Krista Keilty

Outcome of non-invasive positive pressure ventilation in paediatric neuromuscular disease

Background: Non-invasive positive pressure ventilation (NPPV) has a beneficial effect on nocturnal hypoventilation and hospitalisation rates in adults with static or slowly progressive neuromuscular disease and respiratory failure. Its role in children affected with similar disease processes, however, remains unclear. Aims: To investigate the impact of NPPV on hospitalisations and sleep related respiratory parameters in children with neuromuscular disease. Methods: Fifteen children (mean age 11.7, range 3.4–17.8 years) diagnosed with neuromuscular disease who had been started on nocturnal NPPV and had at least one year of follow up since the initiation of such therapy were studied. Patients served as their own controls and comparison was made of the years preceding and following the initiation of NPPV. Results: Children spent 85% fewer days in hospital (mean pre-NPPV 48.0 days, mean post-NPPV 7.0 days) and 68% less days in intensive care after initiation of NPPV (mean pre-NPPV 12.0 days, mean post-NPPV 3.9 days). Sleep study parameters including number of desaturations, apnoea-hypopnoea index and transcutaneous pCO2 levels improved after initiation of NPPV. Conclusions: NPPV can decrease hospitalisations for children with neuromuscular disease and improves sleep related respiratory parameters. A prospective study is now needed to further delineate the role of NPPV in this population of children.

Nocturnal hypoventilation: predictors and outcomes in childhood progressive neuromuscular disease

Objectives To determine: (a) prevalence of clinically unsuspected nocturnal hypoventilation (NH) in a clinic population of children with progressive neuromuscular disease; (b) whether NH can be predicted from clinical/laboratory parameters; and (c) change over 1 year in pulmonary function decline, quality of life and attention in children with NH treated with non-invasive positive pressure ventilation (NPPV) compared with children without NH. Design Prospective cohort study. Setting Two tertiary-care paediatric neuromuscular clinics. Patients 46 children (6–17 years) with progressive neuromuscular disease without neurocognitive impairment or dystrophinopathy. Interventions Polysomnography, pulmonary function, manual muscle strength, quality of life (CHQ-PF50) and Conners questionnaires. Outcome measures (a) Prevalence of NH; (b) predictive value of surrogate clinical measures for NH; and (c) differences in change over 1 year in pulmonary function, muscle strength, quality of life and attention between children with and without NH. Results Prevalence of NH was 14.8%, 95% CI 8.0% to 25.7%. Maximal sensitivity and specificity for NH were achieved with thresholds of forced vital capacity <70% and forced expiratory volume in 1 s <65% predicted (sensitivities: 71.4, 71.4; specificities: 64.1, 79.5). Scoliosis also predicted NH (sensitivity 88.9; specificity 80.4). Over 1 year, those with NH had a greater increase in residual volume/total lung capacity (0.075 (−0.003 to 0.168) vs −0.03 (−0.065 to 0.028)), decline in muscle strength (−0.67 (−0.90 to 0.10) vs 0.53 (−0.05 to 0.90)) and worsened perception of health status. Conclusions 15% of subjects had clinically unsuspected NH, predicted by moderate pulmonary function test impairment and scoliosis. Over 1 year those with NH had increased gas trapping, decline of muscle strength and worse perception of health status, despite NPPV.

An Evaluation of Dyadic Peer Support for Caregiving Parents of Children with Chronic Lung Disease Requiring Technology Assistance

Abstract This study evaluated a dyadic peer support pilot intervention for parents of technology-assisted children with chronic lung disease. These medically-fragile children, living at home in the primary care of their parents, require continuous or intermittent assistance from technological support such as home oxygen, respiratory or cardiac monitors and/or mechanical ventilators. The intervention consisted of matching parents with similar caregiving responsibilities, in order to reciprocally engage in parent-to-parent support. Results identified mixed outcomes based on quantitative and qualitative methods. Participants conveyed benefits and limitations of this modality of peer-based support. They generally favoured peer support as a clinical resource for caregiving parents as the intervention offered a valued opportunity for mutual sharing with another parent who could understand the unique realities particular to caring for a medically-fragile child. Sharing daily experiences was reported to reduce isolation, increase knowledge, and provide an important sense of feeling understood. Challenges associated with peer support included scheduling difficulties and personality incompatibility. Recommendations for program development in clinical settings are described.

