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Predicting Medical Disability in Patients with Pulmonary Arterial Hypertension

C. Abbott

Louise Durst


Page Scovel

F.S. Beery

Michelle Turner

Michael McGoon


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Conference: 2011 PH Professional Network Symposium

Release Date: 09.22.2011

Presentation Type: Abstracts

W. Hill, RN, NP, MSN1; C. Abbott, RN2; L. Durst, RN3; P. Scovel, RN, BSN, CCRC4; F. S. Beery, MS5; M. Turner, MS5; M. McGoon, MD3
1. VA Greater LA Healthcare System/UCLA, Los Angeles, CA
2. University of Colorado Denver, Aurora, CO
3. Mayo Clinic, Rochester, MN
4. University of Iowa, Iowa City, IA
5. ICON Late Phase and Outcomes Research, San Francisco, CA

PURPOSE: PAH is a serious life-limiting disease that has a profound impact on multiple areas of patients' lives. This disease affects every patient differently just as treatment varies from patient to patient. Here we focus on medical disability and its association with PAH. Patients with PAH are often young to middle-aged women and are particularly vulnerable to becoming medically disabled from work or school due to functional and psychological symptoms. The purpose of this analysis is to identify characteristics associated with an increased likelihood for medical disability among patients enrolled in the Registry to EValuate Early And Long-term PAH Disease Management (REVEAL).

METHODS: All REVEAL patients aged ≥19 years except those participating in a blinded clinical trial were included and categorized as either medically disabled or working full- or part-time. Descriptive statistics are presented overall and stratified by disability status at enrollment. Adjusted logistic regression was used to identify enrollment characteristics that were independently associated with medical disability. An alpha of 0.05 was considered statistically significant.

RESULTS: Of the 2070 patients included, 1230 patients were medically disabled and 840 were working full- or part-time. In the descriptive analyses, disabled patients were more likely than non-disabled patients to have familial PAH, connective tissue disease- (CTD), portal hypertension-, drugs and toxins-, or HIV-associated PAH. Adjusted logistic regression showed that characteristics significantly associated with disability included: older age, Hispanic or African American race, worse functional status, decreased 6-minute walk distance, familial PAH, portal hypertension, drugs and toxins-APAH, lower systolic blood pressure, and presence of depression.

CONCLUSIONS: Certain characteristics among patients with PAH are associated with being medically disabled from work or school. These risk factors have previously been shown to be associated with worse prognosis. These findings may be useful for predicting which patients are at an increased risk for medical disability. This may warrant a change in practice patterns to guide patients at risk of disability to meet with social services for assistance either through their medical institution or county. Additional studies are needed to evaluate observed differences in disability among PAH patients with the same risk factors for disability. Further analysis is needed to determine whether specific characteristics, such as depression, were present prior to the diagnosis of PAH.