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making with families. To our knowledge, Markstrom’s and after the initiation of ventilation; and (14) changes in
case series is the only report to date which has described respiratory technology.
the outcomes of an infant cohort.21 NiPPV was shown to Descriptive statistics were used to summarize the entire
effectively reduce the carbon dioxide levels in all study population, as well as ventilation subgroups (i.e.,
infants.21 IMV or NiPPV). Results are presented as frequencies, or
Our aim was to report on the long-term survival of medians and interquartile ranges as appropriate. The
children initiated on NiPPV before the age of one to assess Mann–Whitney U-test was used to compare non-
the safety and efficacy of NiPPV as compared to a parametric continuous variables. The chi-square test
comparator cohort of invasively ventilated children, the was used to compare categorical variables. Paired t-tests
current gold standard for children less than 1 year of age. were used as appropriate. A P-value <0.05 was
considered significant. Graph Pad Prism 5.0 (GraphPad
METHODS Software, Inc., La Jolla, CA) was used for all statistical
analysis.
A prospective clinical database was used for a
The Research Ethics Board (REB) at SickKids,
retrospective chart review of pediatric patients who
Toronto, Canada reviewed and approved the study
were initiated on long-term home mechanical ventilation
protocol (REB 1000020884). As this was a retrospective
(LTMV) and followed in the Home Ventilation Program at
study without any identifying data, the REB deemed
The Hospital for Sick Children (SickKids), Canada
informed consent unnecessary.
between January 1991 and April 2014. For this study,
only patients initiated on LTMV, who were less than
RESULTS
1 year of age, were included. LTMV was defined as the
daily use of invasive mechanical ventilation (IMV) or Fifty-one children initiated on LTHVat less than 1 year
NiPPV for at least 3 months, in the patient’s home or in a of age were included in this review. Twenty-five children
long-term residential facility. Children using NiPPV were (49%) received NiPPV and 26 (51%) receive IMV. The
initially identified and then a comparator cohort of IMV demographic information for this cohort is shown in
children was identified. Children using continuous Table 1. There was no difference (P ¼ 0.1) in the median
positive airway pressure (CPAP) were excluded. Ventila- and interquartile range (IQR) for age at the time of
tion was initiated in either the sleep laboratory or the initiation between the NiPPV group, 0.6 (IQR 0.4–0.7)
intensive care unit (ICU). years versus the IMV group, 0.4 (IQR 0.1–0.7) years.
Variables chosen for examination were as follows: (1) There were no gender differences between both groups
gender; (2) age of patients currently being followed for (P ¼ 1.0). The median duration of ventilation was
ventilation; (3) age at initiation of ventilation; (4) date of different between ventilatory subgroups, 4.2 (IQR 1.6–
initiation; (5) type of hospital ward for the initiation of 9.9) years versus 1.0 (IQR 0.4–3.5) years, P ¼ 0.001, IMV
ventilation; (6) type of ventilation; (7) duration of ventilation and NiPPV, respectively. The majority of the patients
use per 24-hr day; (8) duration of ventilation usage (years); lived at home. There were no significant differences in the
(9) residence at home versus a long-term care facility; (10) use of additional technologies at home between the two
reason for ventilation (adapted from Wallis et al.5); (11) other ventilatory subgroups.
technology used in the home (e.g., supplemental oxygen, The majority of the invasively ventilated patients were
enterostomy feeding tube, ventriculoperitoneal shunt); (12) initiated in the ICU (88.5%). The location of NiPPV
current disposition of the patient; (13) CO2 (mmHg) before initiation was as follows: 72% (n ¼ 18) in the ICU, 21%
Pediatric Pulmonology
Noninvasive Ventilation in Infants 191
98 7
00 9
02 1
3
04 3
06 5
08 7
10 9
12 1
19 199
20 199
20 200
01
20 200
20 200
20 200
20 200
20 201
as capillary blood gases and were taken between 8 am and
-2
-
-
19
4 pm. The median (IQR) pCO2 immediately prior to
NiPPV initiation was 58.0 (50.0–82.3) mmHg. The
Fig. 1. The number of children initiated on noninvasive and
median (IQR) pCO2 after NiPPV initiation with CO2 invasive long-term mechanical ventilation at home from 1996 to
normalization between 35 mmHg to 45 mmHg was 43.0 2013.
(IQR 39.8–45.0) mmHg. The pCO2 significantly im-
proved with the initiation of NiPPV, P ¼ <0.0001 (see
Fig. 2). The median (IQR) length of time between the two
pCO2 measurements was 0.30 (IQR 0.05–0.62) months. category between the two ventilatory subgroups,
Table 3 lists the indications for long-term ventilation by P ¼ 0.002. The reason for ventilation was “musculoskel-
major diagnostic category. There were significant differ- etal” in origin in the largest proportion of total ventilated
ences in the indication for ventilation by diagnostic group, n ¼ 19 (37.2%). The highest percentage of children
TABLE 2— Pre-NIV and Post-NIV Blood Gases for the Study Cohort
Pediatric Pulmonology
192 Kherani et al.
Pediatric Pulmonology
Noninvasive Ventilation in Infants 193
TABLE 5— Descriptive Characteristics of the Patients That Died While Prescribed NIV
care was completed with a one-way extubation. The four safely managed with noninvasive ventilation from
remaining deaths were in patients prescribed NiPPV who infancy.23,24 This is, in part, due to the significant
were not receiving palliative care. Patient NiPPV #3 had improvements in the availability of commercially
congenital myopathy and was prescribed NiPPV for available interfaces for young children, as well as an
hypoventilation. Despite treatment with NiPPV, this emerging option to have custom-made interfaces.25
patient continued experiencing multiple episodes of Furthermore, there have been significant advances in
aspiration pneumonia. Within 6 months of NiPPV the ventilators themselves. There were more IMV
initiation, death occurred at home. The reason for death ventilation initiations over the 2012–2013 period as
is unknown. Patient NiPPV #4 had a diagnosis of compared to any other time periods. Unfortunately, as a
arthrogryposis, scoliosis, pulmonary hypertension, and result of the relatively small patient numbers in our
GERD. She was prescribed NiPPV for nocturnal use cohort, in conjunction with this finding occurring in the
because of hypoventilation. She was found unresponsive last 2 years of the study period, we are unable to comment
during the day at home and received cardiopulmonary on whether this is an isolated finding or an emerging trend.
