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Review Article| Volume 65, ISSUE 1, e29-e50, January 2023
Layers of Loss: A Scoping Review and Taxonomy of HD Caregivers' Spiritual Suffering, Grief/Loss and Coping Strategies
Bethany Faith Leidl, BS
Bethany Faith Leidl
Wake Forest University School of Medicine, (B.F.L., B.M.) Winston-Salem, North Carolina
Debbi Fox-Davis, MBA
Debbi Fox-Davis
HD Reach, (D.F.-D.) Denver, Colorado, USA
Francis O. Walker, MD
Francis O. Walker
Department of Neurology, Emeritus, Winston-Salem, (F.O.W.) North Carolina, USA
Jennifer Gabbard, MD
Jennifer Gabbard
Department of Internal Medicine, Section on Gerontology and Geriatrics (Palliative Care), (J.G., B.M.) Winston-Salem, North Carolina
Buddy Marterre, MD, MDiv
Buddy Marterre
Address correspondence to: Buddy Marterre, MD, MDiv, 5th Floor, Watlington Hall, Medical Center Blvd., Winston-Salem, NC 27157, USA.
Department of General Surgery, (B.M.) Winston-Salem, North Carolina
Published:October 02, 2022DOI:https://doi.org/10.1016/j.jpainsymman.2022.09.010
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Huntington's disease (HD), an incurable, multi-generational, autosomal dominant disorder, creating unique challenges and a myriad of spiritually-related stressors in those affected and their familial caregivers. Spiritual suffering, experiences of grief/loss, and coping strategies have not been systematically studied in HD caregivers.
To comprehensively define spiritual suffering, grief/loss, and coping strategies used by HD caregivers.
A PRISMA-ScR scoping literature review was conducted. Data from included research articles were organized thematically using induction and open coding. A grounded, deductive approach was used to delineate a demarcated taxonomy of themes, which encompasses all three over-arching domains. Four reviewers, employing a modified Delphi approach, ascertained which themes were demonstrated by research participants in each study.
36 of 583 articles met the review criteria; none were published in the palliative care literature. Investigations primarily focused on intrapersonal (self-image) distress and existential angst; only rarely looking deeper into divine/transpersonal suffering, disrupted religious relationships, or meaning distress. HD caregivers experience profound grief/loss, expressed as disenfranchised grief that is associated with the ambiguous loss of their loved one, loss of family structure, social connectedness, and personal losses. Half of the studies reported maladaptive HD caregiver coping strategies—characterized by dysfunctional escape schemes; in contrast, transcendent/creative strategies were often unexplored.
HD caregivers experience prolonged grief and other forms of spiritual suffering as they progressively lose their loved ones and disruption to their own lives. With an improved assessment tool, teams with spiritual and palliative care experts will better be able to support HD family caregivers.
spiritual suffering
HD caregivers experience prolonged grief as they lose their loved ones, their former family roles and experience genetically based existential uncertainty. With improved assessment tools, focusing on spiritual suffering, grief, and coping, teams with palliative and spiritual care experts will be better able to support HD caregivers and their families.
Huntington's disease (HD) is an incurable, autosomal dominant neurodegenerative disorder; which slowly strips away a patient's independence, demarcated by cognitive, psychiatric, and executive decline, behavioral and personality changes, affective problems as well as crippling motor impairments.
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Moreover, HD presents with a painfully long decline, which eventually leads to a patient's death over 20 years after diagnosis and 35 years after symptom onset.
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While many neurodegenerative disorders present in old age, HD typically presents in patients' 30s and 40s when careers are developing and family concerns are paramount. Given the young age of onset, the hereditary risk to offspring of 50%,
and the prolonged disease course, HD presents a unique challenge to patients, families, and clinical care teams alike.
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HD familial care partners often experience prolonged emotional distress and caregiver burden as the disease slowly robs them of their loved ones.
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The very nature of the continuous caregiver experience of progressive loss in HD, where their loved one does not die for many years,
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generates a context for chronic grief
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or prolonged grief disorder.
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Studies show high rates of depression, anxiety, and other psychotic symptoms among care partners.
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Some HD patients even begin manifesting the disease as children.
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Not surprisingly, studies have demonstrated high rates of family dysfunction, adverse parenting, abuse, and stark impacts on all members of a family coping with HD.
This continuous trauma experienced by HD care partners has detrimental effects on caregivers who often struggle to maintain their sense of self;
one seminal article infers that HD "partners have at least as much psychological distress as [patients], but partners have the tendency to draw back."
Furthermore, they observe that "the grief of [patients'] partners is often 'disenfranchised', or not socially recognized, as if they have no right to mourn."
Thus, in addition to all the physical and practical burdens of caregiving in HD, family members must personally deal with overwhelming psycho-socio-spiritual stressors.
Despite excellent primary palliative care provision by neurology teams
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and the support of multiple national advocacy organizations,
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we hypothesize that significant gaps remain—both in the knowledge of the breadth of HD caregiver suffering and in how other providers might help. Managing these gaps could potentially be addressed by palliative care (PC) experts; unfortunately, there is limited data on the role of PC in these caregivers. PC involvement rates with HD patients and their caregivers have been reported as low as 4%, even when they are at the end of their lives.
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J Palliat Med. 2022; 25: 488-505https://doi.org/10.1089/jpm.2021.0308
In addition, spirituality and spiritual concerns are ignored by the instruments usually used to measure caregiver quality of life (as well as by most non-palliative clinicians in family conferences),
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and yet spirituality as a source of support and/or suffering is ubiquitously experienced by patients with devastating serious illnesses.
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Public and medical awareness of HD, including understanding the experiences, burdens, and suffering of families is wanting. This lack of understanding extends to the PC community. We aim to evaluate the current state of HD caregivers' emotional and spiritual suffering by asking: "what is known about the spiritual suffering, grief, and coping strategies for caregivers of HD patients?" We hope to provide a framework for palliative care and spiritual support experts to better address and support caregivers of patients over the unrelenting disease course of HD.
This scoping review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses for Scoping Reviews (PRISMA-ScR) guidelines.
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Only original research studies were eligible, all years were included. Quantitative, qualitative, and mixed-method studies were allowed. Exclusion criteria comprised review articles, studies for tool validation, single case reports (multiple patient case series are included), and articles not available in English (Table 1).
Table 1Study Eligibility Criteria.
Research question: What is known about the spiritual suffering, grief, and coping strategies for caregivers of patients with Huntington's disease?
Literature Search: "Huntington's Disease" AND "caregiver" AND "suffering" / "grief" / "coping" and synonyms
Original research studies on caregivers of patients with Huntington's disease
All publication years up through July 2020
Study design can include:
Quantitative,
Qualitative,
Mixed-Method, and
Other relevant observational studies
Research articles not studying caregivers
Studies developing or validating assessment tools
Single case reports
Commentaries and opinion articles
Studies with goals or outcomes outside our study aim and question
Articles not published in English
The literature search strategy was developed with the input of a research librarian. The databases PubMed, CINAHL, EMBASE, and PsycINFO were searched using the following keywords and their synonyms: "Huntington's Disease," "caregiver" and "suffering" / "grief" / "coping" (Table 2).
Table 2Literature Search Strategy.
We performed a literature search and consulted with a research librarian. The databases PubMed, CINAHL, EMBASE and PsycINFO were searched using the following keywords and their synonyms: "Huntington's Disease" AND "caregiver" AND "suffering" / "grief" / "coping". The following number of potential articles were identified with the corresponding search strategies:
PubMed: 268 articles CINAHL: 105 articles
EMBASE: 143 articles
PsycINFO: 180 articles
Huntington Disease [MeSH] OR "Huntington Disease"[tiab] OR "Huntington's Disease" [tiab] OR "Huntington Chorea" [tiab] OR "Chronic Progressive Hereditary Chorea" [tiab] OR "Huntington's Chorea" [tiab] OR "Late-Onset Huntington Disease" [tiab] OR "Late Onset Huntington Disease" [tiab] OR "Juvenile Huntington Disease" [tiab] OR "Juvenile-Onset Huntington Disease" [tiab] OR "Juvenile Onset Huntington Disease" [tiab] "Huntington Disease" OR "Huntington's Disease" OR "Huntington Chorea" OR "Chronic Progressive Hereditary Chorea" OR "Huntington's Chorea" OR "Late-Onset Huntington Disease"OR "Late Onset Huntington Disease"OR "Juvenile Huntington Disease"OR "Juvenile-Onset Huntington Disease" OR "Juvenile Onset Huntington Disease"
Caregivers [MeSH] OR Caregivers [tiab] OR Caregiver [tiab] OR Caregiving [tiab] OR Family [tiab] OR Spouse [tiab] OR Carer [tiab] OR partner [tiab] OR relative [tiab] OR offspring [tiab] OR dependent [tiab] OR wife [tiab] OR husband [tiab] Caregivers OR Caregiver OR Caregiving OR Family OR Spouse OR Carer OR partner OR relative OR offspring OR dependent OR wife OR husband
"Existential suffering" [tiab] OR "existential distress" [tiab] OR "existential pain" [tiab] OR faith [tiab] OR "spiritual belief" [tiab] OR "religious belief" [tiab] "Stress, Psychological"[Mesh] OR Depression [mesh] OR Quality of Life [mesh] OR Anxiety [mesh] OR Emotions [mesh] OR shame [tiab] OR guilt [tiab] OR distress [tiab] OR anger [tiab] OR grief [tiab] OR Bereavement [tiab] OR Fear [mesh] OR Fear [tiab] OR Fears [tiab] OR Spirituality [tiab] OR religion [tiab] OR God [tiab] Adaptation, Psychological [mesh] OR "Psychological Adaptation" [tiab] OR "Psychological Adaptation" [tiab] OR "Coping Behavior" [tiab] OR "Coping Behaviors" [tiab] OR "Coping Skills" [tiab] OR "Coping Skill" [tiab] OR coping [tiab] OR "Adaptive Behavior" [tiab] OR "Adaptive Behaviors" OR well-being [tiab] OR distress [tiab] OR Suffering [tiab] "Existential suffering" OR "existential distress" OR "existential pain" OR faith OR "spiritual belief" OR "religious belief" OR "Stress, Psychological" OR Depression OR Quality of Life OR Anxiety OR Emotions OR shame OR guilt OR distress OR anger OR grief OR Bereavement OR Fear OR Spirituality OR religion OR God OR Adaptation, Psychological OR "Psychological Adaptation" OR "Psychological Adaptation" OR "Coping Behavior" OR "Coping Behaviors" OR "Coping Skills" OR "Coping Skill" OR coping OR "Adaptive Behavior" OR "Adaptive Behaviors" OR well-being OR distress OR Suffering
The initial literature search identified 575 unique articles. Eight studies, which were not uncovered by the initial search, but thought to possibly meet the research objectives were hand-picked by the authors and added. The title and abstract of each initially identified article were screened for relevance by one reviewer (B.F.L.). 484 studies that met exclusion criteria or were deemed unrelated to our research objectives were removed. The remaining 99 research articles were then read in full by two reviewers (B.F.L. and B.M.) to assess eligibility and to ensure that the study design could demonstrate at least one of the initial themes in our review. 36 articles were deemed suitable for the final group qualitative analysis (Fig. 1).
