Stakeholder Perspectives on the Biopsychosocial and Spiritual Realities of Living with ALS: Implications for Palliative Care Teams

Abstract

Context

Amyotrophic Lateral Sclerosis (ALS) is an all-encompassing, life-limiting disease, resulting in the eventual paralysis of all voluntary muscles and concurrent loss of independence. As the disease advances, both patients and their family caregivers develop complex biological, psychological, and social needs, leading to increasing calls for the involvement of palliative care teams in the management of ALS.

Objective

The purpose of this study was to generate a rich description of the realities of living with ALS, equipping palliative care teams with an in-depth understanding of the experiences and needs of patients with ALS and their family caregivers.

Methods

This study employed a mixed methods design, with quantitative data supplementing a larger body of qualitative data. Semi-structured interviews with 42 key stakeholders, including patients, family caregivers, and healthcare providers, were analyzed for themes essential for effective understanding of ALS.

Results

Identified themes were organized into two broad categories: 1) biopsychosocial needs of ALS patients and family caregivers and 2) the impact of ALS on spiritual and emotional wellbeing. Quantitative data supported the recognized themes, particularly with regard to challenges associated with preserving independence, securing sufficient social support, and managing the emotional complexities of the disease.

Conclusion

Study findings illustrate the intricacies of living with ALS and the importance of eliciting individualized values when caring for patients with ALS and their families. The complex biopsychosocial needs experienced by patients and family caregivers suggest numerous opportunities for meaningful palliative care involvement.

Introduction

Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease that affects motor neurons, resulting in muscular atrophy and eventual paralysis. With an average life expectancy of 3–5 years following diagnosis, patients with ALS experience a loss of autonomy and ability to perform activities of daily living, leading to a decline in quality of life.¹ Along with losing their ability to move and communicate, many ALS patients also experience cognitive and behavioral changes such as apathy and impulsivity.² Faced with a poor prognosis and no foreseeable cure, patients with ALS become progressively dependent on their family caregivers, which often results in both the patient and caregiver becoming socially marginalized. Confronted with numerous stressors and little training, these family caregivers often experience significant caregiver burden.³ The all-encompassing nature of ALS, paired with its profound impact on patient and family wellbeing, has led to increasing calls for the integration of palliative care into ALS management and research on how to incorporate these palliative services in a patient- and family-centered way.⁴

The Biopsychosocial Model of Illness

The biopsychosocial model of illness conceptualizes diseases such as ALS as multidimensional, highlighting the ways in which social, psychological, and behavioral factors interact with the physical realities of the illness.⁵ Healthcare informed by a biopsychosocial conceptualization attends not only to patients’ medical needs, but also promotes their emotional and social quality of life. In addition, a growing recognition of spirituality’s importance for many individuals with life-threatening illnesses has stimulated advocacy for a broader biopsychosocial-spiritual conceptualization of serious illness care.⁶

A biopsychosocial understanding of ALS underpins the American Academy of Neurology’s recommendation that patients with ALS and their families receive care from a multidisciplinary team of providers who are equipped to address patients’ physical and psychosocial needs.^2,7 The reality, however, is that patients’ physical needs are often so great and their functional decline so rapid that these issues become the predominant focus of clinical encounters, leaving numerous social, emotional, and spiritual needs unaddressed.⁸ While integration of specialist palliative care teams into ALS care has been suggested as a strategy to more comprehensively address patients and families’ needs,⁴ some palliative care providers report a lack of extensive experience caring for patients with complex neurological disorders.⁹ Thus, they may benefit from a greater exposure to the realities of living with ALS.

Study Aims

In this paper, we aim to describe the realities of living with ALS to enhance palliative care teams’ ability to meaningfully contribute to the care of patients and families faced with this devastating disease. Informed by a spiritually-inclusive biopsychosocial model of illness, we sought to accomplish the following aims: 1) describe the lived experience of ALS for patients and their family caregivers, and 2) identify the needs of patients with ALS and their families.

