Coping with Huntington’s Disease in Patients and At-Risk Individuals

Abstract

Background:

Huntington’s disease (HD) presents patients and individuals at risk for HD with significant levels of stress. However, relatively little research has examined how individuals cope with stress related to the disease or the association of specific coping strategies with psychological symptoms.

Objective:

This study examined the ways in which HD patients and at-risk individuals cope with HD-related stress using a control-based model of coping and the association of coping strategies with symptoms of depression and anxiety.

Methods:

HD patients (n = 49) and at-risk individuals (n = 76) completed the Responses to Stress Questionnaire – Huntington’s Disease Version to assess coping strategies in response to HD-related stress, as well as standardized measures of depression and anxiety symptoms. Patient health records were accessed to obtain information related to disease characteristics.

Results:

Patients and at-risk individuals reported using comparable levels of primary control coping, secondary control coping, and disengagement coping strategies. In linear regression analyses, only secondary control coping was significantly associated with lower depression (β= –0.62, p < 0.001) and anxiety (β= –0.59, p < 0.001) symptoms in patients and at-risk individuals (β= –0.55, p < 0.001 and β= –0.50, p < 0.001, respectively).

Conclusions:

Secondary control coping may be beneficial for both HD patients and at-risk individuals. Future research using the control-based model of coping in longitudinal studies with the HD population is needed, and future interventions could test the effects of cognitive reframing and acceptance as coping strategies for families affected by HD.

Keywords

Huntington disease coping depression anxiety

INTRODUCTION

Chronic illnesses can place a substantial burden of stress on patients and their families. Huntington’s disease (HD) is a heritable neurodegenerative disease characterized by progressive cognitive, motor, and psychiatric dysfunction that can lead to significant levels of stress for patients and family members. 1 Clinical diagnosis of the disease is typically determined based on the onset of motor symptoms, most frequently occurring in the fourth to fifth decades of life, a time that can be defined for many individuals as critical child-rearing years. Further, because of the autosomal dominant inheritance pattern, children whose parents have the disease are placed at a particular risk for symptoms of depression and anxiety due to their own risk for developing the disease and a chronically stressful environment associated with the disease.2,3, 2,3

Previous research has suggested that individuals at risk for HD commonly experience stressors involving concerns about the future, increased care-taking responsibilities, and feelings of isolation, while HD patients commonly face stressors surrounding symptoms and progression of the disease, uncertainty about the future, and interpersonal effects of the disease on their family members.4,5, 4,5 Further, the cumulative number of stressors experienced by both HD patients and at-risk individuals are related to levels of depression and anxiety. 5 Psychological difficulties in HD patients present in a multitude of ways, including symptoms of apathy, depression, irritability, anxiety, aggression, psychosis, and obsessive-compulsive behaviors.1,2,6–8 , 1,2,6–8 Among the most common psychological difficulties in HD include symptoms of depression and anxiety.1,2, 1,2 While some studies suggest difficulties such as depression and apathy increase over the course of the disease,6,7, 6,7 others find significant heterogeneous patterns of progression.9 –11 Further, research suggests that psychological difficulties often precede the onset of motor symptoms,8,10, 8,10 making assessment and identification of such difficulties critical in at-risk and pre-manifest individuals. However, it remains unclear how psychological difficulties present and progress throughout the course of disease. Despite the potentially high stress burden and prevalence of psychological difficulties faced by HD patients and family members, there is relatively little empirical research examining how patients and at-risk individuals cope with stressors related to the disease and how coping may be related to psychological difficulties associated with HD.

As noted by Zarotti et al., 12 previous research has largely adopted a biomedical framework regarding psychological difficulties in HD, attributing psychological symptoms such as depression and anxiety to underlying pathophysiological changes occurring in the brain. However, in the present study, we view such psychological difficulties as multifactorial in nature, strongly impacted by cognitive functioning and stress and potentially moderated by how individuals cope with stressors in their lives. The primary aim of this study was to learn how HD patients and at-risk individuals cope with HD-related stress and how their coping responses may be associated with symptoms of depression and anxiety. Identifying the coping strategies used by patients and at-risk individuals and examining their correlates with psychological difficulties could provide critical insight for further research and interventions in HD.

