Respiratory Health, Sleep Dysfunction, and Mental Health in Children and Adolescents With a Neuromuscular Disorder: A Descriptive Qualitative Study

Article information

J Sleep Med. 2023;20(1):11-18

Publication date (electronic) : 2023 April 30

doi : https://doi.org/10.13078/jsm.220026

Meg Huston ¹^,²

, Adelaide L Withers ¹^,²^,³

, Jenny Lam ¹

, Andrew Wilson ¹^,²^,³

, Jenny Downs^,¹^,²

¹Telethon Kids Institute, The University of Western Australia, Nedlands, Australia

²Curtin School of Allied Health, Curtin University, Bentley, Australia

³Perth Children’s Hospital, Nedlands, Western Australia, Australia

Address for correspondence Jenny Downs, BApplSci, GradCertPubHlth, MSc, PhD Telethon Kids Institute, The University of Western Australia, 15 Hospital Avenue, Nedlands, Western Australia 6009, Australia Tel: +61-8-6319-1763 E-mail: Jenny.Downs@telethonkids.org.au

Received 2022 December 23; Revised 2023 January 28; Accepted 2023 February 1.

Abstract

Objectives

To explore how respiratory health and sleep problems affect mental health in children and adolescents with neuromuscular disorders (NMDs).

Methods

A qualitative descriptive study was carried out with content analysis of in-depth interviews conducted with children with NMDs and their parents. Data were collected during semi-structured interviews with children with NMDs and their parents. A total of 14 families were recruited (17 parents and 7 children and adolescents with NMD aged 5–17 years). Each interview was conducted with 1–3 family member(s).

Results

Engagement in community activities, meaningful relationships, feeling well, and achieving independence each contributed positively to children’s mental health. Additional challenges to mental health during periods of poor respiratory and/or sleep health included: 1) challenges to behavioral regulation, 2) changes to mood, and 3) challenges to thinking. Parents and children reported nurturing and coping self-management strategies when their physical and mental health were dually challenged in addition to medical management strategies such as non-invasive ventilation (NIV).

Conclusions

This study supports the notion that changes in mental health may indicate poor physical health in children with NMDs. Treating respiratory and sleep problems, such as by early NIV implementation, may improve the mental health of children with NMDs and thus support their overall wellbeing and health outcomes.

Keywords: Neuromuscular diseases; Respiratory diseases; Sleep; Mental health

INTRODUCTION

Neuromuscular disorders (NMDs) are rare conditions characterized by muscle weakness, many of which involve progressive loss of functional abilities [1]. NMDs include Duchenne muscular dystrophy (DMD), spinal muscular atrophy, and other rare disorders such as congenital myopathy and muscular dystrophy [2]. Muscle weakness [3,4] is associated with reduced mobility, pain, and respiratory complications such as hypoventilation, respiratory failure, and sleep dysfunction [4,5]. Medical treatments and rehabilitation strategies focus on delaying functional decline and improving quality of life (QOL) [6] as there are currently no known cures for these disorders [1,2,7].

Individuals with NMD are at risk of frequent respiratory infections, greater healthcare utilization, and increased hospitalization [5,8-10]. Sleep apnea and sleep-related hypoventilation often co-exist in this patient population [8,10]. Specifically, in boys with DMD aged 5–18 years, the prevalence of obstructive sleep apnea, central sleep apnea, and hypoventilation were 64%, 34%, and 17%, respectively [11]. Symptoms of sleepdisordered breathing associated with NMDs include daytime fatigue, morning headaches, snoring, restless sleep, difficulty in rousing, mood changes, and difficulty in focusing [8,10]. Sleepdisordered breathing can be treated with non-invasive ventilation (NIV) [9]. Clinical surveillance of respiratory dysfunction is important for effectively providing respiratory support [1,2,7].

Mental health encompasses how a person operates in their daily lives, including managing everyday stress [12]. Parent-reported data suggest that children with NMDs are at risk of developing difficulties with behavior, communication, and social interaction, and such challenges are often associated with other neurodevelopmental impairments such as intellectual disabilities or autism spectrum disorder [13]. A higher prevalence of internalizing (anxiety and depression) and externalizing (aggression and hyperactivity) problems has been observed in boys with DMD than in healthy populations [14]. Data on these mental health trends have been acquired by parent reporting [13,14], child reporting [13], or clinical evaluations [14,15].

