Family functioning among adolescents with narcolepsy

Abstract

Background

Narcolepsy is a sleep disorder with no cure with onset typically during adolescence. Caring for an adolescent with a lifelong medical condition can negatively impact family structure, cohesion, relationships, and overall functioning. The primary objective of this study was to evaluate family functioning in a cohort of adolescents with narcolepsy using the PedsQL Family Impact Module. The secondary objective was to compare family functioning in adolescents with narcolepsy to adolescents with chronic pain based on published data.

Methods

This was a cross-sectional study of adolescents (aged 10 to 18 years) with narcolepsy. The narcolepsy group was recruited from The Hospital for Sick Children in Toronto, Canada. Family functioning was assessed by the PedsQL family impact module total scores, which was completed by the patient’s caregiver. The PedsQL family impact module yields a total scale which encompasses parent health-related quality of life, daily activities, family relationships, communication, and worry subscales. Lower scores suggest poorer family functioning. Secondary data analyses were used to compare participants’ family functioning to a cohort of adolescents with chronic pain.

Results

Thirty adolescents with narcolepsy participated (mean age=13.8 ± 2.2 years, 76.7% male). Family functioning was impaired in this cohort of adolescents with narcolepsy and similar to adolescents with chronic pain (64.0 ± 19.8 versus 64.7 ± 19.5; P=0.849).

Conclusion

Family functioning is impaired in adolescents with narcolepsy. Clinical teams should assess family functioning at routine clinic visits by asking about concerns and challenges related to caring for an adolescent with narcolepsy and providing resources and support as needed.

Paediatric conditions adversely affect the well-being of the entire family, particularly that of the caregivers (1). Caring for a child with a medical condition can impair family functioning by negatively impacting daily activities, family relationships, and parent health-related quality of life (HRQOL) (2). Family functioning varies across different paediatric conditions (3,4), and may be related to the course of the illness (e.g., progressive, constant, episodic), disease symptoms, side effects of treatment, and outcome (e.g., fatal, nonfatal) (4,5). Family functioning is also associated with demographic factors such as the patient’s age, being a single parent, parental education level, and the number of children in the home (5–7). Additionally, family functioning is associated with poor mental health outcomes (8) and suboptimal treatment adherence (9).

Research on family functioning in the paediatric narcolepsy population is limited. Narcolepsy is a lifelong sleep disorder with no cure that typically has its onset during adolescence (10). The incidence of paediatric narcolepsy has doubled since 2010(11), for reasons that are unclear (12). Symptoms of narcolepsy include excessive daytime sleepiness (EDS), which can manifest as irresistible sleep episodes that occur at inappropriate times (e.g., in public places, while socializing or eating) significantly impacting daily function (13). Patients with narcolepsy also experience cataplexy, which is the sudden loss of skeletal muscle tone causing weakness or complete collapse (13). Other symptoms include sleep paralysis—awakening unable to move, hallucinations, and disturbed nocturnal sleep (13). The time between disease onset and diagnosis can be a challenge in narcolepsy because of its rarity and lack of symptom recognition (14). Often, narcolepsy is only diagnosed after serious concerns such as failing grades at school or motor vehicle accidents (10). The diagnosis delay, constellation of symptoms and consequences are frustrating for both patients and their families alike (15). Family functioning has not been assessed in adolescents with narcolepsy using the PedsQL family impact module. This parent-report tool is useful because it quantifies how multiple areas of family functioning (parent HRQOL, family communication levels of worries, daily activities, and family relationships) are impacted by caring for a child with a medical condition. It is also unclear how family functioning in paediatric narcolepsy compares to families caring for children with other paediatric disease groups, such as chronic pain. Quantifying family functioning in paediatric narcolepsy and comparing it to another paediatric chronic disease group is important as it can contextualize level of impairment a family is experiencing (5,16). Increased understanding of family functioning in the paediatric narcolepsy population is important for clinicians, as they provide family-centred care to patients.

The primary objective of this study was to evaluate family functioning in a cohort of adolescents with narcolepsy using the PedsQL family impact module. The secondary objective was to compare family functioning in a cohort of adolescents with narcolepsy with a cohort adolescents with chronic pain based on published data.