The current study used a well-validated control-based model of coping that includes three factors: primary control engagement coping, secondary control engagement coping, and disengagement coping.13 –15 Primary control coping involves efforts to engage with or change the stressor or one’s emotions and includes strategies such as problem-solving, emotional expression, and emotion regulation. Secondary control coping involves efforts to adapt to a stressor and includes strategies such as cognitive reframing, acceptance, positive thinking, and distraction. Importantly, both primary and secondary control coping involve efforts that acknowledge and engage with a stressor or one’s emotional response to the stressor. Conversely, disengagement coping involves efforts to behaviorally or psychologically evade a stressor and includes strategies such as avoidance, denial, and wishful thinking. This conceptual model is based in theory emphasizing the distinction between voluntary (i.e., coping) and involuntary (i.e., reactivity) responses to stress and characteristics of the experienced stressor. 14 This three-factor structure of coping has been supported by confirmatory factor analysis in studies of adolescents and adults coping with a wide range of medically-related stressors.15 –17

Relatively few studies have investigated coping strategies in patients and family members affected by HD. Five studies have examined the control-based model of coping with HD.18 –22 One qualitative study found that individuals at risk or tested for HD reported using both primary control and secondary control coping strategies; additionally, at-risk individuals reported engaging in more secondary control coping strategies than primary control coping strategies. 18 In two empirical studies, secondary control coping was associated with lower symptoms of depression in patients with HD 19 and individuals at risk for the disease. 20 Further, in two studies examining at-risk offspring of parents affected by HD communicating with their parent about HD-related stress, disengagement coping strategies were related to higher observed negative affect and poorer communication skills in at-risk offspring.21,22, 21,22 Additionally, both primary and secondary control coping were positively related to more adaptive communication skills. Only one study examined all three forms of coping simultaneously (see 22 ). Other studies of coping with HD have been guided by different models of coping and have found that some specific coping strategies (e.g., emotional and instrumental support, positive reframing, acceptance) are associated with lower symptoms of depression and anxiety and better mental health in HD patients.23,24, 23,24 Conversely, other coping strategies (e.g., venting, self-blame, behavioral and mental disengagement) have been associated with higher symptoms of depression and anxiety and poorer mental health.23,24, 23,24 Collectively, these studies suggest that the coping strategies used by HD patients and at-risk individuals have implications for psychological symptoms and mental health.

Prior research in other populations of chronically ill adult patients supports the association of lower depression and anxiety symptoms with the use of primary control coping strategies in patients who perceive more control over their disease.16,25, 16,25 In contrast, secondary control coping has been shown to be related to lower psychological symptoms in both offspring and patients with a neurodegenerative disease.19,20, 19,20 On the other hand, disengagement coping has been shown to relate to higher levels of psychopathology across adults and adolescents coping with illness.16,25, 16,25 Guided by the control-based model of coping and based on these previous findings, we hypothesized that both primary control and secondary control coping would be related to fewer symptoms of depression and anxiety in HD patients, and only secondary control coping would be related to fewer symptoms of depression and anxiety in at-risk individuals. Further, we hypothesized that disengagement coping would be related to higher symptoms of depression and anxiety in both patients and at-risk individuals. Lastly, we included age, sex, and patient cytosine-adenine-guanine (CAG) repeats, a measure of disease severity, in regression analyses to explore the potential influence of these variables on depression and anxiety.

MATERIALS AND METHODS

Participants

The total sample included 57 HD patients and 81 at-risk individuals. A total of n = 8 patients had at least one piece of data missing, including CAG repeats (n = 2), coping data (n = 1), psychological data (n = 2), and both coping and psychological data (n = 3). Patients with any missing data did not differ from those with complete data on any study variables (e.g., CAG repeats, coping factors, psychological symptoms). Five at-risk individuals were missing both coping and psychological symptom data. At-risk individuals with missing data did not differ from those with complete data on any demographic variables (e.g., age or sex). The final sample for analyses included 49 HD patients and 76 at-risk individuals.

