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Nursing and Health Sciences (2014), 16, 454–460
Research Article
Impact of living with pulmonary hypertension: A qualitative exploration Janelle Yorke, PhD, MRes, PGCE, RN,1 Iain Armstrong, M Med Sci, RN2,3 and Sarah Bundock, BSc4 1 University of Manchester, Manchester, 2Pulmonary Hypertension Unit, Royal Hallamshire Hospital, 3Pulmonary Hypertension Association UK, Sheffield and 4HealthEd, Cheshire, UK
Abstract
Little is known about the impact of living with pulmonary hypertension. This paper reports data exploring the experience of living with pulmonary hypertension. Qualitative, semistructured, one-to-one interviews were conducted in participants’ homes to understand their experiences of living with pulmonary hypertension. Thematic analysis was used to identify codes and generate themes from the interview data. The identification of initial codes was conducted independently by the first author, and checked by the second. Thirty patients recruited through the pulmonary hypertension descriptions of living with pulmonary hypertension are presented under five themes that center on the invisibility of pulmonary hypertension, and its complex treatment are presented: (i) living with a hidden illness; (ii) being on a symptomology rollercoaster; (iii) expectations from treatments; (iv) treatment burden; and (v) awareness of financial burden of treatments. Key findings included daily challenges of living with a rare condition that is largely “hidden” and its related complex treatment regimes. People with pulmonary hypertension would benefit if more healthcare professionals, as well as family and friends, would validate their condition and provide them with appropriate support.
Key words
interview, perception, pulmonary hypertension, qualitative.
INTRODUCTION Pulmonary hypertension (PH) is a rare condition that has many causes and is life limiting. Patients with PH suffer from breathlessness, fatigue, decreased exercise capacity, light headedness, and syncope (Cenedese et al., 2006; McDonough et al., 2011). Since the widespread use of the various new treatment options, the long-term prognosis of PH has significantly improved (Kiely et al., 2013). However, none of these therapies are curative, and varying degrees of impairment and symptom experience remain throughout the PH trajectory (Taichman & Mandel, 2007). The focus of research in PH has been clinical trials of therapies, and to a lesser extent, measuring health-related quality of life and symptom severity using a variety of quantifiable questionnaires. There has been a recent increase in the desire to assess health-related quality of life in patients with PH, which is evidenced by the development of a new health-related quality of life tool (emPHAsis-10) (Yorke et al., 2013). While that work has provided valuable quantitative information to measure the impact of PH, there is a Correspondence address: Janelle Yorke, School of Nursing, Midwifery and Social Work, University of Manchester, Room 5.320 Jean McFarlane Building, Oxford Road, Manchester M139PL, UK. Email: [email protected] Present address: Sarah Bundock, Trust Life, Cheshire, UK. Received 11 October 2013; revision received 26 November 2013; accepted 30 January 2014
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need for research that explores patients’ experiences of living with PH in order to gain a greater understanding of patients’ physical and psychosocial needs. Qualitative work exploring the experience of living with PH is currently limited to three studies (Peloquin et al., 1998; Flattery et al., 2005; McDonough et al., 2011), and one study focusing on the pre-diagnostic phase (Armstrong et al., 2012). Peloquin et al. (1998) interviewed three females in the initial stages of prostacyclin therapy and found that the therapy regimen was burdensome and impacted negatively on the patients’ perceived quality of life (Peloquin et al., 1998). Flattery et al. (2005) explored the experiences of 11 patients with PH and found that coping with uncertainties and taxing treatment regimens were both challenging and distressing for the patient (Flattery et al., 2005). Breathlessness has been identified as the most burdensome symptom of PH, and presents patients with activity limitation and a need to redefine their lives (McDonough et al., 2011). Taken together, this small body of work begins to shed light on the challenges of living with PH. Further exploration, however, could help practitioners and researchers develop a greater understanding of the complex issues faced by this patient group and aid the development of specific interventions and support mechanisms for people with PH.This paper reports the data obtained from a program of research exploring the day-to-day impact of PH. doi: 10.1111/nhs.12138
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METHODS In-depth, semistructured interviews were used to capitalize on the intricacies of the experiences of the participants. A semistructured interview style provided the flexibility to ensure that key topics were covered, while enabling the interviewee to disclose additional information that might diverge from the interview guide. The aim of the interviews was to explore participants’ experiences of day-to-day living with PH.
Participants Participants were recruited via the Pulmonary Hypertension Association (PHA) UK membership list. The PHA UK is the only charity in the UK for people with PH, with approximately 3000 members. Potential participants were informed about the study at a PHA UK patient conference in 2009, and contact details were collected from those who expressed an interest in participating. Responders were then purposively sampled from this list to ensure maximal variation, including both men and women of different ages, a range of etiologies and time since diagnosis, and from different geographic areas and specialist PH centers in England. PHA UK staff assisted the researchers to identify participants who met the inclusion criteria and range of demographic details, as described above. The inclusion criteria were patients: (i) diagnosed with PH (mean pulmonary artery pressure of 25 mm Hg or more) of any etiology; (ii) aged 18 years or older; (iii) able to speak and understand English; and (iv) able to provide informed consent. The exclusion criteria were: (i) chronic thrombotic embolic disease suitable for surgery; (ii) patients in the terminal stages of disease; and (iii) known severe and enduring mental illness. The study interviews were conducted in 2010 and took place in the participants’ homes. Interviews were conducted by an experienced health services researcher (SB) not involved in the provision of care for people with PH. Thirty one-to-one, in-depth interviews were conducted with patients. The recruitment of new participants discontinued when no new key themes emerged (Bowen, 2008). Verbal information about the study was provided at the PHA UK 2009 conference and over the telephone when potential participants were contacted to ascertain if they were still interested in participating. Written, informed consent was obtained prior to the interview commencing and was reviewed verbally at the beginning and end of the interview. Ethical approval was obtained from the Pulmonary Hypertension Association Medical Committee (2011–01) and the University of Salford Research Ethics Committee (NURA018).
