Long-term home non-invasive positive pressure ventilation in children: Results from a single center in Japan

Background

Non-invasive positive pressure ventilation (NPPV) in children has recently increased worldwide and is used not only for neuromuscular diseases but for various other diseases. However, there have been few observational studies on long-term NPPV in children in Japan.

Continuous positive airway pressure treatment with nasal pillows in obstructive sleep apnea: long-term effectiveness and adherence

BackgroundsMask-related side effects can negatively influence adherence to continuous positive airway pressure (CPAP). Nasal pillows (P) can be an alternative to the standard nasal masks (N), although there are no data about their long-term efficacy. This study aimed to assess long-term effectiveness and adherence to CPAP therapy delivered with nasal pillows in obstructive sleep apnea syndrome (OSAS) patients.MethodsA retrospective observational design involving a series of consecutive CPAP-naïve patients affected by OSAS. After an initial mask fitting session all patients were allowed to choose the type of nasal interface (N or P) they preferred. Outcomes were assessed 5 days after CPAP titration, and after 2 and 12 months. Patients were offered the option of switching to an alternative mask if needed.ResultsData from 144 patients were analyzed. Subjects were predominantly male (76%), middle aged (58.14 ± 12.86), moderately obese (body mass index: 33.89 ± 7.56), and affected by severe OSAS (apnea-hypopnea index: 47.60 ± 21.31). A total of 102 patients (70.8%) chose P, and 42 (29.2%) chose N. Clinical and polygraphic features, and CPAP pressure levels were similar in P and N groups, both at baseline and at 12 months. A good adherence to treatment was observed in both groups (P, 5.5 ± 1.8 h; N, 5.3 ± 1.5 h). Seventy-six patients (53%) reported at least one side effect during the whole study period, without statistically significant between-group differences. Nostril pain was the most frequent side effect in P.ConclusionsNasal pillows showed equal long-term effectiveness and objective adherence as standard nasal masks.     

Continuous positive airway pressure improves the quality of sleep and oxygenation in obstructive sleep apnea syndrome

We tested the effects of continuous positive airway pressure (CPAP) in 8 patients with obstructive sleep apnea syndrome (OSAS). The comparison of a nocturnal polygraphic study performed during spontaneous breathing with a study during CPAP administration performed the following night showed a significant reduction in stage 1 and increase in REM, the abolition of obstructive apneas and a significant increase in mean oxyhemoglobin saturation. Most patients reported marked relief of symptoms after the first night of treatment. However 3 patients, though confirming the improvement refused further CPAP. We conclude that CPAP is an effective measure for prevention of apneas in OSAS and that in compliant patients it may be regarded as a short-term measure when a permanent correction of the causes is planned, or as a long-term treatment when the latter is not feasible.

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Sommario Abbiamo provato gli effetti dell’applicazione di ventilazione a pressione positiva continua (CPAP) su otto pazienti affetti da sindrome dell’apnea ostruttiva nel sonno (OSAS). Il paragone tra uno studio poligrafico notturno eseguito durante respirazione spontanea, ed uno eseguito la notte successiva durante applicazione di CPAP ha mostrato significativa riduzione dello stadio 1 ed aumento del REM, la scomparsa delle apnee ostruttive ed un aumento significativo della saturazione ossiemoglobinica media. Inoltre la maggioranza dei pazienti ha notato una sensibile attenuazione della sintomatologia già dopo la prima notte di trattamento. Tre pazienti però pur confermando il miglioramento non hanno accettato di proseguire la terapia nelle notti successive. Si può concludere che la CPAP è un’efficace forma di prevenzione delle apnee nell’OSAS e che nei pazienti adattabili può essere considerata per una terapia di breve durata se può essere previsto un intervento correttivo della patologia di base o come una terapia di lunga durata negli altri casi.

     

Longitudinal assessment of noninvasive positive pressure ventilation adjustments in ALS patients

The absence of data guiding optimal titration of noninvasive positive pressure ventilation (NIPPV) over time in ALS patients may contribute to the under-prescribing of NIPPV. We conducted a retrospective, single-center, chart review assessment of NIPPV pressure settings used for symptomatic treatment of ALS patients to determine NIPPV adjustments, and to compare survival between those who were tolerant and intolerant to NIPPV. All subjects were started on nocturnal NIPPV at 8 and 3 cm H2O inspiratory and expiratory pressure, respectively. Of the 18 tolerant subjects identified, 4 (22%) had no NIPPV pressure changes before death; 8 (44%), 1 change; 4 (22%), 2 changes; 1 (6%), 3 changes; and 1 (6%), 5 changes. Most pressure changes occurred during the first year of NIPPV initiation. The maximum pressure needed for comfort by any patient in this study was 19/5 cm H2O, while 4 (22%) found the original 8/3 cm H2O settings to be sufficient until death. Subjects in the tolerant group had better survival, when adjusting for age and site of symptom onset (bulbar versus limb), with a hazard ratio of 0.23 [95% confidence interval: 0.10, 0.54]. The current data suggest that ALS patients who are tolerant to NIPPV typically need at least one upward change in pressure settings. Tolerance to relatively low NIPPV inspiratory pressures is associated with improved survival.     

