Symptom Experience of Pulmonary Arterial Hypertension Patients

McDonough, Annette; Matura, Lea Ann; Carroll, Diane L.

doi:10.1177/1054773810391249

Cited by 44 publications

(45 citation statements)

References 23 publications

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“…These were ''coping with uncertainty'' and ''moving on with life'' in one study [16], and ''holding back'' and ''re-defining life'' in a second study [17]. Qualitative interviews conducted in view of the design of a pulmonary hypertension (PH)-specific tool to measure QoL identified the following PH-specific issues: socialising/interaction with others; being understood, accepted and valued; self-esteem; independence (financial and physical); and security [18].…”

Section: Introductionmentioning

confidence: 99%

Understanding the impact of pulmonary arterial hypertension on patients' and carers' lives

Guillevin¹,

Armstrong²,

Aldrighetti³

et al. 2013

European Respiratory Review

128

214

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Pulmonary arterial hypertension (PAH) is a rare, debilitating and rapidly progressive disease. Although there have been important medical advances in PAH management, the search for a cure continues. Despite an increased understanding of the disease, data on the wider effect of PAH on patients and carers, beyond the clinical symptoms, are still limited. In order to explore this, a large-scale international survey investigated four key areas affected by PAH (physical and practical, emotional, social, and information needs) and provides new insight into patients' and carers' experiences of living with the disease. The results from the survey highlight not only the limited ability of patients to carry out everyday tasks, but also the financial impact and social isolation experienced by both patients and carers. The study confirmed that a decline in a patient's World Health Organization functional class, which indicates an increase in clinical severity of the disease, is associated with greater limitations. Results from the survey demonstrate the need for multidisciplinary PAH management and a comprehensive standard of care to assess and improve all aspects of well-being for both patients and carers. In addition, they underline the need for updated PAH guidelines that address these needs. @ERSpublications PAH has a profound impact on numerous aspects of patients' and carers' lives http://ow.ly/pWWYf This article has supplementary material available from err.ersjournals.comFull survey results, along with a call to action, are available at www.phaeurope.org/projects-activities/pah-patient-andcarer-survey

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Section: Introductionmentioning

confidence: 99%

Understanding the impact of pulmonary arterial hypertension on patients' and carers' lives

Guillevin¹,

Armstrong²,

Aldrighetti³

et al. 2013

European Respiratory Review

128

214

View full text Add to dashboard Cite

show abstract

“…These symptoms are debilitating and force patients to readjust their lives. 3,4 Mortality is high in PAH, although with recent pharmacological advancements, survival has improved with 1-, 3-, and 5-year survival rates of 86%, 69%, and 61%, respectively. 5 Treatments are based on clinical findings including hemodynamics, symptoms, and functional class (FC).…”

mentioning

confidence: 99%

Predictors of Health-Related Quality of Life in Patients With Idiopathic Pulmonary Arterial Hypertension

Matura

McDonough

Carroll

2012

Journal of Hospice & Palliative Nursing

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Idiopathic pulmonary arterial hypertension is a debilitating disease that leads to right ventricular heart failure and death. There is limited research on how this disease affects patients. The purpose of this study was to describe differences in health status, health-related quality of life, and psychological states among functional classes along with predictors of health-related quality of life. A convenience sample of 104 participants with idiopathic pulmonary arterial hypertension was recruited. Participants completed a sociodemographic and clinical data form; Medical Outcomes Study Short-Form 36, US Cambridge Pulmonary Hypertension Outcome Review, and Profile of Mood States surveys. The mean age was 51.9 (SD, 16.6) years, and 81 (78%) were female. Patients with functional class IV had significantly worse self-reported physical health status, health-related quality of life, and psychological distress than the other functional classes. Predictors of health-related quality of life were role-emotional (Medical Outcomes Study Short-Form 36), symptoms and activity (US Cambridge Pulmonary Hypertension Outcome Review), depression (Profile of Mood States), employment, and diuretic and oxygen use, accounting for 86% of the variance. These factors need to be monitored and assessed with patients with idiopathic pulmonary arterial hypertension especially as functional class increases. Effective symptom management is necessary to reduce the negative impact on health-related quality of life. A case study is presented to illustrate these points. KEY WORDShealth status, idiopathic pulmonary arterial hypertension, quality of life P ulmonary hypertension (PH) is a debilitating disease causing progressive functional limitations that ultimately leads to right ventricular heart failure and death. There are five World Health Organization (WHO) classifications of PH. Group I is termed pulmonary arterial hypertension (PAH). Group II is PH associated with left ventricular heart disease. Group III is associated with pulmonary disease. Group IV is associated with chronic thromboembolic disease, and group V is for miscellaneous disorders. 1 Pulmonary arterial hypertension contains idiopathic pulmonary arterial hypertension (IPAH) along with familial PAH, autoimmune diseases, congenital heart disease, HIV, and sickle cell disease. Pulmonary arterial hypertension affects primarily women with a mean age at diagnosis of 50 years. 2 Clinically, patients experience a constellation of symptoms including shortness of breath, fatigue, chest pain, palpitations, and peripheral edema. These symptoms are debilitating and force patients to readjust their lives. 3,4 Mortality is high in PAH, although with recent pharmacological advancements, survival has improved with 1-, 3-, and 5-year survival rates of 86%, 69%, and 61%, respectively. 5 Treatments are based on clinical findings including hemodynamics, symptoms, and functional class (FC). WHO FC I are patients without symptoms with usual physical activity. Those with mild symptoms with ordinary activ...

