Abstract
Study Objectives:
Sleep-disordered breathing is a major cause of morbidity and mortality among pediatric patients with severe neurological disabilities such as cerebral palsy. Despite increasing use of noninvasive ventilation (NIV) in this group, there remains a lack of consensus about its role and indications. We aim to explore the indications, acceptability, and outcomes of a cohort of children with severe, complex neurological disability and sleep-disordered breathing, managed with NIV.
Methods:
Data were retrospectively extracted on children with severe neurological disabilities (Gross Motor Function Classification System V equivalent) initiated on NIV in Queensland over a 5-year period. Demographic, clinical, hospitalization, and polysomnography data were collected, as well as caregiver-reported side effects and NIV adherence.
Results:
Fourteen (median age 9.1 years; 6 female) children were included, 8 with cerebral palsy and 6 with other complex neurological disabilities. Obstructive sleep apnea was the most common indication for NIV (n = 12). The median (interquartile range) apnea-hypopnea index improved on NIV [pre-NIV 21.3 (interquartile range 10.0–28.2) vs post-NIV 12.2 (interquartile range 2.8–15.2)], although this was not statistically significant. There was significant improvement in proportion of time spent with SpO2 < 95% (22.2% pre-NIV vs 7.85% post-NIV; P < .05). Reported side effects were minimal. There was no reduction in hospital admissions in the 12 months post-NIV initiation.
Conclusions:
Our findings suggest that NIV improves polysomnography parameters among children with severe neurological disability. Long-term outcomes and overall impact on quality of life remain unclear. Ethical issues and overall benefit must be considered before embarking on this mode of therapy.
Citation:
Morrison L, Suresh S, Leclerc MJ, Kapur N. Symptom care approach to noninvasive ventilatory support in children with complex neural disability. J Clin Sleep Med. 2022;18(4):1145–1151.
Keywords: noninvasive ventilation, cerebral palsy, sleep-disordered breathing
BRIEF SUMMARY
Current Knowledge/Study Rationale: Children with complex neurological disabilities experience high rates of sleep-disordered breathing and are sometimes treated with noninvasive ventilation. However, indications, acceptability, and outcomes of this treatment among this population are unknown.
Study Impact: Using data from 14 children with severe, complex neurological disabilities (Gross Motor Function Classification System Level V or equivalent) and sleep-disordered breathing, we report that noninvasive ventilation is a feasible option in this group with improvement in respiratory parameters in sleep and good acceptance and adherence.
INTRODUCTION
Children with severe physical and intellectual disability such as cerebral palsy (CP) and other neuropathological syndromes are at an increased risk of developing a number of associated disorders including sleep-disordered breathing,1 epilepsy, scoliosis, gastrointestinal diseases, chronic pain syndromes, and swallowing dysfunction.2,3 Sleep quality among these patients is impaired by epilepsy, muscle spasms, gastrointestinal reflux, and visual impairment (which may impact melatonin release and therefore sleep initiation).4 In particular, sleep-disordered breathing (SDB) including snoring, respiratory related arousals, central sleep apnea. and obstructive sleep apnea (OSA) are all known to be highly prevalent among this population.1,5 Neurological disabilities, including CP, are also associated with a decrease in central respiratory drive during sleep, leading to central sleep apnea or mixed patterns.6 Sleep apnea causes hypoxemia and hypercapnia, increasing risk of pulmonary hypertension and respiratory failure.7
However, the leading causes of morbidity and mortality among these patients are respiratory complications.8,9 Neurologically disabled children frequently experience swallowing dysfunction and aspiration, as well as decreased cough reflex and expiratory muscle dysfunction.7 These factors lead to recurrent lung infections and respiratory insufficiency. The high rates of scoliosis and weakened chest wall muscles among this group also impact respiratory function, leading to mechanical lung restriction and decreased vital capacity and contributing to the risk of respiratory failure and nocturnal hypoventilation and hypoxemia.10,11 Sleep-related hypoventilation caused by muscle weakness, thoracic restriction, or chronic lung disease has also been reported in this cohort.1,12
The exact prevalence of SDB among patients with neurological disabilities is unknown and is thought to be both under-diagnosed and under-treated.1,13 Additionally, no clear guidelines exist for managing these conditions.1,14 Although noninvasive ventilation (NIV) (including continuous positive airway pressure and bilevel positive airway pressure) is typically the treatment offered in children who have persistent SDB despite adenotonsillectomy, its potential for symptom alleviation among children with complex neural disability is unclear. Observational studies demonstrate that some neurologically disabled pediatric patients are being managed with NIV.1 Racca and colleagues15 reported that 13% of 378 children treated with NIV in their Italian cohort had been diagnosed with CP. Other surveys suggest that this figure ranges from 7–13%.15–17 Yet the indications and outcomes for this treatment modality remain uncertain. In addition, there is an ongoing ethical debate regarding role of ventilatory support for purely life-extending purposes in this group, when it may not improve quality of life (QoL), and in some cases may make it worse.18
Through this retrospective study, we aim to describe a cohort of children with severe, complex neurological disability and severe SDB, managed with NIV. Furthermore, our objective is to explore the indications, acceptability, and outcomes of NIV use in this group.