PATIENTS AND METHODS

This was a prospective cross-sectional research study approved by the research ethics board at The Hospital for Sick Children (REB#1000055883) in Toronto, Canada. Patients with narcolepsy were recruited from sleep disorders clinics using the following criteria:

Inclusion criteria

• 1) Participants were required to be between 10 and 18 years of age. This age range was selected as it encompasses the World Health Organization definition of an adolescent (17).

• 2) Participants had to have a confirmed diagnosis of narcolepsy made by the sleep medicine team.

Diagnosis of narcolepsy

Patients with narcolepsy were referred to the sleep medicine team at our institution from community paediatricians or general practitioners. Symptoms were reported by patients and their families. The Epworth sleepiness scale, modified for use in paediatrics (18), was administered to all patients and their families to assess EDS at first clinical presentation, prior to receiving treatment. A label of EDS was made for any individual who scored greater than 10 on the Epworth sleepiness scale (the maximum score was 24). All patients underwent a full clinical examination, including a neurological examination. As per standard of clinical care, all patients with suspected narcolepsy had an overnight polysomnogram to rule out other sleep disorders followed by a daytime multiple sleep latency test with five 20-minute nap periods.

For a diagnosis of narcolepsy, patients had to meet the following criteria which were adapted from the International Classification of Sleep Disorders Diagnostic and Coding Manual (3rd edition) (19).

1. History consistent with narcolepsy (e.g., EDS)

2. Multiple sleep latency test consistent with narcolepsy; defined as two or more sleep onset rapid eye movement periods and a mean sleep latency < 8 minutes

3. Symptoms not explained by another sleep disorder, medical, and/or neurological disorder.

Cerebrospinal hypocretin levels were not assessed as they are not available as a clinical test in Canada.

Exclusion criteria

Participants unable to speak, read, or write English were not eligible to participate. To avoid potential confounding variables, patients with known global developmental delay or a sleep disorder other than narcolepsy (e.g., obstructive sleep apnea) were excluded from the study. All polysomnograms performed in adolescents with narcolepsy were rereviewed to ensure that patients did not have obstructive sleep apnea.

Secondary data analyses

For the secondary data analyses, data were extracted from one study assessing family functioning using the PedsQL family impact module in a cohort of adolescents with chronic pain (20). Approval was obtained from the author of this study to use their published data in a secondary data analyses. Additionally, appropriate Institutional Review Boards for this study and informed consent was obtained from participants.

MEASURES

Family functioning

The PedsQL family impact module is a valid and reliable 36-item questionnaire that was used to measure family functioning that is completed by parents/caregivers. The PedsQL family impact module gives a quantitative indicator of family functioning and parent’s self-reported HRQOL as a result of their child’s health. The PedsQL family impact module yields a total scale score, a parent HRQOL summary (physical, emotional, social, and cognitive functioning), family functioning summary score (daily activities and family relationships subscales), communication subscale score, and a worry subscale score. Parents/caregivers are asked to answer specific questions related to problems about family functioning on the following scale: (0-if it is never a problem, 1-if it is almost never a problem, 2-if it is sometimes a problem, 3-if it is often a problem, 4-if it is almost always a problem). Items are reversely scored and linearly transformed to a 0 to 100 scale so that higher scores on the module indicate better family functioning and less of a negative impact on the family as a result of their child’s health. Scale scores are computed as the sum of the items divided by the number of items. Total and subscale scores demonstrate acceptable to excellent internal consistency (Cronbach α 0.79 to 0.96) (21). Total scores were used as the measure of family functioning in this study as they encompassed items from all scales.

Patient’s demographic and clinical characteristics

Data were collected from narcolepsy patients’ medical records using a standardized case report form. This included current medications (if any), known comorbidities (if any) and patient reported cataplexy (yes or no), sleep paralysis (yes or no), and/or hallucinations (yes or no). The presence of coexisting obesity (yes or no) was calculated by the study team using the patient’s body mass index (BMI) z-score. BMI z score was calculated using measured height and weight according to age and sex-specific growth curves. Obesity was defined as a BMI z-score ≥2.0(22). Demographic data included patient age (years), sex (male or female), highest household education level (<postsecondary education or ≥postsecondary education), single parent household (yes or no), and number of children in household. Demographic characteristics (e.g., age) of the studies included in the secondary data analyses were also extracted. Detailed demographic and clinical characteristics can be found in Table 1.

