EXPRESS: Reasons for Refusing Parenteral Therapy: A Qualitative Study of Patients with Pulmonary Arterial Hypertension

While parenteral prostacyclin (pPCY) therapy, delivered either subcutaneously or intravenously (IV)is recommended for pulmonary arterial hypertension (PAH) patients with severe or rapidly developing disease, some patients refuse this treatment. This study aimed to understand, directly from patients with PAH, why pPCY was refused and, in some cases, later accepted. Interviews were conducted with 25 PAH patients who previously refused pPCY therapy (Group A: Refused/Never Initiated [n=9] and Group B: Refused/Initiated [n=16]). Patients in both groups believed that pPCY could improve their symptoms, slow disease progression, and provide them a greater ability to perform activities. Reasons for refusal included concern over side effects and the perceived limitations of pPCY on daily activities. Group A perceived their decision as a balance between quality of life and prolonging life and most acknowledged they would reconsider pPCY if other treatment options were exhausted. Group B cited they initiated therapy due to a worsening of symptoms, disease progression, to improve quality of life, to be there for their family, or a desire to live. Following initiation, Group B indicated their experience met expectations with reduced symptoms, slowed disease progression, and perception of improved survival; concerns related to pPCY were described as manageable. Given the efficacy of pPCY therapy, clinicians should apply knowledge of these findings in clinical practice. Patients noted improvements to parenteral pump technologies to include smaller size, water resistance, and implantability may increase their acceptance of this modality. Development efforts should focus on technologies that increase the acceptance of pPCY when indicated.

Download Full-text

EXPRESS: Association of daily physical activity with psychosocial aspects and functional capacity in patients with Pulmonary Arterial Hypertension: a cross-sectional study

Pulmonary Circulation ◽

10.1177/2045894021999955 ◽

2021 ◽

pp. 204589402199995

Author(s):

Layse Nakazato Lima ◽

Felipe Mendes ◽

Ilma Paschoal ◽

Daniela Oliveira ◽

Marcos Mello Moreira ◽

...

Keyword(s):

Quality Of Life ◽

Physical Activity ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Functional Capacity ◽

Daily Physical Activity ◽

Cross Sectional ◽

Pulmonary Arterial ◽

The Mean

Pulmonary arterial hypertension (PAH) impairs exercise tolerance and daily physical activity (PA). Aside from the hemodynamic limitations, physical, cognitive and emotional factors may play a relevant and as yet unexplored role. We investigated whether there is an association between the PA level and psychological disorders, health-related quality of life, and daily activities. We also searched for an association of the PA level with clinical factors and functional capacity. This was an analytical, cross-sectional, observational study conducted in a Brazilian University Hospital. Twenty stable PAH subjects wore an accelerometer for a week and completed an activity diary. They answered the quality of life questionnaire (SF-36), as well as the anxiety and depression scale (HADS), and the Manchester Respiratory Activities of Daily Living questionnaire (MRADL). Transthoracic echocardiography, the 6-Minute walk test (6MWT), the 1-minute sit-to-stand test (STST), and spirometry were performed. For statistical analysis we used Chi-square tests or Fisher's test as appropriate and the Mann-Whitney test to compare numerical values between two groups. The relationship between the parameters was assessed using the Spearman correlation test. The mean age was 44.3 years, 80% were women, 80% had idiopathic PAH, and 20% had connective tissue disease . The mean daily step count was 4,280 Â± 2,351, and the mean activity time was 41.6 Â± 19.3 minutes. The distance covered (6MWT) was 451.5 m, and the number of movements (1-STST) was 23.8. Thirty percent scored positive for anxiety, and 15% for depression (HADS). There was a significant correlation between accelerometer data and walking distance (6MWT), number of movements (1-STST), level of daily physical activity (MRADL), and depression symptoms. Our findings support the hypothesis that other aspects beyond physical and hemodynamic ones might impact the daily physical activity of patients with PAH.

Download Full-text

Effect of Pulmonary Arterial Hypertension-Specific Therapies on Health-Related Quality of Life

CHEST Journal ◽

10.1378/chest.13-2634 ◽

2014 ◽

Vol 146 (3) ◽

pp. 686-708 ◽

Cited By ~ 28

Author(s):

Gilles Rival ◽

Yves Lacasse ◽

Sylvie Martin ◽

Sébastien Bonnet ◽

Steeve Provencher

Keyword(s):

Quality Of Life ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Health Related Quality ◽

Related Quality ◽

Health Related ◽

Pulmonary Arterial

Download Full-text

A prescribed walking regimen plus arginine supplementation improves function and quality of life for patients with pulmonary arterial hypertension: a pilot study

Pulmonary Circulation ◽

10.1177/2045893217743966 ◽

2017 ◽

Vol 8 (1) ◽

pp. 204589321774396 ◽

Cited By ~ 5

Author(s):

Mary Beth Brown ◽

Attie Kempf ◽

Catherine M. Collins ◽

Gary M. Long ◽

Matthew Owens ◽

...

Keyword(s):

Quality Of Life ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Current Evidence ◽

Proof Of Concept ◽

Concept Study ◽

Sf 36 ◽

Pulmonary Arterial ◽

Arginine Supplementation

Current evidence suggests that exercise training is beneficial in pulmonary arterial hypertension (PAH). Unfortunately, the standard supervised, hospital-based programs limit patient accessibility to this important intervention. Our proof-of-concept study aimed to provide insight into the usefulness of a prescribed walking regimen along with arginine supplementation to improve outcomes for patients with PAH. Twelve PAH patients (all women) in New York Heart Association (NYHA) functional class (FC) II (n = 7) or III (n = 5) and in stable condition for ≥ 3 months were enrolled. Patients performed home- and fitness-center- based walking at 65–75% heart rate (HR) reserve for 45 min, six sessions/week for 12 weeks. Concomitant L-arginine supplementation (6000 mg/day) was provided to maximize beneficial endothelial training adaptations. Cardiopulmonary exercise testing, 6-min walk testing (6MWT), echocardiography, laboratory studies, and quality of life (QoL) survey (SF-36) were performed at baseline and 12 weeks. Eleven patients completed the study (72 session adherence rate = 96 ± 3%). Objective improvement was demonstrated by the 6MWT distance (increased by 40 ± 13 m, P = 0.01), VO2max (increased by 2 ± 0.7 mL/kg/min, P = 0.02), time-to-VO2max (increased by 2.5 ± 0.6 min, P = 0.001), VO2 at anaerobic threshold (increased by 1.3 ± 0.5 mL/kg/min, P = 0.04), HR recovery (reduced by 68 ± 23% in slope, P = 0.01), and SF-36 subscales of Physical Functioning and Energy/Fatigue (increased by 70 ± 34% and 74 ± 34%, respectively, P < 0.05). No adverse events occurred, and right ventricular function and brain natriuretic peptide levels remained stable, suggesting safety of the intervention. This proof-of-concept study indicates that a simple walking regimen with arginine supplementation is a safe and efficacious intervention for clinically stable PAH patients, with gains in objective function and QoL measures. Further investigation in a randomized controlled trial is warranted.

Download Full-text