Effect of long term noninvasive ventilation in children on parent's quality of life.

OBJECTIVE: Improving or maintaining the quality of life of the family of children treated with long term continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV) is a major concern; but studies are scarce. The aim of the study was to evaluate the impact of long term CPAP or NIV in children on anxiety, depression, quality of sleep, and quality of life of their parents. METHODS: Validated questionnaires evaluating anxiety and depression (hospital anxiety and depression scale), sleep quality (Pittsburgh sleep quality index), daytime sleepiness (Epworth sleepiness scale), and parents' quality of life (PedsQL family impact module) were completed by parents of children who were started on CPAP/NIV before (M0) and after 6-9 months (M6) of treatment. RESULTS: The questionnaires of 36 parents (30 mothers, 6 fathers) of 31 children were analyzed. For the entire group, no significant change was observed in anxiety, depression, sleep quality, daytime sleepiness, and quality of life between M0 and M6. When analyzing questionnaire class changes between M0 and M6: anxiety was relieved in 23% of parents and worsened in 29%, depression was relieved in 14% and worsened in 20%, sleep quality improved in 43% and worsened in 27%, sleepiness improved in 26% and worsened in 17%, with no change in the other parents. CONCLUSION: Long term CPAP/NIV in children had no significant effect on parents' anxiety, depression, sleep quality, and quality of life.

Quality of poly(somno)graphy recordings in children.

The aim of the study was to assess the scorability of the signals of four poly(somno)graphy devices and transcutaneous carbon dioxide tracings (PtcCO2 ) of one device in children. The presence (0%, < 25%, 25%-50%, 50%-75%, 75%-99%, 100% of recording time) and quality (bad, average, good) of the signal of each sensor were analysed. During a 5-month period, 364 poly(somno)graphies were performed in 12 different hospital units. Forty-one children had poor/bad cooperation, and 13 severe behaviour disorders. Seventy-one and 293 poly(somno)graphies were performed in children aged ≤ 2 and > 2 years, respectively; nine poly(somno)graphies failed. For the four poly(somno)graphy devices, the signal was present during 99% of recording time for the electroencephalogram, 99% for thoracic belt, 97% for abdominal belt, 97% for body position, 95% for the microphone, 92% for pulse oximetry, 87% for tracheal sound, 71% for oronasal thermistor, 52% (41% for ≤ 2 years, 55% for > 2 years old) for nasal pressure and 86% for PtcCO2 . The signal was of good quality in 98% of poly(somno)graphies for body position, 96% for microphone, 96% for thoracic belt, 95% for pulse oximetry, 91% for abdominal belt, 91% for tracheal sound, 82% for oronasal thermistor, 78% for electroencephalogram, 73% for nasal pressure and 46% of PtcCO2 recordings. The scorability was comparable between devices. Nasal pressure and oronasal thermistor had the lowest scorability, especially in children aged ≤ 2 years. This underlines the necessity of the development or improvement of alternative, ideally face-free, sensors, or miniaturized devices adapted for infants and children.

Tracheal sounds for the scoring of sleep respiratory events in children.

STUDY OBJECTIVES: Oronasal thermistor and nasal cannula are recommended for the scoring of respiratory events (RE) but these sensors are poorly tolerated in children. The aim of the study was to evaluate tracheal sounds (TS) and suprasternal pressure (SSP) for the scoring of RE during sleep in children. METHODS: We compared the detection and characterization of RE by AASM-recommended sensors ("AASM" scoring), with the detection and characterization of RE by the combination of TS and SSP with respiratory inductance plethysmography-sum (TS-RIP scoring), and TS and SSP only (TS scoring). RESULTS: The recordings of 17 patients were analyzed. The TS, SSP, and RIP flow signals were present during 95%, 95%, and 99% of the validated recording time, respectively, as compared to 79% and 86% for nasal cannula and oronasal thermistor. A total of 1,456 RE were scored with the "AASM" scoring, 1,335 with the TS-RIP scoring, and 1,311 with the TS scoring. Sensitivity for apnea and hypopnea detection was 88% and 84% for the TS-RIP scoring, and 86% and 77% for the TS scoring. For apnea characterization, the TS-RIP scoring sensitivities and specificities were 97% and 100%, 76% and 98%, and 95% and 97%, for obstructive, mixed, and central apnea, respectively. For the TS scoring, they were 95% and 100%, 95% and 97%, and 91% and 97%, respectively. CONCLUSIONS: TS and SSP + RIP-sum has a good sensitivity and specificity for the detection and characterization of apnea and hypopnea in children. TS and SSP alone have good sensitivity and specificity for apnea detection and characterization but lower sensitivity for hypopnea detection.

