[Coping and Supporting Patients and Families of Frontotemporal Lobar Degeneration].

Frontotemporal lobar degeneration (FTLD) is a comprehensive term encompassing a group of clinically overlapping but heterogeneous conditions with selective frontal and temporal lobar neurodegeneration. Among the three clinical subtypes of FTLD, behavioral variant frontotemporal dementia (bvFTD) and semantic dementia (SD) were specified as "designated intractable diseases" by the Japanese Ministry of Health, Labor and Welfare in 2015. Under this designation system, relatively young patient with bvFTD or SD of certain severities can receive partial financial support from the Japanese local government. Here we introduce financial and social support for FTLD, including utilization of the designated intractable disease system and other support available in Japan. In addition, we describe the methods for coping and care of prominent symptoms in FTLD. Lastly, we address the current status of a nationwide Japanese FTLD study cohort named FTLD-J.

Characteristics of Young-Onset and Late-Onset Dementia Patients at a Remote Memory Clinic.

BACKGROUND: Young-onset dementia (YOD) is defined as the onset of dementia symptoms before the age of 65 years and accounts for 2-8% of dementia. YOD patients and their caregivers face unique challenges in diagnosis and management. We aimed to compare the characteristics of rural YOD and late-onset dementia (LOD) patients at a rural and remote memory clinic in Western Canada. METHODS: A total of 333 consecutive patients (YOD = 61, LOD = 272) at a rural and remote memory clinic between March 2004 and July 2016 were included in this study. Each patient had neuropsychological assessment. Health, mood, function, behaviour and social factors were also measured. Both groups were compared using χ2 tests and independent sample tests. RESULTS: YOD patients were more likely to be married, employed, current smokers and highly educated. They reported fewer cognitive symptoms, but had more depressive symptoms. YOD patients were less likely to live alone and use homecare services. YOD caregivers were also more likely to be a spouse and had higher levels of distress than LOD caregivers. Both YOD and LOD patient groups were equally likely to have a driver's licence. CONCLUSIONS: Our findings indicate YOD and LOD patients have distinct characteristics and services must be modified to better meet YOD patient needs. In particular, the use of homecare services and caregiver support may alleviate the higher levels of distress found in YOD patients and their caregivers. Additional research should be directed to addressing YOD patient depression, caregiver distress and barriers to services.

Genuine Smiles by Patients During Marital Interactions are Associated with Better Caregiver Mental Health.

OBJECTIVE: Providing care for a spouse with dementia is associated with an increased risk for poor mental health. To determine whether this vulnerability in caregivers is related to the expression of positive emotion, we examined 57 patients with Alzheimer's disease and behavioral variant frontotemporal dementia and their spouses as they discussed a marital conflict. METHOD: Facial behavior during the discussion was objectively coded to identify Duchenne (i.e., genuine) smiles and non-Duchenne (i.e., polite) smiles. Caregiver mental health was measured using the Medical Outcomes Survey. RESULTS: Greater expression of Duchenne smiles by patients was associated with better caregiver mental health, even when accounting for covariates (i.e., diagnosis, patient cognitive functioning, and caregiver marital satisfaction). Greater expression of non-Duchenne smiles by patients was associated with worse caregiver health, but only when covariates were entered in the model. Expression of Duchenne and non-Duchenne smiles by caregivers was not associated with caregiver mental health. DISCUSSION: Patients' expression of Duchenne and non-Duchenne smiles may reveal important aspects of the emotional quality of the patient-caregiver relationship that influence caregiver burden and mental health.

Family caregiver support groups: spiritual reflections' impact on stress management.

