Overview. Primary palliative care simply refers to the delivery of essential palliative care to any patient with a serious illness by clinicians without subspecialty training in palliative medicine. The American Society for Clinical Oncology (ASCO) Practice Guideline Update on the Integration of Palliative Care into Standard Oncology Care, based on an expert panel using evidence from nine randomized controlled trials, one quasi-experimental trial, and secondary analyses of five randomized controlled trials, cites its recommendation for the essential components of palliative care for people with advanced cancer (Table 2).
Table 2. Essential Components of Palliative Care
| • Rapport and relationship building with the patient and family caregivers |
| • Symptom, distress, and functional status management (eg, pain, dyspnea, fatigue, sleep disturbance, mood, nausea, constipation) |
| • Exploration of understanding and education about illness and prognosis |
| • Clarification of treatment goals |
| • Assessment and support of coping needs |
| • Assistance with medical decision-making |
| • Coordination with other care providers |
| • Provision of referrals to other care providers, as indicated |
All clinicians should be experts in the assessment and management of common physical symptoms, such as pain, nausea, fatigue, anxiety, and depression, and should have excellent communication skills requisite to initiating and guiding patients and their caregivers through iterative discussions about illness understanding, prognosis, goals of care, and adjusting care plans accordingly. All clinicians should be highly competent in providing palliative care, not only because the demand for specialty palliative care clinicians far exceeds their availability, but because patients significantly benefit from receiving such care and guidance from their treating clinicians with whom they already have formed a strong relationship. This is particularly true for people with CNS cancer who receive care from multiple specialists and rely on their guidance about symptom management, psychosocial needs, and addressing advanced care planning and goals of care, especially as their disease progresses. Of note, supportive care largely arose out of the need to provide adults receiving cancer treatment with management of treatment- and disease-related toxicities and symptoms. Although known to be used in lieu of palliative care due to the concerns of clinicians, patients, and their families conflating specialty palliative care with hospice care, supportive care programs may focus largely on symptom and toxicity management without specialty training or certification in palliative medicine, or they may practice within an interdisciplinary team approach and, therefore, are not equivalent to specialty palliative care.
Symptom management. Neuro-oncology clinicians should regularly monitor their patients for new and worsening symptoms and refer patients with refractory or complex symptoms to specialty palliative care. Symptom management is imperative as uncontrolled symptoms worsen quality of life and survival. Instruments have been developed to enable patients to self-report their symptoms and merit wider use. Although clinical factors, such as tumor location, stage, age, functional status, and comorbidities, impact individual symptom burden and neurologic dysfunction, the following addresses common physical symptoms in the adult CNS cancer population and their management.
Fatigue is the most common symptom among all people with cancer, including those with CNS cancer. Its etiology has been attributed to multiple factors: cancer itself, the associated inflammatory cytokines, chemotherapy, radiation therapy, acute and chronic stress, psychological and social distress, medication side effects, sleep disturbance, and changes in activity level, among others (01). The fatigue people experience can be unlike anything they have ever felt. It can be quite debilitating and severely limit their daily functioning. The first step in its management is to assess and address as many of these contributing factors as possible as no medications to date have been found to effectively alleviate fatigue. As in the general cancer patient population, the most efficacious interventions may be energy conservation during intensive treatment, then adding back exercise through increasing daily activity and integrating an exercise regimen, such as was prescribed during physical therapy. However, this may be challenging for those who have neurocognitive dysfunction or significant paresis. Identifying and addressing distress using supportive and psychosocial care resources are key. Optimizing the timing and dosing of supportive medications based on their side effect profiles, such as taking dexamethasone before 3 PM and taking sedating antiemetics in the evening or at bedtime, can help achieve restorative sleep.
