies research group
. Long-term mechanical ventilation equipment for neuromuscular patients: meeting the expectations of patients and prescribers
. Respir Care
. 2014
Jan
;59
(1
):97
–106
.
[PubMed]
0020-132447.
Carlucci
A
, Schreiber
A
, Mattei
A
, Malovini
A
, Bellinati
J
, Ceriana
P
, et al. The configuration of bi-level ventilator circuits may affect compensation for non-intentional leaks during volume-targeted ventilation
. Intensive Care Med
. 2013
Jan
;39
(1
):59
–65
.
[PubMed]
0342-464248.
Khirani
S
, Louis
B
, Leroux
K
, Delord
V
, Fauroux
B
, Lofaso
F
. Harms of unintentional leaks during volume targeted pressure support ventilation
. Respir Med
. 2013
Jul
;107
(7
):1021
–9
.
[PubMed]
0954-611149.
Calderini
E
, Confalonieri
M
, Puccio
PG
, Francavilla
N
, Stella
L
, Gregoretti
C
. Patient-ventilator asynchrony during noninvasive ventilation: the role of expiratory trigger
. Intensive Care Med
. 1999
Jul
;25
(7
):662
–7
.
[PubMed]
0342-464250.
Bach
JR
, Alba
AS
, Shin
D
. Management alternatives for post-polio respiratory insufficiency. Assisted ventilation by nasal or oral-nasal interface
. Am J Phys Med Rehabil
. 1989
Dec
;68
(6
):264
–71
.
[PubMed]
0894-911551.
Mj
T
, editor. Principles and Practice of Mechanical Ventilation
. McGraw-Hill
; 2013
. pp. 761
–75
.52.
53.
Arellano-Maric
MP
, Gregoretti
C
, Duiverman
M
, Windisch
W
. Long-term volume-targeted pressure-controlled ventilation: sense or nonsense?
Eur Respir J
. 2017
Jun
;49
(6
):1602193
.
[PubMed]
0903-193654.
Amato
MB
, Barbas
CS
, Bonassa
J
, Saldiva
PH
, Zin
WA
, de Carvalho
CR
. Volume-assured pressure support ventilation (VAPSV). A new approach for reducing muscle workload during acute respiratory failure
. Chest
. 1992
Oct
;102
(4
):1225
–34
.
[PubMed]
0012-369255.
Jaye
J
, Chatwin
M
, Dayer
M
, Morrell
MJ
, Simonds
AK
. Autotitrating versus standard noninvasive ventilation: a randomised crossover trial
. Eur Respir J
. 2009
Mar
;33
(3
):566
–71
.
[PubMed]
0903-193656.
Kelly
JL
, Jaye
J
, Pickersgill
RE
, Chatwin
M
, Morrell
MJ
, Simonds
AK
. Randomized trial of ‘intelligent’ autotitrating ventilation versus standard pressure support non-invasive ventilation: impact on adherence and physiological outcomes
. Respirology
. 2014
May
;19
(4
):596
–603
.
[PubMed]
1323-779957.
Crescimanno
G
, Marrone
O
, Vianello
A
. Efficacy and comfort of volume-guaranteed pressure support in patients with chronic ventilatory failure of neuromuscular origin
. Respirology
. 2011
May
;16
(4
):672
–9
.
[PubMed]
1323-779958.
Orlikowski
D
, Mroue
G
, Prigent
H
, Moulin
C
, Bohic
M
, Ruquet
M
, et al. Automatic air-leak compensation in neuromuscular patients: a feasibility study
. Respir Med
. 2009
Feb
;103
(2
):173
–9
.
[PubMed]
0954-611159.
Katzberg
HD
, Selegiman
A
, Guion
L
, Yuan
N
, Cho
SC
, Katz
JS
, et al. Effects of noninvasive ventilation on sleep outcomes in amyotrophic lateral sclerosis
. J Clin Sleep Med
. 2013
Apr
;9
(4
):345
–51
.
[PubMed]
1550-938960.
