When some 30,000 oncology practitioners, researchers, and patient advocates gather each year for the annual meeting of the American Society of Clinical Oncology (ASCO), we’ve come to count on some pretty important research updates emerging from that gathering. This year’s meeting—ASCO’s 51st&mdash;proved no exception.

In this month’s cover story, we present some highlights of the meeting, alongside perspectives written by your peers on the impact these developments may have on your practice.

Progress on the immunotherapy front continued to grab headlines at ASCO, among them, nivolumab demonstrating superiority over docetaxel in the treatment of a number of lung cancer subtypes and pembrolizumab showing real promise in the treatment of hard-to-treat advanced head and neck cancer. ASCO brought more good news on the survivorship front, with researchers reporting that survivors of childhood cancers are living longer due to better treatments and improved follow-up care. We cover all of these developments and more in our special 2-credit CE activity, beginning on page 26.

Our editorial team also was on hand for the 9th Annual Joining FORCES Against Hereditary Cancer Conference, held this year in Philadelphia. In this issue, we share expert insights on what the explosion in genetic testing options means for your patients. We also spoke with FORCE’s executive director about the group’s patient-powered ABOUT research registry that’s capturing real-world information from those directly or indirectly affected by hereditary cancer risk. Be sure to visit the OncNurseTV portal on Nursing.OncLive.com for more coverage of the FORCE meeting.

Also in this issue, we hear from oncology nurse navigator and Oncology Nursing News board member Penny Daugherty, who found herself in the unenviable position of switching places when her husband was diagnosed with melanoma. That experience allowed Daugherty to witness firsthand the difference a navigator can make: “After my experience as a wife who was by her husband’s side through his cancer diagnosis and surgery, I can say that I now fully understand the breadth—and the depth&mdash;of our role as nurse navigators.”

We couldn’t agree more, and we hope that you will find the research highlights and peer perspectives you find in this issue helpful in your daily practice.

Articles

From the Chairman

When some 30,000 oncology practitioners, researchers, and patient advocates gather each year for the annual meeting of the American Society of Clinical Oncology (ASCO), we've come to count on some pretty important research updates emerging from that gathering. This year's meeting-ASCO's 51st-proved no exception.


Bringing You the Latest Updates From the Year's Largest Oncology Conference

An increasing number of patients are presenting with peritoneal carcinomatosis, or widespread metastasis of the peritoneum, which can result from a variety of tumors, including digestive and gynecologic malignancies.

 The diagnosis is devastating, with treatment often limited to palliation of symptoms, but a combination of cytoreductive surgical resection (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC)—sometimes referred to as “hot chemotherapy bath”&mdash;has the potential to provide some patients with long-term survival.

This treatment approach is not new. According to Sanjay S. Reddy, MD, a surgical oncologist at Fox Chase Cancer Center, it began back in the 1970s with the use of intraperitoneal chemotherapy for the treatment of ovarian cancer.

“As advances in medicine have continued, more and more patients have undergone this type of therapy for treatment of a wide array of cancers,” he said in an interview with 

. However, despite being around for decades, the procedure continues to be extensive, recovery remains difficult, and there is still no guarantee of improved survival. Reports have shown both success and failure, leading the oncology community to more closely examine which patients are appropriate candidates for this treatment, the techniques involved, and how to better care for patients undergoing this treatment.

Hot Chemotherapy Bath: Standardizing Treatment

HIPEC is administered after CRS, with CRS attempting to remove all visible disease from the abdomen and HIPEC targeting any microscopic disease. A variety of chemotherapeutic agents can be used, depending on tumor type.

According to Jesus Esquivel, MD, national director for HIPEC research, Department of Surgical Oncology, Cancer Treatment Centers of America, mitomycin C is the most frequently used drug for gastrointestinal malignancies, and a platinum-based agent is preferred for gynecologic malignancies. The selected agent is heated before it is infused into the abdominal cavity. The heat is thought to help the chemotherapeutic agent kill any residual cancer cells, and flooding the abdomen with the agent is thought to expose cancer cells to higher concentrations of the selected drug than can be achieved with systemic chemotherapy. After the drug has been deemed to sufficiently circulate through the abdomen (30-120 minutes), it is drained from the body, anastomoses are performed, and the abdomen is closed.

Patients may go on to receive systemic chemotherapy, particularly if they have more extensive or aggressive disease.

HIPEC has been criticized for its lack of standardization, with the literature showing variations in the seven parameters used to characterize the procedure: (1) method, (2) inflow temperature, (3) perfusate volume, (4) drug used, (5) dosage, (6) timing of drug delivery, and (7) total perfusion time.

 Randomized controlled trials are still lacking to shed light on these issues; however, in 2009, an organization was started—the American Society of Peritoneal Surface Malignancies (ASPSM)&mdash;to improve the overall care of patients with peritoneal surface malignancies, and it set HIPEC standardization as its first goal.