Fig. 1PRISMA-ScR Flow Diagram.
Thematic Induction and Taxonomy Synthesis
Thematic synthesis, the process of identifying both initial inductive descriptive and final distinct deductive analytical themes for the literature review, was conducted with a modification of Thomas and Harden's three-step methodology.
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Because we aimed to clearly delineate and define themes from a broad array of fields (including religion, spirituality, chaplaincy, psychology, mental health, grief, coping, and palliative medicine) and since these themes have been described variably by different disciplines, and furthermore, overlap in complex ways, we were concerned that initial open coding of the research studies might be overwhelming. Rather than begin with open coding, literature-based descriptive themes were initially induced to encompass all three over-arching caregiver domains: spiritual suffering, grief/loss, and coping. Similar modifications in qualitative thematic analysis have been shown to provide structure and consistency, achieving evaluation goals without a loss of scientific rigor.
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The domains we induced were spiritual suffering, grief/loss, and coping, each from literature subsets that may address or at least touch on other domains, but come from distinct perspectives. The coping domain was initially split between maladaptive and adaptive strategies, with the latter differentiating emotional management from problem-solving.
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Transcendent / creative coping was added during first-pass open coding when coping mechanisms that went beyond the first two were discovered; this addition was supported by the literature.
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Initial induction of the five themes within the spiritual suffering domain was based on pertinent literature
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and spiritual suffering assessment tools, as described below.
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Because of the overlap between religion and spirituality, when religious elements were key factors in these social disruptions, they were included as a separate theme within spiritual suffering; when they were not, they were assigned to loss of social connectedness within the grief/loss domain.
Following descriptive thematic induction, using the grounding of those themes, more in-depth deductive coding theme development was begun by all four reviewers (B.F.L., F.O.W., D.F-D., and B.M.) with first-pass analysis of 15 of the 36 included research articles. Then, during a multiple-iteration deliberation process by the four reviewers, the similarities and differences within the themes were compared to generate distinct deductive thematic definitions within the taxonomy, ensuring that areas of overlap were minimized and that each final theme was well defined for the final analytical purposes. An additional review for data extraction was performed for articles that demonstrated analytical themes for which the definitions significantly changed.
Initially, descriptive themes were induced, based on medical, psychology, and spirituality literature. We subscribe to Christina Puchalski's and collaborators' national and international consensus definition of spirituality as "the dynamic dimension of human life that relates to the way persons experience, express and/or seek meaning, purpose and transcendence, and the way they connect to the moment, to self, to others, to nature, to the significant, and/or sacred."
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Spiritual suffering—also called spiritual distress or spiritual pain—is defined as significant separations from meaning, purpose, connectedness, and transcendence (e.g., hope).
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Connectedness-based spiritual suffering encompasses significant struggles with self, the divine, and others, all of which are distinct.
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For example, patients with advanced cancer under PC in one study commonly experienced spiritual pain intrapersonally or in intra-psychic terms (e.g., suffering with despair, loss, regret), transpersonally or in relation to the divine (e.g., feeling abandoned by God, being without faith and/or a religious/spiritual community), and interpersonally (e.g., feeling unwanted by family members, feeling disconnected from others).
Existential issues deal with the examination of our existence, with ultimate concerns (or existential suffering sources) that include: finitude/death, isolation/disconnectedness, meaning in life, and freedom (e.g., moral choice).
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Whether the term existential or spiritual is evoked is largely based on one's theological orientation; with theists and/or religiously-oriented people preferring "spiritual" and atheists who focus on the secular and psychological aspects preferring the term "existential."
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Experts on both sides of this semantic divide agree that to be human is to be spiritual (or existential), so we to use the terms interchangeably. Determing the lines differentiating spiritual/existential suffering from psychological distress is also quite challenging.
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We did not investigate psychological suffering, such as depression or anxiety, rather we ensured that our definitions met spiritual suffering criteria or described grief and loss.
A significant theme within spiritual suffering is meaning distress—as emphasized by Dame Cicely Saunders.
Meaninglessness/nihilism is usually delineated from hopelessness/existential fear.
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Although there is certainly overlap between meaning distress and hopelessness/despair,
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we included the latter form of spiritual suffering with existential angst during the thematic synthesis process because they share future orientation. Further, the spiritual struggle inventory literature, in particular, largely demarcates spiritual suffering according to the level of one's disconnectedness into three subsets: 1) divine or transpersonal disruptions; 2) disrupted religious relationships (interpersonal disconnects); and 3) intra-personal, self-image, or integrity distress.
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We ultimately expanded self-image spiritual suffering to include significant moral distress, deep regrets, and purposelessness during the synthesis process; while unshakable attacks by evil spirits were incorporated into divine/transpersonal suffering.
We also expanded the existential angst theme to include uncertainty,
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which is a poignant element in family members who can be tested for and may carry the same HD gene as the affected loved one they care for.
Huniche L
Existential concerns in families with Huntington's disease: towards an understanding of the person in social practice.
Theory Psychol. 2009; 19: 93-113https://doi.org/10.1177/0959354308101421
Because none of the reviewed studies specifically measured or addressed prolonged grief disorder, we elected to analyze anticipatory grief
Holm M
Alvariza A
Fürst CJ
Öhlen J
Årestedt K
Psychometric evaluation of the anticipatory grief scale in a sample of family caregivers in the context of palliative care.
Health Qual Life Outcomes. 2019; 17: 42https://doi.org/10.1186/s12955-019-1110-4
and prevalent grief symptoms together. Concentrating on caregivers of patients with neurodegeneration pressed us to define the ambiguous loss of the caregivers' loved ones' personhood/identity (leading to disenfranchised grief)
Attig T
Disenfranchised grief revisited: discounting hope and love.
Omega (Westport). 2004; 49: 197-215https://doi.org/10.2190/P4TT-J3BF-KFDR-5JB1
Boss P
Dahl CM.
Family therapy for the unresolved grief of ambiguous loss.
in: Kissane DW Parnes F Bereavement Care for Families. Taylor & Francis Group, London2014: 171-182
http://ebookcentral.proquest.com/lib/wfu/detail.action?docID=1588621
discretely from other losses, such as losses of family structure, or other social connectedness, or personal losses.
Professional grief supporters most often separate adaptive coping into two themes: healthy emotional management and problem-solving strategies.
González-Rivera JA
Rosario-Rodríguez A
Spirituality and self-efficacy in caregivers of patients with neurodegenerative disorders: an overview of spiritual coping styles.
Religions. 2018; 9: 276https://doi.org/10.3390/rel9090276
Rexrode KR
Petersen S
The ways of coping scale: a reliability generalization study.
Educ Psychol Meas. 2008; 68: 262-280https://doi.org/10.1177/0013164407310128
Huang MF
Huang WH
Su YC
Coping strategy and caregiver burden among caregivers of patients with dementia.
Am J Alzheimers Dis Other Demen. 2015; 30: 694-698https://doi.org/10.1177/1533317513494446
Monteiro AMF
Santos RL
Kimura N
Coping strategies among caregivers of people with Alzheimer disease: a systematic review.
Trends Psychiatry Psychother. 2018; 40: 258-268https://doi.org/10.1590/2237-6089-2017-0065
Despite how important global meaning-focused, spiritual or faith-based coping is in neurodegenerative disease caregivers,
O'Brien MR
Clark D
Spirituality and/or religious faith: a means for coping with the effects of amyotrophic lateral sclerosis/motor neuron disease?.
Palliat Support Care. 2015; 13: 1603-1614https://doi.org/10.1017/S1478951515000097
clinicians often ignore such transcendent coping strategies. Thus, we built the creative/transcendent coping theme from religious, spirituality, and palliative care literature. It includes many elements, which are difficult to define, such as peace, self-awareness, self-, and other-forgiveness / grace, acceptance with meaning reconstruction, and gratitude.
Bryant-Davis T
Wong EC
Faith to move mountains: religious coping, spirituality, and interpersonal trauma recovery.
Am Psychol. 2013; 68: 675-684https://doi.org/10.1037/a0034380
Neimeyer RA Meaning Reconstruction and the Experience of Loss. American Psychological Association, Washington, DC2001
Bono G
Krakauer M
Froh JJ
The power and practice of gratitude.
in: Joseph S Positive Psychology in Practice: Promoting Human Flourishing in Work, Health, Education, and Everyday Life. 2nd ed. Wiley, Hoboken, NJ2015: 559-575
Jacobsen J
Kvale E
Rabow M
Helping patients with serious illness live well through the promotion of adaptive coping: a report from the Improving Outpatient Palliative Care (IPAL-OP) initiative.
Creatively engaging in the present experience or realizing "flow" and embracing aesthetics/art/music amidst spiritual suffering and loss, were also incorporated into this theme.
Csikszentmihalyi M
Robinson RE
The Art of Seeing: An Interpretation of the Aesthetic Encounter. The J. Paul Getty Museum, Malibu, CA1990
Creativity: Flow and the Psychology of Discovery and Invention. HarperCollins Publishers, New York1996
Flow: The Psychology of Optimal Experience. Harper Perennial Modern Classics, New York2008
Other adaptive coping strategies that help transcend suffering,
many of which were promoted by founding leaders in death/dying and the hospice and PC movement (Elisabeth Kübler-Ross, Cicely Saunders, Balfour Mount), were included in the adaptive transcendent coping definition too, such as mindfulness meditation, reflection, prayer, and hope.