Data collection and analysis An in-depth interview guide was developed to cover the patients’ experience of living with PH, which included the following questions: 1. When you were first diagnosed with PH what were your thoughts and feelings? How do you feel about having PH now?
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2. What impact, if any at all, would you say your PH has had on your life? 3. What impact, if any at all, would you say your PH has had on your family/friends? 4. What difference, if any at all, would you say your treatment for PH has had on your overall quality of life? 5. What do you think your quality of life would be like if you were not having any treatments for your PH? 6. What, if any impact has the diagnosis of PH had on your work life and financial circumstances? 7. What is your opinion about the standard of care you receive to manage your PH? The interview guide was developed during discussions with PH nurse specialists and patient representatives affiliated with the PHA UK. Interviews lasted between 40 and 100 min and were audio-recorded and transcribed verbatim. The methods used to code and categorize data were approaches to thematic analysis described by Braun & Clarke (2006). The identification of initial codes was conducted independently by the first author and checked by the second. Five themes were developed through further data scrutiny and discussion. The transcripts were analyzed line by line and paragraph by paragraph, looking for significant statements and codes according to the topics addressed. The researcher compared the various codes based on differences and similarities, and sorted them into categories. Finally, the categories were formulated into five themes.
RESULTS The characteristics of the 30 patients who participated in the study are detailed in Table 1. The coding used to represent participants was as follows: F, female; M, male; and individual study code and participant’s age (e.g. F2:56 is a female patient, study code 2, and 56 years of age). Participants’ descriptions of living with PH are presented under five themes that center on the invisibility of PH and its complex treatment.
Living with a hidden illness Many participants described living with a “hidden” illness, as “you can’t see anything.” The lack of visible signs of having a chronic condition, that might otherwise alert people to the fact that they were ill, presented participants with a unique challenge. There was a sense that other people did not appreciate how ill participants were, and often felt that they were required to justify why they were tired or short of breath: I think what I don’t like about having pulmonary hypertension is that no-one can see it . . . I got wheelchair assistance at the airport, and they (friends) went, “Well, what’s wrong with you?,” and I was like, “Well, I don’t really need to tell you, but . . .” “Well, you look fine.” (F26:29) © 2014 Wiley Publishing Asia Pty Ltd.
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Table 1. Characteristics of the 30 patients with pulmonary hypertension interviewed for the study Characteristics Sex (female : male) Age (mean years; SD; range) Working status Work full time Work part time Retired Long-term sick Unemployed Education PH etiology Idiopathic Connective tissue disease Congenital heart disease Chronic thromboembolic Heritable Portal hypertension Lung disease Drugs/toxins Time since diagnosis < 1 year 1–< 3 years 3–< 5 years 5–< 10 years > 10 years WHO PH functional class I II III IV
N = 30 18:12 56.3 (38; 26–80) 2 4 11 9 2 2 12 7 5 2 1 1 1 1 2 2 7 12 7 0 9 19 2
PH, pulmonary hypertension; SD, standard deviation; WHO, World Health Organization.
They see me and they think, well you’re just normal, you’re fine, you haven’t got any wheelchairs or contraptions and stuff . . . they don’t understand because I look normal. (F35:14) Cos you can’t see anything, and when people see you they’ll say, “Well, you’re looking really well, how are you doing?,” and even if I’m doing something in the house and I become breathless, somebody might say something like, “Oh, have you been running?” (F49:1) Even when oxygen therapy was used, some participants experienced questioning from other people: “Why are you using oxygen?” Even fellow patients: “What (are) you using oxygen for, you don’t look like you need oxygen?” (F42:15)
We took a life insurance policy, and it said “terminal illness benefit” on it . . . because they don’t know about PH and because they don’t have it on their list and stuff like that . . . they just fob you off with all kinds of stuff . . . they just want the doctor to say that I’m going to die in 12 months, but they’ve tried to tell them that with this condition you could die anytime. (M47:3) Stories about appealing against unsuccessful insurance and disability benefit claims applications were common: I had to appeal. . . . There was a doctor there, and he just said, “Well, yeah.” He said: “Basically you’ve got heart failure.” Degrading, I suppose . . . because you can’t see anything. (F49:1) Benefits, they didn’t know what pulmonary hypertension was. I look obviously extremely well on the outside with no sort of “disabilities.” They found it extremely hard to accept that I couldn’t work. I went through an appeal process. (F35:7) Younger participants in particular found it difficult to access help: At 25 . . . trying to actually get any kind of . . . financial help from the government, any sort of benefit, was extremely difficult. (F35:7) For others, it was not PH itself, but the side-effects of invasive treatments to that expedited benefit claims. In particular, patients receiving subcutaneous infusions and suffering from infusion site pain and related problems could access benefits more easily: I was sent to a government doctor, and she said: “What’s the problem and what are you doing about it?,” and I said: “Well, I’ve got this drug,” and on that day I had it in my thigh, and I said, “But it is so sore,” and she said: “Well, can I see it?” I said: “You can see it, but please do not touch it,” because . . . from my knee joint to my hip joint, it was swollen . . . and bright red, and she said: “Oh my God, right okay, that’s all,” and I then was awarded whatever the benefit was. (M61:10)
Being on a PH symptomatology rollercoaster Participants reported that their PH symptoms, particularly breathlessness, were unpredictable and made planning for the immediate and long-term future difficult:
It’s sort of the assumption that you’re young and you look fit, therefore you should be; it must be something you’ve done yourself, you know, like smoking. (F42:15)
Well, you just thought every single day . . . when you have an episode like that (deterioration), then you just get better and you’ve been better, say, for three weeks, and you feel great again, and then whack, you’re back to square one. (M53:18)
Having a “hidden” and unknown illness made interactions with insurance and government organizations for sickness and disability benefit claims difficult, and at times, degrading for the person with PH:
Because I can be fine for a month, as fine as I can be with this, and then I can have a down period . . . I can be really more breathless than usual. You just have to stay in and deal with that. (F72:23)
This often resulted in a sense of social stigma:
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Living with a fluctuating illness made applying for sickness and disability benefits difficult, and a source of anxiety and frustration for many participants: I did try and put a claim for disability allowance, but they didn’t class me as needing disability . . . if it’s a bad day, I mean, I’ll walk for about . . . not even a minute and then I’m stopping. (F30:17) With my PH, it differs from day to day . . . you don’t want to feel like you’re lying, but you’re not lying, because on your worst days, you can’t . . . I can’t get out of bed, and I can’t move. (F26:29)