resuscitation at home for 25 min prior to the arrival of the The main documented complication in the noninva-
Emergency Medical Services. Brain death was confirmed sively ventilated group was midface hypoplasia (n ¼ 3 of
on day 3 of admission and withdrawal of care occurred the 25) and dermatographism (n ¼ 3 of 25), both of which
next day. The circumstances surrounding the deaths of were seen in a small minority of patients. Midface
patient NiPPV # 6 and # 7 are not available. hyopoplasia is an important consideration for pediatri-
cians initiating NiPPV because by the age of 4 years, only
60% of the adult facial structures have developed.26–28
DISCUSSION
More children were able to be weaned off ventilation in
To the best of our knowledge, we are reporting on the the NiPPV cohort as compared to the IMV comparator
largest cohort of children initiated on NiPPV at less than group. Therefore, NiPPV may be more likely to be
1 year of age. Similar to the Markstrom and Bach studies, prescribed to children with a chance of clinical improve-
we found that NiPPV was effective; there was a ment, an unclear prognosis or as part of a palliative care
significant improvement in PaCO2 levels in all patients approach where symptomatic relief is warranted, such as
after the initiation of NiPPV.21,22 No deaths were SMA type I. On the other hand, IMV was predominantly
attributable to equipment failure in our cohort which is prescribed to children with mostly central nervous system
another significant finding. (CNS) diagnoses and significant neurodisability. These
Our study provides insight into clinical practice patients were perceived as having less chance of disease
patterns over the past two decades. There has been a resolution.
significant increase in the prescription of NiPPV for There were more deaths in the NiPPV cohort. At first
children less than 1 year of age at our center. Twenty-four glance, this would suggest that NiPPV itself may be
of the 25 NiPPV initiations in the last 23 years were within contributing to a higher mortality rate than children
the last 10 years. This is consistent with the emerging initiated on invasive ventilation via tracheostomy.
literature for diagnoses, such as congenital central However, four of the eight deaths occurred in children
hypoventilation syndrome, that these children can be in whom a palliative care approach was taken at the time
Pediatric Pulmonology
194 Kherani et al.
of the initiation of NiPPV and none of the known deaths studies, adherence data for NiPPV are lacking in our
were because of equipment failure. Most of the deaths study. Our study did not report on the healthcare
appeared to be a result of the underlying disease leading to utilization in this cohort that was managed on NiPPV as
progressive respiratory failure. compared to IMV. Future directions will include a
The significant challenges with adherence to therapy provincial review of the healthcare utilization of
for pediatric NiPPV are well established in the litera- invasively and noninvasively ventilated children using
ture29–31 Only one child in our cohort was non-adherent provincial healthcare administration databases to ensure
with therapy as per parental report. This number may in acute care and homecare expenditures are not significant-
fact have been higher but objective assessment of ly greater for children managed noninvasively. Lastly, the
adherence by downloads from the ventilator itself was neurocognitive outcomes of the two cohorts of children
not formally available in the medical records for the were not formally assessed. This would be an important
duration of our retrospective review. Therefore, these data outcome measure for future, prospective, studies of long-
could not be included. Although only one patient was term use of NiPPV and IMV in infants.
completely non-compliant with therapy, we suspect that
the number of children with suboptimal adherence to
CONCLUSION
therapy in our cohort would have been much higher.
The differences in the caregiver requirements for a NiPPV initiated in children less than 1 year of age is
child at home on long-term NiPPV versus IMV, as well as increasingly being used, appears to be an effective
the associated risks, are other important considerations treatment, and many children seem to be able to be
when deciding on the mode of ventilation for a child. eventually weaned off ventilation. Although, the overall
Children who are invasively ventilated via a tracheosto- mortality rate was higher in the NiPPV cohort as
my require “24/7 eyes on care” of the tracheostomy to compared to children maintained on IMV, this appeared
ensure a stable, patent airway. There are also associated to be related to the underlying medical diagnosis as
risks of weakened cough, bleeding, and increased several children were receiving palliative care. None of
secretions related to a tracheostomy. Additionally, these the known deaths were due to equipment dysfunction.
children have the social stigma associated with a Based on the results of this long-term study, NiPPV use in
tracheostomy. On the other hand, the use of NiPPV for infants appears to be a viable long-term ventilation
more than 12 hr is associated with increased risk of skin strategy. Future directions include the development of a
breakdown from the interface. NiPPV needs to be national registry for ventilated children, and the assess-
discontinued during oral feeds and occasionally during ment of the neurocognitive outcomes and relative
enterostomy feeds to minimize the risk of gastroesopha- healthcare costs of children receiving invasive versus
geal reflux and aspiration. Surprisingly in our cohort, non-invasive long-term ventilation at home.
albeit small, very few of these potential complications
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Pediatric Pulmonology