Home continuous positive airway pressure for cardiopulmonary indications in infants and children

ObjectivesA number of reports exist regarding the use of continuous positive airway pressure (CPAP) to manage obstructive sleep apnoea (OSA) in children, which we term ‘conventional CPAP’. In contrast, there are few reports of home CPAP use for other indications, which we have grouped under the term ‘cardiopulmonary’. The aims of this study were to (1) document cardiopulmonary indications for CPAP use in a cohort of infants and children, and (2) evaluate its effectiveness in this group.MethodsHospital records were reviewed for 645 patients who were commenced on long-term CPAP over a 10-year period at a single-tertiary hospital (Children's Hospital at Westmead). This study evaluated the group where the primary indication for CPAP was not OSA (‘cardiopulmonary CPAP’). Data evaluated included: demographics, diagnoses, indications for CPAP, hours of use (compliance) and sleep study results at baseline and on CPAP.ResultsOf 645 children, 148 (23%) used home CPAP for cardiopulmonary indications; and 130 (87.8%) of these were included. For this group, mean age at CPAP initiation was 18.6 ± 33.6 months (range one week to 16.8 years). Cardiopulmonary indications for CPAP use included: primary airway diseases 65 (50%), chronic lung diseases 33 (25.4%), congenital heart disease (CHD) 20 (15.4%), and both CHD and airway malacia 12 (9.2%). All sleep study variables improved on CPAP relative to the diagnostic sleep study (p < 0.0001), including reduced respiratory rate with CPAP use (p < 0.0001). CPAP was well tolerated (>4 h/night).ConclusionsInterstitial lung diseases and other cardiorespiratory disorders, often of congenital origin, can be effectively treated with home CPAP whether they are associated with OSA or not. Sleep studies demonstrated improved gas exchange, sleep and reduced work of breathing with CPAP use.     

早期无创正压通气治疗肌萎缩侧索硬化患者疗效的临床评估

目的 探讨用力肺活量(forced vital capacity,FVC)＜75%的肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)患者早期接受无创正压通气(noninvasive positive pressure ventilation,NPPV)治疗1年后的疗效评估.方法测定60例肌萎缩侧索硬化症(amyothophic lateral sclerosis,ALS)患者肺通气功能,以用力肺活量(FVC%)是否低于75%和是否接受NPPV治疗为标准分3组,其中FVC%＜75%且配合NPPV治疗14例,作为第1组;FVC%＜75%且不能配合NPPV治疗18例,作为第2组;FVC%＞75%为第3组,即阳性对照组,共28例.结果 第1组(FVC%＜75%且配合NPPV治疗)ALS患者1年内生存率与第2组(FVC%＜75%且不能配合NPPV治疗)ALS患者比较有统计学差异(P＜0.05),与第3组(阳性对照组)无统计学差异(P＞0.05).第1组患者FVC%下降的幅度以及ALSFRS-R评分降低的幅度较第2组患者缓慢,差异具有统计学意义(P＜0.05).结论 在ALS患者FVC＜75%时引入NPPV治疗,可延缓疾病的进展,延长患者的生存时间.     

Head circumference and chronic positive pressure ventilation in children: a pilot study

Methods We reviewed the cases of 11 children <12 years of age with complex medical conditions and respiratory failure requiring chronic positive pressure ventilation (CPPV). We conducted a within-group comparison of average head circumference (HC) percentiles with each childs age-expected 50th percentile value and a between-groups comparison with children with no history of ventilation. We examined the relationship between HC, peak levels of positive inspiratory pressure (PIP), and length of time on CPPV.Results We found that children on CPPV had an average HC value at the 71st percentile, significantly greater (p=0.009) than the age-expected 50th percentile; that children on CPPV had a greater (p=0.003) average HC percentile compared with children with complex medical conditions not on CPPV; and that peak levels of PIP had a moderately strong correlation to HC (r=0.689, p=0.019).Conclusion We conclude that children on CPPV have larger than expected HC and HC appears related to the peak level of PIP. Research to further investigate the relationship between HC and CPPV in children appears to be warranted.The results of this study were presented at the European Respiratory Society 13th Annual Congress, 27 September–1 October 2003, Vienna, AustriaThis study was approved by the Institutional Review Board at the Franciscan Hospital for Children, Boston, MA, USA     

