NEOPED RESPIRATORY DISORDERS

Lung Parenchymal Disease  At the same time, the increased surface

RESPIRATORY DISTRESS SYNDROME tension draws fluid from the pulmonary
 also known as hyaline membrane capillaries into the alveoli.
disease, is a disease of prematurity.  In combination, these factors impair
 The incidence increases with decreasing oxygen (O2) exchange and cause
gestational age. severe hypoxemia.
 The major factors in the  The severe hypoxemia and acidosis
pathophysiology of RDS increase pulmonary vascular resistance
o qualitative surfactant deficiency (PVR).
o decreased alveolar surface area  As pulmonary arterial pressure
o increased small airways increases, extrapulmonary right-to-left
compliance shunting increases, and hypoxemia
o presence of a ductus worsens.
arteriosus.  Hypoxemia and acidosis also impair
 Surfactant production depends on both further surfactant production.
the relative maturity of the lung and  Steroids given before birth (antenatally)
the adequacy of fetal perfusion. have been shown to mature surfactant
 Maternal factors that impair fetal blood function in the fetus, decrease the
flow, such as abruptio placentae and severity of RDS, and improve outcomes.
maternal diabetes, also may lead to Clinical Manifestations
RDS.  The first signs of respiratory distress in
Pathophysiology infants with RDS normally appear soon
 In preterm infants, adequate amounts after birth.
of surfactant are present in the lung;  Tachypnea usually occurs first. After
however, the surfactant is trapped tachypnea, worsening retractions,
inside type II cells. paradoxical breathing, and audible
 In infants with RDS, type II cells do not grunting are observed.
release adequate amounts of  Nasal flaring also may be seen. Chest
surfactant. auscultation often reveals fine
 The surfactant that is released is inspiratory crackles.
incompletely formed, so it does not  Cyanosis may or may not be present. If
make tubular myelin and does not central cyanosis is observed, it is likely
decrease alveolar surface tension. that the infant has severe hypoxemia.
 Because the surfactant molecule in the  Certain other conditions, such as
alveolus is structurally abnormal, the systemic hypotension, hypothermia,
type II cells and alveolar macrophages and poor perfusion, can mimic this
have more rapid uptake for recycling. aspect of RDS.
Thus, there is a qualitative deficiency of  A definitive diagnosis of RDS usually is
alveolar surfactant. made with chest radiography. Diffuse,
 A qualitative decrease in surfactant hazy, reticulogranular densities with
increases alveolar surface tension the presence of air bronchograms with
forces, which causes alveoli to become low lung volumes are typical of RDS.
unstable and collapse and leads to  The reticulogranular pattern is caused
atelectasis and increased work of by aeration of respiratory bronchioles
breathing. and collapse of the alveoli.
 NEOPED RESPIRATORY DISORDERS

 Air bronchograms appear as aerated,  Because of the relationship between

dark, major bronchi surrounded by the arterial partial pressure of carbon
collapsed or consolidated lung tissue. dioxide (PaCO2) and functional residual
capacity (FRC), PaCO2 is lowest when
PEEP is used to optimize FRC.
Treatment  The time constant of the lungs in RDS is
 Continuous positive airway pressure short, so the lung empties very quickly
(CPAP) and positive end expiratory with each ventilator cycle. If alveolar
pressure (PEEP) are the traditional ventilation is inadequate, either peak
support modes used to manage RDS. inspiratory pressure or rate should be
 Surfactant replacement therapy and increased.
high-frequency ventilation (HFV) have  For minimizing the possibility of
been added to these traditional volutrauma, the peak inspiratory
approaches. pressure should be kept less than 30 cm
 Unless the infant’s condition is severe, a H2O for larger premature infants, and
trial of nasal CPAP is indicated (4 to 6 even lower peak inspiratory pressure is
cm H2O). indicated for more immature infants.
 Because of the hazards of endotracheal Four surfactants
tubes (ETTs), nasal prongs are  beractant (Survanta; Abbott
preferred. Laboratories, North Chicago, IL),
 If the infant’s clinical condition  calfactant (Infasurf; ONY, Amherst,
deteriorates rapidly, a more aggressive NY)
approach is required.  poractant alfa (Curosurf; Chiesi,
 Endotracheal intubation should be Cheadle, United Kingdom), and
performed under controlled conditions  lucinactant (Surfaxin, Discovery
as an elective procedure. Labs, Warrington, PA).
 Mechanical ventilation with PEEP  Beractant and calfactant are natural
should be initiated if oxygenation does bovine surfactant extracts.
not improve with CPAP or if the patient  Poractant alfa is a natural porcine
is apneic or acidotic. surfactant extract.
 There is significant interest in an  Lucinactant is a completely synthetic
approach comprising intubation, surfactant.
delivery of surfactant, extubation, and  Each of three natural surfactants has
then nasal CPAP. surfactant proteins B and C as part of
 However, more research is needed to the formulation.
understand the risks and benefits of  The synthetic surfactant uses an amino
 this approach. acid sequence that acts like surfactant
 The aim of mechanical ventilation for protein. Surfactant proteins are
RDS is to prevent lung collapse and important for decreasing alveolar
maintain alveolar inflation. surface tension.
 In severe RDS, collapse of alveoli with  All of these preparations are liquid
every breath necessitates very high suspensions that are instilled directly
reinflation pressure. into the trachea.
 To prevent the need for this high  The current standard of care is to
reinflation pressure, use of end-tidal deliver replacement surfactant to all
pressure is necessary. infants with RDS. There are
investigations of an aerosol delivery
 NEOPED RESPIRATORY DISORDERS

system that would not require intubate the infant, administer

intubation for the synthetic surfactant. surfactant, and then extubate the infant
 Currently, there is no evidence to back to nasal CPAP
support the use of a particular brand of
surfactant over another.
 Surfactant replacement therapy also is
used as both a rescue treatment (in Transient Tachypnea of the Newborn
infants who already have RDS) and a  often called type II RDS, is probably the
prophylactic therapy (in the care of most common respiratory
infants delivered prematurely).  disorder of newborns.
 Some centers use prophylactic  The cause of TTN is unclear, but it is
surfactant replacement therapy in the most likely related to delayed
care of all very small infants (<1500 g). clearance of fetal lung liquid.
 Therapies aimed at decreasing  During most births, approximately two-
pulmonary edema, improving cardiac thirds of this fluid is expelled by
output, and weaning from O2 and high thoracic squeeze in the birth canal; the
ventilator pressures are essential in the rest is reabsorbed through the
successful treatment of infants lymphatic vessels during initial
receiving surfactant. breathing.
 Recent evidence supports the use of  These mechanisms are impaired in
noninvasive ventilatory support (e.g., infants born by cesarean section or
bubble CPAP) to support even the infants with incomplete development
smallest of infants. of the lymphatic vessels (preterm or
 All surfactants are delivered by ETT. small-for-gestational-age infants).
Animal studies suggest that surfactant is  The residual lung fluid causes an
rapidly distributed throughout the lung. increase in airway resistance and an
 Each specific surfactant has different overall decrease in lung compliance.
dosing volumes and intervals. The Because compliance is low, the infant
surfactant product insert describes the must generate more negative pleural
positioning of the infant for surfactant pressure to breathe.
delivery.  This process can result in hyperinflation
 Basically, the infant is positioned with of some areas and air trapping in
different sections of the lung dependent others.
so that the surfactant enters that  Most infants with TTN are born at term
section of the lung with gravity flow. If without any specific predisposing
the infant is very sick and cannot be factors in common.
repositioned, surfactant can be  Mothers of neonates who have TTN
administered with the infant in the tend to have longer labor intervals and
supine position. a higher incidence of failure to
 Some infants have severe disease that progress in labor, which leads to
requires intubation and immediate cesarean delivery. In many cases,
administration of surfactant. however, maternal history and labor
 Some infants have only mild disease. and delivery are normal.
These less sick infants may require only Clinical Manifestations
nasal CPAP.  During the first few hours of life, infants
 For infants who have intermediate with TTN breathe rapidly.
disease, at some centers clinicians
 NEOPED RESPIRATORY DISORDERS

