Psychological distress and coping styles of caregivers of patients with amyotrophic lateral sclerosis: a longitudinal study

Abstract

Objectives: To assess psychological distress levels over time in caregivers of patients in the early stage of amyotrophic lateral sclerosis (ALS) and to investigate the association between caregivers’ coping styles and psychological distress over time. Methods: Fifty-four caregivers were followed in a RCT of patients in the early stage of ALS. Questionnaires were administered at baseline, 4, 7, and 10 months. Psychological distress was assessed using the Hospital Anxiety and Depression Scale (HADS) and task-, emotion-, and avoidance-oriented coping styles were identified with the short version of the Coping Inventory for Stressful Situations (CISS-21). Analyses were performed using linear mixed models. Results: Caregivers’ psychological distress increased by 0.24 points on the HADS per month (p = 0.01). An emotion-oriented coping style was positively associated with psychological distress in caregivers (b = 0.90, p < 0.01), but did not influence the development of psychological distress over time. The avoidance-oriented coping style and the task-oriented coping style were not significantly related to psychological distress. Conclusion: Feelings of distress increase in ALS caregivers during the course of the disease of the patient. Emotion-oriented coping is related to psychological distress; however, emotional coping and distress might represent overlapping constructs. Focusing on one coping style in the care for ALS caregivers may be too simplistic for the complex situations they face, as it ignores the fact that people may require different coping strategies in different situations.

Keywords:

• Caregivers
• psychological distress
• coping
• longitudinal
• ALS

Introduction

Due to the rapid and progressive decline in muscle function in amyotrophic lateral sclerosis (ALS), patients become increasingly dependent on others for the provision of care. Informal caregivers, mainly spouses, fulfill a major role in the care for patients with ALS (1). As patients’ disease progresses, caregivers can experience an increase in caregiver burden (2) and psychological distress (3). Psychological distress can be defined as a discomforting, emotional state in response to a stressor (4). Caregivers of patients with ALS are faced with all sorts of possible stressors during the disease course, such as the impact of the unfavorable prognosis, the lack of a curative treatment, the physical deterioration of the patient, cognitive or behavioral changes in the patient and accumulating caregiving demands (5,6). How caregivers respond to these stressors might influence the development of psychological distress (7).

The general manner in which someone attempts to manage a stressor is called coping style (8), and coping styles are relatively stable over time within individuals (9). In the literature, there is no consensus on the total number or nature of distinctions to describe coping styles (8). Lazarus and Folkman (10) suggested that there are two types of coping styles: emotion-orientated coping and task (or problem)-orientated coping. Emotion-oriented coping refers to emotional reactions directed toward oneself while the task-orientated coping is characterized by attempts to solve problems. Later, Endler and Parker (11) identified a third coping style: avoidance-oriented coping. Avoidance-oriented coping is characterized by attempts to avoid stressful situations either by engaging in other activities or by interacting with others. The use of each coping style seems to have its own advantages and disadvantages, dependent on the circumstances (8). In addition, the use of a specific coping style can be beneficial in the short term, but may lead to negative consequences in the long term (12). For example, caregivers who use an avoidance coping style may deny that their partner is diagnosed with a fatal progressive disease, which decreases their feelings of stress on the short term. However, using this coping style on the long term may lead to poor adjustment as the condition of the patient will continue to progress. Requesting professional support and assisting aids may be needed which requires the use of problem-focused coping strategies.

In previous coping research focused on caregivers of patients with traumatic brain injury and Alzheimer’s disease, applying an emotion-oriented coping style was associated with more symptoms of burden and anxiety (13,14). Siciliano et al. (15) investigated coping in 96 ALS caregivers and concluded that only emotional-oriented coping was related to increased levels of depression, anxiety, and burden. However, these studies are cross-sectional and do not provide information about the development of distress over time in relation to coping styles. More knowledge about the association between coping and distress over time could help to identify which caregivers are in need of support and could help to better tailor interventions to caregivers at risk. Therefore, the objectives of the current study are to describe caregivers' psychological distress levels over time and to determine the association of caregivers’ coping styles with psychological distress over time.

