The Hold me Tight Program for Couples Facing Huntington's Disease.

BACKGROUND: A positive predictive genetic test for Huntington's disease (HD) can be a life-changing event for both carriers and their partners, leading to lower wellbeing and increasing the risk for separation and divorce. The 'Hold me Tight' program (HmT), based on emotionally focused couples' therapy, aims at strengthening the couple bond by targeting attachment needs. OBJECTIVE: This study investigates whether the HmT program helps couples strengthen their relationship, as an investment in a future where the disease will affect life in many ways. METHODS: In a multiple baseline design using three baselines of varying length, 15 couples of presymptomatic HD-carriers and their partners were included. In three consecutive groups, couples underwent the intervention (an adapted version of the 8-session HmT program) in four weekly sessions and completed self-report questionnaires throughout the study period of 19 weeks (17 measurements). Attachment style was assessed at baseline, resilience at baseline and at the end of the follow-up, while relationship satisfaction and wellbeing were measured weekly. A multi-level model was applied to the data. RESULTS: Over the course of the study, wellbeing and relationship satisfaction significantly improved; resilience, however, did not. Furthermore, all three outcome measures were moderated by attachment style, with more securely attached individuals showing better outcomes. CONCLUSION: HmT improved wellbeing and relationship satisfaction of couples facing HD. Due to these improvements and high patient acceptability rates, this program could become a standardized procedure in HD care. The program could be adapted for other populations, e.g., couples facing other genetic neurological disorders.

[Psychological and psychiatric care for hereditary brain disorders]. / Psychologische en psychiatrische zorg bij erfelijke hersenziekten.

Hereditary neurological disorders are characterised by a combination of neurocognitive, neuropsychiatric and somatic symptoms, with average onset in mid-adulthood. Offspring of gene carriers is at risk of developing the disorder. Patients, partners and family members may present with various psychological problems that engender several care demands throughout life. For physicians and other medical professionals who occasionally come across these disorders it may be difficult to find adequate care. We describe two patients to illustrate the importance of specialised, multidisciplinary psychological and psychiatric care. First, we present a 43-year-old female patient with Huntington's disease who has had long-time psychiatric problems with a strong negative impact on her life and that of her spouse and children. Second, we present a 31-year-old female gene carrier of CADASIL who has psychological problems that are leading to uncertainty about whether the disease process has started.

An International Validation of a Clinical Tool to Assess Carers' Quality of Life in Huntington's Disease.

Family carers of individuals living with Huntington's disease (HD) manage a distinct and unique series of difficulties arising from the complex nature of HD. This paper presents the validation of the definitive measure of quality of life (QoL) for this group. The Huntington's Disease Quality of Life Battery for Carers (HDQoL-C) was expanded (n = 47) and then administered to an international sample of 1716 partners and family carers from 13 countries. In terms of the psychometric properties of the tool, exploratory analysis of half of the sample demonstrated good internal consistency and reliability. Some items on the full version did not meet psychometric thresholds and a short version (HDQoL-Cs) (n = 23) was developed based on more stringent criteria. This was achieved using standard psychometric item reduction techniques to both increase reliability and reduce the burden of carers completing the scale. Confirmatory factor analysis of the model structure showed a good fit for all factors and indicated that the HDQoL-C and HDQoL-Cs are psychometrically robust measures of QoL. We found that carers who lived with and looked after their spouse/partner had reduced sense of coping, hope for the future, and overall QoL. Carers with children who were at risk carried the gene or were symptomatic also had poorer QoL outcomes. Findings indicated the HDQoL-C and HDQoL-Cs are valid in multiple languages and across varied cultures as measures of self-reported QoL in family carers of individual's living with HD. These psychometrically validated tools can aid and guide the implementation of therapeutic interventions to improve life quality in this population and research into international and cross-cultural carer experiences. The HDQoL-Cs is recommended as the definitive international measure of HD carer QoL.