 Alveolar ventilation, as measured by  Amniotic fluid consists mainly of fetal

arterial pH and PaCO2, usually is lung fluid, fetal urine, and transudate
normal. from the uterine wall.
 The chest radiographic findings, which  Meconium, the contents of the fetal
may initially be indistinguishable from intestine, occasionally is expelled from
pneumonia, are hyperinflation, which is the fetus into the surrounding amniotic
secondary to air trapping, and perihilar fluid.
streaking. The perihilar streaking  Meconium consists of
probably represents lymphatic mucopolysaccharides, cholesterol, bile
engorgement. acids and salts, intestinal enzymes, and
 Pleural effusions may be evident in the other substances.
costophrenic angles and interlobar  Meconium normally is not passed until
fissures. after delivery.
Treatment  Infants who have marked perinatal
 Infants with TTN usually respond readily depression or perinatal asphyxia may
to a low FiO2 by infant O2 hood or pass meconium in utero.
nasal cannula.  The pathophysiologic control
 Infants requiring a higher FiO2 may mechanisms for the passage of
benefit from CPAP. Because the meconium in utero are not completely
retention of lung fluid may be gravity- understood. It is widely accepted that
dependent, frequent changes in the infants can have meconium aspiration
infant’s position may help speed lung in utero.
fluid clearance.  Amniotic fluid stained with meconium is
 Because TTN and neonatal pneumonia found in approximately 12% of all
have similar clinical signs, intravenous births.
administration of antibiotics should be  Meconium-stained amniotic fluid is rare
considered for at least 3 days after among infants younger than 37 weeks
appropriate culture samples are of gestational age.
obtained.  The clinical syndrome develops in 2 of
 Mechanical ventilation is rarely every 1000 infants. Of infants with
needed, and, when it is, this probably inhaled meconium, 95% clear their
indicates a complication. lungs spontaneously.
 Clearing of the lungs evident on a chest  Amniotic fluid infusion into the uterus
radiograph and with clinical before the delivery of infants with
improvement usually occurs within 24 meconium-stained fluid has been
to 48 hours. shown to improve neonatal outcomes.
 A few infants with TTN eventually have  For many years, the aspirated
persistent pulmonary hypertension. meconium itself was considered the
primary cause of MAS. More recent
MECONIUM ASPIRATION SYNDROME evidence suggests that the real
 is a disease of term and near-term causative agent is fetal asphyxia that
infants. precedes aspiration.
 It involves aspiration of meconium into  Fetal asphyxia causes pulmonary
the central airways of the lung. vasospasm and hyperreactivity of the
 It usually is associated with perinatal vasculature, which lead to persistent
depression and asphyxia. pulmonary hypertension.
Pathophysiology
 NEOPED RESPIRATORY DISORDERS

 MAS involves three primary problems:  It is no longer recommended that

pulmonary obstruction, lung tissue vigorous infants with meconium-stained
damage, and pulmonary hypertension. fluid be intubated and suctioned.
 Obstruction occurs because of plugging  However, it is important that an ETT be
of the airways with particulate inserted immediately in severely
meconium. This obstruction often is of depressed infants with thick meconium,
the ball valve type, which allows gas and suction should be applied directly
entry but prevents gas exit. to the ETT.
 Ballvalve obstruction causes air  The ETT is removed and inspected for
trapping and can lead to volutrauma. meconium. If meconium is present, the
 The lung tissue injury caused by MAS is procedure is repeated with a new ETT
chemical pneumonitis. Additionally, until no further meconium is aspirated
there are various chemical effects, or until two to four aspirations have
inflammatory responses, cytokine and been performed. The ETT should be left
chemokine activations, complement in place, and mechanical ventilation
activation, and phospholipase A2 should be started.
activation.  For prevention of hypoxemia, a flow of
 Persistent pulmonary hypertension with warmed 100% O2 should be blown
intracardiac and extracardiac right-to- across the infant’s face during the
left shunting frequently complicates aspiration efforts.
MAS.  No evidence suggests an improved
Clinical Manifestations outcome because of endotracheal
 Before birth, thick meconium, fetal suctioning in the care of infants who
tachycardia, and absent fetal cardiac have meconium and are vigorous and
accelerations during labor are evidence would not otherwise require intubation.
that the fetus is at high risk for MAS.  There is evidence that tracheal lavage
 After delivery, if the infant has a low with dilute surfactant improves the
umbilical artery pH, an Apgar score less clinical course and outcome of infants
than 5, and meconium aspirated from with MAS.
the trachea, intensive care and close  If the infant’s condition worsens, CPAP
observation for MAS are warranted. or mechanical ventilation may be
 Infants with MAS typically have gasping indicated.
respirations, tachypnea, grunting, and CPAP is indicated if the primary problem is
retractions. hypoxemia. By distending the small airways,
 The chest radiograph usually shows CPAP
irregular pulmonary densities, which can sometimes overcome the ball-valve
represent areas of atelectasis, and obstruction and improve
hyperlucent areas, which represent both oxygenation and ventilation. If respiratory
hyperinflation secondary to air acidosis is
trapping. severe or clinical assessment indicates excessive
 Arterial blood gases (ABGs) typically work in breathing,
show hypoxemia with mixed mechanical ventilation should be started. Figure
respiratory and metabolic acidosis. 34-3 shows
 In the most severe cases, there is right the ball-valve effect. At rest, the airway lumen is
to- left shunting and persistent partially
pulmonary hypertension. obstructed. With inspiration, negative
Treatment intrathoracic pressure
 NEOPED RESPIRATORY DISORDERS