Continuous positive airway pressure treatment and anxiety in adults with coronary artery disease and nonsleepy obstructive sleep apnea in the RICCADSA trial

BackgroundAnxiety and obstructive sleep apnea (OSA) coexist among adults with coronary artery disease (CAD) following revascularization. Continuous positive airway pressure (CPAP) is the first line treatment of OSA patients with daytime sleepiness. The current study evaluated the effect of CPAP on anxiety in CAD patients with nonsleepy OSA.MethodsTwo hundred forty-four revascularized CAD patients with nonsleepy OSA (apnea-hypopnea index ≥15/h, Epworth Sleepiness Scale score <10) were randomly assigned to CPAP or no-CPAP between 2005 and 2010. Zung Self-rating Anxiety Scale (SAS) was administered at baseline and after 3 and 12 months with higher scores suggesting more anxiety.ResultsA total of 208 patients with complete SAS scores at baseline and 12-month follow-up were included (CPAP, n = 103; no-CPAP, n = 105). In the intention-to-treat analysis, CPAP had no significant effect on the SAS scores. On-treatment analysis revealed a significant increase in the median of delta SAS score (+3.75) after three months among the participants using the device 2.8 h/day or more while there was a decline in the median of delta SAS score (−1.25) in the non-adherent or no-CPAP group (p = 0.031). The increase in the SAS score (+1.25) in the adherent group, and the decline (−1.25 points) in the non-adherent/no-CPAP group remained significant after one year (p = 0.011). Baseline SAS score predicted non-adherence [adjusted odds ratio 1.11; 95% confidence interval (CI) 1.04–1.18; p = 0.003], and there was an association between the increase in the SAS scores and accumulated CPAP hours/day [standardized β = 0.144 (95% CI 0.005–0.695), p = 0.047].ConclusionOur results suggest that anxiety should be considered in the management of CAD patients with nonsleepy OSA following revascularization.Clinical trial registrationNCT00519597.     

Non-invasive positive pressure ventilation for laryngeal contraction disorder during sleep in multiple system atrophy

We examined the usefulness of non-invasive positive pressure ventilation (NPPV) in the management of nocturnal laryngeal stridor associated with vocal cord dysfunction in five cases of multiple system atrophy (MSA). First, the patients were investigated during sleep induced by a minimal dose of propofol. Laryngoscopy showed paradoxical vocal cord movement resulting in inspiratory stridor. Electromyographic (EMG) study revealed synchronized bursts in the thyroarytenoid muscles and diaphragm in every inspiratory phase whenever the stridor emerged. NPPV was initiated after paradoxical movement was recognized with laryngoscopy. The NPPV mask was equipped with an additional channel for laryngoscopic monitoring. The optimal pressure for treatment was determined according to laryngoscopic and EMG findings. Next, NPPV was applied to natural sleep using the conditions determined in propofol-induced sleep. In all cases, NPPV eliminated nocturnal stridor and oxygen desaturation during natural sleep. Laryngoscopic observation during induced sleep is recommended as a useful procedure to titrate the optimal pressure for NPPV therapy. Since central hypoventilation progresses in the course of MSA, the choice of NPPV rather than continuous positive airway pressure should be encouraged to treat laryngeal contraction disorder associated with MSA.     

Predictors of adherence to positive airway pressure therapy in children: a systematic review and meta-analysis

BackgroundWhile positive airway pressure (PAP) is effective for treating sleep-disordered breathing (SDB) in children, adherence is poor. Studies evaluating predictors of PAP adherence have inconsistent findings, and no rigorous reviews have been conducted. This systematic review aims to summarize the literature on predictors of PAP therapy adherence in children.MethodsStudies evaluating baseline predictors of PAP therapy adherence in children (≤20 years) with SDB were included. We searched MEDLINE, Embase, CENTRAL, CINAHL, Clinicaltrials.gov, and the last four years of conference abstracts. Results were described narratively, with random-effects meta-analyses performed where feasible. Risk of bias and confidence in the evidence were assessed.ResultsWe identified 50 factors evaluated across 28 studies (21 full text articles, seven abstracts). The highest rates of PAP therapy adherence were most consistently found with female sex, younger age, Caucasian race, higher maternal education, greater baseline apnea-hypopnea index (AHI), and presence of developmental delay. Pooled estimates included odds ratios of 1.48 (95%CI: 0.75–2.93) favoring female sex, 1.26 (95%CI: 0.68–2.36) favoring Caucasian race, and a mean difference in AHI of 4.32 (95%CI: −0.61-9.26) events/hour between adherent and non-adherent groups. There was low quality evidence to suggest that psychosocial factors like health cognitions and family environment may predict adherence.ConclusionIn this novel systematic review, we identified several factors associated with increased odds of PAP therapy adherence in children. These findings may help guide clinicians to identify and support children less likely to adhere to PAP therapy and should be considered when developing interventions to improve adherence.     

