Pulmonary arterial hypertension (PAH) is a chronic illness characterized by increased pulmonary pressures resulting in right heart failure and premature death. Common symptoms that impair quality of life and functioning are dyspnea, fatigue and sleep disturbance. This trio of symptoms is highly prevalent and forms a symptom cluster (2 or more symptoms that co-occur) in PAH. From a biological, proinflammatory cytokines are implicated in dyspnea, fatigue and sleep disturbance; there is activation of the sympathetic nervous system (SNS) and an inherent inflammatory process in PAH that contributes to the pathophysiology, but the link to this symptom cluster has not been investigated. One novel, treatment for symptom clusters is slow-paced respiration therapy using the FDA-approved device, RESPeRATE. The device contains headphones and a sensor that attaches to the chest to detect inhalation and exhalation. Musical tones synchronize with the respiratory cycle to slowly guide the user to decrease respirations. RESPeRATE moderates effects of the SNS; lowers blood pressure; improves functional capacity and ejection fraction; and significantly decreases pulmonary pressures in left heart failure. The investigators will enroll 10 women with PAH to use the RESPeRATE device to perform slow-paced respiration for 15 minutes per day for 8 weeks to determine the feasibility and effects on the SNS and inflammatory activity and the symptom cluster. The investigator's overall hypothesis is that, as compared to baseline, after eight weeks of therapy women with PAH who receive slow-based respiration therapy will have lower SNS activity and inflammatory levels, and improved dyspnea, fatigue and sleep disturbance.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
10
The FDA-approved RESPeRATE device contains headphones and a sensor that attaches to the chest to detect inhalation and exhalation. Musical tones synchronize with the respiratory cycle to slowly guide the user to decrease respirations.
University of Pennsylvania
Philadelphia, Pennsylvania, United States
Adherence to slow-paced respiration therapy.
Adherence rates will be assessed by the frequency of days used.
Time frame: 10 weeks
Change in baseline and Week 10 dyspnea scores on the Multidimensional Dyspnea Profile
Time frame: Baseline and 10 weeks
Change in baseline and Week 10 fatigue scores on the Multidimensional Fatigue Inventory
Time frame: Baseline and 10 weeks
Change in baseline and Week 10 sleep scores on the Pittsburgh Sleep Quality Index
Time frame: Baseline and 10 weeks
Change in baseline and Week 10 norepinephrine levels
Time frame: Baseline and 10 weeks
Change in baseline and Week 10 interleukin-6 levels
Time frame: Baseline and 10 weeks
Change in baseline and Week 10 tumor necrosis factor-alpha levels
Time frame: Baseline and 10 weeks
Change in baseline and Week 10 six minute walk test
Time frame: Baseline and 10 weeks
Change in baseline and Week 10 right ventricular systolic pressure
Time frame: Baseline and 10 weeks
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