Engaging Specialist Palliative Care in the Management of Amyotrophic Lateral Sclerosis: A Patient, Family, and Provider-Based Approach

Abstract

Purpose:

To describe key stakeholders’ perspectives on specialist palliative care and its integration into the management of amyotrophic lateral sclerosis (ALS).

Methods:

Qualitative, non-interventional, descriptive study. Data collected via individual interviews of 42 stakeholders (n = 14 patients, n = 16 family caregivers, n = 12 healthcare providers). Transcribed interviews were analyzed using inductive thematic analysis techniques.

Results:

Stakeholders’ general impressions of specialist palliative care were highly variable. Many expressed limited or inaccurate understandings of palliative care’s definition and purpose. Perceptions of palliative care as hospice were common. Stakeholders generally supported the integration of specialist palliative care into ALS management, and many recognized the value of early integration of palliative services in both the community and the clinic setting.

Conclusion:

Key stakeholders readily identify a meaningful role for specialist palliative care in ALS management. Integration of specialist palliative care into existing systems of support would be facilitated by a more comprehensive understanding of the service among patients, family caregivers, and healthcare providers.

Introduction

Each year approximately 5,000 individuals are diagnosed with Amyotrophic Lateral Sclerosis (ALS), an incurable, neuromuscular disease characterized by the relentless degeneration of motor neurons. Following diagnosis, patients with ALS survive only an average 2-5 years and experience symptoms of voluntary muscle paralysis, including loss of speech and ability to swallow, walk, and eventually breathe.¹ Alongside these dramatic physical changes, patients and their families confront numerous emotional, social, and spiritual challenges as they attempt to adapt and cope with this all-encompassing, life-limiting disease.²

Given the complexity of issues accompanying an ALS diagnosis, professional organizations increasingly recommend that ALS patients and their families receive specialist palliative care services concurrent with clinic-based neurological multidisciplinary care.^3,4 However, no broadly accepted guidelines define the optimal integration of palliative care (differing from hospice care) into existing clinical frameworks. In fact, most patients with ALS never interact with a specialist palliative care team at any point over the course of their disease.⁴ When in the disease course to most appropriately introduce palliative care into ALS management also remains in question. While there is general support for palliative care integration early in the disease trajectory, optimal timing of specific providers’ involvement is largely lacking in the professional literature.⁵

A number of factors can complicate discussions regarding the optimal role of specialist palliative care in ALS management. First is the reality that patients, families, and clinical staff alike often equate palliative services with end-of-life care⁵ and, thus, may have never considered a role for palliative providers earlier in the disease trajectory. Second is the fact that, even in the absence of specialist palliative care services, ALS care is typically multidisciplinary in its approach.⁵ Consequently, stakeholders may be unclear about what unique role specialist palliative care providers would play on teams that already include physicians, occupational therapists, social workers, physical therapists, nurses, speech-language pathologists, respiratory therapists, dieticians, and others providing general palliation of ALS symptoms. Informing a patient, family, and provider-based approach to specialist palliative care involvement in the management of ALS demands further research.

Study Purpose and Research Questions

In this study, we sought to describe key stakeholders’ perspectives on the integration of specialist palliative care services into the routine management of ALS. Two research questions guided the study: (1) What are the perceptions of ALS patients, family caregivers, and healthcare providers with regard to specialist palliative care services? and (2) How, if at all, do ALS patients, family caregivers, and healthcare providers believe specialist palliative care teams should be involved in ALS care?

Methods

After securing Institutional Review Board (IRB) approval (Project #2009374 and #2009421), we conducted a qualitative, non-interventional, descriptive study,⁶ employing stratified purposive sampling⁷ to recruit patients with ALS, their family caregivers, and healthcare providers involved in ALS care. All study participants were associated with an ALS Association-certified clinic at an academic medical center in the Midwestern United States. Though the clinic was located in a small metropolitan area, the surrounding service area was rural. Participating patients with ALS were age 18 or older; their adult family caregivers were also invited to participate. Both patients and caregivers had to be English-speaking; however, patients could participate with or without assistance. To ensure representation across the full disease course, we stratified the patient and family caregiver sample based on the King’s clinical staging system, which assigns a patient’s disease to 1 of 5 different stages based on the number of central nervous system regions affected and the need for specific interventions such as a feeding tube or help with breathing.⁸ ALS is considered in Stage 1 when only 1 region is involved, and stages increase in number as the disease progresses, ending in the patient’s eventual death (Stage 5). Receipt of specialist palliative care services was not an inclusion criterion for patients or caregivers; some participants had received this type of care, while others had not. All clinic-associated providers were eligible to participate.