opens the airway and relieves the obstruction. patterns and the requirement for supplemental
Gas enters and O2 at fixed time
expands the alveoli. With expiration, points in the infant’s life. Immaturity, genetics,
intrathoracic pressure malnutrition, O2
changes to a positive force, which narrows the toxicity, and mechanical ventilation all have
airway and causes been suspected to
total occlusion. Gas cannot be expelled and is cause BPD
trapped within nvolves many pathways. The initiating factors
the alveoli. It is difficult to provide ventilation to are related to
infants with atelectrauma (lung collapse) and volutrauma
severe MAS. These infants often retain CO2 and (large tidal volume
need increased [VT]). Factors such as hyperoxia and hypoxia,
ventilator support. Because of high airway mechanical forces,
resistance, the lungs vascular maldevelopment, inflammation,
have a long time constant. High ventilator rates nutrition, and genetics
and pressures contribute to the abnormal development of the
increase the risk for air trapping and lung and
volutrauma. lead to BPD.77,78,80-86 Atelectrauma is a term
Evidence suggests that both HFV and coined to describe
synchronous intermittent loss of alveolar volume that is both a result and
mechanical ventilation decrease the risk for air a cause of lung
leak.63,64 injury. Volutrauma is the term used to describe
Various studies have shown improvement in local overinflation
MAS with the use (and stretch) of airways and alveoli.
of HFV and surfactant.51,54,61,65-67 Nitric Atelectrauma leads
oxide (NO) has become to derecuitment (e.g., areas of alveolar collapse)
a major adjunct in the management of of the lung.
persistent pulmonary Volutrauma leads to damage to airways,
hypertension.47-49,68 Corticosteroids have not pulmonary capillary
yet been shown to endothelium, alveolar and airway epithelium,
improve outcomes for infants with MAS.69 High and basement
mean airway membranes. The combination of atelectrauma
pressures may worsen pulmonary hypertension and volutrauma
and aggravate synergistically increases lung injury.87
 right-to-left cardiac shunting.55 Both atelectrauma and volutrauma cause a
need for increased
Bronchopulmonary Dysplasia supplemental O2 concentrations. This use of
Background. Infants, especially preterm infants, supplemental O2
with severe leads to overproduction of superoxide,
respiratory failure in the first few weeks of life hydrogen peroxide, and
may develop perhydroxyl radicals. Preterm infants are
a chronic pulmonary condition called particularly susceptible
bronchopulmonary to O2 radicals because the antioxidant systems
dysplasia (BPD). BPD is a complex disease that develop in
is poorly the last trimester of pregnancy. Prolonged
defined.71-75 Historical definitions have hyperoxia begins a
included radiographic sequence of lung injury that leads to
inflammation, diffuse
 NEOPED RESPIRATORY DISORDERS

alveolar damage, pulmonary dysfunction, and and fibrosis diffusely intermixed throughout the
death. lung
The response of the lungs to the combination of (Figure 34-5).77,78 ABG measurements reveal
trauma varying degrees of
and O2 toxicity is the production and release of hypoxemia and hypercapnia secondary to
soluble mediators. airway obstruction,
These mediators probably are released from air trapping, pulmonary fibrosis, and atelectasis.
granulocytes There is a
residing in the lung. The release of these marked increase in airway resistance with an
mediators can injure overall decrease in
the alveolar-capillary barrier and cause an lung compliance.
inflammatory Treatment. The best management of BPD is
response.71,79 A “new” BPD is being described prevention.
that shows Prevention of atelectrauma and volutrauma
decreased alveolarization rather than the begins in the delivery
prominent airway room. Establishment of an optimal FRC without
damage of the “old” BPD.77-88 This change in overstretching
the pathologic the lung requires careful attention to detail in
characteristics of BPD is thought to be related providing end-tidal pressure and avoiding large
to improvements VT. Surfactant
in ventilator management, the use of should be delivered early in the course of
surfactant, and processes treatment.
that interrupt alveolar development (e.g., Treatment of infants with BPD involves steps to
postnatal steroid minimize
therapy).74,89,90 additional lung damage and prevent pulmonary
Clinical Manifestations. BPD has various clinical hypertension
manifestations. and cor pulmonale. Infants with severe disease
Some very immature infants may start with little may be dependent
or no on supplemental O2 or mechanical ventilation
O2 requirement and little or no mechanical for months
ventilation requirement. and have symptoms of airway obstruction for
Progressive respiratory distress develops at years. Therapy
approximately usually is supportive throughout the course of
2 to 3 weeks of life, and then the infant needs the disease. An
O2 and mechanical infant with BPD is given respiratory support as
ventilation. Other immature infants may begin needed. Supplemental
with pneumonia O2 can help decrease the pulmonary
or sepsis and need very high levels of O2 and hypertension that
mechanical is common with BPD.80
ventilation. In either of these scenarios, Multiple treatments have been suggested for
progressive vascular infants with
leakage and areas of atelectasis and BPD.73,76,84,90-92 Diuretics are given as
emphysema develop in the needed to decrease pulmonary
lungs, and progressive pulmonary damage edema; antibiotics are given to manage existing
occurs. The chest pulmonary
radiograph in severe disease shows areas of infection.93-95 Chest physical therapy may help
atelectasis, emphysema, mobilize secretions
 NEOPED RESPIRATORY DISORDERS

and prevent further atelectasis. Bronchodilator starts as obstructive apnea and then develops
therapy into central
may help decrease airway resistance.93 Steroid apnea.99,100,104,105
therapy with Cause. Premature infants have immature
dexamethasone can produce substantial short- control of respiratory
term improvement drive in response to O2 and carbon dioxide
in lung function, often allowing rapid weaning (CO2). In
from ventilatory mature animals, an increase in alveolar PaCO2
support. However, steroid therapy has little elicits an increase
effect on in VT and respiratory rate. A decrease in FiO2
long-term outcome such as mortality and below room air
duration of O2 also triggers an increase in VT. Conversely, in
therapy.96 Steroid therapy also has been premature animals,
implicated in decreased an increase in PaCO2 temporarily increases VT
alveolarization and increased developmental but does not
delay.92 Although increase respiratory rate. A decrease in FiO2
steroids are still given in clinical practice, they below room air
should be used decreases VT and respiratory rate. This effect
cautiously and only after the risks have been can lead to apnea
thoroughly in a premature infant. Several factors in
explained to the parents. The use of NO to addition to prematurity
prevent or improve can cause apnea in infants. Table 34-2
BPD is controversial.97,98 summarizes the potential
Control of Breathing causes, associated signs, and diagnostic
Apnea of Prematurity indicators.99,105-107
Background. Apnea of prematurity is a Treatment. Infants with apnea need continuous
common, controllable monitoring
disorder among premature infants. It usually of heart and respiratory rates. Continuous
resolves over noninvasive
time.99-103 Premature infants frequently have monitoring of oxygenation by transcutaneous
periodic respiration, electrode or
which comprises sequential short apneic pulse oximetry is recommended. Most apneic
episodes of 5 to incidents can be
10 seconds followed by 10 to 15 seconds of quickly ended with gentle mechanical
rapid respiration. stimulation, such as
Apneic spells are abnormal if (1) they last longer picking the infant up, flicking the sole of the
than 15 foot, or rubbing
seconds or (2) they are associated with the skin.106,108 If the cause of apnea is not
cyanosis, pallor, hypotonia, prematurity, treatment
or bradycardia. must be directed at resolving the underlying
If no effort to breathe occurs during a spell, the condition. Table
apnea 34-3 outlines current treatment strategies for
is called central apnea. If breathing efforts infants with
occur, but obstruction apnea.86,109 Apnea secondary to prematurity
prevents airflow, the apnea is termed responds well
obstructive. Mixed to methylxanthines, especially theophylline and
apnea is a combination of the central and caffeine.110-112
obstructive types that
 NEOPED RESPIRATORY DISORDERS