Polysomnographic findings in Rett syndrome.

INTRODUCTION: Rett syndrome (RS) is a severe neurodevelopment disorder associated with abnormal breathing during wakefulness and disturbed nocturnal behaviour. Breathing abnormalities during daytime have been extensively reported but polysomnographic (PSG) findings have been poorly studied. MATERIALS AND METHODS: Consecutive patients with RS carrying distinct mutations in MECP2 gene, who underwent a PSG between October 2014 and January 2018, were included in the study. Clinical and PSG data were collected. RESULTS: Seventeen RS girls, mean age 9.5 ± 2.8 years, were included in the study. Mean total sleep time was 366 ± 102 min. Mean sleep efficiency was reduced (66 ± 19%) with only 3 girls presenting a sleep efficiency above 80%. Wake after sleep onset was increased (33 ± 20%) with an arousal index of 7 ± 6 events/hour. Sleep stages were altered with a normal N1 (2 ± 3%), a decreased N2 (34 ± 20%), an increase of N3 (51 ± 23%) and a decrease of REM sleep (12 ± 9%). Mean apnea hypopnea index (AHI) was increased at 19 ± 37 events/hour, with a predominance of obstructive events. Thirteen patients had an AHI > 1.5 event/hour. Four patients had an obstructive AHI >10 events/hour with one patient having associated tonsillar hypertrophy. Two patients had predominant severe central apneas (central AHI 53 and 132 events/hour) which resolved with noninvasive ventilation and nocturnal oxygen therapy respectively. CONCLUSION: Girls with RS have poor sleep quality with alterations in slow wave and REM sleep stages. Obstructive respiratory events are uncommon in patients without adenotonsillar hypertrophy. Central respiratory events are rare. Longitudinal studies should help understanding the natural history of sleep disturbances in RS and their relationship with the neurocognitive decline.

Parent-child co-sleeping in children with co-morbid conditions and sleep-disordered breathing.

PURPOSE: Co-sleeping is common in children with co-morbid conditions. The aim of the study was to analyze the prevalence and determinants of parent-child co-sleeping in children with co-morbid conditions and sleep-disordered breathing and the impact on parental sleep. METHODS: Parents of consecutive children undergoing a sleep study filled in a questionnaire on co-sleeping. RESULTS: The parents of 166 children (80 boys, median age 5.7 years (0.5-21) participated in this study. The most common co-morbid conditions of the children were Down syndrome (17%), achondroplasia (11%), and Chiari malformation (8%). The prevalence of parent-child co-sleeping was 46%. Reasons for co-sleeping were mainly reactive and included child's demand (39%), crying (19%), nightmares (13%), medical reason (34%), parental reassuring or comforting (27%), and/or over-crowding (21%). Sixty-eight percent of parents reported that co-sleeping improved their sleep quality because of reassurance/comforting (67%), reduced nocturnal awakening (23%), and child supervision (44%). Forty percent of parents reported that co-sleeping decreased their sleep quality because of nocturnal awakenings or early wake up, or difficulties initiating sleep (by 77% and 52% of parents, respectively), whereas both positive and negative associations were reported by 29% of the parents. Co-sleeping was more common with children < 2 years of age as compared to older children (p < 0.001). CONCLUSIONS: Parent-child co-sleeping is common in children with co-morbid conditions and sleep-disordered breathing. Co-sleeping was mainly reactive and had both positive and negative associations with parental sleep quality. Co-sleeping should be discussed on an individual basis with the parents in order to improve the sleep quality of the family.

Parents of children referred to a sleep laboratory for disordered breathing reported anxiety, daytime sleepiness and poor sleep quality.

AIM: We evaluated the impact that having a child with sleep-disordered breathing had on their parents, including their own sleep quality. METHODS: Questionnaires were completed by 96 parents of 86 children referred for a sleep study or control of continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV) at the sleep laboratory of the Necker Hospital, Paris, France, between October 2015 and January 2016. The questionnaires evaluated anxiety and depression, family functioning, the parents' quality of life, daytime sleepiness and sleep quality. RESULTS: The children had a mean age of seven ±five years and most of the responses (79%) came from their mothers. These showed that 26% of parents showed moderate-to-severe anxiety, 8% moderate-to-severe depression, 6% complex family cohesion, 59% moderate-to-severe daytime sleepiness and 54% poor sleep quality. Anxiety was higher in mothers than in fathers (p < 0.001). The questionnaire scores did not differ according to the child's age, the results of the sleep studies or the CPAP or NIV treatment. The symptoms seem to be more commonly related to the child's underlying disease than their sleep-disordered breathing. CONCLUSION: The parents of children referred to a sleep laboratory reported frequent anxiety, daytime sleepiness and poor sleep quality.

