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BRONCHOPULMONARY
    DYSPLASIA
   Dr Varsha Atul Shah
Bronchopulmonary
             Dysplasia
•   Summary
•   Introduction
•   Risk Factors
•   Clinical Features
•   Management of BPD in Level 3 Units
•   Respiratory Criteria for Transfer to a Level 2 Hospital
•   Management in the Level 2 SCN
•   Oxygen
•   Home oxygen
•   Follow up
SUMMARY
• the most severely affected babies are the
  most premature, particularly 23-26 week
  gestation babies
  diuretics and corticosteroids are effective in
  achieving short-term improvement in the status
  of ventilator dependant babies. Safety issues
  of steroid use are unresolved. There is no place
  for long term therapy with diuretics in level 2
  SCN's
  there is no consensus on how to wean oxygen in
  babies with BPD

• the transition from a tertiary hospital nursery
  to a level 2 SCN is a difficult time for parents
  as they adjust to different staff and practices
INTRODUCTION-1
• In line with the recommendations from a recent
  workshop in North America the term
  bronchopulmonary dysplasia (BPD) will be used in
  this chapter rather than Chronic Lung Disease.

• Over the past decade the clinical definition of
  BPD has evolved from oxygen dependency at 28
  days of age to  oxygen dependency at 36 weeks
  corrected gestational age.
•
INTRODUCTION-2
• The National Institute for Health in the USA has further
  divided this definition into mild (in supplemental oxygen at
  28 days of age, but in air by 36 weeks corrected age),
  moderate (requiring <30% supplemental oxygen at 36 weeks
  corrected age and severe (in >30% supplemental oxygen
  and/or requiring positive pressure support, CPAP or
  ventilation, at 36 weeks corrected age).

• BPD is the single most important factor determining length
  of stay in babies born at less than 29 weeks. The most
  severely affected babies are the most premature,
  particularly 23 - 26 week gestation babies.
RISK FACTORS
• prematurity 
• peripartum inflammation/infection
  associated with preterm labour and/or
  clinical or subclinical chorioamnionitis
• postnatal lung Injury due to volutrauma,
  barotrauma,oxygen toxicity, hypocarbia or
  infection
CLINICAL FEATURES
• babies dependant on endotracheal
  mechanical ventilation (MV)
• babies dependant of Nasal CPAP
• babies who are oxygen dependant,
  usually by nasal prongs
MANAGEMENT
•   endotracheal ventilation is increasingly being replaced by NCPAP, for even
    the tiniest babies many are being managed with NCPAP from birth. Results
    from a recent randomised controlled trial have shown that 50% of babies
    25-28 weeks gestation are able to manage without ever requiring intubation
    and ventilation, and that infants of this age who commence NCPAP from
    birth have no increased risk of death or BPD, and in fact are less likely to
    be in oxygen at 28 days of age.

•   for those who do require intubation and ventilation there is an intense
    focus on minimizing ventilator associated lung injury from the moment a
    baby is placed on a ventilator. Synchronised modes of MV with close
    monitoring of tidal volumes are key features of current practice. In
    addition there is a more liberal approach to carbon dioxide control, allowing
    CO2 to rise into the 50's and 60's providing the pH remains better than
    7.25
MANAGEMENT 2
•   oxygen damages delicate lung tissue as well as the immature
    retina. Pulse oximetry targets are typically set between 85 to 94%
    in the first weeks after birth


•   ongoing randomised trials (BOOST2 trial in Australia) are
    attempting to determine whether we should be aiming for the
    bottom or top end of this target range. Babies in this trial may be
    transferred to level 2 nurseries with their trial saturation
    monitors in place as we continue to try to answer this question. 