Annane
D
, Orlikowski
D
, Chevret
S
. Nocturnal mechanical ventilation for chronic hypoventilation in patients with neuromuscular and chest wall disorders
. Cochrane Database Syst Rev
. 2014
Dec
;(12
):CD001941
.
[PubMed]
1469-493X61.
Vignaux
L
, Vargas
F
, Roeseler
J
, Tassaux
D
, Thille
AW
, Kossowsky
MP
, et al. Patient-ventilator asynchrony during non-invasive ventilation for acute respiratory failure: a multicenter study
. Intensive Care Med
. 2009
May
;35
(5
):840
–6
.
[PubMed]
0342-464262.
Vrijsen
B
, Chatwin
M
, Contal
O
, Derom
E
, Janssens
JP
, Kampelmacher
MJ
, et al. Hot Topics in Noninvasive Ventilation: Report of a Working Group at the International Symposium on Sleep-Disordered Breathing in Leuven, Belgium
. Respir Care
. 2015
Sep
;60
(9
):1337
–62
.
[PubMed]
0020-132463.
Gregoretti
C
, Foti
G
, Beltrame
F
, Giugiaro
PM
, Biolino
P
, Burbi
L
, et al. Pressure control ventilation and minitracheotomy in treating severe flail chest trauma
. Intensive Care Med
. 1995
Dec
;21
(12
):1054
–6
.
[PubMed]
0342-464264.
Carlucci
A
, Mattei
A
, Rossi
V
, Paracchini
E
, Raineri
SM
, Gregoretti
C
. Ventilator Settings to Avoid Nuisance Alarms During Mouthpiece Ventilation
. Respir Care
. 2016
Apr
;61
(4
):462
–7
.
[PubMed]
0020-132465.
JE
. A. Roundtable conference on poliomyelitis equipment: New. York City, May 28-29, 1953. In: White Plains NYNFfIP, March of Dimes; 1953, editor.1953
.66.
Bach
JR
. Amyotrophic lateral sclerosis: prolongation of life by noninvasive respiratory AIDS
. Chest
. 2002
Jul
;122
(1
):92
–8
.
[PubMed]
0012-369267.
Bach
JR
, Gonçalves
MR
, Hamdani
I
, Winck
JC
. Extubation of patients with neuromuscular weakness: a new management paradigm
. Chest
. 2010
May
;137
(5
):1033
–9
.
[PubMed]
0012-369268.
Bach
JR
. Continuous noninvasive ventilation for patients with neuromuscular disease and spinal cord injury
. Semin Respir Crit Care Med
. 2002
Jun
;23
(3
):283
–92
.
[PubMed]
1069-342469.
Janssens
JP
, Borel
JC
, Pépin
JL
, Somno
NI
; SomnoNIV Group
. Nocturnal monitoring of home non-invasive ventilation: the contribution of simple tools such as pulse oximetry, capnography, built-in ventilator software and autonomic markers of sleep fragmentation
. Thorax
. 2011
May
;66
(5
):438
–45
.
[PubMed]
0040-637670.
Ogna
A
, Nardi
J
, Prigent
H
, Quera Salva
MA
, Chaffaut
C
, Lamothe
L
, et al. Prognostic Value of Initial Assessment of Residual Hypoventilation Using Nocturnal Capnography in Mechanically Ventilated Neuromuscular Patients: A 5-Year Follow-up Study
. Front Med (Lausanne)
. 2016
Sep
;3
:40
.
[PubMed]
2296-858X71.
Gonzalez-Bermejo
J
, Morelot-Panzini
C
, Arnol
N
, Meininger
V
, Kraoua
S
, Salachas
F
, et al. Prognostic value of efficiently correcting nocturnal desaturations after one month of non-invasive ventilation in amyotrophic lateral sclerosis: a retrospective monocentre observational cohort study
. Amyotroph Lateral Scler Frontotemporal Degener
. 2013
Sep
;14
(5-6
):373
–9
.
[PubMed]
2167-842172.
