Charlotte Huff

Gaining access to that top-notch doctor or medical center, never easy for any patient, can present a larger hurdle for someone with an uncommon malignancy, says Marty Rosen, executive vice president of marketing and cofounder at Health Advocate, in Pennsylvania, which provides health advocacy, wellness and other services.

“I think that the challenge is probably exacerbated for these people who have rarer or esoteric disease, for no other reason than that there is probably a smaller universe of these providers, especially top docs or top centers of excellence,” Rosen says. “That probably creates even more of a potential logjam.”

	Given the challenges involved, Rosen and others who work with patients advise them to gather key medical information in advance and prepare to make their most compelling case.

	Ideally, the patient’s local doctor should call on the patient’s behalf, Rosen says. If that’s not feasible, a close family member might be the next best choice, to advocate non-emotionally and articulately to a busy office assistant who is triaging a multitude of ill patients, he says. In just a few sentences, spell out what tests have been conducted, the precise diagnosis and why time is of the essence, he says.

	Be sure to have all of the scans and medical records gathered digitally, so they can be imme- diately transmitted, says Marion Schwartz, chief advocacy officer at the Cholangiocarcinoma (bile duct cancer) Foundation. Request a digital copy of every test as soon as it’s been completed. In some cases, the actual tissue (known as a “block”) may be needed to perform specialized diagnostic tests to determine the tumor type.

	Patients can also compare strategies online, whether it’s getting access to an off-label medi- cation or an overbooked physician, says Beth McNaughton, vice president of community for Inspire.com, which provides links to 200 online support groups. “It’s kind of like tips and tricks,” she says. “They’ll ask if there’s some way to get around it, or to get prioritized.”

	Prepare to be polite, but persistent, Schwartz stresses. “Re-contact, re-email, re-call,” she says. “Call daily until you are heard.” 

 on finding support and medical options when you have a rare cancer]

Articles

Gaining access to that top-notch doctor or medical center can present a larger hurdle for someone with an uncommon malignancy, but it is possible with a few tips.

Getting Booked - Stat

For 18 months before her rare form of cancer was diagnosed, Connie Alexakis and her doctors wrote off a series of frustrating symptoms to other maladies.

First, she experienced intense all-over itching, so constant that she called in an exterminator to search her home for bugs. When none were found, she figured it had something to do with the Chicago environment, as the itching vanished when she moved with her husband to South Carolina in July 2012. By the time the itching erupted again, in August 2013, her doctors blamed hormones, considering she had given birth to her daughter six weeks earlier.

Connie Alexakis (right) has undergone aggressive treatment for late-stage bile duct cancer. Along with her husband, Jerell Chua (left), and daughter, Olympia Chua (center), Alexakis is hopeful that the treatments will succeed in keeping the cancer at bay. Photo by Rachel Fesko. 

A period of relief followed until December 2013, when she again began to itch, along with exhibiting another worrisome sign—her skin and eyes were turning yellow. Even then, Alexakis and her husband, a family physician, thought it might be a drug reaction, as she was taking an influenza medication. It wasn’t until New Year’s Eve last year that numerous tests revealed she had cancer of the bile duct, or cholangiocarcinoma, a disease that develops in at least 2,000 Americans every year. The rare malignancy, which had spread to the nearby liver, was stage 4.

At 32 years old, feeling sleep-deprived from caring for an infant, Alexakis acknowledges that she was a bit in denial about the stakes involved, until she asked her oncologist how long she might live if she put off treatment. About three months, he responded.

Since then, Alexakis and her husband have pursued a battery of chemotherapy and radiation treatments and second opinions, encountering the challenges of access and time common to any rare malignancy.

Individuals with rare cancers particularly benefit from team-based care, in which the oncologist, surgeon and other specialists closely coordinate. 

One out of every four cancers in adults is considered rare, according to an analysis of United States cancer data published in 2010 in the journal 

. As Alexakis learned, a rare cancer might result in a delayed diagnosis, which in turn results in acute time pressure to identify and track down medical care from a more limited pool of specialized physicians.

Patients in urban areas can have an easier time, because they are more likely to live near a National Cancer Institute designated center, says Diane Reidy, a medical oncologist at New York’s Memorial Sloan Kettering Cancer Center who cares for patients with neuroendocrine, adrenal gland and other rare malignancies.

“I always tell my patients that we’re not any smarter, but we just see these rare tumors so frequently that it becomes common practice to us,” she says. In the case of uncommon cancers, Reidy says, “We don’t have these very large randomized (research) trials to tell us exactly what to do. So that’s when the expertise of the physician can really help.”