Procedure

Participants were recruited through a Huntington’s Disease Society of America Level 1 Center of Excellence (COE) in the southeastern U.S. Participants were recruited as part of a larger study designed to investigate psychological and cognitive functioning in families affected by HD. For the larger study, eligibility criteria for HD patients included fluency in English, confirmed HD genetic expansion by genetic testing, and having at least one biological child between the ages of 7 and 39 years old. All patients recruited in the larger study are included in the present sample, regardless of their HD stage. Therefore, the present sample of HD patients ranges on a continuum from premanifest to motor-manifest HD. Eligibility criteria for at-risk individuals included fluency in English, having a biological parent with the HD genetic expansion confirmed by genetic testing, and being between the ages of 7 and 39 years old. In the present study, at-risk individuals younger than the age of 10 were excluded from the sample because these individuals lack self-report survey data. Additionally, individuals who had undergone genetic testing for HD prior to their study visit, and were therefore no longer considered at risk, were excluded from the present sample.

Eligible participants were identified by a member of the medical team at the COE, who made the initial study introduction. Informed consent and assent were obtained prior to study participation. Questionnaires were administered via REDCap, a secure, online website. 26 Participants were compensated after completion of the study visit. The study was reviewed and approved by the medical center Institutional Review Board (IRB #181691).

Measures

Demographic information. Patients and at-risk individuals provided information on their age, sex, race, and highest level of education.

HD characteristics. Patient medical records were reviewed to obtain information about number of CAG repeats.

Coping. The Responses to Stress Questionnaire – Huntington’s Disease Version (RSQ-HD)14,19–22 , 14,19–22 was used to assess patient and at-risk individual HD-related stressors and coping mechanisms. The RSQ includes 57 items and yields three factors of coping: primary control coping, secondary control coping, and disengagement coping. In addition, the RSQ also yields two factors that assess involuntary responses to stress, which are not included in the present analyses. To control for possible response bias in item endorsement, proportion scores were calculated for each factor by dividing the total score for each coping factor by the total RSQ score. 14 The RSQ has been used with HD patients and at-risk individuals previously,5,19–22 , 5,19–22 and has demonstrated excellent internal consistency, test-retest reliability, and convergent and construct validity. Internal consistency for the coping factors ranged from α= 0.77 to 0.87 for patients and α= 0.79 to 0.80 for at-risk individuals.

Depression and anxiety symptoms. Patient depression symptoms were assessed using the Patient Health Questionnaire-9 (PHQ-9), 27 which consists of 9 items reflecting the symptoms of major depressive disorder based on DSM-IV criteria. Patient anxiety symptoms were assessed using the Generalized Anxiety Disorder-7 (GAD-7), 28 which consists of 7 items reflecting symptoms of generalized anxiety disorder based on DSM-IV criteria. For both measures, patients rated each item from 0 to 3, based on how much each symptom had bothered them in the past 2 weeks (0 = not at all; 1 = several days; 2 = more than half the days; 3 = nearly every day). Cut-off scores for the PHQ-9 are minimal (0–4), mild (5–9), moderate (10–14), moderately severe (15–19), and severe (20–27) depression. Cut-off scores for the GAD-7 are minimal (0–4), mild (5–9), moderate (10–14), and severe (15–21) anxiety. The PHQ-9 and GAD-7 have been widely used across clinical populations and with HD patients previously.19,22,29 , 19,22,29 Internal consistency for the PHQ-9 and GAD-7 in the current sample was α= 0.85 and α= 0.90, respectively.

