Research, Life Expectancy & Prognosis for PH

Researching treatments and a possible cure for pulmonary hypertension

Although pulmonary arterial hypertension was discovered in 1891, there were no known treatments for the disease until 1994 when Flolan was introduced. Previous to the release of that medication, the prognosis and life expectancy for a patient with pulmonary hypertension was around 3 years and it took an average of 2 years to accurately diagnose a patient. Flolan was the first drug to increase the life expectancy by up to 5 years for pulmonary hypertension patients. While we do not currently have a cure for the disease, several more treatments have been approved for PAH thanks to research. The most recent studies have shown improved average survival now at 7 years. However, survival at centers of excellence is probably even better. Over the past 20 years, we have gone from no medications to treat PAH to over 10 medications. Even more medications are being studied. We are optimistic that our patients will continue to experience longer survival and better quality of life.

Key players in research for pulmonary hypertension

The advancements in treating pulmonary hypertension patients would not have been possible without research. There are PAH Research teamseveral key players in bringing a drug to market or making it available for patients. The drug companies who set up the research study for the specific drug, the pulmonary hypertension centers that become participating sites for the studies, and most importantly the pulmonary hypertension patients who enroll in the research studies.  Research is such an important piece of bringing new therapies to market that the PHA (Pulmonary Hypertension Association) requires all of their Comprehensive Care Centers to have a fully functioning research department.

Who can participate in pulmonary hypertension research

Each research study has strict rules about what types of patients can be enrolled, how the drug will be administered, and what measurements will be used to determine the results of the study. Many of the studies have requirements of what background therapy a patient can be on before they are enrolled in the study. For example, protocol (rules of the study) might say a patient cannot be on any treatments for pulmonary hypertension prior to enrolling in the study or protocol might say they can be on Adcirca or Revatio but not Letairis or Tracleer. It is becoming more and more difficult to find pulmonary arterial hypertension patients to enroll in studies as many of them are starting therapies with physicians outside of specialty clinics and disqualifying themselves as research candidates.

Steps to participating in research for pulmonary hypertension

Researching PH treatmentsMost pulmonary hypertension centers of excellence participate in research studies. If the physician you are currently working with does not participate, inform them that you would like to learn more about research studies for treatments for pulmonary hypertension. They may be able to refer you to a center currently enrolling patients in studies. Research is completely separate from clinical management of the patient meaning your current physician will continue to participate in your care. It is important for potential research patients to understand the risks and benefits of enrolling in a research study and what will be expected of them throughout the study. The patient will be asked to take home information about the study and read through it and come back to discuss any questions or concerns. Once all of their questions are answered they will be asked to sign an informed consent if they are interested in participating. It is important to understand that while a physician may offer the patient the chance to participate in a study, the decision is always up to the patient. Some patients will want to participate in research but not fit the specific rules of the current research studies enrolling. The sponsor (usually a drug company) of the study is ultimately responsible for deciding which patients are eligible to enroll. Once you are enrolled in a research study, you may choose to stop at any time. This is called withdrawing consent. You will not be punished or suffer any negative consequences from your doctor if you choose to stop participating. Some studies last a few months and others last many years. We are very excited about research. Through your participation we are able to improve the care of all patients with PAH.