Her nervous eyes met mine as I entered the small exam room.

Would I be the person bringing her the results that she so anxiously awaited? I introduced myself and explained to her that I had a few questions and that the physician would be in to see her soon. As we talked, Linda told me the too often heard story of a routine chest x-ray before surgery, and suddenly, the anxiety over having a gallbladder removed was overshadowed by the news that the x-ray revealed a large lung mass. A CT scan and then biopsy were performed after her recovery from surgery, and now she was at our multidisciplinary clinic awaiting the results.

We were interrupted by a knock on the door, and one of the physicians strode into the room. He wasted little time as he explained to her that she had lung cancer, and it was his opinion that it had most likely spread and that further surgical work-up was needed. “Do you have any questions?” he asked, almost as an afterthought. Her shocked expression quickly changed to tears. The physician continued speaking about her plan of care as the tears continued to fall. He concluded with “Do you understand?” and she silently nodded her head, but from her dazed expression it was apparent to me that she had heard nothing beyond, “You have lung cancer.”

The cardiothoracic surgeon came in next, and he seemed to understand that she could take in very little. As he sat and explained, he looked to me for confirmation that I would help answer the questions that he knew would come later. After he left the room, I spent additional time explaining the next steps that we would take and my role as guide in helping her on the journey she was about to begin.

The next few weeks allowed me to get to know Linda much better. Conversations centered on her lung cancer, and her plan of care gradually gave way to sharing her life story. A single mother with a young daughter, she was estranged from her family, and she worked as a waitress to support herself and her daughter. She had worried about paying her bills when she was healthy, and now medical bills threatened to overwhelm her.

Trust was built as we began to work through her concerns one by one, obtaining financial assistance to cover her care, finding resources to help her daughter understand and process her mother’s diagnosis, and referrals to behavioral health to assess her anxiety and fear. In addition, I shared clinical information about her diagnosis, and expected treatment.

The day of her outpatient procedure came, and when I wasn’t able to reach her in the evening I checked our EMR and found her name on the ICU patient list. An unexpected EKG abnormality and subsequent surgery found fluid around her heart. When I spoke to the cardiothoracic surgeon later that day, he told me he had found evidence of cancer within the fluid and even on the surface of her heart. It was apparent that her disease was much more extensive than we had originally thought.

I wasn’t sure what to expect as I entered her room, but her sweet smile was still in place and her hand found mine as she whispered, “Hello.” Over the next few days I visited with her daily in the ICU and then on the pulmonary floor. Confined to the bed and now requiring high levels of oxygen to maintain her saturations, our visits were sometimes spent sitting quietly, while other times she spoke of her estranged family and how much she missed them—her daughter, their life, the unfairness of this diagnosis, and even her belief in God. Waiting for her to take the lead in the conversations allowed her to be in control when she was in control of very little else.

	Providing Autonomy, Respect, and Dignity

Several days later as I entered her room, I found two men sitting at her bedside who introduced themselves as her brother and her nephew. She had reached out to them, and 15 years of estrangement fell away. They had caught an immediate flight to be with her. As they excused themselves to go get something to eat, she indicated the chair next to the bed, and I sat down. “You know they want me to start treatment soon, don’t you?” she asked. I nodded and sat quietly. “I don’t want to do that,” she finally said.

Her voice was low and quiet as she whispered, “I want to go home.” “Home to your house?” I questioned. “No, I want to go home to California where my family is. I want my daughter to be with my family.”

Promising nothing I decided to have a conversation with her admitting physician, a pulmonologist who I knew only slightly. He listened but said, “You know that the oncologists want to start treatment?” “I know, but what she wants is to go home to California.” He hesitated for a second and then said, “You won’t find an airline willing to fly her there.” “But if I can?” I persisted. “Then I will write the orders,” he said.

The process took two days, but in that time I found an airline willing to accommodate her oxygen needs and a private donor willing to pay for her flight. Arrangements were made quickly, and her discharge date was set. I went to say goodbye to her. She hugged me and thanked me: “Don’t say goodbye; say until we meet again because I know we will.” My eyes filled with tears but I managed, “Until we meet again.”

Linda made it to California and was reunited with all of her family. She was able to place her daughter in their care and know that she would be well-cared for. Two weeks later, Linda died with her family at her side having made all of her own decisions, maintaining her dignity and autonomy, the very essence of person-centered care.

Kathleen A. Gamblin is Coordinator, Oncology Patient Navigation, at the Northside Hospital Cancer Institute in Atlanta.