METHODS
For this retrospective, descriptive study, a list of all children (< 18 years of age) initiated on home NIV (continuous positive airway pressure or bilevel positive airway pressure) in Queensland from November 2015 to November 2020 was obtained through the Queensland Children’s Hospital’s Respiratory & Sleep Medicine database. Children with a diagnosis of CP or another severe neurological disability, with a Gross Motor Function Classification System (GMFCS) Level of V or equivalent, were included for further analysis. Severity of CP is categorized by the GMFCS, which ranges from Level I (child can walk and perform gross motor skills with impaired speed, balance, and coordination) to Level V (child is limited in ability to maintain antigravity postures and is transported in a manual wheelchair).19 Other neuropathological syndromes with similar GMFCS level, including genetic diseases (Rett syndrome, leukodystrophy) and epileptic encephalopathy (Lennox Gastaut Syndrome), were also included.
Demographic, clinical, hospitalization, and polysomnography (PSG) data were extracted from their electronic medical records. Clinical details extracted included underlying etiology, associated comorbidities, level of disability, and indication for NIV initiation. Clinical notes were also reviewed for the 12 months following NIV initiation, and all caregiver-reported side effects were recorded. NIV adherence data was recorded by patients’ machines and downloaded at approximately 3-month intervals. Severe OSA was defined as apnea-hypopnea index ≥ 10 events/h and hypoventilation was defined as transcutaneous carbon dioxide > 50 mm Hg for ≥ 25% of the recording. Data were tabulated and analyzed using SPSS version 26. Wilcoxon signed rank test was utilized to compare paired PSG data before and after NIV initiation.
RESULTS
Over the 5-year period, 410 children were initiated on home NIV (272 on continuous positive airway pressure, 138 on bilevel positive airway pressure). Of these, 14 (median age 9.1 years [range 7 months to 15 years]; 6 females) had severe, complex neurological disability with GMFCS Level of V or equivalent and were included for further analysis. Eight children (57%) had CP, 2 had Rett syndrome, and 1 child each had leukodystrophy, Leigh syndrome, 12p partial trisomy syndrome, and Lennox-Gastaut syndrome. Clinical OSA was the most common indication for initiating NIV support (n = 11; 79%), 2 of these had mixed SDB and 1 had central sleep apnea. Seven children had associated hypoventilation. Seizure was the most common comorbidity (n = 10; 72%). Seven patients (50%) were gastrostomy fed. Other comorbidities included scoliosis (n = 5; 36%), visual and hearing impairment (n = 4; 29%), and airway malacia (n = 4; 29%). Only 5 children had had previous adenotonsillectomy, and half (n = 7) had previously been using supplemental oxygen. One child with Rett syndrome died during the follow-up period. The cause of death was unrelated to NIV use. Table 1 describes characteristics of each included child.
Table 1.
Patient characteristics.
