Findings of search

Overall, 19 studies were included in the synthesis (figure 1). Studies were published between 2005 and 2020; however, 18/19 were published in the last 10 years.20 Data reflect over 1900 individuals across Europe, North and South America and Asia. Ages ranged from 19 to 91 years. In all but one study, the samples were predominantly female21—likely reflecting the gender bias observed in PH.22 Only three studies provided sufficient detail regarding sample ethnicity.17 23 24 Participants had been diagnosed with PH ranging from <1 year to 24 years. Ten studies failed to report medical information concerning participant’s functional class—this is important given that experiences seemed to differ depending on disease-specific issues.17 25 The majority (n=14) of studies used research interviews for data collection, whereas greater variation of analytical methods was used (table 3).

• Download figure
• Open in new tab
• Download powerpoint

Figure 1

Preferred Reporting Items for Systematic Reviews and Meta-Analyses diagram. PH, pulmonary hypertension.

Quality assessment

All studies met the majority of the 10 quality assessment items (table 4). Seven studies failed to justify their decision for using a qualitative approach and one study provided a limited description of their data analysis method. Surprisingly, 15 articles did not report their method of reflexivity.

Descriptive themes

Six descriptive themes emerged:

Diagnosis

Participants reported experiencing PH-related symptoms a “long” time [²⁷, p. E19] prior to obtaining a diagnosis.21 26 27 This period was characterised by a decline in physical functioning, QoL and emotional and social difficulties.20 25 28 29 During this time, and thereafter, individuals expressed confusion over the cause of their experiences28—worries that were not necessarily reduced by the diagnosis, which was described as having posed “more questions than it answers”[22, p. 3]. Participants tended to avoid, adapt to or explain away their symptoms: “[I] just assumed that everything was down to smoking” [27, 28, p. 4].

Participants described undergoing a series of examinations, referrals and misdiagnoses to find an explanation for their experiences.26–29 This process left participants feeling ‘frustrated’ [21, p. 103], ‘uncertain’ [26, p. 611], angry and “disillusioned” with the perceived meaning of their symptoms [28, p. 6]. Receiving the diagnosis was described as ‘life-changing’ [37, p. 4]. While people responded differently, for instance, expressing feelings of ‘relief’—as they can now receive treatment [22, p. 3]—‘shock’ [30, p. 38], helplessness30 and confusion,26 a strong emotional response was evident.31 Following the diagnosis, individuals ruminated over the cause of their disease.27

Treatment

Participants discussed their reliance on specialist centres,6 21 which helped reduce feelings of uncertainty by answering questions and dispelling false narratives about PH: ‘it (the diagnosis) wasn’t as devastatingly unhopeful as first appeared’ [28, p. 7]. A disparity in care between services was described,6 specifically, between specialist centres and community services in terms of knowledge, treatments and approach to care.21 28 31 Many participants expressed the need for greater collaboration between services.6 21 26 27

Individuals often discussed how they felt their care could be improved, which included, regular checkups,24 receiving more information,27 32 promoting shared decision making26 and holistic care33 and involving family members.27

Participants did not view medication as a “cure” [29, p. 2], but rather to alleviate symptoms, improve health and ultimately, survival: “[if medication was stopped] Well, all of us would die. It’s as simple as that” [25, 27, 29, 31–33, 38, p. 458]. Many reported an almost immediate reduction in symptoms attributed to treatment,27 34 with a ‘strong emotional attachment’ to medication being noted [25, 33, p. 5].

Side effects of treatments were common, which seemed to mostly manifest physically.20 23 32 There was uncertainty surrounding side effects as participants reflected that it was not always clear whether their symptoms were associated with the disease itself or psychosocial burden of PH.33 35 Side effects posed as a barrier to adherence, as while some viewed the negatives of treatment as a tradeoff: “it’s added years to my life…but it’s bittersweet” [21, p. 102], others stopped despite the risk.36

Other barriers were discussed such as, accessibility and cost of treatment, emotional distress and problems with self-administration.23 24 31–34 36 Lack of perceived need also affected uptake as while many experienced emotional difficulties, some felt they did not need psychological support,34 perceiving: “the disease itself is the problem”[29, p. 4]. However, some expressed the need for help with managing anxiety and low mood.25

Participants described a number of worries associated with their treatment, such as, whether it was effective20 and if so for how long,36 potential risks,31 what other options are available29 31 36 and the wait to receive treatment.17 Visible treatments could be a source of ‘social stigma’ [27, 38, p. 456], leaving participants feeling ‘self-conscious’ [33, p. 4].

