Vni Nas Distrofias Musculares

15/04/23
VNI NAS DISTROFIAS MUSCULARES

QUANDO E COMO INICIAR?
Filipa Soares Pires

Serviço de Pneumologia
Centro Hospitalar de São João
NEUROMUSCULAR
DISEASES
neuropathic
disease
myopathies
disease of neuro-
muscular junction
1
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NEUROMUSCULAR DISEASES
Acquired
Inherited
Ambrosino N. Eur Respir J 2009
MUSCULAR DYSTROPHIES
•  Group of inherited disorders caused by mutaNons in a number of
genes.
•  These geneNc mutaNons cause either a dysfuncNon in, or lack of,
proteins that are essenNal for muscle cell stability, leading to
progressive destrucNon and weakness in the muscles.
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Most prevalent in

childhood
Most prevalent in

adults
Simonds AK. Semin Respir Crit Care Med 2002
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HETEROGENEITY
•  Inheritance paUern
•  Age of clinical manifestaNons
•  DistribuNon of muscle weakness
Duchenne and Becker muscular dystrophy
HETEROGENEITY
•  Inheritance paUern
•  Age of clinical manifestaNons
•  DistribuNon of muscle weakness
FSH dystrophy
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RESPIRATORY COMPROMISE
•  Respiratory muscle weakness

•  Respiratory infecNons
•  Chest-‐wall deformiNes
•  Presence of cardiomyopathy
NIV IN MUSCULAR DYSTROPHIES

Duchenne Muscular Dystrophy
WHY? WHEN? HOW?
5
15/04/23

WHY?

WHY?
Phillips MF. Am J Respir Crit Care Med 2001
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WHY?
Phillips MF. Am J Respir Crit Care Med 2001

WHY?
•  VenNlatory failure as a major cause of death.
•  Inspiratory and expiratory muscle weakness

•  ContribuNon of chest wall restricNon (scoliosis)
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WHY?
•  VenNlatory failure as a major cause of death.
•  Inspiratory and expiratory muscle weakness

•  ContribuNon of chest wall restricNon (scoliosis)

WHY?
EVOLUTION OF
RESPIRATORY FAILURE Normal breathing
IN NEUROMUSCULAR
DISEASES FVC < 60% pred
REM related sleep disordered breathing
FVC < 40% pred

NREM and REM sleep disordered breathing
FVC < 20% pred

DayNme venNlatory failure
A. Simonds
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WHY?
•  Mean survival of 9.7 months from the onset of dayNme
hypercapnia.
•  VC<680 mL (14%) – best predictor of diurnal
hypercapnia in DMD
3000
2000
VC
1000 SENS: 90%

680 SPEC: 95%
Diurnal
0 Normocapnia
Diurnal hypercapnia
M. Toussaint. Chest 2007

WHY? •  Survival
Survival in DMD by tracheostomy and NIV vs no venNlaNon:
227 paNents with DMD were referred from 1964-‐2010.
Ishikawa Y. Neuromuscul Disord 2010
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WHY? •  Survival
Simonds A. Thorax 1998

WHY?
•  Quality of life
Kohler M. Am J Respir Crit Care Med, 2005
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WHY?
•  Quality of life
Simonds A. Thorax, 1998

WHY?
•  Slower rate of decline in lung funcNon
Barbé F. Chest, 1996
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WHEN?
•  PosiNve impact and effects of NIV have been

documented clearly.
•  Controversies about the Nming of iniNaNon.

WHEN? •  PaNents free of dayNme
respiratory failure and FVC
between 20 and 50%. PrevenNve use of NIV?
Control
Control
Premature introducNon can
be counterproducNve.
NIV
NIV
Raphael JC. Lancet 1994
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15/04/23

WHEN?
•  PaNents with neuromuscular disease with nocturnal hypovenNlaNon are
likely to deteriorate with the development of dayNme hypercapnia and/
or progressive symptoms within 2 years and may benefit from the
introducNon of nocturnal NIV before dayNme hypercapnia ensues.
Ward S. Thorax 2005

WHEN?
Ward S. Thorax 2005
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WHEN?

Dyspnea
FaNgue
Morning headaches
WHEN? Frequent nocturnal awakenings
Hypersomnolence
Difficulty with concentraNon
Frequent nightmares
Restless sleep
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WHEN?
(M. Toussaint)

WHEN?
Progression to dayNme venNlaNon
•  95% of DMD paNents experienced diurnal dyspnea

before hypercapnia occurred
Associated with a drop in vital capacity.
•  Dyspnea as a marker of the load on respiratory muscles.

Toussaint M. Thorax 2008
15
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WHEN?
Progression to dayNme venNlaNon
•  When diurnal dyspnea is present, 2 hours diurnal MPV

unloads respiratory muscles.
•  Diurnal Borg dyspnea score >3 (+ symptoms + drop in

VC) may be sufficient to implement diurnal NIV.
Toussaint M. Thorax 2008

WHEN?
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HOW?
Lofaso F. Resp Care 2014

HOW?
17
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HOW?