Articles

Feature Story

 Linda told me the too often heard story of a routine chest x-ray before surgery, and suddenly, the anxiety over having a gallbladder removed was overshadowed by the news that the x-ray revealed a large lung mass.

Building Trust and Giving Voice to the Patient's Wishes

When patients with multiple myeloma relapse or become refractory to prior therapy, FDA approval of additional therapeutic options such as pomalidomide and carfilzomib was certainly welcome news. But for healthcare teams treating patients in the relapsed/refractory setting, careful monitoring remains essential, along with taking a proactive approach in urging patients to communicate any problems they are experiencing, so that side effects can be effectively managed without dose reductions or treatment interruptions.

This was the takeaway message emphasized in a recent interview with Nurse Practitioner Ann McNeill, RN, APN, who outlined important clinical considerations for nurses and other clinicians working in the multiple myeloma setting.

McNeill works in the myeloma division at the John Theurer Cancer Center in Hackensack, New Jersey, where she is an integral member of a team managing patients throughout their treatment cycles, including those who have become resistant to bortezomib-based regimens and whose disease has progressed.

Pomalidomide and carfilzomib are the two newest FDA-approved agents for these patients, and although the therapies are generally well tolerated, McNeill explained, it is critical that patients be educated about the importance of communicating any treatment- or disease-related issues with clinicians:

“When they report back to us and communicate with us, we can then manage appropriately to keep them on therapy for extended periods.”

McNeill said that at her center, for those patients on pomalidomide therapy are frequently and regularly monitored. The policy is to see patients at least every other week—and often weekly&mdash;for the first 2 months.

“Patients know that they have to get frequent checks of their blood counts. We look for significant drops in their neutrophil counts or their platelet counts, or even their hemoglobin, and manage appropriately.” There are a number of issues her team looks out for which are very specific to the agents used to treat patients with relapsed/refractory disease.

These can include thrombocytopenia with the proteasome inhibitors, neutropenia with the immunomodulatory drugs (IMiDs), and peripheral neuropathy with bortezomib and thalidomide. Gastrointestinal changes most typically manifest as diarrhea and/or constipation, rather than nausea or vomiting.

“Fatigue is something that we see very commonly, regardless of the treatment option,” said McNeill. Although fatigue may be related to treatment, McNeill stressed that it is important to rule out other possible root causes in the myeloma patient, such as anemia, dehydration, inactivity, and depression.

McNeill said that bone disease is a common characteristic of myeloma, affecting about 85% of patients. “Our policy is to use monthly bisphosphonate therapy for newly diagnosed patients with bone disease until they go to transplant, or if they don’t go to transplant, for approximately a year or two,” after which the agents are administered on a much less frequent basis, typically about four times per year.

She added that every patient who is on an IMiDcontaining regimen will have anticoagulation prophylaxis, and the agent chosen will depend on the patient. Patients at low risk for blood clots typically would take a daily 81-mg aspirin. As warranted, her team also uses warfarin, low molecular—weight heparin, and, more recently, rivaroxaban (Xarelto). Despite these preventive measures, “We do see some blood clots, but the rate is very low.”

Pomalidomide is typically only discontinued due to disease progression, said McNeill, rather than unacceptable toxicities. “We are fairly aggressive in managing any toxicities, cytopenias, or GI side effects.” In general, patients are able to stay on their full dose of pomalidomide therapy. McNeill said the typical starting does is 4 mg, with few patients receiving less than 3 mg. If the dose is reduced, she said, it’s usually due to persistent neutropenia.

She added that her team has seen some rare cases of confusion with pomalidomide, but “again, we are always looking to rule out other etiologies for a neurologic symptom before we attribute it to the drug.”

Pomalidomide can be used safely in elderly patients, McNeill assured. “If a patient does present with impaired renal function or other comorbidities, we of course monitor these patients more closely. We may want to see them more frequently,” and this monitoring includes phone calls from the navigators on the team.

“In general, many of these agents are very well tolerated, and the patients know what to report to us. We want to keep these patients on full-dose agents,” she emphasized, “because they usually get good disease control, and that’s our goal.”

At her center, McNeill said nurse practitioners see patients autonomously, meaning they assume a central role in disease education and telling patients what to look for in terms of toxicities.

In her patient education role, McNeil said that it is really important to explain the dosing schedule, when to go for laboratory tests, the type of adverse events patients should look out for, and when to call. She said that all members of the care team, including the navigators and infusion room nurses, are well educated about the various agents used to treat myeloma, their potential side effects, and how to manage them appropriately.