Egnew TR
The meaning of healing: transcending suffering.
Ann Fam Med. 2005; 3: 255-262https://doi.org/10.1370/afm.313
Mount B
The 10 commandments of healing.
J Cancer Educ. 2006; 21: 50-51https://doi.org/10.1207/s15430154jce2101_15
Spiritual stages of dying.
in: Puchalski CM A Time for Listening and Caring: Spirituality and the Care of the Chronically Ill and Dying. Oxford University Press, Oxford; New York2006: 55-81
We designated negative and passive spiritual coping strategies
as maladaptive dysfunctional escapes to differentiate those from the three adaptive coping mechanisms. Whereas anger toward God is associated with depression and bereavement adaptation problems,
Smyth JM
Carey MP
Anger toward God: social-cognitive predictors, prevalence, and links with adjustment to bereavement and cancer.
J Pers Soc Psychol. 2011; 100: 129-148https://doi.org/10.1037/a0021716
Prince-Paul M
Root BL
Peereboom KS
The spiritual struggle of anger toward God: a study with family members of hospice patients.
we interpreted it as a collaborative, adaptive form of spiritual struggle,
since it is "normal" in some faith traditions and can also serve as a pivot point in positive spiritual coping.
If I tell others about my anger toward God, how will they respond?" predictors, associated behaviors, and outcomes in an adult sample.
J Psychol Theol. 2011; 39: 304-315https://doi.org/10.1177/009164711103900402
We deemed oscillation between transcendent hope and periodic avoidance or "denial" of reality adaptive
Weisman AD
On Dying and Denying: A Psychiatric Study of Terminality.
1st ed. Behavioral Publications, New York1972
and distinguished it from dysfunctional persistently-avoidant maladaptive escape strategies. Otherwise, the maladaptive coping definition induction was largely built on the antitheses of the three adaptive coping strategies, using tenacious evasion of realities/escape as the central defining element for this theme.
Lowit A
van Teijlingen ER
Avoidance as a strategy of (not) coping: qualitative interviews with carers of Huntington's disease patients.
BMC Fam Pract. 2005; 6https://doi.org/10.1186/1471-2296-6-38
Coding and deductive thematic data charting was completed by four independent reviewers. Their faith tradition self-identifications are Unitarian Universalist, Humanistic Jew, Catholic, and Mystical Christian / Taoist. Once deductive themes had been clearly defined, each reviewer individually read each article in full and when they believed that at least one clear, substantive demonstration of any individual theme was recorded by at least one participant in each study, they charted that along with page number references and germane comments. Thematic reviews were generated from diverse individual perspectives (a medical student; the Executive Director of HD Reach, a nonprofit organization working to improve the care and quality of life for those affected by HD; an HD neurology specialist; and a PC physician; from the above varied faith traditions), without hierarchy (each reviewer had an equal voice). Themes that were merely alluded to were not counted. We then employed a Group Delphi approach to deliberate on all charting judgment discrepancies (if all four reviewers independently believed a theme was demonstrated, no further discussion was undertaken).
Niederberger M
Spranger J
Delphi technique in health sciences: a map.
Front Public Health. 2020; 8: 1-10https://doi.org/10.3389/fpubh.2020.00457
In order to move from any qualitative dissensus in a pragmatic way to consensus, audio-visual deliberation meetings with all four reviewers were held, in every case whenever two reviewers perceived that a theme was demonstrated and two did not. In addition, any solitary dissenter (whether claiming a theme was or was not demonstrated) could plead their case against the other three reviewers when that article was discussed.
Brady SR
Utilizing and adapting the Delphi method for use in qualitative research.
Int J Qual Methods. 2015; 141609406915621381https://doi.org/10.1177/1609406915621381
Four full group discussions were held, covering theme demonstration in about nine articles each time. Whenever any discrepancies in theme demonstration occurred, group discussion resolved these to a final consensus.
Other data were also extracted from the research articles, including author(s), title, publication audience and year, country of origin, research design, number of study participants, and research objectives. Selected representative quotes from the research studies for each theme were extracted as well.
Of the 583 unique study articles originally identified, 36 were included in the final thematic analysis. Journal focuses of the examined studies were nursing (8), sociology (7), genetics (6), neuroscience (6), psychology (4), medical (3), quality of life (1), and thanatology (1). None of the included studies were published in journals with a focus on palliative care (Table 3).
Table 3Included Study Characteristics.
First Author Year Published
Journal Focus (Audience)
Study Participants (Caregivers)
Anderson 2019
Anderson KE
Griffin J
Kinel A
Quality of care for Huntington's disease in the United States: findings from a national survey of patients and caregivers.
JHD. 2019; 8: 509-519https://doi.org/10.3233/JHD-190380
Neuroscience USA Family Members 511 Survey
Aubeeluck 2006
Neuroscience UK Spouse / Partner 5 Other
Stupple E
All the burden on all the carers": exploring quality of life with family caregivers of Huntington's disease patients.
Qual Life Res. 2012; 21: 1425-1435https://doi.org/10.1007/s11136-011-0062-x
Quality of Life UK Family Members 47 Focus Groups
Brewer 2008
Psychology UK Parent 12 Interviews
Brouwer-DudokdeWit 2002
Brouwer-DudokdeWit AC
Savenije A
Zoeteweij MW
Maat-Kievit A
Tibben A
A hereditary disorder in the family and the family life cycle: Huntington disease as a paradigm.
Fam Process. 2002; 41: 677-692https://doi.org/10.1111/j.1545-5300.2002.00677.x
Sociology Netherlands Unspecified 6 Case Studies
Cox 2012
Cox M.
Quality of life among carers of people with Huntington's disease.
BJNN. 2012; 8: 288-294
Nursing USA Unspecified 31 Survey
Dawson 2004
Dawson S
Kristjanson LJ
Toye CM
Flett P
Living with Huntington's disease: need for supportive care.
Nurs Health Sci. 2004; 6: 123-130https://doi.org/10.1111/j.1442-2018.2004.00183.x
Nursing Australia Unspecified 19 Interviews
Decruyenaere 2005
Genetics Belgium Dyad 16 Survey
Domaradzki 2016
Domaradzki J
Family caregivers' experiences with healthcare services - a case of Huntington's disease.
Psychiatr Pol. 2016; 50: 375-391https://doi.org/10.12740/PP/59103
Psychology Poland Family Members 55 Interviews
Etchegary 2011
Etchegary H
Healthcare experiences of families affected by Huntington disease: need for improved care.
Chronic Illn. 2011; 7: 225-238https://doi.org/10.1177/1742395311403637
Medical Canada Family Members 10 Interviews
Halpin 2012
Halpin M
Accounts of suicidality in the Huntington disease community.
Omega (Westport). 2012; 65: 317-334https://doi.org/10.2190/OM.65.4.e
Thanatology Canada and USA Family Members 10 Interviews
Helder 2002
Helder DI
Kaptein AA
van Kempen GMJ
Weinman J
van Houwelingen JC
Living with Huntington's disease: illness perceptions, coping mechanisms, and spouses' quality of life.
Int J Behav Med. 2002; 9: 37-52https://doi.org/10.1207/S15327558IJBM0901_03
Psychology Netherlands Spouse / Partner 90 Survey
Kavanaugh 2014
Kavanaugh MS
Children and adolescents providing care to a parent with Huntington's disease: disease symptoms, caregiving tasks and young carer well-being.
Child Youth Care Forum. 2014; 43: 675-690https://doi.org/10.1007/s10566-014-9258-x
Sociology USA Child 40 Survey
Cho C
Maeda H
Swope C
I am no longer alone": evaluation of the first North American camp for youth living in families with Huntington's disease.
Child Youth Serv Rev. 2017; 79: 325-332https://doi.org/10.1016/j.childyouth.2017.06.029
Sociology Canada and USA Family Members 43 Mixed-method
Keenan 2007
Keenan KF
Miedzybrodzka Z
van Teijlingen E
McKee L
Simpson SA
Young people's experiences of growing up in a family affected by Huntington's disease.
Clin Genet. 2007; 71: 120-129https://doi.org/10.1111/j.1399-0004.2006.00702.x
Genetics UK Child 12 Interviews
Kessler 1993
Kessler S
Forgotten person in the Huntington disease family.
Am J Med Genet. 1993; 48: 145-150https://doi.org/10.1002/ajmg.1320480306
Genetics USA Spouse / Partner 5 Case Studies
Lewit-Mendes 2018
Lewit-Mendes MF
Lowe GC
Lewis S
Corben LA
Delatycki MB
Young people living at risk of Huntington's disease: the lived experience.
Neuroscience North America, Europe, Australasia, Asia Child 84 Survey
Lowit 2005
Medical UK Spouse / Partner 10 Interviews
Mand 2015
Mand CM
Gillam L
Duncan RE
I'm scared of being like mum": the experience of adolescents living in families with Huntington disease.
Neuroscience Australia Child 8 Interviews
Oliveira 2020
Oliveira CR
Mendes Á
Sequeiros J
Sousa L
Management of information within Portuguese families with Huntington disease: a transgenerational process for putting the puzzle together.
Eur J Hum Genet. 2020; 28: 1210-1217https://doi.org/10.1038/s41431-020-0630-z
Genetics Portugal Family Members 9 Interviews
Oliver 1969
Oliver JE
Dewhurst KE
Six generations of ill-used children in a Huntington's pedigree.
Postgrad Med J. 1969; 45: 757-760https://doi.org/10.1136/pgmj.45.530.757
Medical UK Family Members 40 Other
Oosterloo 2020
Neuroscience Netherlands Parent 10 Interviews
Roscoe 2009
Roscoe LA
Corsentino E
Watkins S
McCall M
Sanchez-Ramos J
Well-being of family caregivers of persons with late-stage Huntington's disease: lessons in stress and coping.