Methods

We performed a qualitative-focused mixed methods study, using a concurrent nested design.¹⁰ To recruit patients with ALS, their family caregivers, and healthcare providers involved in ALS care, we employed stratified purposive sampling.¹¹ All study participants had either a direct or indirect association with the single recruitment site, an ALS Association-certified clinic in a small metropolitan area in the Midwestern United States. The University of Missouri Institutional Review Board approved the study (IRB Project #2009374 and #2009421).

To be eligible, participating patients had to be 18 years of age or older, have a clinical diagnosis of definite or probable ALS (based on El Escorial criteria¹²), have experienced at least one visit to the ALS clinic prior to enrolling in the study, and be able to participate with or without assistance in an interview in English. To ensure covering the variability of needs throughout the entire disease course, we stratified our patient sample based on ALS staging using the King’s staging system.¹³ Participating family caregivers were required to be English-speaking adults who identified as a family caregiver or a “support person” for a patient with ALS. Bereaved caregivers who met the eligibility criteria, maintained a relationship with the local ALS care community, and expressed an interest in the study were also invited to participate. During clinic visits and support group meetings, patients and caregivers were informed of the study and, if interested, were contacted by the on-site research coordinator. She provided study details and read aloud the informed consent statement before officially enrolling study participants who provided verbal consent. We compensated all participating patients and family caregivers $70 in appreciation for their time and to cover any costs incurred as part of the study.

We invited all healthcare providers affiliated with the ALS clinic to take part in the study via email. The research coordinator reached out to individuals who expressed interest, providing them with additional information before obtaining their verbal consent. Since providers were granted permission to participate in the study during regular working hours, they were not given additional compensation.

Data Collection

All participants took part in a semi-structured interview in which they discussed the biopsychosocial and spiritual realities of living with ALS as well as any changes that could be made to their current management of ALS. Initial questions were designed to elicit participants’ overall understanding of ALS (e.g., How would you describe ALS to someone who didn’t know much about the disease?). The later portion of the interview focused specifically on how ALS affects the personal, emotional, financial, and spiritual realms of patients and caregivers’ lives. Interviews for patients and caregivers lasted between 34 and 76 minutes (average: 55 min), while interviews with healthcare providers lasted between 34 and 60 minutes (average: 47 min). Except for two patients whose responses were submitted in writing due to communication challenges, the research coordinator conducted interviews either over the phone or in person, based on participant preference. Audible interviews were digitally audio-recorded, de-identified, and transcribed by a third party prior to analysis.

In addition to taking part in a qualitative interview, patient participants completed the 40-item ALS Assessment Questionnaire (ALSAQ-40),¹⁴ which was used to measure patients’ subjective wellbeing. ALSAQ-40 subscales included items focused on physical mobility (10 items), activities of daily living (10 items), eating and drinking (3 items), communication (7 items), and emotional functioning (10 items). Patients completing the ALSAQ-40 were asked to indicate how often during the prior two weeks they had experienced specific problems or challenges related to living with ALS. Responses ranged from never (0) to always (4), with higher scores reflecting poorer subjective wellbeing.

Data Analysis

We used Dedoose Version 8.0.42 (Los Angeles, CA) to identify, organize, and describe the qualitative data using inductive thematic analysis techniques.¹⁵ Following data collection and transcription, the first two authors reviewed all the transcribed content and generated an initial list of descriptive codes. They then independently coded the same four interviews (approximately 10% of the data) and compared coding decisions to make needed refinements, assuring satisfactory (i.e., ≥ 80%)¹⁶ inter-coder agreement. After independently coding the remaining interviews, they merged similarly coded data, resulting in broader themes that described patients and families’ experiences and needs. For example, trouble with cooking was first merged with other practical issues and later organized under the theme of need for additional caregiving help.

We analyzed quantitative data by calculating basic descriptive statistics for all ALSAQ-40 items and subscales using the scoring algorithms established by the instrument’s developers,¹⁷ which transformed subscale scores into a calculated score ranging from 0 to 100, allowing for interpretation according to the following classification: 0–9 = no problems, 10–39 = problems rarely, 40–59 = problems sometimes, 60–79 = problems often, and 80–100 = problems nearly always. By noting areas in which patients reported frequent challenges, we were able to further develop our understanding of the impact of ALS on patients and caregivers’ everyday lives.

