A 25-year old woman develops slowly progressive shortness of breath. After two years of wrong turns in her health care she eventually gets an echocardiogram that suggests pulmonary hypertension. She has a right heart catheterization that confirms severe PAH. She is referred to a local pulmonologist who starts her on one oral medication. She improves a bit but continues to have shortness of breath with exertion and fatigue.
Over the next 6 months her symptoms progress and her local pulmonologist keeps changing oral medications. Three years after she first developed symptoms of shortness of breath the patient is hospitalized at her local hospital with severe right heart failure. She develops kidney and liver failure and dies.
Was this young woman’s death avoidable? Was her treating PAH doctor really an expert in treating PAH? Why was she never placed on continuously infused prostanoid therapy? Why was she never referred to a center of excellence?
PAH is a rare complicated disease. Without proper treatment patients will progress and die from right heart failure. Effective treatments are now available. As more therapies have entered the market decision making around choosing the best therapy has become progressively more complicated.
During this same time, more and more physicians are claiming to have expertise in treating PAH. It seems that many local hospitals are now boasting of having a pulmonary hypertension program. How can this be when each year a small handful of new PAH specialists are trained across the entire country?
The short answer is they are not PAH “Programs”. Perhaps they have a physician or two interested in the disease, perhaps they are excited about all of the research currently taking place around PAH and want to participate, perhaps they see upside in all of the testing (echocardiograms, right heart catheterizations, etc.) required to care for PAH patients. But they are not PAH experts.
How can you tell if your PAH doctor is truly an expert in treating this disease?
First, ask what kind of training your doctor has received in PAH. There are no general pulmonary or cardiology fellowship programs that train doctors adequately to be expert in treating PAH. Being an expert requires additional training. Ask your doctor how many of their PAH patients are being treated with continuously infused therapies like treprostinil (remodulin) or epoprostenol (veletri or flolan). Imagine taking your car to a mechanic who only is comfortable using a few of the wrenches and tools. A PAH physician who only uses oral therapy is not capable of delivering the best PAH care for PAH patients.
How can you tell if your PAH program is really a Center of Excellence?
First and foremost, the PHA has developed a credentialing process recognizing the best PAH programs in America. In order to earn the label Comprehensive Care Center these PAH programs must undergo a rigorous review process. They must have super-specialized physicians who are expert in all PAH medications. Expert nurse coordinators must be part of the team to help patients navigate the complex journey of PAH. The affiliated hospitals must have nurses and staff that are highly trained in the care of PAH patients. The program must participate in clinical research helping to advance the care of PAH patients. These dedicated physicians must publish papers advancing the science of caring for PAH patients. These expert PAH physicians and nurses must be active in local, regional and national education and advocacy for PAH patients.
I have heard from around the county that many local hospitals are setting up PAH programs. The physicians that these hospitals have chosen to participate in these programs often have little PAH experience and the hospitals typically have little or no experience in the care of PAH patients. Nursing staff in the hospitals often have little or no experience and the hospitals often don’t even carry all the needed medications. These same hospitals typically don’t have lung transplant services. These same programs are usually not recognized by the PHA as regional or comprehensive care centers. The fact that the hospital is interested in PAH does not mean that they can deliver the best care. What can you do as a PAH patient or advocate of a PAH patient? Don’t be shy. Do some research on your PAH doctor and the hospital you might visit. If they don’t have the credentials then perhaps ask for a second opinion at a PHA recognized Comprehensive Care Center.