Expectations from treatments Starting PH treatment requires a period of adjustment, with participants learning to live with a life-threatening disease, and for some, beginning an invasive treatment regimen. For most participants, there appeared to be an acceptance that treatment would at best “maintain” their PH: It’s kept me going, nothing fantastic, but I know that with this condition . . . you go downwards . . . I haven’t. . . . Without treatment, I think I would have ended up on the chair (for the) best part of the day. (M70:8) As long as I can get up in the morning, still get myself ready . . . and be independent within the limits of PH. (F35:7) After about 2–3 years, I’ve just felt myself sliding backwards again . . . I seem to be at a level (where) I’m not improving massively, but I’m not getting worse, so I’m kind of at sort of a plateau. (F35:7) Some younger patients acknowledged the fact that they were beginning to decline while only young: Now I’ve gone five years (on a specific treatment regimen), I’ve got in a decent time. These last few months . . . it’s been going downhill again. (M31:12). The perceived time-limiting factor for PH drugs remaining effective made participants fearful of the future: The last resort that the specialist reached for is this drug, and so if this stops working well, then there only is one other ending after that. (M61:10) You still worry, but nobody can tell you, and I don’t suppose many people want to know . . . about what’s going to happen . . . I mean, you worry about how long . . . this medication (will) keep lasting. (M47:3) Commencing new treatment offered optimism and a sense of control: I have been able to feel better from time to time . . . it’s not just that it makes me feel a bit better, you get a bit more optimistic that it is controllable. (M63:11)
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I’m quite looking forward to going on this tablet really . . . I try to stay positive, and hopefully it will make things so much better . . . I’m looking forward to seeing what it does. (F30:17)
Treatment burden For some, the side-effects of medication were viewed as “worse than the breathlessness” and could not be “endured.” The effort required to adhere to different treatments was viewed as causing distress, which patients often hid from loved ones: He (husband) doesn’t know, but I’m often in tears because the effort involved in doing the things . . . but I do it . . . I have to. (F65:13) Got started on this medication, which was fine at first, but then as months went by and the dose went up, it was just getting so painful . . . I stood that for seven months, and then I just couldn’t, I just couldn’t cope with it. (M47:3) The pain suffered at subcutaneous drug infusion sites was so unbearable for some participants that they considered stopping the drug, despite the risk of death associated with doing so. The following interview extract illustrates the determination of a patient to have a painful drug stopped and the willingness of him to risk death as a result. The extract also demonstrates the frustration and anguish of the patient in trying to convince their doctor that their concerns were real: It finally got to the point that, because I was either unconscious (from taking pain killers) or in extreme pain, my wife and I agreed that this had got to stop, and we agreed that the next time I saw the specialist I was going to say: “This drug must stop,” and we’d agreed that if he said: “Okay, that’s your choice, but be aware that there is no alternative,” but by this time I knew that Flolan (epoprostenol sodium) had been recommended, but turned down, and if he’d said: “There’s no alternative, you’re going to be dead within three months,” we’d agreed that the answer to that was going to be: “Well, okay, then that’s what it’ll be, but I cannot endure this drug anymore.” (The doctor said:) “I suppose you want Flolan, do you?,” and I said: “Well, if that’s what you think.” He (doctor) put the line in and connected up the drug and said: “How are you feeling?” I said: “I feel fabulous.” He said “Well, I really don’t understand that, because it’s effectively the same drug that you were on previously and causing such a lot of fuss and bother about.” I said: “But there’s no pain.” Removal of that pain is a gain.” (M61:10) Similarly, excessive side-effects that necessitated certain drugs to be stopped left participants wondering what the alternatives might be: The site pain . . . that really, really was painful, I couldn’t do anything else. I was literally bedridden, and I didn’t like that, but I stayed with it for six months . . . so it’s like . . . what’s next? I suppose. (F49:1) © 2014 Wiley Publishing Asia Pty Ltd.
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I’ve been to see my consultant, I’ve talked about death and how it manifests itself . . . towards the end . . . I would rather die than start messing around, having Hickman’s and permanent things into veins . . . it isn’t me, I’m afraid. (M44:24) For younger participants, in particular, adaptive mechanisms were used in an attempt to hide invasive therapies: I wear it (Hickman catheter) here [points to right chest area], so I wear those attractive vest things. I didn’t tell anybody, I just, again, hid it for some reason . . . I just sort of blamed it on the cold. (F35:14) People indicated that their educational needs and individual circumstances were not always considered by PH specialist staff when prescribing burdensome treatments: He (PH specialist nurse) went and got me some injections there and then . . . he didn’t teach me to inject myself. I had to ring my (general practitioner) surgery and say: “Can somebody please give me a lesson in this tomorrow morning, because I’ve got to do this?” I was angry that nobody had bothered to tell me. (F56:2)
Awareness of the financial burden of treatments All participants were conscious of the financial cost of “keeping themselves alive.” Some felt embarrassed or guilty about the cost of their medications to the National Health Service (NHS), but argued that the only alternative was for people with PH to die: This drug is indecently expensive . . . so the NHS, particularly nowadays, has this wonderful notion, stop this drug, think of the money we would save. Well, all of us would die. It’s as simple as that. (M61:10) It’s a very expensive drug, and I have to admit, I wasn’t happy with that. Then they said I shouldn’t worry about the cost, just . . . you don’t need to worry about cost. (M53:18) I was absolutely amazed at the cost to the Health Service of keeping me alive. Even to the extent of saying to my family: “Am I worth it?” (F72:23) People also spoke of their struggles to be provided with PH medications by local health authorities: We had trouble getting that (PH drug) . . . through the PCT (Primary Care Trust) . . . I was about to get in touch with my MP (Member of Parliament). I would have gone to the local media. (F69:6) It’s a postcode lottery . . . and we’ve had to fight with NICE (National Institute of Health and Care Excellence) all the way because . . . they say: “No, we can’t afford the thing.” NICE need to have more information and not judge us by other diseases, because it’s a different disease from anything else, and not as many people have it, so you can’t go – you can’t compare. (F69:6) © 2014 Wiley Publishing Asia Pty Ltd.