Methods

Participants and procedures

This longitudinal observational study was performed using the data of the FACTS-2-ALS trial in which patients with ALS and their caregivers were followed for 10 months (16). Five outpatient rehabilitation clinics in The Netherlands participated, and caregivers were enrolled between October 2009 and November 2014.

Only informal caregivers, who were the partners of the patients, were included in the trial. Patients’ inclusion criteria for the FACTS-2-ALS trial were: age between 18 and 80 years; life-expectancy of more than 1 year; predicted forced vital capacity of at least 80%; diagnosed at least one month with probable or definite ALS according to the revised El Escorial criteria (17) and having walking and cycling ability. Patients’ exclusion criteria were: severe cognitive impairment, disabling co-morbidity, and psychological disorder, all assessed by the rehabilitation physician using the Cumulative Illness Rating Scale (CIRS) (18). Both patient and caregiver had to have sufficient mastery of the Dutch language.

In this study, patients were randomized to receive either care as usual, usual care plus aerobic exercise therapy, or usual care plus cognitive behavioral therapy for dyads. Patients who scored less than 8 on the Hospital Anxiety and Depression Scale (HADS) were not included in the cognitive behavioral therapy group. Since dyads in the cognitive behavioral therapy group received an intervention aimed at reducing stress, these couples were excluded from analysis. Care as usual for patients with ALS and their next of kin in the Netherlands consists of multidisciplinary care offered by specialized ALS care teams according to international guidelines (19). These specialized ALS care teams include social workers and often also psychologists who offer psychosocial support. These psychosocial professionals provide mainly support to patients but also offer support to next of kin when psychosocial problems arise.

The Medical Ethics Committee of the University Medical Center Utrecht and all participating centers approved the study, which was performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki. All participants provided informed consent.

Measurements and data collection

Caregivers

To measure psychological distress in caregivers, the HADS total score was used (20). The questionnaire comprises 14 items about feelings of anxiety and depression over the past week. Items are scored on a 0–3 scale, with higher sum scores indicating a higher level of psychological distress. Cronbach’s alpha was 0.91 at baseline in the caregivers’ questionnaires, indicating good internal consistency. There are no strict cut-off criteria for the HADS (21). We used a cut-off of 12 points or higher to identify psychologically distressed caregivers, based on a cut-off for patients in outpatient rehabilitation (22).

The short version of the Coping Inventory for Stressful Situations (CISS-21) (11,23) was used to measure caregivers' preference in the use of each of the three coping styles: task-oriented coping, emotion-orientated coping, and avoidance-oriented coping. The questionnaire contains 21 statements on how to cope with a stressful situation. Each coping style is measured with seven statements structured on a 5-point Likert-scale from “strongly disagree” to “strongly agree.” Subscale scores range from 7 to 35, with higher scores indicating a preference for the use of a particular coping style. Cronbach’s alpha of the subscales at baseline ranged from 0.76 to 0.84, indicating acceptable to good internal consistency.

Patients

Patients’ disease severity was measured using the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R), a 12-item questionnaire with response categories ranging from 0 to 4. Lower scores indicate a higher level of disease severity (24).

Psychological distress was measured with the HADS, as in caregivers.

Data collection

Data were collected through questionnaires at baseline and after 4, 7, and 10 months.

Demographic variables and the CISS-21 were self-administered at home. The ALSFRS-R and the HADS were administered by a rehabilitation physician at baseline and by a research assistant at 4, 7, and 10 months.