Berry
RB
, Chediak
A
, Brown
LK
, Finder
J
, Gozal
D
, Iber
C
, et al.; NPPV Titration Task Force of the American Academy of Sleep Medicine
. Best clinical practices for the sleep center adjustment of noninvasive positive pressure ventilation (NPPV) in stable chronic alveolar hypoventilation syndromes
. J Clin Sleep Med
. 2010
Oct
;6
(5
):491
–509
.
[PubMed]
1550-938973.
Georges
M
, Adler
D
, Contal
O
, Espa
F
, Perrig
S
, Pépin
JL
, et al. Reliability of Apnea-Hypopnea Index Measured by a Home Bi-Level Pressure Support Ventilator Versus a Polysomnographic Assessment
. Respir Care
. 2015
Jul
;60
(7
):1051
–6
.
[PubMed]
0020-132474.
Fernandez Alvarez
R
, Rabec
C
, Rubinos Cuadrado
G
, Cascon Hernandez
JA
, Rodriguez
P
, Georges
M
, et al. Monitoring Noninvasive Ventilation in Patients with Obesity Hypoventilation Syndrome: Comparison between Ventilator Built-in Software and Respiratory Polygraphy
. Respiration
. 2017
;93
(3
):162
–9
.
[PubMed]
1423-035675.
Simonds
AK
. Recent advances in respiratory care for neuromuscular disease
. Chest
. 2006
Dec
;130
(6
):1879
–86
.
[PubMed]
0012-369276.
Ozsancak
A
, D’Ambrosio
C
, Hill
NS
. Nocturnal noninvasive ventilation
. Chest
. 2008
May
;133
(5
):1275
–86
.
[PubMed]
0012-369277.
Bach
JR
, Campagnolo
DI
, Hoeman
S
. Life satisfaction of individuals with Duchenne muscular dystrophy using long-term mechanical ventilatory support
. Am J Phys Med Rehabil
. 1991
Jun
;70
(3
):129
–35
.
[PubMed]
0894-911578.
Nauffal
D
, Doménech
R
, Martínez García
MA
, Compte
L
, Macián
V
, Perpiñá
M
. Noninvasive positive pressure home ventilation in restrictive disorders: outcome and impact on health-related quality of life
. Respir Med
. 2002
Oct
;96
(10
):777
–83
.
[PubMed]
0954-611179.
Pehrsson
K
, Olofson
J
, Larsson
S
, Sullivan
M
. Quality of life of patients treated by home mechanical ventilation due to restrictive ventilatory disorders
. Respir Med
. 1994
Jan
;88
(1
):21
–6
.
[PubMed]
0954-611180.
Ingadóttir
TS
, Jonsdottir
H
. Technological dependency—the experience of using home ventilators and long-term oxygen therapy: patients’ and families’ perspective
. Scand J Caring Sci
. 2006
Mar
;20
(1
):18
–25
.
[PubMed]
0283-931881.
Lindahl
B
, Sandman
PO
, Rasmussen
BH
. Meanings of living at home on a ventilator
. Nurs Inq
. 2003
Mar
;10
(1
):19
–27
.
[PubMed]
1320-788182.
Evans
R
, Catapano
MA
, Brooks
D
, Goldstein
RS
, Avendano
M
. Family caregiver perspectives on caring for ventilator-assisted individuals at home
. Can Respir J
. 2012
Nov-Dec
;19
(6
):373
–9
.
[PubMed]
1198-224183.
Gelinas
DF
, O’Connor
P
, Miller
RG
. Quality of life for ventilator-dependent ALS patients and their caregivers
. J Neurol Sci
. 1998
Oct
;160
Suppl 1
:S134
–6
.
[PubMed]
0022-510X84.
Ambrosino
N
, Carpenè
N
, Gherardi
M
. Chronic respiratory care for neuromuscular diseases in adults
. Eur Respir J
. 2009
Aug
;34
(2
):444
–51
.
[PubMed]
0903-193685.
Brooks
D
, King
A
, Tonack
M
, Simson
H
, Gould
M
, Goldstein
R
. User perspectives on issues that influence the quality of daily life of ventilator-assisted individuals with neuromuscular disorders
. Can Respir J
. 2004
Nov-Dec
;11
(8
):547
–54
.