Sleep disturbance in family caregivers of children who depend on medical technology: A systematic review

PURPOSE Society relies on family caregivers of children who depend on medical technology (e.g. mechanical ventilation), to provide highly skilled and vigilant care in their homes 24 hours per day. Sleep disturbance is among the most common complaints of these caregivers. The purpose of this review is to systematically examine studies reporting on sleep outcomes in family caregivers of technology dependent children. METHODS All relevant databases were systematically searched: MEDLINE, EMBASE, PsycINFO and CINAHL. Given the heterogeneity of the studies, a qualitative analysis was completed and thus results of this review are presented as a narrative. RESULTS Thirteen studies were retrieved that met eligibility criteria for inclusion. All of the studies reported on family caregivers of children with medical complexity living at home. Moreover, all of the studies relied entirely on self-report, not objective sleep measures. No intervention studies were found. Sleep disturbance was found to be common (51-100%) along with caregiver reports of poor sleep quality. Sleep quantity was seldom measured, but was found in the few studies that did, to be approximately 6 hours, or less than recommendations for optimal health and daytime function. Multiple caregiver, child and environmental factors were also identified that may negatively influence caregiver sleep, health and daytime function. CONCLUSION Findings of this review suggest that family caregivers of children with medical complexity who depend on medical technology achieve poor sleep quality and quantity that may place them at risk of the negative consequences of sleep deprivation. Recommendations for practice include that health care providers routinely assess for sleep disturbance in this vulnerable population. The review also suggests that studies using objective sleep measurement are needed to more fully characterize sleep and inform the development of targeted interventions to promote sleep in family caregivers of technology dependent children.

Pediatric home mechanical ventilation: A Canadian Thoracic Society clinical practice guideline executive summary

ABSTRACT Over the last 30 to 40 years, improvements in technology, as well as changing clinical practice regarding the appropriateness of long-term ventilation in patients with “non-curable” disorders, have resulted in increasing numbers of children surviving what were previously considered fatal conditions. This has come but at the expense of requiring ongoing, long-term prolonged mechanical ventilation (both invasive and noninvasive). Although there are many publications pertaining to specific aspects of home mechanical ventilation (HMV) in children, there are few comprehensive guidelines that bring together all of the current literature. In 2011 the Canadian Thoracic Society HMV Guideline Committee published a review of the available English literature on topics related to HMV in adults, and completed a detailed guideline that will help standardize and improve the assessment and management of individuals requiring noninvasive or invasive HMV. This current document is intended to be a companion to the 2011 guidelines, concentrating on the issues that are either unique to children on HMV (individuals under 18 years of age), or where common pediatric practice diverges significantly from that employed in adults on long-term home ventilation. As with the adult guidelines,1 this document provides a disease-specific review of illnesses associated with the necessity for long-term ventilation in children, including children with chronic lung disease, spinal muscle atrophy, muscular dystrophies, kyphoscoliosis, obesity hypoventilation syndrome, and central hypoventilation syndromes. It also covers important common themes such as airway clearance, the ethics of initiation of long-term ventilation in individuals unable to give consent, the process of transition to home and to adult centers, and the impact, both financial, as well as social, that this may have on the childs families and caregivers. The guidelines have been extensively reviewed by international experts, allied health professionals and target audiences. They will be updated on a regular basis to incorporate any new information.

Paediatric Patient-Centred Care: Evidence and Evolution

This chapter begins with an overview of paediatric patient-centred care, with a focus on child and family-centered care (CF however, it increasingly points to the positive impact of C&FCC on a range of outcomes and processes related to health, mental health, well-being, satisfaction and family functioning. There is emerging consensus about core elements that comprise C&FCC and, increasingly, the body of literature indicates that C&FCC merits ongoing implementation and evaluation in seeking effective paediatric healthcare.