Physical and mental health have been found to be interrelated [16]. For example, improved sleep health has been associated with better mental health and cognition in the general population [17,18]. Fatigue in adults with NMDs is associated with lower mood [19]. Similarly, children with asthma are more likely to exhibit poor mental health than their healthy counterparts [16], and poor respiratory health in patients with NMDs is believed to have implications for their mental health. Beyond these data, the lived experiences and impacts of fluctuating physical health on mental health have not yet been explored in children and adolescents with NMDs. This study aimed to explore how episodic respiratory and sleep problems affect mental health in children and adolescents with NMDs from the perspectives of parent caregivers and children.

METHODS

This was a descriptive, qualitative study that aimed to gain insights into the lived experiences of children and adolescents with NMD related to respiratory health, sleep, and mental health. Ethical approval was granted by the Child and Adolescent Health Services Human Research Ethics Committee (RGS4295) and Curtin University Human Research Ethics Committee (approval number: HRE2021-0123). Written informed consent was obtained from participating parents and mature minors.

Participants and recruitment

Purposive sampling across a range of characteristics was employed due to the lack of related literature in the field. We aimed to capture the variation in experiences across different ages (5–17 years), diagnoses, and mobility. Participants were recruited via the Muscular Dystrophy Western Australia (MDWA), Perth Children’s Hospital (PCH), and Telethon Kids Institute (TKI). The study was advertised through TKI networks in the clinic at the PCH and through MDWA networks.

Procedures

Prior to commencement, the study design and interview schedule were presented to a Consumer Reference Group comprising parents of children with NMDs to obtain their input. The interview schedule (Supplementary Material in the online-only Data Supplement) was semi-structured with open-ended questions about current health, wellbeing, and episodes of poor respiratory and sleep health followed by probing questions. Interviews were conducted once with each family member by phone or videoconferencing. Training was provided by the senior author (JD) to the primary researcher (MH, a physiotherapist with special interest in disability). All interviews were audio-recorded and transcribed verbatim. Parent caregivers and children were offered the option to be interviewed separately or together depending on their preferences. Preliminary scoping suggested that children and adolescents prefer discussions at the family level.

Data management and analysis

The final interview transcripts were imported to NVivo software (QSR International Pty Ltd., Burlington, MA, USA) and analyzed using content analysis to generate a framework of domains considering relevant findings in the literature. The researchers had prior knowledge in this field, and this study was not oriented toward developing a theory [20]. The primary researcher read the transcripts multiple times and coded the data inductively into categories. Quotes from parent caregivers, children, and adolescents were coded together within categories. The categories were reviewed by the research team to achieve consensus. The data were estimated to be thematically saturated when no new categories or elements were identified within the categories.

Rigor of collected data

Strategies were implemented to ensure the rigor of data collection and address common criteria, including credibility, dependability, confirmability, and transferability [21]. For credibility, the data were peer-debriefed and member-checked with the Consumer Reference Group to discuss the findings. An audit trail using NVivo-12 ensured dependability and confirmability. Thick descriptions in the research allowed for the transferability of conclusions drawn from this sample of children with NMDs to other children with NMDs. To limit the influence of researcher bias on interpretation, audiotapes, transcripts, and coding were examined at length by the full research team.

RESULTS

From a total of 17 families, members from 14 families were recruited, while 3 families declined to participate. Parents and children were offered the opportunity to participate in interviews together or separately; however, no child chose to participate in individual interviews. Interviews were conducted with 14 families comprising 17 parents (14 mothers and 3 fathers) and 7 children/adolescents. As all participants spoke English, interviews were conducted in English. Most children and adolescents included in this study were diagnosed with DMD. The majority of the patients were boys, non-ambulant, and receiving nocturnal respiratory support. The median age of child participants was 14 (range: 7–17) years. Most children reported pain on their usual days. Participant characteristics are presented in Table 1. Families were interviewed between January and June 2021. Interviews lasted a median (range) of 73 min (51–156 min).