At-risk individuals’ depression and anxiety symptoms were assessed using the Depressive Problems and the Anxiety Problems subscales of the Youth Self Report (YSR) 30 and the Adult Self Report (ASR). 31 Individuals ages 10–17 completed the YSR and ages 18 and older completed the ASR. The YSR and ASR include 112 and 126 items, respectively, which reflect a checklist of symptoms and behaviors. Respondents are asked to rate how much each statement describes them within the past 6 months on a 3-point scale (0 = not true; 1 = somewhat or sometimes true; 2 = very true or often true). The YSR and ASR Depressive Problems and Anxiety Problems subscales produce normative T scores (M = 50, SD = 10) derived from a nationally representative sample of adolescents and adults. Of note, the ASR is appropriate for individuals aged 18 to 59; therefore, patients were given different measures to assess symptoms of depression and anxiety due to the wide age range of our sample. The YSR and ASR have demonstrated excellent internal consistency, test-retest reliability, and construct validity. Internal consistency for the Depressive Problems subscale in the current sample was α= 0.81 (YSR) and α= 0.89 (ASR) and for the Anxiety Problems subscale was α= 0.74 (YSR) and α= 0.77 (ASR).

Statistical analyses

All statistical analyses were computed using SPSS (version 29). Descriptive statistics were calculated for all measures. Associations of coping mechanisms with symptoms of depression and anxiety were tested using bivariate Pearson correlations. Corrections for multiple comparisons were made using the Bonferroni adjustment. The significance threshold following Bonferroni adjustments was 0.008 (0.05/6 comparisons) for patient and at-risk individual correlations. Linear regression analyses were then conducted to examine coping mechanisms as predictors of both depression and anxiety symptoms. Age and sex were included as covariates in all regression analyses, and patient analyses also included CAG repeats as a covariate. All data for continuous variables approximated normal distributions, with all skewness and kurtosis statistics within the normal range. All analyses used two-tailed tests to determine statistical significance.

RESULTS

Descriptive statistics

The final sample included 49 HD patients and 76 at-risk individuals. Table 1 presents descriptive statistics for all key participant characteristics. Patients (n = 49) ranged from 31 to 72 years old (M = 47.18, SD = 9.72) and were 55% female. Patients had a mean CAG repeat length of M = 43.53 (SD = 2.85). Most of the patients (98%) identified as White, which is characteristic of the HD population at large. 32 Patients came from a wide range of educational backgrounds: no high school degree (6%), GED or high school graduate (25%), partial or completed college education (49%), to graduate education (18%). One patient (2%) reported other education.

Table 1

Descriptive statistics for key study variables in patients and at-risk individuals

	Patients			At-Risk Individuals
Variable	M	SD	Range	M	SD	Range
Age	47.18	9.72	31–72	18.84	7.09	10–38
CAG Length	43.53	2.85	38–50	–	–	–
Primary Control Coping	0.18	0.04	0.11–0.26	0.17	0.04	0.08–0.28
Secondary Control Coping	0.26	0.06	0.11–0.39	0.28	0.06	0.12–0.41
Disengagement Coping	0.14	0.03	0.07–0.21	0.15	0.03	0.09–0.25
Depression Symptoms	8.86	5.68	0–19	57.55	9.00	50–88
Anxiety Symptoms	7.24	6.01	0–21	56.68	7.03	50–78

Patients’ reported use of primary control coping strategies ranged from 0.11 to 0.26 (M = 0.18, SD = 0.04), secondary control coping strategies ranged from 0.11 to 0.39 (M = 0.26, SD = 0.06), and disengagement coping strategies ranged from 0.07 to 0.21 (M = 0.14, SD = 0.03). Patients’ depression scores ranged from 0 to 19 (M = 8.86, SD = 5.68) and anxiety scores ranged from 0 to 21 (M = 7.24, SD = 6.01), with both means in the range for mild depression and mild anxiety. Approximately half (51%) of patient depression scores were in the minimal to mild depression range and 49% were in the moderate to moderately severe depression range. No patient met criteria for severe depression. Conversely, nearly three-quarters (71%) of patient anxiety scores were in the minimal to mild anxiety range, 10% were in the moderate anxiety range, and 18% were in the severe anxiety range.