The organization strives to foster research collaboration between medical and surgical oncologists at cancer centers that provide HIPEC, and it published its first recommendations in 2014, focusing on the delivery of HIPEC to patients with peritoneal carcinomatosis from colorectal cancer.

 According to Esquivel, who is on the ASPSM’s board of directors, most institutions now follow the ASPSM’s recommendations, leading to more uniformity in how HIPEC is provided.

Although controversy has surrounded various aspects of HIPEC, one matter on which the oncology community agrees is that proper patient selection is essential for success. According to Bassel El-Rayes, MD, director, Gastrointestinal Oncology Translational Research Program, Winship Cancer Institute, three key factors must be considered when making the determination: origin of the tumor, extent of disease, and tumor histology.

“Patients with tumors that are aggressive or have metastasized are not good candidates for this treatment, regardless of where their tumor originated,” said El-Rayes. In a recently published paper he coauthored, the CRS/HIPEC combination showed good results in patients with primary appendiceal mucinous adenocarcinomas.

 El-Rayes said that the results are likely to be favorable for other low-grade tumors and that the evidence for its use in treating higher grade tumors is lacking, though the role may evolve.

Esquivel, who has performed more than 500 CRS/HIPEC procedures, concurs. He said that his ideal patient is one with “a limited amount of peritoneal carcinomatosis and a diagnosis of a not very aggressive tumor, like mucinous tumors of the appendix, ovary, or colon,” noting that the results for more aggressive tumors like sarcomas and pancreatic and gastric tumors are not as good. He explained that the outcome of patients undergoing CRS and HIPEC is directly related to the burden of disease and the histology of the tumor.

“For many patients with low-grade tumors, like pseudomyxoma peritonei of the appendix, this procedure can be done with curative intent. For patients with colon cancer with peritoneal carcinomatosis, the outcomes continue to improve as systemic chemotherapy gets better,” Esquivel said. He also noted that patients now have a median survival of about 3 or more years, whereas it was only 12 months a decade ago when systemic chemotherapy was used alone.

Helping Patients Understand Hot Chemotherapy Bath

When this treatment approach is used, patients need to understand that recovery will take time because of how extensive the procedure is. One recent study that examined quality of life in patients receiving HIPEC for colorectal cancer showed that functional status declined the first 3 months postoperatively, but returned to or exceeded baseline levels over the next 3 to 6 months, with emotional wellbeing improving throughout the first 12 months following surgery.

Because patients receiving HIPEC have complex care needs on their road to recovery, outstanding nursing care is essential, explained Reddy.

“Most patients come out of the operating room with a central line, arterial line, nasogastric decompressive tubes, Foley catheters, and various other drains,” he said, noting that the nasogastric tubes are typically left in for some time because of a high incidence of profound ileus following surgery.

In addition to properly managing all of this equipment, nurses can help improve and potentially speed patients’ recovery.

“Just like with any operation, the more proactive patients are about walking and deep breathing, the quicker the recovery they will make,” said Reddy. But this is not an easy task because inflammation, the metabolic stress response, and postoperative pain are known to impair a patient’s ability or motivation to get up and move.

In addition, for HIPEC patients, the systemic effects of intra-abdominal chemotherapy begin to manifest around postoperative days 6 and 7, Reddy noted. During this period, patients may develop pancytopenia and other complications. Nurses can help promptly identify and manage these and other obstacles on the patient’s road to recovery and communicate any complications, challenges, and progress to the care team.

“Communication between the physician, nursing team, and other ancillary staff is most important,” Reddy said. “Attention to details is essential for a smooth hospital course.”

 asked two nurses what their experience has been treating patients who have received HIPEC. As their perspectives show, outcomes can be quite different, but care can be improved by focusing on the needs of the whole patient.

Julie Christensen, BSN, RN, is a Physician Practice/HIPEC coordinator at the Cancer Treatment Centers of America. Theresa McDonnell, ACNP-BC, is nursing director for the cancer center's outpatient clinic at Massachusetts General Hospital.

What outcomes have you observed for patients who have undergone hot chemotherapy bath, and do you think they are improving? If so, what do you attribute this to?

: As a registered nurse who has cared for patients who have undergone HIPEC through the past 4 years, I have seen this procedure enhance the quality of life and lengthen life. This surgical offering provides hope for patients with advanced-stage cancers who have few other treatment options available to them. I think outcomes continue to improve because technology is improving. We are in an amazing period for cancer treatment as advancements in surgery and critical care are happening every day.

 was published in 2011, I have seen one success story and unfortunately more failures. The success was in a young woman who had limited peritoneal disease. I think that careful selection of patients is important for improving outcomes because currently those with less extensive disease appear to do better.