OBJECTIVES: Caregiving for people with neurodegenerative disease (e.g. Alzheimer's disease (AD); frontotemporal dementia (FTD)) is provided primarily by the family care system. Caregiving is taxing and much of the caregiving research is guided by a stress/adaptation theoretical perspective. Within this theoretical model, the role of spirituality in rendering meaning to the caregiver role remains unexplored. We conducted a qualitative analysis of expressions of spirituality and its meaningfulness during the caregiving journey within the context of an online video conferencing support group intervention program. METHODS: Eighteen AD and six FTD caregivers participated in the Caring for Others evidence-based program consisting of 10 weekly virtual online support group sessions facilitated by experienced gerontological social workers. RESULTS: Content analysis of the video-recorded group interactions yielded recurrent themes of caregivers' spiritual reflections and their associations with managing caregiver stress responses. CONCLUSION: The findings inform professional observations of the importance of spiritual beliefs in understanding how caregivers ascribe both positive and negative meanings to critical life events and their management.

The social and economic burden of frontotemporal degeneration.

OBJECTIVE: To quantify the socioeconomic burden of frontotemporal degeneration (FTD) compared to previously published data for Alzheimer disease (AD). METHODS: A 250-item internet survey was administered to primary caregivers of patients with behavioral-variant FTD (bvFTD), primary progressive aphasia, FTD with motor neuron disease, corticobasal syndrome, or progressive supranuclear palsy. The survey included validated scales for disease staging, behavior, activities of daily living, caregiver burden, and health economics, as well as investigator-designed questions to capture patient and caregiver experience with FTD. RESULTS: The entire survey was completed by 674 of 956 respondents (70.5%). Direct costs (2016 US dollars) equaled $47,916 and indirect costs $71,737, for a total annual per-patient cost of $119,654, nearly 2 times higher than reported costs for AD. Patients ≥65 years of age, with later stages of disease, and with bvFTD correlated with higher direct costs, while patients <65 years of age and men were associated with higher indirect costs. An FTD diagnosis produced a mean decrease in household income from $75,000 to $99,000 12 months before diagnosis to $50,000 to $59,999 12 months after diagnosis, resulting from lost days of work and early departure from the workforce. CONCLUSIONS: The economic burden of FTD is substantial. Counting productivity-related costs, per-patient costs for FTD appear to be greater than per-patient costs reported for AD. There is a need for biomarkers for accurate and timely diagnosis, effective treatments, and services to reduce this socioeconomic burden.

Caregiver burden in atypical dementias: comparing frontotemporal dementia, Creutzfeldt-Jakob disease, and Alzheimer's disease.

BACKGROUND: Caregiver burden is a significant issue in the treatment of dementia and a known contributor to institutionalization of patients with dementia. Published data have documented increased caregiver burden in behavioral variant frontotemporal dementia (bvFTD) compared to Alzheimer's disease (AD). Another atypical dementia with high-perceived caregiver burden is sporadic Creutzfeldt-Jakob disease (sCJD), but no formal studies have assessed this perception. The aim of this study was to compare caregiver burden across atypical dementia etiologies. METHODS: 76 adults with atypical dementia (young-onset AD [YOAD], bvFTD, language variant FTD [lvFTD], and sCJD) were administered an abbreviated version of the Zarit Burden Interview (ZBI), Neuropsychiatric Inventory (NPI-Q), and other assessment instruments during a five-year time period at Johns Hopkins Hospital (JHH). A Cox regression model examined differences between disease categories that impact mean ZBI scores. RESULTS: Mean ZBI scores were significantly different between dementia etiologies, with bvFTD and sCJD having the highest caregiver burden (p = 0.026). Mean NPI-Q caregiver distress scores were highest in bvFTD and sCJD (p = 0.002), with sCJD and bvFTD also having the highest number of endorsed symptom domains (p = 0.012). On regression analyses, an interactive variable combining final diagnosis category and NPI-Q total severity score demonstrated statistically significant differences in mean ZBI scores for sCJD and bvFTD. CONCLUSIONS: This study demonstrates that bvFTD and sCJD have increased levels of caregiver burden, NPI-Q caregiver distress, total severity scores, and number of endorsed symptom domains. These results suggest that higher caregiver burden in bvFTD and sCJD are disease specific and possibly related to neuropsychiatric symptoms.

The Evolution of Caregiver Burden in Frontotemporal Dementia with and without Amyotrophic Lateral Sclerosis.