“With any drug, it’s a risk versus benefit analysis,” said McNeill. “Patients who have relapsed/ refractory disease need to stay on treatment, so we want to manage them through these cycles and keep them on the agent for a long period of time.”

When patients with multiple myeloma relapse or become refractory to prior therapy, FDA approval of additional therapeutic options such as pomalidomide and carfilzomib was certainly welcome news.

Clinical Considerations for Managing Refractory Multiple Myeloma

Fatigue is a very distressing symptom experienced by most individuals with cancer.

This symptom has deleterious effects on many aspects of quality of life including physical, psychological, and social well-being. When an individual has fatigue, it can limit his or her ability to function at home and at work, to socialize, and to participate in previously enjoyable activities.

In comparison to the fatigue experienced by healthy individuals, fatigue experienced by cancer patients is different in that it is often not alleviated by rest or sleep.

 Despite the negative impact of cancer-related fatigue, in general it is underreported by patients and underestimated by providers. One challenge in the assessment and treatment of this symptom is the lack of knowledge about fatigue, specifically the mechanism underlying fatigue.

The cause of cancer-related fatigue is complex and multidimensional, involving many potentially contributing elements. These include tumor-related factors and comorbid medical/ psychological conditions and also side effects associated with cancer treatment and other medications.

 Some of the biologic mechanisms that have been investigated include anemia, cytokine dysregulation, hypothalamic—pituitary–adrenal axis dysregulation, 5-hydroxytryptophan neurotransmitter dysregulation, and alterations in adenosine triphosphate and muscle metabolism. The process that has received the most attention is the dysregulation of cytokines, with a focus on proinflammatory cytokines.

The connection between inflammation and fatigue comes from animal studies where inflammatory cytokines can signal central nervous system (CNS) changes and produce fatigue and other symptoms. Investigators have proposed that tumors and the treatments used to treat them can activate the proinflammatory cytokine network, leading to symptoms of fatigue via cytokine signaling in the CNS.

The inflammatory response can persist long after treatment completion. Studies conducted with cancer patients before treatment, during treatment, and after treatment have shown associations between fatigue and inflammatory markers, although results have not been consistent.

 In order to learn more about this important association, longitudinal studies are needed.

Canhua Xiao, PhD, RN, a research assistant professor at Nell Hodgson Woodruff School of Nursing at Emory University is currently studying the link between fatigue and inflammation in patients with head and neck cancer receiving radiotherapy. Patients with head and neck cancer typically receive radiation treatment and have particularly high rates of fatigue which often lasts after therapy is complete. Her study seeks to clarify and broaden the understanding of the association of fatigue with pro- and anti-inflammatory signaling. In addition, this study investigates genetic mechanisms with a longitudinal descriptive design. Patients receiving radiation for head and neck cancer are followed from before radiation therapy begins to 1 year post radiation therapy. Xiao’s work is motivated by the hope that understanding the biologic mechanisms of fatigue will be the key to its successful management and perhaps also will benefit other related symptoms.

Until the mechanisms of fatigue are better understood, clinicians should concentrate on available resources. The National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology recommend that all cancer patients be screened for fatigue at their initial clinical visit, and then regularly during and after their cancer treatment has been completed.

 One unique challenge in the assessment of fatigue is that a patient’s perception of fatigue can change before, during, and after treatment, meaning that fatigue scores cannot be compared throughout the treatment continuum.

Once it has been determined that a patient is experiencing fatigue, the next step is to determine if there are any potentially treatable factors that could have contributed to the development of cancer-related fatigue, such as anemia, pain, emotional distress, sleep disturbance, malnutrition, medication side effects, and comorbidities.

However, even after treating these conditions, a significant proportion of cancer patients will continue to experience fatigue. For these individuals, intervention with additional therapeutic modalities may be required.

Pharmaceutical agents to treat fatigue have been studied but none have proved to reliably prevent or treat this symptom.

 A variety of nonpharmacologic interventions have shown potentially beneficial effects including physical activity, psychosocial support, and mind—body treatments.

Specific interventions such as cognitive-behavior approaches, acupuncture, mindfulness-based yoga, and biofield therapies have shown promise but need further investigation.3 Because fatigue is such a distressing symptom, clinicians and researchers must continue to work to understand the mechanisms behind this challenging symptom and determine the most effective treatments.

		Koornstra RH, Peters M, Donofrio S, et al. Management of fatigue in patients with cancer—a practical overview. Cancer Treat Rev. 2014;40(6):791- 799.

		Mustian KM, Morrow GR, Carroll JK, et al. Integrative nonpharmacologic behavioral interventions for the management of cancer-related fatigue. Oncologist. 2007;12(suppl 1):52-67.