At-risk individuals (n = 76) ranged from 10 to 38 years old (M = 18.84, SD = 7.09), were 58% female, and mostly (95%) identified as White. At-risk individuals’ education backgrounds similarly had a wide range: 8th grade or less (30%), no high school degree (25%), high school graduate (13%), some college (11%), associate’s degree (5%), bachelor’s or RN degree (12%), and master’s degree (4%).

At-risk individuals’ reported use of primary control coping strategies ranged from 0.08 to 0.28 (M = 0.17, SD = 0.04), secondary control coping strategies ranged from 0.12 to 0.41 (M = 0.28, SD = 0.06), and disengagement coping strategies ranged from 0.09 to 0.25 (M = 0.15, SD = 0.03). At-risk individuals’ T scores for depression symptoms ranged from 50 to 88 (M = 57.55, SD = 9.00) and for anxiety symptoms ranged from 50 to 78 (M = 56.68, SD = 7.03). Both mean depression symptoms and anxiety symptoms were slightly elevated, at over half a standard devation greater than the normative mean. For all at-risk individuals, about 12% and 5% were in the clinical range (T = 70 or greater) for depression and anxiety problems, respectively.

Correlates of patients’ coping

Table 2 shows bivariate Pearson correlations between coping mechanisms and depression and anxiety symptoms in patients. Primary control coping was negatively correlated with depression symptoms (r = –0.34, p = 0.016) and anxiety symptoms (r = –0.39, p = 0.006). Secondary control coping was significantly negatively correlated with depression symptoms (r = –0.59, p < 0.001) and anxiety symptoms (r = –0.60, p < 0.001). Disengagement coping was positively correlated with depression symptoms (r = 0.33, p = 0.020) and anxiety symptoms (r = 0.33, p = 0.021). After correcting for multiple comparisons, only the associations between primary control coping and anxiety symptoms and secondary control coping and depression and anxiety symptoms remained significant. Linear multiple regression analyses predicting depression and anxiety symptoms are shown in Table 3. Age, sex, and CAG repeats were included as covariates. After including all three coping mechanisms, only secondary control coping was significantly associated with lower depression symptoms (β= –0.62, p < 0.001) and anxiety symptoms (β= –0.59, p < 0.001).

Table 2

Correlations between coping and psychological symptoms in patients and at-risk individuals

	Patients		At-Risk Individuals
	Depression	Anxiety	Depression	Anxiety
	Symptoms	Symptoms	Symptoms	Symptoms
Primary Control Coping	–0.34^*	–0.39^**	–0.15	–0.16
Secondary Control Coping	–0.59^**	–0.60^**	–0.45^**	–0.49^**
Disengagement Coping	0.33^*	0.33^*	0.03	0.13

^*p < 0.05, ^**p < 0.01.

Table 3

Regression analyses predicting depression and anxiety symptoms in patients and at-risk individuals

	Depression Symptoms		Anxiety Symptoms
	b	β	b	β
Patients	F = 5.37^**, R² = 0.43		F = 5.39^**, R² = 0.44
Intercept	55.24^*	–	52.92^*	–
Age	–0.15	–0.25	–0.13	–0.21
Sex	0.98	0.09	1.16	0.10
CAG repeats	–0.62	–0.31	–0.49	–0.23
PCC	–7.38	–0.05	–25.25	–0.18
SCC	–55.18	–0.62^**	–55.65	–0.59^**
DC	16.98	0.09	0.48	0.00
At-risk Individuals	F = 4.61^**, R² = 0.25		F = 5.02^**, R² = 0.26
Intercept	94.10^**	–	80.79^**	–
Age	0.02	0.02	–0.12	–0.12
Sex	–2.12	–0.12	–0.06	–0.00
PCC	–20.60	–0.09	–9.87	–0.05
SCC	–79.66	–0.55^**	–57.03	–0.50^**
DC	–65.91	–0.21	–27.44	–0.11

PCC, primary control coping; SCC, secondary control coping; DC, disengagement coping. ^*p < 0.05, ^**p < 0.001.

