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Hard to swallow: a phenomenological exploration of the experience of caring for individuals with myotonic dystrophy and dysphagia.

ABSTRACT

Purpose: Myotonic dystrophy (DM1), a genetic, multisystemic disorder, is the most prevalent adult form of muscular dystrophy. Dysphagia is a common symptom that may be difficult to diagnose and treat and can be associated with increased morbidity and mortality. Preexisting cognitive impairment or apathy, both well described in the DM1 literature, may contribute to management challenges. Caregivers may become important for managing a family member's swallowing dysfunction. Although clinicians place great importance on swallowing difficulties, it is unknown how dysphagia impacts patients and their caregivers. Therefore, the purpose of this study was to explore the experiences of caregivers living with those with DM1 and dysphagia. Methods: An interpretive phenomenological approach was used to study the lived experience of six caregivers for individuals with DM1 and dysphagia. Audio-taped semistructured interviews were used for data collection, and data were analyzed using van Manen's steps for phenomenological analysis. Findings: Despite the potential for dysphagia to cause morbidity and mortality in individuals with DM1, caregivers did not describe this as a problematic symptom. Instead, they highlighted more debilitating symptoms like fatigue or weakness and discussed the caregiving experience. Themes pertaining to participants' lived body, lived relationality, lived time, and lived space were identified. Conclusions: Healthcare providers need to balance issues of clinical concern with those that are important for individuals and their family members. Assessments of caregiver knowledge and burden at each clinic visit may be warranted.

Keywords: caregiver, dysphagia, myotonic dystrophy, phenomenology, qualitative research

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Myotonic dystrophy (DM1) is an inherited, chronic, and progressive neuromuscular disorder that may occur rarely at birth (congenital form) or more commonly manifest during adulthood. Given the autosomal dominant inheritance, DM1 generally affects roughly 50% of every generation of a family. Complications of DM1 include muscle weakness, fatigue, hypersomnolence, cardiac conduction deficits, cognitive dysfunction, apathy, and gastrointestinal manifestations including dysphagia (Harper, 2001; Turner & Hilton-Jones, 2008).

Dysphagia is defined as difficulty swallowing foods or liquids safely that may result in malnourishment or an aspiration pneumonia that occurs when foreign substances enter the lungs (National Institute on Deafness and Other Communication Disorders, 2014). Swallowing difficulties are common in DM1 and may affect between 25% and 80% of patients (Bellini et al., 2006; Ronnblom & Danielsson, 2004). Individuals experiencing dysphagia may report coughing while eating, heartburn, chest pain, or reflux (Bellini et al., 2006). Dysphagia may cause embarrassment and lead to decreased social participation, low self-esteem, and poor quality of life. Relationships with caregivers may also be negatively affected by an individual's inability to share meals (Ekberg, Hamdy, Woisard, Wuttge-Hannig, & Ortega, 2002; Plowman-Prine et al., 2009). Healthcare professionals consider dysphagia particularly serious in DM1 because of the risk of sudden death from choking (Garrett, DuBose, Jackson, & Norman, 1969). Moreover, in addition to respiratory failure and cardiac problems, aspiration pneumonia is a common cause of death for individuals with DM1 (de Die-Smulders et al., 1998; Mathieu et al., 1999; Turner & Hilton-Jones, 2008).

Diagnosis of dysphagia in DM 1 is complicated because patients rarely complain about, or describe difficulties with, their swallowing. Remarkably, patients with DM1 did not report symptoms of dysphagia despite evidence on videofluroscopy suggesting abnormal swallowing function (Leonard, Kendall, Johnson, & McKenzie, 2001). It is possible that muscle weakness and swallowing dysfunction progress so slowly that individuals develop compensatory strategies that leave them unaware of their dysphagia (Leonard et al., 2001). Moreover, findings from a survey study of 360 frail elderly patients suggest that participants believed that dysphagia was untreatable, and therefore, there was little to gain by mentioning symptoms to healthcare providers (Ekberg et al., 2002). It is unknown if this phenomenon occurs in DM1.

Apathy and cognitive impainnent may further complicate the diagnosis and treatment of DM1-related dysphagia (LaDonna, Koopman, & Venance, 2011). There is a well-described cognitive and personality profile for individuals with DM1 that may be characterized by low IQ, an avoidant personality, lack of motivation, and difficulty with executive function (Delaporte, 1998; Meola et al., 2003; Sistiaga et al., 2010). Clinical experience suggests that some individuals with DM1 manifest apathy and therefore may minimize their symptoms, miss clinic appointments, and not express interest in their health (Meola & Sansone, 2007). Although speculative, it is possible that these characteristics impact an individual's ability and/or willingness to recognize and manage swallowing dysfunction.