Health Commun. 2009; 24: 239-248https://doi.org/10.1080/10410230902804133
Sociology USA Family Members 17 Survey
Røthing 2015
Røthing M
Malterud K
Frich JC
Balancing needs as a family caregiver in Huntington's disease: a qualitative interview study.
Health Soc Care Comm. 2015; 23: 569-576https://doi.org/10.1111/hsc.12174
Sociology Norway Family Members 15 Interviews
Caregiver roles in families affected by Huntington's disease: a qualitative interview study.
Scand J Caring Sci. 2014; 28: 700-705https://doi.org/10.1111/scs.12098
Nursing Norway Family Members 15 Interviews
Sherman 2020
Sherman CW
Iyer R
Abler V
Antonelli A
Carlozzi NE
Perceptions of the impact of chorea on health-related quality of life in Huntington disease (HD): a qualitative analysis of individuals across the HD spectrum, family members, and clinicians.
Neuropsychol Rehabil. 2020; 30: 1150-1168https://doi.org/10.1080/09602011.2018.1564675
Neuroscience USA Family Members 17 Focus Groups
Sobel 2003
Sobel S
Cowan CB
Ambiguous loss and disenfranchised grief: the impact of DNA predictive testing on the family as a system.
Fam Proc. 2003; 42: 47-57
Sociology Canada, USA Unspecified 55 Interviews
Soltysiak 2007
Soltysiak B
Gardiner P
Exploring supportive care for individuals affected by Huntington disease and their family caregivers in a community setting.
J Clin Nurs. 2008; 17: 226-234https://doi.org/10.1111/j.1365-2702.2007.02276.x
Nursing UK Unspecified 9 Focus Groups
Sparbel 2008
Sparbel KJH
Driessnack M
Experiences of teens living in the shadow of Huntington disease.
J Genet Counsel. 2008; 17: 327-335https://doi.org/10.1007/s10897-008-9151-6
Genetics Canada, USA Child 32 Focus Groups
Tyler 1983
Sociology South Wales (UK) Family Members 76 Other
Vamos 2007
Psychology Australia Child 40 Survey
Williams 2000
Schutte DL
Holkup PA
Evers C
Muilenburg A
Psychosocial impact of predictive testing for Huntington disease on support persons.
Am J Med Genet. 2000; 96: 353-359
Genetics USA Unspecified 18 Mixed-method
Ayres L
Specht J
Sparbel K
Klimek ML
Caregiving by teens for family members with Huntington disease.
J Fam Nurs. 2009; 15: 273-294https://doi.org/10.1177/1074840709337126
Nursing Canada, USA Child 24 Focus Groups
Barnette JJ
Strategies used by teens growing up in families with Huntington disease.
J Pediatr Nurs. 2013; 28: 464-469https://doi.org/10.1016/j.pedn.2013.02.030
Nursing USA Child 44 Survey
Paulsen JS.
Family carer personal concerns in Huntington disease.
Nursing USA, UK Family Members 227 Survey
Nursing Canada, USA Family Members 42 Focus Groups
The characteristics of the 36 included studies are summarized in Table 3. The earliest was published in 1969, and the latest in 2020. Twenty-four studies were conducted in a single country (8 USA; 8 UK; two each from Australia, Netherlands, Norway; one each from Portugal, Canada); seven were conducted in two countries (six Canada and USA, one UK and USA); one was multinational (North America, Europe, Australia, Asia). Of the 36 included studies, 13 employed interviews, 10 were quantitative survey studies, six reported on focus groups, two were case studies, two used a mixed-method research methodology, and three represented other types of studies. A total of 1,704 participants were reported, ranging from 5 to 511 participants per individual study. The average number of participants was 47; the median was 19. In many instances, study participant (caregiver) relationships to the individual suffering from HD were not specified, or only designated as "family member"; when it was stated, caregivers included children (8), spouses/partners (4), and parents (2); one study specifically studied the patient-caregiver dyad.
Comprehensive Taxonomy of Spiritual Suffering, Grief/Loss, and Coping Strategy Themes
The taxonomy is presented in Table 4. The average number of themes demonstrated in each study was 4.6; the median was five. Two of the 36 studies failed to demonstrate any of the 14 themes; in one article, 13 of the 14 were demonstrated. 21 of the 34 studies that demonstrated one or more themes did not demonstrate a single theme within the spiritual suffering domain, whereas only four failed to demonstrate a grief/loss theme (Fig. 2).
Table 4Comprehensive Taxonomy of Spiritual Suffering, Grief/Loss, and Coping Strategy Themes.
Spiritual Suffering Meaning Distress Struggling with meaninglessness in life or suffering, nihilism; questioning meaning in life: "Why me?" "What's the point?"
Divine / Transpersonal Suffering Divine detachment / alienation. Feeling God has abandoned, is angry at, punishing/judging the patient/caregiver; unshakable attacks by evil spirits. Shattered God Image / world-view; unresolved anger or bitterness toward God. Questioning cosmic justice / fairness, and/or faith / trust relationship with God / the world. Fear of going to Hell.
Existential Angst in Uncertainty Struggling with uncertainty by experiencing significant angst/dread, hopelessness/despair, and/or intense fear regarding the future. [Hope is a meaning-based feasible-if transcendent-goal, with a pathway and agency. It is not outcome- or event-based]. Grave anxiety associated with genetic testing for an inherited disorder.
Disrupted Religious Relationships (Interpersonal) Spiritual (meaning-based and purpose-related) and religious (beliefs and practices) tensions and conflicts with family, friends, and faith leaders and communities. Hurt or offended by failures in one's faith community (e.g., by condemnation for anger at God, exclusion from, gossip about, or failure to openly support one's struggles without bias).
Self-Image Distress (Intrapersonal) Struggling with or loss of personal identity / sense of self, personhood, dignity; unstable values and/or feeling of purpose in life, beliefs and faith practices. Guilt/shame, self-blame, deep regrets; moral distress in caregiving vs. other roles; helplessly witnessing undue suffering in their loved one. Biopsychosociospiritual trauma / violence / abuse wounds.
Grief/Loss Grief Symptoms Explicit: (1) anticipatory grief; and/or (2) symptoms, including overshadowing sadness and/or anger/irritability, numbness, decreased concentration, preoccupation, helplessness; sleep, appetite and functional disturbances.
Ambiguous Loss of Loved One The ambiguous loss of the patient's / loved one's personhood / identity (typically physical presence with cognitive, affective, and/or behavioral decline) leading to disenfranchised grief (wherein the loss cannot be openly acknowledged, socially sanctioned or publicly mourned). Along with other symptoms, spousal caregivers may feel trapped and say, "This is not the person I married" and/or ruminate about their loved one before...
Loss of Family Structure Loss of family structure / cohesion, fractured previously-intimate family relationships / bonds. Displays of familial emotional / relational cutoff, estrangements, conflicts, with unfulfilled longings or no desires for forgiveness / reconciliation.
Loss of Social Connectedness Isolation / loneliness. Loss of important relationships / social connectedness outside the family. Caregivers may feel detached, like no one cares or understands, seek isolation from embarrassment, and/or not have the time to socialize.
Personal Losses Practical personal life losses: Arrested personal and professional / educational development. Personal societal connection losses, e.g., job, school, childhood, financial and living resources.
Adaptive Coping Healthy Emotional Management Processing and expressing lament and anger (including at God), ambivalence, fear/anxiety, guilt, and sadness; practicing self-forgiveness; having a positive affect and attitude.
Active Problem-Solving Accurate appraisal, active problem-solving; seeking psychological, social, and/or spiritual/religious support; new boundary setting, arranging respite; good self-care (exercise, diet, sleep, etc.); exercising agency, acting on plans.
Creative / Transcendent Coping Seeking peace, self-awareness, forgiveness, grace, love. Accepting reality with meaning and purpose reconstruction and hope reframing; experiencing flow [positive life engagement in the now] and joy. Embracing aesthetics/art/music, gratitude, humor, faith, mystery/unknowing. Practicing mindfulness meditation, reflection, contemplative prayer, collaborative (with one's Image of God) spiritual coping; self-compassion.
Maladaptive Coping Dysfunctional "Coping" Escapes Persistent emotional or relational disengagement / escape [not oscillation between hope and fearful "avoidance," which is normal]. Dysfunctional emotional management, e.g., remaining stuck in self-blame, depression, paralyzing anxiety/fear and/or anger. Promoting family dysfunction with emotional cutoff, triangulation, gossip, secrets, projection, passive aggression, blame-shifting; intentional social isolation. Passive religious "coping," e.g., petitionary prayer, clinging to unrealistic wishes. Not acting on plans; lack of self-care / boundaries; substance use (e.g., ETOH, benzodiazepines).
Fig. 2Spiritual Suffering, Grief/Loss, and Coping Strategy Theme Demonstration.
Spiritual Suffering Themes
The domains of spiritual suffering were demonstrated variably across the studies examined, with certain themes confirmed frequently and others declared only sparingly. Many studies (10/36) demonstrated the theme of existential angst in uncertainty. A participant in one study shows this by saying: "I've had three very close family members die of cancer, and you go through it and you don't necessarily get over it, but you do get on with your life … and there is support for the survivor. You know, with Huntington's, the time period is so long and it's just this very slow walk down a very long road. You know each day is just slightly worse than the day before. And there is no end to the tunnel, and you don't know what you're going to face."
Self-image (intrapersonal) distress was also present across many (9/36) of the analyzed studies. One caregiver poignantly said, "I'm very concerned, I have these meltdowns … it scares me because I'm trying to be strong and I'm trying to be a superwoman and be great at my job, be a great wife, be a great mom."
As an example of an understanding and positive interpretation of existential angst in uncertainty by a caregiver who was also a genetic carrier, one 47-year-old—who was depressed following his own diagnosis of HD—recounted his father's suicide, saying: "We had a farm, and one day he [father] just kind of brought in the veterinarian and he put all the animals to sleep on the farm. I guess he felt maybe we couldn't take care of them. Another day he took me aside just to make sure I knew how to run the farm, and stuff like that. And, of course, what happened was he killed himself. … I can certainly understand why he made that decision and I think he really kind of did it for our family's benefit too."
