Although dementia is commonly understood to connote late-life memory loss, the disease has multiple cognitive and behavioral phenotypes, which are reflective of variations in underlying neuropathology (Bertoux etal., Reference Bertoux, O’Callaghan, Flanagan, Hodges and Hornberger2015; O’Connor, Reference O’Connor2017; Ranasinghe, Reference Ranasinghe2016). One form of dementia, behavioral-variant frontotemporal dementia (bvFTD), defies the common definition in that the onset can occur relatively early (i.e., typically before age 65, and even as young as the early 20s; Hogan, Reference Hogan2016; Diehl-Schmid etal., Reference Diehl-Schmid2013). bvFTD also differs from other types of dementia in that it usually involves disinhibition and disruption of social cognition, but leaves memory intact during the early stages of the disease (Mioshi etal., Reference Mioshi2013). Despite its high prevalence, bvFTD is underrecognized even by healthcare professionals (Nunnemann etal., Reference Nunnemann, Kurz, Leucht and Diehl-Schmid2012). It should perhaps come as no surprise, then, that caregivers’ experience and need for support are understudied. A recent scoping review, published in International Psychogeriatrics, by Karnatz and colleagues (Karnatz etal., Reference Karnatz2020), spotlights this issue by summarizing current knowledge and identifying gaps in the literature, with important implications not only for patients, but also for those who love and care for them. Of particular note, they found that caregivers of bvFTD patients reported the highest burden among caretakers of patients with all types of frontotemporal lobar degeneration (FTLD).
The review by Karnatz and colleagues documents a rapidly accelerating pace of research on caregiver burden in FTLD. Of the 69 studies included in the current review, 42 were published after the previous comprehensive review on this topic (Nunnemann etal., Reference Nunnemann, Kurz, Leucht and Diehl-Schmid2012). At the time the previous review was published in 2012, there had been no randomized controlled intervention studies on the burden of caregivers of FTLD, and there was only one extant study on caregiver needs. The current review by Karnatz and colleagues, conducted in April 2017, includes 69 studies, of which 9 assessed support available to caregivers of FTLD patients.
Karnatz and colleagues establish several core facts about how patients with bvFTD receive care. Nearly three-quarters of caregivers are spouses, followed by children, siblings, and other family members. Caregivers of bvFTD patients are, on average, younger than caregivers of patients with other types of dementia, due in large part to the earlier age of onset of bvFTD. Karnatz and colleagues found that caregivers of patients with bvFTD report the highest levels of strain among the forms of dementia reviewed. The most burdensome symptoms are behavioral changes, including disinhibition, blunted effect, and loss of empathy (Bora etal., Reference Bora, Velakoulis and Walterfang2016). Embarrassing behavior and loss of the previously held relationship between patient and caregiver are also significant sources of distress. Early onset of bvFTD contributes to caregiver burden, since caregivers often have underage children and full-time jobs in addition to caring for family members with bvFTD. Indeed, some caregivers of people with bvFTD were themselves underage children. These challenges are not new; the first literature on the “sandwich generation” emerged over 40 years ago (Chassin etal., Reference Chassin, Macy, Seo, Presson and Sherman2010; Miller, Reference Miller1981). Nevertheless, longevity has continued to increase, without commensurate increases in available support for neurodegenerative diseases associated with aging (Chick etal., Reference Chick, Buck, O’Hara, Hantke, O’Hara and Etkin2020; Hantke and Bott, Reference Hantke, Bott, Hantke, O’Hara and Etkin2020).
Karnatz and colleagues found that bvFTD is associated with higher caregiver burden, higher caregiver depression, and less caring relationships between patient and caregiver, as compared to other forms of dementia. Nevertheless, the review found that available support for caregivers of patients with bvFTD is severely lacking, with many care facilities not equipped to support the unique needs of patients with bvFTD.
As the authors argue, another problem contributing to this lack of support is that clinicians frequently misdiagnose or otherwise fail to identify bvFTD. Caregivers expressed frustration with the difficulty and complexity of obtaining a clear diagnosis. The fact that early symptoms can overlap with those from other neurological disorders, including motor neuron disease, corticobasal syndrome, and progressive supranuclear palsy, combined with the younger age of onset, can obfuscate the presence of bvFTD. A significant gap highlighted by Karnatz and colleagues is the lack of appropriate measures of social cognition and limited assessment with those extant measures that can be utilized with dementia patients. Traditional cognitive assessment batteries for dementia do not routinely include measures of social cognition, yet the evidence suggests that such measures may capture bvFTD earlier than standard assessments of episodic memory, language, and naming. Affective problems in bvFTD can suggest a major mood disorder, particularly in younger patients, and this is made all the more complicated by the fact that any observed deficits in executive functioning are also a hallmark of primary affective dysfunction.