Consequently, informal caregivers may play an important role in assessing and managing swallowing dysfunction for their loved ones. To our knowledge, there are relatively few studies researching the experiences of caregivers for adults with muscular dystrophy (Bostrom & Ahlstrom, 2005a, 2005b; Bostrom, Ahlstrom, & Sunvisson, 2006; Boyer, Drame, Morrone, & Novella, 2006; Cup et al., 2011; Timman, Tibben, & Wintzen, 2010). These studies explored a variety of muscular dystrophies using quantitative and/or qualitative methods and did not specifically address the implications of caring for individuals with DM1 and swallowing dysfunction. However, a qualitative study explored nine individuals who cared for loved ones experiencing dysphagia after stroke or other traumatic injuries and found that caregivers prompted their family members about safe eating practices, spent extra time preparing meals, and experienced a sense of fear or worry (Johansson & Johansson, 2009).

Although clinicians provide surveillance for swallowing problems and are concerned about the potential for dysphagia to increase the risk of morbidity or mortality, it is unknown if and how swallowing problems impact the daily lives of patients with DM1 and their identified caregivers. Therefore, it may be important to consider the potential disconnect between healthcare provider's clinical concerns and patients' and family members' perceptions. Much of the DM1 literature considers dysphagia largely from the clinicians' perspectives; therefore, little is known about the experience from the perspectives of the patients and caregivers. The purpose of this research was to explore the experiences of providing care for a person with DM1 and known dysphagia. Findings may influence clinicians' approach to dysphagia diagnosis and treatment; that is, findings may help identify appropriate questions to ask patients and caregivers during clinical encounters and/or to refine management strategies.

Methodology

The methodological framework used to guide this study involved a philosophical inquiry of an interpretive phenomenological nature as articulated by Merleau-Ponty (1962) and van Manen (1990). Using the viewpoints of van Manen and Merleau-Ponty, we explored each participant's experience of embodiment, as being situated and relational or as "being-to-the-world." This refers to the idea that human knowledge is relational, temporal, and present in the world, instead of only being a static object in the world, independent of the researcher. According to van Manen, Merleau-Ponty offers four existentials: "1. Lived body (Corporeality) 2. Lived space (Spatiality), 3. Lived time (Temporality), and 4. Lived human relation (Relationality)" (p. 101). These four existentials were utilized and reflected on throughout the research process to understand the realities of the participants and their lived experiences and to allow the findings to be grounded on the fundamental life world structures, which all human beings experience, although not all in the same ways. The four existentials of lived body, lived space, lived time, and lived human relation are categories for phenomenological questioning, reflecting, and writing (van Manen, 1990).

Methods

For the purposes of this study, a caregiver was defined as a spouse, family member, or friend who provided some degree of supportive care to a person living with DM1. We designed the study and collected and analyzed the data using an interpretive phenomenological approach (Dowling, 2007; van Manen, 1990). Interpretive phenomenology seeks to explore and understand the nuances of lived experience by uncovering taken-for-granted assumptions (van Manen, 1990). Individuals with mild-to-moderate DM1 and swallowing problems (by physician report) who attended an outpatient neuromuscular clinic were invited to identify and approach a caregiver about the study; six caregivers (two women, four men) agreed to participate. Data were collected during semistructured interviews lasting up to an hour; the interviews began by asking participants to reflect on the meaning of the term "caregiver." Next, the interviewer (K. A. L.) used a series of prompts to probe about awareness and understanding of swallowing dysfunction and other DM1 related symptoms, strategies for management, and symptom impact on daily life. All interviews were recorded and transcribed verbatim.

