Home mechanical ventilation in complex chronic disease in children – what is the impact on the sleep quality of the caregivers?


 Background The role caregivers of children with complex chronic disease play may be linked to important sleep disturbances, which may threaten the long-term quality care to their children.Aim Evaluation of the sleep quality of the caregivers of children with complex chronic disease under home mechanical ventilation.Design Observational and analytic study (October 2018 – January 2019).Setting/Participants We assessed 33 caregivers of patients with complex chronic disease under home mechanical ventilation for ≥3 months, followed in the Pneumology Clinic of a tertiary paediatric hospital. Evaluation instrument: Pittsburgh Sleep Quality Index (Portuguese version).Results Most of the caregivers were mothers (88%), median age of 40 years. Most of the children were male (70%), median age of 11 years. The main diagnostic group was genetic/polymorphic syndrome (46%); 52% belonged to group 2 (Association for Children with Life-Threatening or Terminal Conditions). There was a discrepancy between the subjective sleep quality ("Good" in 64%) and the Pittsburgh Sleep Quality Index score ("Poor" in 72%), with significant percentage of daytime sleepiness/dysfunction (75%). The increase in daily hours of ventilation seems to lead to a lower sleep quality (p = 0.669). Caregivers with sleep disorder had lower sleep quality values.Conclusions The sleep quality of the caregivers of children under home mechanical ventilation has ​​similar values to specific groups described in literature, but lower than that of the healthy Portuguese population. It is essential to raise the professionals’ awareness for the evaluation of sleep quality of caregivers, since it may not be adequately perceived by them.

The sleep quality of the caregivers of children under home mechanical ventilation has similar values to specific groups described in literature, but lower than that of the healthy Portuguese population. It is essential to raise the professionals' awareness for the evaluation of sleep quality of caregivers, since it may not be adequately perceived by them.

Background
In the last few decades, there has been a substantial rise in the number of children and adolescents depending on home mechanical ventilation, due to an increase in the number of patients surviving critical illness and due to technological advances. (1)(2)(3)(4) Home mechanical ventilation allows a prolongation of life, palliation of symptoms and an improvement in the quality of life. (1) With this, the care of many of these children has shifted from professionals in the hospital to parents in the family home, and society increasingly relies on the caregivers of dependent children to provide highly skilled and vigilant care in their homes 24 hours a day. (2,3) The caregivers of children who depend on medical technology at home have been found to experience numerous negative outcomes, including high levels of physical, emotional, financial and family stress. (5) The extraordinary responsibility that caregivers undertake may be linked to chronic sleep disturbance, which may threaten their ability to provide long-term quality care for their children. (2) Sleep is recognized as a complex biobehavioral process described as "a reversible behavioral state of perceptual disengagement from, and unresponsiveness to, the environment". (6) Sleep, in adequate quantity and good quality, is considered necessary for an overall health and optimal daytime performance. Shorter sleep duration and higher nocturnal awakenings have been reported, when comparing sleep in caregivers of children with physical disabilities to those of typically developing children.(2) Chronic sleep deprivation is considered a major public health concern and has been associated with caregiver stress and depression. (2,7) In 1993, an home mechanical ventilation program for children and adolescents with chronic respiratory failure began in our hospital. It has already supported hundreds of patients with a positive impact in patients and caregivers´ lives. (4) Methods Observational and analytic study performed between October 2018 and January 2019. The study group was defined based on a convenience sample consisting of parents/caregivers of children/adolescents (0-17 years) with complex chronic disease, followed in the Pneumology Clinic of our hospital. The inclusion criteria were parents/caregivers of children/adolescents with complex chronic disease under home mechanical ventilation for at least 3 months; parents/caregivers who could not read and/or write in Portuguese were excluded.
It was considered as caregiver, the person responsible for most of the care of the patient.
Home mechanical ventilation included invasive ventilation by tracheostomy and noninvasive ventilation provided at the child's home.
Complex chronic disease was defined based on the definition of Feudtner: "Any medical condition that can be reasonably expected to last at least 12 months (unless death intervenes) and to involve either several different organ systems or 1 organ system severely enough to require specialty pediatric care and probably some period of hospitalization in a tertiary care center" (8) and updated according to the ICD-10 diagnostic codes. (9) The diagnoses were grouped according to the diagnostic group into neuromuscular disease, respiratory disease, metabolic/neurodegenerative disease, central hypoventilation and other congenital or genetic defect (which included all the congenital/genetic defects, except central hypoventilation, which constitutes a different group). They were also grouped according to the trajectory, using the classification of the Association for Children with Life-Threatening or Terminal Conditions and their Families (ACT), which reflects the trajectory of diseases requiring palliative care in a pediatric context. Group 1 represents "life-threatening conditions for which curative treatment may be feasible but can fail", group 2 covers "conditions where premature death is inevitable, but long periods of intensive treatment are aimed at prolonging life and allowing participation in normal activities", group 3 includes "progressive conditions without curative treatment options, where treatment is exclusively palliative and may extend over many years" and group 4 represents "irreversible but non-progressive conditions with severe disability and are susceptible to health complications and premature death".
We used the Pittsburgh Sleep Quality Index (PSQI), validated for the Portuguese population, as the evaluation instrument. After obtaining an informed written consent, caregivers completed the questionnaire anonymously.
The PSQI is a generic instrument that subjectively measures the sleep quality of a subject over a 1-month time interval. It consists of 19 questions, grouped into 7 clinical domains related to sleep quality (subjective sleep quality, sleep latency, sleep duration, habitual sleep efficiency, sleep disturbances, use of sleep medication, and daytime dysfunction).
Each component is evaluated on a scale from 0 to 3. The 7 components are combined to obtain the overall value of sleep quality on a scale from 0 to 21. It helps distinguish between "good sleep quality" and "poor sleep quality". A global sum of 5 or greater indicates a "poor" sleeper, with major difficulties in at least two components, or moderate difficulties in over three components. An higher overall value reflects a worse sleep quality.
The information obtained was complemented by a questionnaire which included other variables, such as demographic variables (gender, age, literary abilities, marital status, relationship to the child/adolescent) and clinical variables (presence of sleep disorder) of the caregivers and demographic, clinical and social variables of the child/adolescent (gender, age, diagnosis, attendance of a place of learning quantified in hours, average of daily hours of ventilation and type of interface of ventilation). Ethics Committee provided approval.
Statistical analysis was performed using IBM SPPS Statistics® version 25. The level of significance adopted in the study was 5%.