Results

Below we present our study results and findings, which we have organized into three sections. First, we give an overview of participants’ basic demographic characteristics. Second, we summarize patients’ ALSAQ-40 subscale scores, using quantitative results to describe patients’ subjective wellbeing with regard to physical mobility, activities of daily living, eating and drinking, communication, and emotional functioning.¹⁴ Finally, we present qualitatively-derived themes that describe patients and caregivers’ experiences and needs, providing illustrative quotations and, when helpful in understanding the theme’s meaning, information drawn from individual items from the ALSAQ-40.

Participant Characteristics

We interviewed fourteen patients (7 female and 7 male). All self-identified as Caucasian and were age 50 or over: 50–59 years old (n = 6), 60–69 years old (n = 5), and 70 years old or older (n = 3). ALS clinical staging by the team neurologist showed 2 patients in stage 1 (early stage of disease progression), 7 in stage 2, 4 in stage 3, and one in stage 4 (late stage of disease progression). All patients were living in a private residence in the community. Just over half (8 of 14) were supported by a full-time caregiver. Half (7) used a power wheelchair, 3 received nutrition and hydration through a percutaneous endoscopic gastrostomy (PEG) tube, and 6 used a cough assist machine and mechanical suction to prevent pulmonary aspiration. Six were assisted in breathing via non-invasive ventilation (e.g., bilevel positive airway pressure (BiPAP) machine), and one relied on mechanical ventilation via tracheostomy.

Sixteen family caregivers (13 female and 3 male) were interviewed, including 3 bereaved caregivers. Fifteen self-identified as Caucasian, and 1 self-identified as African American. Caregivers’ ages fell into the following categories: 49 years old or younger (n = 3), 50–59 years old (n = 5), 60–69 years old (n = 5), and 70 years old or older (n = 3). Participating healthcare providers (n = 12) had a wide range of years spent working with patients with ALS: 0–2 years (n = 3), 3–9 years (n = 6) and 10 or more years (n = 3). An equal number of male (n = 6) and female (n = 6) providers took part in the study, including individuals representing a broad range of health professions (e.g., chaplaincy, medicine, nursing, nutrition, social work, speech language pathology).

Quantitative Results: ALSAQ-40 Subscale Scores

Patients’ mean score on the ALSAQ-40 Physical Mobility subscale was 70.71 (SD = 32.91), reflecting frequent problems in this domain. The Activities of Daily Living/Independence subscale mean was 59.29 (SD = 28.85), falling just on the border between “sometimes” and “often” with regard to the frequency of problems experienced in this area. The sample’s Emotional Functioning subscale mean fell into the “sometimes” range ($\stackrel{-}{x}$ = 46.25, SD = 24.41), while the mean subscale scores for Communication $(\stackrel{-}{x}$ = 35.20, SD = 36.79) and Eating and Drinking $(\stackrel{-}{x}$ = 33.93, SD = 36.47) both fell into the “rare” frequency range.

Qualitative Findings: Biopsychosocial Needs and Spiritual Realities of Living with ALS

For organizational clarity, we grouped qualitative themes into two broad categories. In the first category we placed themes and data relating to the biopsychosocial needs of patients with ALS and their families, including the need for additional caregiving help, education on ALS and ALS care, social support, and transportation and other resources to preserve independence. While the first category related to expressed needs, when discussing the spiritual reality of living with ALS, participants described spiritual coping and its effect on their emotional wellbeing. Hence, the second theme category included information about the relationship between the spiritual and emotional aspects of patients’ lives.

Biopsychosocial Needs

Patients, caregivers, and healthcare providers described numerous biopsychosocial needs experienced by patients with ALS and their family caregivers. Table 1 provides illustrative quotations concerning themes derived from these identified needs.

Table 1.