Do nurses face any unique challenges when caring for these patients?

: Nurses face many challenges when caring for surgical oncology patients. This includes caring for the physical, spiritual, and emotional needs of the patient and family. It is important for the nurse to be aware of all of these challenges and gear care toward each specific patient. We must treat the patient as a whole, not just the cancer. And pain control, as with any surgery, can be a unique challenge as well, due to the fact that every person experiences pain differently.

: The psychosocial aspects around recovery and managing the disappointment and sadness when there is recurrence are unique challenges to this population.

How long are HIPEC patients generally hospitalized, and what is the recovery like?

: Patients who undergo HIPEC may be in the hospital between 6 to 14 days. However, the exact number of days varies by patient because there are several factors to consider, including comorbidities, extent of disease, and the recovery period. The typical recovery time is not too extensive and patients are encouraged to get up and walk (with assistance) within 12 hours of surgery.

: It varies by patient, but we are seeing improvement in length of stay and acuity.

What do you think nurses should know when it comes to caring for patients who have undergone this therapy, both in the immediate postoperative period and more long-term?

: Nurses should know that HIPEC is an invasive procedure and every patient will recover differently. It is important to consider the whole patient when providing care. We as nurses are truly blessed to be able to comfort and care for these families on their difficult journey with cancer.

: From an oncology nursing perspective it’s important to understand the potential postoperative complications as well as the potential for recurrence.

			Chia CS, Tan WJ, Wong JF, et al. Quality of life in patients with peritoneal surface malignancies after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. 

			Esquivel J. Current status and future directions of hyperthermic intraperitoneal chemotherapy (HIPEC). http://www.interventionaloncology360.com/content/current-status-and-future-directions-hyperthermic-intraperitoneal-chemotherapy-hipec. Published June 23, 2104. Accessed July 14, 2015.

Feature Story

The diagnosis of peritoneal carcinomatosisis can be devastating, but a combination of cytoreductive surgical resection and hyperthermic intraperitoneal chemotherapy has the potential to provide some patients with long-term survival.

Hyperthermic Intraperitoneal Chemotherapy: Moving Toward Improved Outcomes

Genevieve Ann Hollis is a nurse practitioner in the Department of Radiation Oncology at the Hospital of the University of Pennsylvania and is an Advanced Senior Lecturer-B in the Biobehavioral and Health Sciences Division at the University of Pennsylvania School of Nursing in Philadelphia.

There are approximately 2.8 million prostate cancer (PC) survivors in the United States,

 and 233,000 will be diagnosed with this disease in 2014.

 Approximately 75% to 82% of men diagnosed at age 75 or younger will be treated with surgery, radiation, and/or androgen deprivation therapy (ADT).

 Most of these men will live for many years after completing treatment. Thus, addressing long—term treatment-related toxicities and their impact on health-related quality of life is critical.

At the population level, it is estimated that 52% of all adult men experience erectile dysfunction (ED). Risk factors for ED include coronary artery disease, hypertension, diabetes, depression, neurologic diseases, and associated medications, with advancing age conferring the highest risk.

 Since the incidence of PC also increases with advancing age, many men will have ED and/or its risk factors prior to PC diagnosis. Despite advances in surgical and radiation therapy techniques, a commonly dreaded toxicity of all PC treatments is a markedly increased risk for ED.

A cohort analysis from the multicenter Prostate Cancer Outcomes and Satisfaction With Treatment Quality Assessment (PROSTQA) clearly demonstrated that the ED risk in this patient population is greatly influenced by disease state, treatment modalities, and various baseline patient characteristics.

 Immediately post-prostatectomy virtually all men experience ED that may improve over time, typically by 18-24 months.

 The ED risk increases from 17% pre-prostatectomy to 65% (range 30%-94%) at 2 years after prostatectomy.

In comparison, erectile function is essentially unchanged immediately after radiation therapy but can decline over the subsequent 6-30 months.

 The ED risk increases from 47% pre-external beam radiation therapy (EBRT), to 63% (range 8%-84%) at 2 years after EBRT. Furthermore, the ED risk increases from 33% pre-brachytherapy to 57% (range 8%-84%) at 2 years after brachytherapy.

Younger age, nerve-sparing prostatectomy, and pre-prostatectomy PSA ≤10 is protective in those who undergo prostatectomy. Absence of neoadjuvant ADT and pre-EBRT PSA ≤4 are protective in the EBRT cohort. Younger age, African American race/ethnicity, and lower body mass index were protective in the brachytherapy cohort.

 Men who receive ADT typically have a profound loss of libido and are rarely sexually active.

