Original Article
Symptom Prevalence, Symptom Severity, and Health-Related Quality of Life Among Young, Middle, and Older Adults With Pulmonary Arterial Hypertension
American Journal of Hospice & Palliative Medicine® 2016, Vol. 33(3) 214-221 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/1049909114554079 ajhpm.sagepub.com
Lea Ann Matura, PhD, RN1, Annette McDonough, PhD, RN2, and Diane L. Carroll, PhD, RN, FAAN3
Abstract Pulmonary arterial hypertension (PAH) is a chronic, life threatening illness that affects primarily women. The purpose of this study was to describe the prevalence of PAH symptoms and to determine whether there are differences in symptom severity and HRQOL in PAH symptoms among young, middle, and older adults with PAH. A cross sectional design was utilized. For all the age groups, shortness of breath (SOB) on exertion and fatigue were the two most prevalent symptoms. SOB on exertion had the highest symptom severity scores followed by fatigue for all groups. Symptom severity was significantly different among the groups for palpitations, abdominal swelling and nausea. For components of HRQOL, physical functioning worsened with age. All groups had diminished general health, role physical and vitality levels. There are some differences in symptom prevalence, symptom severity and HRQOL among young, middle and older adults. Awareness of these differences is important for healthcare providers to know and assess overtime. Palliative care should be an integral part of caring for patients with PAH. Keywords symptoms, quality of life, pulmonary, hypertension, aging, severity
Introduction Pulmonary arterial hypertension (PAH) is a chronic illness caused by elevated pulmonary artery pressures, which leads to increased pulmonary vascular resistance and ultimately right heart failure. Pulmonary arterial hypertension is predominantly diagnosed in women (80%).1 Pulmonary arterial hypertension affects all races yet death is higher in African Americans and Asians.2 Epidemiology studies estimate PAH at 7.6 cases per million and a prevalence rate of 26 cases per million.3 The average time from the onset of symptoms to diagnosis is approximately 2 years resulting in increased disease severity at diagnosis. Mortality is high, but advances in treatment have increased survival rates from 1-year survival of 68% in 1980s to 97% today.4 There are several etiologies of PAH with approximately 50% being idiopathic PAH. Other associated forms of PAH are related to connective tissue disease, HIV, portopulmonary hypertension, congenital heart disease, and drugs (eg, anorexigens).5 Medical regimens can be complex for some patients including prostanoid analog infusions either continuously or subcutaneously.1 Some common symptoms reported by people with PAH are dyspnea on exertion, fatigue, and sleep disturbance.6-8 These symptoms can be severe and negatively impact health-related
quality of life (HRQOL).7,9-11 However, we do not know whether there are differences in symptoms and HRQOL among different age-groups of patients with PAH. As survival increases, the need to understand how PAH affects people at different stages of their life is necessary. Other disorders have also demonstrated differences between younger and older adults with regard to symptom severity and symptom perception. Older patients with esophageal reflux reported less heartburn and acid reflux severity than younger patients although older adults had increased acid exposure and mucosal injury.12 Depressive symptoms among adulthood demonstrated that older adults also experienced an increase in distress with age.13 Furthermore, in a cohort of patients
1
School of Nursing, University of Pennsylvania, Philadelphia , PA, USA Massachusetts General Hospital, University of Massachusetts, Lowell, MA, USA 3 Munn Center for Nursing Research, Institute for Patient Care, Boston, MA, USA 2
Corresponding Author: Lea Ann Matura, PhD, RN, School of Nursing, University of Pennsylvania, Claire M. Fagin Hall, 418 Curie Blvd, Room 322, Philadelphia, PA 19104, USA. Email: [email protected]
Matura et al receiving palliative care, the oldest adults were less likely to report pain, anxiety, and nausea than younger patients; however, there were no differences between the age-groups for dyspnea and depression levels.14 Symptom recognition in heart failure shows older patients have more difficulty detecting and interpreting dyspnea than younger patients.15 Older patients undergoing a 6-minute walk test (6MWT) were twice as likely to report differing levels of dyspnea than the research assistants assessing them during the 6MWT. Differing levels of symptom perception as patients age may be a key component for patients’ ability to perform selfcare and impact hospital readmissions.16 Symptoms such as dyspnea and fatigue were present for 5 to 7 days before seeking heart failure treatment. Older patients did not often attribute increasing symptom severity with the need to seek medical care. Understanding differences in symptom experiences among the age-groups is important in order to formulate a treatment plan and teach patients how to interpret their symptom experiences and when to seek medical treatment. Chronic illness in the older adult can impact their ability to perform activities of daily living and negatively impact HRQOL.17 Along with difficulties with activities of daily living, depression and problems with memory are associated with HRQOL in older adults.18 The ability to recognize symptoms is necessary in order for older patients with heart failure to perform self-care and the confidence older patients have to perform self-care is also associated with HRQOL.19 Older adults with impaired ability to perform activities of daily living are strong predictors of disability and negatively affect HRQOL.20 Understanding how symptoms and HRQOL differ as patient’s age is important in order to inform care. To date, there has not been a study investigating if there are differences in symptom prevalence and severity and HRQOL among different age-groups of patients with PAH. The purpose of this study was to describe the prevalence of PAH symptoms and to determine whether there are differences in symptom severity and HRQOL in PAH symptoms among young, middle, and older adults with PAH.
Methods Design and Participants This was a cross-sectional study that included a convenient sample of patients with PAH. Inclusion criteria included adults aged 18 and older who were able to read and speak English. All participants self-identified as having a PAH diagnosis. The PAH diagnoses included idiopathic, heritable, along with associated PAH (connective tissue disease, HIV, portopulmonary hypertension, congenital heart disease, and drug use such as anorexigens). Participants were recruited and enrolled over a 2-year period (May 2009-May 2011). Two hundred and seventy-six participants were recruited. One hundred and ninety-one participants returned completed surveys, resulting in a 69% response rate. Young adults were defined as those 18 to 40 years old, middle adults were those 41 to 64 years old, and those 65 and older were considered older adults.
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Procedures This study was approved by the university and hospital institutional review boards before recruitment of participants began. Participants were recruited from several sites including a PAH clinic, PAH support groups in the Northeast, the Pulmonary Hypertension Association (PHA) Web site, and local and international PAH conferences. Potential participants were approached by the advanced practice nurse in the PAH clinics to introduce the study. Interested participants were given the principal investigator’s (PI) contact information. At the monthly PAH support group meetings and the conferences, the PI was introduced. Interested participants would approach the PI if they were interested in participating. The administrator for the PHA Web site posted an advertisement for the study. Interested participants contacted the PI for more details regarding the study. Participants either completed the questionnaires and returned to the PI at the conferences and PAH support group meetings or they mailed the questionnaires back to the PI via the self-addressed, stamped envelopes that were provided. All participants completed a sociodemographic and clinical data form, the Pulmonary Arterial Hypertension Symptom Scale (PAHSS) and the Medical Outcomes Study Short Form36 (SF-36).
