Symptom burden, quality of life, and attitudes toward palliative care in patients with pulmonary arterial hypertension: Results from a cross-sectional patient survey

doi:10.1016/j.healun.2012.08.010

The Journal of Heart and Lung Transplantation

Volume 31, Issue 10, October 2012, Pages 1102-1108

https://doi.org/10.1016/j.healun.2012.08.010 Get rights and content

Background

Pulmonary arterial hypertension (PAH) is a complex disease with variable clinical manifestations; nevertheless, morbidity and mortality associated with PAH are considerable. This study examined quality of life (QOL) in PAH patients and assessed use of palliative care (PC) for addressing QOL issues and what barriers might exist regarding early PC implementation for patients with PAH.

Methods

An Internet-based survey was distributed to Pulmonary Hypertension Association patient-related listservs. Symptom burden and QOL were assessed using Linear Analog Self Assessment (LASA) QOL items and the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR).

Results

Of 774 eligible patients with active e-mail addresses, 315 returned surveys (41% overall response), and 276 (88%) contained analyzable responses. Responders (mean age, 48.9 years ± 16.0) were predominantly white (85%), female (86%), and with idiopathic PAH (42%). Profound deficiency in overall QOL (40%), fatigue (57%), physical well-being (56%), social activity (49%), emotional well-being (49%), and pain (38%) were reported. Most patients believed their PAH physician had excellent understanding of PAH progression/plan of care (92%), but less were satisfied with care regarding QOL management (77%). Few patients considered PC (8%), or had pain management (4%) or PC involved (1%). Most common reasons were beliefs that patients were doing well/not sick (63%) or that PC had not been suggested (22%).

Conclusions

PAH may result in symptoms or QOL impairment persisting despite optimal PAH therapy. However, PC awareness or use by PAH patients and providers is low. Opportunities may exist to integrate PC into care for PAH patients.

Section snippets

Methods

With permission from the Pulmonary Hypertension Association and the approval of the Mayo Clinic Institutional Review Board, we contacted members of 4 Pulmonary Hypertension Association-maintained listservs via e-mail. Patients, caregivers, and medical providers who subscribed to these listservs were invited to participate in an Internet-based survey. Separate surveys were created for each group; the analysis contained in this report focuses on the survey of patients with PAH. If patients were

Respondent characteristics

Of 774 patients and caregivers with active e-mail addresses registered with the Pulmonary Hypertension Association, 315 returned completed surveys (41% overall response). Of these 315 responders, 276 (88%) contained eligible unique patient-specific responses that could be analyzed. Those that were disqualified only completed caregiver information and did not complete patient-related outcomes. Of the responders, 92.7% were from 45 of 50 states in the United States, with the remaining 7.3%

Discussion

The findings of this study are concordant with previous studies11, 12, 13, 14, 15, 16 that demonstrated patients with PAH have a profound and multifactorial symptom burden that affects QOL and may persist even with optimal PAH therapy. Despite these facts, PC use is low in patients with PAH, and several misperceptions of PC exist.

The barriers to PC access may partly explain the low use of PC in PAH despite the potential for benefits. PC is a field that continues to evolve and define itself, and

Disclosure statement

This study was funded with internal, institutional funding from the Mayo Clinic, Department of Medicine.

The initial abstract and poster were presented at Pulmonary Hypertension Association's Ninth International Pulmonary Hypertension Conference and Scientific Sessions, Garden Grove, California, June 24–27, 2010, and in an oral presentation at International Society of Heart and Lung Transplantation, Prague, Czech Republic, April 18, 2012.

