rationale and benefits of niv in lung...
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Rationale and benefits of NIV in
lung diseasesBrigitte Fauroux
Pediatric noninvasive ventilation and sleep unitResearch unit INSERM U 955
Necker university Hospital, Paris, France
InsermInstitut nationalde la santé et de la recherche médicale
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Rationale and benefits of NIVin lung diseases
• Introduction• Cystic fibrosis• Bronchiolitis obliterans• Bronchopulmonary dysplasia• Interstitial lung disease
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Two types of chronic respiratory failure
Abnormalities of the respiratory mechanics
� PaCO2 � PaO2
� Respiratoryload
Respiratory control
� Respiratory muscle capacity
NM diseases
Lung and airwaydiseases
Abnormalities of thealveolar-capillary barrier
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Chronic lung diseases in children
• Obstructive lung diseases– Cystic fibrosis– BO– BPD
• Interstitial lung disease
Alveolar hypoventilation=
Hypercapnia & hypoxemia
Hypoxemia
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Two types of respiratory treatments
Abnormalities of the respiratory mechanics
Abnormalities of thealveolar-capillary barrier
� Respiratoryload
Respiratory control
� Respiratory muscle capacity
Mechanicalventilation
O2
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Respiratory diseasesexaggerate during sleep
Sleep
Ventilatory drive
Respiratory muscles
Respiratory mechanics
� Central drive� Chemoreceptor
sensitivity
Preservation of the activity of the diaphragm� Intercostal and upper airway muscles
Ventilation/perfusion mismatch� Airflow resistance� Functional residualcapacity
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• If you can not measure it, you can not improve it.
William Thomson (1824 - 1907) or « Lord Kelvin »
physician, founder of the thermodynamics
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Measurement of the work of breathing
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Rationale and benefits of NIVin lung diseases
• Introduction• Cystic fibrosis• Bronchiolitis obliterans• Bronchopulmonary dysplasia• Interstitial lung disease
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100
150
200
250
300
350
400
450
500
550
600
650
PT
Pes
/min
(cm
H2O
.s.m
in-1
)
10 15 20 25 30 35 40 45 50
r = 0.-55; p =0.001
Hart et al. AJRCCM 2002;166:61
Respiratory mechanics incystic fibrosis
5
10
15
20
25
30
35
40
45
RR
(b
pm)
10 15 20 25 30 35 40 45 50
r = 0.38; p =0.03
.20
.25
.30
.35
.40
.45
.50
.55
.60
.65
.70
VT
(L
)
10 15 20 25 30 35 40 45 50
r = 0.37; p = 0.04
5
6
7
8
9
10
11
12
PaO
2 (K
Pa)
10 15 20 25 30 35 40 45 50
r = 0.76; p < 0.0001
4.5
5
5.5
6
6.5
7
7.5
cPaC
O2 (
Kp
a)
10 15 20 25 30 35 40 45 50
% Pred FEV1
r = 0.70; p < 0.0001
VEMS %
VEMS %
PTPes
RR
VT
PaO2
PaCO2
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Noninvasive ventilation unloads the respiratory muscles in CF
86 88 90 92 94 96 seconds
-25 -20 -15 -10 -5.00.05.010
cmH
2O
Poe
so
-25 -20 -15 -10 -5.00.05.010
cmH
2O
Pga
s
-1.0
-0.5
0.0
0.51.0
l/s
Déb
it pa
t
-5.0
0.0
5.0
10
cmH
2O
P p
at
72 74 76 78 80 82 seconds
-40 -35 -30 -25 -20 -15 -10 -5.0
cmH
2O
Poe
so
-30
-20
-10 0.0
cmH
2O
Pga
s
-1.0
-0.5
0.0
0.5
1.0
l/s
Déb
it pa
t
0.0
5.0
10
15
cmH
2O
P p
at
Spontaneous breathing Pressure support (14 cmH2O)
Pes
Pgas
Flow
Paw
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NIV decreasesthe work of breathing in CF
Fauroux et al. Crit Care Med 2001;29:2097
8 children with CF, mean age 13 years, mean FEV1 25%
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NIV improves gas exchange in CF
Fauroux et al. Crit Care Med 2001;29:2097
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NIV improves (daytime and) nocturnal gas exchange
Milross et al. AJRCCM 2001;163:129
10 adult CF patients3 nights: air, O2, NIV
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NIV prevents oxygendesaturation and
respiratory muscle fatigue during chest physiotherapy
Fauroux et al. Pediatrics 1999:103:e32
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NIV is associated with a decrease in dyspnea in CF
Fauroux et al. Crit Care Med 2001;29:2097
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The level of inspiratorypressure should be sufficient