		Bower JE. Cancer-related fatigue-mechanisms, risk factors, and treatments [published online August 12, 2014]. Nat Rev Clin Oncol.

		Mock V, Atkinson A, Barsevick A, et al. NCCN Practice Guidelines for Cancer-Related Fatigue. Oncology (Williston Park). 2000;14(11A):151-161.

		Minton O, Richardson A, Sharpe M, et al. Drug therapy for the management of cancer-related fatigue. Cochrane Database Syst Rev. 2010;7(7):CD006704.

Partner Perspectives

Cancer-Related Fatigue-Complex, Multidimensional, and Challenging to Treat

		Lisa Schulmeister, RN, MN, APRN-BC, OCN®, FAAN

Oncology Nursing Consultant, Adjunct Assistant Professor of Nursing

		Louisiana State Health Sciences Center in New Orleans, Louisiana

My eye doctor recently added an extra wide chair in his waiting room, which got me thinking about the prevalence of obesity. According to the CDC, 35% of adults are obese (body mass index [BMI] ≥30 kg/m2) and 69% are overweight (BMI ≥25 kg/m2, and includes the obese).You would think that obesity would be more socially acceptable now, since it’s so prevalent; however, this is not the case and apparently healthcare providers are among the worst offenders for being weight biased.

Weight bias can be expressed nonverbally (eg, rolling eyes) as well as verbally via derogatory comments and jokes, which really aren’t funny when considering that weight-related stigmas and discrimination reduce quality of life and have long-term consequences for emotional and physical health.

Although we would like to think that healthcare providers are nonjudgmental, we’re not. In several studies, it’s been suggested that physicians and nurses across a range of specialty areas harbor negative stereotypes toward obesity, such as perceptions that patients who are obese lack self-control, are unmotivated or lazy, and are more likely to be noncompliant with treatment (interestingly, researchers have not found a correlation between obesity and noncompliance).

Medical and nursing students bring weight bias with them when they enter school. Researchers have found that the majority of these students possessed both explicit (outwardly expressed) and implicit (automatic, unconscious) weight biases. Many students expressed frustration about caring for patients who are obese, viewed them to be difficult to deal with, and were pessimistic that obesity counseling and treatment could be successful.

In addition, the students reported observing weight bias frequently in the clinical setting; 63% of students had witnessed other students making jokes or negative comments about patients with obesity, 40% had observed instructors making negative comments, and 65% had witnessed healthcare providers making negative comments.

What’s troubling about this statistic is that when combined with data on healthcare utilization that found that people affected by obesity tend to avoid preventive healthcare services, it’s the perfect set-up for delayed diagnosis of disease and missed opportunities for screening and early detection. In fact, one study found that women avoided or delayed routine gynecologic care not because of fear of finding something wrong, but rather, because of fear and shame associated with being put on a scale.

Weight bias is a clinical concern that cannot be ignored. First and foremost, as clinicians we need to examine our own perceptions about people who are overweight or obese (it may also be helpful to consider if negative stereotypes also exist about patients who are significantly underweight). We need to think about how we express these perceptions and consider our actions in the healthcare setting. All too often, the patient’s weight is not discussed at all; consequently, an opportunity to provide health education is missed.

Weight-related discussions, which really are health-related discussions, need to occur in a safe and supportive environment for both the patient and healthcare provider. We need to be educated about complex etiology of obesity and its biological and genetic factors, and be aware of the challenges of achieving significant long-term weight loss. We also need to do a better job talking about obesity and engage in proactive and supportive communication to elicit health behavior change. Lastly, as role models for students entering the healthcare professions, we need to be thoughtful about how we describe our patients and need to avoid perpetuating weight bias and negative stereotypes.

One strategy is to use what’s called “people-first” language. We already do this in oncology care; for instance, we take care of people with cancer and not “cancer patients.” Similarly, terms such as “obese patient” need to be replaced by people-first language, such as "people with obesity" or "individuals affected by obesity."

Oftentimes, we forgo opportunities to intervene and provide health education about obesity because of our lack of knowledge. Educational resources on weight bias in the healthcare setting, including videos, toolkits, informational handouts, and continuing medical education, are available at the Yale Rudd Center for Food Policy and Obesity (www.yaleruddcenter.org). If every one of us took the time to better educate ourselves about the epidemic of obesity and became more sensitive and skilled in addressing obesity, the health of America would surely improve. 

Nurse's Notes

My eye doctor recently added an extra wide chair in his waiting room, which got me thinking about the prevalence of obesity.

