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Inserm. Institut national de la santé et de la recherche médicale. Noninvasive Positive Pressure Ventilation ERS School Courses Hannover - Germany - 2007. Acute and chronic NPPV in children. Brigitte Fauroux Pediatric Pulmonology & Research unit INSERM UMR S-719 Armand Trousseau Hospital
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Inserm Institut national de la santé et de la recherche médicale Noninvasive Positive Pressure Ventilation ERS School Courses Hannover - Germany - 2007 Acute and chronic NPPV in children Brigitte Fauroux Pediatric Pulmonology & Research unit INSERM UMR S-719 Armand Trousseau Hospital Paris - France
Acute and chronic NPPV in children • Rationale for NPPV • Acute applications of NPPV • Indications and outcome • Technical requisites • Chronic applications of NPPV • Indications and long term benefits • Technical requisites
General requisites for NPPV • Medical requisites • respiratory failure can be improved, reversed, or cured by NPPV • hypercapnic respiratory failure (acute + chronic) • hypoxemic respiratory failure (acute) • patient has a minimal respiratory autonomy • NPPV is possible • no obstacle in the nose (closure of the mouth) • minimum level of co-operation • no excessive secretions • Technical requisites • adequate interface (nasal > face mask) • ventilator, settings and alarms adapted for children
Acute and chronic NPPV in children • Rationale for NPPV • Acute applications of NPPV • Indications and outcome • Technical requisites • Chronic applications of NPPV • Indications and long term benefit • Technical requisites
Acute applications of NPPV Goals :avoid intubation, reverse ARF reduce mortality, morbidity
National survey on NPPV in the PICU in France 2006 • 27/31 do use NPPV: duration of experience < 2 years n=6 • 2 - 6 years n=13 • 6 - 10 years n=6 • > 10 years n=2
NPPV in children with acute asthma 20 children with acute asthma 2 hours of NPPV (bilevel 10/5) NPPV was associated with respiratory rate clinical asthma score No change in PO2 and PCO2 Thill et al. Pediatr Crit Care Med 2004;5:337
NPPV in bronchiolitis • Not new ! : Beasley and Jones BMJ 1981;283:1506 • Successfull experience (case series) • before the ICU: Pirret et al. Intensive Crit Care Nurs 2005;21:314 • in the PICU: Martinon-Torres et al. Respir Med 2006;100:1458 • 15 infants received NPPV with heliox • H1: in clinical score, respiratory rate and PaCO2 • duration of NPPV: 2 – 14 days • one patient required intubation
NPPV unloads the respiratory muscles in cystic fibrosis PTPdi (cm H2O.s.min-1) 10 patients mean age 13 years mean FEV1 25% p<0.05 Fauroux et al. Crit Care Med 2001:29:2097
NPPV improves alveolar hypoventilation in cystic fibrosis *p<0.05 Fauroux et al. Crit Care Med 2001; 29:2097
NPPV unloads the respiratory muscles in infants with upper airway obstruction Noninvasive CPAP 10 cm H2O Spontaneous breathing Pes (cm H2O) Pgas (cm H2O) Pdi (cm H2O)
NPPV unloads the respiratory muscles in infants with upper airway obstruction 10 infants with severe upper airway obstructionMean age 11.5 ± 5.4 months, weight 7.2 ± 0.4 kg Essouri et al. Intensive Care Med 2006;7:329 * p< 0.005
NPPV in ARF in children with neuromuscular disorders Avoid intubation Reverse ARF Indication to start long term NPPV NPPV 3-year old boy, congenital myopathy Volume targeted NPPV (VT: 220 ml) Spontaneous breathing (VT: 100 ml)
NPPV in children with SMA type I • 11 SMA type I infants • Age at first intubation or episode requiring ventilatory support : 5 – 28 months • 48 intubations for 28 episodes of acute respiratory failure : • 20 Non-protocol extubations: success 2 • 28 Protocol extubations: success 23 Bach et al. Chest 2000;117:1100
NPPV in an acute situationHypoxemic respiratory failure 28 patients, mean age 8 years (4 to 204 months) pneumonia: n=22 Fortenberry et al. Chest 1995;108:1059
NPPV during hypoxemic respiratory failure (4 children with acute leukemia) Piastra et al. Intensive Care Med 2004;30:472
NPPV in the pediatric intensive care 9 5-yr experience in the PICU 114 children age 15 days to 17 yrs Intubation rate (%) 61 114 23 12 9 Essouri et al. Ped Crit Care Med 2006;7:329
Predictive factors of NPPV success in the pediatric intensive care *p<0.05 Essouri et al. Ped Crit Care Med 2006;7:329
Predictive factors of NPPV success in the pediatric intensive care *p=0.002 *p=0.099 Bernet et al. Ped Crit Care Med 2005;6:660
NPPV in an acute situationCardiogenic pulmonary edema 6 years old boy, on heart transplant list Spontaneous breathing PS 10 cm H2O
NPPV in an acute situationCardiogenic pulmonary edema 6 years old boy, on heart transplant list
Acute and chronic NPPV in children • Rationale for NPPV • Acute applications of NPPV • Indications and outcome • Technical requisites • Chronic applications of NPPV • Indications and long term benefit • Technical requisites