Biopsychosocial Needs of ALS Patients and Family Caregivers

Identified Need	Illustrative Quotations
Additional caregiving help	“You just get so tired, you know? …. I didn’t think he’d last until Christmas, and then when we made it through Christmas and we made it through that year’s anniversary, probably depression kind of set in. I never labeled it that because I never wanted him to think that I was depressed about him still hanging on.” (Caregiver 15)
	“It was impossible to hold a job … and worry about coming home and finding her on the floor.” (Caregiver 7)
	“[Overwhelmed patients] have to pay out-of-pocket for somebody to come in and take care of them, and if they don’t have that money, then they have to go to a different state …. We have no nursing homes or skilled nursing facilities [in the state] that will take ventilators.” (Provider 2)
Education on ALS and ALS care	“Maybe it’s denial, but he still feels that if she went and used those muscles and worked really hard that she could regain a lot of what she’s lost. It’s just not the way we understand ALS to be.” (Caregiver 14)
	“How to help them shower and bathe, sponge bathing, just practical advice on how to physically care for them as they start to deteriorate - we’re starting to reach that point - that would be really helpful for me. Who do I call or do I go visit with who can just give me some tips or even show me a video?” (Caregiver 8)
	“It would be good, instead of having to ask somebody as important as [the neurologist], … to have an individual that is capable of explaining the symptoms and what’s occurring.”(Patient 8)
Social Support	“People don’t pay attention to you. I’ve always been a very chatty person …. People don’t engage you. I sound like I’ve been drinking, and that’s probably what they think. They’re like, ‘Oh, wow. 7:00 in the morning, she’s already drunk. ’” (Patient 10)
	“Nobody calls or hangs around. And you get to a point where you don’t want to go out. We’re homebodies. And that has a lot to do with pride again, because he doesn’t want people to see him like that, because he was so active. I’m like, ‘Let’s go to [another city] and visit this or that person,’ and he doesn’t want to do that because he doesn’t want them to see.” (Caregiver 11)
	“People are afraid of people in a wheelchair. They’re afraid of people who are sick. It’s weird and different, there’s stigma involved …. The patient and the families start becoming isolated from their prior social contacts, their social group. It’s extremely detrimental.” (Provider 13).
Transportation and other resources to preserve independence	“They get in this wonderful chair that gives them their freedom, their independence, and their mobility needs, and then we have no way of transporting them to go grocery shopping or go pay bills or just go out on a date with … their spouse.” (Provider 3)
	“Trying to find a vehicle [was really hard for us]. ‘Oh my gosh, he’s getting this wheelchair delivered, but there’s no way [to transport it].’ And I purchased one of those cargo carrier things that hook into your hitch with the ramp, but then after finding out how much that wheelchair costs, there is no way I was going to tow it on the back of the car …. [Now] we have a van, it’s a 12 passenger. We took out some of the seats because his wheelchair is so big. It’s come in very handy, but he can’t be transported in it [while in the wheelchair] because he’s so tall [and] the wheelchair is so tall.” (Caregiver 11)

Additional caregiving help

The most frequently cited need was for additional caregiving support in the home and community, which was identified 395 times in the dataset. Specific needs included finding affordable skilled nursing personnel or care facilities, locating appropriate respite care resources, and addressing practical needs in the home (e.g., cooking, tending to hygiene, transferring the patient). Several caregivers discussed their personal experiences and fears of being forced to relocate to neighboring states due to the lack of skilled nursing facilities offering ventilator support in the state in which they resided. Others described significant challenges finding affordable and qualified direct care providers, as neither informal “sitters” nor certified nursing assistants (CNAs) were able to fully substitute for the family caregiver. For example, one caregiver cited the practical limitations of leaving patients with ALS to be cared for by CNAs who were not permitted to perform tracheostomy care. Patients also broadly endorsed that additional caregiving support was a pressing need. Nearly all patients (93%) reported that they sometimes, often, or always worried about being a burden to others.