Data were analyzed using van Manen's (1990) suggested steps for phenomenological analysis. Two researchers (K. A. L. and W. J. K.) began by reading each transcript twice to gain a holistic sense of the data and then independently chose a sentence or phrase that captured the essence of each transcript. "Caregiving is a full-time job" and "caregiving is a partnership" are examples of sentences that described the essence of two transcripts. Next, a selective reading approach was used to code the data and extract statements that described the caregiving experience. K. A. L. and W. J. K. independently underlined key passages from the text related to dysphagia or caregiving and developed a list of codes using words or phrases that reflected the participants' experiences. K. A. L. and W. J. K. then met to discuss their codes and combined them into overarching preliminary themes. Key quotes supporting the themes were extracted from the data. The themes and quotations were then discussed with all authors, and a final list of findings was developed by consensus. Recruitment ceased when no further themes emerged. This does not mean, however, that new information would not have been gained by further data collection; rather, the data collected were sufficient to allow a rich understanding of the phenomenon under study. As suggested by van Manen, the use of temporal, spatial, relational, and bodily existentials to guide the analysis yields a richly textured understanding of the embodied nature of caregiving for those with DM1 and dysphagia.

To further ensure the rigor of our study (Morse, Barrett, Mayan, Olson, & Spiers, 2002), we sent a letter to participants that described the preliminary findings and invited each caregiver to participate in a second interview to comment on the emerging themes. Two participants agreed to participate in follow-up interviews.

The Western University Research Ethics Board approved this study. To protect the confidentiality of our research participants, we have assigned each person a pseudonym. At the time of his follow-up interview, Sam was a 79-year-old man living with his moderately affected wife. Sam has three children with DM1 and a grandchild with congenital DM1; Sam also provides some degree of care for his moderately affected son. Laura is in her 60s and lives with her mildly affected husband. Laura also has a son with moderate DM1 for whom she does not consider herself a caregiver, although she often run errands for him. Daisy is a caregiver for her moderately affected husband, and she also works outside the home. Michael is a retired gentleman in his 60s who lives with his mildly affected wife, and John is in his 50s and cares for his mildly affected wife. Finally, Greg is in his late 40s and provides care for his mildly affected partner and for his son with congenital DM1. Greg's affected father-in-law died unexpectedly after a choking episode.

Results

Although dysphagia is one of the most life-threatening symptoms of DM1 (Harper, 2001; de Die-Smulders, 1998), five of the caregivers in this study did not consider it to be their family members' most problematic symptom. Instead, most identified fatigue and weakness as the symptoms that impacted their affected family members' function and quality of life. Despite the interviewer's attempts to circle the conversation back to the experience of caring for someone with dysphagia, participants chose to focus on their role as caregivers rather than on their loved ones' symptoms. Although caregivers offered insight into their beliefs about DM1 and swallowing dysfunction and were able to describe strategies for managing symptoms, the participants continually returned to discuss their caregiving roles and responsibilities. This divergence from the original research question is not wholly unexpected given that the purpose of interpretive phenomenology is to have participants reflect on their lived experience and to uncover taken-for-granted assumptions (van Manen, 1990). We identified five themes that reflected the bodily, spatial, temporal, and relational existentials described by van Manen (1990): "relationships drive perceptions," "the meaning of caregiving," "the physical experience of caregiving," "carving out a space for respite," and "looking into a crystal ball."

Relationality: Relationships Drive Perceptions

Relationality refers to the lived relations and spaces that are shared with others (van Manen, 1990). In particular, sharing human relationships with others develops impressions of others that are confirmed-or not--through interactions. For instance, participants' relationships with their affected family members influenced their beliefs about the meaning of caregiving and the impact of DM1 symptoms on their lives. It was evident that healthcare providers were concerned about the swallowing function of several patients with DM1 because Sam, Daisy, and Michael all stated that their family member had been referred for a videofluroscopic swallowing assessment, yet participants were largely unconcerned about their swallowing dysfunction. This was despite being shown video evidence of their loved ones' swallowing abnormalities. Sam described that it was "the most fascinating thing to see this food go back, stop, and then go down," yet he did not consider swallowing to be a particularly troubling symptom because his day-to-day experience of living with his wife reinforced his belief that she had developed appropriate compensatory strategies. However, a healthcare provider would likely be concerned about the potentially serious strategies he described:
   So, as far as that's concerned, she has a way ... we
   have morning vitamin pills. There was about 6 or
   7 when washed down and they go down then
   the Omega 3, 6, 9, they're a lot bigger so but she
   has an excellent way of popping them back up.
   So in that sense she doesn't have a problem
   swallowing. But like yesterday, we had stew so she
   just put it through the blender. Because I think
   more than just swallowing problem it is her
   muscles are weak--too weak--and so she says
   that's enough ... and that's obviously related to the
   myotonic. But, so in a way, swallowing she
   handles quite well.