Can the analysis of built-in software of CPAP devices replace polygraphy in children?

OBJECTIVES: Polysomnography (PSG) is the gold standard for the scoring of residual respiratory events during continuous positive airway pressure (CPAP). Studies comparing PSG scoring with automatic scoring by the built-in software of CPAP devices have reported acceptable agreements except for the hypopnea index (HI) in adult patients, but no study has yet been conducted in children. The aim of the present study was to compare the automatic scoring by CPAP device and manual scoring using the software tracings of the CPAP device integrating pulse oximetry (SpO2) with in-lab polygraphy (PG). METHODS: Consecutive clinically stable children treated with constant CPAP (ResMed) for at least one month and scheduled for a nocturnal PG were recruited. A pulse oximeter was connected to the CPAP device. The PG apnea-hypopnea index (AHIPG), scored according to modified AASM guidelines, was compared with the automatic AHI reported by the CPAP device (AHIA CPAP) and the manual scoring of the AHI on the CPAP software (AHIM CPAP). RESULTS: Fifteen children (1.5-18.6 years) were included. Mean residual AHIPG was 0.9 ± 1.2/hour (0.0-4.6/hour) vs. AHIA CPAP of 3.6 ± 3.6/hour (0.5-14.7/hour) (p < 0.001), and AHIM CPAP of 1.2 ± 1.6/hour (0.0-5.1/hour) (p = 0.01). Correlation between AHIPG and AHIA CPAP was good (r = 0.667; p = 0.007), and improved when considering AHIM CPAP (r = 0.933; p < 0.001). Strong correlations were also observed between the PG apnea index (AI) and HI, and the manually scored AI and HI on CPAP, respectively. CONCLUSIONS: Manual scoring of respiratory events on the built-in software tracings of CPAP devices integrating SpO2 signal may be helpful. These results have to be confirmed in patients with higher AHI.

Sleep study as a diagnostic tool for unexplained respiratory failure in infants hospitalized in the PICU.

PURPOSE: The aim of the study was to analyze the diagnostic and therapeutic value of a polygraphy (PG) in infants hospitalized for unexplained respiratory failure or life-threatening events in the PICU. MATERIAL AND METHODS: The PG of 13 infants (4 girls), mean age 6.8±7.7months, were analyzed. RESULTS: Eight infants were admitted for unexplained respiratory failure and 5 for life-threatening events. PG showed features suggestive of respiratory muscle weakness in 5 infants whose final diagnoses were nemaline rod myopathy (n=2), congenital myasthenia (n=2), and diaphragmatic dysfunction (n=1). Four of these patients were successfully treated with noninvasive ventilation (NIV). PG was suggestive of brainstem dysfunction in 4 infants; 2 were treated successfully with NIV and another with caffeine. PG showed obstructive sleep apnea in 3 infants; 2 were treated successfully with NIV and one patient was lost during follow up. A typical pattern of congenital central hypoventilation syndrome was observed in the last patient who was treated successfully with invasive ventilation. One patient with diaphragmatic dysfunction and one with brain stem dysfunction died. CONCLUSIONS: PG may assist the diagnosis and guide the management of unexplained respiratory failure or life-threatening events in infants hospitalized in the PICU.

A comparison of pulse oximetry and cerebral oxygenation in children with severe sleep apnea-hypopnea syndrome: a pilot study.

