A qualitative exploration of parental caregivers' experience caring for children who have survived medulloblastoma.

BACKGROUND: Approximately 70% of children diagnosed with a medulloblastoma will become long-term survivors. Medulloblastoma therapy frequently causes long-term morbidities in survivors, which places a considerable burden on parental caregivers. We aimed to explore the experience of parental caregivers caring for medulloblastoma survivors. METHODS: We conducted a qualitative study using grounded theory thematic analysis. We used semi-structured parental caregiver interviews to explore family experiences, social circumstances, and family-reported impact within families of children who had survived medulloblastoma. Parental caregivers were recruited from specialized survivor clinics at two large quaternary centers in Toronto, Canada. RESULTS: Sixteen of 22 eligible families participated, and 20 parental caregiver interviews were completed. Survivors were a median age of 6 years (range: 1-9 years) at diagnosis, and were 9.5 years (range: 5-12 years) from treatment at the time of the interview. Three major themes and associated subthemes emerged: (i) parental caregivers described significant long-term challenges associated with their child's survivorship. Subthemes included medical treatment sequelae, school issues and behavioral concerns, and surveillance and access to care. (ii) Parental caregivers recognized the impact that their child's quality of life (QOL) had on both their personal and family QOL. Subthemes included parental QOL, parental mental health and coping, spousal relationships, and effects on the family unit as a whole. (iii) Parental caregivers reported experiencing conflicting emotions related to their child's survivorship status and long-term effects. Subthemes included feeling happiness with concurrent worry, fear, and stress, as well as concerns about the future. CONCLUSIONS: Parental caregivers of medulloblastoma survivors experience long-term challenges, with personal and family impacts. Further work is needed to improve care models and support systems for families with a child who has survived medulloblastoma.

Factors Associated with Changes in Invasive and Noninvasive Positive Airway Pressure Therapy Settings during Pediatric Polysomnograms.

STUDY OBJECTIVES: Our aim was to identify clinical predictors associated with changes in settings for pediatric invasive and noninvasive positive airway pressure therapy, which could help inform the allocation of limited polysomnogram (PSG) resources. METHODS: A retrospective review was conducted in children who underwent one or more PSGs for technology titration. Children were included if they were using continuous positive airway pressure (CPAP) therapy, bilevel positive airway pressure (BPAP) therapy, or invasive positive pressure ventilation (IPPV) the night of the PSG. The primary outcome measure for the study were predictors of change in settings during IPPV, CPAP, and BPAP titration studies. RESULTS: During the study period, 274 children using CPAP, BPAP, or IPPV underwent one or more titration PSGs. The mean (standard deviation [SD]) age of the children at the time of the first titration PSG was 10.52 (5.11) y. Fifty percent (n = 136) of the study participants were male. Most patients underwent BPAP titration studies (n = 166), followed by CPAP (n = 83) and then IPPV (n = 25). A total of 623 technology titration PSGs were completed. Reason for respiratory technology, type of respiratory technology, and time between ventilation initiation and the PSG were significant predictors of a change in settings in the multivariable regression model. CONCLUSIONS: Children were more likely to have a change in their technology settings during a PSG if there was a shorter period of time from the original technology initiation, if they were using BPAP (as compared to CPAP or IPPV) and/or if they had a primary central nervous system or musculoskeletal diagnosis.

A comparison of invasive and noninvasive ventilation in children less than 1 year of age: A long-term follow-up study.

BACKGROUND: We report on the long-term survival of children initiated on invasive and noninvasive positive pressure ventilation (NiPPV) before the age of 1 to assess the safety and efficacy of long-term ventilation at home. METHODS: A chart review was performed of children initiated on long-term home mechanical ventilation (LTHV) before the age of 1 year, at The Hospital for Sick Children (SickKids), Canada, between January 1991 and April 2014. RESULTS: We report on 51 children. Twenty-five children (49%) received NiPPV and 26 (51%) received invasive mechanical ventilation via tracheostomy (IMV). There was one NiPPV initiation between 1991 and 2001, the rest were in subsequent years. Most children had a "musculoskeletal disorder" in the NiPPV cohort, n = 14 (56%) and a "central nervous system" disorder in the IMV cohort, n = 13 (50%). The pCO2 improved with the initiation of NiPPV, P = < 0.0001. Of the 25 subjects initiated on NiPPV, eight (32%) are currently being followed as compared to 22 (84%) in the IMV cohort. Seven (28%) of the NiPPV group were weaned off ventilation as compared to three (11.5%) in the IMV cohort. There were two NiPPV treatment failures. There were more deaths in the NiPPV cohort: eight (32%) versus two (7.6%) in the IMV cohort. Four of the deaths in the NiPPV cohort were in children in whom a palliative approach was taken. None were due to NiPPV technical failure. CONCLUSIONS: Based on this long-term follow-up study, NiPPV use in infants appears to be a viable long-term ventilation strategy.