Moreover, Greg's father-in-law died after a choking episode, an occurrence that made his partner more vigilant about her swallowing function. However, Greg was less concerned about swallowing abnormalities; instead, he focused on his partner's difficulty breathing.
   My concern is the breathing more so than her
   choking. Everybody can get out of a choking
   situation, but the breathing problem is the big
   issue. She keeps losing percentages of her lungs'
   capacity because of her diaphragm. That's my
   big concern.


This was surprising given his father-in-law's unexpected and sudden death from choking and his partner's concern about her swallowing dysfunction. Respiratory failure is a slowly progressive symptom as opposed to choking, which can cause an acute complication like aspiration pneumonia or sudden death. However, Greg seemed confident that he could help his partner should she choke, yet seemed to feel helpless about the progressive decline of her lung capacity. The fear of losing his partner to a symptom or event that he could not control may have influenced how he evaluated the impact of his partner's symptoms and, in turn, where he chose to focus his energy.

In general, dysphagia appeared to be a symptom that participants felt the individual with myotonic dystrophy had compensated for and managed; therefore, swallowing dysfunction did not seem to cause great concern or to affect shared experiences like mealtimes or social activities. Caregivers described that their loved ones were aware of their limitations and had identified strategies like avoiding certain foods, cutting things into smaller pieces, or having liquids with meals to facilitate safer swallowing.

As she told the fellow we were talking to, she's very careful how she eats. And by careful, I mean she cuts small pieces. She eats very slow. I could probably eat two meals to her one. (Michael)

Instead, caregivers considered weakness and fatigue far more troubling symptoms. Fatigue impacted the social participation of caregivers and their affected family members and seemed to negatively affect the marital relationship. Daisy described that it was not uncommon for her husband to fall asleep at inappropriate times, and Greg's partner stated that Greg must feel like he "lives alone" because she falls asleep so frequently. Sam noted that his wife's fatigue, coupled with her multitude of complications, made it difficult for her to be motivated to be active or social. He regularly had to prompt her to leave the house to "get some fresh air." Laura and Michael described changing their expectations about, and opportunities for, social outlets. Michael described staying home to play cards instead of engaging in activities outside the home, and Laura prepared special meals to ensure that her husband could participate in meals with friends:
   We know they (husband and son) have muscular
   dystrophy and we accommodate time or meals
   so that ... basically, if we have company and I'll
   make sure it it's something that maybe not
   100% of the meal (husband) can eat but a good
   portion--75% maybe even 80%--and he wants
   that too because then it's--he's not depriving me
   of doing things.


Relationality: The Meaning of Caregiving

Although participants did not speak at length about assisting their affected family member with managing swallowing dysfunction, they did speak indepth about the overall caregiving experience. The data suggested that there were three main reasons for being a caregiver: (a) It is the nature or personality of the individual to be a carer, (b) there is no one else to do the caregiving, and (c) caregiving is done out of love. Five of six participants endorsed that "caregiver" was a term that they would use to describe themselves. Sam put caregiving for his wife in context with his volunteer work as a palliative caregiver in a hospice:
   Well, it's interesting. Because in a way, it's
   different than with the palliative care. I always
   felt that we gained an awful lot by giving time to
   chat and so on. It was very enriching in many
   cases. With my own wife, it's not so much
   enriching as to feel that I have made it a little
   more comfortable and 1 have been able to assist
   her in something that she found difficult or because
   we've been married for 48 years ... so we've
   exchanged a lot.... And, it isn't quite the same.


On the other hand, Laura felt that she was not a caregiver because her spouse and child "manage on their own." For Laura, a "caregiver" is a defined role and title that must be earned by performing specific tasks. She viewed her work checking on her son and preparing meals for her husband not as caregiving duties but as intrinsic parts of her lived relations as a wife and mother.
   Um ... well, the word caregiver is used and it's
   used for people who actually need that kind
   of care and I think--I guess mostly personal
   care, feeding and all of that. So, I'm not a
   caregiver for either one of them. But I help
   when I can.


Similarly, for Sam and Michael, caregiving was seen as part of the marital relationship that would be present even without factoring in a chronic disease.