Correlates of at-risk individuals’ coping

Table 2 shows bivariate Pearson correlations between coping mechanisms and depression and anxiety symptoms in at-risk individuals. Primary control coping was not significantly correlated with either depression or anxiety symptoms. Secondary control coping was significantly negatively related to depression symptoms (r = –0.45, p < 0.001) and anxiety symptoms (r = –0.49, p < 0.001). Disengagement coping was not significantly correlated with either depression or anxiety symptoms. Both associations for secondary control coping remained significant following correction for multiple comparisons. Linear multiple regression analyses predicting depression and anxiety symptoms from all three coping mechanisms are shown in Table 3. After controlling for age and sex, only secondary control coping was significantly associated with lower depression symptoms (β= –0.55, p < 0.001) and anxiety symptoms (β= –0.50, p < 0.001).

DISCUSSION

The primary aim of the current study was to understand how HD patients and at-risk individuals cope with stressors related to the disease and examine the associations of coping strategies with symptoms of depression and anxiety. Results show that both patients and at-risk individuals on average report using secondary control coping strategies (e.g., cognitive reframing, acceptance, distraction) proportionally highest out of all three coping factors. Partial support was found for our hypothesis regarding associations between HD patient coping and symptoms of depression and anxiety. While both primary control and secondary control coping were related to lower symptoms of depression and anxiety in bivariate correlations in patients, only secondary control coping was significantly associated with lower psychological symptoms in the context of other coping strategies. In at-risk individuals, full support was found for our hypothesis that only secondary control coping would be related to fewer symptoms of depression and anxiety. Our hypotheses that disengagement coping would be related to higher symptoms of depression and anxiety in patients and at-risk individuals only had partial support in patient bivariate correlations. Disengagement coping was not significantly related to symptoms of depression and anxiety in regression analyses for patients or at-risk individuals.

Secondary control coping, examined in the context of additional coping strategies, was the only significant predictor of depression and anxiety symptoms for both patients and at-risk individuals. Secondary control coping involves the use of strategies including cognitive reframing, acceptance, positive thinking, and distraction as efforts to adapt to a stressor. Both patients and at-risk individuals likely benefit from engaging in such strategies in the face of HD-related stress because HD may be perceived as both unpredictable and uncontrollable. The use of secondary control coping strategies may provide benefit to patients when they experience worsening symptoms or to at-risk individuals when they consider their own risk for the disease or observe a family member’s declining health. This pattern of findings mirrors those found in studies examining this control-based model of coping in other contexts. For example, studies have found that breast cancer patients have lower symptoms of depression when they engage in higher secondary control coping. 16 Further, symptoms of depression and anxiety were lower in children of depressed parents who engaged in greater secondary control coping strategies. 33 In many circumstances, secondary control coping proves to be beneficial for both patients with a disease and children in families affected by a chronic illness. The present study in our sample of HD patients and at-risk individuals echoes these findings from other populations.16,33, 16,33

On the other hand, primary control coping involves efforts to engage with or change a stressor or one’s emotional response through the use of strategies including problem-solving, emotion regulation, or emotional expression. In HD patients, primary control coping strategies may include communicating with doctors about their symptoms and managing the quality of their care, including decisions about their medical care and medication adjustments. Results showed that while primary control coping was related to lower symptoms of depression and anxiety in correlational analyses, it was not significantly associated with depression and anxiety symptoms for patients when examined in the context of secondary control and disengagement coping. It is possible that patients do not perceive themselves to have a significant amount of control over the progression of the disease, and therefore, they engage in fewer strategies aimed at engaging with or changing their symptoms or medical care or these strategies may be less effective in reducing their distress. Primary control coping was also not significant in correlational or regression analyses for at-risk individuals. While this finding in patients contrasts with results of other studies examining primary control coping in adults with a chronic illness, 16 this result in at-risk individuals supports other studies examining children of parents with major depressive disorder. 33