Table 1.

Characteristics of the participants from the families (n=14)

Participants described how engagement in community activities, fostering meaningful relationships, and feeling independent contributed to their child’s mental health. Periods of poor respiratory health and difficulties with sleep (i.e., lower respiratory tract infections, poor sleep quality, and snoring) additionally contributed to the child’s mental health in three categories: 1) difficulties regulating behaviors, 2) changes to mood, and 3) challenges to thinking (Fig. 1). Parents and children reported nurturing and self-management coping strategies in addition to medical management such as NIV, which improved sleep and supported the child’s ability to manage everyday challenges. Sample quotes illustrating these categories are presented in Tables 2–4.

Fig. 1.

Illustration of the key categories factors of the child’s mental health and the influences of poor sleep and respiratory illness.

Table 2.

Elements and sample quotes in the challenges to regulation of behaviours category

Table 3.

Elements and sample quotes in the changes to mood category

Table 4.

Elements and sample quotes in the challenges to thinking category

Difficulties regulating behavior

Table 2 presents elements and sample quotes for this category.

Cranky and argumentative

Many parents observed their children becoming cranky and more argumentative during episodes of poor respiratory health or sleep. For example, children were described as lacking patience and being resistant to completing household chores, with a tendency to react intensely rather than regulating their responses: “So his brother might make a comment that normally wouldn’t bother him because he would just be able to ignore it, but he’ll get quite cranky and fire off a response.” This argumentative disposition contrasted with the even-tempered behaviors observed when the child was well.

Impulsivity

After episodes of poor respiratory health or impaired sleep, parents described occasions where their child would throw objects or knock over equipment. These behaviors were not routinely observed when the child was well. As one parent described: “So, on a day where there’s been sleep, that behavior may happen just once within the day, but if he’s had a poor night’s sleep, we’ll see those behaviors four or five times in a day.”

Obsessive behaviors

Some children included in this study have been diagnosed with obsessive–compulsive disorder (OCD) or autism spectrum disorder. Parents observed an increase in obsessive or repetitive behaviors during episodes of poor respiratory health or sleep. Examples include placing items in order and panicking when different foods are mixed on their dinner plate. One parent commented: “So he has significant OCD and I think what happens is that when he becomes more fatigued and more tired, whether that’s due to respiratory or what, his behaviors escalate.”

Meltdowns and tantrums

Episodes of poor sleep and respiratory health were reported to lead to increased tantrums and meltdowns. For example, “Then, it is like a complete meltdown, to the point where he has thrown himself on the floor.” Parents also reported actions such as throwing items.

Changes to mood

Table 3 presents elements and sample quotes for this category.

Anxiety and worry

Tiredness commonly results in anxiety in children and adolescents. For children with underlying anxiety, anxiety escalates with poor respiratory health and sleep. One parent commented that “the anxiety is probably the stem of a lot of the problems, it’s there most of the time, but it escalates very quickly when there’s tiredness.”

Comfort seeking

Many parents commented on their children seeking extra comfort and affection during episodes of poor respiratory health and sleep disturbances. This included wanting to cuddle more with parents, seeking favorite foods and warm drinks, and snuggling with blankets and soft toys. These behaviors were observed less frequently when children were in good health and tended to be more independent.

Irritability

Episodes of poor health and fatigue were considered to induce increased irritability. Some behaviors were described as angry, blunt, snappy, frustrated, and overly sensitive. Other children became irritated with everyday tasks because these tasks were more effortful, as parent commented: “[name]’s form of irritability would be that he gets a bit snappy, and short with people … he does things with a huff.”

Somberness and withdrawal

Some children were described as quiet, drained, having a low mood, and isolating themselves when unwell. One parent stated that “he will just look very dull, and very, very tired… and just not being himself, he won’t talk much. You can tell that something is coming.” These quiet and somber moods contrasted with vibrant and playful attitudes observed when children were well.