•   babies who require endotracheal ventilation are aggressively
    weaned and extubated to NCPAP often within 1-2 days of birth
DRUG THERAPY-
              Corticosteroids
•
•   dexamethasone is effective in achieving short-term improvement in the status of
    ventilator dependant babies as well as longer term reductions in BPD, however, there
    is now level evidence showing that dexamethasone in the first week of life is
    associated with an increased risk of cerebral palsy in survivors
•   safety of corticosteroids used later in the course of evolving BPD between 14 - 28
    days is unresolved and the Cochrane review of current evidence suggests reserving
    the use of late corticosteroids to infants who cannot be weaned from mechanical
    ventilation, and minimising the dose and duration of treatment.
•   in light of this evidence frequency of the use of steroids for BPD in NICUs has
    dramatically declined in the past 5 years  and a "low" dose regimen (eg 0.15 - 0.25mg/
    kg/day) weaned and ceased over a 7 - 10 day period is recommended
•   typical clinical scenarios where steroids would be considered are a baby > 2 weeks of
    age who is unable to be weaned from endotracheal MV
•   there is no place for the use of steroids in the treatment of BPD outside a tertiary
    neonatal unit
•   early use of inhaled steroids is ineffective in preventing BPD, although there is some
    evidence they can be used to assist extubation in ventilator dependant infants. More
    work is needed in this area before this approach is taken.
NUTRITION
•   provision of adequate calories in a nutritionally appropriate form
    is  critical, infants with BPD utilise 20-40% more kcal than infants
    without BPD
•   caloric requirements of babies with moderate to severe lung
    disease can be as high as 130 - 150 calories/kg/day
•   babies with BPD tolerate fluid overload poorly and are modestly
    fluid restricted (150 - 160mL/kg/day) and fed fortified breast
    milk or low birthweight formula
•   growth is closely monitored and caloric intake titrated
    against growth
•   Vitamin A supplementation reduces death and oxygen requirements
    at 36 weeks corrected gestational age. Vitamiin A is currently
    given as Pentavite to all infants from day 5 until discharge.
•   babies sufficiently stable to transfer to a level 2 unit should not
    require a caloric density of > 24 cal/30mls
OXYGEN
•   oxygen is the one constant in the treatment of BPD but it has been poorly studied
•   once weaned from NCPAP, oxygen is delivered by nasal prongs using low flow
    (<0.5L/min)
•   there is no consensus on how to wean oxygen in babies with BPD
•   weaning is dictated to some extent by the equipment available. Some tertiary centres
    have only recently moved from having flow meters with a lower limit of 0.25L/min/O2.
    Other units use flow meters that allow weaning down to as low as 0.005mL/min/O2.
    Most believe that weaning should be slow, but what this means varies from institution
    to institution
•   babies with BPD have a degree of pulmonary hypertension, and are therefore likely to
    benefit from a more generous oxygen administration regimen rather than from a
    restrictive policy, however the corrected gestational age and the state of
    vascularistion of the retina needs to be taken into consideration before saturation
    targets are liberalised . Whilst there is some logic to this approach, there is indirect
    evidence from randomized controlled trials conducted for other reasons suggesting
    that a more liberal oxygen policy in these babies can actually increase the pulmonary
    morbidity. RCT's are in progress to address this question
Criteria for Home Oxygen - General

• Appropriate social/home environment including reasonable
  accessibility to medical care
• Baby is on 4 hourly or demand oral feeding regimen
• Baby is normothermic in an open cot
• Satisfactory growth
• All babies discharged from tertiary units on home oxygen
  have specific Paediatric Thoracic specialist follow up. It is
  strongly recommended that Paediatricians manage babies on
  home oxygen in collaboration with a Paediatric Thoracic
  physician
Criteria for Home Oxygen -
         Respiratory

• Baby must pass an "air test". The oxygen is
  turned off, the nasal prongs removed and the
  baby monitored over 30 minutes
• If saturations are maintained >86% for 30
  minutes the test should be repeated in 48 hours.
  If a second test is satisfactory the baby is
  eligible for discharge on home oxygen on
  respiratory grounds. In other words the baby has
  demonstrated a reasonable level of respiratory
  reserve
FOLLOW UP
•   These babies require term follow up throughout childhood
•   There is an increased pulmonary morbidity in the first 2 years of
    life. Parents should be counselled about this morbidity and ways to
    minimise it.
•   Influenza vaccine is recommended for infants with ongoing
    cardiac, respiratory or neurological illnesses at 6 months of age.
    Recommendations are 2 doses, 4 weeks apart
•   Influenza vaccine is not officially recommended for these babies
•   RSV prophylaxis is not routinely recommended, the American
    Academy of Pediatrics recommends use of RSV prophlaxis for
    infants <2yrs, with BPD requiring treatment within the last 6
    months, who are discharged home prior to the RSV season. The
    Department of Health in the UK recommends its use in children
    <2yrs with BPD on home oxygen or who have had prolonged use of
    oxygen 
DRUG THERAPY0-Diuretics

•   insufficient studies of suitable size reporting on important outcomes exist
    to strongly support the use of diuretics for the treatment of BPD
•   diuretics are an effective short term therapy for ventilated babies
•   There is no evidence for efficacy in non ventilated babies therefore
    diuretic therapy should be weaned and ceased once babies are stable off
    mechanical ventilation
•   typical combinations include hydrochlorothiazide and spironolactone
•   NaCl and KCl supplementation are commonly required
•   chronic frusemide administration is generally avoided, as it has been
    associated with the development of nephrocalcinosis and hyperchloremic
    metabolic alkalosis
•   there is no place for long term therapy with diuretics in level 2 SCN's