BACKGROUND AND AIMS: Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) represent a disease spectrum. Caregiver burden in subtypes of FTD has not yet been directly compared with those patients who have co-existent FTD and ALS (ALSFTD). METHOD: Perceived caregiver burden was evaluated using the short Zarit Burden Interview (ZBI) in patients with behavioral-variant FTD (bvFTD, n = 21), semantic dementia (SD, n = 18), and ALSFTD (n = 15) at the initial clinical presentation and follow-up assessments. The Mini-Addenbrooke's Cognitive Examination (M-ACE) and the Motor Neuron Disease Behaviour Scale (MiND-B) were also used. Linear mixed effects models examined longitudinal changes on the ZBI, M-ACE, and MiND-B across groups. RESULTS: Burden at baseline was highest for the bvFTD group. Longitudinally, perceived burden increased for the SD and ALSFTD groups whereas in bvFTD, the level of burden which was high at baseline and remained high with disease progression. The severity of abnormal behaviors at baseline, as assessed by the MiND-B, correlated with baseline levels of caregiver burden and further accounted for 23% of the variance in caregiver burden at clinical follow-up. CONCLUSIONS: The trajectory of perceived burden differs across the FTD-ALS spectrum, with SD and ALSFTD caregivers demonstrating an increased burden that develops over time, compared to a persistently high level for bvFTD caregivers, evident throughout the disease course. The evolution of burden in these three syndromes likely reflects the initial presentation and clinical characterization that develops with time. Psycho-education programs for caregivers, which provide better coping strategies for challenging behaviors, may reduce levels of burden experienced with disease progression.

The Role of Coping Strategies in Psychological Outcomes for Frontotemporal Dementia Caregivers.

BACKGROUND: Caregiving for a person with frontotemporal dementia (FTD) is related to poor caregiver outcomes. Coping strategies adopted by caregivers are known to influence psychological outcomes in other dementia caregiver populations, however, their influence on psychological outcomes in FTD caregivers is poorly understood at present. METHODS: Questionnaire data for 94 German primary caregivers (mean [M] 59.11 years, 68 females) of FTD care-recipients living in the community (M 63.94, 30 females) were investigated. Standardized measures completed by the caregiver included the Caregiver Strain Index (CSI), Beck Depression Inventory II (BDI-II), Quality of Life-Alzheimer's Disease (QoL-AD), and the Brief Coping Orientations to Problems Experienced (COPE). Care-recipients' nursing care level was collected as a measure of the intensity of their care needs. RESULTS: Mediation analyses showed that the effect of the intensity of care-recipients' care needs on caregiver well-being depended on caregivers' experience of strain. High levels of caregiver strain did not predict depression (-0.22, 95% confidence interval CI: [0.16 to 2.04]) but predicted reduced QoL (-0.44, CI: [-1.15 to -.16]). Moreover, caregivers' experience of strain was exacerbated by their use of dysfunctional coping (ß = .21; p = .04), care-recipients' intensity of care needs (ß = .25; p = .01), and fewer financial resources (ß = .23; p = .02). In turn, caregivers' use of dysfunctional coping as a response to their strain increased the levels of depression (0.46, CI: [0.19-0.82]). By contrast, use of problem-focused coping strategies increased caregivers' QoL (0.10, CI: [0.00 to 0.31]). SIGNIFICANCE: This study identifies variables amenable to clinical interventions that can improve caregivers' well-being: specifically, caregiver strain and coping strategies. For a disease without cure yet increasing prevalence and cost, ameliorating the caregiver experience through targeted interventions is essential.

Caregiving for a loved one with dementia at the end of life: an emergent theory of rediscovering.

Millions face the challenges of caregiving for a loved one with dementia. A classic Glaserian grounded theory methodology was used to discover the problem that caregivers of individuals with dementia face at the end of life and how they attempt to resolve that problem. Data were collected from a theoretical sample of 101 participants through in-person interviews, online interviews, book and blog memoirs of caregivers, and participant observation. Constant comparative method revealed a basic social psychological problem of role entrapment. Caregivers attempt to resolve this problem through a 5-stage basic social psychological process of rediscovering including missing the past, sacrificing self, yearning for escape, reclaiming identity, and finding joy. Health care professionals can support caregivers through this journey by validating, preparing caregivers for future stages, and encouraging natural coping strategies identified in this process. This study provides a substantive theory that may serve as a framework for future studies.