[PubMed]
1198-224186.
Tsara
V
, Serasli
E
, Voutsas
V
, Lazarides
V
, Christaki
P
. Burden and coping strategies in families of patients under noninvasive home mechanical ventilation
. Respiration
. 2006
;73
(1
):61
–7
.
[PubMed]
0025-793187.
Vitacca
M
, Escarrabill
J
, Galavotti
G
, Vianello
A
, Prats
E
, Scala
R
, et al. Home mechanical ventilation patients: a retrospective survey to identify level of burden in real life
. Monaldi Arch Chest Dis
. 2007
Sep
;67
(3
):142
–7
.
[PubMed]
1122-064388.
Findeis
A
, Larson
JL
, Gallo
A
, Shekleton
M
. Caring for individuals using home ventilators: an appraisal by family caregivers
. Rehabil Nurs
. 1994
Jan-Feb
;19
(1
):6
–11
.
[PubMed]
0278-480789.
Dale
CM
, King
J
, Nonoyama
M
, Carbone
S
, McKim
D
, Road
J
, et al.; CANuVENT group
. Transitions to Home Mechanical Ventilation: The Experiences of Canadian Ventilator-Assisted Adults and Their Family Caregivers
. Ann Am Thorac Soc
. 2017
Dec
.
[PubMed]
2325-662190.
Laakso
K
, Markström
A
, Idvall
M
, Havstam
C
, Hartelius
L
. Communication experience of individuals treated with home mechanical ventilation
. Int J Lang Commun Disord
. 2011
Nov-Dec
;46
(6
):686
–99
.
[PubMed]
1368-282291.
Simonds
AK
. Risk management of the home ventilator dependent patient
. Thorax
. 2006
May
;61
(5
):369
–71
.
[PubMed]
0040-637692.
Srinivasan
S
, Doty
SM
, White
TR
, Segura
VH
, Jansen
MT
, Davidson Ward
SL
, et al. Frequency, causes, and outcome of home ventilator failure
. Chest
. 1998
Nov
;114
(5
):1363
–7
.
[PubMed]
0012-369293.
Chatwin
M
, Heather
S
, Hanak
A
, Polkey
MI
, Simonds
AK
. Analysis of home support and ventilator malfunction in 1,211 ventilator-dependent patients
. Eur Respir J
. 2010
Feb
;35
(2
):310
–6
.
[PubMed]
0903-193694.
Farre
R
, Lloyd-Owen
SJ
, Ambrosino
N
, Donaldson
G
, Escarrabill
J
, Fauroux
B
, et al. Quality control of equipment in home mechanical ventilation: a European survey
. Eur Respir J
. 2005
Jul
;26
(1
):86
–94
.
[PubMed]
0903-193695.
Bach
JR
, Intintola
P
, Alba
AS
, Holland
IE
. The ventilator-assisted individual. Cost analysis of institutionalization vs rehabilitation and in-home management
. Chest
. 1992
Jan
;101
(1
):26
–30
.
[PubMed]
0012-369296.
O’Donohue
WJ
Jr, Giovannoni
RM
, Goldberg
AI
, Keens
TG
, Make
BJ
, Plummer
AL
, et al. Long-term mechanical ventilation. Guidelines for management in the home and at alternate community sites. Report of the Ad Hoc Committee, Respiratory Care Section, American College of Chest Physicians
. Chest
. 1986
Jul
;90
(1
Suppl
):1S
–37S
.
[PubMed]
0012-369297.
Gladman
M
, Zinman
L
. The economic impact of amyotrophic lateral sclerosis: a systematic review
. Expert Rev Pharmacoecon Outcomes Res
. 2015
Jun
;15
(3
):439
–50
.
[PubMed]
1473-716798.
Nonoyama
ML
, McKim
DA
, Road
J
, Guerriere
D
, Coyte
PC
, Wasilewski
M
, et al. Healthcare utilisation and costs of home mechanical ventilation
. Thorax
. 2018
Jan
;73
(7
):thoraxjnl-2017-211138
.