| Subject No. | Agea (y) | Diagnosis | NIV Type | A&Tb | Indication for NIV | Comorbidities | AHI (Pre-NIV) | AHI (On NIV) | Ave. NIV Usec |
|---|---|---|---|---|---|---|---|---|---|
| 1 | 12 | CP | CPAP | Adenectomy only | OSA | Chronic esophagitis, microcephaly, visual impairment | 21.3 | 3.9 | 8:38 |
| 2 | 15 | CP | BPAP | No | Dystonic breath holding spells | Tracheomalacia, gastrostomy, eosinophilic esophagitis | N/Ad | 0 | 8:12 |
| 3 | 7 | CP | BPAP | No | OSA | Epilepsy, tracheomalacia, communicating hydrocephalus, scoliosis, gastrostomy | N/Ad | 9.8 | 8:47 |
| 4 | 9 | Rett Syndrome | BPAP | No | CSA with hypoventilation | Epilepsy, GERD, scoliosis, recurrent LRTI, osteopenia, Jejunostomy | 10 | 2.8 | 5:38 |
| 5 | 5 | CP | CPAP | Yes | OSA with hypoventilation | Epilepsy, scoliosis, Soto’s Syndrome | 99.7 | 15.2 | N/A: Did not tolerate |
| 6 | 15 | Rett Syndrome | BPAP | No | OSA with hypoventilation | Epilepsy, aspiration pneumonia, gastrostomy, osteopenia, scoliosis | 7.4 | 25.9 | 5:25 |
| 7 | 2 | Leuko-dystrophy | CPAP | Adenectomy only | OSA with hypoventilation | Vision impairment, gastrostomy, GERD | 28.2 | 25.3 | 3:38 |
| 8 | 14 | Lennox Gastaut Syndrome | BPAP | No | OSA with hypoventilation | Epilepsy, vision & hearing impairment, gastrostomy | 10.7 | 12.8 | 8:10 |
| 9 | 5 | Leigh's disease | CPAP | Yes | OSA | Visual impairment | 4.5 | 1.6 | 10:14 |
| 10 | 0.5 | 12p partial trisomy syndrome | CPAP | Adenectomy only | OSA with hypoventilation | Epilepsy, laryngomalacia, visual & hearing impairment | 22.5 | 2.2 | 13:32 |
| 11 | 3 | CP | BPAP | No | Mixed SDB | Epilepsy, tracheomalacia, laryngomalacia, cardiac hypertrophy, bulbar dysfunction, GERD, hearing loss | N/Ad | 0.8 | 8:33 |
| 12 | 13 | CP | CPAP | Yes | Mixed SDB with hypoventilation | Epilepsy, hypotonia, scoliosis, gastrostomy | 104.5 | 12.2 | N/A: Discontinued |
| 13 | 13 | CP | CPAP | No | OSA | Epilepsy | N/Ad | 0.7 | 10:33 |
| 14 | 1.5 | CP | BPAP | No | Symptom management, support for airway clearance | Epilepsy, bronchiectasis, bilateral hearing loss | N/Ad | 4.4 | N/A: Recently initiated |
aAge at NIV initiation. bAdeno- and/or tonsillectomy prior to NIV initiation. cAverage nightly NIV use (hours: minutes). dNo baseline polysomnography performed. AHI = apnea-hypopnea index, Ave. = average, BPAP = bilevel positive airway pressure, CP = cerebral palsy, CPAP = continuous positive airway pressure, CSA = central sleep apnea, GERD = gastroesophageal reflux disease, N/A = not applicable, NIV = noninvasive ventilation, OSA = obstructive sleep apnea, SDB = sleep-disordered breathing. LRTI = lower respiratory tract infection.
Polysomnographic characteristics
Diagnostic PSG prior to NIV initiation was available for 9 children, and 2 additional children had pre-NIV overnight oximetry studies available. Paired oxygen saturation (SpO2) data were therefore available for 11 children, with paired apnea-hypopnea index data available for 9. Among the available baseline (pre-NIV) studies, the median apnea-hypopnea index was 21.3 (n = 9; interquartile range [IQR] 10.0– 28.2), median of mean SpO2 was 95.3% (n = 11; IQR 93.9–97.2%), and the median of maximum transcutaneous carbon dioxide was 55.5 mm (n = 9; IQR 52.0–59.3 mm).
Post-NIV PSG was available for all 14 children. Half (n = 7) were initiated on continuous positive airway pressure and the other half (n = 7) on bilevel positive airway pressure support to manage their SDB. The median of mean SpO2 showed improvement with NIV initiation in 9 children, although the overall results were not statistically significant (n = 11; pre-NIV 95.3% [IQR 93.25–96.75] vs post-NIV 96.4% [IQR 96.25–97.25%]; Figure 1A). The 2 children who showed a reduction in SpO2 had the diagnostic study on supplemental oxygen, hence the diagnostic and titration values were not comparable.
Figure 1. Pre-NIV and post-NIV comparison of polysomnography parameters.