Measures Sociodemographic and Clinical Data Form. Variables were collected by self-report and included age, gender, race/ethnicity, educational level, occupation, employment status along with date of PAH diagnosis, PAH etiology, oxygen use, and medications. The PI (LAM) determined the PAH World Health Organization (WHO) functional class based on the self-reported symptoms.1 World Health Organization functional class I are those with PAH who do not have any symptoms of dyspnea, fatigue, or syncope with physical activity. World Health Organization functional class II are those who are not symptomatic with usual activities while those with WHO functional class III have symptoms with less than usual activity but they are comfortable at rest. World Health Organization functional class IV are those patients with PAH who have symptoms at rest.1
Pulmonary Arterial Hypertension Symptom Scale The PAHSS is an investigator-developed symptom severity scale that assess 17 PAH symptoms.8 Participants rate the severity of their symptoms over the past month. The rating scale is 0 to 10 with 0 representing ‘‘absence’’ of the symptom and 10 indicating ‘‘extremely intense.’’ Symptoms included on the measure are shortness of breath (SOB) with exertion, SOB lying down, SOB at rest, awakening at night SOB, fatigue, difficulty sleeping, chest pain, abdominal swelling, swelling of ankles and feet, syncope, palpitations, dizziness, cough, nausea, loss of appetite, and hoarseness Raynaud’s phenomenon (cold, numbness of extremities, etc). Initial psychometrics have been established with good validity and reliability.8
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216 Table 1. Sociodemographic and Clinical Variables Among Age-Groups. Variable (N ¼ 191) Mean + SD age, yearsa Gender: female Ethnicity: caucasiana Marital status: marrieda Living arrangements with family/friends Education: college graduate Employment statusa Full-time Part-time Retired Disabled PAH etiology Idiopathic Connective tissue disease Congenital heart disease Drugs (fenfluramine/phentermine) Functional class I II III IV Oxygen usea Years since diagnosis Medications Calcium channel blockers Endothelin receptor Antagonists phosphodiesterase type-5 inhibitors (PDE-5) Prostanoid analogs Diuretics Digoxin
Total (N ¼ 191)
Young adults (n ¼ 39)
Middle adults (n ¼ 101)
Older adults (n ¼ 51)
53.2 + 15.1 162 (85%) 168 (88%) 97 (51%) 141 (74%) 113 (59%)
30.2 + 6.4 33 (85%) 30 (77%) 11 (28%) 28 (72%) 26 (67%)
53.3 + 6.4 89 (88%) 89 (88%) 51 (51%) 75 (74%) 65 (64%)
71.0 41 49 35 38 22
+ 4.2 (80%) (96%) (69%) (75%) (43%)
36 (19%) 21 (11%) 43 (23%) 77 (43%)
13 (33%) 7 (18%) 0 (0%) 13 (33%)
21 (21%) 8 (8%) 7 (7%) 59 (58%)
2 6 36 5
(4%) (12%) (71%) (10%)
104 (55%) 34 (18%) 8 (4%) 8 (4%)
25 (64%) 5 (13%) 0 (0%) 1 (3%)
52 (52%) 20 (20%) 6 (6%) 1 (1%)
27 9 2 6
(53%) (18%) (4%) (12%)
22 (12%) 36 (19%) 54 (28%) 79 (41%) 115 (60%) 6.3 + 6.6
7 (18%) 9 (23%) 9 (23%) 14 (36%) 13 (33%) 5.3 + 6.7
12 (12%) 18 (18%) 28 (28%) 43 (43%) 67 (67%) 5.6 + 6.2
3 9 17 22 35 6.2
(6%) (18%) (33%) (43%) (69%) + 7.9
46 (24%) 95 (50%) 110 (58%) 75 (39%) 117 (61%) 29 (15%)
5 (13%) 20 (51%) 27 (69%) 19 (49%) 20 (51%) 10 (26%)
25 (25%) 53 (53%) 54 (54%) 41 (41%) 69 (69%) 13 (13%)
16 22 29 15 28 6
(31%) (43%) (57%) (29%) (55%) (12%)
Abbreviations: PAH, pulmonary arterial hypertension; SD, standard deviation. a P < .05.
Medical Outcomes Study Short Form-36 The SF-36 is a widely used measure of generic HRQOL. It contains both physical and mental components.21 There are 8 subscales: physical functioning, role physical, bodily pain, general health, vitality, social functioning, role emotional, and mental health. These subscales are combined to form composite physical and mental summary scores. Scores range from 0 to 100 with higher scores indicating better HRQOL. Psychometric properties have been established.22,23
Data Analysis Sociodemographic and clinical variables were analyzed using descriptive statistics. Frequencies and percentages were used for categorical variables; means and standard deviations were used to characterize continuous variables. Based on distributional property analysis, normalizing transformations were not necessary to perform. Outliers were assessed by visual inspection of distributions. Comparisons among the age-groups for continuous variables were performed using 1-way analysis of variance (ANOVA) models. Chi-square statistics were used for
comparisons among categorical variables. The 17 PAH symptoms and HRQOL are compared between the 3 age-groups (ie, young, middle, and older) using 1-way ANOVA models and the F statistic with post hoc pair-wise comparisons analyzed using the Dunnett’s tests. No adjustments were made for multiplicity. All analyses were performed using SPSS version 21.24 Statistical significance was set at .05 level.
Results The total sample included 191 participants. The mean age was 53.2 + 15.1 years. The majority of the sample was female (85%) and caucasian (88%). Forty-three percent of the sample reported that they were disabled. Over half of the sample was idiopathic PAH, 41% were WHO functional class IV, and 60% were using oxygen. The mean age since diagnosis was 6.3 + 6.6 years. Over half of the population were taking endothelin receptor antagonists, phosphodiesterase type-5 inhibitors, and diuretics. Among the age-groups, 20% were classified as young; 53% were middle; and 27% were older adults. The groups significantly differed among ethnicity, employment status, and oxygen use, as shown in Table 1.
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Table 2. Symptom Severity Among Age-Groups. Symptom intensity (0-10), mean + SD SOB with exertion Fatigue Difficulty sleeping Swelling of ankles/feet Fast heart beat/palpitationsa Raynaud’s phenomenon Dizzy/lightheaded Abdominal swellingb Cough Chest pain/discomfort Loss of appetite SOB at rest SOB while lying down Nauseaa Hoarseness Awaken at night SOB Passing out
Total (N ¼ 191) 6.2 + 5.0 + 3.2 + 2.9 + 2.9 + 2.7 + 2.6 + 2.3 + 2.3 + 2.2 + 1.9 + 1.8 + 1.7 + 1.5 + 1.5 + 0.9 + 0.4 +
Young adults (n ¼ 39)
2.7 2.5 3.4 3.1 2.6 3.4 2.5 3.2 2.6 2.4 2.5 2.3 2.2 2.4 2.3 1.8 1.5
5.6 4.8 3.4 2.3 3.6 1.7 2.9 2.2 2.5 2.9 2.5 1.9 1.7 2.5 1.2 1.1 0.9
+ 2.7 + 3.0 + 3.7 + 3.0 + 2.8 + 2.8 + 3.0 + 3.0 + 3.0 + 2.7 + 2.9 + 2.4 + 2.3 + 3.3 + 2.2 + 1.9 + 2.3
Middle adults (n ¼ 101) 6.3 5.2 3.4 3.2 3.0 3.1 2.7 2.9 2.1 2.1 1.6 1.8 1.9 1.4 1.6 1.0 0.3
+ 2.9 + 2.3 + 3.4 + 3.0 + 2.6 + 3.5 + 2.4 + 3.4 + 2.4 + 2.3 + 2.3 + 2.4 + 2.4 + 2.2 + 2.3 + 1.8 + 1.3
Older adults (n ¼ 51) 6.6 + 4.7 + 2.7 + 2.6 + 2.1 + 2.9 + 2.2 + 1.1 + 2.6 + 1.6 + 1.9 + 1.6 + 1.2 + 0.8 + 1.7 + 0.5 + 0.3 +
2.5 2.5 3.1 3.1 2.4 3.4 2.3 2.6 2.7 2.2 2.5 2.0 1.8 1.5 2.3 1.5 1.1
Abbreviations: SOB, shortness of breath; SD, standard deviation. a Significant differences between young and older adults at P < .05. b Significant differences between middle and older adults at P < .05.