R.P.F. and M.D.M. have done consulting work for the

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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
Physicians who specialize in pulmonary arterial hypertension (PAH) care for patients facing a serious, life-limiting illness. Palliative care is underutilized in patients with PAH, and little is known about how best to provide palliative care to this patient population.
Using a qualitative approach, assess physicians’ perspectives on barriers and facilitators to the use of palliative care in PAH.
Participants were board-certified pulmonologists and cardiologists recruited from the Pulmonary Hypertension Association's list of physician specialists and academic center websites. We performed one-on-one semi-structured interviews that were recorded, transcribed, and analyzed using thematic analysis.
Twelve physicians participated in the study, with a median age of 48.5 years and 20.5 years of clinical experience caring for patients with PAH. We identified the following themes and associated barriers and facilitators to effective implementation of palliative care for patients with PAH: a tailored approach to the individual patient; a PAH-specialist-led culture of care; effective collaboration with palliative care clinicians; and limitations imposed by health systems.
PAH physicians are open to palliative care for their patients and are willing to partner with palliative care clinicians to implement this effectively and in the right setting. Areas for targeted improvement in enhancing palliative care for patients with PAH exist, especially enhancing collaboration between PAH physicians and palliative care specialists and navigating barriers in health systems.
Palliative care referrals in patients with pulmonary arterial hypertension: The Pulmonary Hypertension Association Registry
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In the only comprehensive review of palliative care and PAH-related hospital admissions across the United States to date, 2.2% of 30,495 admissions from 2001 to 2017 involved inpatient palliative care consultation, although the rate did increase over time [18]. Furthermore, in two patient and physician surveys on palliative care in PAH, while 72% of physicians responded that they had referred to palliative care at least once in the past, only 8% of patients had considered palliative care [19,20]. To our knowledge, no study has examined the rate of outpatient palliative care consultation in PAH.
Pulmonary arterial hypertension (PAH) is a life limiting disease with substantial symptom burden and healthcare utilization. Palliative care alleviates physical and emotional symptoms for patients with serious illness, and has been underutilized for these patients.
To characterize patients with PAH referred to palliative care and identify predictors of referral.
We conducted an observational study of adult patients enrolled in the Pulmonary Hypertension Association Registry from January 2015 through June 2021, performing descriptive statistics on patient characteristics at baseline for all patients and the subset referred to palliative care. These characteristics were modeled in a backwards elimination Cox regression with time to referral to palliative care as the primary outcome.
92 of 1,578 patients were referred to palliative care (5.8%); 43% were referred at their last visit prior to death. Referrals were associated with increasing age per decade (hazard ratio 1.35 [95% confidence interval 1.16–1.58]), lower body mass index (hazard ratio 0.97 [95% confidence interval 0.94–0.998]), supplemental oxygen use (hazard ratio 2.01 [95% confidence interval 1.28–3.16]), parenteral prostanoid use (hazard ratio 2.88 [95% confidence interval 1.84–4.51]), and worse quality of life, measured via lower physical (hazard ratio 0.97 [95% confidence interval 0.95–0.99]) and mental (hazard ratio 0.98 [95% confidence interval 0.96–0.995]) scores on the 12-item Short Form Health Survey.
Patients with PAH are infrequently referred to palliative care, even at centers of excellence. Referrals occur in sicker patients with lower quality of life scores, often close to the end of life.
A Path Forward for Palliative Care in Pulmonary Arterial Hypertension
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Pulmonary arterial hypertension (PAH) is a progressive disease associated with significant morbidity and mortality. Despite the negative impact of PAH on quality of life and survival, data on use of specialty palliative care services (PCS) is scarce.
We sought to evaluate the inpatient use of PCS in patients with PAH.
Using the National (Nationwide) Inpatient Sample, 30,495 admissions with a primary diagnosis of PAH were identified from 2001 through 2017. The primary outcome of interest was temporal trends and predictors of inpatient PCS use in patients with PAH.
The inpatient use of PCS was low (2.2%), but increased during the study period from 0.5% in 2001 to 7.6% in 2017, with a significant increase starting in 2009. White race, private insurance, higher socioeconomic status, hospital-specific factors, higher comorbidity burden (Charlson Comorbidity Index), cardiac and noncardiac organ failure, and use of extracorporeal membrane oxygenation and noninvasive mechanical ventilation were independent predictors of increased PCS use. PCS use was associated with a higher prevalence of do-not-resuscitate status, a longer length of stay, higher hospitalization costs, and increased in-hospital mortality with less frequent discharges to home, likely because these patients were also sicker (higher comorbidity index and illness acuity).
The inpatient use of PCS in patients with PAH is low, but has been increasing over recent years. Despite increased PCS use over time, patient- and hospital-specific disparities in PCS use continue. Further studies evaluating these disparities and the role of PCS in the comprehensive care of PAH patients are warranted.
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On the individual level, the Task Force experts promote emphasis on narrative medicine where patients are able to share their concerns and struggles and healthcare providers can be aware and involved in the full perspective of the patients so they can provide the best holistic management. One area of significant potential improvement is access to palliative care as surveys consistently show low rates of awareness and access to palliative care in patients who have low HRQoL and significant disease burden [49]. On a patient population level, there is great importance to support groups, patient associations, and structured resources for patient education and public awareness [14].
Pulmonary arterial hypertension is a devastating progressive disease mediated by different pathophysiologic pathways that result in progressive increase in pulmonary vascular resistance along with right ventricular failure and eventually premature death. Despite significant advances in the understanding of the underlying mechanisms and development of a number of targeted therapies, pulmonary arterial hypertension remains a challenging condition with high morbidity and mortality. New therapies are being actively sought, and early recognition remains of paramount importance. In an effort to improve the detection and management of pulmonary hypertension, the 6th World Symposium on Pulmonary Hypertension came up with most recent statements in 2018. The goal of this review is to summarize some key updates from the proceedings of the Symposium pertaining to different aspects of evaluation and management of patients with pulmonary arterial hypertension.

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Original clinical scienceSymptom burden, quality of life, and attitudes toward palliative care in patients with pulmonary arterial hypertension: Results from a cross-sectional patient survey

Background

Methods

Results

Conclusions

Section snippets

Methods

Respondent characteristics

Discussion

Disclosure statement

J Card Fail

Mayo Clin Proc

Chest

Chest

Chest

Respir Med

Psychosomatics

J Heart Lung Transplant

J Heart Lung Transplant

J Pain Symptom Manage

Mayo Clin Proc

Leuk Res

The Seattle Heart Failure Model: prediction of survival in heart failure

Circulation

Estimating prognosis in heart failure: time for a better approach

Heart

Discordance between patient-predicted and model-predicted life expectancy among ambulatory patients with heart failure

JAMA

Palliative care in heart failure: a position statement from the palliative care workshop of the Heart Failure Association of the European Society of Cardiology

Eur J Heart Fail

Palliative care for patients with heart failure

JAMA

Original clinical science
Symptom burden, quality of life, and attitudes toward palliative care in patients with pulmonary arterial hypertension: Results from a cross-sectional patient survey