These agents stimulate the central nervous of prematurity are not at higher risk for sudden
system and increase infant death
the infant’s responsiveness to CO2. For infants syndrome (SIDS) than other infants.
with apnea that Apnea monitoring can allow infants who are
does not respond to treatment with otherwise ready
theophylline, doxapram can for discharge but still having occasional
be used.113-115 However, doxapram is episodes of apnea to go
delivered by continuous home.103,109,118 However, the presence of a
infusion and has multiple toxicities. home apnea monitor
The “Back to Sleep” program initiated by the is a significant inconvenience to the family.
American Home monitors lack
Academy of Pediatrics has significantly the sophisticated filtering systems of hospital
decreased the incidence monitors, and
of SIDS.116 It is thought that when a newborn, they have very frequent false alarms.118 Also,
which has a relatively there is no evidence
heavy head and weak neck muscles, manages to that apnea monitors prevent SIDS.
position Pulmonary Vascular Disease
its face into a soft surface (e.g., mattress, pillow, Persistent Pulmonary Hypertension
bunting, etc.) of the Newborn
it will develop increased CO2 retention and Background. Persistent pulmonary
become apneic. hypertension of the
Unlike older infants, newborns will not have a newborn (PPHN) is a complex syndrome with
response to many causes.119-132
awaken and move, they become apneic, then The common denominator in PPHN is a return
become bradycardic, to fetal circulatory
then have cardiopulmonary arrest.100-116 pathways, usually because of elevated PVR. This
CPAP also can be used to manage infant condition
apnea.117 Although results in further right-to-left shunting, severe
the mechanism of action is not established, hypoxemia, and
CPAP probably metabolic and respiratory acidosis.
increases FRC and improves arterial partial Pathophysiology. In the uterus, the fetus does
pressure of oxygen not use the
(PaO2) and PaCO2. CPAP may stimulate vagal lungs as a gas-exchange organ. PVR is high, and
receptors in the systemic vascular
lung, increasing the output of the brainstem resistance (SVR) is low. This condition produces
respiratory centers. a PVR/
Severe or recurrent apnea that is unresponsive SVR ratio greater than 1. A fetus has two
to these interventions anatomic shunts that
may necessitate mechanical ventilatory are not present in older infants, children, or
support. adults: the foramen
As the respiratory control mechanisms mature, ovale and ductus arteriosus. With a PVR/SVR
apnea of ratio greater than
prematurity normally resolves without 1 and the anatomic shunts, blood flow bypasses
intervention. Apneic the lung either
spells begin to disappear by weeks 37 to 44 of at the atrial level (foramen ovale) or at the
postmenstrual pulmonary artery
age with no apparent long-term effects. Infants (ductus arteriosus). Intrauterine total
who have apnea pulmonary blood flow
 NEOPED RESPIRATORY DISORDERS

and systemic arterial O2 saturation (SaO2) are disease detected with chest radiography or
low. PaCO2 measurement.
In the transition to extrauterine life, PVR In infants with a significant shunt through the
decreases owing to ductus
gas filling the lungs and increasing PaO2 in the arteriosus, there usually is a substantial gradient
pulmonary (>5%) between
venous circulation. SVR increases with the preductal and postductal O2 saturation. This
removal of the placenta gradient can be
from the circulation, and this makes the found easily if two pulse oximeters are placed
PVR/SVR ratio on the infant, one
less than 1. If PVR does not decrease to allow on the right arm and the other on either leg.
the PVR/SVR ratio Treatment. Initial therapy for PPHN is removal
to become less than 1, the infant has PPHN. of the
The three fundamental types of PPHN are underlying cause, such as administration of O2
vascular spasm, for hypoxemia,
increased muscle wall thickness, and decreased surfactant for RDS, glucose for hypoglycemia,
cross-sectional and inotropic
area of pulmonary vessels.123 Vascular spasm agents for low cardiac output and systemic
is an acute event hypotension. If correction
that can be triggered by many different of the underlying problem does not correct
conditions, including hypoxemia,
hypoxemia, hypoglycemia, hypotension, and the infant needs intubation and mechanical
pain. Increased ventilation. Because
muscle wall thickness is a chronic condition that pain and anxiety may contribute to PPHN, the
develops in infant may need
utero in response to several different causative sedation and, frequently, paralysis. If these
factors, including measures do not
chronic fetal hypoxia, increased pulmonary improve oxygenation, the next step is HFV. This
blood flow (e.g., mode of ventilation
intrauterine closure of the ductus arteriosus), allows a higher FRC without a large VT. Inhaled
and pulmonary NO is
venous obstruction (e.g., total anomalous considered the next intervention.68,133-136 If
pulmonary venous all of these modalities
return with obstructed below-diaphragm fail to improve oxygenation, the infant may be a
return). Decreased candidate
cross-sectional area is related to hypoplasia of for extracorporeal membrane oxygenation
the lungs and (ECMO).137-139 Also,
occurs with congenital diaphragmatic hernia, types 3 and 5 phosphodiesterase inhibitors are
Potter sequence being used in
(absent kidneys), and oligohydramnios infants and children with refractory pulmonary
syndromes (decreased hypertension.
amniotic fluid). 140-143 Even with all of these treatments,
Clinical Manifestations. PPHN should be PPHN remains a
suspected when complex disease with high morbidity and
an infant has rapidly changing O2 saturation mortality.
(SaO2) without Congenital Abnormalities
changes in FiO2 or has hypoxemia out of Affecting Respiration
proportion to the lung
 NEOPED RESPIRATORY DISORDERS