Group of caregivers and patients
PSQI were filled by 29 mothers (87.9%) and 4 fathers (12.1%), with no other type of caregivers included. The sociodemographic details are presented in Table 1. Of the 33 caregivers included in the study, 24.2% suffered from some type of pathology or sleep disorder.

Sleep quality
The values obtained in each of the components of the PSQI and its global value are presented in Table 3. The PSQI global values are presented in Table 4 by diagnostic and ACT group, characteristics of the home mechanical ventilation and attendance of a place of learning. Nevertheless, it is possible to infer that a greater number of hours of home mechanical ventilation tends to cause a poorer global sleep quality of the caregiver, through a scatter plot (Fig. 1).
The relationship between the global value of sleep quality and the presence of pathology/sleep disorder in the caregiver was studied using extreme diagrams and quartiles (Fig. 2).

Main findings of the study
Sleep disturbance is a highly common problem, with multiple contributing factors, and is Sleep quality of the caregivers didn't vary significantly with the attendance of a place of learning. In a study of children with respiratory disease, no differences in primary sleep outcomes were found between caregivers who completed questionnaires during the school year versus the summer/holidays. (14) The type of interface and the number of daily hours using home mechanical ventilation didn't seem to interfere with sleep quality. The duration of home mechanical ventilation and the time of the day when it was performed were identified as important factors for sleep disruption, with a greater negative impact with the increase in daily hours of home mechanical ventilation. (18) However, the longer-term use of non-invasive ventilation appears to result in an adjustment by the caregiver, with consequent improvement in sleep quality. (19) Strength and limitations of the study Within the limitations of the study, it is to highlight that the sample size is relatively small, making it difficult to find significant associations.
PSQI doesn't provide detailed information on specific factors, which may vary depending on the underlying pathology and, therefore may influence the sleep quality of caregivers.
The study was based on a subjective self-completion tool that can lead the caregiver to underestimate/overestimate the quality of the measured parameters. The fact that about 25% of the sample presented a sleep disorder may constitute a bias to the results found.
Sleep may be influenced by other variables not considered in this study (environmental issues, sleep hygiene habits, presence of one or more caregivers, child/adolescent's disease stage).
It would be useful to carry out a prospective and longitudinal study, which would allow the questionnaires to be applied immediately before the onset of home mechanical ventilation and after a period of 3 to 6 months, in order to determine the impact of home mechanical

Conclusion
Sleep quality of caregivers of children/adolescents under home mechanical ventilation is lower than that recorded in the healthy Portuguese population and presents values similar to those described in particular groups in the literature.
Given the data obtained, the authors consider that it is essential to sensitize health professionals to assess sleep quality of the caregivers of patients with complex chronic disease, given the relevance, not only in performing of their duties, but also in their own health and quality of life. Therefore, taking care of the caregivers should be one of the axes of the medical intervention.  Appendix.docx