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DISCUSSION This study provides a descriptive summary of the participants’ experiences of living with PH. Key themes included the daily challenges of living with a rare condition that is largely “hidden” and its related complex treatment regimes. Description of these themes offers insight into the experience of living with and managing PH, and helps identify social, policy, and healthcare issues that affect those suffering from the disease. The most significant finding from this study was the level of difficulty experienced by patients in relation to demonstrating how unwell they are to people, including family, friends, benefit claims officials, and healthcare professionals. This lack of understanding about PH contributes to social withdrawal and isolation. Patients described awkward situations that negatively affected their self-confidence and sense of identity. The variable presence of PH symptoms, in particular breathlessness and fatigue, also changed the way patients lived their lives. The results of this study are consistent with previous studies assessing the impact of fibromyalgia (Cunningham & Jillings, 2006) and other chronic illnesses (Charmaz, 1983). In particular, the experience of people living with fibromyalgia appears to mirror the experience of PH, as neither condition has an obvious physical ailment to alert or “remind” people of the individual’s illness. Participants even described a need to remind family and friends that they had a chronic illness. Others decided not to disclose how much they were suffering. The invisibility of PH, despite its intrusiveness, presented patients with additional social difficulties, as a constant battle to be recognized as a legitimately unwell person ensued. Having a chronic condition and being different from the general population subjects a person to possible stigmatization by those who do not have the illness or understand the reality of it (Undeland & Malterud, 2007). Coping with stigma involves a variety of strategies, including the decision about whether to disclose the condition and suffer further stigma, or attempt to conceal the condition, or aspects of the condition, in an attempt to be seen as normal (Joachim & Acorn, 2001). For the participants in the current study, this ranged from stigmatization from loved ones to administrative people assessing a person’s right to claim social security. This was compounded by the lack of knowledge about PH and added to experiences of stigmatization and discrimination, as reported in other long-term conditions (Wahl et al., 2002; Lempp et al., 2006; Mendelson, 2006). A lack of knowledge about PH from the perspective of the general public and healthcare professionals has been previously noted as a considerable cause for concern in the prediagnostic phase of PH (Armstrong et al., 2012). Knowledge of the disease was a key factor in patients receiving a timely and correct diagnosis. The current study is the first to describe the impact of living with a rare and often “hidden” condition. In some regards, participants were struggling for credibility and affirmation. The uncertainty surrounding the PH illness trajectory and prognosis led to struggles with applications for social security benefits. In the UK, PH care is assigned to six designated specialist PH centers (National
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Pulmonary Hypertension Centres of the UK and Ireland, 2008). These centers focus on providing optimal clinical care, yet might not fully aware of and able to meet the social needs of this population. The treatment burden in PH can be significant, especially in the presence of invasive drug therapies (Peloquin et al., 1998; Flattery et al., 2005; McDonough et al., 2011). Our findings are comparable with these previous accounts. Further to this, we found that patients’ expectations of PH treatments were largely focused on limitations. Many expected that their current treatment would eventually stop working. As a result, participants had readjusted their expectations of their abilities and believed that maintenance, as opposed to improvement, was acceptable. We found that side-effects could be extremely distressing, and patients often had to convince healthcare providers and social security assessors before they were taken seriously. For some patients, even death was considered an alternative to continuing a treatment with intolerable side-effects. Participants in the current study were acutely aware that PH medications incur a significant financial cost to the healthcare system. This led to some legitimizing their existence and questioning their own worth. Examples of how people would go to extreme lengths to secure their treatments, such as contacting their local MP, were heard. Linked with this was the acknowledgment that PH was a relatively unknown illness and that there was a perceived need to lobby in order to ensure that treatments would be available to patients when they needed them. A range of factors, including past experiences with medications, relationships with healthcare providers, and the cost of medication, all influenced patients’ expectations of their medications (Dolovich et al., 2008). No participants in the current study disclosed non-adherence tendencies toward prescribed PH medicines. Nonetheless, a more comprehensive discussion between patients and their healthcare providers about these factors could ascertain whether medications are used optimally, and this warrants further investigation. This study has some limitations. This is a cross-sectional study, and therefore, we are not able to report on the longitudinal experiences of participants and how these might change over time. The study only reports the views and perceptions of patients. Future research should include the views of family members, where they are interviewed separately from people with PH and healthcare professionals.
Conclusion It is clear that the themes mentioned by participants in this study play an integral role in living with PH. The data presented here highlight the challenges experienced by patients with PH, including coping with invasive treatment regimens, uncertainty about the future, and a perceived lack of understanding from those around them. This experience often leads to stressful and frustrating encounters with loved ones, healthcare professionals, and people conducting benefit entitlements. This study found that people with PH would benefit
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if healthcare professionals, family, and friends, would validate their condition, and in doing so, provide them with appropriate support. Future research should explore patients’ experiences of living with PH over time and consider the views of family members and healthcare professionals. In particular, we recommend that focusing on patients’ quality of life and interventions designed to support patients and families living with PH should be a priority for future research and service development.
ACKNOWLEDGMENTS Funding for the research was provided by the Pulmonary Hypertension UK (PHA) UK. Editorial assistance was provided by Kerren Davenport.