(A) Effect of NIV on mean oxygen saturation (n = 11). (B) Effect of NIV on AHI in Gross Motor Function Classification System V Cohort (n = 9). (C) Effect of NIV on % study time SpO2 < 95% (n = 9). AHI = apnea-hypopnea index, NIV = noninvasive ventilation, SpO2 = oxygen saturation.
The median (IQR) apnea-hypopnea index improved on NIV (pre-NIV 21.3 [IQR 10.0–28.2) vs post-NIV 12.2 (IQR 2.8–15.2)], although this was also not statistically significant (Figure 1B). There was significant improvement in the median proportion of time spent with SpO2 < 95% (pre-NIV 22.2% vs 7.85% post-NIV, P < .05; Figure 1C).
Adherence
Two children did not tolerate NIV, and therapy was ceased within 4 months of initiation. Adherence data for 1 child were unavailable. The remaining 11 patients had at least 1 download of NIV adherence data, with a median daily usage of 8:33 hours per night (range 3:32 hours to 13:32 hours) (Figure 2). The average percentage days used for our cohort was 79%.
Figure 2. NIV adherence by number of days used and time used.
Adherence data: percentage of days used (A) and time used (days used) (B). NIV = noninvasive ventilation.
Adverse effects
Four children did not report any adverse effects of NIV use. In the first 12 months of NIV use, mask-related pressure areas were the most commonly reported adverse effect (n = 6, 43%). Five children (36%) reported discomfort due to mask leakage; 3 (21%) reported increased secretions; 2 (14.3%) reported dry mouth, throat, or nose; and 1 (7.1%) reported blocked or runny nose.
Hospitalizations
Nine children had hospitalization data for the 12 months preceding and following initiation on NIV. The other 5 either initiated NIV treatment within the last year or did not have a complete 12 months of data before discontinuing treatment. Of the 9 with complete data, 5 experienced fewer hospitalizations (including emergency department visits and inpatient visits) after being initiated on NIV. Overall, the cohort had a combined 33 hospitalizations in the year prior to NIV initiation, and an identical 33 inpatient or emergency visits in the year following NIV initiation. Hospitalization data are summarized in Figure 3.
Figure 3. Hospitalizations in 12 months before and after NIV initiation.
NIV = noninvasive ventilation.
DISCUSSION
Neurologically disabled children with sleep-disordered breathing present a multifaceted clinical picture with complex respiratory comorbidities and a paucity of research to guide management decisions.20 Using data from 14 children with severe, complex neurological disabilities (GMFCS Level V or equivalent) with severe sleep-disordered breathing, we report that NIV is a feasible option in this group, with improvement in respiratory parameters in sleep and good acceptance and adherence. While our study did not show a clear clinical benefit in overall hospitalization rates with NIV use, adverse effects were minimal and were able to be managed without discontinuation of therapy.
There are a number of possible benefits of NIV treatment among this population, such as reduced respiratory complications, improved quality of sleep and survival, and slowed functional decline.1 In a cohort of 21 children with CP and OSA, Grychtol and Chan1 reported improved gas exchange and PSG parameters on NIV. Similar improvements in respiratory parameters, sleep, and/or QoL measures have also been reported among neurologically disabled children initiated on NIV in other small observational studies.21,22 Among 10 children with severe neurodevelopmental disabilities, Marcus et al reported improved care-giver sleep quality, caregiver concern, and daytime functioning after NIV initiation.22 In addition, the findings of a 2014 systematic review by Gogou et al23 suggest that treating OSA in children may decrease seizure activity—a potentially important finding for neurologically disabled children with severe epilepsy.
Based on previous reported reduction in hospitalization rates with NIV in children with severe neuromuscular disabilities, we had postulated that our group were likely to show this benefit.24 While overall we did not see a reduction in total hospitalization rates with NIV usage, the number of respiratory, emergency, otolaryngology and gastroenterology admissions declined, with 5 children showing reduction in overall admission rate. Based on these small numbers, we are unable to make any reasonable conclusion regarding the effect of NIV on hospitalization rates in this group. While conjectural, it is possible that a longer follow-up period may have shown a clearer trend.