During regularly scheduled clinics and support group meetings, providers informed patients and family caregivers of the opportunity to participate in the study and connected interested individuals with the research coordinator. Email was used to inform healthcare providers about the study and invite them to participate. The research coordinator met with all individuals who expressed interest, provided additional study information, and obtained verbal consent from study enrollees, consistent with the IRB-approved protocol. All patient and family caregiver participants received $70 USD to cover any costs incurred as part of their study participation. Providers were allowed to take part in the study during regular working hours and were not provided additional compensation.

Data Collection

Using a semi-structured interview guide, the research coordinator individually interviewed all participants in person or via telephone. Initial questions elicited participants’ existing general impressions and understandings of palliative care. Participants were then provided the Center to Advance Palliative Care’s (CAPC) definition of palliative care.⁹ In addition, the interviewer clarified distinctions between end-of-life care, general palliation, and specialist palliative care services. This understanding allowed more focused questioning for the remainder of the interview, which focused on strategies to integrate specialist palliative care into existing ALS services. Interviews were digitally audio-recorded and ranged in length from 34 to 76 minutes, with an average of 55 minutes. Transcribed interview content and detailed research field notes comprised the final dataset.

Data Analysis

Two researchers used Dedoose, Version 8.0.42 to conduct an inductive thematic analysis.¹⁰ After reviewing the full dataset, the analysts generated an initial list of descriptive codes. They then independently coded approximately 10% of the dataset before collaboratively refining the list of the codes and corresponding definitions. Following coding completion, the researchers consolidated existing codes into potential themes that reflected broader ideas. Themes that demonstrated goodness-of-fit with the data, meaningfully addressed the research questions, and accurately reflected the participants’ perceptions were then finalized.

Results

Three primary themes emerged from the analysis: participants’ general impressions of palliative care, understanding of palliative care, and perspectives on the integration of palliative care into ALS management. Each primary theme and corresponding subthemes are described below, accompanied by illustrative quotations where appropriate. To preserve confidentiality, participant quotations are labeled with alphanumeric study identifiers. Table 1 summarizes characteristics of the 42 study participants, and Table 2 presents additional data in support of identified themes.

Table 1.

Participant Characteristics^a

Characteristic		Patients (n = 14)	Caregivers (n = 16)	Providers (n = 12)
Gender, n (%)b
	Male	7 (50%)	3 (19%)	6 (50%)
	Female	7 (50%)	13 (81%)	6 (50%)
Race, n (%)
	Black/African American	0 (0%)	1 (6%)	0 (0%)
	White/Caucasian	14 (100%)	15 (94%)	12 (100%)
Patient’s ALS Stagec , n (%)
	1	2 (14%)	0 (0%)	-
	2	7 (50%)	5 (31%)	-
	3	4 (29%)	3 (19%)	-
	4	1 (7%)	6 (38%)	-
	5 (death/bereaved)	-	2 (13%)	-
Age Range, n (%)
	< 50 years	0 (0%)	3	-
	50-59 years	6 (43%)	5	-
	60-69 years	5 (36%)	5	-
	70-79 years	2 (14%)	3	-
	80-89 years	1 (7%)	0	-
Relationship to Patient, n (%)
	Spouse	-	10 (63%)	-
	Adult Child	-	2 (13%)	-
	Friend	-	2 (13%)	-
	Parent	-	1 (6%)	-
	Sibling	-	1 (6%)	-

^aNot all characteristics were obtained for all study participant types

^bPercentages may not total 100 due to rounding

^cBased on King’s clinical staging system at the time of study enrollment

Table 2.

