Post on 27-Oct-2019
Holistic Approach to ChildrenHolistic Approach to Children
ith Ch i L Diwith Chronic Lung Disease
Suchada Sritippayawan, MD.
Div. Pulmonology & Crit Care
Wanida Pao-in, MD.
Div. Pulmonology & Crit Care gy
Dept. Pediatrics,
Faculty of Medicine
gy
Dept. Pediatrics,
Faculty of Medicine
Chulalongkorn UniversityThammasart University
Case illustration
• Triplet, GA 25 weeks
• Triplet A (male) 635 g, triplet B (female) 720 g, triplet C (female) 605 gtriplet C (female) 605 g
• APGAR: A-2,7, B-1,7, C-1,7• Surfactant, on mechanical ventilator,
antibiotics since birthantibiotics since birth• Triplet B died at DOL 2 because of pulmonary
hemorrhage
Case illustration
Triplet C• BW 605 g, HC 21 cm, Ht 30, APGAR 1,7• Ventilator support: conventional mode• Ventilator support: conventional mode
Initial setting: Conventional mode PIP 15 cmH2O, PEEP 4 cmH2O, IMV rate 60/min, T 0 34 sec FiO 0 8TI 0.34 sec, FiO2 0.8
Case illustration
• Antibiotics, antifungal• Dopamine to maintain BP
PRC furosemide• PRC, furosemide• Monitor fluid, electrolytes • Indomethacin for PDA closure• PPN • Enteral feeding: start at DOL 3, full feed at te a eed g sta t at O 3, u eed at
DOL 13
Chest x-ray at DOL 1
Triplet C at DOL 28
• BW 670 g• Ventilator support: PIP 19 cmH2O, PEEP 4
cmH O IMV rate 70/min T 0 34 sec FiO 0 8cmH2O, IMV rate 70/min, TI 0.34 sec, FiO2 0.8 • PE: active, PR 130, RR 79, BP 50/28, BT 36.7
Heart: normal S1S2, no murmur, no hyperactive precordiumhyperactive precordiumLung: subcostal retraction, occ. coarse crepitations and wheezing Others: unremarkableOthers: unremarkable
Triplet C at DOL 28
Labs • CBG: pH 7.23, PCO2 70, PO2 43, HCO3 28
BUN 25 Cr 1 5• BUN 25, Cr 1.5• Eye exam: no ROP• U/S: no IVH
Chest x-ray at DOL 28
Problem lists
• Premie 25 weeks• Ventilator dependent since birth• PDA (S/P close with indomethacin)• PDA (S/P close with indomethacin)• Bacterial / fungal infection• Renal insufficiency
Poor weight gain• Poor weight gain
Questions
Q1: Does this patient have CLD?Q2: What are respiratory problems and their
pathophysiology?pathophysiology?Q3: How can we treat respiratory problems?Q4: What are other comorbids and their
pathophysiology?pathophysiology?Q5: How can we treat these comorbids?Q6: Is there any long term sequelae and
how to follow up?how to follow up?
Problem lists
• Premie (GA 25 weeks)• Ventilator dependent since birth• PDA (S/P close with indomethacin)PDA (S/P close with indomethacin)• Bacterial / fungal infection • Renal insufficiency• Poor weight gain• Poor weight gain
Respiratory problems
• Premie (GA 25 weeks)• Ventilator dependent
at DOL 28at DOL 28• CBG: CBG: pH 7.23,
PCO2 70, PO2 43, HCO3 28C f• CXR: Bilateral hyperinflation, radiolucent areas plus strands of radiodensity ad o uce t a eas p us st a ds o ad ode s ty
Q1: Does this patient
have CLD?
What is chronic lung disease?