CONTRIBUTIONS Study design: JY, IA, SB. Data collection and analysis: JY, IA, SB. Manuscript writing: JY, IA, SB
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Nursing and Health Sciences (2014), 16, 454–460
Research Article
Impact of living with pulmonary hypertension: A qualitative exploration Janelle Yorke, PhD, MRes, PGCE, RN,1 Iain Armstrong, M Med Sci, RN2,3 and Sarah Bundock, BSc4 1 University of Manchester, Manchester, 2Pulmonary Hypertension Unit, Royal Hallamshire Hospital, 3Pulmonary Hypertension Association UK, Sheffield and 4HealthEd, Cheshire, UK
Abstract
Little is known about the impact of living with pulmonary hypertension. This paper reports data exploring the experience of living with pulmonary hypertension. Qualitative, semistructured, one-to-one interviews were conducted in participants’ homes to understand their experiences of living with pulmonary hypertension. Thematic analysis was used to identify codes and generate themes from the interview data. The identification of initial codes was conducted independently by the first author, and checked by the second. Thirty patients recruited through the pulmonary hypertension descriptions of living with pulmonary hypertension are presented under five themes that center on the invisibility of pulmonary hypertension, and its complex treatment are presented: (i) living with a hidden illness; (ii) being on a symptomology rollercoaster; (iii) expectations from treatments; (iv) treatment burden; and (v) awareness of financial burden of treatments. Key findings included daily challenges of living with a rare condition that is largely “hidden” and its related complex treatment regimes. People with pulmonary hypertension would benefit if more healthcare professionals, as well as family and friends, would validate their condition and provide them with appropriate support.
Key words
interview, perception, pulmonary hypertension, qualitative.
INTRODUCTION Pulmonary hypertension (PH) is a rare condition that has many causes and is life limiting. Patients with PH suffer from breathlessness, fatigue, decreased exercise capacity, light headedness, and syncope (Cenedese et al., 2006; McDonough et al., 2011). Since the widespread use of the various new treatment options, the long-term prognosis of PH has significantly improved (Kiely et al., 2013). However, none of these therapies are curative, and varying degrees of impairment and symptom experience remain throughout the PH trajectory (Taichman & Mandel, 2007). The focus of research in PH has been clinical trials of therapies, and to a lesser extent, measuring health-related quality of life and symptom severity using a variety of quantifiable questionnaires. There has been a recent increase in the desire to assess health-related quality of life in patients with PH, which is evidenced by the development of a new health-related quality of life tool (emPHAsis-10) (Yorke et al., 2013). While that work has provided valuable quantitative information to measure the impact of PH, there is a Correspondence address: Janelle Yorke, School of Nursing, Midwifery and Social Work, University of Manchester, Room 5.320 Jean McFarlane Building, Oxford Road, Manchester M139PL, UK. Email: [email protected] Present address: Sarah Bundock, Trust Life, Cheshire, UK. Received 11 October 2013; revision received 26 November 2013; accepted 30 January 2014
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need for research that explores patients’ experiences of living with PH in order to gain a greater understanding of patients’ physical and psychosocial needs. Qualitative work exploring the experience of living with PH is currently limited to three studies (Peloquin et al., 1998; Flattery et al., 2005; McDonough et al., 2011), and one study focusing on the pre-diagnostic phase (Armstrong et al., 2012). Peloquin et al. (1998) interviewed three females in the initial stages of prostacyclin therapy and found that the therapy regimen was burdensome and impacted negatively on the patients’ perceived quality of life (Peloquin et al., 1998). Flattery et al. (2005) explored the experiences of 11 patients with PH and found that coping with uncertainties and taxing treatment regimens were both challenging and distressing for the patient (Flattery et al., 2005). Breathlessness has been identified as the most burdensome symptom of PH, and presents patients with activity limitation and a need to redefine their lives (McDonough et al., 2011). Taken together, this small body of work begins to shed light on the challenges of living with PH. Further exploration, however, could help practitioners and researchers develop a greater understanding of the complex issues faced by this patient group and aid the development of specific interventions and support mechanisms for people with PH.This paper reports the data obtained from a program of research exploring the day-to-day impact of PH. doi: 10.1111/nhs.12138
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METHODS In-depth, semistructured interviews were used to capitalize on the intricacies of the experiences of the participants. A semistructured interview style provided the flexibility to ensure that key topics were covered, while enabling the interviewee to disclose additional information that might diverge from the interview guide. The aim of the interviews was to explore participants’ experiences of day-to-day living with PH.
Participants Participants were recruited via the Pulmonary Hypertension Association (PHA) UK membership list. The PHA UK is the only charity in the UK for people with PH, with approximately 3000 members. Potential participants were informed about the study at a PHA UK patient conference in 2009, and contact details were collected from those who expressed an interest in participating. Responders were then purposively sampled from this list to ensure maximal variation, including both men and women of different ages, a range of etiologies and time since diagnosis, and from different geographic areas and specialist PH centers in England. PHA UK staff assisted the researchers to identify participants who met the inclusion criteria and range of demographic details, as described above. The inclusion criteria were patients: (i) diagnosed with PH (mean pulmonary artery pressure of 25 mm Hg or more) of any etiology; (ii) aged 18 years or older; (iii) able to speak and understand English; and (iv) able to provide informed consent. The exclusion criteria were: (i) chronic thrombotic embolic disease suitable for surgery; (ii) patients in the terminal stages of disease; and (iii) known severe and enduring mental illness. The study interviews were conducted in 2010 and took place in the participants’ homes. Interviews were conducted by an experienced health services researcher (SB) not involved in the provision of care for people with PH. Thirty one-to-one, in-depth interviews were conducted with patients. The recruitment of new participants discontinued when no new key themes emerged (Bowen, 2008). Verbal information about the study was provided at the PHA UK 2009 conference and over the telephone when potential participants were contacted to ascertain if they were still interested in participating. Written, informed consent was obtained prior to the interview commencing and was reviewed verbally at the beginning and end of the interview. Ethical approval was obtained from the Pulmonary Hypertension Association Medical Committee (2011–01) and the University of Salford Research Ethics Committee (NURA018).
