Chest
Volume 136, Issue 2, August 2009, Pages 597-603
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Commentary
Evaluating Health-Related Quality of Life, Work Ability, and Disability in Pulmonary Arterial Hypertension: An Unmet Need

https://doi.org/10.1378/chest.08-1260Get rights and content

To our knowledge, there are no specific and validated measures of quality of life (QoL) or degree of disability for pulmonary arterial hypertension (PAH). A review of the literature shows that, with the exception of one recently designed specifically for pulmonary hypertension, QoL questionnaires used in PAH studies are generic measures. These are selected because of shared symptoms that do not necessarily correlate well with functional or physiologic measures and have not been validated for applicability in PAH.

In this review, we present the available QoL tools for pulmonary artery hypertension and describe the need for more specific instruments that consider the physical and emotional implications of the diseases associated with PAH and the impact of various treatment options. We also discuss the impact of PAH on work ability and the need for provisions to address medical disability status and Social Security benefit status.

Section snippets

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

References (0)

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