[A nursing experience using the props-integrated communicative approach to ameliorate aggression in a frontotemporal dementia patient].

This report introduces the nursing caring experience with a male patient with frontotemporal dementia (FTD) who was hospitalized in an acute psychiatric ward from March 5th to April 30th, 2012 due to the clinical manifestations of verbally expressive impairment, aggression, and subsequent caregiver burden. The patient was assessed according to the guidelines of clinical competencies for mental health nursing assessments developed by the Psychiatric Mental Health Nurses Association. Three clinical diagnoses were identified after this assessment, including (1) impaired verbal communication, (2) chronic confusion, and (3) caregiver role strain. The current report focuses only on the clinical issue of impaired verbal communication. We adopted a props-integrated communicative approach by integrating props and physical motions with communicative strategies. This approach enabled us to formulate a patient-centered communicating model and prompt for active expression and adequate communication, which ultimately resolved the patient's aggression problem. In addition, we provided psychoeducation to the family members in order to teach them the relevant knowledge, skills, and approaches that caregivers may use to enhance their caring capabilities and reduce the burden of caregiving. This successful experience may be used as a reference in caring for FTD patients with communicative impairments. Our proposed approach integrates props with simple language and develops an appropriate communicating model to provide high quality care for patients.

Differentiating subtypes of apathy to improve person-centered care in frontotemporal degeneration.

Apathy, a reduction in goal-directed behavior (GDB), affects 90% of individuals with behavioral variant frontotemporal degeneration, which is a common cause of early onset neurodegenerative disease. The cognitive and neural impairments associated with apathy make it difficult to initiate, plan, and self-motivate activities toward a specific goal, such as dressing or bathing. These impairments are associated with significant decline in functional ability, caregiver burden, and increased cost of care due to early institutionalization. The current article reviews the evidence suggesting that apathy arises from the interruption of one or any combination of three GDB processes: initiation, planning, and motivation. From this perspective, three subtypes of apathy related to dysfunction at the level of GDB and the corresponding neuroanatomy are explored. Further research is required to confirm and measure these subtypes of apathy for use in clinical and research settings. A more precise classification of apathy by subtype will allow implementation of the most appropriate person-centered, individualized therapy.

Living with semantic dementia: a case study of one family's experience.

Semantic dementia is a variant of frontotemporal dementia and is a recently recognized diagnostic condition. There has been some research quantitatively examining care partner stress and burden in frontotemporal dementia. There are, however, few studies exploring the subjective experiences of family members caring for those with frontotemporal dementia. Increased knowledge of such experiences would allow service providers to tailor intervention, support, and information better. We used a case study design, with thematic narrative analysis applied to interview data, to describe the experiences of a wife and son caring for a husband/father with semantic dementia. Using this approach, we identified four themes: (a) living with routines, (b) policing and protecting, (c) making connections, and (d) being adaptive and flexible. Each of these themes were shared and extended, with the importance of routines in everyday life highlighted. The implications for policy, practice, and research are discussed.

Life enhancing activities for family caregivers of people with frontotemporal dementia.

Aberrant psychological and behavioral symptoms are common in patients with dementia. These symptoms have negative consequences for family caregivers, causing stress and burden. Frontotemporal dementia (FTD) symptoms cause more pronounced stress and burden on caregivers than those associated with Alzheimer dementia. In this randomized, attention control pilot study, we delivered 5-weekly, one-on-one, positive affect intervention sessions to family caregivers of people with FTD. The program, Life Enhancing Activities for Family Caregivers: LEAF was conducted in-person or by videoconference with caregivers across the United States. Measures of affect, caregiver mood, stress, distress, and caregiver burden were assessed at baseline, end of sessions, and 1 month after completion. Twenty-four caregivers (12 intervention and 12 attention control) participated. At the end of the intervention, scores on positive affect, negative affect, burden, and stress all improved in the intervention compared with the control group. These scores continued to show improvement at the assessment done 1 month after intervention. Subjects were receptive to the skills and the delivery methods. The positive emotion skill-building intervention proved feasible especially in the internet videoconference delivery format. The intervention promoted positive affect and improved psychological outcomes for family caregivers of people with FTD.