Fauroux et al. Eur Respir J 2004;24:624� Mean level 16 cmH2O
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Expiratory pressure should be low
Hart et al. AJRCCM 2002;166:61
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13 children, FEV1 35-79%
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Benefits of NIV in cystic fibrosis
Proven benefits• Improvement in gas
exchange• Improvement in the
tolerance of chest physiotherapy
• Decrease in dyspnea• Improvement in exercise
tolerance• Stabilisation of the
decline in lung function
Expected benefits• Increase in survival• Increase in sleep quality• Increase in quality of life• Increase in weigth• Decrease/correction of
PHT• Improved outcome of
lung transplantation
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Rationale and benefits of NIVin lung diseases
• Introduction• Cystic fibrosis• Bronchiolitis obliterans• Bronchopulmonary dysplasia• Interstitial lung disease
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Spontaneous breathing 12/4 cmH2O 13/5 cmH2O
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Rationale and benefits of NIVin lung diseases
• Introduction• Cystic fibrosis• Bronchiolitis obliterans• Bronchopulmonary dysplasia• Interstitial lung disease
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Poe
s (c
mH
2O)
-60
-40
-20
0
Pga
s (c
mH
2O)
-2
0
2
4
6
8
10
12
14
16
Paw
(cm
H2O
)
0
2
4
6
8
10
12
Time (sec)
0 1 2 3 4 5
Pdi
(cm
H2O
)
-10
0
10
20
30
40
-60
-40
-20
0
-2
0
2
4
6
8
10
12
14
16
0
2
4
6
8
10
12
Time (sec)
0 1 2 3 4 5-10
0
10
20
30
40
-60
-40
-20
0
-2
0
2
4
6
8
10
12
14
16
0
2
4
6
8
10
12
-10
0
10
20
30
40
0 1 2 3 4 5
-60
-40
-20
-10
Time (sec)
Spontaneous breathing Clinical CPAP Physiological CPAP
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SB CPAP clin CPAP Physio CPAP Physio + 1
Sw
ing
Exp
irato
ry P
gas
(cm
H2O
)
0
2
4
6
8
10
12
14
16
UAOBPD
Expiratory abdominal activity
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SB CPAP clin CPAP PhysioCPAP Physio + 1
Sw
ing
Poe
s (c
mH
2O)
0
10
20
30
40
50
60
70
UAOBPD
SB CPAP clin CPAP Physio CPAP Physio + 1S
win
g P
di (
cmH
2O)
0
10
20
30
40
50
UAOBPD
Decrease in the respiratory effortwith CPAP
Khirani et al. Crit Care 2013;17:R167
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Rationale and benefits of NIVin lung diseases
• Introduction• Cystic fibrosis• Bronchiolitis obliterans• Bronchopulmonary dysplasia• Interstitial lung disease
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No benefit of NIV in ILD13 year old girl with « end-stage » ILD
PoesocmH2O
PgascmH2O
AirflowL/sec
PawcmH2O
PdicmH2O
240.00000 245.00000seconds
-30.0
-15.0
0.0
15.0
cmH
2O
Poe
so
-20.0
-15.0
-10.0
-5.0cm
H2O
Pga
s
-2.0
-1.0
0.0
1.0
2.0
l/s
Déb
it pa
t
-20.0-10.0
0.0
10.020.0
cmH
2O
P p
at
-30.0
-15.0
0.0
15.0
30.0
cmH
2O
Pdi
135.00000 140.00000
-30.0
-15.0
0.0
15.0
cmH
2O
-35.0
-30.0
-25.0
-20.0
cmH
2O
-1.0
-0.5
0.0
0.5
1.0
l/s
0.0
5.0
10.0
15.0
cmH
2O
-30.0
-15.0
0.0
15.0
cmH
2O
140.0seconds
Spontaneousbreathing Nasal CPAP 6 cmH2O Nasal bilevel PAP 12/6cm H2O
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OSAS in common inadults with interstitial lung
disease
Troy et al. World J Clin Cases 2014;2:828
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OSAS is common in ILD
Lancaster et al. Chest 2009;136:772
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AHI predicts survival in ILD
Kolilekas et al. J Clin Sleep Med 2013;9:593
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Marie, 10 years, ILD
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13 year old girl witha mutation of NKX2-1
Polysomnography
Normal sleep quality andstructureNo apneas9 hypopneas/h70% are obstructive
Mean SpO2 92%Minimal SpO2 86%Desaturation index 10/h11% of time with SpO2<90%
Mean PtcCO2 35 mmHgMaximal PtcCO2 40 mmHg
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Conclusion
• Physiological studies show that there is a rationale for NIV in pediatric lung diseases– CF, BO, selected BPD, ILD ?
• Necessity of more systematic – sleep studies– physiological measurements
• Future studies should aim at defining:– patients who could benefit most from NIV– initiation criteria for NIV– clinical benefits from NIV