Expert medical specialty care for rare diseases like pulmonary arterial hypertension was recently the subject of review in an article by Sean M. Studer, MD, MS, Chief of Medicine, Woodhull Hospital Medical Center, NY. The article, entitled “Do Patients With Pulmonary Arterial Hypertension Benefit From Referral to a Specialized Center?” and published in the journal Advances in Pulmonary Hypertension, explored the possibility of improved care for patients suffering from PAH in specialized centers, since direct care for patients outside these centers is not common practice. According to the author, “to best support the argument for referral, this anticipated benefit should be evidence-based, include independent accreditation of specialty centers, and offer multiple opportunities to improve outcome and quality of life in PAH.”
In order to refer a patient to an expert/specialized center, some standards proposed in the fifth annual World Symposium on Pulmonary Hypertension (WSPH) need to be considered, such as evidence that a certain treatment is beneficial to the patient, based either on expertise consensus or clinical studies.
This model for specialty centers offering PAH care seems to have successfully developed in many countries, with PAH patients gaining improved access to much-needed emergency treatments and leading to higher satisfaction among patients, the lowest possible complication rates, the shortest lengths of hospital stays, and the best value for healthcare payers. In agreement with the WSPH’s recommendations, the Pulmonary Hypertension Association (PHA) has developed a pioneering program, the PHA-Accredited Pulmonary Hypertension Care Centers (PHCC) initiative, aiming to improve outcomes for PH patients while providing education to reinforce the correct use of the guidelines, thus promoting continued clinical research. Some experts in the field have noted that different PAH studies suffer from diagnostic inaccuracy, underutilized guideline-based testing, and failure to prescribe adequate treatment in a timely manner in patients suffering from advanced disease. As such, these gaps should be identified by specialty centers in order to optimize PAH outcomes.
As a concluding remark, the author summarizes the potential benefits of referral to and collaboration with specialized treatment centers for PAH, such a diagnostic confirmation, early treatment, therapy monitorization, controlled medication side effects, adjustable therapy throughout time, diverse medication, RV function evaluation over time, PAH support groups, increase awareness of possible clinical trials and optimize timing and access to lung transplantation. Overall, there seems to be sufficient evidence indicating that PAH patients followed and treated in specialty centers can significantly improve disease outcome.
The journal that this new article appeared in, Advances in Pulmonary Hypertension, is an effort by the Pulmonary Hypertension Association to provide a forum to both clinicians and patients alike who wish to follow and partake in the latest research and treatment developments for PH.