• Bronchopulmonary
dysplasia
P l d MV• Prolonged MV use
• Chronic aspirationChronic aspiration
• Chronic infection
• BronchiectasisChronic ILD• Chronic ILD
• Asthma, etc.,
Chronic lung disease
AJRCCM 2003;168:356-96
Definition of BPD
Old definition (Bancalari’s criteria)• MV at least 3 days in neonate plus• Oxygen dependent at DOL 28 plus• Oxygen dependent at DOL 28 plus• Radiologic changes
Clin Pediatr (Phila) 2002;41:77-85
Definition of BPD
Old definition (Bancalari’s criteria)• Radiologic changes
- RDS (D1-3)RDS (D1 3)- Opacification of both lungs (D4-10) - Small rounded areas of radiolucency in both lungs (D10-20)both lungs (D10 20)
- Enlarged radiolucent areas + strands of radiodensity
Clin Pediatr (Phila)2002;41:77-85
Definition of BPD
New definition (NIH consensus)• Time point of assessment
GA < 32 wk : 36 wk PMA or D/C homeGA < 32 wk : 36 wk PMA or D/C homeGA > 32 wk : > 28 d but < 56 d of postnatal age or D/C home
• Treatment with oxygen > 21% for at least 28Treatment with oxygen 21% for at least 28 days plus
Clin Pediatr (Phila)2002;41:77-85
Definition of BPD
New definition (NIH consensus)• Severity of BPD
Mild BPDMild BPDGA < 32 wk : Breathing RA at 36 wk PMA or D/C homeGA > 32 wk : Breathing RA at 56 d of postnatalGA 32 wk : Breathing RA at 56 d of postnatal age or D/C home
Clin Pediatr (Phila)2002;41:77-85
Definition of BPD
New definition (NIH consensus)• Severity of BPD
Moderate BPDModerate BPDGA < 32 wk : Need FiO2 ≥ 0.3 and/or positive pressure at 36 wk PMA or D/C homeGA > 32 wk : Need FiO2 ≥ 0.3 and/or positiveGA 32 wk : Need FiO2 ≥ 0.3 and/or positive pressure at 56 d of postnatal age or D/C home
Clin Pediatr (Phila) 2002;41:77-85
Definition of BPD
New definition (NIH consensus)• Lung parenchymal disease plus• Clinical features of respiratory distress plusClinical features of respiratory distress plus• Not acute event
Clin Pediatr (Phila) 2002;41:77-85
Q2: What are respiratory problems
d h i h h i l ?and their pathophysiology?
Pulmonary system
Abnormal lung Abnormal AbnormalAbnormal lung parenchyma &
chest wall
Abnormal airway
Abnormal cardiorespiratory
control duringchest wall control during sleep
Other bid
Abnormal gas
comorbids
gexchange and lung
function during gawake and sleep
Chronic lung disease: BPD
• Age at the onset
• Types and severity of
respiratory insults
• DurationSeverity of
BPDDuration
• Body responseBPD
• Treatment
• Genetics
Pediatr Respir Rev 2003;4:28-39.
Pulmonary system
Abnormal lung Abnormal AbnormalAbnormal lung parenchyma &
chest wall
Abnormal airway
Abnormal cardiorespiratory
control duringchest wall control during sleep
Other bid
Abnormal gas
comorbids
gexchange and lung
function during gawake and sleep
Pathogenesis of CLD• Oxygen free radical
↓ ti id tAcute lung injury • Ventilator-induced
PDA l l d• ↓ antioxidant
enzymes system Recruitment of
• PDA, vol. overload
• Infections
inflammatory cells
• Age at the onset
• Types, severity,
Acute inflammatory reactions
yp y
duration of
respiratory insults
Resolved Persistent inflammation
respiratory insults
• Body response
• Treatment
No CLDLung destruction & damage
• Treatment
• Genetics
No CLDCLD
Pathogenesis of BPD
N Engl J Med 2007; 357:1946-55
Old BPD vs. New BPD
Old BPD New BPD
• Alternating atelectasis
with hyperinflation
• Less regional heterogeneity
of lung diseasewith hyperinflation
• Severe airway epithelial
of lung disease
• Rare airway epithelial
lesions
• Marked airway smooth
lesions
• Mild airway smoothMarked airway smooth
muscle hyperplasia
y
muscle thickening
• Extensive diffuse fibroproliferation
• Rare fibroproliferative changes
Lancet 2006;367:1421-31
p
Old BPD vs. New BPD
Old BPD New BPD
• Hypertensive remodeling
of pulmonary arteries
• Fewer arteries but
dysmorphicof pulmonary arteries
• Decreased alveolarisation
dysmorphic
• Fewer, larger and and surface area simplified alveoli
Lancet 2006;367:1421-31
Pulmonary system
Abnormal lung Abnormal AbnormalAbnormal lung parenchyma &
chest wall
Abnormal airway
Abnormal cardiorespiratory
control duringchest wall control during sleep
Other bid
Abnormal gas
comorbids
gexchange and lung
function during gawake and sleep
Airway abnormalities in CLD
• Prolonged intubation, PPV
• Cyanotic spell
• Inappropriate intubation,
suctioning Cyanotic spell
• Wheezing not respond
g• Concomitant infections
Central & upper airway obstruction
to bronchodilator
• Recurrent atelectasis
• Lobar emphysema• Glottic and subglottic stenosis
• Tracheobronchial stenosis • Failed extubation• Ventilator dependent
Tracheobronchial stenosis
• Granuloma formation
AJRCCM 2003;168:356-96
• Tracheobronchomalacia
Abnormal lung function in CLD
CLD
Abnormal Abnormal lungAbnormal Other co-morbid
airway Abnormal lung parenchyma chest wall
mechanics
Other co morbid
• RAD
• GER• etc.