Well, I guess I could look at it that way too.... definitely [she] does what she's capable of doing. And I guess I do that for her too. In my opinion, she would be a caregiver of me too. To me it's a 50/50 deal. You don't know what life's going to deal you. You've got to make the best of what you have and go from there. (Michael)

In addition, some participants seemed to have a nurturing nature in all facets of life that influenced their experience of caregiving. For Sam, his experience of caregiving transcended caring for his family members with a chronic, progressive disease. Sam's caregiving took many forms throughout his life, both as an educator and as a volunteer in a hospice. He perhaps chose these vocations because of an inherent need to serve and support; in turn, he used these experiences to shape how he cared for his wife.
   I was a teacher for 31 years and there definitely
   is caring for the kids that they succeed. Now with
   [spouse] it definitely is love, I mean she is my
   wife. And it has become, or it has become a little
   bit more. Just last night she said, how did she put
   it, you are doing things that I used to do....


In contrast, John's experience of caregiving was one of burden and burnout. John described being responsible for all of the household chores in addition to working full time, and he resented what he perceived as his wife's learned helplessness: "She needs to get back on her CPAP and it's just finding the phone number for her. And she expects me to do that but that's not my job. 'I'm not doing that for you dear. That's drawing the line there, you can call.'" When asked if there were any rewards of caregiving, he noted that the one positive aspect of caregiving was the knowledge that his wife was receiving appropriate care:

No. Other than I know she's getting good care. So, you know, that's the biggest thing. She's being taken care of. 'Cause I know what else is out there.... But, no, other than I know she's getting good care and being taken care of. Not being abused by anybody or nothing, so ... Yeah, that's about it. (John)

Corporeality: The Physical Experience of Caregiving

"Lived body (corporeality) refers to the phenomenological fact that we are always bodily in the world" (van Manen, 1990). For most participants, their physical body affected either how they interpreted their loved ones' symptoms or how they were able to provide care. Despite not being cognizant of the extent of their loved ones' swallowing dysfunction, some caregivers had good awareness of their loved ones' bodies. Caregivers knowledgably discussed the main DM1 symptoms and the variability of disease progression. Caregivers were readily able to describe the "lived body" of fatigue, cardiac and respiratory problems, dysphagia, and weakness as primary symptoms affecting their family members. For example, Michael was mindful about taking objects from his wife because her myotonia made it difficult for her to release her grip:
   Well, probably lots of times I help her get
   dressed, maybe do up her shoes for her. The
   big thing is getting stuff out of the cupboards
   because of with her grip. I'm going to have to do
   that type of thing. Or you've got to be careful
   when ... if she hands me something I've got to
   realize that it takes her a while to let go of it, so
   if I don't, like sometimes pull her over or pull
   her arm type of thing.


Participants described that the experience of caregiving involved both physical and emotional work. Most caregivers cared not only for their family member with DM1 but also for other affected family members and/or aging parents. Participants described that the work of caregiving involved the bodily work of cooking, cleaning, heavy lifting, running errands, and assisting with bathing and dressing. Participants also discussed that attending and asking questions at medical appointments were important aspects of their roles that facilitated the work of caregiving. The task of "prompting," whether to take medication or chew slowly or for their family member to be social, was described. Moreover, Sam used his body to provide comfort and healing for his wife: "And I do Reiki, 1 give her Reiki treatment. We do massage, light massage with maybe a bit of aromatic oil." However, caregivers' physical limitations sometimes impacted their ability to perform the physical tasks of caregiving. John described having a pinched nerve, and Sam stated that a shoulder injury affected his ability to assist his wife in lifting or carrying heavy objects.

Caregiving also enacted an emotional toll on Greg and John who described that the constant duties of caregiving were "annoying" and frustrating. In particular, John stated that he felt "down," and his emotions affected him both physically and emotionally to the point that he was on a number of prescription medications to treat his anxiety and depression. Overall, caregivers described that much of the perceived effort of caregiving was found to be emotional, with "worry" as a prominent theme. Participants disclosed a variety of concerns, including whether their family member was eating enough, the impact of DM1 on their own physical and emotional health, increased caregiving tasks as DM1 progresses, and the constant fear of the likelihood of an early or unexpected death.

You know, that's the most important right there cause you never know one day he could be lying dead somewhere and I'd never know it.... Like I say, you never know if he could end up one day in bed dead, you know, and I wouldn't know it.... That's something I don't want to think about. You know, nobody wants to think about that but if it happens it happens. (Daisy)

Spatiality: Carving Out a Space for Respite

Spatiality, or lived space, is "felt space" that describes how individuals experience spatial dimensions in their everyday lives (van Manen, 1990). In general, participants described that it was difficult to find physical spaces that accommodated individuals with a disability, and navigating in public spaces was impacted by their loved ones' bulky mobility aids like wheelchairs or oxygen tanks. As a result, social circles and outlets diminished.