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Bronchopulmonary dysplasia

  • 1. BRONCHOPULMONARY DYSPLASIA Dr Varsha Atul Shah
  • 2. Bronchopulmonary Dysplasia • Summary • Introduction • Risk Factors • Clinical Features • Management of BPD in Level 3 Units • Respiratory Criteria for Transfer to a Level 2 Hospital • Management in the Level 2 SCN • Oxygen • Home oxygen • Follow up
  • 3. SUMMARY • the most severely affected babies are the most premature, particularly 23-26 week gestation babies diuretics and corticosteroids are effective in achieving short-term improvement in the status of ventilator dependant babies. Safety issues of steroid use are unresolved. There is no place for long term therapy with diuretics in level 2 SCN's there is no consensus on how to wean oxygen in babies with BPD • the transition from a tertiary hospital nursery to a level 2 SCN is a difficult time for parents as they adjust to different staff and practices
  • 4. INTRODUCTION-1 • In line with the recommendations from a recent workshop in North America the term bronchopulmonary dysplasia (BPD) will be used in this chapter rather than Chronic Lung Disease. • Over the past decade the clinical definition of BPD has evolved from oxygen dependency at 28 days of age to  oxygen dependency at 36 weeks corrected gestational age. •
  • 5. INTRODUCTION-2 • The National Institute for Health in the USA has further divided this definition into mild (in supplemental oxygen at 28 days of age, but in air by 36 weeks corrected age), moderate (requiring <30% supplemental oxygen at 36 weeks corrected age and severe (in >30% supplemental oxygen and/or requiring positive pressure support, CPAP or ventilation, at 36 weeks corrected age). • BPD is the single most important factor determining length of stay in babies born at less than 29 weeks. The most severely affected babies are the most premature, particularly 23 - 26 week gestation babies.
  • 6. RISK FACTORS • prematurity  • peripartum inflammation/infection associated with preterm labour and/or clinical or subclinical chorioamnionitis • postnatal lung Injury due to volutrauma, barotrauma,oxygen toxicity, hypocarbia or infection
  • 7. CLINICAL FEATURES • babies dependant on endotracheal mechanical ventilation (MV) • babies dependant of Nasal CPAP • babies who are oxygen dependant, usually by nasal prongs
  • 8. MANAGEMENT • endotracheal ventilation is increasingly being replaced by NCPAP, for even the tiniest babies many are being managed with NCPAP from birth. Results from a recent randomised controlled trial have shown that 50% of babies 25-28 weeks gestation are able to manage without ever requiring intubation and ventilation, and that infants of this age who commence NCPAP from birth have no increased risk of death or BPD, and in fact are less likely to be in oxygen at 28 days of age. • for those who do require intubation and ventilation there is an intense focus on minimizing ventilator associated lung injury from the moment a baby is placed on a ventilator. Synchronised modes of MV with close monitoring of tidal volumes are key features of current practice. In addition there is a more liberal approach to carbon dioxide control, allowing CO2 to rise into the 50's and 60's providing the pH remains better than 7.25
  • 9. MANAGEMENT 2 • oxygen damages delicate lung tissue as well as the immature retina. Pulse oximetry targets are typically set between 85 to 94% in the first weeks after birth • ongoing randomised trials (BOOST2 trial in Australia) are attempting to determine whether we should be aiming for the bottom or top end of this target range. Babies in this trial may be transferred to level 2 nurseries with their trial saturation monitors in place as we continue to try to answer this question.  • babies who require endotracheal ventilation are aggressively weaned and extubated to NCPAP often within 1-2 days of birth
  • 10. DRUG THERAPY- Corticosteroids • • dexamethasone is effective in achieving short-term improvement in the status of ventilator dependant babies as well as longer term reductions in BPD, however, there is now level evidence showing that dexamethasone in the first week of life is associated with an increased risk of cerebral palsy in survivors • safety of corticosteroids used later in the course of evolving BPD between 14 - 28 days is unresolved and the Cochrane review of current evidence suggests reserving the use of late corticosteroids to infants who cannot be weaned from mechanical ventilation, and minimising the dose and duration of treatment. • in light of this evidence frequency of the use of steroids for BPD in NICUs has dramatically declined in the past 5 years  and a "low" dose regimen (eg 0.15 - 0.25mg/ kg/day) weaned and ceased over a 7 - 10 day period is recommended • typical clinical scenarios where steroids would be considered are a baby > 2 weeks of age who is unable to be weaned from endotracheal MV • there is no place for the use of steroids in the treatment of BPD outside a tertiary neonatal unit • early use of inhaled steroids is ineffective in preventing BPD, although there is some evidence they can be used to assist extubation in ventilator dependant infants. More work is needed in this area before this approach is taken.