Table 3. Health-Related Quality of Life Among Age-Groups. Subscale PAH (mean + SD) General health Physical functiona,b Role physical Pain Vitality Social function Mental health Role emotional Composite summary mental health Composite summary physical health
Total (N ¼ 191) 37.1 + 25.9 41.5 + 25.9 36.4 + 40.3 66.4 + 27.6 41.3 + 22.7 65.5 + 26.4 71.9 + 19.3 67.0 + 41.5 61.5 + 21.0 45.4 + 22.7
Young adults (n ¼ 39) 45.0 + 52.1 + 45.5 + 67.9 + 47.8 + 70.9 + 72.3 + 71.8 + 65.7 + 52.6 +
25.1 24.1 44.7 25.7 23.4 24.4 16.0 37.1 19.5 25.6
Middle adults (n ¼ 101) 34.7 + 19.6 40.7 + 24.6 35.7 + 40.0 65.7 + 28.1 39.1 + 21.8 64.3 + 26.6 72.4 + 20.2 64.0 + 43.4 60.0 + 21.1 44.2 + 21.5
Older adults (n ¼ 51) 35.8 + 35.0 + 30.9 + 66.3 + 40.4 + 63.7 + 70.7 + 69.3 + 61.0 + 42.0 +
20.1 27.5 37.3 27.9 23.5 27.5 20.0 41.0 21.7 21.6
Abbreviations: PAH, pulmonary arterial hypertension; SD, standard deviation. a Significant differences between young and middle adults at P < .05. b Significant differences between young and older adults at P < .05.
For the total sample, the most prevalent of the 17 PAH symptoms were SOB on exertion (98%), fatigue (95%), and palpitations (78%). The highest mean symptom scores included SOB on exertion (6.2 + 2.7), fatigue (5.0 + 2.5), and difficulty sleeping (3.2 + 3.4), as shown in Table 2. The lowest HRQOL measures represented by the SF-36 subscales for the total sample included general health (37.1 + 25.9), physical function (41.5 + 25.9), role physical (36.4 + 40.3), and vitality (41.3 + 22.7) along with the SF-36 composite summary physical health (45.4 + 22.7), as shown in Table 3. Among the age-groups, there were significant differences between the young and middle adult groups and the young and older adult groups for the HRQOL scores on the SF-36 physical function subscale. Among the age-groups, there were significant differences between the young and older adult mean scores for palpations and nausea. There were also differences between the middle
and older adult mean scores for abdominal swelling. The most prevalent symptoms for the young adult group (Figure 1) were similar to the total sample: SOB on exertion (100%), fatigue (92%), and palpitations (87%). The highest mean severity scores for the young adult group included SOB on exertion (5.6 + 2.7), fatigue (4.8 + 3.0), and palpitations (3.6 + 2.8). The most prevalent symptoms for the middle adult group included SOB with exertion (98%), palpitations (82%), and dizziness (77%; Figure 2). The highest mean scores for the middle adult group included SOB on exertion (6.3 + 2.9), fatigue (5.2 + 2.3), and difficulty sleeping (3.4 + 3.4). The most prevalent symptoms for the older adult group included SOB with exertion (98%), fatigue (92%), and cough (68%; Figure 3. The highest mean scores for the old adult group included SOB on exertion (6.6 + 2.5), fatigue (4.7 + 2.5), and Raynaud’s phenomenon (2.9 + 3.4).
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Discussion
Figure 1. Pulmonary arterial hypertension (PAH) symptom prevalence for young adults.
Figure 2. Pulmonary arterial hypertension (PAH) symptom prevalence for middle adults.
Figure 3. Pulmonary arterial hypertension (PAH) symptom prevalence for older adults.
This is the first study to describe the prevalence and differences in PAH symptoms and HRQOL among young, middle, and older adults with PAH. These results show similarities and differences in the symptom experience and HRQOL among different age-groups in patients with PAH. More of the older adults use oxygen compared to the other age-groups. Shortness of breath on exertion and fatigue were the 2 most prevalent symptoms for all age-groups. The next most prevalent symptom for the young adult group was palpitations in comparison to difficulty sleeping for the middle adults and cough for the older adults. Physical functioning was significantly different among the groups with the older adult group having the worst physical functioning. Comparison of symptom prevalence and severity scores in other disorders reveals similar and contrasting patterns. In a study of patients with chronic obstructive pulmonary disease (COPD), all patients reported breathlessness25 similar to the findings of this study where 98% to 100% of the patients among the different PAH age-groups reported SOB on exertion. This study reported a diverse prevalence of 70% to 92% for fatigue in comparison to another study of patients with COPD who reported 72% with fatigue.25 Of the 21 studies included in a recent review of symptom prevalence and severity in people receiving active cancer treatment, only 44% of patients reported difficulty breathing/dyspnea with a mean severity score of 2.8 (based on 0-10 scale).26 In contrast, among the age-groups in this study 98% to 100% reported SOB on exertion with a mean symptom severity score ranging from 5.6 to 6.6. Oxygen use also increased incrementally with age as did the means SOB with exertion scores possibly indicating worsening disease with age. These differences among studies may not be unexpected due to the nature of PAH involving the heart and lungs and the Reilly et al. review including only 20% of patients with lung cancer who may experience increased levels of SOB. Compared to the Bentsen et al.’s review, results of this study showed a much higher prevalence of fatigue among the agegroups (70%-92%) and slightly higher mean severity scores (4.7%-5.2%). The review revealed that generalized fatigue was reported in 60% of the patients with a mean severity score of 4.6 (based on 0-10 scale).26 Another study investigated whether fatigue levels increased in age by comparing the US general population and a sample of patients with cancer.27 Those with cancer reported more severe fatigue than the general population and there was increased fatigue with age in the cancer group similar to the findings of this study. In another study, women living with chronic illness reported fatigue as a barrier to performing activities of daily living and impaired their HRQOL.28 Similarly, a study investigating symptoms and HRQOL in heart failure found the most prevalent symptom was lack of energy (66%) while SOB was reported by 56%.29 The pathophysiology of PAH resulting in impairment in cardiopulmonary function and right heart failure may contribute to a higher proportion of patients with PAH experience fatigue.1
Matura et al This study had 77% of the middle-aged group report dizziness with a mean severity score of 2.7 while the review of people receiving cancer treatment a smaller proportion (26%) reporting dizziness, the mean score was much higher (4.7). Dizziness could be treatment related in both PAH and cancer or in the case of PAH it may be related to hemodynamic changes where blood is not effectively pumped from the impaired right heart to the left heart and the rest of the body. Palpitations were prevalent in the young (87%) and middle (82%) adult group. Palpitations are abnormal sensations of irregular, rapid, or pulsations in the chest30 and are common symptoms reported in outpatient settings and emergency departments (EDs).31 In a survey of people seeking care at EDs for palpitations (N ¼ 684 000), 22% were young adults and 34% were middle adults with over half being female (61%). Thirty-eight percent of those with palpitations were attributed to cardiac disease. In this study, palpitations were the third highest mean score (3.6) for the middle adult group. Although the severity is in the mild range, it is prevalent symptom for this group. Assessing the presence and the severity may be important to prevent ED visits or possible hospital admissions. Difficulty sleeping had the highest mean symptom severity score (3.4) for the middle adult group. On the PAHSS, sleep is assessed by asking patients to rate their ‘‘difficulty sleeping’’ over the past month on a 0 to 10 scale. Patients who respond that they are having difficulty sleeping may need further sleep assessments including self-report questionnaires (eg, Pittsburgh Sleep Quality Index),32 actigraphy,33 or polysomnography.34 While the mean score is in the mild range, sleep is an important symptom to assess. Sleep disturbance in PAH includes