HOW?
•  The choice of the MV mode depends on the daily
duraNon of mechanical venNlaNon.
18
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HOW?
Nocturnal NIV Preferred mode
Pressure control mode
•  More comfortable during non leak condiNons

•  Leak compensaNon

HOW?
Nocturnal and dayNme NIV Preferred mode
Volume control mode
•  BeUer performance for mouthpiece venNlaNon

•  Allows paNent to disconnect from the venNlator (expiraNon,
speech…)
•  Able to generate the high pressures necessary for effecNve
airstacking or lung-‐volume recruitment.
•  Assisted cough
19
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HOW?
•  Interface

HOW?
Nocturnal NIV
•  Non-‐invasive venNlaNon with pressure cycled bilevel devices or volume

cycled venNlators.
•  In bilevel or pressure support modes of venNlaNon, add a backup rate.
•  Recommended interfaces include: a nasal mask or a nasal pillow. Others
can be used.
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HOW?
DayNme NIV
•  Non-‐invasive venNlaNon with portable volume cycled or volume-‐pressure

venNlators. Bilevel devices are an alternaNve.
•  A mouthpiece interface is strongly recommended during day use of
volumetric venNlaNon.
•  Other venNlator-‐interface combinaNons can be used based on clinician
preference and paNent Comfort.

HOW?
High venNlatory dependence
VS.
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15/04/23

HOW?
•  Is tracheostomy needed for conNnuous venNlatory
support?
1. Effec6veness
NIV has never been ineffec0ve for con0nuous ven0latory support in competent hands unless:
-‐  the ven0lator se;ngs or interfaces are inadequate
-‐  pa0ents are too mentaly impaired to cooperate with it
-‐  bulbar innervated musculature is too impaired to protect the airway.
Cough aids fundamental!!
Bach JR. Eur Respir J 2007

HOW?
support?
2. Complica6ons
There were significantly fewer hospitalisa0ons and pulmonary complica0ons in the NIV group as
compared with the tracheostomy group.
Bach JR. Eur Respir J 2007
22
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HOW?
support?
3. Pa6ent preference
Bach JR. Chest 1993
23

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15/04/23

VNI NAS DISTROFIAS MUSCULARES

Filipa Soares Pires

Ambrosino N. Eur Respir J 2009

Most prevalent in

Most prevalent in

Simonds AK. Semin Respir Crit Care Med 2002

• Age of clinical manifestaNons

• DistribuNon of muscle weakness

Duchenne and Becker muscular dystrophy

• Age of clinical manifestaNons

• DistribuNon of muscle weakness

• Respiratory muscle weakness

NIV IN MUSCULAR DYSTROPHIES

WHY? WHEN? HOW?

NIV IN MUSCULAR DYSTROPHIES

NIV IN MUSCULAR DYSTROPHIES

Phillips MF. Am J Respir Crit Care Med 2001

NIV IN MUSCULAR DYSTROPHIES

Phillips MF. Am J Respir Crit Care Med 2001

NIV IN MUSCULAR DYSTROPHIES

• VenNlatory failure as a major cause of death.

• Inspiratory and expiratory muscle weakness

NIV IN MUSCULAR DYSTROPHIES

• VenNlatory failure as a major cause of death.

• Inspiratory and expiratory muscle weakness

NIV IN MUSCULAR DYSTROPHIES

FVC < 40% pred

FVC < 20% pred

NIV IN MUSCULAR DYSTROPHIES

1000 SENS: 90%

NIV IN MUSCULAR DYSTROPHIES

Ishikawa Y. Neuromuscul Disord 2010

NIV IN MUSCULAR DYSTROPHIES

Simonds A. Thorax 1998

NIV IN MUSCULAR DYSTROPHIES

Kohler M. Am J Respir Crit Care Med, 2005

NIV IN MUSCULAR DYSTROPHIES

Simonds A. Thorax, 1998

NIV IN MUSCULAR DYSTROPHIES

• Slower rate of decline in lung funcNon

Barbé F. Chest, 1996

NIV IN MUSCULAR DYSTROPHIES

• PosiNve impact and eﬀects of NIV have been

NIV IN MUSCULAR DYSTROPHIES

Raphael JC. Lancet 1994

NIV IN MUSCULAR DYSTROPHIES

Ward S. Thorax 2005

NIV IN MUSCULAR DYSTROPHIES

Ward S. Thorax 2005

NIV IN MUSCULAR DYSTROPHIES

NIV IN MUSCULAR DYSTROPHIES

NIV IN MUSCULAR DYSTROPHIES

NIV IN MUSCULAR DYSTROPHIES

• 95% of DMD paNents experienced diurnal dyspnea

Associated with a drop in vital capacity.

• Dyspnea as a marker of the load on respiratory muscles.

•  Age of clinical manifestaNons

•  DistribuNon of muscle weakness

•  Age of clinical manifestaNons

•  DistribuNon of muscle weakness

•  Respiratory muscle weakness

•  VenNlatory failure as a major cause of death.

•  Inspiratory and expiratory muscle weakness

•  VenNlatory failure as a major cause of death.

•  Inspiratory and expiratory muscle weakness

•  Slower rate of decline in lung funcNon

•  PosiNve impact and eﬀects of NIV have been

•  95% of DMD paNents experienced diurnal dyspnea

•  Dyspnea as a marker of the load on respiratory muscles.

•  When diurnal dyspnea is present, 2 hours diurnal MPV

•  Diurnal Borg dyspnea score >3 (+ symptoms + drop in

•  More comfortable during non leak condiNons

•  BeUer performance for mouthpiece venNlaNon

•  Non-‐invasive venNlaNon with pressure cycled bilevel devices or volume

•  Non-‐invasive venNlaNon with portable volume cycled or volume-‐pressure