Other than going places--that's the biggest drawback, going and figuring out whether we need to take her machine and all the stuff, and when it's really hot out she doesn't bother going outside, it's so humid. (John)

Moreover, the emotional toll that Greg and John described also affected their ability to feel comfortable and relaxed in their homes because they always felt that they should be performing tasks or supporting their partners. Therefore, for John and Greg, caregiving constrained their ability to achieve a physical distance from their duties and responsibilities.

They're (caregiving duties) intense right now as far as I'm concerned because I can't go out on my own without worrying about her. As I said, we travel with cell phones, so if I'm out and about, I'm always close. I can't go anywhere without always worrying about her. So I don't go too far away. I stay close to the house. So it's really restricted my life in that respect. I don't want to go out and get a part-time job, because if I do that, I'm on the job, she phones you up and she has trouble, what am I going to do? (Greg)

Therefore, there was a sense that burden was part of the experience of caregiving for John and Greg. To cope with the stress of caregiving, they attempted to carve out both an emotional and a physical lived space for respite.

She just takes it (caregiving) for granted. Which can be real annoying. I try to sit down and relax and she's going jibber, jibber at me. Shhh, I'm trying to relax. So I just end up going sitting on the porch or going out in the back yard with the dogs. Here we go, here's some relaxing. (John)

Temporality: Looking Into a Crystal Ball

Temporality refers to subjective time rather than "clock time," and an individual's "temporal landscape" is colored by the past, present, and future (van Manen, 1990). Before their loved ones' diagnosis, few participants had knowledge about DM: "Like I've watched the Jerry Lewis Telethon and there's a bunch of families there with different muscular dystrophy diseases but coming up with myotonic dystrophy--no. No, I [had] never heard of the word before (Daisy)." Daisy also reflected on the changes in their lives since her husband's diagnosis; they had enjoyed parties and being social, but now, they spent more time at home alone. Furthermore, Sam discussed the past with a sense of regret and wondered whether he and his wife would have had children had they known about the genetic nature of DM1: "We haven't dwelt on that. Once in a while it came up and you know what 'That's i t... that's the way it is.'"

However, most participants focused on the present and living in the moment with their loved ones and described living with their loved ones' symptoms and adjusting to them as they progressed. Therefore, swallowing was not considered problematic because the affected individual was coping and managing in the here and now. Overall, there was a sense that, although the complications of DM1 (i.e., fatigue and weakness) affected daily life, DM 1 was manageable. Therefore, while some caregivers described a sense of regret or fear of the unknown about DM1, others like Laura described DM1 as "no big deal."

We work with him rather than it dictates what we do. It's just--you just do it.... there's no hardship at all. (Laura)

There was, however, greater concern for other symptoms including breathing difficulties and progressive weakness. Many participants had a "crystal ball" mentality in which they envisioned that their partners' decline in time was inevitable and that they would have to take on an increased caregiving role. Caregiving was already viewed as time lived as a consuming "24/7 job" (Greg) that was done in addition to full-time employment:

Like it's hard for me because I work and coming home, you know making meals and stuff, keeping our place clean and whatever, you know it's really hard, really really hard. (Daisy)

However, Michael, Greg, and John discussed that their retirement afforded them more time as lived to devote to caregiving; this was positive for Michael and Greg but not for John who was overwhelmed at the thought of being a full-time caregiver.

The experience of caregiving also involved future temporality as making plans and being prepared for eventual complications, and participants discussed their varying degrees of preparedness for the future. Only two participants (Sam and Greg) had previous experience with CPR or the Heimlich maneuver; however, they took first aid courses for employment or other purposes, not in preparedness for their loved ones' impaired swallowing function. When prompted, however, all caregivers expressed an interest in learning more about what might be done in an emergency or unforeseen situation, but no one had plans to be formally trained. Despite this, most caregivers believed that they would be able to appropriately respond should a choking episode occur in future time.
   Well if she were to choke, I could do the Heimlich
   on her. I plan on getting my CPR certificate. I'm
   working on that in (location). I'm waiting for a
   clinic to open up in case I ever need it. Then I'm
   certified. I used to be a trainer for a boy's hockey
   team. I had to get certified for that. So general first
   aid and stuff like that, I'm familiar with. I'm not
   totally lost when it comes to something like that.
   But CPR is what I've got to follow through on.