insomnia, excessive daytime sleepiness,35 and restless leg syndrome.36 Poor sleep quality is associated with depression, dyspnea, and poor quality of life.35 Cough was another prevalent symptom (68%) in the older adult group with a mean score of 2.6 in comparison to 52% with a mean score of 5.1 in patients receiving cancer treatment. Cough may be related to the tumor involvement in lung cancer or metastatic lung disease or possibly a comorbidity such as COPD or gastroesophageal reflux disease.37 In PAH, there may be dilation of the pulmonary vessels causing cough.38 Although only 12% of the total sample reported syncope of which 10% were older adults, this is an important symptom to assess in patients with PAH. In PAH, syncope is a poor prognostic sign39 as there is a risk for injuries from falls especially with the older adults.40 The SF-36 measured HRQOL in this study. For the US normative data, scores higher than 50 are considered above average or signifying better HRQOL.23,41 Components of HRQOL were diminished in this study for the middle and older adult groups (general health, physical function, role physical, vitality, and the composite summary physical health). The younger adult group had similar HRQOL except for the physical functioning subscale which was slightly above the US normative average indicating slightly better physical function. HIV is an associated etiology for PAH. HRQOL in HIV was associated with the increased number of chronic illnesses42
219 making it necessary for health care providers to be aware of those people who develop PAH from their HIV disease that their HRQOL may be further impacted and needs to be monitored. Another study investigating HRQOL in chronic illnesses determined that HRQOL was most impaired by those with stroke and least by those with cancer compared to those with no current acute or chronic illness.43 Multiple chronic illnesses were associated with greater HRQOL impairments. Healthrelated quality of life in heart failure was moderately compromised.29 Health-related quality of life was strongly associated with symptom distress, comorbidities, female sex, and functional impairment demonstrating the need to assess symptoms and HRQOL in PAH. In relation to physical functioning, 43% of the total sample reported that they were disabled. Of those reporting disability, 33% were young adults and 58% were middle-aged adults. This is important for health care providers to understand in order to mitigate/manage symptoms and enhance functional status. Not only is physical functioning related to prognosis44 it can also impact HRQOL. As new treatments, both pharmacological and nonpharmacological, continue to emerge, mortality may continue to decrease increasing the older adult population with PAH. Assessing symptoms that are prevalent, severe, and impair HRQOL is needed. There are several limitations to this study. First, a nonrandom sample was recruited and enrolled in this study. Second, all participants self-reported their PAH diagnosis and PAH etiology. There was no confirmation of their diagnosis with medical records. Third, there may be recall bias with selfreport measures. Furthermore, a more robust sample including more men and additional ethnicities is warranted. We did not collect other clinical variables which may contribute to the understanding of symptoms in PAH and physical functioning such as hemodynamic parameters and the 6MWT. Longitudinal studies are needed to understand how symptoms and HRQOL change over time as patients age. In summary, this study adds to the literature on the similarities and differences in the symptom prevalence, severity, and HRQOL among age-groups in patients with PAH. For all the age-groups, SOB on exertion and fatigue were the 2 most prevalent symptoms. Palpitations were also prevalent in the young adult group; difficulty sleeping was common in the middle adult group; and cough was prevalent in the older adult group. Shortness of breath on exertion had the highest symptom severity scores followed by fatigue for all groups. Symptoms that were significantly different among the groups were palpitations, abdominal swelling, and nausea. Of those symptoms that were significantly different among the age-groups, the older adult group had the lowest severity means. For components of HRQOL, physical functioning worsened with age. All groups had diminished general health, role physical, and vitality levels. Palliative care is an integral part of caring for patients with PAH and timely referrals to the palliative care team are essential to optimize symptom management. Clinicians caring for patients with PAH need to understand the
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220 similarities and differences in symptoms as patients age and the need to employ palliative care throughout the disease trajectory and life span.
13.
Declaration of Conflicting Interests The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
14.
Funding This work was partially supported by a Bouve College of Health Sciences, Northeastern University intramural grant and Sigma Theta Tau International Gamma Epsilon grant.
15. 16.
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and young patients with gastro-oesophageal reflux. Age Ageing. 2000;29(2):125-130. Sutin AR, Terracciano A, Milaneschi Y, An Y, Ferrucci L, Zonderman AB. The trajectory of depressive symptoms across the adult life span. JAMA Psychiatry. 2013;70(8):803-811. Olden AM, Holloway R, Ladwig S, Quill TE, van Wijngaarden E. Palliative care needs and symptom patterns of hospitalized elders referred for consultation. J Pain Symptom Manag. 2011;42(3): 410-418. Riegel B, Dickson VV, Cameron J, et al. Symptom recognition in elders with heart failure. J Nurs Scholarsh. 2010;42(1):92-100. Jurgens CY, Hoke L, Byrnes J, Riegel B. Why do elders delay responding to heart failure symptoms? Nurs Res. 2009;58(4): 274-282. Barile JP, Thompson WW, Zack MM, Krahn GL, Horner-Johnson W, Haffer SC. Activities of daily living, chronic medical conditions, and health-related quality of life in older adults. J Ambul Care Manag. 2012;35(4):292-303. Baernholdt M, Hinton I, Yan G, Rose K, Mattos M. Factors associated with quality of life in older adults in the United States. Qual Life Res. 2012;21(3):527-534. Buck HG, Lee CS, Moser DK, et al. Relationship between self-care and health-related quality of life in older adults with moderate to advanced heart failure. J Cardiovasc Nurs. 2012;27(1):8-15. Groessl EJ, Kaplan RM, Rejeski WJ, et al. Health-related quality of life in older adults at risk for disability. Am J Prev Med. 2007; 33(3):214-218. Covinsky KE, Wu AW, Landefeld CS, et al. Health status versus quality of life in older patients: does the distinction matter? Am J Med. 1999;106(4):435-440. Ware JE Jr, Sherbourne CD. The MOS 36-item short-form health survey (SF-36). I. Conceptual framework and item selection. Med Care. 1992;30(6):473-483. Brazier JE, Harper R, Jones NM, et al. Validating the SF-36 health survey questionnaire: new outcome measure for primary care. BMJ. 1992;305(6846):160-164. IBM. Statistical Package for the Social Sciences (SPSS) Armonk, NY: IBM, Inc.; 2012. Bentsen SB, Gundersen D, Assmus J, Bringsvor H, Berland A. Multiple symptoms in patients with chronic obstructive pulmonary disease in Norway. Nurs Health Sci. 2013;15(3):292-299. Reilly CM, Bruner DW, Mitchell SA, et al. A literature synthesis of symptom prevalence and severity in persons receiving active cancer treatment. Support Care Cancer. 2013;21(6):1525-1550. Butt Z, Rao AV, Lai JS, Abernethy AP, Rosenbloom SK, Cella D. Age-associated differences in fatigue among patients with cancer. J Pain Symptom Manag. 2010;40(2):217-223. Kralik D, Telford K, Price K, Koch T. Women’s experiences of fatigue in chronic illness. J Adv Nurs. 2005;52(4):372-380. Blinderman CD, Homel P, Billings JA, Portenoy RK, Tennstedt SL. Symptom distress and quality of life in patients with advanced congestive heart failure. J Pain Symptom Manag. 2008;35(6): 594-603. Kroenke K, Arrington ME, Mangelsdorff AD. The prevalence of symptoms in medical outpatients and the adequacy of therapy. Arch Intern Med. 1990;150(8):1685-1689.