Our knowledge of how PC treatments contribute to ED continues to evolve, but it clearly is a multifactorial pathogenesis. These factors include damage to the cavernosal nerve bundles that arise from the pelvic plexus, lie lateral and posterior to the prostate, and innervate the highly vascularized corpora cavernosa in the dorsal aspect of the penis. It has been theorized that PC treatments also can cause vascular leakage that ultimately leads to deterioration and fibrosis of the corpora cavernosa structures.

Penile rehabilitation is the use of medical interventions to decrease corpora cavernosa hypoxia and fibrosis to potentially preserve spontaneous erectile function after PC treatment.

 While controversial, advances in understanding how PC treatments impact the physiology of erections have led to increased interest in penile rehabilitation interventions for these patients. The use of phosphodiesterase type 5 (PDE5) inhibitors has gained particular interest due to studies which suggest that these medications are potent protectors of endothelial and smooth muscle tissue in the corpora cavernosa and that they may have neuroprotective and regeneration properties.

Most of the PC penile rehabilitation research has been conducted with men post- prostatectomy. The recognition that cavernosal nerve severance during prostatectomies caused irreversible ED led to nerve-sparing surgical approaches which markedly improved post-prostatectomy ED rates.

 While this nerve dysfunction is likely, the major contributor to ED post prostatectomy, substantial venous leakage and corpora cavernous structure fibrosis also occur in the early post-prostatectomy time period and continues for many months.

Post-prostatectomy studies of therapies that aim to improve penile blood flow include those using vacuum constriction devices (VCD)

 and intracavernosal alprostadil injections,

 and the PDE5 inhibitors sildenafil and vardenafil.

 While most of the studies demonstrated at least short-term improvement in ED rates with these interventions, limitations include use of various interventions; the timing of interventions relative to prostatectomy; intervention schedules ranging from on-demand to daily for 6-12 months; generally small sample sizes; frequent lack of randomization and placebo, and short patient follow-up.

Venous leakage with corpora cavernosa fibrosis is theorized to be the major contributor to ED after radiation therapy and to a lesser degree neural injury.

 Two studies randomized men undergoing treatment with EBRT to sildenafil or placebo for 6 months. While men receiving sildenafil had lower ED rates while taking the medication, this benefit decreased over time and was not sustained when no longer taking the sildenafil.

 Unfortunately a recent randomized controlled study of daily tadalafil or placebo for 6 months in men receiving EBRT or brachytherapy did not demonstrate improved erectile function in the tadalafil arm.

Nurses play a critical role in assisting the PC survivor and his partner in adapting to potential and actual ED. This includes acknowledging that ED is a common concern, providing counseling regarding patient’s individualized risk using the PROSTQA model, and ongoing assessment for the presence of ED using validated self-report tools such as the Expanded Prostate Cancer Index Composite (EPIC-26) or International Index of Erectile Dysfunction (IIEF-5). It is also important to assess for psychological distress, problems with intimacy or relationships, and self-esteem in PC survivors experiencing ED.

Given the findings and limitations of the current evidence, widespread use of early penile rehabilitation to prevent ED is controversial. More studies are needed to more fully understand the underlying pathology as well as determine the most effective interventions and schedules. If ED symptoms are present, these same therapies can be used “on demand” prior to planned sexual activity. It is important to note that not all these interventions are covered by insurance, limiting their availability to many patients outside a clinical trial. Side effects or cardiovascular comorbidity will limit PDE5 inhibitor use by some patients and use of penile pumps or intracavernosal injections will not be acceptable to others. Surgical placement of a penile prosthesis can be considered for refractory ED.

Skolarus TA, Wolf A, Erb NL, et al. American Cancer Society Prostate Cancer Survivorship Care Guidelines. CA Cancer 

Siegel R, Desantis C, Vigo K, et al. Cancer treatment and survivorship statistics CA Cancer 

Feldman HA, Goldstein I, Hatzichristou DG, et al. Impotence and its medical and psychosocial correlates: results of the Massachusetts Men Aging Study.

Alemazaffar M, Regan MM, Cooperberg MR, et al. Prediction of erectile function following treatment for prostate cancer. 

Dall’Era JE, Mills JN, Koul HK, Meacham RB. Penile rehabilitatin after radical prostatectomy: important therapy or wishful thinking? 

Zelefsky MM, Eid JF. Elucidating the etiology of erectile dysfunction after definitive therapy for prostate cancer. 

Jung, J., Jo, H.W., Kwon, H., Jeong, N. Y. (2014). Clinical neuroanatomy and neurotransmitter-mediated regulation of penile erection.

Walsh PC, Donker PJ. Impotence following radical prostatectomy: insight into etiology and prevention. 

Raina R, Agarwal A, Ausmundson S, et al. Early use of vacuum constriction device following radical prostatectomy facilitates early sexual activity and potentially earlier return of erectile function.