Prognosis

Participants were saddened,30 “terrified”[35, p. 162] and anxious over the prospect of their death.17 25 Individuals described many uncertainties associated with their prognosis including, how their disease would progress,23 35 what their future would hold,34 the impact of their death on loved ones17 21 25 and how long they had remaining: “I am clueless as to how long my heart will hold out” [29, p. 3]. One women wondered if her death would make it “easier” due to the impact of PH on her family [17, p. 4]. The pervasive fear over when or how their symptoms would worsen ‘plagued’ participants [24, p. 129], resulting in emotional and psychological difficulties.23 34

Healthcare professionals

Developing a therapeutic relationship with a PH “expert” was important [33, p. 5]. Professionals were described as a source of support,34 advice,6 knowledge,31 reassurance,28 motivation and ‘hope’ [29, 31, p. 4]. That said, participants reported some difficulties accessing or discussing their worries with professionals,26 27 as well as previous encounters in which professionals lacked competency,32 empathy or sensitivity: one participant, for example, reported being told “It’s [PH] worse than cancer, we can’t treat it” [28, p. 3].

Participants expressed their frustration over healthcare professionals’ poor understanding of PH.28 31 32 Individuals spoke of pleading or taking it on themselves to explain the disease to staff.21 27

Impact

A range of symptoms were experienced by participants including, dyspnoea, pain, dizziness, fatigue, palpitations and cognitive difficulties.20 21 23 26 32 35 Breathing problems, however, were described as the “main symptom” [27, p. E16], which was associated with feelings of breathlessness, ‘suffocating’, ‘choking’ and ‘pain’—this was closely interlinked with anxiety [24, 27, p. E16]. Breathing could become a ‘conscious act’ [27, p. E16], as participants provided precise details over which activities would cause them shortness of breath [27, p. E17]. That said, some symptoms were ‘unpredictable’ and likened to being on a ‘rollercoaster’ [30, 38, p. 456].

Decline in physical ability, mobility and energy levels were prevalent,25 30 which could be caused by any form of activity.23 26 31 Participated expressed ‘fear’ of engaging in activities [33, p. 4] due to the onset of symptoms, pain23 or it requiring too much energy.34 There seemed to be a discrepancy between participant’s ideal and actual self, in terms of ability,26 which manifested in feelings of ‘anger’ [24, p. 129], ”failing” [22, p. 4], ‘disappointment’ [32, p. 38] and shame.34

Individuals had to reduce or cease many activities,17 26 37 leading them to relate their situation to a “shackle” [33, p. 4]. Reflected in this, was the feeling of restriction, the nature of which had a considerable impact on profession, family and social roles.17 21 25 26 30 32 33 This appeared to be, somewhat, moderated by age, gender and stage of the disease.17 25 For example, younger participants had to limit themselves to activities that matched their ‘activity tolerance’ opposed to interests [33, p. 4]. Participants reported sexual difficulties largely due to fatigue and shortness of breath, which negatively affected their relationship.17 26 34 Financial problems associated with the cost of care and loss of earnings.25 30 32 Practical and emotional problems caused by travelling.31 34 Male and female participants discussed difficulties associated with having or caring for their children.17 21 25 27 34

There was a general sense that the disease could be invisible and “hidden” from others [38, p. 456]. Family, friends, the public, local authorities, insurance companies and even healthcare professionals were all described, at times, as failing to understand the disease and participant’s physical limitations.17 20 26 27 32 34 36 Participants found themselves having to ‘battle’ for their illness to be recognised [38, p. 458], and justify their difficulties.27 34

Life with PH could have a negative effect on participant’s mental health, with anxiety, low mood, isolation23 25 29 32 35 and suicidal ideation being discussed.30 37

Coping

Participants required a period of time to adjust to life with PH.20 23 30 36 Individuals coped differently, for example, an international study observed two approaches: ‘disease dominated’ whereby participants harboured a ‘passive attitude towards PH’, were dependent on others, and likely to experience depression; whereas ‘solution seekers’ developed strategies to manage, were less dependent and maintained a social life [33, p. 4].

A common approach to coping was preparation,21 ‘extensive planning’ and developing ‘daily routines’ [30, 33, p. 5]. Individuals discussed weighing up how activities would impact them, rather than “act on impulse” [24, p. 127]. Participants reported feeling like they had to ‘hold back or take it [daily activities] slow’ [21, 24, p. 127]. Personal limits were learnt through ‘testing’ their own ability [30, p. 39].