Congenital abnormalities that affect respiration atresia with a proximal fistula, esophageal
can be divided atresia with a
into several groups: airway diseases, lung distal fistula, esophageal atresia with both a
malformations, chest proximal and a
wall abnormalities, abdominal wall distal fistula, esophageal atresia without either
abnormalities, and diseases fistula, and an
of neuromuscular control. intact esophagus with a so-called H fistula.144
Airway Diseases The most common
Airway abnormalities have three fundamental of these malformations is esophageal atresia
mechanisms: with a distal fistula,
internal obstruction, external obstruction, and which accounts for 85% to 90% of all TEFs. The
disruption. least common
Internal obstruction includes common is the H fistula. All of these malformations
problems, such as manifest as difficulty
laryngomalacia, that cause obstructive apnea. swallowing, bubbling and frothing at the mouth,
Less common and choking,
problems caused by internal obstruction are particularly during attempts at feeding. These
tracheomalacia, anomalies can
laryngeal webs, tracheal stenosis, and occur in isolation or as part of an association of
hemangiomas. All of these defects. The
diseases usually manifest as a combination of most common is the VATER or VACTERL
inspiratory association of vertebral
stridor, gas trapping, expiratory wheezing, and anomalies, imperforate anus, TEF, and renal or
accessory respiratory radial
muscle activity. anomalies. In VACTERL, cardiac anomalies are
External compression can be caused by added, and
hemangiomas, neck renal and limb anomalies replace renal or radial
or thoracic masses, and vascular rings. These anomalies in
lesions are far less the acronym. These associated anomalies must
common than diseases caused by internal be sought in any
obstruction, but they infant with TEF. TEF is managed with surgical
are not rare. The symptoms are similar to those ligation of the
of internal fistula (tying it closed) and reconnection of the
obstruction. Neck masses usually are obvious at interrupted
visual inspection. esophagus.145 Most infants with TEF have a
Intrathoracic masses and vascular rings must be good outcome;
suspected however, some infants have severe
on the basis of the clinical manifestations: noise malformations that can cause
during the chronic problems. Infants with TEF usually need
respiratory cycle that worsens with exertion. only supportive
The infant may respiratory care. They usually do not have lung
have difficulty with swallowing. disease.
Airway disruptions usually are related to However, some infants need HFV because the
tracheoesophageal air leak through
fistula (TEF) in a newborn. This malformation the fistula can become larger than the airflow to
usually is associated the alveoli.
with esophageal atresia. There are five types of Lung Malformations
TEF: esophageal
 NEOPED RESPIRATORY DISORDERS

There is a broad spectrum of rare lung Lobar emphysema is an airway malformation

malformations that that causes gas
occur in the newborn period.106-108 These trapping in a lobe of the lung. These
lesions are thought to malformations manifest
be part of a continual spectrum of diseases that as space-occupying masses within the thorax.
originate as They usually are
defects in lung segmentation. The most treated by surgical removal.
common is congenital Congenital Diaphragmatic Hernia
pulmonary adenomatoid malformation (CPAM); Congenital diaphragmatic hernia is a severe
this was previously disease that usually
known as cystic adenomatoid malformation of manifests in newborns as severe respiratory
the lung. distress.148-150 The
CPAM is classified into five types on the basis of pathophysiologic mechanism is a complex
the type and combination of lung
size of the cyst.146,147 The disease may affect hypoplasia, including decreased alveolar count
entire lobes of the and decreased
lung. The affected parts of the lung do not pulmonary vasculature, pulmonary
exchange gas and hypertension, and unusual
can become infected. The usual treatment is anatomy of the inferior vena cava.148-150 This
surgical removal disorder varies
of the affected lobe. There is also the potential between asymptomatic (rare) and severe life-
for malignant threatening disease
transformation. (frequent). There are two types of hernia:
Some affected fetuses can develop hydrops in Bochdalek hernia
utero. Most (lateral and posterior defect, usually on the left)
infants with CPAM have symptoms of lung and Morgagni
volume loss. As the hernia (medial and anterior, may be on either
mass expands, the normal surrounding lung is side). Hernias
compressed. that occur in the right hemidiaphragm may be
Some CPAMs resolve spontaneously. A few less severe
infants have severe because the liver can block the defect and
cardiorespiratory compromise and need decrease the volume
respiratory support of abdominal contents that can enter the
and emergency surgery. However, better results thorax.150
are seen when Some authors speculate that the diaphragmatic
surgery can be performed electively. hernia
Other, less common lung malformations include complex is a developmental field defect and not
pulmonary just a simple
sequestration and lobar emphysema. Both of cascade of events related to a hole in the
these diseases diaphragm. This theory
involve maldevelopment of lobes of the lung. is partly based on long-term outcomes of
Sequestration is survivors with diaphragmatic
a primitive, frequently cystic, lung lobe that is hernias. These survivors frequently have severe
not in communication scoliosis
with the tracheobronchial tree and frequently in the direction of the diaphragm defect. They
receives also
no pulmonary vascular blood flow. frequently have severe esophageal reflux
disease.
 NEOPED RESPIRATORY DISORDERS

Most cases of congenital diaphragmatic hernia aortic arch and coarctation of the aorta.
can be diagnosed Hypoplastic left heart
in utero with ultrasonography. Physical syndrome has several accepted treatments,
examination may including a palliative
yield the following findings: scaphoid abdomen surgical procedure (Norwood) and
(because the transplantation.181-184 Neither
abdominal contents are in the thorax), the Norwood procedure nor transplantation is
decreased breath sounds, ideal, and
displaced heart sounds (because the heart is each option has significant associated problems.
pushed away from The decision
the hernia), and severe cyanosis (from lung must be made in consultation with the family.
hypoplasia and NEONATAL RESUSCITATION
pulmonary hypertension). The diagnosis is Resuscitation of the newborn is a subset of
established with resuscitation techniques.
chest radiography. Most infant resuscitations occur in the delivery
The general treatment of infants with room.
congenital diaphragmatic Although these resuscitations can range from
hernia involves neonatologists and pediatric minimal intervention
surgeons. to full resuscitation, more than 90% of them can
Initial treatment is insertion of an ETT, paralysis, be
and mechanical successfully dealt with by stimulation, ensuring
ventilation. A large sump tube is placed in the the presence of
stomach and an airway, and providing breathing support.185-
connected to continuous suction. These 188 RTs are very
therapies allow adequate important members of any resuscitation team.
ventilation and oxygenation and prevent gas Their expertise
insufflation in establishing and supporting an airway and
of the intestine. Most centers delay surgical initiating respiratory
repair for several support is essential. It is beyond the scope of
days to allow the natural decrease in PVR. On this chapter
day 7 to 10 of to delineate the guidelines of neonatal
life, a surgeon closes the defect. This scenario resuscitation. The reader
occurs only should refer to the neonatal resuscitation
for infants with easily correctable pulmonary guidelines published
hypertension. by the American Academy of Pediatrics
Infants with severe pulmonary hypertension (AAP).185-188
may need HFV PEDIATRIC RESPIRATORY DISORDERS
Initial treatment is intravenous administration Compared with the common cardiopulmonary
of prostaglandin diseases in
E1. Most infants with these defects need the neonatal period, the pulmonary conditions
support with mechanical that occur
ventilation. These infants do not have lung among older infants and children commonly
disease. The result from airway
pressures and rates used should be set obstruction caused by bacterial or viral
appropriately. infections. Other enti-
There are standard surgical repairs for both ties discussed in this section include asthma,
interrupted SIDS, gastroesophageal
reflux disease, and cystic fibrosis.
 NEOPED RESPIRATORY DISORDERS