A disease is classified as rare if it impacts fewer than 200,000 Americans at any given time, according to the definition in the Orphan Drug Act of 1983. (The 

 analysis applied a somewhat different threshold, cited at a National Cancer Institute workshop, defining a rare cancer as one diagnosed in fewer than 40,000 people annually.) The 1983 federal law has jumpstarted research into treatments for so-called orphan diseases, including malignancies, by providing incentives to drug companies. Just 10 new treatments for such diseases were developed during the decade prior to the law’s passage, versus more than 460 approved for clinical use in the years since, according to the National Organization for Rare Disorders (NORD).

Individuals with rare cancers particularly benefit from team-based care, in which the oncologist, surgeon and other specialists closely coordinate, Reidy says. “It’s so critical that we’re there from the start to quarterback the plan,” she says. “If somebody had a sloppy surgery, it’s impossible for us to fix that.”

Too often, though, patients stick with the local doctor who made the diagnosis, which might preclude their access to research trials or other medical options, says Marion Schwartz, chief advocacy officer at the Cholangiocarcinoma (bile duct cancer) Foundation. With bile duct cancer, for example, there’s an ongoing effort to educate patients and doctors that a small minority might be eligible for a liver transplant, Schwartz says. But patients are disqualified if they’ve already had a liver biopsy, she says.

[Getting Booked - Stat: Getting that appointment with a top-notch doc]

To track down an experienced physician, patients should start by asking the local doctor who made the diagnosis, Reidy suggests. If that physician doesn’t have an immediate suggestion, they can search the Internet for related publications and research trials to identify relevant experts, she says.

When meeting with specialists, don’t hesitate to quiz them a bit, Reidy says. How many patients with a similar diagnosis have they treated in the last year? What research trials are available for their patients? Keep in mind, she says, that a senior doctor at a large cancer center might not necessarily be the best choice. A less experienced physician might be just as smart, provide the same level of care and have more time to devote to explain the malignancy and care options, she says.

If the local doctor isn’t much help in terms of referrals, ask for suggestions through online support groups or find related disease-specific organizations, says Mary Dunkle, vice president for communications at the National Organization for Rare Disorders. The disease-specific group also might list its medical advisors on its website, indicating some of the experts for that malignancy, she says.

Can’t travel due to financial reasons or responsibilities closer to home? Major cancer centers typically will review pathology reports long-distance, to verify the cancer diagnosis and staging, Reidy says. Many also will review medical records and provide treatment recommendations long-distance, she says. “It’s not perfect because they can’t see you,” she adds. “There’s definitely something to be said about personalizing the therapy by meeting the patient.”

Alexakis and her husband, Jerell Chua, adopted a hybrid strategy, with a mix of local treatment and second opinions. Her cancer had advanced to a stage that made surgery impossible. So Alexakis completed three months of gemcitabine/cisplatin, a typical chemotherapy regimen, with cancer specialists in nearby Charlotte, N.C. But when scans proved disappointing, the couple searched further, sending her records to Emory University in Atlanta and traveling to Memorial Sloan Kettering.

In the months since, Alexakis has combined an oral form of chemotherapy, Xeloda (capecitabine), with a two-pronged radiation approach. She underwent a series of radiation sessions in the spring, designed to target the bile duct tumor itself, and was hospitalized three times for chemoembolization, which delivers intense doses of chemotherapy through a catheter in an artery that feeds the liver.

Alexakis considered traveling for treatment, but ultimately decided to stay with her Charlotte medical team. “We have a little one,” she says. “If this does turn lethal very quickly, I don’t want to think that I spent my entire time traveling back and forth on planes, instead of being home with my husband and my daughter.”

As they contend with their diagnosis and treatment options, patients with a rare malignancy can sometimes feel isolated, says Beth McNaughton, vice president of community for Inspire, a social network for patients and caregivers, especially if they have to “educate friends and family about their particular rare cancer.”

Rare cancers are “lonely diseases,” agrees Schwartz, of the Cholangiocarcinoma Foundation, who had never heard of the disease before her husband received the diagnosis in 2006, dying nearly a year later. Alexakis describes reactions from friends and family as falling into one of two camps: Some default to peppy optimism; others research online and assume a funereal attitude after encountering write-ups that are “completely pessimistic,” she says.

By early September, Alexakis was permitting herself some cautious optimism, with the latest set of summer scans indicating that the radiation-chemotherapy combo had gained traction against the tumors. “There is some hope now that maybe I could become a surgical candidate,” she says.

Her last of four chemoembolization sessions was scheduled for mid-September, followed soon after by another scan. Then she and her husband would decide the next step, working with their local oncologist, she says. They plan to cast their net wide, and have been building a list of potential experts to consult.

Always, Alexakis keeps a CD with her, loaded with her latest scan. “In my opinion, I don’t know when I might meet this world-class surgeon. I might be at the airport. I might be at McDonald’s. I want to be ready,” she says.

“The best thing is to turn me from inoperable to operable. For me, I know that’s my only chance at a cure.”