Do Attachment Style and Emotion Regulation Strategies Indicate Distress in Predictive Testing?

Predictive genetic testing for a neurogenetic disorder evokes strong emotions, and may lead to distress. The aim of this study is to investigate whether attachment style and emotion regulation strategies are associated with distress in persons who present for predictive testing for a neurogenetic disorder, and whether these psychological traits predict distress after receiving test results. Self-report scales were used to assess attachment insecurity (anxiety and avoidance) and maladaptive emotion regulation strategies (self-blame, rumination, catastrophizing) in adults at 50 % risk for Huntington's Disease (HD), Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL), and Hereditary Cerebral Hemorrhage With Amyloidosis - Dutch type (HCHWA-D), when they presented for predictive testing. Distress was measured before testing and twice (within 2 months and between 6 and 8 months) after receiving test results. Pearson correlations and linear regression were used to analyze whether attachment style and emotion regulation strategies indicated distress. In 98 persons at risk for HD, CADASIL, or HCHWA-D, attachment anxiety and catastrophizing were associated with distress before predictive testing. Attachment anxiety predicted distress up to 2 months after testing. Clinicians may consider looking for signs of attachment anxiety and catastrophizing in persons who present for predictive testing, to see who may be vulnerable for distress during and after testing.

Offspring of a parent with genetic disease: childhood experiences and adult psychological characteristics.

OBJECTIVE: To investigate childhood experiences and psychological characteristics in offspring of a parent with genetic disease. METHOD: Self-report scales were used to assess adverse childhood experiences (ACEs), adult attachment style, mental health, and psychological symptomatology in offspring of a parent with a neurogenetic disorder (i.e. Huntington's Disease, HD; Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy, CADASIL; and Hereditary Cerebral Hemorrhage With Amyloidosis-Dutch type, HCHWA-D), and in offspring of a parent affected with Hereditary Breast/Ovarian Cancer (HBOC). These groups were compared to persons who did not have a parent with one of these genetic diseases. Associations between childhood experiences and adult psychological characteristics were investigated. RESULTS: Compared with the reference group (n = 127), offspring of a parent with a neurogenetic disorder (n = 96) reported more parental dysfunction in childhood, and showed more adult attachment anxiety and poorer mental health. Offspring of a parent with HBOC (n = 70) reported more parental loss in childhood and showed poorer mental health. Offspring who experienced parental genetic disease in childhood had more attachment anxiety than offspring who experienced parental disease later in life. In the group of offspring, a higher number of ACEs was associated with poorer mental health and more psychological symptomatology. CONCLUSIONS: This cross-sectional study indicates that adult offspring of a parent with genetic disease may differ in attachment style and mental health from persons without one of these genetic diseases in their family, and that this may be related to adverse childhood experiences.

Attachment in families with Huntington's disease. A paradigm in clinical genetics.

OBJECTIVE: Based on the premise that attachment experiences lead to a working model for social relationships throughout life, this study investigates if there is a difference between adult attachment representations in individuals who were brought up by a parent with Huntington's disease (HD), compared to a non-clinical population. Specific events in the parents' disease process, especially those leading to trauma and loss will receive attention. METHODS: Using the Adult Attachment Interview, adult attachment representations were investigated in 32 unaffected adults at 50% risk for HD who were raised by an affected parent. RESULTS: We found a lower percentage of secure attachment representations, a higher percentage of preoccupied representations, and a higher percentage of unresolved/disorganized representations in our sample, compared to a non-clinical population. A relatively late start of the parent's HD career was associated with a secure adult attachment representation. Death of the HD parent before the child's 18th birthday was associated with an unresolved/disorganized adult attachment representation. CONCLUSION: Growing up in a family where one of the parents has Huntington's disease appears to affect the offspring's adult attachment representation. PRACTICE IMPLICATIONS: This study can be of relevance for genetic counselling, as well as for counselling and intervention in childrearing matters.