Identified Themes and Supporting Data

Theme	Subtheme	Interview Excerpt
General impressions of palliative care	No impression	Provider 8a: “I’m just not 100% [informed] on what [palliative care teams] do.” Family caregiver 11c: “I know [palliative care providers] came to visit at the clinic, but even at that time I was still clueless as to what they had to offer.”
	Negative impression	Patient 9p: “I have not met one [palliative care provider] that wasn't a jerk. In fact, the first one that they sent me to, I fired him immediately. When I come in, they start asking … ‘How do you want to die? Do you want holes in your stomach? Do you want holes in your throat? This is what's going to happen. That’s what’s going to happen.’ To me, that is not … patient caring.” Patient 5p: “[The palliative care provider] was trying to explain to me different medications and stuff, and … I told her that [my current medication] seemed to be working just fine …. I wasn’t having any pain, and … I felt like she was trying to get me on [more] pain medication.”
	Positive impression	Family caregiver 7c: “Anything to do with stress, depression, [or] anxiety [helps].” Provider 5a: “We have … some families that really may not have the skills to know how to deal with kind of that emotional, social, spiritual … part, … so having somebody that’s specifically trained to kind of help navigate that … would be positive.”
Understanding of palliative care	Misunderstanding of palliative care	Provider 9a: “I’ve had plenty of folks say …. ‘I always thought [palliative care meant that] everybody’s written me off, and it’s time to go die.” Interviewer: “When you hear ‘palliative care,’ is that what you think of? End of life?” Family caregiver 1c: “Mmm hmm (affirmative). Is that not it?”
	Limited understanding of palliative care	Provider 10a: “First let me make sure I am remembering the distinction. So, hospice is like we are basically providing minimal comfort care because the patient is going to be dying soon, and [with] palliative care, all treatment is still in full force, but we’re also just providing comfort? Right?” Patient 4p: “How would I interface with [the palliative care team] versus [the ALS clinic providers]? …. I don’t know what the difference would be.”
	Comprehensive understanding of palliative care	Provider 12a: “With ALS … it can go either way, right? It can be … patients catastrophizing or families catastrophizing and not understanding that it's a slow process and no one knows exactly what the timeline's going to be. Or it can be the other way, where people think, ‘Oh, you know, I’ve got time, and it's not a big deal’ … [Palliative care’s role is] helping them understand more clearly, both now and in planning for the future, what they need to prepare for.” Provider 1a: “[Palliative care providers have] specialized training in meeting the complex needs - both symptoms and other things that [one] might need - for patients and families with serious illness, and ALS, by definition, is a serious, progressive illness …. The main focus is to help [patients and families] with where [they] are right now to find an added layer of support to get what [they] need. And so it’s not about dying, it’s about living well with where [they] are right now.”
Integration of palliative care	Timing of palliative care integration	Family caregiver 4c: “[The palliative care team] definitely came in like in a really progressed, terrible stage, almost like, ‘All right, we’re death knocking at your door’ … and that’s how [the patient] was taking it 100% …. She felt like they were asking her to pick out her casket color and outfit … If [palliative care] was introduced in an early stage, and they were with her from the beginning, yeah, that would have definitely [been different].” Provider 12a: “Transition periods. I think that’s [when palliative care] can be especially helpful … because things are changing … but it’s also really helpful … to build a relationship with people before an acute event.”
	Recommended palliative care services	Family caregiver 3c: “Spiritual and emotional [support] would be great for me because sometimes I get to the clinic and I want to address [an upsetting situation], but I don’t have anyone to address it to, you know, a neutral person.” Provider 4a: “There have been issues … when the patients are more homebound and can’t get to clinic. Then how can we help them? … I think that’s a place where [care is] lacking.”

General Impressions of Palliative Care

Prior to being provided with a definition of palliative care, participants’ general impressions were roughly equally divided among three categories: no impression, negative impression, and positive impression. In some instances, participants’ general impressions shifted from no impression or negative impression to positive impression after the interviewer provided specific examples of the services that might be offered by specialist palliative care teams. Additionally, while some participants’ impressions were shaped by their actual experiences with specialist palliative care, several participants had no prior interaction with palliative care providers. Those individuals’ impressions were informed by general knowledge, their understanding of others’ experiences, and their reactions to the definition of palliative care provided during their interviews.