Near infrared spectroscopy (NIRS) has been used to assess the impact of obstructive sleep apnea-hypopnea syndrome (OSAHS) on cerebral oxygenation. However, the relationship between the variations in the cerebral tissue oxygen saturation (ΔTOI) and pulse oximetry (ΔSpO2 ) has not been assessed in children with OSAHS. Consecutive clinically stable children with severe OSAHS [apnea-hypopnea index (AHI) >15 events h-1 ] diagnosed during a night-time polygraphy with simultaneous recording of cerebral oxygenation with NIRS (NIRO-200NX, Hamamatsu Photonics KK) were included between September 2015 and June 2016. Maximal ΔSpO2 (SpO2 drop from the value preceding desaturation to nadir) and concomitant variations in transcutaneous carbon dioxide (ΔPtcCO2 ), maximal ΔTOI and maximal variations in cerebral oxygenated (O2 Hb) and deoxygenated (HHb) haemoglobin were reported. The relationships between ΔSpO2 , ΔPtcCO2 and ΔTOI, ΔO2 Hb and ΔHHb were investigated. The data from five children (three boys, aged 9.6 ± 6.7 years, AHI 16-91 events h-1 ) were analysed. Strong correlations were found between ΔSpO2 and ΔTOI (r = 0.887, P < 0.001), but also with ΔO2 Hb and ΔHHb with a particular pattern in the youngest child with a dark skin pigmentation. Mean ΔSpO2 was 20 ± 17% and mean ΔTOI was 8 ± 7%. Maximal ΔSpO2 of approximately 70% were coupled with ΔTOI of no more than 35%. ΔPtcCO2 correlated only weakly with the cerebral oxygenation indexes. This pilot study shows a strong relationship between pulse oximetry and cerebral oxygenation in children with OSAHS, with lower changes in TOI compared to SpO2 . Future studies should address the clinical impact of respiratory events on cerebral oxygenation and its consequences.

Obstructive sleep apnea in Down syndrome: Benefits of surgery and noninvasive respiratory support.

Children with Down syndrome are at increased risk of obstructive sleep apnea (OSA). The aim of the study was to describe the management of OSA in a large cohort of children with Down syndrome. A retrospective analysis of sleep studies and consequent management was performed for all consecutive Down syndrome patients evaluated between September 2013 and April 2016. The data of 57 patients were analyzed: 51/53 had an interpretable overnight polygraphy and 4 the recording of nocturnal gas exchange. Mean age at baseline sleep study was 6.2 ± 5.9 years. Eighteen patients (32%) had prior upper airway surgery. Mean apnea-hypopnea index (AHI) was 14 ± 16 events/hr with 41 of the 51 (80%) patients having OSA with an AHI >1 event/hr and 20 patients (39%) having an AHI ≥10 events/hr. Consequently, eight patients (14%) had upper airway surgery. OSA improved in all patients except two who needed noninvasive respiratory support. Nineteen (33%) patients required noninvasive respiratory support. Mean age at noninvasive respiratory support initiation was 7 ± 7 years. On 11 patients with objective adherence data available, mean compliance at 2 ± 1 years of treatment was excellent with an average use per night of 8 hr46 ± 3 hr59 and 9 patients using the noninvasive respiratory support >4 hr/night. Noninvasive respiratory support was associated with an improvement of nocturnal gas exchange. The prevalence of OSA is high in Down syndrome. Upper airway surgery is not always able to correct OSA. Noninvasive respiratory support represents then an effective treatment for OSA and good compliance may be achieved in a majority of patients.

Sleep-disordered breathing and its management in children with achondroplasia.

Sleep-disordered breathing is a common feature in children with achondroplasia. The aim of our study was to review the poly(somno)graphic (P(S)G) findings and consequent treatments in children with achondroplasia followed in the national reference center for skeletal dysplasia. A retrospective review of the clinical charts and P(S)G of 43 consecutive children (mean age 3.9 ± 3.5 years) with achondroplasia seen over a period of 2 years was performed. Twenty four (59%) children had obstructive sleep apnea (OSA). Thirteen children had an obstructive apnea-hypopnea index (OAHI) < 5/hr, four had an OAHI between 5 and 10/hr, and seven had an OAHI ≥ 10/hr. Ten of the 15 children who had previous upper airway surgery still had an abnormal P(S)G. All the patients with an AHI ≥ 10/hr were under 7 years of age and none had a prior tonsillectomy. The children who underwent adeno-tonsillectomy, coupled in most cases with turbinectomy, were significantly older (mean age 7.5 ± 3.5 vs. 3.5 ± 1.7 years old, P = 0.015) and had significantly better P(S)G results than those who underwent only adeno-turbinectomy. No correlation was observed between the mean AHI value at the baseline P(S)G and the type of academic course (standard, supported or specialized). In conclusion, OSA is common in children with achondroplasia. The observation of a reduced prevalence of OSA after (adeno-)tonsillectomy is in favor of this type of surgery when possible.