The authors also found that higher caregiver burden was associated with higher dementia severity, lower functional status, and younger caregiver age. Interestingly, despite the relationship between patient characteristics and caregiver burden, the authors found that interventions providing direct support to caregivers were more effective in alleviating caregiver strain than interventions that aimed to reduce patients’ FTD symptoms. However, the authors noted that there is little extant research on caregiving in cultures outside of Western industrialized nations, which may have different needs for support (Do etal., Reference Do, Cohen and Brown2014). As Karnatz and colleagues point out, as the literature on bvFTD grows, so too does the literature on interventions for their caregivers. Yet, only a limited number of studies to date have focused on the specific needs of caregivers of patients with FTLD, relative to similar research on caregivers of patients with Alzheimer’s disease or vascular dementia. The extant literature suggests that generally, non-pharmacological caregiver interventions are viewed as helpful, particularly if they had an emphasis on social contacts and mutual support from other caregivers with similar needs.
The review by Karnatz and colleagues is an extremely valuable contribution to the field that yields clear and significant implications for practice, treatment, and future research. First, increasing clinicians’ awareness of diagnostic criteria for bvFTD, and of differences from criteria for psychiatric and neurodegenerative disorders with overlapping symptoms, could help to connect patients and their caregivers with support earlier in the course of disease progression (Ducharme etal., Reference Ducharme, Price, Larvie, Dougherty and Dickerson2015). Second, increased research on identifying the most effective social cognitive measures for better capturing bvFTD, and developing batteries that standardly include such measures in the clinical trenches, could also support earlier diagnosis and connection to support. Third, increased public awareness of bvFTD symptoms could also ease the social burden of embarrassment and isolation. Fourth, more research is needed both to understand optimal ways to support caregivers of patients with bvFTD, and to examine how these needs differ in different cultures. Fifth, there is a substantial need for interventions targeted specifically to the characteristics of caregivers of patients with bvFTD, particularly given their younger age and likelihood of experiencing competing caregiver demands of caring for their own offspring and their loved ones with bvFTD. Finally, this in-depth review from Karnatz and colleagues reminds us how different the dementia caregiving experience can be, depending upon which type of dementia the patient is experiencing. With the burgeoning population of older adults over the coming decades, we need to increasingly attend to the varied needs of caregivers of dementia patients, and to provide more care facilities and support systems that accommodate the special needs of patients with bvFTD and their caregivers.
As long as increases in longevity outpace the development of treatments for age-related neurodegenerative diseases – and as long as support for caregivers of patients with these diseases lags behind the need for such supports – the stress associated with caretaking will continue to be the rule, not the exception.
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Although dementia is commonly understood to connote late-life memory loss, the disease has multiple cognitive and behavioral phenotypes, which are reflective of variations in underlying neuropathology (Bertoux etal., Reference Bertoux, O’Callaghan, Flanagan, Hodges and Hornberger2015; O’Connor, Reference O’Connor2017; Ranasinghe, Reference Ranasinghe2016). One form of dementia, behavioral-variant frontotemporal dementia (bvFTD), defies the common definition in that the onset can occur relatively early (i.e., typically before age 65, and even as young as the early 20s; Hogan, Reference Hogan2016; Diehl-Schmid etal., Reference Diehl-Schmid2013). bvFTD also differs from other types of dementia in that it usually involves disinhibition and disruption of social cognition, but leaves memory intact during the early stages of the disease (Mioshi etal., Reference Mioshi2013). Despite its high prevalence, bvFTD is underrecognized even by healthcare professionals (Nunnemann etal., Reference Nunnemann, Kurz, Leucht and Diehl-Schmid2012). It should perhaps come as no surprise, then, that caregivers’ experience and need for support are understudied. A recent scoping review, published in International Psychogeriatrics, by Karnatz and colleagues (Karnatz etal., Reference Karnatz2020), spotlights this issue by summarizing current knowledge and identifying gaps in the literature, with important implications not only for patients, but also for those who love and care for them. Of particular note, they found that caregivers of bvFTD patients reported the highest burden among caretakers of patients with all types of frontotemporal lobar degeneration (FTLD).
The review by Karnatz and colleagues documents a rapidly accelerating pace of research on caregiver burden in FTLD. Of the 69 studies included in the current review, 42 were published after the previous comprehensive review on this topic (Nunnemann etal., Reference Nunnemann, Kurz, Leucht and Diehl-Schmid2012). At the time the previous review was published in 2012, there had been no randomized controlled intervention studies on the burden of caregivers of FTLD, and there was only one extant study on caregiver needs. The current review by Karnatz and colleagues, conducted in April 2017, includes 69 studies, of which 9 assessed support available to caregivers of FTLD patients.