Education on ALS and ALS care

Study participants identified an overwhelming need for patient and caregiver education on ALS, both in terms of the disease itself and with regard to caring for a person with ALS. This need was identified 389 times in the dataset. Some described needs pertained to caregiver training for routine daily functions (e.g., using adaptive equipment for transfers, fall prevention, hygienic care). Others focused on the need for support in care coordination, including help navigating the complexities of health insurance and organizing more feasible access to community resources. Patients and caregivers exhibited a varying degree of basic disease understanding, suggesting a need for more education on disease mechanisms and the expected progression of ALS. Study participants also described how individuals’ coping responses could influence their understanding of ALS. For example, some suggested that patients and families who used avoidance coping mechanisms were unlikely to seek out information on ALS and ALS care, which resulted in potential knowledge and skill deficits.

Social support

In 243 instances, study participants noted a significant loss of social interaction and support for patients with ALS and their family caregivers. Caregivers cited a decrease in personal time, limited contact with friends and family, and feelings of guilt associated with caregiving burden. Over half of patients (57%) reported sometimes, often, or always experiencing feelings of boredom or loneliness. Some described purposefully limiting social interactions due to fear of embarrassment associated with functional limitations (e.g., need for a caregiver to cut food prior to eating, risk of choking, slurred speech). Both patients and caregivers acknowledged social groups as beneficial when coping with loss of independence, loss of privacy, and guilt. Several caregivers specifically cited a need for a network composed of relatable peers to prompt the exchange of information and experiences.

Transportation and other resources to preserve independence

Resources to preserve independence were the fourth most often identified need, mentioned on 211 occasions. Participants identified physical mobility as the most salient factor affecting patients’ independence. A lack of transportation that accommodated the relatively large wheelchairs used by many individuals with ALS left many patients predominantly homebound. Most patients and families found the substantial cost of owning or leasing a wheelchair accessible van prohibitive, and study participants cited numerous barriers to other transportation options, particularly in more rural areas. Lack of accessible transportation was cited as a reason for decreased social interaction, absence from religious services, and missed clinical appointments.

Impact of ALS on Spiritual and Emotional Wellbeing

On 157 occasions, patients, caregivers, or healthcare providers discussed the impact of ALS on patients’ spiritual and emotional wellbeing. Table 2 provides illustrative quotations for this category.

Table 2.

Impact of ALS on Spiritual and Emotional Wellbeing

“Reframing hope to ‘Today’s going to be a good day. We’re going to be able to maximize time with family,’ things like that, is … the big picture piece about what palliative care delivers.” (Provider 1)

“For the … first short period of time, you know … emotionally it was, ‘Woe is me, poor me. Why, why me?’ But that was just a short period of time. From then on … I turned over my thoughts and my feelings and my emotions to the Lord. I just said, you know, ‘This is something beyond me. There’s nothing I can do. You’re the only one that can help me through this, so I turn it over to you, Lord.’ So I haven’t given up hope …. Sometimes I get down, but … I always hang onto hope.” (Patient 4)

“I think those who might be more involved in their spiritual community … tend to have more [people who] might come and help them out, whether it be that they help with patient care, whether it be making meals, whether it be just giving a family member time away from home. And, you know, just the thought of prayer. I think there’s a lot of power in prayer.” (Provider 8)

We asked participants about ALS’ effect on patients’ spirituality, feelings toward God or a higher power, and perception of life’s meaning. Patients expressed multiple ways ALS affected their spirituality. Most (n = 9; 64%) identified ways ALS had strengthened their spirituality, three (22%) reported no change in their spirituality, and two (14%) reported negative spiritual consequences of ALS such as confusion or anger at God. Positive spiritual effects of ALS included increased religious service attendance and interactions with a spiritual community (reported by 29% of patients), and an increase in prayer and self-reflection (reported by 35% of patients). Among those who qualitatively reported an increase in engagement with a spiritual community following their ALS diagnosis, we noted lower self-reports of hopelessness and depression. When responding to the ALSAQ-40, patients who reported an increase in religious service attendance and interactions with spiritual community reported never, rarely, or sometimes feeling hopeless about their future or feeling depressed.