Technical requisites for acute NPPV Ventilator: ICU ventilator Interface: adapted for children
Acute and chronic NPPV in children • Rationale for NPPV • Acute applications of NPPV • Indications and outcome • Technical requisites • Chronic applications of NPPV • Indicationsand long term benefit • Technical requisites
Chronic applications of NPPV Physiopathology of respiratory failure Respiratory muscles capacity Neuromuscular disorders Ventilatory drive Ondine’s course Respiratory load Cystic fibrosis COPD Upper airway obstruction Alveolar hypoventilation PaO2 and PaCO2
Mechanical ventilation unloads the respiratory muscles Respiratory muscles Respiratory load Mechanical ventilation
34% 8% 9% 30% 17% 2% Long term home NPPV in children (France - 2000) Number of patients Fauroux et al, Pediatr Pulmonol 2003;35:119
Long term NPPV use is associated with an increase in survival in DMD patients Total Duchenne muscular dystrophy population in Denmark 1977-2001 Mortality 4.7 2.6 / 100 years NPPV users 0.9 43.4 per 100 105 Jeppesen et al. Neuromuscular Disorders 2003;13:804
NPPV is administered preferentially during sleep Sleep Ventilatory drive Respiratory mechanics Respiratory muscles • central drive • chemoreceptor sensitivity V/Q mismatch airflow resistance FRC preservation of the diaphragm upper airway muscles
NPPV is associated with an improvement in nocturnal gas exchange in children with neuromuscular diseases (n=40) SaO2 (%) Transcutaneous PCO2 (mm Hg) p=0.03 p=0.03 Simonds et al. Eur Resp J 2000;16:476
Long term NPPV use is associated with an improvement in gas exchange in children with NM disorders 30 children Age 12.3 ± 4.1 yrs NPPV for 25 ± 13 months % of total sleep time mm Hg Mellies et al, Eur Respir J 2003;22:631
NPPV improves nocturnal hypoventilation in CF patients Milross et al. AJRCCM 2001;163:129
NPPV improves nocturnal hypoventilation in CF patients Change in PtcPCO2 (mmHg) from NREM to REM sleep % of time with SaO2 > 90% * ** * ** *p<0.05 **p<0.01 Milross et al. AJRCCM 2001;163:129
Improvement of nocturnal gas exchange with NPPV in infants with OSA 12 infants with severe upper airway obstruction Fauroux et al. AJRCCM 2001:164:1874
Effectiveness of CPAP therapy in children with OSA 29 children with OSA age 2 to 16 years CPAP: n=13 Bilevel PAP: n=16 p=0.003 Marcus et al. Pediatrics 2006;117:e442
Adherence to CPAP therapy in children with OSA 29 children with OSA Randomly assigned to CPAP or Bilevel PAP 1/3 dropped out < 6 months Mean nightly use was 5.3 ± 2.5 hours Parents overestimated CPAP use Marcus et al. Pediatrics 2006;117:e442
Long term NPPV use in children with NM disorders • Effects on lung function • IVC in Duchenne patients (-183 ml) • IVC remained stable in other NM diseases • MIP • in all patients (40% to 44% predicted, NS) • Quality of life • in the 13 patients who were evaluated • Lung growth and nutrition ? Mellies et al, Eur Respir J 2003;22:631
Efficient respiratory movements are necessary for normal lung and chest wall growth Abnormal development of the diaphragm and the intercostal muscles leads to lung hypoplasia in mice Ackerman et al. PLoS Genetics 2005;1:e10 Inanglou et al. Int J Dev Biol 2003;47:363 Inanlou et al. Dev Dyn 2005;323:43
Long term NPPV use (1 months) in patients with cystic fibrosis • 4 adult CF patients • FEV1 11 – 14% Piper et al. Chest 1992;102:846
Long term NPPV use in patients with severe OSA • Correction of sleep disordered breathing • Improvement in quality of life (avoids tracheostomy) • Allows progressive nutritional autonomy Fauroux et al. AJRCCM 2001; 164:1874 Essouri et al. Intensive Care Med 2005
Criteria to initiate NPPV in the chronic setting • Diurnal hypercapnia (NM patients) • After an acute respiratory failure (NM patients) • Nocturnal hypoventilation ? • Sleep disruption, daytime hypersomnolence, excessive fatigue, morning headaches • Polysomnography ++ • No precise criteria • Mean SaO2 ?, % of time spent with a SaO2 < 90% ? • Level of transcutaneous or end tidal CO2 ? • Sleep fragmentation, apnea - hypopnea index ?
NPPV for nocturnal hypoventilationwith daytime normocapnia 48 patients with NMD or chest wall disease age 7– 51 years all VC < 50% Ward et al. Thorax 2005;60:1019
NPPV for nocturnal hypoventilationwith daytime normocapnia Ward et al. Thorax 2005;60:1019
Acute and chronic NPPV in children • Rationale for NPPV • Acute applications of NPPV • Indications and outcome • Technical requisites • Chronic applications of NPPV • Indications and long term benefit • Technical requisites
Technical requisites for long term (home) NPPV • Ventilators designed for pediatric patients • appropriate VT, triggers, alarms, battery • Appropriate circuits and expiratory valves • Interfaces (nasal masks) designed for children • Other supportive therapy • oxygen therapy • suction equipment • physiotherapy, assisted cough • nutrional support
Interface adapted for the child