Karnatz and colleagues establish several core facts about how patients with bvFTD receive care. Nearly three-quarters of caregivers are spouses, followed by children, siblings, and other family members. Caregivers of bvFTD patients are, on average, younger than caregivers of patients with other types of dementia, due in large part to the earlier age of onset of bvFTD. Karnatz and colleagues found that caregivers of patients with bvFTD report the highest levels of strain among the forms of dementia reviewed. The most burdensome symptoms are behavioral changes, including disinhibition, blunted effect, and loss of empathy (Bora etal., Reference Bora, Velakoulis and Walterfang2016). Embarrassing behavior and loss of the previously held relationship between patient and caregiver are also significant sources of distress. Early onset of bvFTD contributes to caregiver burden, since caregivers often have underage children and full-time jobs in addition to caring for family members with bvFTD. Indeed, some caregivers of people with bvFTD were themselves underage children. These challenges are not new; the first literature on the “sandwich generation” emerged over 40 years ago (Chassin etal., Reference Chassin, Macy, Seo, Presson and Sherman2010; Miller, Reference Miller1981). Nevertheless, longevity has continued to increase, without commensurate increases in available support for neurodegenerative diseases associated with aging (Chick etal., Reference Chick, Buck, O’Hara, Hantke, O’Hara and Etkin2020; Hantke and Bott, Reference Hantke, Bott, Hantke, O’Hara and Etkin2020).
Karnatz and colleagues found that bvFTD is associated with higher caregiver burden, higher caregiver depression, and less caring relationships between patient and caregiver, as compared to other forms of dementia. Nevertheless, the review found that available support for caregivers of patients with bvFTD is severely lacking, with many care facilities not equipped to support the unique needs of patients with bvFTD.
As the authors argue, another problem contributing to this lack of support is that clinicians frequently misdiagnose or otherwise fail to identify bvFTD. Caregivers expressed frustration with the difficulty and complexity of obtaining a clear diagnosis. The fact that early symptoms can overlap with those from other neurological disorders, including motor neuron disease, corticobasal syndrome, and progressive supranuclear palsy, combined with the younger age of onset, can obfuscate the presence of bvFTD. A significant gap highlighted by Karnatz and colleagues is the lack of appropriate measures of social cognition and limited assessment with those extant measures that can be utilized with dementia patients. Traditional cognitive assessment batteries for dementia do not routinely include measures of social cognition, yet the evidence suggests that such measures may capture bvFTD earlier than standard assessments of episodic memory, language, and naming. Affective problems in bvFTD can suggest a major mood disorder, particularly in younger patients, and this is made all the more complicated by the fact that any observed deficits in executive functioning are also a hallmark of primary affective dysfunction.
The authors also found that higher caregiver burden was associated with higher dementia severity, lower functional status, and younger caregiver age. Interestingly, despite the relationship between patient characteristics and caregiver burden, the authors found that interventions providing direct support to caregivers were more effective in alleviating caregiver strain than interventions that aimed to reduce patients’ FTD symptoms. However, the authors noted that there is little extant research on caregiving in cultures outside of Western industrialized nations, which may have different needs for support (Do etal., Reference Do, Cohen and Brown2014). As Karnatz and colleagues point out, as the literature on bvFTD grows, so too does the literature on interventions for their caregivers. Yet, only a limited number of studies to date have focused on the specific needs of caregivers of patients with FTLD, relative to similar research on caregivers of patients with Alzheimer’s disease or vascular dementia. The extant literature suggests that generally, non-pharmacological caregiver interventions are viewed as helpful, particularly if they had an emphasis on social contacts and mutual support from other caregivers with similar needs.
The review by Karnatz and colleagues is an extremely valuable contribution to the field that yields clear and significant implications for practice, treatment, and future research. First, increasing clinicians’ awareness of diagnostic criteria for bvFTD, and of differences from criteria for psychiatric and neurodegenerative disorders with overlapping symptoms, could help to connect patients and their caregivers with support earlier in the course of disease progression (Ducharme etal., Reference Ducharme, Price, Larvie, Dougherty and Dickerson2015). Second, increased research on identifying the most effective social cognitive measures for better capturing bvFTD, and developing batteries that standardly include such measures in the clinical trenches, could also support earlier diagnosis and connection to support. Third, increased public awareness of bvFTD symptoms could also ease the social burden of embarrassment and isolation. Fourth, more research is needed both to understand optimal ways to support caregivers of patients with bvFTD, and to examine how these needs differ in different cultures. Fifth, there is a substantial need for interventions targeted specifically to the characteristics of caregivers of patients with bvFTD, particularly given their younger age and likelihood of experiencing competing caregiver demands of caring for their own offspring and their loved ones with bvFTD. Finally, this in-depth review from Karnatz and colleagues reminds us how different the dementia caregiving experience can be, depending upon which type of dementia the patient is experiencing. With the burgeoning population of older adults over the coming decades, we need to increasingly attend to the varied needs of caregivers of dementia patients, and to provide more care facilities and support systems that accommodate the special needs of patients with bvFTD and their caregivers.
As long as increases in longevity outpace the development of treatments for age-related neurodegenerative diseases – and as long as support for caregivers of patients with these diseases lags behind the need for such supports – the stress associated with caretaking will continue to be the rule, not the exception.
Description of authors’ roles
Both authors drafted and edited the manuscript.