Discussion

This study explored the complex realities of living with ALS, with particular focus on the biopsychosocial needs of both patients and family caregivers. Consistent with other research,^18,19 these findings make clear that ALS takes a severe physical and psychological toll. As ALS patients experience diminished physical function and become unable to move, communicate, tend to personal hygiene, and live independently, they experience diminished autonomy and, often, reduced feelings of self-worth. Similarly, family caregivers are confronted with progressing levels of dependency, increasing physical care demands, and the need for potentially long-term life adjustments, which stress caregivers’ mental and physical health. The urgency of this matter is reflected by the majority of patients who reported that they sometimes, often, or always worried about being a burden to others, in conjunction with numerous caregiver narrations identifying the immediate need for home help assistance with practical tasks such as cooking and bathing. Patients and caregivers also identified a need for support in overcoming guilt associated with feeling like a burden (patients) or feeling burdened (caregivers).

Study findings emphasizing the importance of transportation and other resources to promote patient independence are also supported by existing literature. For example, a recent study identified travel demands and reduced mobility as one of the main barriers to patient attendance at multidisciplinary ALS clinics, thus affirming the importance of accessible transportation.²⁰ Research has also previously acknowledged a need for education on ALS and ALS care, as was identified in the present study. For example, a recent study found that over 80% of ALS patients and caregivers sought out additional information after receiving an ALS diagnosis, despite being initially satisfied with the way in which their diagnosis was disclosed and explained.²¹ Extant research also shows support for spirituality as a coping resource for patients with ALS and their families, as indicated by the present study findings. Specifically, studies have shown that personal spirituality and participation in a community of faith can sustain hope and diminish despair,²² while religiousness has been shown to have a positive correlation with caregiver quality of life.²³

Implications for Palliative Care Teams

Study findings reveal numerous opportunities for palliative care teams to support patients with ALS and their family caregivers. Participants voiced a need for patients and caregivers to access additional caregiving help, assistance in obtaining resources and coordinating care, and increased social, spiritual, and emotional support, all of which are fundamental to the practice of the interdisciplinary palliative care team.²⁴ While all members of the palliative care team share responsibility for promoting patients and families’ overall quality of life, nurses and social workers are especially well equipped to help patients navigate the complex healthcare system, while chaplains and social workers possess expertise to address spiritual and emotional needs. In addition, the progressive deterioration of patients’ physical abilities, increasing caregiver dependency, and the need for ongoing symptom management highlight clear roles for palliative physicians and nurses.

While best practices regarding integration of palliative care into ALS management are still being defined, palliative care providers are typically accustomed to working from a consultation model,²⁵ and are well versed in negotiating roles and responsibilities with other care teams.²⁶ These characteristics of palliative teams would likely prove useful in integration efforts. Further, while the field continues to grapple with the best time to introduce palliative care to patients with ALS,²⁵ the present study identified numerous opportunities for palliative care involvement early in the disease trajectory as patients and families begin to come to terms with the diagnosis and plan for the future. Thus, our findings align nicely with arguments for early palliative care intervention in ALS management due to the typically rapid progression of the disease and need for a proactive versus reactive plan of care.²⁷

Study Strengths and Limitations

A number of study strengths and limitations warrant attention. This study was strengthened by its mixed methods design,¹⁰ which facilitated an in-depth exploration of the realities of living with ALS alongside an examination of quantitative data measuring patients’ wellbeing in a broad range of key domains. An additional strength of the study was our inclusion of numerous stakeholders, which allowed us to capture a variety of different perspectives, including those of providers who drew on years of experience in ALS care in discussing the experiences and needs of patients with ALS and their family caregivers. With regard to limitations, because we collected data from only one clinic in a small, predominantly Caucasian metropolitan area, the generalizability or transferability of our research findings are limited. It is also important to note that several of the study participants traveled to the clinic from more rural parts of the state and may, thus, have reported experiences and needs unique to rural settings. Further, in this region, patients who receive most of their healthcare from Veterans Affairs (VA) providers receive ALS care in the clinic from which we recruited participants, which operates outside of the VA system. Because veterans may be entitled to benefits not available to all individuals with ALS,²⁸ our findings likely differ from those that would have been generated from a study of exclusively VA or non-VA patients. Finally, participants may have provided responses believed to be viewed as favorable by the research team. Future studies would benefit from inclusion of larger samples drawn from multiple clinical sites and involvement of researchers less familiar to potential study participants.