Montorsi F, Guazzoni G, Strambi LF, et al. Recovery of spontaneous erectile function after nerve sparing radical retropubic prostatectomy with and results of a prospective, randomized trial without early intracavernous injections of alprostadil. 

Bannowsky A, Schulze H, Van Der Horst C, et al. Recovery of erectile function after nerve-sparing radical prostatectomy: improvement with nightly low-dose sildenafil. 

Padma-Nathan H, McCullough AR, Levine LA, et al. Randomized, double-blind, placebo-controlled study of postoperative nightly sildenafil citrate for the prevention of erectile dysfunction after bilateral nerve-sparing radical prostatectomy.

Montorsi F, Brock G, Lee J, et al. Effect of nightly versus on-demand vardenafil on recovery of erectile function in men following bilateral nerve-sparing radical prostatectomy. 

Ilic D, Hindson B, Duchesne G, Millar JL. A randomised, double-blind, placebo-controlled trial of nightly sildenafil citrate to preserve erectile function after radiation treatment for prostate cancer. 

Zelefsky MJ, Shasha D, Branco RD, et al. (2014). Prophylactic sildenafil citrate improves select aspects of sexual function in men treated with radiotherapy for prostate cancer. 

Pisansky TM, Pugh SL, Greenberg RE, et al. Tadalafil for Prevention of Erectile Dysfunction After Radiotherapy for Prostate Cancer The Radiation Therapy Oncology Group [0831] Randomized Clinical Trial. 

Partner Perspectives

While controversial, advances in understanding how prostate cancer treatments impact the physiology of erections have led to increased interest in penile rehabilitation interventions for these patients. 

Penile Rehabilitation in Prostate Cancer Patients

		Deborah Lee is a PhD student at the Michigan State University College of Nursing in East Lansing.

Chemotherapy and radiation treatment for many cancers do not come without risk and can result in treatment-related toxicities that produce serious side effects. Short- and long-term side effects of chemotherapy often worsen a patient’s quality of life and overall risk of mortality.

The number of cancer survivors in the United States is projected to reach 19 million individuals by 2024,

 and of these, 5.3 million are considered to be at high risk for cardiotoxicity, with breast and prostate cancer having the largest percentage of patients at risk.

		Intravenous bolus administration of anthracyclines

		Receiving other cardiotoxic medications

		Increased length of time since cancer treatment

		Elevation of biomarkers during and after treatment

Chemotherapy-induced cardiotoxicity (CIC) is defined as a poisonous or detrimental effect upon the heart that can delay cancer treatment, decrease survival, and increase morbidity.

 Acute and chronic heart failure is the second most prevalent comorbidity for childhood cancer survivors due to treatment with anthracyclines and chest radiation therapy, and it affects up to 48% of adult cancer survivors.

The mechanism of CIC is unknown; however, researchers hypothesize that it is due to increased oxidative stress on the cardiomyocytes. Prevention strategies such as iron chelation, antioxidants, beta blockers, angiotensin-converting enzyme inhibitors, and angiotensin receptor blockers have not been helpful.

Three approaches are used for primary prevention of anthracycline-induced cardiomyopathy to reduce a patient’s risk: (1) administration of dexrazoxane during anthracycline treatment; (2) continuous anthracycline infusion over 6 hours (as opposed to 30-minute infusion); and (3) use of anthracycline derivatives instead of doxorubicin.7 Dexrazoxane is the only FDA-approved medication for use in children and adults to prevent cardiotoxicity. Use of the dietary supplement coenzyme Q10 does not have sufficient data yet to support its use clinically for its potential protective effects for CIC.

Nurses can be instrumental in a variety of different clinical settings in implementing secondary prevention strategies for CIC, and this in includes careful clinical assessment of patients. Nurses are on the front lines of early detection for potential cardiovascular side effects during and after treatment for cancer, evaluating a patient for subtle signs and symptoms of cardiac dysfunction. These include minor impairment of exercise capacity and resting tachycardia with a heart rate greater than 100.

 Auscultation of an S3 murmur can also detect early heart failure.

The most common symptoms of cardiac problems due to CIC include pain, dyspnea, weight gain, edema, weakness, fatigue, palpitations, dizziness, syncope, and swelling of an extremity with deep vein thromboembolism.

In addition, nurses can aid in optimizing blood pressure management to decrease a patient’s afterload on the heart during anticancer treatment, as elevated blood pressure has been shown to increase CIC severity.

The European Society for Medical Oncology guidelines also recommended an echocardiogram 6 months after the conclusion of treatment, annually for 2 to 3 years, and then every 3 to 5 years for life.

			Dempsey KS (2008). Chemotherapy-induced cardiotoxicity in women. 

			The State of Cancer Care in America, 2015: a report by the American Society of Clinical Oncology. 

