A patient in his 30s with a history of gastric cancer presented with fever, dyspnea, and hypoxia. We met his wife and young children on admission when the suspected diagnosis was a parapneumonic effusion. Repeat testing revealed evidence of lymphangitic spread of his tumor. Informing him of these results and assessing his goals of care was emotionally challenging for all involved.
Adolescents and young adults (AYA) with cancer are defined as those aged 15-39.1 Advanced care planning discussions with AYA patients can be especially emotional for patients, families, and clinicians, all of whom want to maintain hope. The transition from family caregiver to the patient, loss of employment, and changes in self-identity are causes of suffering in AYA patients. We approach this case from a Palliative Medicine perspective to raise awareness of these AYA-specific issues and highlight the importance of early Palliative Medicine consultation to address the physical, psychosocial, and spiritual needs of AYA patients with cancer.
When engaging in advanced care planning discussions with AYA patients, the lurking pitfall is the hidden assumption that those wanting aggressive treatments are “not ready for Palliative Care.” This has its roots in the synonymous use of Palliative Medicine with “end-of-life care,” which can feel antithetical to supporting a culture of hope for both the patient and provider.2 AYA patients with cancer are more likely to receive aggressive treatments in their last month of life, less likely to receive hospice and palliative care services, and carry a higher burden of undertreated physical symptoms.3 In one study of AYA patients, Palliative Medicine was consulted an average of only 4 to 16 days before death.3
Many AYA patients exit pediatric care but have not established care in adult medical homes, and present to the Emergency Department or hospital with uncontrolled cancer symptoms. Unique points for distress include autonomy from parents, changes in self-identity, social isolation, loss of employment, welfare of children and significant others, fertility preservation, and wellness in survivorship.1 Understanding the breadth of these issues is important to ensure that goals of care discussions with this patient encompass both “what treatments are wanted” and these multiple facets essential to the patient’s quality of life. Recognizing family structure and dynamics allows for referrals to child life services to support his children. Knowing the fertility implications of specific chemotherapy treatment can prompt referrals to aid in family planning and reproductive health. Advocacy and peer support programs that exist through AYA foundations (e.g., LIVESTRONG) can help provide age-appropriate peer support systems.
Advanced Care Planning
As we discussed his preferences for ongoing treatment, he simply nodded affirmatively as each potential treatment modality was explained. His goals of care were clear—he wanted to receive all treatments necessary to prolong life. However, it was not clear that he was ready to discuss the possibility that these treatments may not work. We consulted Palliative Medicine to facilitate discussion of his prognosis and the patient’s priorities to ensure his quality of life. After a therapeutic thoracentesis, his breathing improved and wished to return home prior to the Palliative Medicine consultation.
How can we ensure that our patients want to engage in advanced care planning discussions? Evidence shows that AYA patients are ready for these conversations. Asking these brief “yes” or “no” screening questions can help assess a patient’s readiness for these discussions:4
- It might be helpful for me to talk about what would happen if treatments were no longer effective.
- Talking about medical care plans ahead of time to make sure my wishes are followed in the case that treatment options are limited or there are no more treatment options available would upset me very much.
- I feel comfortable writing down or discussing what I would want to happen to me if treatments were no longer effective.
The advanced care planning tool “5 Wishes®” was evaluated in patients 16-28 years old, and 95% of participants felt that such a document would be “helpful or very helpful”.4 While clinicians may have a significant interest in “the kind of medical treatment I would want,” AYA patients perceived this question as being the most stressful. They often requested more discussion on how they would want to be remembered and what they want their loved ones to know to leave their legacy. If patients are ready, writing letters to their children or loved ones can provide a sense of control of the legacy that they will leave. Best practice is to start these conversations at diagnosis and build over time, monitoring for changes in functional status, disease progression, or other life changes that impact patient goals or prognosis.
At this point, symptom management, allowing time to communicate wishes with family, elucidating end-of-life wishes, and helping children understand their parent’s disease and symptoms would be key Palliative Medicine objectives. If not addressed early, there may be a time when it is “too late” for Palliative Care, and the patient is unable to convey their wishes.
Unfortunately, our patient did not make it to his Palliative Care appointment. He returned shortly after his discharge with respiratory failure due to worsening pulmonary tumor burden. We expressed our concerns to the patient and family that he was nearing the end of life. We discussed the possibility of transitioning to comfort care; however, he wished to continue to pursue treatments, including chest tube placement, intubation, and a time trial of immunotherapy.
First, let us focus on the meaning of “transition,” as its significance may differ between patients and clinicians. Understanding a patient’s wishes about end of life care, prioritizing symptom management, and beliefs about “a good death” can occur in parallel with ongoing treatment rather than ‘in-sequence’ as they transition to end-of-life care. Waiting to discuss these concepts until an AYA patient has “transitioned” to no longer believing treatment could be effective, unfortunately, leads to the observed delays in Palliative Medicine referral.
Second, patients such as ours challenge a conceptual framework that aggressive treatments towards the end of life and death in the hospital represent failures of advanced care planning. The quality of advanced care planning is not measured simply on the absence of these outcomes. Late aggressive treatments and ‘in-hospital death’ on their own do not indicate poor quality of care or that a “good death” did not occur. Characteristics constituting a “good death” are shaped by multiple cultural, social, and personal circumstances. For many patients, dying at home is not wanted as it may increase their sense of being a burden to loved ones. Studies suggest that the place of death is no longer a benchmark of providing quality end of life care.5
Our patient ultimately transitioned to comfort care and died in the hospital, surrounded by loved ones. While the end seemed peaceful, we witnessed the emotional toll of rapidly transitioning from novel immunologic therapies to end of life care on his wife and family. Did he have enough time and guidance to communicate with his family, and did they, in turn, have the support in place to minimize the trauma of watching their loved one go through this process?
While the features of this case stand out due to their emotional impact, they also sadly confirm the common clinical course for AYA cancer patients. To address gaps in care for this population, many hospitals have designed specialized AYA units where there is collaborative care between pediatric and adult oncology. Providing early interdisciplinary support across all psychosocial and physical realms to patients and their families can result in a higher quality of life and lower symptom burden. However, early integration of Palliative Medicine remains suboptimal and exceptionally challenging in young adults. General internists play a critical role in bridging this gap. Internists can provide holistic care to AYA patients thru awareness of their unique needs and ensuring communication with the multiple subspecialists involved. We hope clinicians will consider earlier referral to Palliative Medicine to assist their patients with advanced care planning. As the disease progresses, regular visits help elucidate personal values and provide strategies to mitigate social, spiritual, psychological, and physical distress.