Early effects of continuous positive airway pressure in a rodent model of allergic rhinitis

BackgroundContinuous positive airway pressure (CPAP) is the most commonly used treatment in obstructive sleep apnea. In a previous rat model study, we demonstrated that nasal CPAP induces early rhinitis expressed by nasal neutrophil extravasation. Here we hypothesized that nasal CPAP would worsen nasal inflammation on a previously inflamed mucosa. The objective of this study was to evaluate the early nasal CPAP effects of allergic rhinitis (AR) in a rodent model.MethodsTwenty Sprague-Dawley rats were sensitized with intraperitoneal ovalbumin (OVA). Nasal inflammation was induced by the administration of intranasal OVA during consecutive days. The same procedure was performed in 20 control rats treated with saline solution. The allergic (AR) and non-allergic (NAR) rats were then randomized to nasal CPAP at 10 cm H₂O for five hours or to sham CPAP. The degree of nasal inflammation was assessed by evaluating the percentage of neutrophils, eosinophils, basophils, and lymphocytes in the nasal mucosa. An unpaired Mann−Whitney test was used to analyze differences between groups.ResultsThe greatest inflammation was observed in the group of AR without CPAP (1.24% ± 0.94%), followed by NAR with CPAP (0.64% ± 0.30%), AR with CPAP (0.64% ± 0.40%), and NAR without CPAP (0.21% ± 0.29%).ConclusionsAdministration of nasal CPAP or allergy sensitization can produce, individually, neutrophil extravasation on the nasal mucosa of a rat model. The application of both stimuli is not responsible for increased inflammation. Therefore, this study suggests that rhinitis is not a major limitation for CPAP administration.     

Monitoring noninvasive ventilation in neuromuscular patients: feasibility of unattended home polysomnography and reliability of sleep diaries

BackgroundIn-hospital polysomnography (PSG) often is performed to monitor neuromuscular patients under noninvasive ventilation (NIV), but success of home PSG has not been established for that purpose. Reliability of sleep diaries in neuromuscular patients is unknown. The aims of our study were to evaluate feasibility, quality, and acceptability of unattended home PSG, as well as the reliability of sleep diaries in neuromuscular patients on long-term NIV.MethodsFifty-two neuromuscular patients underwent unattended home or hospital PSG during NIV. Patients were questioned about their sleep during the PSG and their attitudes towards the procedure.ResultsOne home and one hospital PSG were scored as failure or low quality due to prolonged signal loss or sleep duration of <3 h. Objective and subjective sleep duration and efficiency often showed large differences. Subjective awakenings reflected objective awakenings lasting for >4 min in 86.5% patients. Preference for home PSG was expressed by 82% subjects.ConclusionsIn neuromuscular patients under NIV unattended home PSG is feasible and preferred, with a low failure rate. The degree of reliability of different parameters of subjective sleep assessment should be considered when used as a complement of nocturnal cardiorespiratory recordings.     

Obstructive sleep apnea: effect of comorbidities and positive airway pressure on all-cause mortality

ObjectiveMost studies have used cardiovascular and cerebrovascular disease (CVD) end-points to measure the effect of continuous positive airway pressure (CPAP), but pre-diagnostic morbidities involve a range of comorbidities that may influence the consequences of obstructive sleep apnea (OSA). We therefore aimed to evaluate all-cause total mortality after a diagnosis of OSA, the effect of CPAP in women and men, and the potential effect of major comorbidities.MethodsWe used national registry data as a historical cohort and included 22,135 OSA patients aged 20 years or more with comorbidity data three years before diagnosis and follow-up morbidity and mortality over a period of 17 years.ResultsA total of 8129 (37%) accepted CPAP for more than six months; 14,006 (63%) were CPAP non-user/non-compliant. Those treated with CPAP tended to have higher mortality rates. Patients treated with CPAP were more likely to be male, elderly, suffer from diabetes, and present with more cardiovascular diseases than those not treated with CPAP. After a diagnosis of OSA, more patients in the CPAP-treated group developed cardiovascular complications. Female gender was associated with lower mortality, whereas age, diabetes type 2, and hypertension prior to OSA diagnosis were associated with negative effects on outcome. After an OSA diagnosis, male gender, age, diabetes (types 1 and 2), hypertension, and heart failure were all associated with greater mortality. CPAP treatment had a positive effect in middle-aged and elderly people, whereas CPAP in females had no effect on all-cause mortality.ConclusionsCPAP-treated patients present more comorbidities before and after diagnosis compared with non-treated/non-compliant patients, which explains the higher mortality in this group. CPAP treatment is associated with lower mortality rates in middle-aged and elderly (aged 60+ years) males, but only after adjustment for multiple comorbidities. No effect of CPAP treatment on all-cause mortality in female OSA patients was found. Males with OSA, older than 40 years, with comorbidities and a low educational level presented a particularly high mortality risk.     