Matura et al 31. Probst MA, Mower WR, Kanzaria HK, Hoffman JR, Buch EF, Sun BC. Analysis of emergency department visits for palpitations (from the National Hospital Ambulatory Medical Care Survey). Am J Cardiol. 2014;113(10):1685-1690. 32. Buysse DJ, Reynolds CF 3rd, Monk TH, Berman SR, Kupfer DJ. The Pittsburgh sleep quality index: a new instrument for psychiatric practice and research. Psychiatry Res. 1989;28(2):193-213. 33. Avi S. The role and validity of actigraphy in sleep medicine: an update. Sleep Med Rev. 2011;15(4):259-267. 34. Kushida CA, Littner MR, Morgenthaler T, et al. Practice parameters for the indications for polysomnography and related procedures: an update for 2005. Sleep. 2005;28(4):499-521. 35. Batal O, Khatib O, Bair N, Aboussouan L, Minai O. Sleep quality, depression, and quality of life in patients with pulmonary hypertension. Lung. 2011;189(2):141-149. 36. Minai OA, Malik N, Foldvary N, Bair N, Golish JA. Prevalence and characteristics of restless legs syndrome in patients with pulmonary hypertension. J Heart Lung Transplant. 2008;27(3):335-340. 37. Yancy JW, McCrory DC, Coeytaux RR, et al. Efficacy and tolerability of treatments for chronic cough: a systematic review and meta-analysis. Chest. 2013;144(6):1827-1838.
221 38. Heath D, Whitaker W, Brown JW. Idiopathic pulmonary hypertension. Br Heart J. 1957;19(1):83. 39. Kawut SM, Al-Naamani N, Agerstrand C, et al. Determinants of right ventricular ejection fraction in pulmonary arterial hypertension. Chest. 2009;135(3):752-759. 40. Costantino G, Furlan R. Syncope risk stratification in the emergency department. Cardiol Clin. 2013;31(1):27-38. 41. Ware JE Jr, Sherbourne CD, Achat H, et al. MOS 36-item shortform health survey. Optimism and depression as predictors of physical and mental health functioning. Normative Aging Study. 2000;22(2):127-130. 42. Balderson BH, Grothaus L, Harrison RG, McCoy K, Mahoney C, Catz S. Chronic illness burden and quality of life in an aging HIV population. AIDS Care. 2012;25(4):451-458. 43. Wikman A, Wardle J, Steptoe A. Quality of life and affective well-being in middle-aged and older people with chronic medical illnesses: a cross-sectional population based study. PLoS ONE. 2011;6(4):e18952. 44. Snow JL, Kawut SM. Surrogate end points in pulmonary arterial hypertension: assessing the response to therapy. Clin Chest Med. 2007;28(1):75-89.
Symptom Prevalence, Symptom Severity, and Health-Related Quality of Life Among Young, Middle, and Older Adults With Pulmonary Arterial Hypertension
American Journal of Hospice & Palliative Medicine® 2016, Vol. 33(3) 214-221 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/1049909114554079 ajhpm.sagepub.com
Lea Ann Matura, PhD, RN1, Annette McDonough, PhD, RN2, and Diane L. Carroll, PhD, RN, FAAN3
Abstract Pulmonary arterial hypertension (PAH) is a chronic, life threatening illness that affects primarily women. The purpose of this study was to describe the prevalence of PAH symptoms and to determine whether there are differences in symptom severity and HRQOL in PAH symptoms among young, middle, and older adults with PAH. A cross sectional design was utilized. For all the age groups, shortness of breath (SOB) on exertion and fatigue were the two most prevalent symptoms. SOB on exertion had the highest symptom severity scores followed by fatigue for all groups. Symptom severity was significantly different among the groups for palpitations, abdominal swelling and nausea. For components of HRQOL, physical functioning worsened with age. All groups had diminished general health, role physical and vitality levels. There are some differences in symptom prevalence, symptom severity and HRQOL among young, middle and older adults. Awareness of these differences is important for healthcare providers to know and assess overtime. Palliative care should be an integral part of caring for patients with PAH. Keywords symptoms, quality of life, pulmonary, hypertension, aging, severity
Introduction Pulmonary arterial hypertension (PAH) is a chronic illness caused by elevated pulmonary artery pressures, which leads to increased pulmonary vascular resistance and ultimately right heart failure. Pulmonary arterial hypertension is predominantly diagnosed in women (80%).1 Pulmonary arterial hypertension affects all races yet death is higher in African Americans and Asians.2 Epidemiology studies estimate PAH at 7.6 cases per million and a prevalence rate of 26 cases per million.3 The average time from the onset of symptoms to diagnosis is approximately 2 years resulting in increased disease severity at diagnosis. Mortality is high, but advances in treatment have increased survival rates from 1-year survival of 68% in 1980s to 97% today.4 There are several etiologies of PAH with approximately 50% being idiopathic PAH. Other associated forms of PAH are related to connective tissue disease, HIV, portopulmonary hypertension, congenital heart disease, and drugs (eg, anorexigens).5 Medical regimens can be complex for some patients including prostanoid analog infusions either continuously or subcutaneously.1 Some common symptoms reported by people with PAH are dyspnea on exertion, fatigue, and sleep disturbance.6-8 These symptoms can be severe and negatively impact health-related
quality of life (HRQOL).7,9-11 However, we do not know whether there are differences in symptoms and HRQOL among different age-groups of patients with PAH. As survival increases, the need to understand how PAH affects people at different stages of their life is necessary. Other disorders have also demonstrated differences between younger and older adults with regard to symptom severity and symptom perception. Older patients with esophageal reflux reported less heartburn and acid reflux severity than younger patients although older adults had increased acid exposure and mucosal injury.12 Depressive symptoms among adulthood demonstrated that older adults also experienced an increase in distress with age.13 Furthermore, in a cohort of patients
1
School of Nursing, University of Pennsylvania, Philadelphia , PA, USA Massachusetts General Hospital, University of Massachusetts, Lowell, MA, USA 3 Munn Center for Nursing Research, Institute for Patient Care, Boston, MA, USA 2
Corresponding Author: Lea Ann Matura, PhD, RN, School of Nursing, University of Pennsylvania, Claire M. Fagin Hall, 418 Curie Blvd, Room 322, Philadelphia, PA 19104, USA. Email: [email protected]
Matura et al receiving palliative care, the oldest adults were less likely to report pain, anxiety, and nausea than younger patients; however, there were no differences between the age-groups for dyspnea and depression levels.14 Symptom recognition in heart failure shows older patients have more difficulty detecting and interpreting dyspnea than younger patients.15 Older patients undergoing a 6-minute walk test (6MWT) were twice as likely to report differing levels of dyspnea than the research assistants assessing them during the 6MWT. Differing levels of symptom perception as patients age may be a key component for patients’ ability to perform selfcare and impact hospital readmissions.16 Symptoms such as dyspnea and fatigue were present for 5 to 7 days before seeking heart failure treatment. Older patients did not often attribute increasing symptom severity with the need to seek medical care. Understanding differences in symptom experiences among the age-groups is important in order to formulate a treatment plan and teach patients how to interpret their symptom experiences and when to seek medical treatment. Chronic illness in the older adult can impact their ability to perform activities of daily living and negatively impact HRQOL.17 Along with difficulties with activities of daily living, depression and problems with memory are associated with HRQOL in older adults.18 The ability to recognize symptoms is necessary in order for older patients with heart failure to perform self-care and the confidence older patients have to perform self-care is also associated with HRQOL.19 Older adults with impaired ability to perform activities of daily living are strong predictors of disability and negatively affect HRQOL.20 Understanding how symptoms and HRQOL differ as patient’s age is important in order to inform care. To date, there has not been a study investigating if there are differences in symptom prevalence and severity and HRQOL among different age-groups of patients with PAH. The purpose of this study was to describe the prevalence of PAH symptoms and to determine whether there are differences in symptom severity and HRQOL in PAH symptoms among young, middle, and older adults with PAH.