Headaches are highly prevalent among people with CNS cancer. Headaches may be worse at initial diagnosis, during radiotherapy, at disease recurrence or progression, and at the end of life, though they may start or worsen at any time through the illness trajectory. A cardinal sign of increased intracranial pressure, hemorrhage, or leptomeningeal disease, headaches can have various other etiologies, such as post-craniotomy muscle spasms or neuralgia, temporomandibular joint dysfunction, muscle tension, referred pain from the skull base, migraine, medication side effects, dehydration, sleep disturbances, carbon dioxide retention, and NSAID or caffeine overuse. Chronic headache management in neuro-oncology does not significantly differ from general neurology practice other than more frequently prescribing dexamethasone to manage vasogenic edema as the tumor burden increases (21). Migraine prophylaxis with gabapentin, pregabalin, duloxetine, or a tricyclic antidepressant is advised with a reasonable abortive regimen (NSAIDs, antiemetic, hydration, etc.). When choosing a prophylactic medication, it is particularly important to consider its entire effect profile to leverage the use of those advantageous to the patient (choosing valproic acid if seizures and mood lability are comorbid symptoms) and to defer on those likely to cause cognitive dysfunction or sedation. Judicious use of dexamethasone is recommended given its myriad of untoward side effects (07).
Nausea may be related to the disease process, especially if advanced, but it is more commonly a side effect of tumor-directed treatment or medications. One of the most feared and common adverse effects of cancer treatment, chemotherapy-induced nausea and vomiting (CINV), can cause significant worsening of patients’ quality of life. The American Society for Clinical Oncology’s antiemetic guideline provides recommendations on anticancer agent and radiotherapy emetogenicity and respective antiemetic regimens as well as adjunctive drugs, cannabinoids, complementary and alternative therapies, and breakthrough and anticipatory nausea and vomiting (14). Of note, temozolomide’s emetogenic potential is dose dependent; doses up to 75 mg/m2 have low, and doses more than 75 mg/m2 have moderate, emetogenic potential (22). Constipation is a well-recognized side effect of both 5-HT3 antagonists and temozolomide; severe constipation may warrant using an antiemetic other than a 5-HT3 antagonist, such as dopamine antagonists and NK1 receptor antagonists. Olanzapine has randomized controlled trial data supporting its use in preventing CINV in the general cancer population (13). However, its use within neuro-oncology is constrained by its potential to exacerbate confusion and sedation.
Psychological distress management. Receiving a diagnosis of CNS cancer is a major life event. People have valid fears about treatment and their prognosis as well as the implications of their illness on nearly every aspect of their life and that of their family members. Anxiety, depressive symptoms, grief reaction, adjustment disorder, major depression, and posttraumatic stress disorders are highly prevalent among people with CNS cancer. In addition, neuropsychiatric effects secondary to direct CNS involvement by cancer and its treatment are common. These cumulative neuropsychological factors have notable detrimental effects on functional status, symptom burden, symptom interference with daily activities, and health-related quality of life for those living with CNS cancer. Patients should be regularly screened for distress along with physical symptoms, and distress should be appropriately assessed and managed when present.
No high-level evidence demonstrating benefit of any specific psychiatric medication for depression or anxiety in the CNS tumor patient population exists; therefore, treatment should follow standard clinical guidelines. Notable specific drug-drug and drug-cancer interactions and contraindications have been thoroughly reviewed elsewhere (18; 24; Yap et al 2011; 06; 12; 23). A selective serotonin or norepinephrine reuptake inhibitor, such as citalopram, escitalopram, or sertraline, may be considered in addition to facilitating the receipt of mental health services, stopping medications with untoward mood effects, optimizing sleep, and encouraging exercise, as feasible. People with CNS cancer and their close others should be encouraged early after diagnosis to establish care with therapists or counselors who are specifically trained in serious illness and grief. Support groups available locally, in-person, or virtually through national patient advocacy organizations, such as the American Brain Tumor Association, may also be very helpful for patients and caregivers. For those with high psychological distress or high or complex symptom burdens, early referral to specialty palliative care for consultation is recommended.
Advanced care planning and discussing prognosis and goals of care. Strong communication skills are crucial for all clinicians caring for people with CNS cancer given the many sensitive and difficult conversations that need to be held throughout the course of the illness. There are multiple discussions that must be conducted with great transparency, directness, and compassion, such as breaking the news of the diagnosis, deciding on treatment options, designating a durable healthcare power of attorney, and iterative discussions about prognosis and goals of care, especially when the disease has progressed. Although the involvement and assistance of specialist palliative care may be helpful or needed at certain junctures, it is critically important that primary teams have high-quality communication with patients and their caregivers. Primary teams have the advantage of a strong relationship built over time with the patient, which also allows for gaining a sense of patients’ values, hopes, and priorities, in addition to knowing their disease trajectory and the anticipated course. Learning how to empathetically respond to emotion, deliver serious news, and discuss transitions in goals of care are requisite skills for all clinicians.