Ob t ti d f t• Hypoxemia • Obstructive defect
• Restrictive defect
• Hypoxemia
• Hypercarbia Can persist • Diffusion defect• BHR
until adult• O2 dependent
• BHR• MV dependent
Pulmonary system
Abnormal lung Abnormal AbnormalAbnormal lung parenchyma &
chest wall
Abnormal airway
Abnormal cardiorespiratory
control duringchest wall control during sleep
Other bid
Abnormal gas
comorbids
gexchange and lung
function during gawake and sleep
Cardiorespiratory control during sleep in CLDsleep in CLD
CLD
Abnormal Abnormal lung AbnormalOther co-morbid
airway Abnormal lung mechanics
Abnormal chest wall mechanics
• RAD• GERGER
Sleep-related hypoxemia, hypercarbia
Abnormal hypoxic ventilatory and arousal
• Poor RV function
response during sleep
AJRCCM 2003;168:356-96
Poor RV function• Abnormal autonomic control of HR
Q3: How can we treat
respiratory problems?p y p
Management of Respiratory ProblemRespiratory Problem
Ventilatory strategiesVentilatory strategies
After Birth
• Early initiation of nasal CPAP
After Birth
Early initiation of nasal CPAP• Nasal intermittent positive pressure
ventilation (NIPPV)ventilation (NIPPV)• Patient-triggered ventilation (SIMV, assist-
control, and pressure support ventilation)control, and pressure support ventilation)• High-frequency ventilation (HFV)• Volume targeted ventilation:• Volume targeted ventilation:• Permissive hypercapnia
P i i h i• Permissive hypoxemia
Ventilatory strategies
Established CLD
• Minimizing ventilatory support (e.g. nCPAP, NIPPV whenever possible)
• Tolerating higher PaCO (55-60 mm Hg• Tolerating higher PaCO2 (55-60 mm Hg provided pH >7.25)
• Target SpO2 : 89-94%• If on IMV: consider using PTV• If on IMV: consider using PTV • Oxygen therapy to maintain SpO2 > 92-93%
Other strategiesg
• Methylxanthines• Steroids: considered in infants after 10-14Steroids: considered in infants after 10 14
days of age• Diuretics for features of pulmonary edema • Bronchodilators for bronchospasmBronchodilators for bronchospasm • Sedation and muscle relaxation for ‘BPD
spells’
Wheezing in CLDI
• Airway hyperresponsiveness• Airway inflammation
A t i l d f t b l tti t i• Anatomical defect: subglottic stenosis, airway malacia
• Interstitial edemaH t f il• Heart failure
• Aspiration syndromep y• Infection
Q4: What are other comorbidsQ4: What are other comorbids
and their pathophysiology?and their pathophysiology?