Although there was a sense that caregivers understood that their loved ones' condition would decline, the participants continually circled back to the present time. Overall, the experience of caregiving seemed to be one of love and duty, tempered by the expectation that caregiving responsibilities would intensify in future lived time. The experience of caregiving for an individual with DM1, therefore, was influenced by their corporeality or lived body in lived time, lived space, and lived relations when being with their affected family members.

Discussion

Given the attention, concern, and surveillance that healthcare providers impart on DM1-affected individuals with dysphagia, we set out to explore caregivers' perceptions about, and their experiences with, their family members' swallowing dysfunction. However, the goal of interpretive phenomenology is to uncover taken-for-granted assumptions (van Manen, 1990), and study findings suggest that the overall caregiving experience, coupled with troubling symptoms like weakness and fatigue, have a far greater impact on the participants' lived experiences than dysphagia. Therefore, the study evolved into an in-depth examination of participants' knowledge and attitudes about DM1 and the meaning of their caregiving experiences. This speaks to the uncertain, fluid, and emergent nature of qualitative inquiry (Lincoln, 1995; Whittemore, Chase, & Mandle, 2001).

The participants in our study described their beliefs about DM1 and dysphagia--the impact of DM1 symptoms on their lived body, lived time, lived space, and lived relations; in turn, the four existentials described by van Manen (1990) also influenced caregivers' descriptions of the meaning and work of caregiving. The relative lack of concern about dysphagia echoed other studies suggesting that individuals with DM1 are often not aware or concerned about swallowing or other consequences of their condition (Bostrom & Ahlstrom, 2005a; Meola & Sansone, 2007) despite significant concern from healthcare providers. This disconnect has been explored in populations with other neurological disease, but to our knowledge, it has not been studied in DM1. For example, a study exploring multiple sclerosis care found that physicians focus on mobility issues, while patients are more concerned with cognitive decline (Heesen, Kopke, Richter, & Kasper, 2007). In Parkinson disease, although clinicians may attend preferentially to the motor features of the disease, it is often the non-motor features including mood problems, nocturia, and drooling that most impact patients' health-related quality of life (Martinez-Martin, Rodriguez-Blazquez, Kurtis, Chaudhuri, & NMSS Validation Group, 2011). Similarly, there is evidence that the cognitive and psychiatric features of Huntington disease are far more disabling than chorea, yet research is often geared toward the motor symptoms (Bonelli & Hofmann, 2004). Finally, clinicians and patients may differ in their perception of the severity of Huntington disease symptoms, with clinicians objectively assessing symptom impact on disability and patients subjectively assessing symptom impact on daily life. This suggests that quality-of-life measures may be more indicative of symptom impact than disability scores (Banazkiewicz et al., 2012).

Arguably, patients' description of symptoms may impact their caregivers' beliefs about how they are living with and managing their condition. In turn, clinical management may be influenced by patients' and caregivers' subjective reports of symptom impact on functional ability (Shulman et al., 2006). However, caregivers were aware of healthcare professionals' concerns that dysphagia increases individual risk of morbidity and mortality related to choking, aspiration, and sudden death. Others have hypothesized that this dichotomy may occur because there may be a distinction between knowing the symptoms of DM1 and fully understanding their implications (Bostrom & Ahlstrom, 2005a). We speculate that caregivers' lack of concern mirrors that of patients because the swallowing dysfunction evolves gradually over time, allowing for strategies to compensate. A survey of 286 older adults with osteoporosis or osteoarthritis found that participants used a variety of strategies to compensate for their declining function including asking for help, giving up certain activities, performing behaviors that optimize their current function, and using assistive devices (Gignac, Cott, & Badley, 2000). Although this study did not speak to dysphagia, it does illustrate how individuals with chronic disease compensate for progressive functional loss. Our findings suggest that individuals with DM1 compensate for their abnormal swallowing by avoiding certain foods, having liquids with meals, or regurgitating objects that were not safely swallowed. For caregivers, therefore, there is no additional perception that swallowing is difficult to manage or is a hardship because their lived relations with their partners reinforce the belief that their loved ones have compensated appropriately.

However, similar to the experiences of 30 caregivers for individuals with muscular dystrophy (10 individuals with DM1) described in a questionnaire study by Boyer et al. (2006), the participants in our study expressed a sense of emotional and physical burden, primarily related to the anxiety of disease progression and sudden death. Further in line with our findings regarding the "work" of caregiving, five partners of individuals with DM1 described that they increasingly became responsible for prompting their family members, for doing household chores, and for planning social activities (Cup et al., 2011).

