Silver Spring, Md. (March 23, 2018) – The Pulmonary Hypertension Association (PHA) patient registry is giving more than 500 people with two deadly, often-misdiagnosed breathing diseases a way to advance patient care – for themselves and others. The national nonprofit established the PHA Registry (PHAR) in 2015 as part of its PH Care Center (PHCC) accreditation program, launched a year earlier to raise the overall quality of care and improve long-term outcomes for PH patients.
PH is a general term for high blood pressure of the lungs. One form of PH, pulmonary arterial hypertension (PAH), is characterized by blood vessels in the lungs that become stiff and narrow, causing enlargement of the right side of the heart as it works extra harder to move blood through to the lungs, which can lead to right heart failure and death. Common symptoms are non-specific and include shortness of breath, fatigue and chest pain. Consequently, this progressive disease is often misdiagnosed—for example, as asthma—resulting in delays in proper diagnosis and treatment and costing patients valuable time. PH can be associated with many rare diseases—for example connective tissue diseases such as lupus and scleroderma, HIV infection, sickle cell anemia—as well as more common conditions and exposures such as congestive heart failure, COPD, liver disease, methamphetamine use, and other illnesses.
The PHAR is a multi-center, observational registry that measures quality of care in newly diagnosed patients at PHCCs with PAH or chronic thromboembolic pulmonary hypertension (CTEPH), two forms of PH for which targeted treatments are available that can extend and improve patients’ lives.
“The PHA registry serves as a great tool to help newly diagnosed patients learn about the necessity of standards of care and the importance of receiving care at a PHA accredited site,” said Martha Royse, APRN, MS, research coordinator at Kentuckiana Pulmonary Associates, a PHAR-participating PHCC. “The registry is not only an introductory way for our patients to learn about the need for research about this rare condition, but also marks the patient’s initial involvement in the journey toward curing this disease.”
As of March 2018, 509 patients cared for at 31 adult PHCCs and three accredited pediatric centers are enrolled in the PHAR. PHAR-participating PHCCs are among a larger network of 54 centers – 46 adult facilities and 8 that treat children – designated as PHCC Centers of Comprehensive Care (CCCs). With the recent addition of three Regional Care Program (RCP) – designated PHCCs, a total of 57 centers in 28 states are now PHA-accredited.
PHAR centers collect information when a patient who is interested in participating is initially diagnosed with follow-up data processed at approximately six-month intervals. In addition to measuring quality of care — including assessing differences in centers’ adherence to evidence-based guidelines and establishing benchmarks for health outcomes — the PHAR helps determine the relationship between expert recommended care strategies and patient outcomes. The PHAR gives participating centers a platform to perform their own research and quality improvement initiatives and assess patient-reported outcomes, including health-related quality of life. Each PHAR site may access its own data in real-time, enabling the center to compare its performance to others in the registry. PHAR sites collect and enter patient-reported outcomes, including two health-related quality-of-life surveys, as well as data such as exact diagnosis and hospitalization rates. To view all PHAR-participating sites, visit https://phassociation.org/phar/clinical-sites/.
By mid-2018, PHA’s PHCC program will release its first PHAR annual report, which will include diagnosis, treatment, overall health, demographic, lifestyle, health insurance information and other data points that could be helpful in advancing the care of people with PH.
“Each patient is reminded of their contribution to the PH community every time they follow-up in the registry,” Royse said.
PHAR participation is open to any accredited PHCC that wishes to enroll patients newly diagnosed with PAH or CTEPH. PHAR centers may author scientific manuscripts using registry data; access their own raw data and real-time center-level summary information for national benchmarking; compare real-time statistics on their center to PHAR averages; and use the registry for local quality improvement initiatives.
About the Pulmonary Hypertension Association
Headquartered in Silver Spring, Md., the Pulmonary Hypertension Association (PHA) is the country’s leading pulmonary hypertension organization. PHA’s mission is to extend and improve the lives of those affected by PH; its vision is a world without PH, empowered by hope. PHA achieves this by connecting and working together with the entire PH community of patients, families, health care professionals and researchers. For more information and to learn how you can support PH patients, visit www.PHAssociation.org and connect with PHA on Twitter and Instagram @PHAssociation and on Facebook at facebook.com/PulmonaryHypertensionAssociation.