Chronic lung diseases
Multidisciplinary (Holistic)(Holistic)
approach and follow-up isfollow-up is
required
AJRCCM 2003;168:356-96
Comorbids in CLD
Abnormal CVS in CLD
• PHT, Cor pulmonale
• LVH, LVF
• Systemic HT
• ↑systemic-to-pulmonary collateral circulation• ↑systemic-to-pulmonary collateral circulation
Lancet 2006; 367:1421-31
AJRCCM 2003;168:356-96
Pathophysiology of PHT in CLD• Hypoxemia
H bi ↓ Al l• Premie
• Hypercarbia
• MetabolicPulmonary
vasoconstriction↓ Alveolar
development• Hypoxemia
• HYperoxiaacidosis
Vascular remodeling
• Intrauterineinfection
↓ Pulmonary vascular Endothelial
ll i j developmentcell injury
I ti lIntimal proliferation
Pulmonary hypertension RHF
Lancet 2006; 367:1421-31
AJRCCM 2003;168:356-96
Pathophysiology of HT & LVH in CLD• Hypoxemia
H bi ↑ R i ↑ N ti• Hypercarbia
• Metabolic
↑ Renin-angiotensin
activity
↑ WOB↑ Negative intrathoracic pressure
acidosis
↑↓ ↑ LV afterload↓ Pulmonary endothelial
function
↑ PVR
SystemicLVH↓ Clearance of
norepinephrine Systemic HT
norepinephrine
AJRCCM 2003;168:356-96
Pathophysiology of GER in CLD
• ↑ intrathoracic –ve
pressure
• Low, flat diaphragmLow, flat diaphragm
Abnormal KUB function in CLD
↑ PVR Prolonged loop diuretic use
• Hypoxia
↑ RAP
diuretic use• Hypotension
• Nephrotoxic
• ↑ ANP
• Hypercalciuria
• Nephrocalcinosis
pdrug
• ↑ Vasopressin
p
• Hyperphosphaturia
↓ M K
• ↑ water retention
• ↓ Mg, K
• Metabolic alkalosisTubular injury
• ↓ Na
Clin Pediatr 2002; 41:77-85.
Other comorbids in CLD
• ↑REE
• ↓ intake
• Drugs
• Other comorbids
Q5: How can we treat
these comorbids?
Management of Oth C bidOther Comorbids
Management of ComorbidsManagement of Comorbids
• PHT
• GERDOxygen GERD
• KUB
Calcium channel blockersProstacyclin Phosphodiesterase inhibitor: sildenafil• KUB
N t iti
Phosphodiesterase inhibitor: sildenafil Endothelin receptor antagonists: bosentan
• Nutrition
GERDGERD
• Feeding• Drugs: - prokineticsDrugs: prokinetics• - H2-receptor antagonists• - proton-pump inhibitors
• FundoplicationFundoplication
nutritionnutrition
– Calorie intake to 120 to 150 Kcal/kg/d– Breast milk fortified with HMF– Fat supplementation (e.g. MCT oil) – Multivitamin to meet RDA Multivitamin to meet RDA
Q6: Is there any long term
sequelae and how to seque ae a d o to
follow up?follow up?
Abnormal lung function in CLD
CLD
Abnormal Abnormal lungAbnormal Other co-morbid
airway Abnormal lung parenchyma chest wall
mechanics
Other co morbid
• RAD
• GER• etc.
Ob t ti d f tH i • Obstructive defect
• Restrictive defect
• Hypoxemia
• Hypercarbia Can persist • Diffusion defect• BHR
• Exerciseintolerance
until adult
• BHRintolerance
Long term sequelae
Management of Long Term SequelaeLong Term Sequelae
Management of Long Term SequelaeManagement of Long Term Sequelae
• Respiratory system• Infection prevention• Infection prevention• Neurologic/developmentalg p• Hearing/vision• Growth• Other
Triplet C at DOL 1 - 4.5 months
• NSS neb, chest PT• On nasal CPAP at age 2 months• Seretide® Ventolin® MDI at age 2 months• Seretide®, Ventolin® MDI at age 2 months
(2 weeks) • O2box/cannula at age 2.5 months (36 wk PMA)
Discharge at age 4 5 mo (corrected age 1 mo)• Discharge at age 4.5 mo (corrected age 1 mo)– BW 2,700 g, HC 35 cm, length 48 cm – Home med: MTV, FeSO4
Triplet C at DOL 9 months
Age 9 mo (corrected age 6 mo)• 5.6 kg, HC 39.5 cm, length 61 cm
• spastic diplegia • DENVER II: PS 6 mo FM 4 mo L 4 mo• DENVER II: PS 6 mo, FM 4 mo, L 4 mo,
GM 6 mo