Sudden Infant Death Syndrome event occurs when an infant becomes apneic,
SIDS is the leading cause of death (40%) among cyanotic, or limp
infants younger enough to frighten the parent or caregiver. The
than 1 year in the United States. Approximately prone sleeping
7000 infants die position has been strongly associated with
of SIDS each year in the United increased risk for
States.99,102,189,190 A presumptive SIDS. It is difficult to differentiate death from
diagnosis is based on the conditions of death in SIDS and death
which a previously from intentional suffocation. The possibility of
healthy infant dies unexpectedly, usually during intentional suffocation
sleep. must be investigated but with great
Autopsy shows that many infants who die of sensitivity.109,191,192
SIDS have evidence Prevention
of repeated episodes of hypoxemia or ischemia. Because of the unknown causation and
Factors unexpected occurrence,
associated with increased frequency of SIDS are there is no therapy for SIDS. Prevention is the
presented in goal. Successful
Box 34-1. If the infant is found and resuscitation prevention requires that infants at high risk be
is successful, identified
the diagnosis would be apparent. through a history of risk factors and
Cause documented monitoring
The cause of SIDS is unknown. Apnea of or event recording. After identification that an
prematurity is not a infant is at risk,
predisposing factor, and there is no evidence the family is trained in apnea monitoring and
that immaturity cardiopulmonary
of the respiratory centers is a cause. Although resuscitation. The AAP recommends placing
infants in families infants in either
in which two or more SIDS deaths have the supine or the side-lying position for the first
occurred are at slightly 6 months
higher risk, there is no evidence of a genetic of life and reducing soft objects in the infant’s
link. The best sleeping
knowledge of SIDS comes from population or environment.109,191-193 To define the need
epidemiologic and appropriate
studies and is summarized in Box 34-2. An approach for home monitoring of infants, the
infant who dies of AAP has
SIDS typically is a preterm African-American boy developed a policy statement on infantile apnea
born to a and home
poor mother younger than 20 years of age who monitoring.109,191 The AAP recommendations
received inadequate for the need for
prenatal care. Infants 1 to 3 months old are and use of home monitoring are summarized in
most susceptible, Box 34-3.
and death is most likely to occur at night during Gastroesophageal Reflux Disease
the Gastroesophageal reflux disease (GERD) is the
winter. The risk for SIDS also is high among regurgitation
infants who previously of stomach contents into the esophagus and is
experienced an apparent life-threatening event. common in
Such an childhood. Some causes of GERD are not
pathologic. There is
 NEOPED RESPIRATORY DISORDERS

general agreement that there are important commonly occurs soon after a viral upper
interactions between respiratory tract
GERD and various disorders of the respiratory infection. The infant may have a slight fever
system.194 with an intermittent
Respiratory problems caused by cough. After a few days, signs of respiratory
gastroesophageal reflux include distress develop,
reactive airways disease, wheezing, aspiration particularly dyspnea and tachypnea. Progressive
pneumonia, inflammation
laryngospasm, stridor, chronic cough, choking and narrowing of the airways cause inspiratory
spells, and and expiratory
apnea.194-198 GERD should be considered wheezing and increase airway resistance. A
when an infant has chest radiograph
faced a sudden life-threatening event and when shows signs of hyperinflation with areas of
an older child consolidation. The
has unexplained chronic head and neck diagnosis of RSV infection can be established by
problems. GERD can immunofluorescent
be diagnosed with esophageal pH testing, upper assay the same day and assists in the
gastrointestinal implementation of
contrast studies, and gastric scintiscan. When a treatment plan.
GERD has been Prophylaxis. In recent years, passive
diagnosed, medical therapy can begin.199-205 immunization for RSV
Occasional cases has become available.208,210-216 Initially,
that do not respond to medical management passive immunization
may require surgical was recommended only for preterm infants
intervention. with BPD. However,
Bronchiolitis passive immunization is now recommended for
Bronchiolitis is an acute infection of the lower high-risk infants
respiratory tract, younger than 2 years of age who require
usually caused by respiratory syncytial virus medical therapy
(RSV). Nearly 1 in for chronic lung disease, infants born at less
10 infants younger than 2 years of age acquires than 32 weeks of
a bronchiolitis gestational age, and infants with congenital
infection. The outcome is generally good, heart disease who
although approxi- have cardiovascular compromise (Box 34-4).216
mately 1% of infants hospitalized for Treatment. Treatment of a patient with
bronchiolitis die of respiratory bronchiolitis varies
failure. Infants most prone to respiratory failure with the severity of the infection and the clinical
as a signs and
consequence of bronchiolitis are very young symptoms. Many patients can be treated at
and immunodeficient home with humidification
and have a comorbidity, such as congenital and oral decongestants. Patients with more
heart disease, severe
BPD, CF, or childhood asthma.206-210 symptoms (apnea) and comorbidity usually are
Clinical Manifestations hospitalized,
The clinical manifestations of bronchiolitis are and treatment is directed at relieving the airway
inflammation obstruction
and obstruction of the small bronchi and and associated hypoxemia. Hospitalized
bronchioles. Bronchiolitis children frequently are
 NEOPED RESPIRATORY DISORDERS

treated with systemic hydration and O2 via occur. A radiograph of the upper airway is
nasal cannula, highflow helpful in confirming
nasal cannula, hood, croup tent, or nasal the diagnosis and ruling out epiglottitis but is
cannula and usually not
assisted with airway needed in most cases of croup. Classic croup is
clearance.208,212,215,217-220 Antibiotics may seen on an
be administered to control secondary bacterial anteroposterior radiograph as characteristic
infections. If subglottic narrowing
bronchiolitis progresses to acute respiratory of the trachea, called the steeple sign (Figure
failure, mechanical 34-8).
ventilation is required. Because of the Treatment
obstructive nature of this The evaluation and treatment of a child with
disorder, low respiratory rates and long croup must focus
expiratory times may on the degree of respiratory distress and
be needed to prevent air trapping. Heliox has associated clinical findings.
been used for If stridor is mild or occurs only on exertion and
severe airways disease requiring mechanical cyanosis
ventilation.219 Vigorous is not present, hospitalization is generally not
bronchial hygiene, occasionally including required and the
tracheobronchial child is treated at home. If there is stridor at
aspiration, usually is needed to maintain a rest (accompanied
patent airway by harsh breath sounds, suprasternal
(Box 34-5). retractions, and cyanosis
Croup with breathing of room air), hospitalization is
Croup is a viral disorder of the upper airway indicated.
that normally The traditional treatment of a child with mild to
results in subglottic swelling and obstruction. moderate
Termed laryngotracheobronchitis, croup has involved cool mist therapy with or
viral croup is usually caused by the without supplemental
parainfluenza O2. However, there is no evidence that this
virus and is the most common form of airway practice is
obstruction beneficial.221 Corticosteroids and epinephrine
in children 6 months to 6 years old. RSV and have been shown
influenza virus to have the greatest benefit for decreasing the
are less common as causative agents. Bacterial length and severity
superinfection of respiratory symptoms associated with viral
with Staphylococcus aureus, group A croup.222-225
Streptococcus pyogenes, or The addition of budesonide has been shown to
Haemophilus influenzae may worsen croup. reduce the
Clinical Manifestations severity of symptoms in mild to moderate cases
Symptoms become evident after 2 or 3 days of of croup.222-225
nasal congestion, Progressive worsening of the clinical signs
fever, and coughing. A child typically has slow, despite treatment
progressive indicates the need for intubation and
inspiratory and expiratory stridor and a barking mechanical ventilation.
cough. As the Heliox has been used for infants and children
disease progresses, dyspnea, cyanosis, with severe
exhaustion, and agitation
 NEOPED RESPIRATORY DISORDERS