However, only a single study demonstrated the themes of either meaning distress or divine/transpersonal suffering (that article showed a clear demonstration of both).
The theme of disrupted religious relationships (interpersonal) was similarly rarely addressed and was confirmed by only two of the studies.
Although one article exhibited all five spiritual suffering themes,
58% of the articles (21/36) failed to demonstrate even one.
Grief/Loss Themes
The themes of grief and loss were well-represented across the evaluated studies. 86% (31/36) of the articles demonstrated at least one grief/loss theme; three demonstrated all five. Loss of family structure (22/36), loss of social connectedness (21/36), and loss on a personal level (21/36) were quite commonly demonstrated. Loss of the caregiver's affected loved one's identity/personhood, which is associated with disenfranchised grief, was also a common theme, demonstrated in 16 studies. Compared to other topics surrounding loss, the theme of general grief symptoms and anticipatory grief was demonstrated in fewer studies overall (5/36).
Coping Themes
75% (27/36) of the reviewed articles demonstrated at least one coping theme; one study demonstrated all four.
Adaptive coping mechanisms were demonstrated across multiple studies. Active problem-solving was the most demonstrated theme and was present in 19 of the 36 studies. Healthy emotional management was demonstrated in 12 articles, and creative/transcendent coping mechanisms were present in 10 of the studies. Maladaptive coping mechanisms were demonstrated in half (18/36) of the analyzed studies.
Representative Thematic Quotes
Many of the research studies included quotes from participant caregivers. Representative quotes demonstrating each of the 14 themes from various studies were extracted and reproduced in Table 5.
Table 5Representative Study Participant Quotes Demonstrating Each Theme.
Author (Year)
Quotes (page number)
Meaning Distress Sobel (2003) "You hear all these percentages and I've always thought, 'big deal for the one who gets it—with anything—heart disease, whatever.' You're the loser, and that's the one thing we have to prepare ourselves for. We can be the losers in the game." (51)
Divine / Transpersonal Suffering Sobel (2003) A 16-yr-old brother of a sister who tested positive told their mother: "God doesn't have a plan. He just sets up a guideline and away we all go." Their mother replied: "Yes. Your sister lost the lottery." (53)
Existential Angst in Uncertainty Cox (2012) "I may also develop the disease myself one day. No one wants to settle down with someone who may develop HD." (292)
Keenan (2007) "I love my mum to bits, I always want to help her as much as I can, but it just gets so much as times ... And every time when I go in and open her door, I'm always dreading in case I see her lying there with a cracked skull." (124)
A 26-yr-old, who supported her 12- and 16-yr-old cousins and their mother (with HD): "… when my gran was away on holiday I was about tearing my hair out … anytime there's an argument, her mum storms out saying 'Oh I'm going to take pills, I'm going to kill myself,' stuff like this. And I'm like at my work thinking what the hell can I do? You see she hasn't got her husband anymore; he left..." (123)
Mand (2015) "I think I'd feel a lot of guilt if I was gene-negative, because even though I'd dodged a bullet, my brother and sister were still in the firing line, and I wouldn't breathe easy until I knew they didn't have it." (213)
Disrupted Religious Relationships Sobel (2003) "People at church brushed it off with, 'God will provide a miracle,' and they weren't allowing me to face the reality." (52)
Self-Image Distress (Intrapersonal) Sobel (2003) "He [the father] was extremely upset because he felt he had done this to his daughter. He started to hate himself. I think he loathes himself and wants an excuse not to live anymore." (52)
Williams (2009b) "I'm very concerned, I have these meltdowns … it scares me, because I'm trying to be strong and I'm trying to be a superwoman and be great at my job, be a great wife, be a great mom." (793)
Grief Symptoms Williams (2000) Anticipatory grief after their loved one's positive genetic test: "The main thing that bothers me is that I know how she's going to be and I'd like to spend more time with her and my job just doesn't allow it. I feel like I'm missing the best part of her." (357)
Ambiguous Loss of Loved One Aubeeluck (2012) "I have always enjoyed my wife's company, but … I get these alternating feelings of sadness at the gradual passing of a warm and loving relationship with my wife, then anger at the behaviour. I think the worst thing is this roller-coaster nature of the mood swings so I never quite know who I'm going home to." (1431)
Brewer (2008) "Adam … doesn't appear to be anything like what you've ever seen … in HD. Adam is just really badly behaved; doesn't listen, doesn't do as he's told, hyperactive, like he's been charged up, doesn't listen … totally different, like nothing I've ever seen." (8)
Dawson (2004) "I guess in a way the grieving of [loss of a husband] is over. I realize that I've now got a dependent whose personality is quite different to the man I married. Quite totally different actually so I now look at him as a 12, 13-yr-old male child." (126)
"I miss the companionship. Having a dependent who's no longer a husband that you can no longer talk to anymore because you can't have conversations anymore unless he's starting the conversation." (127)
A parent, regarding an adult son: "He was getting very aggressive like we were walking on eggshells towards the end. He was starting to get aggressive and hitting … into the children [grandchildren], … and we couldn't cope with that." (127)
Røthing (2014) "What hurts me most is that you lose the one you loved. A complete different person is sitting in the dining room today, another person than the one who was my husband yrs ago. That is sad for me. I am more like a caring person now, not his spouse and loved one as I was." (702)
"Yes, there is a lot of frustration because me and my sister were best friends, super friends, sharing everything, clothes, boyfriends, and excitements, and now she is not here…" (702)
Loss of Family Structure Etchegary (2011) "People don't know that kids need counseling too. They are living in a verbally abusive environment." (231)
Mand (2015) "It's a pretty negative situation in my dad's family … when we go to grandma's we can't mention it, it's like it's something to hide because that's how they all view it, like it's shameful … my aunties and uncle are like 'it's dad's disease now.'" (212)
Williams (2011) "I wanted to stand by her forever, but now I realise I am not a saint who can go on giving and giving without getting any love or peace. I don't expect we will manage to remain a family much longer." (142)
Loss of Social Connectedness Brewer (2008) "I don't go shopping with him ... I wouldn't go … into the town centre, because he just wanders off … he pokes everybody, … and you're on a bus and lots of people look. … I've always said I'd rather … cut out a few of the things in the day, … because that's making him quite socially unacceptable. People find that hard to tolerate … Especially where you're in a situation where other people may look." (11)
Cox (2012) "Social circle is very small because of HD." (292)
Oosterloo (2020) "Maybe she wouldn't have been so alone and isolated from other children. She was so alone, that was incredibly sad. Of course, I don't know how she experienced it." (9)
Personal Losses Aubeeluck (2006) "The suffocation of my space and time, I miss out on so many things and instead I am on the never-ending relentless journey towards her death." (101)
Aubeeluck (2012) "…the other part that goes with it is the costings. We won't have anything to do with it as the carers we, we make sure that - there is a real implication between what the government are defining as healthcare and social care and when you back to the finances cos they're all interlinked there are a number of hoops that you've already got with a neurological terminal condition, it's ridiculous, there should be a much simpler way, um and I think a a a way of relief to come …" (1431)
Cox (2012) "I am stuck at home caring for my mom. I love her and want to care for her, but I am stuck, which is killing my independence and my ability to find happiness in relationships." (292)
Keenan (2007) "Before school we had to get my mum bathed, changed, get her breakfast, plus get us showered and changed. Plus you'd have to do all the tidying up before you went … and then coming home tea would have to get made. I missed most of my third yr at school because of it. I didn't like leaving mum here on her own during the day while me and my sister were at school and my dad was out working so some days I would skive off just to make sure she was all right." (122)
Mand (2015) "I don't go to school, I quit in April. I just help dad around the house, he needs it because he works full time and my brother is at school, so I stay home and look after the house … Most of my friends are still at school so I don't get to see them often." (212)
Sparbel (2008) A teenager: "My dad [with HD]… he doesn't eat, you have to feed him. If he's not cooperating, then she [my unaffected mother] has to take care of everything else … so I try to watch over her [my mom] too … I'll run her errands, I'll clean the house, cook some food, so she takes care of my dad. There's always something that needs to be done." (332)
Williams (2009b) "I may get, if I'm lucky, some nights maybe an hour or two hours of sleep … I've had 5 surgeries myself and … my husband would call me to help him in the bathroom and I could barely take care of myself." (793)
Adaptive Coping: Healthy Emotional Management Kavanaugh (2017) An 18-yr-old, in response to a youth support camp: "The camp has affected me in such a positive way. HD does not frighten me anymore. I am not scared of my future. It has … been life changing." (330)
A 17-yr-old, in response to a youth support camp: "I am much more positive and not so embarrassed. I am better in dealing with stressful situations in positive ways. HD doesn't scare me anymore." (330)
Williams (2009a) A teenager explains sadness and anxiety management: "… trying to, you know, enjoy the last proofs of my mom before something really bad happens." (282)
Adaptive Coping:
Active Problem-Solving Aubeeluck (2012) "When I was helping to care for my father, I remember how difficult it was. I started going to one [a support group]. It's small and I travel 150 miles round trip, but it is so worth it. That would also give you a day to … just you, take a little extra time and treat yourself to dinner or something. Take care of yourself first. You are no good to anyone else if you're falling apart." (1430)
Kavanaugh (2017) "I met lots of friends who have connections to HD along with skills to cope with the disease. I learned a lot to help me deal with everyday life." (330)
Keenan (2007) "We're starting to change the structure of the house so that it's accessible for my mum, and the bathroom is not big enough. So these are all things that we're gradually changing and gradually looking at. It's all about managing your parents – the tables have turned!" (125)
Oliveira (2020) "I have never hidden her disease from anyone, … we would go out for a walk or to the hairdresser … or even to the same restaurant." (5)
Røthing (2015) "I have chosen to work full-time, actually now also with the possibility of working at home, to reduce overtime work. … But, I prefer to go to work because it is better. Somehow, my function at work is better than my function at home; my work keeps me going, with good colleagues. It means a lot to me, it is the only place I can socialize." (573)