Statistical analysis

Participant characteristics were summarized using means, standard deviations, medians and interquartile ranges for continuous variables and proportions for categorical variables. Statistical analyses were performed using linear mixed models. Since there was some variation in the timing of the follow-up measurements, we converted the moments of measurement into months after baseline based on the assessment date. To assess the relationship between each coping style at baseline and caregivers’ psychological distress over time, models with and without the interaction between time and coping style were fitted. The factors time and coping were centered prior to the interaction analysis. In order to account for individual trajectories, we assessed the need for a random intercept and slope in every model. A random intercept and random slope were only included if this improved the model fit based on the likelihood ratio test. Since longitudinal data on coping styles of the caregiver were available, the stability of coping was evaluated using linear mixed models. All statistical analyses were performed using IBM SPSS Statistics for Windows, version 22.0.

Results

Number of participants and follow-up

A total of 64 caregiver-patient couples were included in the FACTS-2-ALS trial, 10 of whom were enrolled in the CBT-group. Therefore, data from 54 caregivers were included in the current study. At the 4, 7, and 10 months measurements, 47, 41, and 38 caregivers completed the questionnaires, respectively. Of the total of 16 caregivers who did not complete the last questionnaire, 5 dropped out due to the death of the patient, 1 caregiver dropped out due to feelings of burden, 1 caregiver died, 1 caregiver did not receive the questionnaire, and 8 caregivers did not report a reason for not filling in the questionnaires.

Demographics

Table 1 reports the descriptive characteristics for caregivers and the ALS patients for whom they provided care. Almost half of the caregivers were psychologically distressed at baseline (n = 23, 42.6%). Mean HADS total scores at baseline were 10.8 (SD 7.2) and 7.7 (SD 4.6) for caregivers and patients, respectively. On average, caregivers scored highest on the task-oriented coping style at baseline compared to the other coping styles. Eighty-nine percent of the caregivers indicated using all three coping styles to some degree and eleven percent reported a preference for only two different coping styles.

Table 1 Baseline characteristics.

Caregivers (N = 54)
Age, years mean (SD)	58.7 (10.9)
Female, n (%)	38 (70.4)
Relationship status^a
Living together, n (%)	50 (94.3)
Living seperately, n (%)	3 (5.7)
Highest level of education^b
High school, n (%)	10 (19.2)
Vocational training, n (%)	19 (36.6)
College or university degree, n (%)	23 (44.2)
HADS total score, mean (SD)	10.8 (7.2)
Caregivers psychologically distressed, n (%)^c	23 (42.6)
CISS: task-oriented coping style, mean (SD)	24.3 (5.3)
CISS: emotion-oriented coping style, mean (SD)	15.2 (5.0)
CISS: avoidance-oriented coping style, mean (SD)	18.7 (5.6)
Patients (N = 54)
Age, years mean (SD)	60.2 (9.7)
Female, n (%)	15 (27.8)
Time since diagnosis, months median (Q1–Q3)	3.3 (2.2–5.1)
ALSFRS-R, mean (SD)	42.5 (3.8)
HADS total score, mean (SD)	7.7 (4.6)

N: number of caregivers or patients; SD: standard deviation; HADS: Hospital Anxiety and Depression Scale; CISS: Coping Inventory for Stressful Situations; Q1: first quartile; Q3: third quartile; ALSFRS-R: Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised. ^aOne caregiver did not answer the question.

^bTwo caregivers did not answer the question. ^cIdentified by HADS total score ≥ 12.

Course of psychological distress over time

Caregivers’ psychological distress increased significantly over time, with 0.24 points on the HADS per month (p = 0.01; Table 2). Including a random intercept and a random slope for time in this model resulted in a better fit of the model, indicating that the course of psychological distress differed between caregivers.

Table 2 Caregivers’ psychological distress over time (N = 54).

	b (95% CI)	SE b	p-Value
Intercept	10.61 (8.69–12.54)	0.96	<0.001
Change per month	0.24 (0.06–0.43)	0.09	0.012

Random effects: random intercept and random slope. Covariance structure: Autoregressive structure (1) Heterogeneous.

N: number of caregivers; CI: confidence interval; SE: standard error.