> Someone with a rare cancer should search for a doctor who is an expert in treating that disease, and who may be able to recommend clinical trials.

> When patients don’t want to travel, large cancer centers will often review pathology reports long-distance to confirm a diagnosis or staging, or to offer treatment recommendations.

Finding specialized care is worth the effort for patients with uncommon cancers.

Rare Finds: A Rare Cancer Diagnosis

Given the mixed messages involved, it’s not surprising that women with ductal carcinoma in situ (DCIS) can become anxious and sometimes unduly alarmed, says Ann Partridge, a medical oncologist at Boston’s Dana-Farber Cancer Institute.

They’re told the good news, at least where cancer is involved: that DCIS is confined to the milk ducts of the breast and thus doesn’t pose an imminent life-threatening risk. Then they’re bombarded with a battery of treatment options and decisions, starting with surgery and potentially followed by weeks of radiation and years of tamoxifen.

“At least emotionally, they feel just like an average patient with invasive breast cancer,” Partridge says. “They feel like they were walking along and had the carpet ripped out from under their feet in terms of their trust in their body.”

The result: confusion and an inflated risk perception. In one 2008 study that Partridge helped author, 28 percent of recently diagnosed women described their risk of the noninvasive cancer spreading, not only within the breast itself but also to other parts of the body, as moderate or higher. The likelihood of such a metastasis occurring, according to studies, is less than 1 percent. Of the 487 women surveyed, 10 percent also reported substantial anxiety.

Partridge credits a mix of potential factors in explaining the perception-risk disconnect, including the high-profile scrutiny on breast cancer in today’s media and the failure of some doctors to devote sufficient time to explaining the relatively low-risk diagnosis. Moreover, doctors might not recognize these women’s psychological needs, as they care for patients with brain metastases and other life-threatening malignancies.

But, Partridge stresses, the emotional whiplash felt by patients with DCIS is very real and justified. “It still stinks to lose a piece of your breast or your whole breast, even if you’re not going to die of something,” she says.

Could the name itself play a role in DCIS anxiety, with the word “cancer” embedded within? In a 2013 commentary published in the 

Journal of the American Medical Association

, a working group organized by the National Cancer Institute has argued that language does matter, going so far as to suggest removing the word “carcinoma” or “cancer” from the names of pre-malignant conditions.

A name change, though, might dissuade women from pursuing treatment that could reduce the likelihood that one day the lesion might morph into invasive breast cancer, says Thomas Julian, a surgeon at Allegheny Cancer Center. “By eliminating that part of the name, you’ve really made it sound like, `Oh, this diagnosis is a big nothing,’ ” he says. “Patients need to discuss this diagnosis (DCIS) with their oncology physicians to determine their best treatment.”

Meanwhile, patients with DCIS describe feeling a bit isolated amid the pink-ribbon swirl of the breast cancer community, says Sandie Walters, who has been treated for DCIS and is the co-founder of the blog 

 Walters, who has mentored patients, says some women with DCIS question whether they qualify as breast cancer survivors. “It’s stage 0 cancer, but then it’s not like what people traditionally think of with cancers,” she says.

For that reason, a traditional cancer support group, where another woman sitting nearby might be coping with hair loss and longer odds, might not be the best place for patients with DCIS to start, Partridge says. “It’s like the skinny person going to Weight Watchers who wants to lose five pounds.”

Instead, she recommends that women with DCIS read up on the noninvasive cancer, hopefully allaying their fears with facts. If their anxiety is eroding their quality of life, they should consider talking with a mental health professional. Or they could ask their cancer center to match them up with someone who also has stage 0 cancer, with whom they can speak more freely.

Above all, the fear and angst shouldn’t be ignored or discounted,  Partridge says. “I think anxiety can lead people to both feel badly in the moment, as well as to make decisions that might have repercussions for their health and wellbeing in the future.”

Given the mixed messages involved, it's not surprising that women with ductal carcinoma in situ can become anxious and sometimes unduly alarmed by the diagnosis.

Coping with the Uncertainty of DCIS

	Lobular carcinoma in situ, or LCIS, sounds similar to its linguistic cousin DCIS (ductal carcinoma in situ). But its diagnosis is typically considered less worrisome.

	Rather than being a precancerous lesion like DCIS, the lobular lesion is considered only a marker of elevated risk for invasive cancer down the road, according to the National Cancer Institute. Irene Wapnir, chief of breast surgery at Stanford School of Medicine in Stanford, Calif., explains that doctors approach LCIS as a high-risk lesion, unlike DCIS, which is regarded as a cancerous lesion that requires breast biopsy and then either lumpectomy with radiation or mastectomy.