Family caregivers of individuals with frontotemporal dementia: examining the relationship between coping and caregiver physical and mental health.

To identify strategies to assist family caregivers of individuals with frontotemporal dementia (FTD) in dealing with their caregiving demands, nurses must understand these family members' unique needs and how they currently deal with their demands. The purpose of this study was to examine the relationship between coping and caregiver physical and mental health among FTD family caregivers. Participants were primary caregivers of individuals with FTD (with behavioral symptoms) living at home (N = 61). A small positive association was noted between problem-focused coping and caregiver physical health (r = 0.29, p < 0.05), and a small but nonsignificant positive correlation was noted between emotion-focused coping and caregiver mental health (r = 0.21, p = 0.10). However, multiple regression analysis showed that emotion-focused coping (ß = 0.46, p < 0.05) made a statistically significant, unique contribution to caregiver mental health and explained approximately 14% of its variance. These findings support the potential value of emotion-focused coping strategies when dealing with behavioral symptoms manifested by individuals with FTD.

Caring for loved ones with frontotemporal degeneration: the lived experiences of spouses.

There is an abundant literature about the experience of caregiving for a spouse living with Alzheimer's disease (AD), but there are very few qualitative studies about caregiving for persons living with Frontotemporal Degeneration (FTD). FTD causes a change in personality and affected persons may lose the ability to adhere to social norms. Thus, the emotional loss caregivers experience is often confounded by anger in response to embarrassing and socially inappropriate behaviors. In this paper, we offer a glimpse of this lived experience through the voices of two spouses whom we interviewed, each with experience caring for persons living with FTD. We suggest that FTD caregivers experience a loss of emotional attachment to their spouse because of their partner's behavioral symptoms. This loss gives rise to feelings of isolation and anger as caregivers assume new roles and reimagine their future. The findings from these interviews illuminate the need for more research and greater attention and support for FTD caregivers early in the disease trajectory.

The meaning of long-term caregiving for patients with frontal lobe dementia.

Nursing staff that work with patients with frontal lobe dementia (FLD) experience challenges that may lead to physical and psychiatric distress. The aim of this study was to capture the feelings, experiences, and reflections of the health staff regarding interactions with and caring for patients with FLD and to highlight what it means for health staff to care for patients with FLD through their daily work. This is a qualitative study with a phenomenological hermeneutic approach. Ten health staff members who work with patients with FLD were interviewed using semistructered interviews. The focus during the interview was the experiences of the staff through their everyday work. The interviews were recorded and then transcribed verbatim. The material was analyzed using a phenomenological hermeneutic approach. The result of the study identifies three themes that highlight the meaning of caregiving for patients with FLD, that is, being aware of the relationship with the patients, being insecure, and being safe. The patients' unpredictable behaviour puts the relationship between the staff and the patients on trial. It is essential in caregiving to see the human behind the disease and the behaviour. The interest of finding new solutions in the caregiving is awakened through the relation with the patients, through reflections with colleagues, external guidance and by support from the staff leader.

Frontotemporal degeneration in the patient with amyotrophic lateral sclerosis: helping the caregiver cope.

Research in the previous decade has found a link between amyotrophic lateral sclerosis (ALS) and frontotemporal degeneration (FTD). It is estimated that as many as 50% of all people with ALS will have some degree of cognitive impairment and that approximately 10%-25% of patients will meet the Neary criteria for FTD. For the caregivers of persons with both ALS and FTD, the burden of care can be quite high. Nurses are in a position to help the caregivers cope.