Sleep-disordered breathing in children with chronic kidney disease.

BACKGROUND: The aim of our study was to ascertain the prevalence and type of sleep-disordered breathing (SDB) in paediatric patients with severe chronic kidney disease (CKD) based on the results of polysomnograms (PSGs). METHODS: Overnight PSGs were conducted on children with CKD stages 3-5 (dialysis dependent). Data were collected on patient demographics from the medical records. Study participants and/or their caregivers completed the paediatric modification of the Epworth Sleepiness Scale Score, the Pediatric Sleep Questionnaire (PSQ) and the Pediatric Quality of Life Inventory at the time of the PSG. RESULTS: Nineteen children were included in the study, of whom seven were on dialysis. The median (interquartile range) age at the time of the PSG was 13.5 (5.4-16.5) years, and eight (42%) of the children were male. There was a 37% (n = 7) prevalence of SDB in this cohort based on the PSG results. Central sleep apnea and obstructive sleep apnea were found in three children each. The PSQ scores did not correlate with the obstructive apnea-hypopnea index. CONCLUSIONS: There was a high prevalence of SDB in this cohort of children with CKD. The PSG and validated sleep questionnaires yielded discordant results, reinforcing the limitations of diagnosing SDB in the CKD population based solely on sleep questionnaires.

The association between sleep-disordered breathing and magnetic resonance imaging findings in a pediatric cohort with Chiari 1 malformation.

BACKGROUND: The prevalence of sleep-disordered breathing (SDB) reported in the literature for Chiari malformation type 1 (CM1) is uniformly high (24% to 70%). In Canada, there is limited access to pediatric polysomnography (PSG). Therefore, the identification of clinical features would be invaluable for triaging these children. OBJECTIVE: To identify demographic features, clinical symptoms/signs and radiological findings associated with SDB in a large pediatric cohort with CM1. METHODS: A retrospective review was conducted on children with CM1 who underwent baseline PSG. Data were collected on patient demographics (age, sex, weight, height, body mass index), clinical symptoms (chart review and clinical questionnaires), diagnostic imaging of the brain and cervicothoracic spine, and medical history at the time of referral. RESULTS: A total of 68 children were included in the review. The mean (± SD) age of the children at the time of PSG was 7.33 ± 4.01 years; 56% (n=38) were male. There was a 49% prevalence of SDB in this cohort based on the overall apnea-hypopnea index. Obstructive sleep apnea was the predominant type of SDB. Tonsillar herniation was significantly correlated with obstructive apnea-hypopnea index (r=0.24; P=0.036). CONCLUSIONS: A direct relationship between the degree of cerebellar tonsillar herniation and obstructive sleep apnea was demonstrated. However, further prospective studies that include neurophysiological assessment are needed to further translate the central nervous system imaging findings to predict the presence of SDB.

Pediatric long-term home mechanical ventilation: twenty years of follow-up from one Canadian center.

BACKGROUND: Canadian longitudinal data from a pediatric domiciliary long-term mechanical ventilation (LTMV) program is lacking. OBJECTIVE: Our aim was to report on the clinical characteristics and trends of children followed in one of Canada's pediatric home ventilation programs over the past 20 years. METHODS: A retrospective chart review was conducted on patients receiving long-term domociliary mechanical ventilation between January 1, 1991 and December 31, 2011 in a single center. Domiciliary long-term mechanical ventilation was defined as the daily use of invasive mechanical ventilation (IMV) or noninvasive positive pressure ventilation (NiPPV) for at least 3 months, in the users' home or in a long-term residential facility. RESULTS: Between 1991 and 2011, a total of 379 children were identified (313 [83%] with noninvasive ventilation). The median age at initiation was 9.6 years (interquartile range [IQR] 2.9-13.9), the median duration of ventilation was 2.2 years (IQR 0.8-4.9) and 53% were male. Ninety-nine percent of children were cared for at home. The reason for ventilation was "musculoskeletal" in origin for the majority of children. The number of children receiving long-term mechanical ventilation at home increased from 2 in 1991 to 156 children as of December 2011. There was a twofold increase in the number of invasive ventilation initiations in the second 10 years, n = 45 (2001-2011) as compared to the first 10 years, n = 21 (1991-2000). However, there was more than a fivefold increase in the number of noninvasive initiations in the first 10 years, n = 50 (1991-2000) as compared to the second 10 years, n = 263 (2001-2011). The largest growth was in the 13-18 years age group. There were 55 (15%) mortalities over the study period. CONCLUSIONS: In summary, our 20-year retrospective study has shown that there has been an exponential growth in the number of children receiving domiciliary LTMV with the majority of children having favorable outcomes. Our study represents a step towards developing a Canadian registry to design and implement programmatic change for this medically complex population to ensure best practice for these children as well as their families.