Table 3 Coping styles of caregivers over time (N = 54).

	b (95% CI)	SE b	p-Value
Emotion-oriented coping
Intercept	15.38 (14.09 to 16.67)	0.64	<0.001
Change per month	−0.10 (−0.22 to 0.02)	0.06	0.110
Task-oriented coping
Intercept	24.11 (22.74 to −25.50)	0.69	<0.001
Change per month	−0.25 (−0.41 to −0.09)	0.07	0.003
Avoidance-oriented coping
Intercept	18.59 (17.08 to 20.09)	0.75	<0.001
Change per month	−0.06 (−0.18 to 0.06)	0.06	0.314

Random effects: random intercept and random slope. Covariance structure: Autoregressive structure (1) Heterogeneous.

N: number of caregivers; CI: confidence interval; SE: standard error.

Coping in relation to distress

The stability of the preference for the use of each coping style was analyzed (Table 3). Scores on emotion-oriented and avoidance-oriented coping did not change over time. The preference for the use of task-oriented coping style decreased over the study period (b = -0.25, p = 0.003).

The results of the analyses assessing the association of caregivers’ coping styles with psychological distress over time are presented in Table 4. These results show that higher scores on the emotion-oriented coping style subscale are associated with psychological distress in caregivers (b = 0.90, p = 0.001). The interaction between emotion-oriented coping style and time was not significant, indicating that the scores on the emotion-oriented coping subscale did not influence the development of distress over time. The task-oriented coping style (p = 0.24) and the avoidance-oriented coping style (p = 0.63) were not significantly associated with psychological distress in caregivers.

Table 4 Coping styles associated with caregivers’ psychological distress over time (N = 54).

	b (95% CI)	SE b	p-Value
Model 1^a
Intercept	10.75(9.20 to 12.30)	0.77	<0.001
Change per month	0.22 (0.30 to 0.41)	0.10	0.026
Change per point on emotion-oriented coping	0.90 (0.64 to 1.17)	0.13	<0.001
Model 2^a
Intercept	10.75 (8.84 to 12.65)	0.95	<0.001
Change per month	0.24 (0.06 to 0.43)	0.34	0.012
Change per point on task-oriented coping	−0.21 (−0.56 to 0.14)	0.17	0.237
Model 3^a
Intercept	10.63 (8.71 to 12.55)	0.96	<0.001
Change per month	0.24 (0.06 to 0.43)	0.34	0.012
Change per point on avoidance-oriented coping	−0.08 (−0.42 to 0.25)	0.17	0.631

Random effects: random intercept and random slope. Covariance structure: Autoregressive structure (1) Heterogeneous.

N: number of caregivers; CI: confidence interval; SE: standard error.

^a The interaction between time and coping style was not significant and did not improve the fit of the model

Discussion

The levels of psychological distress increased in caregivers over the 10 months of follow-up. Furthermore, our results indicate that caregivers with an emotion-oriented coping style experience higher levels of psychological distress, but this coping style does not influence the development of distress over time. No significant relations were found between use of task-oriented coping and avoidance-oriented coping and psychological distress. Moreover, this study suggests that there is variety in the development of distress between caregivers which emphasizes the need to monitor the levels of psychological distress in individual caregivers of patients with ALS.

Our results are in contrast to two previous longitudinal studies in ALS caregivers that reported no change in distress over time (3,25). This might be due to the fact that we included caregivers of patients who were diagnosed more recently (median of 3.3 months) (3,25). The increase in levels of distress shortly after the diagnosis may be caused by changes in the perspective of the future and the confrontation with the consequences of the disease. This is comparable to research findings in caregivers of cancer patients, which shows that having a partner who has recently been diagnosed with a potentially life-threatening disease is associated with psychological distress (26,27).

The relationship between the use of an emotion-oriented coping style and caregivers’ psychological distress was also found in studies in caregivers of patients with ALS (15), dependent older persons (28), and in healthy adults (Endler et al. 29). Emotion-oriented reactions such as emotional responses, self-preoccupation or fantasizing could possibly lead to emotional complaints, which could accumulate over time, resulting in psychological distress. Another explanation for the relation between emotional coping and distress might be related to the content of the items. Items of the emotion-orientated scale of the CISS refer to self-blame, experiencing worry and feeling upset and seem to be contaminated with distress (e.g. “Become very upset”) (30). It is likely that overlap between the content of the items is responsible for the associations between the CISS emotion-oriented scale and distress found in both the present study and in previous studies.