Methods Design and Participants This was a cross-sectional study that included a convenient sample of patients with PAH. Inclusion criteria included adults aged 18 and older who were able to read and speak English. All participants self-identified as having a PAH diagnosis. The PAH diagnoses included idiopathic, heritable, along with associated PAH (connective tissue disease, HIV, portopulmonary hypertension, congenital heart disease, and drug use such as anorexigens). Participants were recruited and enrolled over a 2-year period (May 2009-May 2011). Two hundred and seventy-six participants were recruited. One hundred and ninety-one participants returned completed surveys, resulting in a 69% response rate. Young adults were defined as those 18 to 40 years old, middle adults were those 41 to 64 years old, and those 65 and older were considered older adults.
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Procedures This study was approved by the university and hospital institutional review boards before recruitment of participants began. Participants were recruited from several sites including a PAH clinic, PAH support groups in the Northeast, the Pulmonary Hypertension Association (PHA) Web site, and local and international PAH conferences. Potential participants were approached by the advanced practice nurse in the PAH clinics to introduce the study. Interested participants were given the principal investigator’s (PI) contact information. At the monthly PAH support group meetings and the conferences, the PI was introduced. Interested participants would approach the PI if they were interested in participating. The administrator for the PHA Web site posted an advertisement for the study. Interested participants contacted the PI for more details regarding the study. Participants either completed the questionnaires and returned to the PI at the conferences and PAH support group meetings or they mailed the questionnaires back to the PI via the self-addressed, stamped envelopes that were provided. All participants completed a sociodemographic and clinical data form, the Pulmonary Arterial Hypertension Symptom Scale (PAHSS) and the Medical Outcomes Study Short Form36 (SF-36).
Measures Sociodemographic and Clinical Data Form. Variables were collected by self-report and included age, gender, race/ethnicity, educational level, occupation, employment status along with date of PAH diagnosis, PAH etiology, oxygen use, and medications. The PI (LAM) determined the PAH World Health Organization (WHO) functional class based on the self-reported symptoms.1 World Health Organization functional class I are those with PAH who do not have any symptoms of dyspnea, fatigue, or syncope with physical activity. World Health Organization functional class II are those who are not symptomatic with usual activities while those with WHO functional class III have symptoms with less than usual activity but they are comfortable at rest. World Health Organization functional class IV are those patients with PAH who have symptoms at rest.1
Pulmonary Arterial Hypertension Symptom Scale The PAHSS is an investigator-developed symptom severity scale that assess 17 PAH symptoms.8 Participants rate the severity of their symptoms over the past month. The rating scale is 0 to 10 with 0 representing ‘‘absence’’ of the symptom and 10 indicating ‘‘extremely intense.’’ Symptoms included on the measure are shortness of breath (SOB) with exertion, SOB lying down, SOB at rest, awakening at night SOB, fatigue, difficulty sleeping, chest pain, abdominal swelling, swelling of ankles and feet, syncope, palpitations, dizziness, cough, nausea, loss of appetite, and hoarseness Raynaud’s phenomenon (cold, numbness of extremities, etc). Initial psychometrics have been established with good validity and reliability.8
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216 Table 1. Sociodemographic and Clinical Variables Among Age-Groups. Variable (N ¼ 191) Mean + SD age, yearsa Gender: female Ethnicity: caucasiana Marital status: marrieda Living arrangements with family/friends Education: college graduate Employment statusa Full-time Part-time Retired Disabled PAH etiology Idiopathic Connective tissue disease Congenital heart disease Drugs (fenfluramine/phentermine) Functional class I II III IV Oxygen usea Years since diagnosis Medications Calcium channel blockers Endothelin receptor Antagonists phosphodiesterase type-5 inhibitors (PDE-5) Prostanoid analogs Diuretics Digoxin
Total (N ¼ 191)
Young adults (n ¼ 39)
Middle adults (n ¼ 101)
Older adults (n ¼ 51)
53.2 + 15.1 162 (85%) 168 (88%) 97 (51%) 141 (74%) 113 (59%)
30.2 + 6.4 33 (85%) 30 (77%) 11 (28%) 28 (72%) 26 (67%)
53.3 + 6.4 89 (88%) 89 (88%) 51 (51%) 75 (74%) 65 (64%)
71.0 41 49 35 38 22
+ 4.2 (80%) (96%) (69%) (75%) (43%)
36 (19%) 21 (11%) 43 (23%) 77 (43%)
13 (33%) 7 (18%) 0 (0%) 13 (33%)
21 (21%) 8 (8%) 7 (7%) 59 (58%)
2 6 36 5
(4%) (12%) (71%) (10%)
104 (55%) 34 (18%) 8 (4%) 8 (4%)
25 (64%) 5 (13%) 0 (0%) 1 (3%)
52 (52%) 20 (20%) 6 (6%) 1 (1%)
27 9 2 6
(53%) (18%) (4%) (12%)
22 (12%) 36 (19%) 54 (28%) 79 (41%) 115 (60%) 6.3 + 6.6
7 (18%) 9 (23%) 9 (23%) 14 (36%) 13 (33%) 5.3 + 6.7
12 (12%) 18 (18%) 28 (28%) 43 (43%) 67 (67%) 5.6 + 6.2
3 9 17 22 35 6.2
(6%) (18%) (33%) (43%) (69%) + 7.9
46 (24%) 95 (50%) 110 (58%) 75 (39%) 117 (61%) 29 (15%)
5 (13%) 20 (51%) 27 (69%) 19 (49%) 20 (51%) 10 (26%)
25 (25%) 53 (53%) 54 (54%) 41 (41%) 69 (69%) 13 (13%)
16 22 29 15 28 6
(31%) (43%) (57%) (29%) (55%) (12%)
Abbreviations: PAH, pulmonary arterial hypertension; SD, standard deviation. a P < .05.
Medical Outcomes Study Short Form-36 The SF-36 is a widely used measure of generic HRQOL. It contains both physical and mental components.21 There are 8 subscales: physical functioning, role physical, bodily pain, general health, vitality, social functioning, role emotional, and mental health. These subscales are combined to form composite physical and mental summary scores. Scores range from 0 to 100 with higher scores indicating better HRQOL. Psychometric properties have been established.22,23
Data Analysis Sociodemographic and clinical variables were analyzed using descriptive statistics. Frequencies and percentages were used for categorical variables; means and standard deviations were used to characterize continuous variables. Based on distributional property analysis, normalizing transformations were not necessary to perform. Outliers were assessed by visual inspection of distributions. Comparisons among the age-groups for continuous variables were performed using 1-way analysis of variance (ANOVA) models. Chi-square statistics were used for
comparisons among categorical variables. The 17 PAH symptoms and HRQOL are compared between the 3 age-groups (ie, young, middle, and older) using 1-way ANOVA models and the F statistic with post hoc pair-wise comparisons analyzed using the Dunnett’s tests. No adjustments were made for multiplicity. All analyses were performed using SPSS version 21.24 Statistical significance was set at .05 level.