Weight Bias: A Clinical Concern That Cannot Be Ignored

The demand for genetic services has never been greater. Vast advances in genetic technology, Angelina Jolie’s disclosure that she is a BRCA mutation carrier, and the Supreme Court ruling on gene patents have hurled genetic services into the mainstream. Since the Supreme Court ruling last year, the cost of germline (hereditary) genetic testing has plummeted and now includes panels of genes.

The error rates in ordering and interpreting genetic testing were already high, resulting in incorrect recommendations for surveillance—and even prophylactic surgeries&mdash;across the country.  The move from single gene testing to multigene panels will, unfortunately, drive this error rate even higher.

Add to this a genetic testing laboratory, desperate to retain a share of the lucrative BRCA testing market, has launched a campaign recruiting mammography technicians with little if any education in genetics, to order genetic testing themselves, and it is easy to predict that the error rate will increase even further. 

Some insurance companies, including Cigna, have reacted to the mass misordering and misinterpretation of genetic testing by requiring that their members receive genetic counseling by a certified professional 

 having certain types of genetic tests. It is not difficult to see how all of these events will cause a logjam in genetics services. 

Many premier health centers recognize the dire need for genetics services and are expanding their teams of certified genetic counselors to handle these needs.  However, even with these increases, many centers have a wait time of many weeks or months for nonurgent referrals.  What can we do to help ease these needs?

Increase the nurse’s role in genetic evaluation

Nurses often have close, repeated contact with patients and their families.  They are perfectly positioned to:

		Take a detailed personal and family history of cancer

		Determine which patients require a referral to a certified genetic counselor and facilitate those referrals

		Discuss the basic pros and cons of genetic testing with the patient, including the benefits for other family members

		Dispel myths that often prevent patients from having genetic testing in the first place (

eg, my health insurance will discriminate against me; my insurance won’t cover testing; testing involves a painful, invasive procedure; my only option if I test positive is prophylactic surgery).

		Nurses can increase their knowledge of somatic 

 testing, which will be used more and more to guide personalized medical treatment. Such testing can also uncover underlying germline (hereditary) genetic mutations, and the nurse can facilitate a referral for genetic counseling when such findings arise. When genetic mutations are found that are 

 in the tumor, and are not hereditary, nurses can help explain these complex findings to patients and their families.

Utilize phone counseling for patients who cannot access in-person counseling

Due to distance, long wait times, preference, or because the patient is unable or unwilling to travel, some patients may be better served by utilizing a certified genetic counselor by telephone. There are now several reputable services available that accept health insurance, including:

Understand direct-to-consumer (DTC) genetic testing services

There has been great resistance by healthcare providers to embrace DTC genetic testing services due to concerns about the quality of testing and reporting and the nature of testing offered (SNP-based risk estimates vs. mutation-based; tests with known vs questionable scientific validity and utility).

Some of these concerns have been warranted and have prompted the FDA to examine these practices. These examinations will likely prompt improvements to DTC services.

There is a huge consumer desire for these services, and they will likely continue to increase over time. Oncology nurses can deploy a frontline approach in interpreting DTC testing by:

		Requesting a copy of the patient report

		Deciphering what testing was actually performed (eg, the common Jewish BRCA mutations vs full sequencing of BRCA1 and BRCA2)

		Determining if that patient requires additional testing and a referral to genetic counseling.

		Concluding that if a patient with a striking personal and/or family history has tested “negative” via DTC testing, the history warrants further evaluation with a genetic counselor.

Genetic services will play an increasingly large role in medical care as we move forward. We will need to continually evaluate new genetic service models while maintaining the highest standards of care to minimize errors and maximize benefits as these exciting new technologies unfold. 

Ellen T. Matloff, MS, CGC, is president & CEO of Gene Counsel in New Haven, Connecticut (www.mygenecounsel.com). 

Genetic Counseling

The demand for genetic services has never been greater. Vast advances in genetic technology, Angelina Jolie's disclosure that she is a BRCA mutation carrier, and the Supreme Court ruling on gene patents have hurled genetic services into the mainstream. Since the Supreme Court ruling last year, the cost of germline (hereditary) genetic testing has plummeted and now includes panels of genes.

September 2014

Building Trust and Giving Voice to the Patient's Wishes

Clinical Considerations for Managing Refractory Multiple Myeloma

Cancer-Related Fatigue-Complex, Multidimensional, and Challenging to Treat

Weight Bias: A Clinical Concern That Cannot Be Ignored

Is it Time to Think Outside the Box?: New Approaches to Genetics Services