Similar to the findings of a cross-sectional study among caregivers of patients with ALS (15), the current study showed that task-oriented and avoidance-oriented coping styles were not related to psychological distress. In caregivers of patients with dementia, task-oriented coping was associated with a decrease in psychological distress (31,32). This protective effect of the task-oriented coping style was not, however, found in caregivers of patients with ALS. Furthermore, the preference for using the task-oriented coping style was found to decrease over time. Although we should interpret these results cautiously, this might be related to the rapidly progressing disability of the patient which constantly leads to new challenges that often cannot be solved. Continuing to use a task-oriented coping style might be difficult in these circumstances. In addition, caregivers and patients are repeatedly informed that they are not able to influence the disease progression of ALS, which may diminish the use of a task-oriented coping style.

Another possible explanation for not finding a relationship between these coping styles and distress might be the way we measured coping. The CISS provides a general inventory of coping styles used. However, in order to manage the complex situation of caring for an ALS patient, a wide range of coping strategies might be needed, depending on the situation. Recent approaches have recognized the importance of situation-specific variations in coping. Being able to effectively modify one’s coping strategies according to the demands of different stressful situations is called coping flexibility (33). Coping flexibility seems to lead to more adaptive outcomes and improved well-being (33). Focusing on one coping style in the care for these ALS caregivers may be too simplistic for the complex situations they face. Care professionals may support caregivers by focusing on situations that are related to feelings of distress. Coaching caregivers in applying different coping strategies in these different stressful situations might be beneficial for the wellbeing of these caregivers. However, further research is needed to investigate the applicability of coping flexibility in ALS caregivers.

Strengths and limitations

A strength of our study was the longitudinal design and the inclusion of patients in the early stage of the disease, which provided insights into the development of distress in caregivers during the first months of the disease trajectory and into when supportive interventions may be needed.

This study has several limitations. Because we conducted a secondary analysis on data of a randomized controlled trial, the study population was limited to the in- and exclusion criteria used in the trial, which were primarily based on patients. Consequently, it may not be correct to fully generalize the findings in our study population to the entire population of caregivers of ALS patients. Furthermore, we could not use data from the 10 patient-caregiver couples of the CBT-group, in which caregivers and patients were randomized if patients had higher psychological distress levels. Since psychological distress levels in patients and caregivers are correlated (34), the psychological distress levels we found might be an underestimation of psychological distress in the population as a whole.

The small sample size allowed only a limited number of covariates in our analysis. Due to these limitations, future studies are required to test the generalizability of the results and to determine the role of other caregiver factors (e.g. self-efficacy and professional support received) and patient factors (e.g. disease severity and cognitive and behavioral changes) in relation to the development of distress in caregivers.

Conclusions

Caregivers of patients with ALS experience increasing levels of psychological distress in the first phase of the disease. Over 40% of the caregivers seem to be psychologically distressed. Therefore, we advocate the monitoring of caregivers’ distress symptoms over time by the multidisciplinary ALS care team in order to identify when supportive interventions are needed. Emotional coping is related to increased levels of psychological distress, but did not influence the development of distress over time. However, emotional coping and distress seem to be overlapping constructs. Measuring coping styles in general and tailoring the support to this coping style, may not do justice to the complex situation of ALS caregivers. As caregivers are confronted with various stressful situations in which they might need to apply different coping strategies, more knowledge about coping flexibility is required.

Declaration of interest

The authors report no conflict of interest.

Availability of data

The data that support the findings of this study are available from the corresponding author, JMAVM, upon reasonable request.

Additional information

Funding

The research was supported by The Netherlands Organization for Health Research and Development (ZonMw) and the Prinses Beatrix Spierfonds.