Lack of enthusiasm and motivation

Children were reluctant to engage in their daily routine. One parent commented on their child’s lack of enthusiasm and motivation before starting NIV, where the child’s quality of sleep was poor: “she was reluctant to get moving, reluctant to do activities”. The parents noted improvement in engagement in everyday activities after commencing NIV, which was attributed to better sleep quality. When fatigued or unwell, the children were less likely to extend themselves academically, as one child articulated: “…not really motivated to do anything. Like if there’s like extension work offered and if I’ve slept well and I’ve got energy, I will do that. But if I haven’t slept well, I just go back to doing the bare minimum. I just won’t extend myself.”

Challenges to thinking

Table 4 presents elements and sample quotes for this category.

Attention to tasks and concentration

Participants commented that focus, concentration, and following instructions improved following a good night’s sleep. Many parents noted improvements in their children’s attention and concentration after using NIV. One parent described the following comparison: “Before he started [NIV], he would just go in moments of being in a daze and not hearing what was going on around him … so he’s clearer and can think better with the NIV, a good night’s sleep works miracles.”

Capacity to engage

Children’s capacity to engage socially and participate in routine was reduced with poorer health, including lack of initiative to organize themselves for the day (e.g., getting dressed, packing school bags, brushing teeth, etc.). One parent commented that “he would sit on the lounge and not do anything, wait for breakfast to be brought to him.” With good sleep, children typically showed a greater capacity to engage and, hence, more enthusiasm to participate.

Indecision

Some children experienced difficulties in making decisions in relation to episodes of respiratory illness and poor sleep. This indecisiveness was observed over simple lifestyle or routine choices, such as breakfast or beverage preferences. Physical and mental exhaustion due to tiredness or illness made decision-making too effortful for the children, as one parent reported their child saying “I can’t make a decision. Can you just make one for me?”

Nurturing and coping strategies

In addition to implementing medical treatment, children adopted self-management strategies to address their mental health challenges. These included self-pacing, taking rest days, and following regular routines. To manage anxiety and feelings of sadness, some children practiced mindfulness activities, including relaxation, meditation, positive self-talk, and sensory play. Other children adopted more specific coping strategies, such as rating their anxiety on a scale, counting backward from 100, and listening to audiobooks.

DISCUSSION

Children and adolescents with NMDs are at risk of poor mental health [13-15,22]. This can be exacerbated in individuals with DMD owing to the regular use of exogenous corticosteroids, which can alter mood, behavior, and sleep [7,23]. Factors such as reduced participation in the community may also play a role [24]. Similar to our findings, children with increased asthma symptoms (wheezing and night waking) have been reported to exhibit more anxiety, hyperactivity, impulsivity, and oppositional behavior [16,25]. Our data are consistent with relationships described between poor sleep and behavioral problems in children with NMDs [8,10] and between fatigue and mood in adults with NMDs [19]. This is the first qualitative study to explore how episodic respiratory illness and sleep dysfunction impact mental health in children and adolescents with NMDs. Our findings expand on previous NMD literature and include observations of more obsessive behaviors and poorer concentration during these episodes.

Sleep-disordered breathing in children and adolescents in the general population causes inattention and poor concentration [17,18,26]. Our findings suggest that sleep dysfunction may significantly impair thinking processes in children and adolescents with NMDs. The reverse was also true in that the participants described improvements in concentration and focus after experiencing good-quality sleep after NIV treatment was initiated. It is important to note that neuroimpairments, such as intellectual disability, can lead to inattention and poor concentration in individuals with DMD [13-15]. Our interviews aimed to identify specific changes that occurred during episodes of poor respiratory health and sleep in DMD and other NMDs. Everyday cognitive tasks for children and adolescents with NMDs were more challenging during these episodes irrespective of pre-existing neurological or cognitive impairments.

Children’s self-management strategies were supported by medical management such as NIV, which is known to delay respiratory decline, reduce respiratory infection [27,28], and improve sleep and QOL [29]. Other factors also contribute to the mental health of children and adolescents with NMDs. For example, strong social networks (friendships and participation) and better family functioning improve resilience and coping in boys with DMD [30]. In our study, participants similarly identified meaningful community engagement, strong familial and peer relationships, and developing their independence as other factors that contributed to their mental health when they were well and unwell. Exploring other factors that affect mental health was not the aim of this study, which focused on respiratory and sleep issues. However, we confirmed that NMD requires complex care for many impairments and difficulties in maintaining good health, everyday functioning, and participation in the community.