No impression.

Several participants reported having no impression of palliative care prior to participating in the study. This was true for family caregiver 5c, who replied, “What’s the definition of that?” when asked about palliative care. In some instances, receipt of palliative care failed to leave an overall impression on patients and family caregivers. For example, upon initially being asked about palliative care, family caregiver 4c replied, “I’ve never heard of it.” After further explanation, she determined that she had previously met with palliative care providers, despite not recalling having been introduced to the term.

Negative impression.

Some participants expressed generally negative impressions of palliative care. This was particularly true among those who equated palliative care with end-of-life or hospice care. For example, when asked to describe a clinical encounter between a patient and palliative physician, caregiver 1c recounted her daughter’s negative reaction, stating that “she didn’t want to hear that, about end of life and all that kind of stuff.” Some study participants were wary of palliative care because they assumed that palliative care patients would not be financially covered for concurrent disease-directed treatment, as they believed was true for patients receiving hospice. Participant 9c explained that she had been cautioned against palliative care: “Everybody’s like, ‘Don’t go through them. They’re going to charge you an arm and a leg as soon as you do this and that.’” Similarly, immediately after being read the definition of palliative care, family caregiver 6c asked, “Now would that be an insurance thing?” Several other factors led to negative impressions of palliative care including an aversion to spiritual care (e.g., patient 12p: “I feel that is a personal choice and not to be addressed by the healthcare community”), a lack of privacy in the ALS clinic (e.g., caregiver 12c:” “[The patient] would want to be sitting right there …. listening to everything [I] said”), and a perception that palliative providers were overly focused on pain medications (see Table 2).

Positive impression.

Participants expressing a positive impression of palliative care described appreciation for a holistic healthcare approach. Patient 13p described specialist palliative care as “wonderful to help meet [his] needs,” while family caregiver 5c noted the value in support across “a journey not only of the patient, but … of everybody that’s involved.” While some participants noted an aversion to spiritual care as part of specialist palliative services, others viewed this positively. Patient 10p, for example, identified deeper spiritual support as potentially beneficial because “when faced with [ALS], [faith issues] do come up.” Several others’ positive impressions of palliative care were due in part to the availability of extra emotional support. Patient 6p, for example, stated that it is “good to know that even if you were someone who was experiencing depression and needed some extra help with that symptom of the disease, that [the palliative care team] would help … manage that.”

Some individuals’ impressions of specialist palliative care grew more positive after they received a definition of palliative care and were provided with specific examples of how palliative specialists might be involved in ALS care. For example, after hearing the definition of palliative care, patient 3p responded, “it’s a little bit more in depth than I was actually thinking.” Similarly, caregiver 16c stated, “I didn’t really realize [palliative care] also encompassed the caregiver and the family.”

Understanding of Palliative Care

Researchers identified three subthemes that characterized participants’ understanding of palliative care: misunderstanding of palliative care, limited understanding of palliative care, and comprehensive understanding of palliative care. In some cases, participants expressed views consistent with more than one of these subthemes. For example, a participant might have indicated that palliative care is only available at end of life (which is inaccurate and, thus, an example of a misunderstanding of palliative care) but also described palliative services in vague or superficial terms (e.g., “keeping people comfortable”), which reflected an accurate but limited understanding of palliative care.

Misunderstanding of palliative care.

The most common misunderstanding of palliative care was its equation with end-of-life care. Provider 4a explained that the first thing that came to mind when she heard the term “palliative care” was “care for an individual who’s dying.” Similarly, family caregiver 9c described palliative care as “total end-of-the-line care.” Many providers indicated that this misperception was a common barrier to the integration of palliative care into ALS management. Furthermore, confusing palliative care with hospice was described as specifically problematic, as negative hospice experiences had been shared widely in an effort to warn patients and families about possible restrictions on the treatment options covered under hospice. As a result, some held beliefs similar to provider 3a, who stated, “I don’t like hospice organizations. Period. End of story.”