"I try to stay healthy. Sleep as much as I can and go for walks alone. I live a regulated life. Sometimes, it happens, I find time for a little trip, alone or with my children. It is rare. I need something to look forward to and something nice to think of later. I have to, if not, I know I will be depressed." (573)
Adaptive Coping: Creative / Transcendent Coping Aubeeluck (2012) "When I was helping to care for my father, I remember how difficult it was (sigh) especially looking and feeling like I was somehow seeing my own future played out before me. I'm glad the Lord God is your strength, I can relate. He is mine also. As difficult as it is sometime, remember that is a tremendous amount of strength." (1430)
"… as a family we can get through whatever life's challenges are. HD has brought us closer." (1432)
Halpin (2012) "…my attitude is like life is like a gift every day. Every day is like a free day to me. And not every day is a good day and not every day is equal but in general it's pretty good. … That's sort of how I see life, and I've had all these nice mental thinking days of my life." (327)
Keenan (2007) "I see everybody else as different and my mum's normal because I've lived with it … whereas everybody else would see my mum as different but I don't. … I'll walk down the street with mum and not care what anybody else thinks." (125)
Lowitt (2005) "You can't really plan for it – I just take one day at a time. It's so slow you just adapt." (5)
Oliveira (2020) "Shame? What shame? I am not ashamed! I have a sick son, I'm not ashamed." (5)
Williams (2009a) "When this is over, and if you don't get it, you'll be a better person in the long haul. You'll have, uh, spent your time in hell, so … if you don't get this … then, your life's going to be great because you went through hell already." (284)
Williams (2009b) "You look for little things. I got a great family, I got great friends. But our faith and my church has gotten me through more." (793)
Maladaptive Coping: Dysfunctional "Coping" Escapes Aubeeluck (2012) "…we don't want anyone to tell us, we don't want anybody in the house, we'll cope with this on our own and its all the burden on all the carers then. Cos you have that burden that they don't want anybody in the house and they deny that they are ill." (1432)
"We only found out that HD was in the family recently and it was such a shock. We are still having troubles getting information from any of the family, … now my brother-in-law is acting the same, in the space of one conversation he told my husband that he had the test and … then told him that if my husband's test comes out negative, then he will get tested himself. … I don't understand why he would lie like that." (1432)
Keenan (2007) "I dreaded going home because I got beaten up [by my dad] … So I turned into a bit of a rebel at 15 – alcohol, drugs, coming home late." (126)
Lowit (2005) "Well you know, he wouldn't talk about it at all. It was difficult to find anyone to talk to within his family. I didn't know about it (HD) and they weren't going to tell me anything about it." (4)
Oliveira (2020) "They don't tell much, because my father's family was very afraid of what others could think or say … this [HD] was a taboo, one would not speak nor admit it because they'd be ashamed." (4)
"My older sister never did the test. She doesn't deal well with the disease, she drinks, she's not working. She believes she has the disease and that she will die and so she has to enjoy life now." (5)
Røthing (2014) Speaking of caring for her mother and younger siblings: "I could not tell about it to my father. I isolated myself from others, and in dark times, after I had put smaller siblings to bed, their schoolbags were packed, food for the next day made ready and the house cleaned up, I had ten minutes for myself." (702)
"Our son moved out early, but our daughter who stayed at home was like a chopping block for her HD affected mother. When something went wrong she was always the one to blame. She must have suffered a lot." (703)
Sobel (2003) "There were some wicked fights because all of our emotions were too close to the surface. It wasn't very pretty. It was pretty harsh. Nobody knew what to do with what they were feeling." (52)
This study confirms the hypothesis that HD patient caregivers' burdens are high and that they have significant unmet needs. As one participant said, "Caregivers have enough responsibilities on their hands to spend hours researching for help."
Variables such as income, economic pressure, and social support predict life quality among caregivers of people with progressive neurologic diseases,
O'Connor EJ
McCabe MP
Predictors of quality of life in carers for people with a progressive neurological illness: a longitudinal study.
Qual Life Res. 2011; 20: 703-711
and yet these factors only begin to scratch the surface of the multifaceted forms of suffering that they experience. Caregiver quality of life is a different concept than caregiver burden.
The suffering associated with caring for a patient with HD outstrips the burden in relation to the realms of physical, emotional, time, financial, and other social drains.
A Quality Care Gap Well-Suited to Palliative Care
There is a pressing need for increased involvement of PC experts to collaborate with neurology teams to help support patients with all neurodegenerative diseases,
Oliver DJ
Borasio GD
Caraceni A
A consensus review on the development of palliative care for patients with chronic and progressive neurological disease.
Eur J Neurol. 2016; 23: 30-38https://doi.org/10.1111/ene.12889
and this specifically includes the needs of their caregivers, including long-term psychological and emotional support
and empathic communication skills.
In recent years, the advantages of PC involvement in patients with ALS and their caregivers have been increasingly demonstrated.
Gillespie J
Przybylak-Brouillard A
Watt CL
The palliative care information needs of patients with amyotrophic lateral sclerosis and their informal caregivers: a scoping review.
Several recent reviews have documented a high burden of symptoms in late-stage HD patients potentially amenable to palliative care interventions.
Hosken R
Barriers and facilitators to end of life care in Huntington's disease–a review of the literature.
AJON. 2019; 29: 33-43https://doi.org/10.21307/ajon-2017-020
Aoun SM
Oldham L
Palliative care and support for people with neurodegenerative conditions and their carers.
Int J Palliat Nurs. 2006; 12: 368-377https://doi.org/10.12968/ijpn.2006.12.8.368
In one study, 2/3 of such patients lacked advanced directives.
Booij SJ
Engberts DP
Marinus J
Thinking about the end of life: a common issue for patients with Huntington's disease.
J Neurol. 2014; 261: 2184-2191https://doi.org/10.1007/s00415-014-7479-4
Furthermore, HD patients most in need of palliative care are often too disabled to access the specialty clinics where they have established therapeutic relationships.
Religious faith and spirituality have been shown to provide a powerful avenue for meaning-making, well-being, and coping in ALS patients and their caregivers.
Fegg MJ
Meaning in life in patients with amyotrophic lateral sclerosis.
Amyotroph Lateral Scler. 2010; 11: 469-474https://doi.org/10.3109/17482961003692604
Calvo A
Moglia C
Ilardi A
Religiousness is positively associated with quality of life of ALS caregivers.
Amyotroph Lateral Scler. 2011; 12: 168-171https://doi.org/10.3109/17482968.2011.560947
Pagnini F
Lunetta C
Rossi G
Existential well-being and spirituality of individuals with amyotrophic lateral sclerosis is related to psychological well-being of their caregivers.
Spirituality has also been shown to improve the quality of life and reduce the risk of disease and death for caregivers of geriatric oncology patients.
Balducci L
Geriatric oncology, spirituality, and palliative care.
Meaning distress in bereaved caregivers, as they cope with the loss of a loved one has also been demonstrated.
Spirituality has not been specifically investigated as a means of coping for caregivers of HD patients,
however patients with serious illnesses frequently experience their disease through a spiritual hermeneutic, both as a source of support for coping and transformation and/or as a basis of concern/suffering.
The Huntington's Disease Peer Workgroup concluded that we should "improve support for families in caregiving – and in grief. And we can educate, counsel, and support HD families across generations."
Huntington's Disease Peer Workgroup. Lifting the veil of Huntington's disease: recommendations to the field from the huntington's disease peer workgroup. Available at:http://hdsa.org/wp-content/uploads/2015/03/Lifting-the-Veil-of-HD.pdf. Accessed January 9, 2020.
They also recommended the following call to action: "Screen for and respond to spiritual distress expressed by patients with HD and their families, extending spiritual care to them and supporting their spiritual experience. The potentially changing spiritual experience and needs of people with HD must be met throughout the disease."
A recent report on challenges in delivering complicated palliative neurology care to patients with motor neuron disease highlighted: 1) prolonged timelines of disease progression, 2) threats to personhood, 3) prognostic uncertainty, and 4) existential distress specific to neurological disease, including emotional, psychological and spiritual distress resulting from loss of function, autonomy and death.
Gofton TE
Chum M
Schulz V
Gofton BT
Sarpal A
Watling C
Challenges facing palliative neurology practice: a qualitative analysis.
J Neurol Sci. 2018; 385: 225-231https://doi.org/10.1016/j.jns.2017.12.008
A recent review promoted integrated spiritual care for people with neurodegenerative conditions and their caregivers, specifically suggesting developing and delivering belief- and value-based care models, to prevent health-related suffering.
Paal P
Lex KM
Brandstötter C
Weck C
Lorenzl S
Spiritual care as an integrated approach to palliative care for patients with neurodegenerative diseases and their caregivers: a literature review.
Ann Palliat Med. 2020; 9: 2303-2313https://doi.org/10.21037/apm.2020.03.37
Ideally, caregivers of patients with such devastating chronic illnesses as HD should be introduced to generalist palliative care and "referral for specialist palliative care support should be made at any time when physical, social, psychological, or spiritual unmet needs are not able to be satisfactorily resolved by the primary caring team."
Hawley P
Barriers to access to palliative care.
Palliat Care Res Treat. 2017; 10: 1-10https://doi.org/10.1177/1178224216688887
Lacking formal training in palliative and spiritual care, HD specialists who focus on the clinical needs of patients are often limited in their ability to manage the spiritual suffering, grief and coping needs of caregivers.
Thematic Demarcation between Grief/Loss, Spiritual Suffering, and Coping
By identifying and inducing prevalent themes from various literature and then deliberating on the similarities and differences within themes, we have generated the first comprehensive taxonomy of distinct thematic definitions for 10 types of spiritual suffering, grief, and loss, and four additional themes relating to coping. The subjects of grief/loss and spiritual/existential suffering, in particular, are intermingled in complex ways. As an example of how complicated the interrelationships are between various types of suffering, the following participant quote from a focus group study touches on at least four distinct themes in our taxonomy: "I know that there must be others who have HD and also are the sole caregivers to a parent, but it feels like there is no place to go where I can express the pain involved in this and the guilt because there are times I feel as though I can't take it anymore. It is often a painful place mixed with cries of despair and then times when all goes as well as can be expected but it seems lately I am running out of the heart to go on being the one responsible for mum's needs while feeling the disease myself but there is no-one else to help, it is my job."