Receiving a CNS cancer diagnosis is overwhelming and extremely distressing for people and their loved ones. Initial visits with the primary team often focus on delivering important medical information about the tumor, discussing treatment options, and deciding on a treatment plan. In this time of distress and anxiety, people and their caregivers seek information on prognosis and what to expect across the disease trajectory. Conversations about these topics should start by first asking how much information the patient and their family members prefer to receive; some may be detail-oriented and want to know lots of information, and others might prefer to only receive specific information. Further, caregivers often have different informational needs than patients. For those who want to discuss prognosis in detail, providing prognosis in time ranges (eg, years, months to years, weeks to months, days to weeks, hours to days) and setting the expectation that discussions about prognosis are an ongoing dialogue that will be readdressed as events necessitate are recommended. People and their caregivers should anticipate revisiting this topic during future visits, and clinicians should feel comfortable openly addressing the re-evaluation of prognosis when there is radiographic disease reoccurrence or progression, clinical progression (worsening neurologic or functional status), or significant disease or treatment complications.
When discussing advanced care plans and goals of care, it is important to first elicit concerns and respond empathetically to foster emotional processing and then to address concerns with appropriate information. Next, elicit goals, such as attending a wedding or reconciling an important relationship, and values by asking open-ended questions about what their biggest goals are and what in their life is most valuable to them right now. Next, clinicians can maintain hope in balance with reality by inquiring about what they are hoping for and providing honest yet kind and compassionate responses by acknowledging any unachievable hopes and hopes that could be fulfilled, such as relieving a symptom or spending time with someone, and what care is available to provide emotional and practical support, symptom control, preservation of autonomy, and dignity. Encourage people to state their wishes for their care, and use this opportunity to explain that there is care available no matter what happens. This avoids the false dichotomy that unless cancer-directed treatment is given, there is no care available and that they face abandonment and have no healthcare options if they do not receive cancer treatment, which is untrue. Furthermore, this is an optimal time to address the potential to refer to specialty palliative care in the case of any significant quality-of-life concerns, high or complex symptom burden, or high support needs and to explain that hospice care is end of life care that focuses on quality of life and that maximizing symptom management is available when tumor-directed care is no longer desired or feasible. In the context of this discussion, clinicians should then ask what the person would find acceptable or unacceptable in terms of their quality of life by asking whether there is a degree of disability they believe they could not cope with? Are there symptoms that they would find tolerable (eg, severe pain, unrelenting nausea)? How do they feel about prolonged hospitalization, or a nursing facility stay? Would they accept artificial feeding for a short period of time, or indefinitely? What is their understanding of resuscitation if they die in the hospital?
The modern conceptualizing of advanced care planning includes caregivers hearing the patients’ responses as it is likely the patient will lack decision-making capacity late in their disease trajectory, and the caregiver will be asked to relay their understanding of the patient’s wishes. Also, patients should designate a medical/healthcare durable power of attorney (DPOA) early in the course of the disease; the DPOA may not be the caregiver. If no DPOA is designated, the state law indicates who the legal next of kin is, usually starting with a legal spouse or parent. Several toolkits and guides exist that can guide people and their medical DPOA to have advanced care planning conversations, including The Conversation Project, Five Wishes, etc.
In summary, the basic tenets of communication in delivering primary palliative care in neuro-oncology practice include the following:
A. Eliciting patients’ preferences around receiving information. |
B. Transparent, compassionate, high-quality communication about prognosis that provides the needed guidance patients and their caregivers seek. |
C. Iteratively eliciting patients’ values, hopes, and goals as well as acceptable limits for medical interventions. |
D. Ensuring the patient has identified and legally established a durable medical power of attorney early in the course of the disease who knows them well and can be part of the conversations about what they would want and not want in advanced stages of illness and at the end of life. |