Strengths and limitations

Our data were supported by member checking and peer review to avoid misinterpretation of the data. The inclusion of children and adolescents in the interviews increased the authenticity of children’s reported experiences. There was variability in the participant characteristics representing different ages, NMD diagnoses, and respiratory support use, providing some capacity to transfer these findings to similar patient groups. This study could have been strengthened by more child involvement, although our questions were carefully constructed to acknowledge and involve each child as much as possible.

Conclusions

Respiratory, sleep, and mental health are interrelated in children and adolescents with NMD. This information can be applied to the comprehensive evaluation and treatment of NMD [13,22] with the aim of optimizing health, participation, and QOL. Our findings provide useful domains for the clinical evaluation of physical and mental health. Clinicians can recognize changes in mental health that may indicate worsening physical health of the child when they exhibit behaviors that deviate from their baseline. Further evaluations of the relationships between respiratory, sleep, and mental health using quantitative study designs are needed in future studies. Furthermore, we recommend that the effects of respiratory treatments and sleep interventions, such as early NIV implementation, on the mental health of this population are evaluated.

Supplementary Materials

The online-only Data Supplement is available with this article at https://doi.org/10.13078/jsm.220026.

SUPPLEMENTARY MATERIAL

Interview schedule

jsm-220026-supplementary-1.pdf

Notes

Data availability

Data for this study were obtained from transcripts of interviews carried out by the research team. Coding trees, but not interview transcripts, are available to researchers from the corresponding author on reasonable request.

The authors have no potential conflicts of interest to disclose.

Author Contributions

Conceptualization: Jenny Downs. Data curation: Meg Huston, Jenny Downs. Formal analysis: Meg Huston, Jenny Lam, Jenny Downs. Funding acquisition: Andrew Wilson. Investigation: Meg Huston, Jenny Downs. Methodology: Meg Huston, Adelaide L Withers, Jenny Downs. Project administration: Meg Huston, Jenny Lam, Jenny Downs. Supervision: Jenny Downs Writing—original draft: Meg Huston. Writing—review & editing: all authors.

Funding Statement

This study was supported by the National Health and Medical Research Council (GTN1160578), which provided the infrastructure for this study. JD is supported by a Fellowship from the Stan Perron Charitable Foundation.

Acknowledgements

The authors would like to thank all participating families, parents, children, and adolescents for taking the time to share their experiences and thoughts. The authors thank the Consumer Reference Group for their opinions and advice on the study design, methods, and interpretation of the findings. We also thank Muscular Dystrophy Western Australia for advertising the study through their news forums and connecting to counterpart organizations in Queensland, Victoria, New South Wales, South Australia, and Tasmania.

References

1. Vuillerot C, Hodgkinson I, Bissery A, et al. Self-perception of quality of life by adolescents with neuromuscular diseases. J Adolesc Health 2010;46:70–76. https://doi.org/10.1016/j.jadohealth.2009.05.005.

2. Vanasse M, Paré H, Zeller R. Medical and psychosocial considerations in rehabilitation care of childhood neuromuscular diseases. Handb Clin Neurol 2013;113:1491–1495. https://doi.org/10.1016/B978-0-444-59565-2.00019-8.

3. Cimete G, Arslan S. Home care needs of children with neuromuscular diseases and the difficulties their families face. Revista Brasileira em Promoção da Saúde 2008;21:103–111. https://ojs.unifor.br/RBPS/article/view/668.

4. Skalsky AJ, McDonald CM. Prevention and management of limb contractures in neuromuscular diseases. Phys Med Rehabil Clin N Am 2012;23:675–687. https://doi.org/10.1016/j.pmr.2012.06.009.

5. Benditt JO, Boitano LJ. Pulmonary issues in patients with chronic neuromuscular disease. Am J Respir Crit Care Med 2013;187:1046–1055. https://doi.org/10.1164/rccm.201210-1804CI.

6. Rohmer-Cohen A, Ferrari M, Thoumie P, Bungener C, Mane M. Impact of balance rehabilitation on self-esteem for patients with neuromuscular disease: a preliminary study. J Rehabil 2019. 8523–26. https://doi.org/10.1164/rccm.201210-1804CI.