[PubMed]
0040-637699.
Murphy
PB
, Douiri
A
. Patients, caregivers and health system costs of home ventilation
. Thorax
. 2018
Mar
;73
(7
):thoraxjnl-2018-211659
.
[PubMed]
0040-6376100.
Stuart
M
, Weinrich
M
. Integrated health system for chronic disease management: lessons learned from France
. Chest
. 2004
Feb
;125
(2
):695
–703
.
[PubMed]
0012-3692101.
Ambrosino
N
, Vitacca
M
, Dreher
M
, Isetta
V
, Montserrat
JM
, Tonia
T
, et al.; ERS Tele-Monitoring of Ventilator-Dependent Patients Task Force
. Tele-monitoring of ventilator-dependent patients: a European Respiratory Society Statement
. Eur Respir J
. 2016
Sep
;48
(3
):648
–63
.
[PubMed]
0903-1936102.
Chatwin
M
, Hawkins
G
, Panicchia
L
, Woods
A
, Hanak
A
, Lucas
R
, et al. Randomised crossover trial of telemonitoring in chronic respiratory patients (TeleCRAFT trial)
. Thorax
. 2016
Apr
;71
(4
):305
–11
.
[PubMed]
0040-6376103.
Pinto
A
, Almeida
JP
, Pinto
S
, Pereira
J
, Oliveira
AG
, de Carvalho
M
. Home telemonitoring of non-invasive ventilation decreases healthcare utilisation in a prospective controlled trial of patients with amyotrophic lateral sclerosis
. J Neurol Neurosurg Psychiatry
. 2010
Nov
;81
(11
):1238
–42
.
[PubMed]
0022-3050104.
Bertini
S
, Picariello
M
, Gorini
M
, Renda
T
, Augustynen
A
, Villella
G
, et al. Telemonitoring in chronic ventilatory failure: a new model of survellaince, a pilot study
. Monaldi Arch Chest Dis
. 2012
Jun
;77
(2
):57
–66
.
[PubMed]
1122-0643105.
Hazenberg
A
, Kerstjens
HA
, Prins
SC
, Vermeulen
KM
, Wijkstra
PJ
. Initiation of home mechanical ventilation at home: a randomised controlled trial of efficacy, feasibility and costs
. Respir Med
. 2014
Sep
;108
(9
):1387
–95
.
[PubMed]
0954-6111106.
Vitacca
M
, Bianchi
L
, Guerra
A
, Fracchia
C
, Spanevello
A
, Balbi
B
, et al. Tele-assistance in chronic respiratory failure patients: a randomised clinical trial
. Eur Respir J
. 2009
Feb
;33
(2
):411
–8
.
[PubMed]
0903-1936107.
Masefield
S
, Vitacca
M
, Dreher
M
, Kampelmacher
M
, Escarrabill
J
, Paneroni
M
, et al. Attitudes and preferences of home mechanical ventilation users from four European countries: an ERS/ELF survey
. ERJ Open Res
. 2017
Jun
;3
(2
):00015-2017
.
[PubMed]
2312-0541108.
Garuti
G
, Bagatti
S
, Verucchi
E
, Massobrio
M
, Spagnolatti
L
, Vezzani
G
, et al. Pulmonary rehabilitation at home guided by telemonitoring and access to healthcare facilities for respiratory complications in patients with neuromuscular disease
. Eur J Phys Rehabil Med
. 2013
Feb
;49
(1
):51
–7
.
[PubMed]
1973-9095109.
Vitacca
M
. Telemonitoring in patients with chronic respiratory insufficiency: expectations deluded?
Thorax
. 2016
Apr
;71
(4
):299
–301
.
[PubMed]
0040-6376110.
Bourke
SC
. Respiratory involvement in neuromuscular disease
. Clin Med (Lond)
. 2014
Feb
;14
(1
):72
–5
.
[PubMed]
1470-2118111.