Suffering complexity generates areas where various thematic definitions "touch" others. This is well illustrated by a unique study that captured the experiences of caregivers through a 'photovoice' method, wherein spousal caregivers of HD patients took photographs and then artistically described them.
One such participant's photo showed a children's swing set and slide with a bicycle, soccer ball, and scooter piled in front of the swing, with the caption: "Toys in the garden. With a genetic disease it means that we choose not to have children – our future is taken away from us."
Grief and Loss in HD Caregivers
Each person's grief experience is unique, and yet similar aspects can be characterized. Within the domain of grief/loss, many HD caregivers experience disenfranchised grief, which is related to the gradual ambiguous loss of their attachment to their loved one's personhood and identity before they die. Loss in disenfranchised grief may be socially negated, socially unspeakable, or both. Such socially-unsanctioned grief leaves many caregivers mourning in solitude without support as if they feel they have no right to mourn.
Some spousal caregivers do not want their children exposed to the experience of watching their affected parent "fall apart" or deteriorate before their eyes.
Other times, caregivers' grief processes are thwarted by the absence of finding meaning in the situation they find themselves in.
One study stressed that finding meaning was crucial to combat feelings of helplessness and despair; and that without finding meaning, caregiver grief remains unresolved and disenfranchised.
Not only do caregivers lose their loved ones as they knew them, but they also experience a loss of their former family structure. Patients with HD's mood and behavioral problems can drastically alter their family's relationships.
HD families commonly experience a loss of family structure as evidenced by fractured previously-intimate relationships and/or conflicts that may have escalated to emotional cutoffs and estrangements.
Bowen M.
Family Therapy in Clinical Practice. Jason Aronson, Princeton, NJ1978
The Bowen Center for the Study of the Family
Bowen Theory.
http://www.thebowencenter.org/bowen-theory-1
These broken family bonds may leave caregivers further isolated. Withdrawal from social networks and isolation can be a dynamic and two-way process, since both the shame felt over people's reactions to loved ones with HD (societal pressures) and the intense time-constraint burdens of caregiving play important roles in fostering intentional seclusion.
One study suggested that parental caregivers often find it challenging to ask for help and support, increasing their isolation.
Some children caregivers—many of whom have had their adolescence interrupted by role reversal and the ambiguous loss of their parent's identity—also find it difficult to discuss HD or their situation with their peers. The majority of caregivers in one study had quit attending support meetings, claiming that although they valued and praised them, that meeting other caregivers made things worse because they were presented with an even more dismal vision of their future.
In addition to the loss of time and other living resources, such as financial strains, personal losses include arrested personal and professional/educational development, as tragically demonstrated when children care for their parent with HD. As a parent with HD begins deteriorating, many children experience instrumental and emotional parentification, which is frequently overwhelming. For example, children—sometimes as young as grammar-school-aged—may carry out household tasks, such as washing, shopping, cooking, cleaning;
and may feed, bathe, toilet, provide company for and emotionally console their parent with HD;
sometimes relaying school problem experiences or skipping school to do so.
In this way, the child's parentification and the parent's dependency warps the traditional family structure, generating profound personal losses such as loss of innocence, and loss of time with friends, along with educational, developmental, and individuation delays associated with interrupted adolescence. One research study found that the frequency of childhood caregiving directly correlated with higher conflict with the ill parent and more problems with school;
others concluded that young adult and teenage caregivers act as important supportive linchpins in the family system.
Spiritual Suffering in HD Caregivers
Spiritual suffering among HD caregivers may be devastating. Spirituality has been defined in the PC literature as encompassing four major themes: meaning-making, purpose, connectedness, and transcendence, with or without a relationship with what most of us call "God."
HD family caregivers' spiritual suffering is likely persistent/chronic and may manifest as recurrent struggles in meaning-making, loss of their own identity/personhood/dignity, as well as that of their affected loved one, relationship/connectedness strain (with others, themselves, and for theists, God), isolation, shattered God images—including anger at and/or feelings of abandonment by God—as well as psycho-spiritual suffering, such as hopelessness, despair, and feelings of unfairness.
Marterre B
Clayville K
Navigating the murky waters of hope, fear, and spiritual suffering: an expert co-captain's guide.
Surg Clin North Am. 2019; 99: 991-1018https://doi.org/10.1016/j.suc.2019.06.013
Spiritual suffering screening and assessment tools,
which have only recently begun to be described and validated, provide an opportunity for quantifying this experience.
The themes within the domain of spiritual suffering were not demonstrated nearly as frequently in this review as those in the grief/loss domain, however, this may be because of a relative de-emphasis on spiritual suffering in the studies we reviewed. This is supported by our finding that one study showed a clear demonstration of all five spiritual suffering themes.
Also, of the 10 studies that employed surveys, only one used an inventory that queried spirituality as a resource for coping;
neither it nor any of the others probed spiritual suffering. After anyone in a family receives an HD diagnosis, caregivers' whole worlds change. Their plans for the future and their family get turned upside down overnight. This existential dread can manifest as hopelessness/despair,
Ready RE
Mathews M
Leserman A
Patient and caregiver quality of life in Huntington's disease.
Mov Disord. 2008; 23: 721-726https://doi.org/10.1002/mds.21920
and/or intense fear regarding the future. Grave anxiety over pre-symptomatic genetic testing in HD further elongates the timeline of uncertainty because it cannot determine the date of symptom onset.
Self-image distress includes significant struggling with or loss of personal identity or loss of the caregiver's sense of self, personhood, or dignity; as well as instability of values and/or their feeling of purpose in life, beliefs, and/or faith practices. Guilt, shame, self-blame, deep regrets, and moral distress in how much time and energy to expend in caregiving vs. other life roles are other manifestations of self-image (intrapersonal) distress. Huntington's disease patients and their possibly-unaffected siblings
Sørensen SA
Fenger K
Causes of death in patients with Huntington's disease and in unaffected first degree relatives.
J Med Genet. 1992; 29: 911-914
and children,
Figueiredo M. Young people caring for Huntington's disease parent worry "this could be me." Huntington's Disease News. Available at: https://huntingtonsdiseasenews.com/2018/11/29/young-people-caring-for-huntingtons-disease-parent-worry-this-could-be-me/. Accessed January 9, 2020.
frequently consider attempting, and sometimes complete suicide.
Hubers AAM
Reedeker N
Giltay EJ
van Duijn E
van der Mast RC
Suicidality in Huntington's disease.
J Affect Disord. 2012; 136: 550-557https://doi.org/10.1016/j.jad.2011.10.031
Wetzel HH
Gehl CR
Dellefave–Castillo L
Schiffman JF
Shannon KM
Suicidal ideation in Huntington disease: the role of comorbidity.
Psychiatry Res. 2011; 188: 372-376https://doi.org/10.1016/j.psychres.2011.05.006
As a testament to the depth of existential/spiritual suffering in HD families, the prevalence of suicidal ideation in HD is alarming and significantly higher than in other neurodegenerative disorders. Caregiver self-image distress can be either exacerbated or confirmed by their loved ones' suicidality.
Meaning distress, divine or transpersonal spiritual suffering, and disruptions of very meaningful religious relationships were uncommonly demonstrated in this review. They were uncovered, however. A few articles mentioned these, once as a mixture of disrupted religious relationships and divine/transpersonal suffering, demonstrated by how one caregiver rejected their religious upbringing and became an atheist after his loved one attempted suicide.
We suspect that divine or transpersonal suffering and disrupted religious relationships are under-reported in our review, because of the spirituality and spiritual suffering knowledge gap on the part of many of the researchers. The actual incidence of spiritual suffering themes can only be determined by research methods that balance the investigation of this domain with querying other forms of pain and loss.
Coping in HD Caregivers
Caregiving coping research has been traditionally guided by Lazarus and Folkman's 1984 model, which asserts that coping begins when a loved one's state is appraised as threatening, harmful, or challenging, and goes on to address the management of that distress.
Lazarus RS
Coping and adaptation.
in: Gentry WD Handbook of Behavioral Medicine. The Guilford Press, New York1984: 282-325
Following this, Folkman began stressing one's affective appraisal of the threats (positive, negative, or mixed),
which furthered the distinction between problem-solving and healthy emotional management. Other coping psychology experts defined coping as a self-regulatory model of behavior, which describes the relationship between illness perceptions, coping mechanisms, and peoples' quality of life, implicating coping as a significant mediator for well-being.
More recently, Folkman and Park have described a third distinct type of coping: meaning-focused coping, which draws on values, goals, and beliefs.
Meaning-making coping has been hypothesized to be especially important in stressful situations that are prolonged and chronic,
such as the context that HD family caregivers face. It incorporates faith-based religious and spiritual coping
and goes beyond the typical psychological processes of problem-solving and emotion-focused strategies, so we chose to label it creative/transcendent coping. In the studies we reviewed, the most commonly demonstrated HD caregiver coping strategies were adaptive coping through active problem-solving (19) and dysfunctional or maladaptive escapes (18). Twelve studies revealed that HD caregivers used adaptive healthy emotional management strategies and 10 demonstrated adaptive transcendent/creative coping mechanisms.
One area in which we struggled during our taxonomy development was in determining how best to characterize caregiver avoidance, such as in social situations. Are these behaviors adaptive or maladaptive?
HD patient caregivers are often, but not always socially avoidant. For example, although participants in one study avoided support groups, out of fear of a dismal portrayal of their future;
the assessment of camps for children growing up in a family riddled with the HD gene are uniformly positive.
Supporting youth affected by Huntington disease: results from the North American youth summer camp evaluation.
Neurotherapeutics. 2017; 14: 232
The social and educational impact of attending a Huntington disease (HD)-specific camp.
Neurotherapeutics. 2018; 15: 1206
Another study of families struggling with juvenile HD found that long-term support from a social worker was highly valued.
While some forms of such avoidance may be maladaptive, such as persistent emotional or relational disengagement, two psycho-spiritual models suggest that intermittent avoidance may be adaptive, allowing much-needed respite from caregiving distress. The oscillation model of existential angst (fluctuation between "denial" and acceptance across the dynamic "space" of "middle knowledge"), as originally described by Avery Weisman;
and re-illustrated by Jacobsen, et al,
demonstrates how periodic avoidance is normal—adaptive—human behavior. Weisman describes "middle knowledge" as a paradoxical "area of uncertain certainty, … somewhere between open acknowledgment of death and its utter repudiation."