			DeSantis CE, Lin CC, Mariotto AB, et al. Cancer treatment and survivorship statistics, 2014. 

			Oeffinger KC, Mertens AC, Sklar CA, et al. Chronic health conditions in adult survivors of childhood cancer. 

			Conway A, McCarthy AL, Lawrence P, Clark RA. The prevention, detection, and management of cancer treatment-induced cardiotoxicity: a meta-review. 

			Curigliano G, Cardinale D, Suter T, et al. Cardiovascular toxicity induced by chemotherapy, targeted agents and radiotherapy: ESMO Clinical Practice Guidelines. 

			Vejpongsa P, Yeh ET. Prevention of anthracycline-induced cardiotoxicity: challenges and opportunities. 

Chemotherapy-induced cardiotoxicity is defined as a poisonous or detrimental effect upon the heart that can delay cancer treatment, decrease survival, and increase morbidity.

Chemotherapy-Induced Cardiomyopathy

 The explosion in genetic testing options is undoubtedly helping individuals with a family history of cancer to clarify their risk, yet these advancements are also yielding a lot of genetic information for which there are not yet any actionable medical management guidelines.

This ever-changing genetic landscape can be baffling and worrisome for patients, explained Jill Stopfer, MS, CGC, in her talk at the annual meeting of the group, Facing Our Risk of Hereditary Cancer Empowered (FORCE), held in Philadelphia June 19-20, 2015.

“There are a lot of new testing opportunities and a lot of new genes out there that can be looked at—some we know a fair amount about, and some we know almost nothing about”&mdash;a situation which Stopfer deemed emblematic of both the “promise and the peril of genetic testing.”

Stopfer, a senior genetic counselor, cautioned that the abundance of new genetic tests, while beneficial in many ways, also has limitations.

		May identify source of cancer in the individual’s family and help their relatives to better understand their risk

		May help clinicians make specific medical recommendations to protect the patient’s health

		More may be learned about the individual’s results in the future that may better enable them to take control of their health.

		It’s more economical to test for multiple genes at once.

		A mutation may be found in a gene that is not well understood.

		There may be a finding in a gene the lab doesn’t know how to classify, also known as a variant of uncertain significance.

		The test may find a mutation in a gene inconsistent with an individual’s personal or family history.

		There may not be a clear way to care for a person based on the results.

Medical guidelines exist for treating individuals with a pathogenic mutation in a high-risk gene like 

, which is associated with a higher risk of breast, ovarian, prostate, and pancreatic cancer, as well as melanoma. Stopfer noted that the new panels can be very helpful in stratifying risk for an individual who is positive for 

“Increasingly, we are learning that the specific position of the mutation within the gene is sometimes related to differences in risk. We also think that in families, there are other genes that interact with this powerful gene—we call these genes ‘modifier genes’&mdash;that can dial the risk up or dial it down … yet even for the 

 genes that are so well characterized, there is still more work to be done.”

Other pathogenic mutations may be found in moderate-risk genes, Stopfer explained, for which the cancer risk, and, importantly, any medical management, is unclear. Each of these genes may increase risk, but less dramatically, and it may require a set of genes, or genetic profile, to significantly raise an individual’s cancer risk.

“When the risk for cancer is only a little bit higher than the average person, what’s appropriate? We’re still debating and wrestling with these questions as more and more moderate-risk genes are being identified,” said Stopfer.

Finally, pathogenic mutations may been found in a newly characterized gene for which cancer risks and medical management are unknown, and these variants of uncertain significance (VUS) are especially prevalent in newer genes.

“There are many genes that labs will happily test you for that we know almost nothing about. We can’t tell you what cancers are associated … we can’t tell you what the magnitude of the risk is. We can’t tell you what’s recommended for people who carry mutations in this gene, because it’s unknown.”

And, Stopfer added, “most of these variants of uncertain significance will go away and be reclassified as nothing more than normal difference between people … I tell everyone to expect variants of uncertain significance. Just expect them.”

The growth in panel testing makes the need for genetic counseling even greater to maximize informed decision making: “Pretest genetic counseling is a must,” stressed Stopfer. When patients find VUS or other possibly concerning findings after a multigene panel, they’ll likely wonder: “now, what?” It can be challenging for genetic counselors and other oncology professionals to offer the right guidance.

“We may find a gene that is inconsistent with [a patient’s] personal family history,” said Stopfer. “There may not be a clear-cut way to care for an individual with these results,” which can also make them anxious and worried; some may even face scrutiny from other family members as to why they pursued the testing in the first place.

“The bottom line is that all of this needs to be tailored to the individual,” Stopfer continued: “Do you want this test? What are you going to do with this information? What choices are you going to make?