The effect of noninvasive positive pressure ventilation in children with severe motor and intellectual disabilities with respiratory insufficiency

We investigated the clinical course of 20 children (persons) with severe motor and intellectual disabilities (SMID) who were treated with noninvasive positive pressure ventilation (NPPV) for respiratory insufficiency. NPPV was effective in 10 of 11 patients treated for acute respiratory failure, and in 7 of 9 patients treated for chronic respiratory failure. Twelve patients were treated with NPPV for more than one year. There were no complications associated with NPPV in any of the patients. NPPV improved ventilation impairment soon after ventilation was started, and avoided the need for the endtracheal intubation by adjusting airway management and the choice of mask in all but one of the patients with acute respiratory failure. NPPV in combination with wearing a chin strap was highly effective in patients with open state or upper airway obstruction. Five patients were successfully weaned off the ventilator soon after recovery from acute respiratory failure using NPPV, whereas 5 patients who continued NPPV during the chronic phase after recovery did not experience recurrent episodes of acute respiratory failure. We conclude that NPPV may be an effective treatment for SMID with respiratory insufficiency.     

Continuous positive airway pressure for children: a discussion on how to maximize compliance

As pediatric sleep facilities and resources expand, increasing numbers of children with sleep-disordered breathing requiring continuous positive airway pressure (CPAP) treatment are being identified. Despite extensive expertise in treating adults with CPAP, many centres have little experience using CPAP in the pediatric population. The successful initiation and continued effective treatment with CPAP requires a unique and specialized approach to the pediatric patient and their family. Nearly, half of children needing CPAP will be uncooperative upon initial exposure to this unusual treatment. This review aims to outline an approach to the successful initiation of CPAP treatment in children including some trouble-shooting strategies to maximize initial and ongoing compliance with prescribed therapy.     

A randomised controlled trial on the effect of mask choice on residual respiratory events with continuous positive airway pressure treatment

IntroductionIt has been found that mask style can affect the amount of continuous positive airway pressure (CPAP) required to reduce an apnoea/hyponoea index (AHI) to <5/h on a titration study. However, it was not previously known whether switching from one CPAP mask style to another post titration could affect the residual AHI with CPAP. The purpose of this study was to investigate the differences in residual AHI with CPAP treatment between oronasal and nasal masks.MethodsTwenty-one subjects (age mean (M) = 62.9, body mass index (BMI) M = 29.6 kg/m²) were randomised (14 subjects completed the protocol) to undergo an in-laboratory CPAP titration with either a nasal mask or an oronasal mask. Subjects were then assigned this mask for 3 weeks of at-home CPAP use with the optimal treatment pressure determined on the laboratory study (CPAP M = 8.4 cm of H₂O). At the end of this 3-week period, data were collected from the CPAP machine and the subject was given the other mask to use with the same CPAP settings for the next 3 weeks at home (if the nasal mask was given initially, the oronasal one was given later and vice versa). On completion of the second 3-week period, data on residual AHI were again collected and compared with the first 3-week period on CPAP.ResultsA Wilcoxon Signed-Rank Test (two-tailed) revealed that residual AHI with CPAP treatment was significantly higher with the oronasal compared with the nasal mask (z = −3.296, p < 0.001). All 14 subjects had a higher residual AHI with the oronasal versus nasal mask, and 50% of the subjects had a residual AHI >10/h in the oronasal mask condition, even though all of these subjects were titrated to an AHI of <5/h in the laboratory.ConclusionA higher residual AHI was seen in all patients with the use of an oronasal mask compared with a nasal mask. Switching to an oronasal mask post titration results in an increase in residual AHI with CPAP treatment, and pressure adjustment may be warranted.