Many of our findings resonate with the experiences of 36 caregivers for individuals with muscular dystrophy (19 DM1 caregivers were included) ascertained through inductive content analysis (Bostrom et al., 2006). In particular, the sense that caregiving is shared with the individual living with muscular dystrophy and is done out of love or obligation were similar to our findings. In addition, worry about disease progression and an increased care load, and the idea that individuals and families living with other conditions or life situations are worse off, resonated with our participants' experiences. Using a hermeneutic qualitative approach, Cup et al. (2011) found that the marital relationship can be affected by DM1 but that the five couples studied found ways to cope including giving each other space, respecting each other's abilities, and allowing each partner to pursue his or her own interests. Moreover, we found that individuals with DM1 and their caregivers who see caregiving as part of the marital relationship and/or those who provide care out of love tend to view caregiving more positively. This is similar to studies that suggest that being in a healthy relationship was associated with the psychological well-being and better quality of life for individuals with muscular dystrophy and their partners (Bostrom & Ahlstrom, 2005b; Timman et al., 2010).

This is a small study reporting the experiences of six caregivers of individuals with DM1 and dysphagia and provides preliminary data that may inform future research questions. A longitudinal study with a larger sample that explores the experiences and processes of caregiving for individuals with DM1 and dysphagia may be warranted. Moreover, an exploration of patients' experience of dysphagia or an observational study exploring the daily activities, food choices, and eating patterns of individuals with DM1 may be helpful to guide the development of educational initiatives or interventions. In particular, future research might consider appropriate questions to ask during clinical encounters or an examination of patients' condition-specific knowledge and their uptake of clinical information. Furthermore, areas of education for healthcare providers might attempt to bridge the chasm between healthcare providers' areas of concern with the complications that are most impactful to patients and their family members. Finally, a condition-specific assessment tool measuring swallowing function for individuals with DM1 may be useful (LaDonna et al., 2011) to quickly assess for dysphagia during follow-up visits. Although often outside a routine clinic visit, an assessment of caregiver burden for those caring for family members with DM1 may be necessary (Boyer et al., 2006). Healthcare providers may need to pay particular attention to the effect of DM1 on daily life for individuals and their families (Cup et al., 2011; Edwards & Ruettiger, 2002). Although our results may not be immediately generalizable to the broader population with DM1, the findings may resonate with caregivers and other friends and family of those living with DM 1.

Conclusion

Although we set out to explore the experiences of caregivers for individuals with DM 1 and dysphagia, we found that caregivers did not consider dysphagia overly concerning. Instead, they focused on other troubling symptoms like weakness or fatigue and the impact of caregiving on their lived body in lived time, space, and relations. This raises questions about the dichotomy between the concerns of healthcare providers and patients and their families and how clinicians can assess the needs important to families while addressing clinical concerns (Bogardus et al., 2001). We suggest that clinicians emphasize the importance of monitoring and managing potentially life-limiting symptoms like dysphagia while also assessing how DM1 symptoms impact the quality of life for patients and their caregivers.

Acknowledgments

We thank the participants for sharing their experiences with us. We would also like to thank Dr. Christopher J. Watling for his thoughtful review of this manuscript. This research was generously supported by the Port Elgin Rotarians.

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Kori A. LaDonna, PhD, is a Postdoctoral Fellow, Centre for Education Research & Innovation, Schulich School of Medicine & Dentistry, Western University, London, Ontario, Canada.

Wilma J. Koopman, RN(EC) MScN NP, is Nurse Practitioner, University Hospital, London Health Sciences Centre, London, Ontario, Canada.

Susan L. Ray, RN PhD CNS APN, is Associate Professor, Arthur Labatt School of Nursing, University of Western Ontario, London, Ontario, Canada.

Questions or comments about this article may be directed to Shannon L. Wnance, MDPhDFRCPCatShannon.Wnance@lhsc.on.ca. She is an Associate Professor, Clinical Neurological Sciences, Schulich School of Medicine and Dentistry, and University Hospital, London Health Sciences Centre, London, Ontario, Canada.

The authors declare no conflicts of interest.

DOI: 10.1097/JNN.0000000000000178
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Author:LaDonna, Kori A.; Koopman, Wilma J.; Ray, Susan L.; Venance, Shannon L.
Publication:Journal of Neuroscience Nursing
Article Type:Report
Date:Feb 1, 2016
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