If you don't remember your password, you can reset it by entering your email address and clicking the Reset Password button. You will then receive an email that contains a secure link for resetting your password
If the address matches a valid account an email will be sent to __email__ with instructions for resetting your password
Wake Forest University School of Medicine, (B.F.L., B.M.) Winston-Salem, North CarolinaDepartment of Internal Medicine, Section on Gerontology and Geriatrics (Palliative Care), (J.G., B.M.) Winston-Salem, North CarolinaDepartment of General Surgery, (B.M.) Winston-Salem, North Carolina
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.
Objectives
To comprehensively define spiritual suffering, grief/loss, and coping strategies used by HD caregivers.
Methods
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.
Results
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.
Conclusion
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.
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.
Introduction
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.
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.
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%,
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.
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
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.
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),
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.
Methods
This scoping review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses for Scoping Reviews (PRISMA-ScR) guidelines.
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
Inclusion Criteria:
•
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
Exclusion Criteria:
•
Research articles not studying caregivers
•
Studies developing or validating assessment tools
•
Review articles
•
Single case reports
•
Commentaries and opinion articles
•
Studies with goals or outcomes outside our study aim and question
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:
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).
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.
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.
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.
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.
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.”
Spiritual suffering—also called spiritual distress or spiritual pain—is defined as significant separations from meaning, purpose, connectedness, and transcendence (e.g., hope).
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).
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.”
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.
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.
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.
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.
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)
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.
in: Joseph S Positive Psychology in Practice: Promoting Human Flourishing in Work, Health, Education, and Everyday Life. 2nd ed. Wiley,
Hoboken, NJ2015: 559-575
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.
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.
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
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,
Helping patients with serious illness live well through the promotion of adaptive coping: a report from the Improving Outpatient Palliative Care (IPAL-OP) initiative.
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.
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).
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.
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.
Results
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).
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.
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.
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).
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).
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.
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)
“…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.”
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,
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.
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.
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.”
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.
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.
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.”
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.”
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.
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.
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.
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;
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;
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.
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.
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,
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
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.
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.
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;
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;
Helping patients with serious illness live well through the promotion of adaptive coping: a report from the Improving Outpatient Palliative Care (IPAL-OP) initiative.
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).
We deemed these forms of oscillation as adaptive, transcendent coping, because of the intra-personal, meaning-making, spiritual formation nature of the process.
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;
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.
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.
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 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.
Furthermore, this tool has been shown to have limited psychometric value, hampering its usefulness and suitability in measuring HD caregivers emotional and psychological distress.
Quality of life in Huntington's disease: critique and recommendations for measures assessing patient health-related quality of life and caregiver quality of life.
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.
Limitations
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.
Next Steps
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.
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
in: Farazmand A Global Encyclopedia of Public Administration, Public Policy, and Governance. Springer International Publishing,
Cham, Switzerland2018: 5736-5742
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;
in: Farazmand A Global Encyclopedia of Public Administration, Public Policy, and Governance. Springer International Publishing,
Cham, Switzerland2018: 5736-5742
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.
in: Farazmand A Global Encyclopedia of Public Administration, Public Policy, and Governance. Springer International Publishing,
Cham, Switzerland2018: 5736-5742
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.
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;
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.
00907-1&id=gr1.jpg){kind=link}
00907-1&id=gr2.jpg){kind=link}
00907-1&id=gr3.jpg){kind=link}