Finally, results for disengagement coping were only found for HD patients in the present study. Disengagement coping involves efforts to evade a stressor through the use of strategies including avoidance, denial, and wishful thinking. In patients, higher use of disengagement coping strategies was associated with higher symptoms of depression and anxiety in bivariate correlations. However, once taken into context with other coping strategies, disengagement coping was no longer significantly associated with psychological symptoms. This finding suggests while disengagement coping alone may have a small negative effect on depression and anxiety symptoms in patients, when taken into account with the use of other coping strategies, only secondary control coping has a significant effect on depression and anxiety symptoms and any effect of disengagement coping may be overshadowed or better accounted for by the benefits of secondary control coping. In contrast, disengagement coping was not related to psychological symptoms in correlational or regression analyses for at-risk individuals. At-risk individuals reported engaging in comparable levels of disengagement coping strategies as patients; however, it is possible that the use of these strategies is neither harmful nor beneficial for at-risk individuals. Previous research 21 found that adolescents and young adults at risk for HD who engage in high levels of disengagement coping strategies had higher psychological symptoms when their parent also reported using high levels of disengagement coping strategies. Further research is needed to understand the influence of disengagement coping on psychological difficulties in at-risk individuals and in what context such strategies may be most impactful.

The present study has several limitations. First, the relatively small sample of patients may have constrained our ability to find small effects for primary control coping or disengagement coping. Likewise, a larger sample of at-risk individuals may help identify any associations between primary control coping or disengagement coping and psychological difficulties in this population. Additionally, the use of cross-sectional data limits us from determining causality, as all data was collected at the same timepoint. The present study also only included self-report data, which may be vulnerable to respondent and memory bias. Future research may use longitudinal designs with more objective measures of coping and psychological symptoms to examine how coping strategies directly affect symptoms of depression and anxiety over time. Lastly, due to the nature of the larger study from which the present data are drawn, our inclusion criteria limited the sample of HD patients to only those who have children. Future research should investigate the control-based model of coping in HD patients broadly, and may also investigate whether coping strategies differ between patients with and without children.

Several future directions for research and interventions are implicated in the current study. First, further research using the control-based model of coping in longitudinal designs in the HD population is required to understand whether patients engage in more primary control coping strategies and in what ways these strategies may be helpful in HD over time. Additionally, the association between disengagement coping and psychological difficulties remains unclear, particularly in terms of for whom these coping strategies are either beneficial or detrimental. Further, future interventions for families affected by HD should focus on education and skill-strengthening practices on the use of secondary control coping strategies including reframing, acceptance, and distraction. Such strategies may be built into frameworks advising psychological care in HD (e.g.,34,35, 34,35), including interweaving coping education into cognitive behavioral therapy paradigms to reduce symptoms of depression and anxiety. In particular, given the recent trends in HD clinical research focusing more on pre-manifest gene carriers, it is of critical importance to enact and test interventions in younger individuals who may later develop the disease or become caregivers for other family members.

In conclusion, the present study provides evidence that coping strategies involving cognitive reframing, acceptance, and distraction (i.e., secondary control coping) are significantly associated with fewer depression and anxiety symptoms in both HD patients and at-risk individuals. For patients, the use of primary control coping strategies may provide some benefit, while the use of disengagement coping strategies may potentially be detrimental, though further research is required. The associations among primary control and disengagement coping strategies with psychological symptoms in at-risk individuals are less clear and require further examination. These findings implicate the importance of teaching secondary control coping strategies to individuals and families faced with stress related to HD.

Footnotes

ACKNOWLEDGMENTS

The authors are grateful to the families who participated in this research and to Elizabeth Huitz, Abigail Pine, Marissa Roth, Cara Guthrie, Jennifer Newton, Louis DeLuna, and Nora Wang for their assistance with data collection.

FUNDING

This research was supported by funds from the Griffin Family Foundation, the CHDI Foundation, grant R01HD104188 from the Eunice Kennedy Shriver National Institute of Child Health and Human Development, and grant T32MH18921 from the National Institute of Mental Health.

CONFLICT OF INTEREST

The authors have no conflict of interest to report.

DATA AVAILABILITY

The data surrounding the findings of this study are available upon reasonable request from the corresponding author. The data are not publicly available due to privacy, ethical restrictions, and other concerns.

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