disease. There is some evidence to show short- cause further and immediate swelling of the
term benefit; epiglottis and
however, long-term benefit has not yet been abrupt and total upper airway obstruction.
shown.226 Children with suspected
Epiglottitis epiglottitis should be accompanied by personnel
Epiglottitis is an acute and often life- expert
threatening infection of in emergency intubation during any transport
the upper airway that causes severe obstruction for diagnostic
secondary to procedures.
supraglottic swelling. Evidence suggests that the Extubation of any patient should take into
incidence of consideration the
epiglottitis is decreasing among children and pathophysiologic condition that led to
increasing in intubation. In this case,
adults,227 probably because of the use of the RT should look for evidence that the
vaccines. The most infection is resolving
common cause is H. influenzae type B infection. and that the upper airway is no longer inflamed.
Other organisms A lack of fever
that are increasingly found to be causes of for at least 12 hours and visual inspection of the
acute epiglottitis throat that
include group A S. pneumoniae, S. aureus, reveals minimal inflammation would be most
Klebsiella pneumoniae, helpful. After
Haemophilus parainfluenzae, and beta- extubation, close monitoring must be
hemolytic streptococci performed for evidence
(groups A, B, C, and F).228 of airway compromise. Cool mist may be helpful
Clinical Manifestations to minimize
A child with epiglottitis usually has a high fever, inflammation after extubation.
sore throat, Treatment
stridor, and labored breathing.228-233 The Children with epiglottitis need elective
patient does not have intubation under general
a croupy bark but instead has a muffled voice. anesthesia in the operating room.
Older children Tracheostomy may be needed
may report a sore throat and difficulty if the patient’s condition warrants it; however,
swallowing. Difficulty this procedure is
swallowing may cause drooling. Lateral rarely used. There should be no attempt to lie
radiographs of the neck the child down
(Figure 34-9) show the epiglottis is markedly or attempts to intubate until the child is
thickened and sedated. Premature
flattened (thumb sign) and the aryepiglottic attempts at intubation can precipitate acute
folds are swollen; airway obstruction
the vallecula may not be visualized. Visual and respiratory arrest. After an airway is
examination of the secured, a sample
upper airway is dangerous in these children and for bacterial culture should be obtained and
always should antibiotic
be performed in a controlled setting by therapy should be started. Corticosteroids may
personnel expert in decrease the
emergency intubation. Inadvertent traction of swelling.228,229,231,233 Children with an ETT
the tongue can should be sedated and
 NEOPED RESPIRATORY DISORDERS

restrained to prevent inadvertent extubation. Patients with CF primarily experience

Extubation should abnormalities in the
not be attempted until an upper airway leak is respiratory, digestive, and reproductive
readily detected. tracts.240 Complications
Cystic Fibrosis of lung disease are the leading cause of death in
Cystic fibrosis (CF) is one of the most common patients with
life-limiting CF.241 The decreased airway surface liquid
autosomal recessive diseases, occurring in secondary to CFTR
approximately 1 in dysfunction leads to impaired mucus clearance,
every 3500 newborns in the United States.234 It resulting in
affects approximately inflammation and infection of the airways238
30,000 persons in the United States and 70,000 that causes a
persons patient to have a chronic productive cough.
worldwide.234 The incidence varies by race and Chronic airway
ethnicity affecting infections can occur early in life, most
approximately 1 : 3200 whites, 1 : 9500 frequently with S. aureus,
Hispanics, 1 : 15,000 H. influenzae, or Pseudomonas aeruginosa.234
African Americans, and 1 : 31,000 in Asian Certain organisms,
Americans.235 CF is including P. aeruginosa, methicillin resistant S.
caused by mutations in the gene that encodes a aureus, and
multifunctional Burkholderia cepacia, have been associated
protein called the CF transmembrane with greater declines
conductance regulator in lung function.242-244 There are infection
(CFTR).236 One of the main functions of this control guidelines to
protein is to serve reduce the risk for acquiring pathogens that can
as an apical chloride channel in airway, be detrimental
intestinal, and exocrine to the health of patients with CF.245 As the
cells.237 The movement of chloride ions and disease progresses,
regulation of the cycle of inflammation, infection, and lung
sodium ions is important to the proper damage results
regulation of the water in lung hyperinflation and bronchiectasis.237
content of secretions.238 The dehydrated Patients with endstage
viscous secretions that CF lung disease have severe debility from
result from the CFTR abnormality lead to organ respiratory
dysfunction failure and may develop pulmonary
resulting in the clinical manifestations of the hypertension and cor
disease.238 There pulmonale.241
are over 1900 known CFTR mutations,239 which Approximately 85% of patients with CF have
are grouped exocrine pancreatic
into six classes that result in varying levels of insufficiency.234 CFTR dysfunction in the
CFTR production pancreas dramatically
and dysfunction.237 The variety of CFTR reduces the amount of digestive enzymes,
mutations explains leading to
some of the variability in the severity of the malabsorption of fats and proteins and less so
clinical manifestations for carbohydrates.
of the disease. 246 Malabsorption results in bulky, greasy, foul-
Clinical Manifestations smelling
 NEOPED RESPIRATORY DISORDERS