Sleep disturbance in family caregivers of children who depend on medical technology

Objectives Family caregivers of children who depend on medical technology (CMT) provide highly skilled care up to 24 hours per day. Sleep disruption places family caregivers at risk for poor health and related outcomes that threaten their long-term caregiving capacity. Few studies exist that have measured sleep in family caregivers, and most have relied entirely on subjective measures. Methods In a prospective cohort study, family caregivers of CMT (n=42) and caregivers of healthy children (n=43) were recruited. Actigraphy data and a concurrent sleep diary were collected for 6 days/7 nights. Measures of sleep quality, depression, sleepiness, fatigue and quality of life were also administered. Results Family caregivers of CMT averaged fewer hours of sleep per night (mean (SD)) (6.56 ± 1.4 vs 7.21 ± 0.6, p=0.02) of poorer quality (7.75 ± 2.9 vs 5.45 ± 2.8, p<0.01) than the control group. Three times as many family caregivers of CMT scored in the range for significant depressive symptomatology (12(33%) vs 4(10%), p=0.01) and experienced excessive daytime sleepiness (16(38%) vs 5(12%), p<0.01). Fatigue was also more problematic among family caregivers of CMT (22.12 ± 9.1 vs 17.44 ± 9.0, p=0.02). Conclusions Family caregivers of CMT are at risk of acute and chronic sleep deprivation, psychological distress and impaired daytime function that may threaten their capacity for sustained caregiving. Family caregivers of CMT may be important targets for screening for sleep disorders and the development of novel sleep-promoting interventions.

Paediatric Patient-Centred Care at SickKids: Advancing Child & Family-Centred Care

The delivery of ‘child and family-centred care’ (C&FCC), used interchangeably with ‘paediatric patient-centred care’, is consistent with the vision, mission and values of The Hospital for Sick Children (SickKids). As innovators in child health, SickKids leads and partners to improve the health of children provincially, nationally and internationally through the integration of clinical care, research and education. Institutional aims as outlined in the mission of the hospital include providing the best in complex and specialized C&FCC. Components of C&FCC are discussed and examples of innovation in C&FCC at SickKids are identified.

Experiences with unregulated respite care among family caregivers of children dependent on respiratory technologies

Increasingly, children with respiratory conditions who are dependent on medical technology (e.g. ventilators and tracheostomies) are cared for at home by family caregivers who are at risk for significant health, financial and social burdens. In many jurisdictions, access to quality respite is varied and often the availability of regulated (nursing) providers is insufficient. Rather than go without, some families have secured alternative and unregulated providers to supplement formal home care systems. The purpose of this study was to explore the experiences of family caregivers of children dependent on respiratory technologies who have used unregulated providers for in-home respite care. Through an interpretative description approach, data was gathered from 20 semi-structured parent interviews and analysed using constant comparative analysis. Four themes emerged from the data, which were conceptualized as both opportunities and tensions that parents experienced with both unregulated and regulated home care providers: finding the right fit for the child and family; trusting the provider is everything; using unregulated providers offers unique advantages; and accepting that regulated and unregulated care present challenges. Findings signal that unregulated providers play a pivotal role in supporting parents of children who are dependent on respiratory technologies. Implications for practice, policy and future research initiatives are discussed.

Section 12: Transition from pediatric to adult care

With improvements in care and technological support, increasing numbers of children on long-term ventilation are now surviving into adulthood. At some point in the child’s life, ongoing care in a pediatric environment ceases to be desirable or optimal, and transition to an adult care program therefore becomes necessary. Transition has been defined as the “purposeful, planned movement of adolescents with chronic medical conditions from child-centred to adult-oriented health care.” Transition to adult care is a major and predictable event in the child’s life, one that requires careful consideration and planning. To ensure a smooth transfer of the child’s care that ensures all the child’s ongoing needs are met (not just medical, but emotional and social), many components need to be in place in both the pediatric and adult health systems. Otherwise, there are significant risks that this transfer may be associated with significant patient and family anxiety, reduction in adherence to treatment or loss to follow-up, resulting in a deterioration in health status. Although we were unable to find any studies specifically addressing this issue for children on home mechanical ventilation (HMV), there is a significant literature dealing with transition of care in to adulthood for many other chronic childhood illnesses. Position statements from the Canadian Paediatric Society and American Academy of Paediatrics, although not specifically directed towards HMV, do have sufficient commonality to inform the care of patients on HMV. Most of the literature in this area consists of qualitative, observational studies and consensus statements, with relatively little objective evidence with respect to effectiveness of differing models of transition. There is a growing consensus on the components required for transfer to adult care, though a paucity of objective data.