Results The total sample included 191 participants. The mean age was 53.2 + 15.1 years. The majority of the sample was female (85%) and caucasian (88%). Forty-three percent of the sample reported that they were disabled. Over half of the sample was idiopathic PAH, 41% were WHO functional class IV, and 60% were using oxygen. The mean age since diagnosis was 6.3 + 6.6 years. Over half of the population were taking endothelin receptor antagonists, phosphodiesterase type-5 inhibitors, and diuretics. Among the age-groups, 20% were classified as young; 53% were middle; and 27% were older adults. The groups significantly differed among ethnicity, employment status, and oxygen use, as shown in Table 1.
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Table 2. Symptom Severity Among Age-Groups. Symptom intensity (0-10), mean + SD SOB with exertion Fatigue Difficulty sleeping Swelling of ankles/feet Fast heart beat/palpitationsa Raynaud’s phenomenon Dizzy/lightheaded Abdominal swellingb Cough Chest pain/discomfort Loss of appetite SOB at rest SOB while lying down Nauseaa Hoarseness Awaken at night SOB Passing out
Total (N ¼ 191) 6.2 + 5.0 + 3.2 + 2.9 + 2.9 + 2.7 + 2.6 + 2.3 + 2.3 + 2.2 + 1.9 + 1.8 + 1.7 + 1.5 + 1.5 + 0.9 + 0.4 +
Young adults (n ¼ 39)
2.7 2.5 3.4 3.1 2.6 3.4 2.5 3.2 2.6 2.4 2.5 2.3 2.2 2.4 2.3 1.8 1.5
5.6 4.8 3.4 2.3 3.6 1.7 2.9 2.2 2.5 2.9 2.5 1.9 1.7 2.5 1.2 1.1 0.9
+ 2.7 + 3.0 + 3.7 + 3.0 + 2.8 + 2.8 + 3.0 + 3.0 + 3.0 + 2.7 + 2.9 + 2.4 + 2.3 + 3.3 + 2.2 + 1.9 + 2.3
Middle adults (n ¼ 101) 6.3 5.2 3.4 3.2 3.0 3.1 2.7 2.9 2.1 2.1 1.6 1.8 1.9 1.4 1.6 1.0 0.3
+ 2.9 + 2.3 + 3.4 + 3.0 + 2.6 + 3.5 + 2.4 + 3.4 + 2.4 + 2.3 + 2.3 + 2.4 + 2.4 + 2.2 + 2.3 + 1.8 + 1.3
Older adults (n ¼ 51) 6.6 + 4.7 + 2.7 + 2.6 + 2.1 + 2.9 + 2.2 + 1.1 + 2.6 + 1.6 + 1.9 + 1.6 + 1.2 + 0.8 + 1.7 + 0.5 + 0.3 +
2.5 2.5 3.1 3.1 2.4 3.4 2.3 2.6 2.7 2.2 2.5 2.0 1.8 1.5 2.3 1.5 1.1
Abbreviations: SOB, shortness of breath; SD, standard deviation. a Significant differences between young and older adults at P < .05. b Significant differences between middle and older adults at P < .05.
Table 3. Health-Related Quality of Life Among Age-Groups. Subscale PAH (mean + SD) General health Physical functiona,b Role physical Pain Vitality Social function Mental health Role emotional Composite summary mental health Composite summary physical health
Total (N ¼ 191) 37.1 + 25.9 41.5 + 25.9 36.4 + 40.3 66.4 + 27.6 41.3 + 22.7 65.5 + 26.4 71.9 + 19.3 67.0 + 41.5 61.5 + 21.0 45.4 + 22.7
Young adults (n ¼ 39) 45.0 + 52.1 + 45.5 + 67.9 + 47.8 + 70.9 + 72.3 + 71.8 + 65.7 + 52.6 +
25.1 24.1 44.7 25.7 23.4 24.4 16.0 37.1 19.5 25.6
Middle adults (n ¼ 101) 34.7 + 19.6 40.7 + 24.6 35.7 + 40.0 65.7 + 28.1 39.1 + 21.8 64.3 + 26.6 72.4 + 20.2 64.0 + 43.4 60.0 + 21.1 44.2 + 21.5
Older adults (n ¼ 51) 35.8 + 35.0 + 30.9 + 66.3 + 40.4 + 63.7 + 70.7 + 69.3 + 61.0 + 42.0 +
20.1 27.5 37.3 27.9 23.5 27.5 20.0 41.0 21.7 21.6
Abbreviations: PAH, pulmonary arterial hypertension; SD, standard deviation. a Significant differences between young and middle adults at P < .05. b Significant differences between young and older adults at P < .05.
For the total sample, the most prevalent of the 17 PAH symptoms were SOB on exertion (98%), fatigue (95%), and palpitations (78%). The highest mean symptom scores included SOB on exertion (6.2 + 2.7), fatigue (5.0 + 2.5), and difficulty sleeping (3.2 + 3.4), as shown in Table 2. The lowest HRQOL measures represented by the SF-36 subscales for the total sample included general health (37.1 + 25.9), physical function (41.5 + 25.9), role physical (36.4 + 40.3), and vitality (41.3 + 22.7) along with the SF-36 composite summary physical health (45.4 + 22.7), as shown in Table 3. Among the age-groups, there were significant differences between the young and middle adult groups and the young and older adult groups for the HRQOL scores on the SF-36 physical function subscale. Among the age-groups, there were significant differences between the young and older adult mean scores for palpations and nausea. There were also differences between the middle
and older adult mean scores for abdominal swelling. The most prevalent symptoms for the young adult group (Figure 1) were similar to the total sample: SOB on exertion (100%), fatigue (92%), and palpitations (87%). The highest mean severity scores for the young adult group included SOB on exertion (5.6 + 2.7), fatigue (4.8 + 3.0), and palpitations (3.6 + 2.8). The most prevalent symptoms for the middle adult group included SOB with exertion (98%), palpitations (82%), and dizziness (77%; Figure 2). The highest mean scores for the middle adult group included SOB on exertion (6.3 + 2.9), fatigue (5.2 + 2.3), and difficulty sleeping (3.4 + 3.4). The most prevalent symptoms for the older adult group included SOB with exertion (98%), fatigue (92%), and cough (68%; Figure 3. The highest mean scores for the old adult group included SOB on exertion (6.6 + 2.5), fatigue (4.7 + 2.5), and Raynaud’s phenomenon (2.9 + 3.4).
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Discussion
Figure 1. Pulmonary arterial hypertension (PAH) symptom prevalence for young adults.
Figure 2. Pulmonary arterial hypertension (PAH) symptom prevalence for middle adults.
Figure 3. Pulmonary arterial hypertension (PAH) symptom prevalence for older adults.