Limited understanding of palliative care.

Several participants had a general idea of what palliative care entailed but struggled to provide specific examples of palliative services. These stakeholders were characterized as having a limited understanding of palliative care. Individuals expressing this perspective used vague phrases to describe palliative care such as “providing comfort” (10a) or “just like a pain management thing” (5p). Among those with a limited understanding of palliative care, several indicated that the definition of palliative care they received as part of the study protocol was insufficiently specific. Patient 6p, for example, remained confused after reading the definition, stating, “I guess … I’m trying to envision the difference between [my neurologist] and palliative care doctors.”

Comprehensive understanding of palliative care.

Some participants appeared to have a more comprehensive understanding of palliative care. Individuals expressing this perspective easily distinguished between palliative and end-of-life care. Rather than providing general descriptions of palliative care, they gave detailed examples of ways palliative care could be helpful to individuals with ALS, as exemplified in provider 12a’s discussion of emotional and spiritual support (see Table 2). A comprehensive understanding of palliative care was not identified among patients and family caregivers. Those who appeared to have an in-depth understanding of certain aspects of palliative care (e.g., advance care planning) also demonstrated either a misunderstanding of palliative care (e.g., equating it with end-of-life care) or a limited knowledge of palliative services (e.g., unawareness of family caregiver support).

Integration of Palliative Care

In addition to discussing their general impressions and understanding of palliative care, study participants shared their thoughts on if and how specialist palliative care teams should be engaged in ALS management. Two subthemes were identified under the primary theme integration of palliative care: timing of palliative care integration and recommended palliative care services.

Timing of palliative care integration.

Study participants were specifically asked to share their views on the ideal timing of palliative care integration. There was broad consensus among participants that the specific pattern of palliative care’s involvement should be based on assessment of patients’ and families’ unique needs; however, views on when palliative care should be first introduced were more variable. A majority of participants supported early introduction of palliative care into the management of ALS, with the caveat that initial meetings should focus on building rapport rather than detailed planning for end of life. Provider 2a explained, “They don’t necessarily have to immediately start talking … about everything, but at least the introduction needs to be there. And then if they would like to … get into [advance care planning], I think it should be up to the patient and the family.” Caregiver 4c attributed stakeholders’ equation of palliative and end-of-life care to the reality that many patients and families are not offered palliative services until the disease is very advanced, suggesting that earlier palliative care involvement would help dispel this misperception (see Table 2). A few participants opposed early palliative care integration due to its perceived association with dying. Provider 6a, for example, suggested that the primary ALS care team should slowly introduce the idea of palliative care, giving patients and families time to “get used to that word and [not just] associate it with death.”

Participants also focused on more specific aspects of the timing of palliative care integration. In the healthcare system from which study participants were recruited, patients met with numerous different providers during a typical visit. As a result, several study participants worried about additional appointments in patients’ clinic days. Provider 7a explained, “Sometimes [patients and families] will get really, really upset by having to wait [in clinic]. They drove three hours …. They started out at 5:00am, and it’s 10:00am now, and they’re tired, and they’re hungry, and they’re waiting … on someone to come in.” On the other hand, patient 2p viewed palliative care as a potential solution to the problem of wait times rather than an additional scheduling challenge. From her perspective, palliative care could easily be scheduled for otherwise empty time slots: “There’s gaps [in clinic days] …. When we went in July, we were … there for probably a couple of hours and didn’t see anybody.”

Recommended palliative care services.

Most participants were able to easily identify valuable services that were or could potentially be delivered by palliative care providers. These recommended palliative care services spanned the full biopsychosocial and spiritual realm. Specific physical needs included management of symptoms such as pain and shortness of breath. Caregiver 2c, for example, explained how palliative care providers helped address her mother’s problems with swallowing: “They were able to … make suggestions of other … medications that she might try because things didn’t seem to be working …. [They suggested] things we [hadn’t previously had] a clue about.” Participants also identified a need for support in managing challenging family dynamics in addition to more general psychological and existential distress. For example, caregiver 8c described a highly conflicted relationship with her mother-in-law, the patient’s mother. She stated, “I have asked my doctor for medication to deal with [her] because she upsets me so badly and hurts me so badly.” In describing a potential role for palliative care, she explained, “Even if it’s just somebody to validate my frustration and my sense of being overwhelmed and attacked, that’s all I need …. Nobody can fix it, but if they can just give me some tips and tools on how to just stay sane and set healthy boundaries [it would be helpful].”

