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CommentaryEvaluating Health-Related Quality of Life, Work Ability, and Disability in Pulmonary Arterial Hypertension: An Unmet Need
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Background
The term pulmonary hypertension (PH) defines a group of diseases characterized by an increase in pulmonary artery pressure, whose natural history varies considerably depending on cause and pathophysiology.1 The Venice 2003 modification of the World Health Organization (WHO) classification of PH encompasses five classes (groups) as summarized in Table 1.1 Patients with PAH in WHO group 1 share a related pathobiology, but the natural history, survival from time of diagnosis, and response to
QoL Assessment: What Is Different About PAH?
Since 1948, when the WHO defined health as being not only the absence of disease and infirmity but also the presence of physical, mental, and social well-being, QoL issues have become steadily more important in health-care practice and research.3 QoL refers to one's holistic evaluation of satisfaction with one's life in areas (domains) that he or she considers important.4 Specifically, health-related quality of life (HRQoL) refers to the subjectively perceived impact of one's health on the
QoL Measures in PH
In a review of the available literature in PubMed from 1970 to January 2008 using key words “pulmonary hypertension” AND “quality of life,” we found 146 English-language articles with at least an abstract available. In a review of methods in each of the studies with full text available, < 10% used a standardized questionnaire to quantify QoL.
Questionnaires are the basic instruments to evaluate HRQoL. Several classic HRQoL indicators have been used in PH, and a PH-specific questionnaire was
Measures of Psychosocial Symptoms in PAH
Patients with PAH have relatively unique concerns in the spectrum of cardiopulmonary diseases that can impact their QoL. They suffer from the uncertainty of the disease, the time delay and rapid progression of symptoms prior to an accurate diagnosis, the ominous feeling of breathlessness, the feeling of being alone with an orphan disease, and the statement that “there is no cure.” Each of these thoughts can results in anxiety, panic, depression, anger, and often hopelessness. Support systems
Work Ability and Disability in PAH
The concept of work ability can be defined as the ability of a worker to perform his or her job, taking into account the specific work demands, individual health condition, and mental resources.25 Chronic cardiopulmonary diseases are associated with excess absenteeism, lower performance, and disability,26 each of which can impact a person's sense of well-being.27
PAH most often affects working-aged people, the majority young to middle-aged women,14 and can rapidly lead to severe disability and
Conclusions and Future Directions
Standard generic QoL measures and those specific to chronic lung and heart disease do not properly reflect the clinical status or prognosis of persons with PAH.
A Delphi exercise (a structured process for collecting and distilling knowledge from a group of experts by means of a series of questionnaires interspersed with controlled opinion feedback) was recently conducted among 78 experts (EPOSS-OMERACT group) to define appropriate outcome measures in PAH related to systemic sclerosis.32 Of the
Acknowledgments
Author contributions: Dr. Rubenfire is the senior author, contributed to the study design, and was the principle writer and mentor of the fellows while they were in the United States. Dr. Lippo is a research fellow who performed the literature search for the bibliography, created the tables, and wrote drafts of some paragraphs in the article. Dr. Bodini is a research fellow who performed the literature search for certain segments of the article, helped to create the tables, and wrote drafts of
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A World of Maximalist Medicine: Physician Perspectives on Palliative Care and End-of-life for Patients With Pulmonary Arterial Hypertension
2023, Journal of Pain and Symptom ManagementCitation Excerpt :Patients with PAH experience progressive symptoms before eventual right ventricular failure and premature death.1 Life expectancy has improved with the advent of new therapies,1–6 but patients still experience significant symptom burden and premature death.7–12 Palliative care implementation for patients with PAH is underutilized7,13 contrasting with observations that it provides benefits for patients with other serious illnesses.14,15
Patient Journey and Disease-Related Burden in Japanese Patients With Chronic Thromboembolic Pulmonary Hypertension: A Mixed Methods Study
2021, Value in Health Regional IssuesCitation Excerpt :When comparing disease burdens in the present study with the results of previous studies20,21 examining those of common diseases with symptoms similar to CTEPH, such as chronic obstructive pulmonary disease and bronchial asthma, the present results showed that patients with CTEPH experienced mental and social burdens in addition to the common characteristics related to the physical burden imposed by symptoms and treatment. The physical burden, such as breathlessness, and the mental burden, such as awareness of the intractable nature of the disease and feeling isolated owing to lack of understanding among family members and friends, were consistent with the results of the previous studies in patients with pulmonary hypertension.9,22,23 In contrast, the results of this study emphasized the importance of a social network for patients with CTEPH.
Impact of psychological factors on the health-related quality of life of patients treated for pulmonary arterial hypertension
2018, Journal of Psychosomatic ResearchCitation Excerpt :In addition, the fact that women are usually overrepresented in PAH studies (from 81 to 88% of cases) [4,6,16–19] may bias results concerning emotional responses focussed on anxiety, depression or QoL [20]. Depression is nearly always investigated using self-questionnaires sent to the patient's home, whereas these symptoms merit a face to face clinical evaluation [21–23]. Finally, with a few rare exceptions [12,17], most such studies focussing on psychological disorders involve no or few psychologists and/or researchers in psychology.
Symptom interference severity and health-related quality of life in pulmonary arterial hypertension
2016, Journal of Pain and Symptom ManagementCitation Excerpt :Even though a symptom may not be reported as severe, it may dramatically interfere with a patient's ability to perform activities of daily living and care for themselves and/or their families. Patients with PAH have impairment in the ability to function that adversely affects their HRQOL.11,12 Others have found decreased HRQOL in PAH with increased symptom burden and a lack of awareness of the availability of palliative care.13
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