7. Cowen L, Mancini M, Martin A, Lucas A, Donovan JM. Variability and trends in corticosteroid use by male United States participants with Duchenne muscular dystrophy in the Duchenne registry. BMC Neurol 2019. 1984. https://doi.org/10.1186/s12883-019-1304-8.

8. Birnkrant DJ, Bushby K, Bann CM, et al. Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management. Lancet Neurol 2018;17:347–361. https://doi.org/10.1016/S1474-4422(18)30025-5.

9. Rose L, McKim D, Leasa D, et al. Respiratory health service utilization of children with neuromuscular disease. Pediatr Pulmonol 2018;53:1378–1386. https://doi.org/10.1002/ppul.24145.

10. Bourke SC. Respiratory involvement in neuromuscular disease. Clin Med (Lond) 2014;14:72–75. https://doi.org/10.7861/clinmedicine.14-1-72.

11. Sawnani H, Thampratankul L, Szczesniak RD, Fenchel MC, Simakajornboon N. Sleep disordered breathing in young boys with Duchenne muscular dystrophy. J Pediatr 2015. 166640–645.e1. https://doi.org/10.1016/j.jpeds.2014.12.006.

12. World Health Organisation. Mental health action plan 2013-2020 [Internet]. Geneva: World Health Organisation, 2013 [cited 2020 Sep 7, page 7]. Available from: https://www.who.int/publications/i/item/9789241506021.

13. Darke J, Bushby K, Le Couteur A, McConachie H. Survey of behaviour problems in children with neuromuscular diseases. Eur J Paediatr Neurol 2006;10:129–134. https://doi.org/10.1016/j.ejpn.2006.04.004.

14. Ricotti V, Mandy WP, Scoto M, et al. Neurodevelopmental, emotional, and behavioural problems in Duchenne muscular dystrophy in relation to underlying dystrophin gene mutations. Dev Med Child Neurol 2015;58:77–84. https://doi.org/10.1111/dmcn.12922.

15. Colombo P, Nobile M, Tesei A, et al. Assessing mental health in boys with Duchenne muscular dystrophy: emotional, behavioural and neurodevelopmental profile in an Italian clinical sample. Eur J Paediatr Neurol 2017;21:639–647. https://doi.org/10.1016/j.ejpn.2017.02.007.

16. Arrondo G, Solmi M, Dragioti E, et al. Associations between mental and physical conditions in children and adolescents: an umbrella review. Neurosci Biobehav Rev 2022;137:104662. https://doi.org/10.1016/j.neubiorev.2022.104662.

17. Spruyt K. Neurocognitive effects of sleep disruption in children and adolescents. Child Adolesc Psychiatr Clin N Am 2021;30:27–45. https://doi.org/10.1016/j.chc.2020.08.003.

18. Tarokh L, Saletin JM, Carskadon MA. Sleep in adolescence: physiology, cognition and mental health. Neurosci Biobehav Rev 2016;70:182–188. https://doi.org/10.1016/j.neubiorev.2016.08.008.

19. Alschuler KN, Jensen MP, Goetz MC, Smith AE, Verrall AM, Molton IR. Effects of pain and fatigue on physical functioning and depression in persons with muscular dystrophy. Disabil Health J 2012;5:277–283. https://doi.org/10.1016/j.dhjo.2012.07.002.

20. Hsieh HF, Shannon SE. Three approaches to qualitative content analysis. Qual Health Res 2005;15:1277–1288. https://doi.org/10.1177/1049732305276687.

21. Lincoln YS, Guba EG. Naturalistic inquiry Newberry Park: Sage Publications; 1985.

22. Birnkrant DJ, Bushby K, Bann CM, et al. Diagnosis and management of Duchenne muscular dystrophy, part 3: primary care, emergency management, psychosocial care, and transitions of care across the lifespan. Lancet Neurol 2018;17:445–455. https://doi.org/10.1016/S1474-4422(18)30026-7.