In bereavement, a similar model that normalizes oscillation has been promoted by Margaret Stroebe and associates. She claims that the dynamic process of oscillating between confronting and avoiding both the stressors of loss- and restoration-oriented coping, which describes the temporary "denial" of one's losses and/or restorative tasks, is both normal and beneficial (Fig. 3).
Stroebe M
Schut H
The dual process model of coping with bereavement: rationale and description.
Death Stud. 1999; 23: 197-224https://doi.org/10.1080/074811899201046
We deemed these forms of oscillation as adaptive, transcendent coping, because of the intra-personal, meaning-making, spiritual formation nature of the process.
Nelson JM.
Psychology, Religion, and Spirituality. Springer, New York, NY2009
Persistent emotional, meaning-making, and/or relational disengagement, on the other hand, is maladaptive. Although grief work can occur with the help of others, it is essentially intrapersonal.
Worden's tasks of mourning include 1) accepting the reality of the loss, 2) processing the pain, 3) adjusting to a world without the loved one, and 4) finding a way to remember them while embarking on the rest of one's life journey.
His mourning process tasks can be applied to prolonged caregiver grief during long-term neurodegenerative decline before a loved one's death. Importantly, his third task embraces intrapersonal spiritual adjustments to how grieving individuals view the world without the person they were so intimately attached to;
these are chiefly meaning-making endeavors.
Fig. 3Transcendent Adaptive Oscillations in Coping with Grief and Loss.
As grief-stricken people embark on the necessary tasks of coping; grapple with existential finitude, decline or any significant loss; they oscillate between confrontation of concrete realities (below) and what may appear to be denial (above), but what is actually healthy, adaptive periodic avoidance. The intra-personal journey through this messy process, from left to right and across Weisman's dark "middle knowledge" space of "uncertain certainty"
is normal, adaptive transcendent coping. The meaning-reconstruction that occurs in this dark space ultimately leads to acceptance (to the right), which empowers the grief-stricken individual to adjust to a new world.
Transcendence means moving beyond concrete notions of oneself, the physical world, and connecting outside oneself, whether that connection is to God or something sacred, typically to provide a grounding for meaning (re-)construction. Transcendent coping includes dynamic—perhaps by oscillation—acceptance of (concrete) realities as well as developing equanimity in the face of struggling with the unknown, again, accompanied by meaning reconstruction. This type of coping is frequently expressed by caregivers of HD patients as "taking one day at a time;"
which we interpreted as positively surrendering to life "in the now", similar to Mihaly Csikszentmihalyi's creative invention or experiencing "flow" as a momentary transcendent engagement with everyday life.
Csikszentmihalyi M.
Finding Flow: The Psychology of Engagement with Everyday Life. Basic Books, New York, NY1998
Transcendent coping includes embracing activities and attitudes that go beyond emotional, cognitive, and physical processes, manifested by employing humor, faith, the arts, or experiencing joy and practicing self-compassion.
We sometimes found differentiating transcendent coping from active problem-solving or healthy emotional management challenging. Similarly, teasing transcendence out of following religious traditions can be tricky. Sometimes, meaning-making spiritual formation includes a caregiver's rejection of earlier images of God or religious constructs that they have found counterproductive to their life journey. For example, one study participant—who became an atheist—followed up "… my attitude is like life is like a gift every day. Every day is like a free day to me." with "And I regard my religious days as not fully living. I was living in fear of funny things. Superstitious things."
On the other hand, Roscoe, et al found that high levels of spiritual involvement and beliefs were indicative of successfully finding meaning in times of hardship, allowing caregivers of HD patients to accept things as they are.
Helder, et al administered the 14-item COPE inventory
Carver CS
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Weintraub JK
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to 90 HD spousal caregivers and found that they ranked "acceptance" first and "reinterpretation and growth" fourth; whereas "turning to religion" was ranked ninth.
Maladaptive "coping" is highlighted by persistent deliberate emotional and relational avoidance or escape mechanisms, manifesting as family secrets,
closed family systems,
or obstinate intentional social isolation.
Maladaptive strategies also include blame-shifting, emotional cutoff, alcoholism and substance use, and in the most horrific cases, physical and sexual abuse and even incest.
Current HD Caregiver Well-Being and Suffering Assessment Tools
The 34-item measure of HD caregiver quality of life (HD-QoL-C) that is most often employed primarily assesses caregiver's feelings and satisfaction, only superficially addressing two aspects of spiritual suffering; four aspects of grief/loss with none that query the ambiguous loss of their loved one's identity; and only lightly addressing coping without any specific inventory of active problem solving or maladaptive strategies.
Stupple EJN
Schofield MB
An international validation of a clinical tool to assess carers' quality of life in Huntington's disease.
Front Psychol. 2019; 10https://doi.org/10.3389/fpsyg.2019.01658
Furthermore, this tool has been shown to have limited psychometric value, hampering its usefulness and suitability in measuring HD caregivers emotional and psychological distress.
Hagell P
Smith S
A psychometric comparison of two carer quality of life questionnaires in Huntington's disease: implications for neurodegenerative disorders.
Mestre TA
Ho AK
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The three research publications that yielded the most robust results in this review, demonstrating 9, 10, and 13 of the 14 themes, were all interview studies.
This highlights how surveys tend to be focused on only one or at most two of the domains, and often overlook spiritual suffering and transcendent coping. Thus, we conclude that a better screening tool is needed, importantly combined with personal interviews of HD caregivers to further explore nuances—such as distinguishing between healthy oscillation (Fig. 3) and persistent denial/escape—as well as investigating meaning distress and transpersonal suffering or transcendent coping, which have been uncovered by such screening. Ideally, this screening and exploration should be undertaken by experts who excel in empathic communication and understand all three domains as well as how various themes touch upon other themes. While this is outside the scope of specialist neurology care, it could be accomplished by the integration of concordant palliative and spiritual care experts.
The prevalence of any form of spiritual suffering, grief/loss, and adaptive or maladaptive coping among caregivers of patients with HD cannot be ascertained by a literature review, as the data are influenced by each research team's focus. This review included a variety of research study methods, many of which were descriptive, and only some of which were subjected to rigorous qualitative analysis. The research quality of the included studies was not ascertained. The articles we reviewed were conducted on a fairly homogeneous group of participants; a more diverse population may yield different results. Despite these limitations, it has been possible to formulate compelling conclusions.
Our findings generate health policy, clinical and research implications, not just for caregivers of patients who are suffering from HD, but for all patients and their caregivers who have serious, debilitating, life-threatening illnesses. Unfortunately, medicine often neglects spirituality.
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Balboni M, Balboni T. Do spirituality and medicine go together? Center for Bioethics, Harvard Medical School; Boston, MA. Available at: https://bioethics.hms.harvard.edu/journal/spirituality-medicine. Accessed August 22, 2022.
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It has been suggested that this separation between religion/spirituality and medicine is promulgated by the following beliefs: 1) hospitals provide technologies and cures, rather than humanistic care; 2) physicians are scientists and health managers rather than whole-person healers; 3) humans are divisible into the material and immaterial, with no connection between body and soul/spirit, so health and well-being are not related to spiritual notions; and 4) the engagement of existential fear can only be dealt with by religious communities and spiritual leaders, not clinicians.
Healthcare policies to overcome these barriers and system-wide cultural biases include: 1) requiring education in basic spirituality and suffering screening
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and training in primary spiritual care skills for all clinicians;
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https://www.proquest.com/docview/1019243627/abstract/702E57385E9244C4PQ/1
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2) requiring that medical institutions partner with local religious communities and incorporate spiritual care experts, such as chaplains,
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to protect the personal and human aspects of compassion and care; 3) routinely incorporating spiritual care into the medical care of seriously ill patients by generalists;
and 4) requiring honest determinations of what is necessary to provide whole patient-centered care with professionalism, including sensitive inquiry and engagement of each individual's values, particularly as they affect their medical decision-making.
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From clinical and research standpoints, existing HD caregiver quality of life instruments do not address spiritual suffering, grief/loss, and coping comprehensively. Spiritual suffering is probably underreported because it is under-investigated. Seminal PC articles illuminate the need for spiritual suffering screening and assessment tools that are rigorously developed, clinically relevant, and adapted to a diversity of clinical settings.
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Handzo GF
State of the science of spirituality and palliative care research part I: definitions, measurement, and outcomes.
State of the science of spirituality and palliative care research part II: screening, assessment, and interventions.
Spiritual suffering assessment tools are now available, many of which we used in our initial thematic induction. Three are highlighted here: the PC-7, developed for patients with advanced illnesses where end of life issues are pertinent;
the Religious and Spiritual Struggles Scale (RSS);
and the Spiritual Distress Assessment Tool (SDAT), which was developed for inpatient geriatric rehabilitation.
Neither the SDAT nor the RSS assesses grief or loss; the PC-7 does, but without addressing the ambiguous loss of a loved one's personhood; none of the three assesses coping. Although the RSS is a survey instrument, the PC-7 and SDAT are ascertained through interviews. In addition, all three were developed and validated in patients as opposed to caregivers. A broader comprehensive screening tool—which focuses on caregivers and assesses grief/loss and coping too—is needed. For effective research and comprehensive clinical screening, our taxonomy can be used as a springboard to develop one, not only for those caring for HD loved ones but also for those caring for patients with other disabling illnesses. Perhaps a modification of one or more of these tools—using the taxonomy that we developed (Table 4) as a guide—incorporating non-redundant elements of validated caregiving coping assessments (e.g., selected questions from the 14-item brief RCOPE
to fill the adaptive transcendent coping query gap) is the answer. Ultimately, we hope to engage spiritual care experts to develop such a comprehensive, practical (shorter) assessment tool for screening and engage the HD community to pilot, administer and validate it, proving its feasibility and reliability. Then palliative and spiritual care experts can be better prepared to join with HD care teams to aid in screening for and exploring spiritual suffering, grief/loss, and coping in more depth; further supporting caregivers with active, engaged empathic listening, which will by its very nature promote healing and provide compassionate holistic care.