Table 1.

Narcolepsy patient characteristics (N=30)

Demographic Variables
Age (years)	13.8 ± 2.2
Sex (% male)	76.7
Highest Household Education (%)*
•≤Post secondary	50
•>Post secondary	46.7
Single Parent Household (%Yes)	16.7
Number of Children in Household	2.2 ± 0.9
Clinical Variables
•Body Mass Index Z Score	1.4 ± 0.8
•Obesity (%)	23.3
Patient Reported Symptoms (%)
•Cataplexy	75.9
•Sleep paralysis	17.2
•Hypnagogic Hallucinations	10.3
Epworth Sleepiness Scale Score	13.1 ± 4.5
Patients prescribed medication for narcolepsy (% Yes)**	73.3

Values reported as mean and ± standard deviation unless otherwise specified.

*One patient’s family selected ‘refuse to answer’ for this question (N=29).

**Medication included modafinil (3.3%), Ritalin (26.7%), Dexedrine (6.7%), Strattera (3.3%), clomipramine (3.3%); 26.7% of the patients were not taking medication.

Statistical analyses

Descriptive statistics were applied to evaluate demographics information and PedsQL family impact module scores in the narcolepsy group, and were presented as mean (standard deviation [SD]) for continuous variables and frequency (percentage) for categorical variables. Comparison between the family functioning scores in the narcolepsy group to a cohort of adolescents with chronic pain was performed using student t-tests. Statistical analyses were performed using SPSS version 23.0 and an interactive statistics webpage (http://www.usablestats.com/calcs/2samplet&summary=1). Statistical tests were two-sided and significance level was set at 0.05.

RESULTS

Thirty-five adolescents with narcolepsy were eligible for this study based on the inclusion and exclusion criteria. Three eligible adolescents with narcolepsy and their families could not be contacted about the study (did not come to clinic/answer phone calls). One eligible family was not interested in participating in research. Thirty-one adolescents with narcolepsy and their families provided consent to participate. One adolescent and their family withdrew after consent due to the study time commitment. Detailed patient characteristics can be found in Table 1. The characteristics of the adolescents with chronic pain (N=458) can be found in the published study (20). In summary, adolescents with chronic pain were 13.7 ± 2.7 years of age, which were not significantly different from this cohort of adolescents with narcolepsy (P=0.813). Adolescents with chronic pain were experiencing pain for at least three months in forms such as headache (34%), musculoskeletal pain (26%), abdominal pain (23%), and complex regional pain syndrome (3%). Other reasons for pain (14%) included generalized pain, pain secondary to trauma, or an unclear pain diagnosis (20).

PedsQL family impact module total scores communication scores (e.g., talking about the child’s health to other people), and worry scores (e.g., worries related to treatment side-effects) can be found in Table 2. All scores in the narcolepsy group were similar to the chronic pain group. Scores in the worry domain were the lowest of all domains, which asks about worries related to medical treatments efficacy, treatment side-effects, others reaction to their child’s illness, illness affecting other family members, and the child’s overall future.

Table 2.

PedsQL family impact module scores in adolescents with narcolepsy and chronic pain

PedsQL Family Impact Module Scores	Narcolepsy (N=30)	Chronic Pain (N=458)	t test statistic	df	P value
Total	64.0 ± 19.8	64.7 ± 19.5	−0.188	32	0.849
Parent HRQOL	69.4 ± 19.8	67.4 ± 20.7	0.535	33	0.619
Family Summary (Relationships and Daily Activities)	64.4 ± 26.0	66.1 ± 23.4	−0.349	32	0.702
Worry	42.3 ± 21.8	46.8 ± 22.6	−1.092	33	0.282
Communication	70.4 ± 24.2	74.3 ± 23.9	−0.856	32	0.398

All values reported as mean and SD unless otherwise specified.

df Degrees of freedom; HRQOL Health-related quality of life.

DISCUSSION

This study evaluated family functioning using the PedsQL family impact module total scores among adolescents with narcolepsy and compared the scores with a cohort of adolescents with chronic pain using secondary data analyses. Family functioning in a cohort of adolescents with narcolepsy is impaired and similar to a cohort of adolescents with chronic pain.