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“…It has also been shown a switch of type I muscolar fibres towards type II muscolar fibres (prone to anaerobic metabolism) (23). In patients who have PAH, fatigue may be associated not only with primary factors, which are related to the disease process itself, but also to secondary factors such as sleep disturbance and depression (24). The interactions among these contributors to fatigue severity are confluent and difficult to isolate.…”

Section: Physiopathology Of Exercise Limitation In Pahmentioning

confidence: 99%

Exercise training for patients with pulmonary arterial hypertension

Confalonieri¹

2014

SOB

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ly activity, reducing dyspnoea and fatigue, and enhancing the quality of life and muscle strength. So, the final message from the available literature reports is a strong recommendation to adopt exercise training and cardiopulmonary rehabilitation programs for any patient suffering from exertional dyspnoea and fatigue. Many questions remain unanswered regarding the optimization of the exercise training and rehabilitation programs. Ongoing rehabilitative trials in patients with PAH will help answer some of the remaining questions.KEY WORDS: pulmonary arterial hypertension; dyspnoea; exercise limitation; exercise training; pulmonary rehabilitation. IntroductionPulmonary arterial hypertension (PAH) is a hemodynamic condition defined by a pre-capillary resting pulmonary artery pressure at or above 25 mm Hg mean value that can be the result of a variety of diseases of different causes, but similar clinical picture and virtually identical pathological changes of the distal pulmonary arteries (1, 2). During the Fifth World Symposium on pulmonary hypertension (PH) held in 2013 in Nice, the clinical classification of pulmonary hypertension (2) identified five groups of disorders according to pathological, pathophysiological and therapeutic characteristics, but the term PAH may only be applied to a subgroup of the group 1 including eight rare clinical conditions characterized by pre-capillary PH in absence of other causes of pre-capillary PH such as lung diseases, chronic pulmonary thromboembolism (CTEPH), or rare diseases related-PH with unclear multi-factorial mechanisms. Despite a number of specific drugs are available by now for PAH treatment, exertional dyspnoea is a common progressively disabling symptom, shared either by idiopathic and secondary PAH, still affecting well treated patients. Exercise training (ET), a major component of pulmonary rehabilitation programs, is an established treatment to enhance exercise limitation for patients with chronic obstructive pulmonary disease (COPD), systolic left heart failure, and a variety of chronic respiratory diseases (3 SummaryIn spite of evidence-based consensus recommendations on exercise training for patients with pulmonary arterial hypertension (PAH) its use outside specialized rehabilitation Centers has been accounted a handful of reports. Dyspnoea and fatigue are common symptoms reducing the exercise tolerance of subjects with PAH even when specific drug are correctly administered. Moreover, the mechanisms of exercise limitation in subjects with PAH are known and thandful of reports. There is evidence of impaired signaling between the periphery and central hemodynamic performance. Exercise training has recently been proven to be effective and safe to enhance physical performance in patients with PAH already treated with specific drug therapy. The rationale of training in PAH has been specifically proven also in experimental animal models of PAH. Moreover, repeated positive results in randomized controlled trials and observational studies in humans gave convincing ...

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Symptom Experience of Pulmonary Arterial Hypertension Patients

Cited by 44 publications

References 23 publications

Understanding the impact of pulmonary arterial hypertension on patients' and carers' lives

Understanding the impact of pulmonary arterial hypertension on patients' and carers' lives

Predictors of Health-Related Quality of Life in Patients With Idiopathic Pulmonary Arterial Hypertension

Exercise training for patients with pulmonary arterial hypertension

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