23. Jeronimo G, Nozoe KT, Polesel DN, Moreira GA, Tufik S, Andersen ML. Impact of corticotherapy, nutrition, and sleep disorder on quality of life of patients with Duchenne muscular dystrophy. Nutrition 2016;32:391–393. https://doi.org/10.1016/j.nut.2015.09.004.

24. Stroobach A, Wilson AC, Lam J, Hall GL, Withers A, Downs J. Factors influencing participation in home, school, and community settings by children and adolescents with neuromuscular disorders: a qualitative descriptive study. Dev Med Child Neurol 2022. Oct. 27. [Epub]. https://doi.org/10.1016/j.nut.2015.09.004.

25. Goodwin RD, Robinson M, Sly PD, et al. Severity and persistence of asthma and mental health: a birth cohort study. Psychol Med 2013;43:1313–1322. https://doi.org/10.1017/S0033291712001754.

26. O’Brien LM. The neurocognitive effects of sleep disruption in children and adolescents. Child Adolesc Psychiatr Clin N Am 2009;18:813–823. https://doi.org/10.1016/j.chc.2009.04.008.

27. Dohna-Schwake C, Podlewski P, Voit T, Mellies U. Non-invasive ventilation reduces respiratory tract infections in children with neuromuscular disorders. Pediatr Pulmonol 2008;43:67–71. https://doi.org/10.1002/ppul.20740.

28. Mellies U, Ragette R, Dohna Schwake C, Boehm H, Voit T, Teschler H. Long-term noninvasive ventilation in children and adolescents with neuromuscular disorders. Eur Respir J 2003;22:631–636. https://doi.org/10.1183/09031936.03.00044303a.

29. Alves RS, Resende MB, Skomro RP, Souza FJ, Reed UC. Sleep and neuromuscular disorders in children. Sleep Med Rev 2009;13:133–148. https://doi.org/10.1016/j.smrv.2008.02.002.

30. Fee RJ, Hinton VJ. Resilience in children diagnosed with a chronic neuromuscular disorder. J Dev Behav Pediatr 2011;32:644–650. https://doi.org/10.1097/DBP.0b013e318235d614.

Article information Continued

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Characteristic	Number
Child neuromuscular disorder diagnosis
Duchenne muscular dystrophy	8
Other^*	6
Child age (yr)
7–12	6
13–17	8
Sex of child
Male	10
Female	4
Nocturnal respiratory support
Continuous positive airway pressure	4
Bilevel positive airway pressure	4
Nil	6
Child ambulatory status
Non-ambulant	11
Ambulant	3
Pain score of the child on a usual day^†
Median (range)	2.75 (0–9)

Element	Child/adolescent and parent quotes
Cranky and argumentative	I was tired the next day and grumpy… and then telling everyone to get lost. – 14-year-old girl
	I won’t make a big deal about it. But if I’m tired, yeah, that’s a bit of a different story. – 16-year-old boy
	Oh, she can just be grumpy, like snappy. Rude, like really barky, barking orders. She is really sharp and not very polite. And yeah, that’s when I know she hasn’t had a very good night’s sleep. – Parent of 14-year-old girl
Impulsivity	Because he had had a poor night’s sleep he was running into things and kind of doing silly things, doing risky, silly things. And some might say they look like attention seeking behaviours, you know, he might try and push into a bookcase or throw things on the floor. – Parent of 14-year-old boy
Impulsivity	He actually gets from 0 to 100 quite easily. So that impulse control, he gets very agitated at school, and in his agitation, he actually can yell at the teacher, or he can yell at a friend. – Parent of 7-year-old boy
Obsessive behaviours	But I would definitely say that if he’s had a poor night’s sleep, the following day, we would expect to see more of those OCD and high anxiety behaviours. – Parent of 14-year-old boy
Obsessive behaviours	So, with [name], if things aren’t put back the way he wants it, he will be so obsessed about putting it back, and you’re better off just letting him put it back – Parent of 8-year-old boy
Meltdowns and tantrums	He had a big tantrum where he shouted back at the teacher ‘I’m not disrespectful.’ – Parent of 7-year-old boy
	He would just get upset really quickly. He will be happy staying at home and playing, but then when you suggest doing something different, he would get upset pretty quickly and then has a tantrum. – Parent of 7-year-old boy
	There have been times where, I think where he actually has thrown his tablet, before I have even taken it off him. So, they are the sort of meltdowns that he experiences. – Parent of 8-year-old boy

