Associations of Fatigue and Patient-Reported Outcomes in Pulmonary Arterial Hypertension

1. Associations of Fatigue and Patient-Reported Outcomes in Pulmonary Arterial Hypertension Lea Ann Matura, PhD, RN Assistant Professor University of Pennsylvania School of Nursing

2. Thank You • Funding- Bouve College of Health Sciences, Northeastern University intramural grant and Sigma Theta Tau International Gamma Epsilon grant • Co-Investigators • Diane L. Carroll, PhD, RN • Annette McDonough, PhD, RN • Collaborators • Greg Ball, PhD • Students • Participants No conflicts of interest for the investigators

3. World Health Organization (WHO) Pulmonary Hypertension Groups • WHO Group I – Pulmonary Arterial Hypertension (PAH) • WHO Group II - Pulmonary hypertension due to left heart disease • Atrial or ventricular disease • Valvular disease • WHO Group III - Pulmonary hypertension due to lung diseases or hypoxemia • Chronic Obstructive Pulmonary Disease (COPD), Interstitial Lung Disease • Sleep-disordered breathing, alveolar hypoventilation • Chronic exposure to high altitude

4. WHO Group IV - Pulmonary hypertension due to chronic thrombotic and/or embolic disease • Pulmonary embolism in the proximal or distal pulmonary arteries • Embolization of other matter • WHO Group V – Miscellaneous • Sarcoidosis (Galiè et al., 2009)

5. Pulmonary Arterial Hypertension (PAH) • Elevated mean pulmonary artery pressure (PAP) > 25 mmHg (normal mean PAP ~ 9-12 mmHg) and pulmonary capillary wedge pressure < 15 mmHg • Etiology • Idiopathic • Familial • Connective tissue disease, congenital shunts between systemic and pulmonary circulation, sickle cell, portal hypertension, HIV infection, drugs/toxins

6. Pathobiology • Vasoconstriction • Remodeling of the pulmonary vessel and • Thrombosis (Galiè, 2009)

7. Pulmonary Arterial Hypertension (PAH) • Primary symptoms-dyspnea on exertion and fatigue • Affects women (~78%), mean age diagnosis ~50 years • Approximately 20,000-30,000 cases in US • Mean 2 years from symptom onset to diagnosis • Mortality- 61% at 5 years (Galiè, et al., 2009; Badesch, et al., 2010)

8. Fatigue • Definition- a multidimensional concept defined as an overwhelming, debilitating, and sustained sense of exhaustion that decreases one’s ability to carry out daily activities, to work effectively, and to function (Cellaet al., 2007)

9. Purpose • To determine those socio-demographic/clinical variables and patient-reported outcomes (symptoms, health-related quality of life) associated with fatigue in people with PAH.

10. Methods • Design-Cross sectional, descriptive • Sample- A convenience sample of adults with World Health Organization Group I etiology (PAH) were recruited from an outpatient clinic, support groups • Univariate- descriptive statistics • Bivariate analysis determined variables associated with fatigue to include in multivariate analysis (multiple regression) (p<.20) • Data analysis-SPSS 19

11. Measures • Socio-demographic/clinical data form • PAH Symptom Scale- rating symptom intensity on 17 PAH symptoms • Fatigue measured on this scale for current study • Score range: 0-10; higher score indicates worse symptom intensity (cronbach alpha= .90) • Medical Outcomes Study Short Form-36 v. 2-generic measure of health status/HRQOL • Score range 0-100; higher scores indicate better health status/HRQOL (cronbach alpha=.84) (Brazier et al., 1992) .

12. US Cambridge Pulmonary Hypertension Outcome Review (US CAMPHOR) to measure HRQOL (higher scores indicate worse symptoms, functioning and quality of life) • Score range: Energy 0-10; Breathlessness 0-8; Mood 0-7; Symptom total 0-25; Activity total 0-30) (cronbach alpha= .89) (McKenna et al., 2008) • Profile of Mood States-Short Form (POMS) to measure psychological states • Score range- Higher scores indicate increased distress, except for vigor; Sub scores range from 0-20; Total scores range from -20-100 (cronbach alpha=.65) (McNair et al., 2009)

13. PAH Symptom Scale

15. Results

16. Socio-Demographics & Clinical Characteristics

19. Symptom Prevalence

20. Symptom Prevalence

21. Symptom Intensity Scores

22. SF-36

24. USCAMPHOR

26. POMS

28. Regression Results

29. Fatigue

30. Limitations • Convenience sample • Self-identified PAH • Recruitment sites • Self-report measures • Low cronbach alpha for POMS

31. Implications • Assessment of symptoms are imperative • Fatigue is a moderate-severe symptom in PAH • Fatigue is associated with HRQOL • Improving symptoms such as fatigue may improve HRQOL • Although medications may improve mortality, but may worsen symptoms or induce side effects that affect HRQOL/function

32. Conclusions • Fatigue associated with: • Shortness of breath with exertion • Swelling of ankles/feet • Loss of appetite • Physical composite • Mental composite • ERA use

33. Future • Design, test, implement interventions • Exercise? • Target symptom clusters?

34. Thank You! Questions?