  • 11. NUTRITION • provision of adequate calories in a nutritionally appropriate form is  critical, infants with BPD utilise 20-40% more kcal than infants without BPD • caloric requirements of babies with moderate to severe lung disease can be as high as 130 - 150 calories/kg/day • babies with BPD tolerate fluid overload poorly and are modestly fluid restricted (150 - 160mL/kg/day) and fed fortified breast milk or low birthweight formula • growth is closely monitored and caloric intake titrated against growth • Vitamin A supplementation reduces death and oxygen requirements at 36 weeks corrected gestational age. Vitamiin A is currently given as Pentavite to all infants from day 5 until discharge. • babies sufficiently stable to transfer to a level 2 unit should not require a caloric density of > 24 cal/30mls
  • 12. OXYGEN • oxygen is the one constant in the treatment of BPD but it has been poorly studied • once weaned from NCPAP, oxygen is delivered by nasal prongs using low flow (<0.5L/min) • there is no consensus on how to wean oxygen in babies with BPD • weaning is dictated to some extent by the equipment available. Some tertiary centres have only recently moved from having flow meters with a lower limit of 0.25L/min/O2. Other units use flow meters that allow weaning down to as low as 0.005mL/min/O2. Most believe that weaning should be slow, but what this means varies from institution to institution • babies with BPD have a degree of pulmonary hypertension, and are therefore likely to benefit from a more generous oxygen administration regimen rather than from a restrictive policy, however the corrected gestational age and the state of vascularistion of the retina needs to be taken into consideration before saturation targets are liberalised . Whilst there is some logic to this approach, there is indirect evidence from randomized controlled trials conducted for other reasons suggesting that a more liberal oxygen policy in these babies can actually increase the pulmonary morbidity. RCT's are in progress to address this question
  • 13. Criteria for Home Oxygen - General • Appropriate social/home environment including reasonable accessibility to medical care • Baby is on 4 hourly or demand oral feeding regimen • Baby is normothermic in an open cot • Satisfactory growth • All babies discharged from tertiary units on home oxygen have specific Paediatric Thoracic specialist follow up. It is strongly recommended that Paediatricians manage babies on home oxygen in collaboration with a Paediatric Thoracic physician
  • 14. Criteria for Home Oxygen - Respiratory • Baby must pass an "air test". The oxygen is turned off, the nasal prongs removed and the baby monitored over 30 minutes • If saturations are maintained >86% for 30 minutes the test should be repeated in 48 hours. If a second test is satisfactory the baby is eligible for discharge on home oxygen on respiratory grounds. In other words the baby has demonstrated a reasonable level of respiratory reserve
  • 15. FOLLOW UP • These babies require term follow up throughout childhood • There is an increased pulmonary morbidity in the first 2 years of life. Parents should be counselled about this morbidity and ways to minimise it. • Influenza vaccine is recommended for infants with ongoing cardiac, respiratory or neurological illnesses at 6 months of age. Recommendations are 2 doses, 4 weeks apart • Influenza vaccine is not officially recommended for these babies • RSV prophylaxis is not routinely recommended, the American Academy of Pediatrics recommends use of RSV prophlaxis for infants <2yrs, with BPD requiring treatment within the last 6 months, who are discharged home prior to the RSV season. The Department of Health in the UK recommends its use in children <2yrs with BPD on home oxygen or who have had prolonged use of oxygen 
  • 16. DRUG THERAPY0-Diuretics • insufficient studies of suitable size reporting on important outcomes exist to strongly support the use of diuretics for the treatment of BPD • diuretics are an effective short term therapy for ventilated babies • There is no evidence for efficacy in non ventilated babies therefore diuretic therapy should be weaned and ceased once babies are stable off mechanical ventilation • typical combinations include hydrochlorothiazide and spironolactone • NaCl and KCl supplementation are commonly required • chronic frusemide administration is generally avoided, as it has been associated with the development of nephrocalcinosis and hyperchloremic metabolic alkalosis • there is no place for long term therapy with diuretics in level 2 SCN's