Data collection and analysis An in-depth interview guide was developed to cover the patients’ experience of living with PH, which included the following questions: 1. When you were first diagnosed with PH what were your thoughts and feelings? How do you feel about having PH now?
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2. What impact, if any at all, would you say your PH has had on your life? 3. What impact, if any at all, would you say your PH has had on your family/friends? 4. What difference, if any at all, would you say your treatment for PH has had on your overall quality of life? 5. What do you think your quality of life would be like if you were not having any treatments for your PH? 6. What, if any impact has the diagnosis of PH had on your work life and financial circumstances? 7. What is your opinion about the standard of care you receive to manage your PH? The interview guide was developed during discussions with PH nurse specialists and patient representatives affiliated with the PHA UK. Interviews lasted between 40 and 100 min and were audio-recorded and transcribed verbatim. The methods used to code and categorize data were approaches to thematic analysis described by Braun & Clarke (2006). The identification of initial codes was conducted independently by the first author and checked by the second. Five themes were developed through further data scrutiny and discussion. The transcripts were analyzed line by line and paragraph by paragraph, looking for significant statements and codes according to the topics addressed. The researcher compared the various codes based on differences and similarities, and sorted them into categories. Finally, the categories were formulated into five themes.
RESULTS The characteristics of the 30 patients who participated in the study are detailed in Table 1. The coding used to represent participants was as follows: F, female; M, male; and individual study code and participant’s age (e.g. F2:56 is a female patient, study code 2, and 56 years of age). Participants’ descriptions of living with PH are presented under five themes that center on the invisibility of PH and its complex treatment.
Living with a hidden illness Many participants described living with a “hidden” illness, as “you can’t see anything.” The lack of visible signs of having a chronic condition, that might otherwise alert people to the fact that they were ill, presented participants with a unique challenge. There was a sense that other people did not appreciate how ill participants were, and often felt that they were required to justify why they were tired or short of breath: I think what I don’t like about having pulmonary hypertension is that no-one can see it . . . I got wheelchair assistance at the airport, and they (friends) went, “Well, what’s wrong with you?,” and I was like, “Well, I don’t really need to tell you, but . . .” “Well, you look fine.” (F26:29) © 2014 Wiley Publishing Asia Pty Ltd.
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Table 1. Characteristics of the 30 patients with pulmonary hypertension interviewed for the study Characteristics Sex (female : male) Age (mean years; SD; range) Working status Work full time Work part time Retired Long-term sick Unemployed Education PH etiology Idiopathic Connective tissue disease Congenital heart disease Chronic thromboembolic Heritable Portal hypertension Lung disease Drugs/toxins Time since diagnosis < 1 year 1–< 3 years 3–< 5 years 5–< 10 years > 10 years WHO PH functional class I II III IV
N = 30 18:12 56.3 (38; 26–80) 2 4 11 9 2 2 12 7 5 2 1 1 1 1 2 2 7 12 7 0 9 19 2
PH, pulmonary hypertension; SD, standard deviation; WHO, World Health Organization.
They see me and they think, well you’re just normal, you’re fine, you haven’t got any wheelchairs or contraptions and stuff . . . they don’t understand because I look normal. (F35:14) Cos you can’t see anything, and when people see you they’ll say, “Well, you’re looking really well, how are you doing?,” and even if I’m doing something in the house and I become breathless, somebody might say something like, “Oh, have you been running?” (F49:1) Even when oxygen therapy was used, some participants experienced questioning from other people: “Why are you using oxygen?” Even fellow patients: “What (are) you using oxygen for, you don’t look like you need oxygen?” (F42:15)
We took a life insurance policy, and it said “terminal illness benefit” on it . . . because they don’t know about PH and because they don’t have it on their list and stuff like that . . . they just fob you off with all kinds of stuff . . . they just want the doctor to say that I’m going to die in 12 months, but they’ve tried to tell them that with this condition you could die anytime. (M47:3) Stories about appealing against unsuccessful insurance and disability benefit claims applications were common: I had to appeal. . . . There was a doctor there, and he just said, “Well, yeah.” He said: “Basically you’ve got heart failure.” Degrading, I suppose . . . because you can’t see anything. (F49:1) Benefits, they didn’t know what pulmonary hypertension was. I look obviously extremely well on the outside with no sort of “disabilities.” They found it extremely hard to accept that I couldn’t work. I went through an appeal process. (F35:7) Younger participants in particular found it difficult to access help: At 25 . . . trying to actually get any kind of . . . financial help from the government, any sort of benefit, was extremely difficult. (F35:7) For others, it was not PH itself, but the side-effects of invasive treatments to that expedited benefit claims. In particular, patients receiving subcutaneous infusions and suffering from infusion site pain and related problems could access benefits more easily: I was sent to a government doctor, and she said: “What’s the problem and what are you doing about it?,” and I said: “Well, I’ve got this drug,” and on that day I had it in my thigh, and I said, “But it is so sore,” and she said: “Well, can I see it?” I said: “You can see it, but please do not touch it,” because . . . from my knee joint to my hip joint, it was swollen . . . and bright red, and she said: “Oh my God, right okay, that’s all,” and I then was awarded whatever the benefit was. (M61:10)
Being on a PH symptomatology rollercoaster Participants reported that their PH symptoms, particularly breathlessness, were unpredictable and made planning for the immediate and long-term future difficult:
It’s sort of the assumption that you’re young and you look fit, therefore you should be; it must be something you’ve done yourself, you know, like smoking. (F42:15)
Well, you just thought every single day . . . when you have an episode like that (deterioration), then you just get better and you’ve been better, say, for three weeks, and you feel great again, and then whack, you’re back to square one. (M53:18)
Having a “hidden” and unknown illness made interactions with insurance and government organizations for sickness and disability benefit claims difficult, and at times, degrading for the person with PH:
Because I can be fine for a month, as fine as I can be with this, and then I can have a down period . . . I can be really more breathless than usual. You just have to stay in and deal with that. (F72:23)
This often resulted in a sense of social stigma:
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Living with a fluctuating illness made applying for sickness and disability benefits difficult, and a source of anxiety and frustration for many participants: I did try and put a claim for disability allowance, but they didn’t class me as needing disability . . . if it’s a bad day, I mean, I’ll walk for about . . . not even a minute and then I’m stopping. (F30:17) With my PH, it differs from day to day . . . you don’t want to feel like you’re lying, but you’re not lying, because on your worst days, you can’t . . . I can’t get out of bed, and I can’t move. (F26:29)