Family functioning may be impaired in adolescents with narcolepsy because of the burden associated with caring for a child with a chronic condition, leaving less time for enjoyable activities (16,23). Family functioning in adolescents with narcolepsy may be comparable to adolescents with chronic pain, because both conditions have no cure, limited treatment options, and unpredictable symptoms (20,24). The unpredictable symptoms experienced by both groups of adolescents (e.g., cataplexy, pain) may severely interfere with the family’s daily activities (e.g., taking the child to school or attending social events as a family). The rarity of these conditions may also cause families to feel isolated and may find it difficult to relate to other families.

Scores in the worry domain of the PedsQL Family Impact Module were the lowest of all domains in the narcolepsy group. Worrying is a common issue among caregivers of children with chronic conditions, as their disease course is ambiguous and they are unable to predict their child’s health outcomes and overall future (25). Kippola-Paakkonen et al. reported that caregivers of paediatric narcolepsy patients worry about being unable to the help their own child and entire family cope with the illness as well as the lack of available support (26). Kippola-Paakkonen et al. asked families of paediatric narcolepsy patients about who they relied on for support with respect to their child’s condition, and found 77 per cent of the caregivers relied on their spouses and only 27 per cent relied on their child’s clinical team (26).

Clinicians caring for adolescents with narcolepsy should assess family functioning at routine clinic visits to address concerns and identify avenues of support for patients and their families. Families of patients with narcolepsy appreciate peer-support from other families affected by narcolepsy as it helps them feel understood and less isolated (26). One possible way that clinical teams can help families with narcolepsy feel supported is by hosting narcolepsy family education days or peer-support groups at their institution to provide patients and their families with an opportunity to connect and network with other families whom they can relate to. Clinical teams could also potentially address family worries by asking families what their worries are and what type of support they would like to receive (e.g., psychosocial, educational). For instance, caregivers of paediatric narcolepsy patients worry about their child’s setbacks in the school setting due to symptoms such as EDS and poor nocturnal sleep affecting academic success (26). Clinical teams may want to provide school-aged narcolepsy patients with a letter explaining the diagnoses and lead educational seminars on the condition for school personnel. Several limitations of this study should be taken into consideration. First, the sample size of the narcolepsy group is small and rendering potential limitations to the generalizability of the results, so future studies with larger sample sizes are needed to confirm these results. Second, family functioning is a complex construct with multiple determinants that could potentially confound results. Therefore, a more detailed presentation of sociodemographic characteristics (e.g., family income level, type, and level of both social and economic support that was available and utilized by the family) would have been useful. Another limitation is the interpretation of the PedsQL family impact module scores in the narcolepsy group, as to our knowledge there is no literature validating this tool in the paediatric narcolepsy population. As this was a cross-sectional study, the baseline family functioning (prior to the narcolepsy diagnoses) of patients is unknown. Longitudinal research is needed to understand family functioning over time, as families are constantly changing and adapting to illness related demands. Finally, secondary data analyses impose multiple limitations. It is difficult to compare both groups as there were inconsistencies on the reporting of socioeconomic and demographic characteristics.

This was the first study to quantify family functioning and parent HRQOL using the PedsQL Family Impact Module in the paediatric narcolepsy population. The PedsQL family impact module is a useful tool because it focuses on assessing family functioning in the context of caring for a child with a medical condition. Family functioning is a multi-dimensional construct that can be influenced by multiple factors such as parental employment, relationships, health and socioeconomic status (5). Other tools used to assess family functioning such as the Family Assessment Device (27) ask about family functioning in a more general context, which makes it difficult to gauge how the child’s medical condition itself is affecting the family. This study underscores challenges in family functioning in adolescents with narcolepsy and, therefore, the need for family-centred care in the clinical setting.

Funding Information: There are no funders to report for this submission.

Potential Conflicts of Interest: All authors: No reported conflicts of interest. All authors have submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest. Conflicts that the editors consider relevant to the content of the manuscript have been disclosed.

Institutional Address for all authors: The Hospital for Sick Children, 555 University Ave, Toronto, Ontario M5G 1X8, Canada.