Palliative Care and Advance Care Planning in Pulmonary Hypertension

INTRODUCING PALLIATIVE CARE IN THE PH PATIENT

Figure 1 shows the current model of palliative care involvement in the setting of advanced illness, illustrating the complementary nature of palliative care with the primary specialist.24 At the beginning of the disease course, the goals are still geared toward maximal, life-prolonging treatments and the initial encounters with the palliative care provider may focus on rapport building and basic symptom inventory. As the illness progresses, the availability and/or efficacy of treatments decreases and symptom burden increases, the role of the palliative care provider expands accordingly to encompass aggressive symptom management, psychological and emotional support, further in-depth advance care planning discussions, and transition to end-of-life or hospice care when appropriate.

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There are currently no guidelines to direct the timing of palliative care involvement in PH. Ideally, many palliative care groups advocate involvement of palliative care as early in the disease course as possible; however, the scarcity of palliative care providers makes this unrealistic at this time. It is possible to extrapolate some of the common practices used in heart failure programs that utilize palliative care to the PH population.

Table 1 shows the World Health Organization (WHO) classification of functional status of patients with PH, which in many ways mirrors the New York Heart Association (NYHA) functional classification in heart failure.25 Both classifications rely on functional status and symptom burden as their assessment tool and class IV on both scales represents the inability to perform any significant physical activity as well as signs of dyspnea at rest. It would be reasonable to consider referral to palliative care once a PH patient reaches class IV, or class III for patients with significant serious medical comorbidities, as is done in an increasing number of heart failure practices that have access to palliative care.

Table 1. World Health Organization Classification of Functional Status of Patients With Pulmonary Hypertension

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It is important to emphasize to both providers and patients that palliative care involvement at this time is a supportive service with the goal of maximizing a patient's quality of life and does not pose any limitations on therapies offered by the provider.

At this point in the disease course, there may be a great deal of prognostic uncertainty with the possibility of improvement with aggressive treatment and extended life span matched by the possibility of a precipitous further decline in functional status and death; therefore, this period offers an ideal window to introduce palliative care with the goals of optimizing the patient's quality of life and addressing more detailed aspects of advance care planning. Table 2 lists several other common “triggers” used to guide palliative care involvement in many medical centers.

Table 2. Possible Triggers for Palliative Care Involvement in PH Patients

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ADVANCE CARE PLANNING IN PH

Advance care planning (ACP) can be described as a process of communication between the health care provider and the patient and family/health care proxy for the purpose of identifying and documenting a health care surrogate as well as individualized goals of care near the end of life. Although many physicians report having had ACP discussions with their patients, most of these discussions tend to be limited to designation of a health care proxy and rarely address end-of-life preferences in detail, driven in part by the physician's discomfort at such discussions or their concern about upsetting the patient. In one survey of PH patients only 38% reported having completed an ACP discussion with a loved one and only 14% reported having discussed these issues with their PH physician.

As Figure 1 illustrates, ACP is not a one-time discussion, but a dynamic, evolving conversation throughout the entire course of the illness with the purpose of enhancing the patient and family understanding of their illness, including prognosis and possible outcomes. It is important to keep in mind that a patient's wishes and goals of care will likely shift frequently depending on a patient's condition; it is not uncommon to have a patient endorse wanting full, aggressive measures at the outset and then change their mind as their condition worsens, only to switch back during a period of improvement. In addition, engagement of the patient's family and/or primary caretaker throughout this entire process is extremely important as they usually have a great deal of input and opinions regarding a patient's treatment goals and will be people responsible for making medical decisions on their behalf, should they become too ill to make their own choices.

At the beginning, these discussions may be limited to basic exploration of the patient's understanding of their disease and designation of a health care power of attorney or proxy. As the disease progresses, as well as symptom burden, it becomes more important to revisit these discussions in a more nuanced fashion, particularly as the treatments become more intensive and the possibility of complications increases. Depending on the disease trajectory, patient preferences regarding specific interventions should be addressed such as dialysis, intubation, long-term intravenous infusions, and their wishes in the setting of serious, long-term disability. Once again, lessons can be learned from the previous experiences of palliative care in heart failure, particularly in the management of mechanical circulatory support (MCS) and left ventricular assist devices (LVAD).26 Similar to many newer treatments for PH, these are advanced interventions that have the possibility of greatly increasing a patient's quality and quantity of life, but that also carry a not-insignificant risk of life-limiting complications, all in the greater context of a progressive, life-limiting illness with significant morbidity and eventual mortality.

Swetz et al2728 discuss the role of “preparedness planning” in MCS, which they describe as “a process that explores a patient's goals, values, and preferences regarding MCS and other quality-of-life–related outcomes … and covers more challenges than do typical advance directive discussions.” Many of the topics addressed in this process, as well as much of the language used in these discussions, can be extrapolated onto the PH patient population. Table 3 gives some examples of sample statements that may be used during this process, especially as patients become sicker and begin considering more intensive therapies. These questions range from fundamental issues such as designation of a health care power of attorney to a more in-depth exploration about a patient's views regarding life-sustaining interventions that may become necessary toward the end of the disease course.

Table 3. Sample Statements for Preparedness Planning in Advanced Pulmonary Hypertension

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As noted previously, only a small percentage of PH patients report having had any form of ACP discussions with their physician. This finding is mirrored in other specialties dealing with life-limiting illnesses including oncology, cardiology, and nephrology.2930 A large part of this low number is driven by the physician's own discomfort at having these conversations. Many physicians report being concerned that discussions centered on disease progression and end-of-life wishes may distress their patients or cause them to “give up,” despite the fact that studies have shown evidence to the contrary3132; several studies centered on open communication in advanced cancer patients revealed that all patients reported wanting honesty from their providers, as well as some indication of their overall prognosis.3334 Palliative care providers can assist with these conversations as their training focuses on eliciting a patient's own hopes and values and working with the patient to ensure that these values are reflected in their treatment plan, as well as discussing serious prognoses in a frank, yet sensitive manner.35 Introducing palliative care into a patient's care team earlier in the course of an illness (as opposed to the older model of utilizing palliative care only at the very end of a patient's life) can allow for greater rapport building and ability to hold these conversations over a longer period of time, in addition to maximizing a patient's quality of life through increased symptom control and supportive care.

CONCLUSION

Despite substantial progress and the development of new treatments, PAH remains a rapidly progressive disease with a poor outcome. Referral with palliative care specialists is uncommon in the care of PAH patients. However, early collaboration with palliative care providers may offer substantial benefits to patients and their families by providing an opportunity for patients to make health care decisions and plans before disease progression. Further study is needed to determine the optimal role of palliative care providers in the management of PAH patients.