Element	Child/adolescent and parent quotes
Anxious and worried	Catastrophising yeah, I do that a lot. – 16-year-old boy
Anxious and worried	He has more anxiety on those days because he chews on the pencil a lot more. – Parent of 7-year-old boy
Comfort seeking	Cuddling up to me, sort of just lying on the couch, being close to me. Being close to her brother, having like a comfort toy with her – Parent of 7-year-old girl
Comfort seeking	He just wants to be all over me. He wants to be cuddled, and he’s very smoochie. Sometimes it can become quite sooky – Parent of 8-year-old boy
Irritability	Mostly just sad and I can come across a bit frustrated or angry – 16-year-old boy
Irritability	…Like the dog barking as well, it really irritates him, any kind of noise, or any kind of repeated information like ‘…don’t do this. Don’t do this. Don’t do this’ you know, repeated two, three times, it just really annoys him – Parent of 7-year-old boy
Sombre and withdrawn	I tend to just probably isolate myself a bit as much as I can, I just do my own thing. I don’t really want to talk to people and I’m normally very chatty – 16-year-old boy
Sombre and withdrawn	She is the complete opposite of a quiet person. But, when she’s sick, she’s not herself, and you can definitely tell that she’s sick, even at school they could tell when she starting to get sick. You can tell when [name] is sick, she is just very quiet, which is not normal for her. She becomes very tired. She’s not herself in any way shape or form. It’s almost like a mirror image or the dark side, you’ve got this light and vibrant person and then you’ve got this. – Parent of 7-year-old girl
Lack of enthusiasm and motivation	I’m like, really like tired and lazy. Like, I don’t really do anything and I’m not as energetic – 15-year-old girl
	When he had that chest infection back in March, it just flattened him, he’s just tired, he needs to sleep, like he’s clearly unwell. He doesn’t want to get out of bed, he wants to lay and make himself comfortable – Parent of 14-year-old boy
	He stopped talking much… He’s not excited. Yeah, he’s not enthusiastic, because he’s normally quite enthusiastic about stuff – Parent of 7-year-old boy

Element	Child/adolescent and parent quotes
Attention to task and concentration	Yeah, and concentration is probably a big thing. I struggle with homework because once I get home, I start to not be able to concentrate as well as I normally can. – 16-year-old boy
	[after commencing NIV] Yeah I definitely felt like I could do more in the day – 14-year-old girl
	He could be in a small group, perhaps reading a book and will find it difficult to stay on task with the book and will lose interest and will wander off around the room in his wheelchair looking for something to grab his interest. – Parent of 14-year-old boy
	[after commencing NIV] He was having a deeper sleep and he was sleeping longer. And I think that, he seems a lot better with his thinking and all of that the next day – Parent of 11-year-old boy
Capacity to engage	The mornings are always, same as probably anyone, mornings can be hard to get going – 16-year-old boy
	Yeah, so I didn’t engage with anyone – 14-year-old girl
	I would remember him getting up out of bed and he’d normally go straight to the lounge and sit there and yawn a bit and then like, wouldn’t be able to think about what he wanted on his toast. – Parent of 11-year-old boy
	Just generally, they say, well he’s obviously pretty outgoing and he’s suggestive with things and ideas and all that sort of stuff, he is so different to anyone else in the classroom. And I think they notice that change between when he’s well, and when he’s not well – Parent of 7-year-old boy
	Yes so, reluctant to engage in conversation, difficult to wake up and probably more emotional – Parent of 16-year-old boy
Indecision	Yeah, that metacognition. He will sometimes say things like, ‘I can’t make a decision. Can you just make one please?’ And, you know, shows that awareness that he knows he’s not ready for the day. – Parent of 16-year-old boy
Indecision	Indecisive… That’s a big one. But yeah, it could be anything, ‘would you like juice or milk with your tablets?’ ‘I don’t know.’ You know, if gets to that point where he’s really tired, we just pick – Parent of 16-year-old boy