Piepers
S
, van den Berg
JP
, Kalmijn
S
, van der Pol
WL
, Wokke
JH
, Lindeman
E
, et al. Effect of non-invasive ventilation on survival, quality of life, respiratory function and cognition: a review of the literature
. Amyotroph Lateral Scler
. 2006
Dec
;7
(4
):195
–200
.
[PubMed]
1748-2968112.
Aboussouan
LS
, Khan
SU
, Banerjee
M
, Arroliga
AC
, Mitsumoto
H
. Objective measures of the efficacy of noninvasive positive-pressure ventilation in amyotrophic lateral sclerosis
. Muscle Nerve
. 2001
Mar
;24
(3
):403
–9
.
[PubMed]
0148-639X113.
Birnkrant
DJ
, Bushby
K
, Bann
CM
, Apkon
SD
, Blackwell
A
, Colvin
MK
, et al.; DMD Care Considerations Working Group
. Diagnosis and management of Duchenne muscular dystrophy, part 3: primary care, emergency management, psychosocial care, and transitions of care across the lifespan
. Lancet Neurol
. 2018
May
;17
(5
):445
–55
.
[PubMed]
1474-4422114.
Leonardis
L
, Dolenc Grošelj
L
, Vidmar
G
. Factors related to respiration influencing survival and respiratory function in patients with amyotrophic lateral sclerosis: a retrospective study
. Eur J Neurol
. 2012
Dec
;19
(12
):1518
–24
.
[PubMed]
1351-5101115.
Aboussouan
LS
, Lattin
CD
, Kline
JL
. Determinants of long-term mortality after prolonged mechanical ventilation
. Hai
. 2008
Sep-Oct
;186
(5
):299
–306
.
[PubMed]
0436-5712116.
Hazenberg
A
, Kerstjens
HA
, Prins
SC
, Vermeulen
KM
, Wijkstra
PJ
. Is chronic ventilatory support really effective in patients with amyotrophic lateral sclerosis?
J Neurol
. 2016
Dec
;263
(12
):2456
–61
.
[PubMed]
0340-5354117.
Kohler
M
, Clarenbach
CF
, Böni
L
, Brack
T
, Russi
EW
, Bloch
KE
. Quality of life, physical disability, and respiratory impairment in Duchenne muscular dystrophy
. Am J Respir Crit Care Med
. 2005
Oct
;172
(8
):1032
–6
.
[PubMed]
1073-449X118.
Vandenberghe
N
, Vallet
AE
, Petitjean
T
, Le Cam
P
, Peysson
S
, Guérin
C
, et al. Absence of airway secretion accumulation predicts tolerance of noninvasive ventilation in subjects with amyotrophic lateral sclerosis
. Respir Care
. 2013
Sep
;58
(9
):1424
–32
.
[PubMed]
0020-1324119.
Peysson
S
, Vandenberghe
N
, Philit
F
, Vial
C
, Petitjean
T
, Bouhour
F
, et al. Factors predicting survival following noninvasive ventilation in amyotrophic lateral sclerosis
. Eur Neurol
. 2008
;59
(3-4
):164
–71
.
[PubMed]
0014-3022120.
Chiò
A
, Ilardi
A
, Cammarosano
S
, Moglia
C
, Montuschi
A
, Calvo
A
. Neurobehavioral dysfunction in ALS has a negative effect on outcome and use of PEG and NIV
. Neurology
. 2012
Apr
;78
(14
):1085
–9
.
[PubMed]
0028-3878121.
Gruis
KL
, Brown
DL
, Schoennemann
A
, Zebarah
VA
, Feldman
EL
. Predictors of noninvasive ventilation tolerance in patients with amyotrophic lateral sclerosis
. Muscle Nerve
. 2005
Dec
;32
(6
):808
–11
.
[PubMed]
0148-639X122.
Deo
P
, Bach
JR
. Noninvasive ventilatory support to reverse weight loss in Duchenne muscular dystrophy: A case series
. Pulmonology
; 2018
. 123.