stools, fat-soluble vitamin deficiencies, and poor Since 2010, screening for CF in newborns
weight gain indicating persistent
with failure to thrive. Some newborns present hypertrypsinogenemia is performed in all 50
with a condition states and the
called meconium ileus secondary to bowel District of Columbia.234 The diagnosis is
obstruction of thick confirmed by a sweat
and hardened meconium that sometimes chloride test performed at an accredited CF
results in intestinal center. The skin is
perforation.247 Rectal prolapse also can occur stimulated to produce sweat (pilocarpine
as a result of iontophoresis), and a
malabsorption and elimination of bulky sweat chloride level greater than 60 mEq/L
stools.248 Patients confirms the diagnosis
with pancreatic sufficiency can have recurrent of CF.258 The diagnosis in some infants is
bouts of pancreatitis. challenging
249 Liver disease can result in prolonged because they have two CFTR mutations, but a
obstructive normal sweat
jaundice in the newborn period.250 Some chloride (<30 mEq/L in infants younger than 6
children and adults months).258
have progressive liver disease leading to The diagnosis of CF in patients not identified by
cirrhosis and portal newborn
hypertension.251 screening can be made by performing a sweat
Males with CF have obstructive azospermia as a test in children
result of or adults with signs or symptoms suggestive of
congenital absence of the vas deferens.252 CF, including
Males and females recurrent sinus or lung infections,
with CF often have pubertal delays.253 Females bronchiectasis, nasal polyps,
also have reduced digital clubbing, malabsorption, failure to gain
fertility.254 weight as
As children with CF age, the risk for diabetes expected, recurrent pancreatitis, salt-losing
increases, and syndromes, and
by adulthood the majority of patients have male infertility resulting from obstructive
abnormal glucose azoospermia, or if
tolerance testing.234 they have a sibling with CF.258 Having two
The electrolyte composition of sweat in CF sweat chloride test
patients is abnormal results greater than 60 mEq/L confirms the
because of the higher content of salt.255 diagnosis.258
Increased salt losses Pancreatic insufficiency is most often diagnosed
in the sweat may lead to the initial presentation with a stool
of some fecal elastase testing.259
CF patients with hyponatremic hypochloremic Monitoring
metabolic CF patients should be managed at an accredited
alkalosis.256 The sweat chloride test used for CF center.234
the diagnosis of The CF Foundation recommends that newborns
CF is based on the abnormal concentration of with CF are
chloride in the seen soon after a positive newborn screen
sweat of patients with the disease.257 result and then at
Diagnosis least on a monthly basis until 6 months of age,
then every 2
 NEOPED RESPIRATORY DISORDERS

months until age 1 year, and every 2 to 3 DNA.261 The routine daily use of inhaled DNase
months thereafter.259 has been shown
It is recommended that older children and to improve pulmonary function and reduce
adults are seen quarterly exacerbations in
at a minimum. Because CF lung disease is patients with CF.261,262 Inhaled DNase is
progressive, recommended for daily
patients are closely monitored by symptom use in patients 6 years of age and older.263
assessment, physical Nebulized 7% hypertonic
examinations, sputum cultures to monitor saline is thought to improve mucociliary
airway flora, and clearance and
objective measurements with spirometry and has been shown to improve lung function and
chest radiography. reduce exacerbations.
234 The nutritional status of each patient is also 264 Nebulized 7% hypertonic saline is currently
monitored recommended
very closely, including their growth, body mass for twice-daily use in patients 6 years and
index, protein older.263 High
stores, and fat-soluble vitamin levels. doses of the antiinflammatory drug ibuprofen,
Treatment when used at
Multiple therapies are used to maintain a doses resulting in appropriate levels, reduce the
patient’s lung health. progression of
Airway clearance has been a mainstay of CF lung disease and is recommended for use in
therapy in CF.260 There children 6 to
are many options for airway clearance, 17 years of age with a forced expiratory volume
including percussion in 1 second
and postural drainage, positive expiratory greater than 60% predicted.263 The regular use
pressure, autogenic of azithromycin
drainage, autocycle of breathing technique, possibly through its antiinflammatory and
oscillatory positive antimicrobial properties,
expiratory pressure, and high-frequency chest helps preserve lung function and decreases the
compression.260 frequency
Airway clearance therapies have been shown to of pulmonary exacerbations. Azithromycin is
increase sputum recommended
production, improve exercise tolerance, and in patients 6 years and older.263 Patients taking
decrease the rate of azithromycin
lung function decline.260 In general, no specific should be monitored for the acquisition of
airway clearance nontuberculous
technique is superior to another; therefore mycobacteria, at which time their azithromycin
airway clearance monotherapy
must be tailored to the individual patient.260 should be discontinued.263
As a result of the cellular debris from chronic The lungs of CF patients are chronically
infection and colonized with
inflammation, there is free DNA in the airways bacteria. One of the most common organism is
that contributes P. aeruginosa,
to the viscosity of secretions. To treat this, 234 which has been associated with more rapid
inhaled recombinant decline in
deoxyribonuclease (DNase) is used to degrade lung function and decreased survival.242
the viscous Inhaled antibiotics directed
 NEOPED RESPIRATORY DISORDERS

against this organism are recommended for 41.1 years.234 In the near future, the number
eradiation of adults will outnumber
and chronic suppression. Currently two inhaled the children with CF.234
antibiotics— ROLE OF THE RESPIRATORY
inhaled tobramycin and inhaled aztreonam— THERAPIST IN NEONATAL
are available AND PEDIATRIC RESPIRATORY
for use.263,265,266 DISORDERS
More recently developed therapies are aimed As with any clinical situation, the role of the RT
at correcting in the special
the underlying CFTR defect or potentiating its environment of neonatal and pediatric care is to
function.237 In use his or her
2012, ivacaftor, a potentiator that activates expertise and knowledge to improve patient
defective CFTR, was outcome. Because
approved for use in the United States in there are significant differences in the diseases,
patients with a specific pathophysiologies,
variant of CF—the G551D CFTR mutation. In and function of respiratory support equipment
2014 the approval between
for use of ivacaftor was expanded to some adult and pediatric patients, the RT must be
other gating CFTR thoroughly familiar
mutations. Ivacaftor has been shown to with all aspects of pediatric care. The old adage
improve lung function “children are
and significantly reduce pulmonary not little adults” is true. Equally, newborns are
exacerbations.267 Ivacaftor not little children.
also was observed to significantly decrease the Each of these age groups has unique
sweat chloride characteristics that
concentration. require specialized knowledge and experience.
Lung transplantation is an option for patients The RT is an
with advanced important part of a team that is dedicated to
severe CF lung disease. the health and
The malabsorption secondary to deficiency of well-being of these fragile patients.
pancreatic Also, the RT has an important role in providing
enzymes is managed with pancreatic enzyme education
supplementation and emotional support, not only of the pediatric
and vitamin supplementation.240 Some patient but
patients also require also of the families and caregivers. Frequently,
oral calorie supplements to assist with their the RT is at the
nutritional needs. bedside of patients when the parents or
Prognosis caregivers are present.
When CF was first described in 1938, children The RT is invaluable in helping patients and
lived approximately parents understand
6 months.268 As a result of improvements in CF the respiratory goals of each individual patient.
care and
advances in therapies, survival has significantly
improved.234 In
2002 the median age of survival was 31.3 years,
and 10 years
later in 2012, the median survival of patients
with CF rose to