Intolerance to NIV was previously reported as a significant barrier to successful initiation among severely neurologically impaired children. Grychtol and Chan1 reported a high rate of intolerance to NIV among GMFCS V children and suggested that increased aspiration risk due to severe motor impairment, uncontrolled upper airway secretion, and nocturnal seizure risk make implementation challenging. Sensory intolerance and increased discomfort or secretions due to NIV may exacerbate poor sleep quality and promote nontolerance.1,25 However, among our cohort, the majority (86%) tolerated NIV well, with an average of more than 8 hours of usage per night. This is equivalent to NIV adherence rates among the general pediatric population, with reported rates of 90% and above.1,26 Only 1 child in our cohort discontinued therapy due to nontolerance.
The high burden of care for patients with severe disability has previously been well reported.27 Jacquier and Newman27 reported that cosleeping was common for children with motor disabilities, and that two-thirds of parents provided special care for their disabled children throughout the night, including addressing seizures. For these caretakers, managing NIV treatment may add to this burden and cause further sleep disruption and stress.1 Caretaker burden and poor sleep quality has also been reported to be an important factor resulting in nonacceptance of this modality long term.1 In the current cohort, 1 of the children was unable to initiate home NIV due to caregiver burden, despite finding significant improvement in PSG results while using NIV during titration study. The majority of caregivers reported no difficulty in using NIV and also reported improvement in sleep symptoms. Although we did not include QoL as a primary outcome via a structured questionnaire, this study provides ample evidence to pursue further structured research on QoL.
Ultimately, the appropriateness and feasibility of NIV treatment must be decided by a thorough risk-benefit analysis, individualized to the specific needs of a given patient. Grychtol and Chan1 suggest a pragmatic approach, which considers risks, benefits, and the possible added burden of care. Alternative treatments for SDB include supplemental oxygen, nasopharyngeal prongs, and surgical intervention (including adenotonsillectomy and supraglottoplasty).28 Unfortunately, the outcomes associated with each of these treatment modalities for patients with CP and other neurological disabilities are also unknown. The current study found that a number of the patients in the sample previously had their adenoids and/or tonsils removed, and half had tried supplemental oxygen in the past. However, larger clinical trials are required to compare the outcomes of NIV with other treatment modalities.
Decision-making for SDB is further complicated by the ethical debate surrounding life-sustaining treatment for severely neurologically disabled children.29 While NIV has been shown to improve gas exchange, both in this study and in previous research, the full effects of NIV on QoL among these patients remain unknown.21,22 This presents the potential for NIV to contribute to ongoing life while a patient suffers, without the possibility of long-term survival or improved symptoms. Conversely, if NIV were shown to improve QoL, it may have a role in palliative care for these patients. These issues further emphasize the need for additional research. The most recent consensus statement on ventilatory support in children, endorsed by Australasian Sleep Association and Thoracic society of Australia and New Zealand, has given some guidance for use of NIV in this group, stating that the involvement of the family and all health stakeholders in decision making cannot be over emphasized.30
There were several limitations to our study. The retrospective nature of the study precluded us from having an a priori criteria for NIV initiation. Moreover, the sample size was small, which could be one of the reasons for no significant difference seen in hospitalization rates. We also may have missed visits to non-hospital-based health care providers. Furthermore, we did not have information on the effect on QoL postintervention. This is especially significant given one of the important purported benefits of NIV is an improved QoL for the patient and family. Despite these limitations, this is the first study in children with severe neurological disability reporting accurate adherence data on NIV and exploring possible barriers in adherence in this group.
CONCLUSIONS
It is well recognized that respiratory complications, including sleep-disordered breathing, are a major cause of morbidity and mortality among pediatric patients with severe neurological disabilities such as cerebral palsy. While we report that NIV can be successfully initiated and sustained in most children with severe neurological disability and can lead to improvement in respiratory parameters, its overall benefit on clinical outcomes is unknown. Lack of consensus regarding the indications and role of NIV in this group makes management complex and inconsistent. Further research is needed to understand the effectiveness of NIV in improving quality of life and mitigating symptoms of SDB among this population.
ABBREVIATIONS
- CP
cerebral palsy
- GMFCS
Gross Motor Function Classification System
- IQR
interquartile range
- NIV
noninvasive ventilation
- OSA
obstructive sleep apnea
- PSG
polysomnography
- QoL
quality of life
- SDB
sleep-disordered breathing
- SpO2
oxygen saturation
DISCLOSURE STATEMENT
All authors have seen and approved this manuscript. Work for this study was performed at Children’s Health Queensland Hospital and Health Service. The authors report no conflicts of interest.
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