Adults’ experiences of living with pulmonary hypertension: a thematic synthesis of qualitative studies

Abstract

ObjectivesPulmonary hypertension is a life-shortening disease that has a considerable impact on quality of life. Improving our understanding of how individuals are affected and cope with the disease will help to improve services and outcomes. This review synthesises the published qualitative research that has listened to adults discuss their experiences of living with the disease.DesignA comprehensive systematic search of four databases was conducted in May 2020: Web of Science, PubMed, PsycINFO and Cochrane Library. Suitable studies were evaluated using the Critical Appraisal Skills programme. Findings from the studies were extracted and subjected to a thematic synthesis.ResultsNineteen articles were identified reflecting the experiences of over 1900 individuals impacted by pulmonary hypertension from Europe, North and South America and Asia. Ten studies did not report participant’s WHO functional class of pulmonary hypertension, which resulted in comparing experiences between different severity difficult. All studies met the majority of the quality assessment items. Six descriptive themes emerged discussing participant’s experiences of diagnosis, treatment, prognosis, healthcare professionals, impact and coping with pulmonary hypertension. Four higher order analytical themes were developed from the descriptive themes, reflecting: (i) uncertainties and anxiety that participants encountered related to pulmonary hypertension; (ii) lack of recognition of the impact of the condition; (iii) frustration at the paucity of awareness of pulmonary hypertension in society and healthcare settings and (iv) participant’s accounts of transitioning through different stages of living with the disease.ConclusionsThese findings form the first synthesis of experiences of life in individuals impacted by pulmonary hypertension and illustrate the multifaceted impact of the condition. The voices of numerous groups are missing from the literature highlighting the need for additional research. The results have implications for clinical practice emphasising the role of educational and psychological therapies to support those with the disease.

Consequences of a Diagnostic Label: A Systematic Scoping Review and Thematic Framework

“…This may include increased psychological distress, preference for invasive treatments, greater sick role behaviour, and restriction of independence ( 11 – 14 ). Additionally, research indicates the impact of a label is diverse and varies depending on your perspective as an individual labelled ( 15 , 16 ), family/caregiver ( 15 , 17 , 18 ), or healthcare professional ( 15 , 19 ).…”

Individually Tailored Remote Physiotherapy Program Improves Participation and Autonomy in Activities of Everyday Life along with Exercise Capacity, Self-Efficacy, and Low-Moderate Physical Activity in Patients with Pulmonary Arterial Hypertension: A Randomized Controlled Study

“…Highlight the approved effectiveness of the studied intervention, the individually tailored physiotherapy program added to stable target medical therapy, in patients with PAH to maintain the opportunity to participate in activities related to frequency of social contacts in follow-up should be done in the face of PAH as a long-term disease. At the same time, in our study, no substantial deterioration or differences between groups were found in the IPA subscale SL that mainly describes the quality of social relationships, which can be explained by the relatively older age of the participants, and it is highly possible that other issues arise in younger patients, as current evidence from qualitative research suggests that quality concerns of social life would be more relevant in younger patients with PAH as well as in patients with less time after confirmation of diagnosis [ 3 ]. Recognizing that social participation, for example, meeting friends, volunteer work, club activities, and quality of social relationships, described as sense of belonging, respect, inclusion, are important predictors of well-being in persons living with long-term health problems [ 32 ], interventions to support participation are highlighted as relevant part of the interdisciplinary treatment approach.…”

“…Besides, Rawling et al (2020) in the synthesis of qualitative studies on the experience of adult patients living with PH described four main topics, one of which emphasized the transitional nature of PH. Coming to the stage with the aim of stabilizing the disease, maintaining a good quality of life and survival, the person faces the challenge of finding a balance between restrictions, a sense of loss, and an attempt to live a previous life despite the exacerbation of symptoms and possible worsening, while forming a new identity and life [ 3 ].…”

“…Recognizing that social participation, for example, meeting friends, volunteer work, club activities, and quality of social relationships, described as sense of belonging, respect, inclusion, are important predictors of well-being in persons living with long-term health problems [ 32 ], interventions to support participation are highlighted as relevant part of the interdisciplinary treatment approach. Furthermore, note that studies of the patient’s perspective [ 3 , 4 ] agree that patients with PAH often describe feeling insecure, isolated, and as living with an invisible disease, therefore, to fully eliminate the impact of PAH on a patient’s life, complex interventions in addition to medical treatment of the underlying disease are important, and therefore our treatment approach has the potential to support an individual living with PAH to cope with illness. The observed benefits of the investigated program to improve the perceived chance of participation in activities related to frequency of social contacts in the PAH patients might be due to the change in the perception about their health condition and the improved sense of self-control abilities, as is approved relevant correlations between these factors in other studies [ 33 ].…”

“…It is a chronic, progressive and life-threatening disease, nevertheless, the appropriate target medical treatment today allows patients with PAH not only to survive, but also to live a relatively normal life by adjusting their lifestyle and establishing the optimal adaptation. However, adults living with PAH acknowledge many uncertainties, feel unsecure and isolated, and face the challenge of adapting and maintaining a good quality of life (QoL) [ 3 ]. To minimize the impact of PAH on a patient’s life, it is important to consider an interdisciplinary treatment approach with complex interventions related not only to the medical treatment of the underlying disease [ 4 ].…”