In addition to services recommended for the clinic setting, several study participants emphasized the need for palliative services in the community. Provider 1a explained, “Office-based palliative care doesn’t meet the need when something happens and you need help today …. A just-in-time, home-based palliative care delivery system [is needed].” Many patients and family caregivers cited in-home help (e.g., a bath aide) as one of their most pressing needs. While provider 9a cautioned that personal care may fall outside palliative care’s scope of practice (“It wouldn’t be the kind of home health that [families are] hoping for”), several participants identified coordination of these community-based services as a potential role for palliative care, citing a need for a care “manager” or “coach.”

Discussion

Patients, family caregivers, and healthcare professionals are key stakeholders in ALS care.¹¹ The findings generated in this study highlight the specific importance of these individuals’ general impressions and understanding of palliative care and their views on the integration of palliative care into existing healthcare offerings.

Prior research provides important context for these study findings. In some respects, the themes identified in this study are consistent with previous studies. For example, a 2016 review of research on barriers to greater integration of specialist palliative care identified insufficient knowledge and misperceptions of palliative care as significant problems.¹² A more recent analysis of nationally representative survey data found that approximately 71% of Americans have no knowledge of palliative care, consistent with individuals in this study who were found to have no impression of the service.¹³ In addition, the value of early palliative care integration has been recognized often enough to have been incorporated into the official recommendations of numerous professional organizations.⁴ However, this study also adds to the existing knowledge base by shedding light on specific concerns of ALS stakeholders. For example, many individuals in the present study who equated palliative and end-of-life care opposed the integration of palliative care into ALS management specifically due to concerns about a lack of coverage for treatments such as tube feedings and mechanical ventilation. In most cases, after study participants learned that palliative care was not synonymous with hospice and could be offered concurrent with other treatments, their attitudes toward palliative care improved considerably. This study’s findings also highlight patients’ and family caregivers’ views on the unique advantages of home-based palliative care over clinic-based services, particularly given many patients’ significant mobility limitations and in-home care needs.

Practice Implications

These research findings have a number of implications for practice. First, it is clear that many stakeholders would benefit from a more comprehensive understanding of palliative care. Palliative care should be differentiated from hospice and other end-of-life care, and specific, concrete examples of services available through the specialist palliative care team should be provided. Second, study participants’ perspectives offer support for existing recommendations⁴ that palliative care be introduced early in the ALS disease trajectory. Early integration would allow more time for providers to develop rapport with patients and families before addressing potentially sensitive subjects and would increase opportunities to connect families with resources to reduce caregiving burden, which has been identified as a significant concern.² Early integration would also reinforce the idea that palliative care is appropriate at all stages of disease progression. Finally, on a broader practice level, these findings highlight the potential value of community-based palliative care for patients with ALS and their families. Arranging transportation and accommodating patients’ mobility limitations can make clinic visits decidedly stressful, and patients’ complex care needs often necessitate appointments with many different providers on clinic days, leading to fatigue.¹⁴ Furthermore, clinic settings may offer limited space for private (and, when necessary, separate) meetings with patients and their families. Community-based palliative care could address many of these concerns, while delivering services where they are most needed. It would be imperative, however, to inform patients and family caregivers about any service limitations, such as an inability to provide regular assistance with personal care, to avoid unrealistic expectations.

Study Limitations

Several study limitations warrant consideration. All study participants were recruited from the same ALS clinic, and the study sample lacked diversity. Potential transferability of these research findings to other settings may therefore be limited. Furthermore, not all study participants were initially familiar with specialist palliative care, which meant that some were discussing the integration of a service about which they had limited knowledge. Future studies including a broader range of participants who have directly experienced specialist palliative care are needed to build on the research findings detailed here.