Expectations from treatments Starting PH treatment requires a period of adjustment, with participants learning to live with a life-threatening disease, and for some, beginning an invasive treatment regimen. For most participants, there appeared to be an acceptance that treatment would at best “maintain” their PH: It’s kept me going, nothing fantastic, but I know that with this condition . . . you go downwards . . . I haven’t. . . . Without treatment, I think I would have ended up on the chair (for the) best part of the day. (M70:8) As long as I can get up in the morning, still get myself ready . . . and be independent within the limits of PH. (F35:7) After about 2–3 years, I’ve just felt myself sliding backwards again . . . I seem to be at a level (where) I’m not improving massively, but I’m not getting worse, so I’m kind of at sort of a plateau. (F35:7) Some younger patients acknowledged the fact that they were beginning to decline while only young: Now I’ve gone five years (on a specific treatment regimen), I’ve got in a decent time. These last few months . . . it’s been going downhill again. (M31:12). The perceived time-limiting factor for PH drugs remaining effective made participants fearful of the future: The last resort that the specialist reached for is this drug, and so if this stops working well, then there only is one other ending after that. (M61:10) You still worry, but nobody can tell you, and I don’t suppose many people want to know . . . about what’s going to happen . . . I mean, you worry about how long . . . this medication (will) keep lasting. (M47:3) Commencing new treatment offered optimism and a sense of control: I have been able to feel better from time to time . . . it’s not just that it makes me feel a bit better, you get a bit more optimistic that it is controllable. (M63:11)
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I’m quite looking forward to going on this tablet really . . . I try to stay positive, and hopefully it will make things so much better . . . I’m looking forward to seeing what it does. (F30:17)
Treatment burden For some, the side-effects of medication were viewed as “worse than the breathlessness” and could not be “endured.” The effort required to adhere to different treatments was viewed as causing distress, which patients often hid from loved ones: He (husband) doesn’t know, but I’m often in tears because the effort involved in doing the things . . . but I do it . . . I have to. (F65:13) Got started on this medication, which was fine at first, but then as months went by and the dose went up, it was just getting so painful . . . I stood that for seven months, and then I just couldn’t, I just couldn’t cope with it. (M47:3) The pain suffered at subcutaneous drug infusion sites was so unbearable for some participants that they considered stopping the drug, despite the risk of death associated with doing so. The following interview extract illustrates the determination of a patient to have a painful drug stopped and the willingness of him to risk death as a result. The extract also demonstrates the frustration and anguish of the patient in trying to convince their doctor that their concerns were real: It finally got to the point that, because I was either unconscious (from taking pain killers) or in extreme pain, my wife and I agreed that this had got to stop, and we agreed that the next time I saw the specialist I was going to say: “This drug must stop,” and we’d agreed that if he said: “Okay, that’s your choice, but be aware that there is no alternative,” but by this time I knew that Flolan (epoprostenol sodium) had been recommended, but turned down, and if he’d said: “There’s no alternative, you’re going to be dead within three months,” we’d agreed that the answer to that was going to be: “Well, okay, then that’s what it’ll be, but I cannot endure this drug anymore.” (The doctor said:) “I suppose you want Flolan, do you?,” and I said: “Well, if that’s what you think.” He (doctor) put the line in and connected up the drug and said: “How are you feeling?” I said: “I feel fabulous.” He said “Well, I really don’t understand that, because it’s effectively the same drug that you were on previously and causing such a lot of fuss and bother about.” I said: “But there’s no pain.” Removal of that pain is a gain.” (M61:10) Similarly, excessive side-effects that necessitated certain drugs to be stopped left participants wondering what the alternatives might be: The site pain . . . that really, really was painful, I couldn’t do anything else. I was literally bedridden, and I didn’t like that, but I stayed with it for six months . . . so it’s like . . . what’s next? I suppose. (F49:1) © 2014 Wiley Publishing Asia Pty Ltd.
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I’ve been to see my consultant, I’ve talked about death and how it manifests itself . . . towards the end . . . I would rather die than start messing around, having Hickman’s and permanent things into veins . . . it isn’t me, I’m afraid. (M44:24) For younger participants, in particular, adaptive mechanisms were used in an attempt to hide invasive therapies: I wear it (Hickman catheter) here [points to right chest area], so I wear those attractive vest things. I didn’t tell anybody, I just, again, hid it for some reason . . . I just sort of blamed it on the cold. (F35:14) People indicated that their educational needs and individual circumstances were not always considered by PH specialist staff when prescribing burdensome treatments: He (PH specialist nurse) went and got me some injections there and then . . . he didn’t teach me to inject myself. I had to ring my (general practitioner) surgery and say: “Can somebody please give me a lesson in this tomorrow morning, because I’ve got to do this?” I was angry that nobody had bothered to tell me. (F56:2)
Awareness of the financial burden of treatments All participants were conscious of the financial cost of “keeping themselves alive.” Some felt embarrassed or guilty about the cost of their medications to the National Health Service (NHS), but argued that the only alternative was for people with PH to die: This drug is indecently expensive . . . so the NHS, particularly nowadays, has this wonderful notion, stop this drug, think of the money we would save. Well, all of us would die. It’s as simple as that. (M61:10) It’s a very expensive drug, and I have to admit, I wasn’t happy with that. Then they said I shouldn’t worry about the cost, just . . . you don’t need to worry about cost. (M53:18) I was absolutely amazed at the cost to the Health Service of keeping me alive. Even to the extent of saying to my family: “Am I worth it?” (F72:23) People also spoke of their struggles to be provided with PH medications by local health authorities: We had trouble getting that (PH drug) . . . through the PCT (Primary Care Trust) . . . I was about to get in touch with my MP (Member of Parliament). I would have gone to the local media. (F69:6) It’s a postcode lottery . . . and we’ve had to fight with NICE (National Institute of Health and Care Excellence) all the way because . . . they say: “No, we can’t afford the thing.” NICE need to have more information and not judge us by other diseases, because it’s a different disease from anything else, and not as many people have it, so you can’t go – you can’t compare. (F69:6) © 2014 Wiley Publishing Asia Pty Ltd.