“For some people, even if we don’t know a lot about the gene that has been identified, it’s a placeholder. It’s something to follow and pay attention to, to follow the literature and learn more about as more research comes out,” she continued. “Some people like that: ‘It’s okay if we don’t know everything right now. I would like to know as much as possible, and we’ll just follow it.’”

Stopfer also cautioned against the overestimation of risk, especially as more discoveries emerge from the genetic research pipeline. This can be due to multiple factors, among them, that negative studies are not published as frequently. Another problem which she described as the “winner’s curse,” occurs when initial excitement over findings indicating a possible genetic link may overestimate the actual risk. There is also the issue of bias in sample ascertainment, because gene studies often involve highly affected families:

“Risk estimates from highly selected families may not reflect average risks for all comers,” added Stopher, who reminded her audience that in general, genetic risk for cancer remains “overall, pretty rare.”

“The truth is, for a lot of people with cancer, it is due to something called 

. Cancer is a complex endpoint. There are a lot of ways the cells are no longer behaving the way they should be,” including genetics, environment, lifestyle, and, “very importantly, random cell errors.”

Looking ahead, Stopfer is hopeful that the already intense research interest in genetic cancer risk will shed light on at least some of the current quandaries to help genetic counselors and oncology professionals better understand any cancer risk implications of these discoveries.

Prospective Registry of Multiplex Testing (PROMPT)

, a national research registry under the auspices of the Abramson, Dana-Farber, and Memorial Sloan Kettering Cancer Centers and the Mayo Clinic for people who have had multiplex testing and have at least one mutation or VUS in a gene other than 

. Patients self-enroll through an easy-to-use, secure web portal (

“More studies are always needed to replicate what someone has found,” said Stopfer. “It has to happen over and over again before you feel comfortable with the finding.

“As we saw with the story of BRCA, we do know a lot about what to do, what helps, what interventions make sense, and that’s because of research … we really need to pool this information quickly.”

The abundance of new genetic tests, while beneficial in many ways, also has limitations.

Beyond BRCA: The Promise-and Peril-of Multigene Panel Testing

		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

Like many nurses, I look at the veins in the forearms of cashiers, restaurant servers, and any other arms that appear in front of me, and lately I’ve noticed that many people are wearing a fitness tracker on their wrists. These devices have exploded onto the marketplace and some predict that they’re in their infancy and will become even more sophisticated in the future. They’ve also become addictive; we all know people who check their fitness trackers until they’ve reached the magic 10,000 steps in a day.

In addition to tracking the number of steps a person takes in a day (and in a week and longer), many of the trackers also perform monitoring functions, such as tracking heart rate and sleep quality. Wireless syncing allows the data to appear on a smartphone or computer. There are even scales that transmit a person’s weight directly to another device, and this information can be stored and/or sent on to healthcare providers.

The Health Research Institute of PricewaterhouseCoopers (

) surveyed consumers and found that one in five owns wearable technology, and about half wear the technology every day. The survey found that the top three pieces of information consumers want from wearables are health-related: 77% want wearables to increase activity, 75% want them to collect and track medical information, and 67% want wearables to help them eat better. Adopters of wearable technology tend to be younger, more affluent, and tech savvy. However, the survey also found that 80% of the general population is familiar with at least one wearable device on the market today.

Potential benefits of wearable technology include reducing obesity, improving sleep quality, prompt detection of health issues, and ongoing monitoring of vital signs or medication adherence, for example. However, experts note that information without action is meaningless. Wearable technology is only effective when people act on the information provided. Drawbacks and concerns about this technology include vulnerability to security breaches, dependence on technology, and further decline in personal social interactions. In healthcare, this could mean fewer in-person clinic visits, as patients transmit health data to their healthcare providers and receive a response electronically or by phone.

Employers and insurers are taking notice of the impact of wearable technology. Some have begun to pay for the technology, or incentivize it (eg, the employee is reimbursed for a fitness tracker after walking a required distance). The Walgreens pharmacy chain has a program where customers can transmit their fitness data to Walgreens in exchange for points that can be used in the stores to buy products. The Apple Watch, equipped with a heart-rate sensor and accelerometer, has an activity app that tracks minutes standing and calories burned, and has a workout app, which provides information about activity. A companion app on the iPhone shares the data with researchers.

Some of the wearable technology products in development with applications to healthcare include the Google X smart contact lens, which has a tiny electronic circuit and microchip embedded in the lens that measures the person’s blood sugar level in tear fluid and transmits the data to a smartphone or other device. The Google X nanoscale “cancer hunters” are nanoparticles in a pill that make their way into the bloodstream to attach to cancer cells. Patients wear a device that creates a magnetic field that pulls the particles to a place where they are counted.

