Furthermore, complications of PAH treatment can have a tremendous impact on patients and their QoL. Central line infection after initiation of intravenous prostaglandins can result in severe debility and even long-term damage to multiple organ systems. This can drastically change a patient’s QoL even if there is no effect on the PAH itself. Replacing a line for intravenous therapy after a previous line becomes infected can be a source of significant anxiety for the patient. Taking these fears and worries into account, along with the patient’s goals and preferences, is essential to creating a care plan that is appropriate for each individual patient.
Patients who are having substantial symptoms or side effects may not always feel comfortable telling their PAH physician if they are having second thoughts about continuing or adding invasive treatments. A palliative physician can explore these concerns and help address them with the patient and with the PAH physician in a collaborative way that helps the patient be heard without fear of disappointing or offending the PAH physician.
Dr Zamanian: PAH not only affects a patient’s physical status and symptoms but also emotional well-being. End-stage PAH is often complicated by progressive symptoms but also aggressive therapies (such as parenteral prostacyclin, which requires daily mixing and self-administration via a pump and central line) that have an effect on QoL. Although patients require lung transplantation when the disease is in its final stages, fewer than 50% either qualify for or receive the life-saving surgery.8
Palliative interventions are essential in the care of these patients to address the physical and emotional toll of the disease, but also to improve QoL. I am not aware of data regarding the underutilization of palliative care in PAH, but I suspect that access to a palliative care team and the stigma around end-of-life care influence such utilization.
Pulmonology Advisor: What are some of the barriers to implementing palliative care in PAH, and what are other important takeaways for clinicians treating these patients?
Dr Goodman: The stigma around the phrase “palliative care” is a significant barrier to its implementation in every patient population. It is often equated with hospice or end-of-life care, and frequently patients, families, and physicians all feel it is premature to provide palliative care. True palliative care, however, is not limited to end-of-life care and can benefit patients with serious and potentially life-limiting illnesses at any point in their course. Palliative care involves both symptom management and advance care planning, which includes discussing the patient’s goals, values, and preferences regarding certain problems and uncertainties related to their disease.
The availability of trained palliative care physicians is a significant barrier, as is a lack of training in both symptom management and communication skills for PAH physicians. There is also a paucity of research on symptom management in this patient population aside from the effects of medications used to treat PAH.
Ongoing open communication with patients and their families about their symptoms and side effects is essential. Letting them know that these are common and can be managed is key. Honesty about likely and possible complications and treatment benefits and limitations and what those limitations and possible complications would mean to the patient is vital. Managing uncertainty in this way helps patients have a far better understanding of their condition and their options.
Dr Zamanian: As mentioned previously, access to a palliative care team is essential. Also, some groups, especially end-of-life or hospice groups, do not provide parenteral prostacyclin because of the cost, thus putting a terminal patient in an awkward situation. We have resolved this issue for some patients by asking manufacturers of these drugs to provide them as compassionate products, which they are sometimes willing to do. Finally, the taboo about end-of-life and palliative care plays a major role here, and patients could benefit from more education.
Pulmonology Advisor: What are the remaining needs in this area, in terms of research or otherwise?
Dr Goodman: Ideally, PAH and palliative care physicians would have some degree of cross-training, so that each specialty understands the possibilities and limitations of the other. Also, the model of “see as many patients as you can, as quickly as you can” could be modified to allow PAH physicians to have the time to invite their patients to discuss symptoms, side effects, and concerns. Probably more feasible would be the creation of multispecialty clinics, in which palliative care and PAH physicians are both available to evaluate and comanage patients with significant symptoms and concerns.
Finally, further assessment of the mortality effects of the many new treatments and combinations of treatments for PAH, as well as predictors of mortality, is essential to give physicians in both fields improved prognostic capabilities, and enable them to share prognostic awareness with patients so they can more effectively plan for end of life as it approaches.
Dr Zamanian: Studies are needed that address patient perceptions about access, utility, and optimization of such programs. Patients with PAH should be viewed the same as patients with end-stage or advanced cancer. Education for both patients and providers is needed on this topic.
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- Lopez-Meseguer M, Quezada CA, Ramon MA, et al; on behalf of the REHAP Investigators. Lung and heart-lung transplantation in pulmonary arterial hypertension [published November 21, 2017]. PLoS One. doi:10.1371/journal.pone.0187811