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DISCUSSION This study provides a descriptive summary of the participants’ experiences of living with PH. Key themes included the daily challenges of living with a rare condition that is largely “hidden” and its related complex treatment regimes. Description of these themes offers insight into the experience of living with and managing PH, and helps identify social, policy, and healthcare issues that affect those suffering from the disease. The most significant finding from this study was the level of difficulty experienced by patients in relation to demonstrating how unwell they are to people, including family, friends, benefit claims officials, and healthcare professionals. This lack of understanding about PH contributes to social withdrawal and isolation. Patients described awkward situations that negatively affected their self-confidence and sense of identity. The variable presence of PH symptoms, in particular breathlessness and fatigue, also changed the way patients lived their lives. The results of this study are consistent with previous studies assessing the impact of fibromyalgia (Cunningham & Jillings, 2006) and other chronic illnesses (Charmaz, 1983). In particular, the experience of people living with fibromyalgia appears to mirror the experience of PH, as neither condition has an obvious physical ailment to alert or “remind” people of the individual’s illness. Participants even described a need to remind family and friends that they had a chronic illness. Others decided not to disclose how much they were suffering. The invisibility of PH, despite its intrusiveness, presented patients with additional social difficulties, as a constant battle to be recognized as a legitimately unwell person ensued. Having a chronic condition and being different from the general population subjects a person to possible stigmatization by those who do not have the illness or understand the reality of it (Undeland & Malterud, 2007). Coping with stigma involves a variety of strategies, including the decision about whether to disclose the condition and suffer further stigma, or attempt to conceal the condition, or aspects of the condition, in an attempt to be seen as normal (Joachim & Acorn, 2001). For the participants in the current study, this ranged from stigmatization from loved ones to administrative people assessing a person’s right to claim social security. This was compounded by the lack of knowledge about PH and added to experiences of stigmatization and discrimination, as reported in other long-term conditions (Wahl et al., 2002; Lempp et al., 2006; Mendelson, 2006). A lack of knowledge about PH from the perspective of the general public and healthcare professionals has been previously noted as a considerable cause for concern in the prediagnostic phase of PH (Armstrong et al., 2012). Knowledge of the disease was a key factor in patients receiving a timely and correct diagnosis. The current study is the first to describe the impact of living with a rare and often “hidden” condition. In some regards, participants were struggling for credibility and affirmation. The uncertainty surrounding the PH illness trajectory and prognosis led to struggles with applications for social security benefits. In the UK, PH care is assigned to six designated specialist PH centers (National
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Pulmonary Hypertension Centres of the UK and Ireland, 2008). These centers focus on providing optimal clinical care, yet might not fully aware of and able to meet the social needs of this population. The treatment burden in PH can be significant, especially in the presence of invasive drug therapies (Peloquin et al., 1998; Flattery et al., 2005; McDonough et al., 2011). Our findings are comparable with these previous accounts. Further to this, we found that patients’ expectations of PH treatments were largely focused on limitations. Many expected that their current treatment would eventually stop working. As a result, participants had readjusted their expectations of their abilities and believed that maintenance, as opposed to improvement, was acceptable. We found that side-effects could be extremely distressing, and patients often had to convince healthcare providers and social security assessors before they were taken seriously. For some patients, even death was considered an alternative to continuing a treatment with intolerable side-effects. Participants in the current study were acutely aware that PH medications incur a significant financial cost to the healthcare system. This led to some legitimizing their existence and questioning their own worth. Examples of how people would go to extreme lengths to secure their treatments, such as contacting their local MP, were heard. Linked with this was the acknowledgment that PH was a relatively unknown illness and that there was a perceived need to lobby in order to ensure that treatments would be available to patients when they needed them. A range of factors, including past experiences with medications, relationships with healthcare providers, and the cost of medication, all influenced patients’ expectations of their medications (Dolovich et al., 2008). No participants in the current study disclosed non-adherence tendencies toward prescribed PH medicines. Nonetheless, a more comprehensive discussion between patients and their healthcare providers about these factors could ascertain whether medications are used optimally, and this warrants further investigation. This study has some limitations. This is a cross-sectional study, and therefore, we are not able to report on the longitudinal experiences of participants and how these might change over time. The study only reports the views and perceptions of patients. Future research should include the views of family members, where they are interviewed separately from people with PH and healthcare professionals.
Conclusion It is clear that the themes mentioned by participants in this study play an integral role in living with PH. The data presented here highlight the challenges experienced by patients with PH, including coping with invasive treatment regimens, uncertainty about the future, and a perceived lack of understanding from those around them. This experience often leads to stressful and frustrating encounters with loved ones, healthcare professionals, and people conducting benefit entitlements. This study found that people with PH would benefit
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if healthcare professionals, family, and friends, would validate their condition, and in doing so, provide them with appropriate support. Future research should explore patients’ experiences of living with PH over time and consider the views of family members and healthcare professionals. In particular, we recommend that focusing on patients’ quality of life and interventions designed to support patients and families living with PH should be a priority for future research and service development.
ACKNOWLEDGMENTS Funding for the research was provided by the Pulmonary Hypertension UK (PHA) UK. Editorial assistance was provided by Kerren Davenport.
CONTRIBUTIONS Study design: JY, IA, SB. Data collection and analysis: JY, IA, SB. Manuscript writing: JY, IA, SB
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