Chiò
A
, Mora
G
, Leone
M
, Mazzini
L
, Cocito
D
, Giordana
MT
, et al.; Piemonte and Valle d’Aosta Register for ALS (PARALS)
. Early symptom progression rate is related to ALS outcome: a prospective population-based study
. Neurology
. 2002
Jul
;59
(1
):99
–103
.
[PubMed]
0028-3878124.
Lahrmann
H
, Wild
M
, Zdrahal
F
, Grisold
W
. Expiratory muscle weakness and assisted cough in ALS
. Amyotroph Lateral Scler Other Motor Neuron Disord
. 2003
Apr
;4
(1
):49
–51
.
[PubMed]
1466-0822125.
Gomez-Merino
E
, Bach
JR
. Duchenne muscular dystrophy: prolongation of life by noninvasive ventilation and mechanically assisted coughing
. Am J Phys Med Rehabil
. 2002
Jun
;81
(6
):411
–5
.
[PubMed]
0894-9115126.
Hill
NS
. Neuromuscular disease in respiratory and critical care medicine
. Respir Care
. 2006
Sep
;51
(9
):1065
–71
.
[PubMed]
0020-1324127.
Benditt
JO
. Full-time noninvasive ventilation: possible and desirable
. Respir Care
. 2006
Sep
;51
(9
):1005
–12
.
[PubMed]
0020-1324128.
Bach
JR
, Bakshiyev
R
, Hon
A
. Noninvasive respiratory management for patients with spinal cord injury and neuromuscular disease
. Tanaffos
. 2012
;11
(1
):7
–11
.
[PubMed]
1735-0344129.
McKim
DA
, Griller
N
, LeBlanc
C
, Woolnough
A
, King
J
. Twenty-four hour noninvasive ventilation in Duchenne muscular dystrophy: a safe alternative to tracheostomy
. Can Respir J
. 2013
Jan-Feb
;20
(1
):e5
–9
.
[PubMed]
1198-2241130.
Soudon
P
, Steens
M
, Toussaint
M
. A comparison of invasive versus noninvasive full-time mechanical ventilation in Duchenne muscular dystrophy
. Chron Respir Dis
. 2008
;5
(2
):87
–93
.
[PubMed]
1479-9723131.
Pinto
T
, Chatwin
M
, Banfi
P
, Winck
JC
, Nicolini
A
. Mouthpiece ventilation and complementary techniques in patients with neuromuscular disease: A brief clinical review and update
. Chron Respir Dis
. 2017
May
;14
(2
):187
–93
.
[PubMed]
1479-9723132.
Bach
JR
, Bianchi
C
, Aufiero
E
. Oximetry and indications for tracheotomy for amyotrophic lateral sclerosis
. Chest
. 2004
Nov
;126
(5
):1502
–7
.
[PubMed]
0012-3692133.
Boussaïd
G
, Lofaso
F
, Santos
DB
, Vaugier
I
, Pottier
S
, Prigent
H
, et al. Impact of invasive ventilation on survival when non-invasive ventilation is ineffective in patients with Duchenne muscular dystrophy: A prospective cohort
. Respir Med
. 2016
Jun
;115
:26
–32
.
[PubMed]
0954-6111134.
Bach
JR
, Gonçalves
MR
, Hon
A
, Ishikawa
Y
, De Vito
EL
, Prado
F
, et al. Changing trends in the management of end-stage neuromuscular respiratory muscle failure: recommendations of an international consensus
. Am J Phys Med Rehabil
. 2013
Mar
;92
(3
):267
–77
.
[PubMed]
0894-9115135.
Markström
A
, Sundell
K
, Lysdahl
M
, Andersson
G
, Schedin
U
, Klang
B
. Quality-of-life evaluation of patients with neuromuscular and skeletal diseases treated with noninvasive and invasive home mechanical ventilation
. Chest
. 2002
Nov
;122
(5
):1695
–700
.
[PubMed]
0012-3692136.