Many acknowledged the importance of developing a helpful or positive “mindset” [26, 29, p. 4], for example, focusing on activities that were still within their ability23: “I tell myself that I have to look at what I can still do…” [30, p. 39]. Self-talk,31 focusing on having overcome previous adversity,37 faith20 and self- humour were also used to cope.20 21

Altering expectations of ability, recovery and future25–27 36 allowed some to develop a new sense of purpose31 or “redefine’”their lives [24, p. 128, 27, 35]. Accepting their limits gave participants the perception that they were not letting their disease “dominate” [25, 26, 33, p. 4] and provided a sense of control23: “…it’s just my life, it’s just what I do” [21, p. 102]. Keeping active was described as helping to shift their focus away from their symptoms,35 control disease progression and have psychological benefits.6

Family, friends and employers were described as offering practical and emotional support.25 29 30 34 35 However, participants appeared conflicted, describing their own position as “enforced dependency” [17, 33, p. 3]. Social support could also be a barrier or counterproductive to coping, as how others dealt with the disease was not always helpful.32 34

Participants spoke about concealing, avoiding, diminishing and making excuses for their difficulties.17 21 29 30 36 37 This helped reduce some of the emotional impact of PH, as well as feeling less of a burden or protecting others.31 34 37 However, this could lead to social withdrawal and isolation, further straining relationships and causing psychological difficulties.25 29 30

Peer supports groups and organisations were common sources of reassurance, support and advice.32 34 Individuals sought validation and normalisation of their symptoms from people with shared experience35 and also looked to help others.24

Analytical themes

Four key analytical themes emerged:

Managing uncertainty

Participants acknowledged many uncertainties caused by internal, such as bodily sensations, or external events linked to PH, including the reaction of others26 or what the future may hold.17 Uncertainty was often associated with a perception of risk or threat, which precipitated and perpetuated psychological, emotional and physiological distress. Many of the approaches that participants employed to manage their difficulties related to uncertainty seemed to only provide brief relief, until the next unknown situation occurred, or their strategy fed back into their level of distress, for example:

Participants sought information and reassurance from a range of sources,20 in the hope of gaining knowledge, understanding and some sense of certainty.28 Unfortunately, given the complexity and lack of understanding of PH, this could result in even greater uncertainty.28

Individuals seemed to engage in worry as a helpful means to problem solve, plan and minimise negative outcomes. However, planning could be impractical due to the unpredictable nature of PH36 or it removed ‘spontaneity in their life’ [24, p. 127]. Worrying was also closely linked with hypervigilance to the impact of everyday activities23 and somatic changes as an ‘indicator’ to alter behaviour [22, 27, p. E17]. This resulted in anticipatory anxiety and panic.23

Some described avoiding25 37 or concealing their disease, however, this often prolonged distress and resulted in emotional difficulties, such as anxiety, guilt and loneliness.17

Physical nature of PH

PH was described as a physically limiting disease,32 as was there a tendency for individuals to report somatic experiences.21 23 26 Furthermore, despite experiencing emotional difficulties, participants often resisted the idea or need for psychological support.25 32 37 A notion further reflected in the relief on hearing the disease was not psychosomatic in nature.28

Conversely, participant’s social environment did not always recognise the physical manifestations of the disease—participants assumed because “you can’t see anything” and most of the time they “look fine” as there was a lack of visible signs [26, 38, p. 455]. Discordance between the meaning and impact participants and others attached to the disease seemed most salient on occasions when physical adaptations were required due to fatigue or breathlessness—symptoms of which could be invisible to others.25 34 It seemed the support participant’s received was, at times, influenced by other’s representation of the disease.21 Likewise, participant’s own perceptions of their circumstance may have affected their experiences of coping, which could help, in part, to explain resistance to psychological support.

Living with the rarity of PH

The rarity of PH was highlighted in discussions,25 26 no more so than when individuals expressed their frustration at the lack of PH awareness in society and healthcare.27 32 Lack of understanding and misconceptions over PH resulted in participants feeling stigmatised and discriminated against.21 25 32 Participants found themselves having to justify and legitimise their sick role, despite having an incurable disease32 36 or withdrawing to avoid difficulties.17 Individuals felt the need to become experts in PH, which made it all the more frustrating when others assumed that they knew better or when participant’s voices were unheard in care.21 27 Living with a rare condition left individuals feeling lonely and isolated: “forced to carry a burden no one wants, or understands fully” [33, p. 4]. Participants found understanding from experts in PH and PH communities.20

Transitional nature of PH

Participants progressed through different transitions living with PH.25 At first, experiencing a decline in general functioning, which appeared to improve following the diagnosis and administration of treatment.28 Participants next faced the process of adapting to life with PH and treatment,20 31 the goal of which was to stabilise the disease,6 maintain a good QoL and survive.25 Participants had to find a balance between feeling underactive, in which they perceived their identity as changing17 and themselves “failing” [22, p. 4], and overactive, whereby participants attempted to resume normal life despite the risk and increase in PH symptoms, or dissonance feeling torn between societal norms and their own ability.23 This balance was often achieved through ‘trial and error’ [30, p. 39] and becoming more aware of physical, psychological, family and society cues.