The Sunfriend is a watch-like device that measures UVA and UVB exposure, which has the potential to decrease skin cancer incidence. HyGreen and Biovigil are devices that are in development to improve hand hygiene compliance and effectiveness by “reading” hand cleanliness. The Propeller Health GPS-enabled inhaler has location sensors to track the time and place of each use of the inhaler, and provides data to better identify asthma attack triggers. We can expect to see these devices, and more, in the future.

Nurse's Notes

Potential benefits of wearable technology include reducing obesity, improving sleep quality, prompt detection of health issues, and ongoing monitoring of vital signs or medication adherence.
 

More Patients Tracking Health Data With Wearable Technology

This past spring I had a personal experience which totally altered my view of my vocation as an oncology nurse navigator. I very deliberately use the word vocation, because for me, navigation opened the door to an occupation that utilizes all of my capacities as an oncology nurse. And, with that realization, came that wonderful sense of peace that comes with discovering one’s true calling.

And thus begins my story: I was attending an ONS Congress presentation when I got the text from my husband saying, "Yep! It's melanoma." He was referring to the results of a biopsy from a small, odd-looking freckle on his back that the dermatologist biopsied the previous week.

Not only did that get my attention, but I had absolutely no idea what the rest of the presentation was about, and as soon as it concluded, I called him to hear him say that the dermatologist would remove it surgically. I, of course, told him that he would be going to Jonathan Lee, MD, a superb surgical oncologist (specializing in melanoma) at Northside Hospital Cancer Institute where I work. I also informed Bobby (my protesting husband) that resistance was futile and having been married to me for 44 years, he quietly agreed.

The following Monday I returned to work and called Penny Bauman, RN, OCN, Northside’s melanoma/sarcoma nurse navigator, and asked for her help. She called me back and asked if my husband could see Dr. Lee at 3 PM that same day! She met us there and stayed with us all during that initial visit—which was lengthy&mdash;meaning she had to drive home in the thick of our notorious Atlanta rush hour. She left smiling, and true to her nature, hugged Bobby and me, saying she would be with us all the way.

That Friday I drove my husband to the hospital at 7:30 AM, registered in the pre-op area, and suddenly experienced a personal paradigm shift: I wasn't the lab-coated nurse gliding through my hospital with the "just-between us exclusivity" of my professional self. No, today I sat down next to my husband—with all of the other family members who sat by those they cherished&mdash;and I was afraid for him, as the word 

 turned into a small, ugly monster in my mind's eye, and I recalled some of the wide excisions we did in the operating room when I worked in Florida … knowing that 'little odd looking freckle' can be merely the tip of something dangerous indeed.

Along came Penny B, with her smile and that warm hug, and she was our sunshine for that terrifying morning. She came in after Bobby was tucked into his pre-op stretcher and was quietly and warmly supportive for us. She checked to see how Bobby did during the surgery and later came to the post-op visit with Dr. Lee just in case there were any questions. She was a quiet warm support, ever smiling.

Because my husband was a patient with an oncology nurse for a wife, it might have occurred to Penny B that she could have used her time somewhere else, but what did occur to her, as it occurs to all of us who are nurse navigators, is that here was a patient with an emergent and frightening diagnosis who could use some support to get through the experience, and a wife who was worried and frightened and who had been transformed from the stable persona of her professional role into a fearful human being who could really use that hug and that smile and that warm sense of support. Thankfully, my husband's melanoma was stage I and required no further treatment, but it was an “aha” moment for us both.

As a nurse navigator, I've come in to the hospital at 5 AM to be with a family during pre-op for support; I've come to the hospital to be with a dying patient who had her daughter's wedding skyped in; I've gone to patient funerals so that the patient's family will know that I truly realized the uniqueness of their loved one, and I've visited long-term patients in hospice, so they know I didn't drop them from my list when they left the hospital.

I support my patients in many intangible, unquantifiable ways, and occasionally, I wonder if I am just being too expansive in my interpretation of the navigator’s role. Yet, after my experience as a wife who was by her husband’s side through his cancer diagnosis and surgery, I can say that I now fully understand the breadth—and the depth&mdash;of our role as nurse navigators.

Sometimes at our multidisciplinary conference, when a doctor is asked if the patient is appropriate for navigation, he or she replies that the individual has good family support, and hence, no need for navigator referral. Having now assumed both roles—as a navigator, and even more so as a wife&mdash;I believe that every patient needs that warm smile, hug, and touch to guide them through the fear that inevitably replaces reason, when a cancer diagnosis collides with our everyday lives.

Penny Daugherty is an oncology nurse navigator specializing in gynecologic oncology and multiple myeloma at the Northside Hospital Cancer Institute in Atlanta. Penny Bauman is an oncology nurse navigator with the melanoma/sarcoma program at Northside.

An oncology nurse navigator learns just how essential the role is, when her own husband is diagnosed with melanoma and a colleague provided just the right support.