Narayanaswami
P
, Bertorini
TE
, Pourmand
R
, Horner
LH
. Long-term tracheostomy ventilation in neuromuscular diseases: patient acceptance and quality of life
. Neurorehabil Neural Repair
. 2000
;14
(2
):135
–9
.
[PubMed]
1545-9683137.
Ceriana
P
, Surbone
S
, Segagni
D
, Schreiber
A
, Carlucci
A
. Decision-making for tracheostomy in amyotrophic lateral sclerosis (ALS): a retrospective study
. Amyotroph Lateral Scler Frontotemporal Degener
. 2017
Nov
;18
(7-8
):492
–7
.
[PubMed]
2167-8421138.
Veronese
S
, Valle
A
, Chiò
A
, Calvo
A
, Oliver
D
. The last months of life of people with amyotrophic lateral sclerosis in mechanical invasive ventilation: a qualitative study
. Amyotroph Lateral Scler Frontotemporal Degener
. 2014
Dec
;15
(7-8
):499
–504
.
[PubMed]
2167-8421139.
Hirose
T
, Kimura
F
, Tani
H
, Ota
S
, Tsukahara
A
, Sano
E
, et al. Clinical characteristics of long-term survival with noninvasive ventilation and factors affecting the transition to invasive ventilation in amyotrophic lateral sclerosis
. Muscle Nerve
. 2018
Apr
.
[PubMed]
0148-639X140.
Cheng
HW
, Chan
KY
, Chung
YK
, Choi
CW
, Chan
CH
, Cheng
SC
, et al. Supportive & palliative interventions in motor neurone disease: what we know from current literature?
Ann Palliat Med
. 2018
Jul
;7
(3
):320
–31
.
[PubMed]
2224-5820141.
Dangers
L
, Laviolette
L
, Georges
M
, Gonzalez-Bermejo
J
, Rivals
I
, Similowski
T
, et al. Relieving dyspnoea by non-invasive ventilation decreases pain thresholds in amyotrophic lateral sclerosis
. Thorax
. 2017
Mar
;72
(3
):230
–5
.
[PubMed]
0040-6376142.
Servera
E
, Sancho
J
, Zafra
MJ
, Catalá
A
, Vergara
P
, Marín
J
. Alternatives to endotracheal intubation for patients with neuromuscular diseases
. Am J Phys Med Rehabil
. 2005
Nov
;84
(11
):851
–7
.
[PubMed]
0894-9115143.
Bédard
ME
, McKim
DA
. Daytime Mouthpiece for Continuous Noninvasive Ventilation in Individuals With Amyotrophic Lateral Sclerosis
. Respir Care
. 2016
Oct
;61
(10
):1341
–8
.
[PubMed]
0020-1324144.
de Visser
M
, Oliver
DJ
. Palliative care in neuromuscular diseases
. Curr Opin Neurol
. 2017
Dec
;30
(6
):686
–91
.
[PubMed]
1350-7540145.
Arias
R
, Andrews
J
, Pandya
S
, Pettit
K
, Trout
C
, Apkon
S
, et al. Palliative care services in families of males with Duchenne muscular dystrophy
. Muscle Nerve
. 2011
Jul
;44
(1
):93
–101
.
[PubMed]
0148-639X146.
Abbott
D
, Prescott
H
, Forbes
K
, Fraser
J
, Majumdar
A
. Men with Duchenne muscular dystrophy and end of life planning
. Neuromuscul Disord
. 2017
Jan
;27
(1
):38
–44
.
[PubMed]
0960-8966147.
Kettemann
D
, Funke
A
, Maier
A
, Rosseau
S
, Meyer
R
, Spittel
S
, et al. Clinical characteristics and course of dying in patients with amyotrophic lateral sclerosis withdrawing from long-term ventilation
. Amyotroph Lateral Scler Frontotemporal Degener
. 2017
Feb
;18
(1-2
):53
–9
.
[PubMed]
2167-8421148.
Hardiman
O
. Management of respiratory symptoms in ALS
. J Neurol
. 2011
Mar
;258
(3
):359
–65
.
[PubMed]
0340-5354© 2019 S. Karger AG, Basel
2019
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