This is the first study to describe the prevalence and differences in PAH symptoms and HRQOL among young, middle, and older adults with PAH. These results show similarities and differences in the symptom experience and HRQOL among different age-groups in patients with PAH. More of the older adults use oxygen compared to the other age-groups. Shortness of breath on exertion and fatigue were the 2 most prevalent symptoms for all age-groups. The next most prevalent symptom for the young adult group was palpitations in comparison to difficulty sleeping for the middle adults and cough for the older adults. Physical functioning was significantly different among the groups with the older adult group having the worst physical functioning. Comparison of symptom prevalence and severity scores in other disorders reveals similar and contrasting patterns. In a study of patients with chronic obstructive pulmonary disease (COPD), all patients reported breathlessness25 similar to the findings of this study where 98% to 100% of the patients among the different PAH age-groups reported SOB on exertion. This study reported a diverse prevalence of 70% to 92% for fatigue in comparison to another study of patients with COPD who reported 72% with fatigue.25 Of the 21 studies included in a recent review of symptom prevalence and severity in people receiving active cancer treatment, only 44% of patients reported difficulty breathing/dyspnea with a mean severity score of 2.8 (based on 0-10 scale).26 In contrast, among the age-groups in this study 98% to 100% reported SOB on exertion with a mean symptom severity score ranging from 5.6 to 6.6. Oxygen use also increased incrementally with age as did the means SOB with exertion scores possibly indicating worsening disease with age. These differences among studies may not be unexpected due to the nature of PAH involving the heart and lungs and the Reilly et al. review including only 20% of patients with lung cancer who may experience increased levels of SOB. Compared to the Bentsen et al.’s review, results of this study showed a much higher prevalence of fatigue among the agegroups (70%-92%) and slightly higher mean severity scores (4.7%-5.2%). The review revealed that generalized fatigue was reported in 60% of the patients with a mean severity score of 4.6 (based on 0-10 scale).26 Another study investigated whether fatigue levels increased in age by comparing the US general population and a sample of patients with cancer.27 Those with cancer reported more severe fatigue than the general population and there was increased fatigue with age in the cancer group similar to the findings of this study. In another study, women living with chronic illness reported fatigue as a barrier to performing activities of daily living and impaired their HRQOL.28 Similarly, a study investigating symptoms and HRQOL in heart failure found the most prevalent symptom was lack of energy (66%) while SOB was reported by 56%.29 The pathophysiology of PAH resulting in impairment in cardiopulmonary function and right heart failure may contribute to a higher proportion of patients with PAH experience fatigue.1
Matura et al This study had 77% of the middle-aged group report dizziness with a mean severity score of 2.7 while the review of people receiving cancer treatment a smaller proportion (26%) reporting dizziness, the mean score was much higher (4.7). Dizziness could be treatment related in both PAH and cancer or in the case of PAH it may be related to hemodynamic changes where blood is not effectively pumped from the impaired right heart to the left heart and the rest of the body. Palpitations were prevalent in the young (87%) and middle (82%) adult group. Palpitations are abnormal sensations of irregular, rapid, or pulsations in the chest30 and are common symptoms reported in outpatient settings and emergency departments (EDs).31 In a survey of people seeking care at EDs for palpitations (N ¼ 684 000), 22% were young adults and 34% were middle adults with over half being female (61%). Thirty-eight percent of those with palpitations were attributed to cardiac disease. In this study, palpitations were the third highest mean score (3.6) for the middle adult group. Although the severity is in the mild range, it is prevalent symptom for this group. Assessing the presence and the severity may be important to prevent ED visits or possible hospital admissions. Difficulty sleeping had the highest mean symptom severity score (3.4) for the middle adult group. On the PAHSS, sleep is assessed by asking patients to rate their ‘‘difficulty sleeping’’ over the past month on a 0 to 10 scale. Patients who respond that they are having difficulty sleeping may need further sleep assessments including self-report questionnaires (eg, Pittsburgh Sleep Quality Index),32 actigraphy,33 or polysomnography.34 While the mean score is in the mild range, sleep is an important symptom to assess. Sleep disturbance in PAH includes insomnia, excessive daytime sleepiness,35 and restless leg syndrome.36 Poor sleep quality is associated with depression, dyspnea, and poor quality of life.35 Cough was another prevalent symptom (68%) in the older adult group with a mean score of 2.6 in comparison to 52% with a mean score of 5.1 in patients receiving cancer treatment. Cough may be related to the tumor involvement in lung cancer or metastatic lung disease or possibly a comorbidity such as COPD or gastroesophageal reflux disease.37 In PAH, there may be dilation of the pulmonary vessels causing cough.38 Although only 12% of the total sample reported syncope of which 10% were older adults, this is an important symptom to assess in patients with PAH. In PAH, syncope is a poor prognostic sign39 as there is a risk for injuries from falls especially with the older adults.40 The SF-36 measured HRQOL in this study. For the US normative data, scores higher than 50 are considered above average or signifying better HRQOL.23,41 Components of HRQOL were diminished in this study for the middle and older adult groups (general health, physical function, role physical, vitality, and the composite summary physical health). The younger adult group had similar HRQOL except for the physical functioning subscale which was slightly above the US normative average indicating slightly better physical function. HIV is an associated etiology for PAH. HRQOL in HIV was associated with the increased number of chronic illnesses42
219 making it necessary for health care providers to be aware of those people who develop PAH from their HIV disease that their HRQOL may be further impacted and needs to be monitored. Another study investigating HRQOL in chronic illnesses determined that HRQOL was most impaired by those with stroke and least by those with cancer compared to those with no current acute or chronic illness.43 Multiple chronic illnesses were associated with greater HRQOL impairments. Healthrelated quality of life in heart failure was moderately compromised.29 Health-related quality of life was strongly associated with symptom distress, comorbidities, female sex, and functional impairment demonstrating the need to assess symptoms and HRQOL in PAH. In relation to physical functioning, 43% of the total sample reported that they were disabled. Of those reporting disability, 33% were young adults and 58% were middle-aged adults. This is important for health care providers to understand in order to mitigate/manage symptoms and enhance functional status. Not only is physical functioning related to prognosis44 it can also impact HRQOL. As new treatments, both pharmacological and nonpharmacological, continue to emerge, mortality may continue to decrease increasing the older adult population with PAH. Assessing symptoms that are prevalent, severe, and impair HRQOL is needed. There are several limitations to this study. First, a nonrandom sample was recruited and enrolled in this study. Second, all participants self-reported their PAH diagnosis and PAH etiology. There was no confirmation of their diagnosis with medical records. Third, there may be recall bias with selfreport measures. Furthermore, a more robust sample including more men and additional ethnicities is warranted. We did not collect other clinical variables which may contribute to the understanding of symptoms in PAH and physical functioning such as hemodynamic parameters and the 6MWT. Longitudinal studies are needed to understand how symptoms and HRQOL change over time as patients age. In summary, this study adds to the literature on the similarities and differences in the symptom prevalence, severity, and HRQOL among age-groups in patients with PAH. For all the age-groups, SOB on exertion and fatigue were the 2 most prevalent symptoms. Palpitations were also prevalent in the young adult group; difficulty sleeping was common in the middle adult group; and cough was prevalent in the older adult group. Shortness of breath on exertion had the highest symptom severity scores followed by fatigue for all groups. Symptoms that were significantly different among the groups were palpitations, abdominal swelling, and nausea. Of those symptoms that were significantly different among the age-groups, the older adult group had the lowest severity means. For components of HRQOL, physical functioning worsened with age. All groups had diminished general health, role physical, and vitality levels. Palliative care is an integral part of caring for patients with PAH and timely referrals to the palliative care team are essential to optimize symptom management. Clinicians caring for patients with PAH need to understand the
American Journal of Hospice & Palliative Medicine® 33(3)
220 similarities and differences in symptoms as patients age and the need to employ palliative care throughout the disease trajectory and life span.
13.
Declaration of Conflicting Interests The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
14.
Funding This work was partially supported by a Bouve College of Health Sciences, Northeastern University intramural grant and Sigma Theta Tau International Gamma Epsilon grant.
15. 16.
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