	In some cases of LCIS, surgery might be recommended: for example, if the LCIS is scattered throughout the breast, Wapnir says. But for most women, taking the estrogen blockers tamoxifen or raloxifene is the recommended approach. In one large-scale prevention study, which included 830 women with a history of LCIS, use of tamoxifen reduced the risk of later developing an invasive cancer by more than half.

Lobular carcinoma in situ, or LCIS, is considered a risk factor for invasive cancer.Â 

Another Lesion: LCIS

	Sandie Walters’ breast cancer was diagnosed in 2007 during a routine screening mammogram, and soon after, the 58-year-old Grand Rapids, Mich., woman was sitting in a surgeon’s office. His recommendation: a mastectomy, preferably immediately.

	She says her doctor even seemed reluctant to postpone the surgery until after she and her husband had returned from their imminent two-week summer vacation. “He said, ‘It’s probably OK, but I can’t officially say that it would be OK.’ Well, if you think that’s not scary—that’s scary.”

	It’s a story that sounds unsettlingly familiar to patients who undergo a routine test only to find themselves thrust into the frightening position of making a life-changing decision about cancer treatment. However, Walters’ story comes with a notable twist—one that’s affecting an increasing number of women with breast cancer. Her diagnosis was ductal carcinoma in situ (DCIS), also referred to as stage 0 cancer, a nonmalignant but potentially risky condition that’s creating stress and controversy for patients and physicians alike.

	That news launched Walters on a seven-year quest. She first steeped herself in studies and second opinions before choosing more limited treatment followed by ongoing screening. It’s not an approach that she regrets, despite receiving unsettling test results in June. “When I think back on it, I don’t think I would have done anything differently,” says Walters, shortly after an imaging scan identified a stage 2 tumor in her healthy breast.

	The abnormal cells of DCIS are described as stage 0 because they’re confined to the milk ducts of the breast, where they could remain, perhaps forever, without causing additional problems. But DCIS also is considered a risk factor for invasive breast cancer, which occurs when those cells spread beyond the ducts to elsewhere in the breast and potentially, albeit rarely, much farther. “The problem is that it’s difficult to pick out the bad actors in terms of cancer,” says Ann Partridge, a medical oncologist at Boston’s Dana-Farber Cancer Institute.

	The result is that some women decide to use the surgical equivalent of a sledgehammer on a not-quite-cancerous lesion. Getting a double mastectomy for a small area of DCIS is typically considered an excessive measure by clinicians, Partridge says. The comedian Wanda Sykes took this surgical route after her DCIS diagnosis in 2011, telling viewers of the “Ellen” show that she preferred to slash her worry and recurrence risk to near zero. Meanwhile, women like Walters pursue second opinions as they weigh not only the type of surgery, but whether to later undergo radiation treatments or take tamoxifen, a hormonal agent that blocks the cancer-promoting effects of estrogen. At the opposite end of the spectrum, still a small group, are those women who opt for “active surveillance,” which includes aggressive screening, sometimes combined with hormonal medications, instead of surgery.

	It’s a valid approach for some women with DCIS, says Laura Esserman, a breast surgeon at the University of California, San Francisco, who has spoken repeatedly about her overtreatment concerns. “I do think there is a large majority of DCIS [cases] that are detected today that are low or no risk,” she says. “One size does not fit all. And DCIS is not one condition.”

	While cancer physicians might differ about the degree of overtreatment, they do coalesce around one point: Any diagnosis that includes the word “carcinoma” can trigger understandable palpitations. But with DCIS, patients have plenty of time to research and consider options, says Thomas Julian, a breast surgeon at Allegheny General Hospital in Pittsburgh.

	“First of all, very few people die from DCIS,” Julian says. So he tells patients to take the time to work through it. “There’s not a biological rush” that demands treatment within a few weeks, he adds.

	The rapid increase in DCIS over the past several decades has been attributed to increasing numbers of women who undergo routine screening mammography, as the noninvasive breast cancer seldom can be felt as a lump and usually detected on mammographic screening as calcium specs (microcalcifications). Today, roughly 25 percent of breast cancer diagnoses are DCIS, whereas prior to 1980 it was rarely detected.

	Despite the unsettling name, the survival rates are quite good. One analysis, which examined long-term results from two major studies, found that the breast cancer-related mortality rate within 15 years was 3.1 percent among women who received only a lumpectomy. It was even lower, but not statistically different (2.3 percent) for women who completed radiation and tamoxifen after surgery, according to the findings published in 2011 in the 

. “Yes, we are overtreating many patients,” says Irene Wapnir, a study author and chief of breast surgery at Stanford School of Medicine in Stanford, Calif. “But on the other hand, lumpectomy, radiation and tamoxifen achieve excellent long-term results.”

	Still, these medical interventions are not without personal costs, including potential side effects, says Shelley Hwang, chief of breast surgery at Duke Cancer Institute in Durham, N.C. She worries that some doctors don’t take time to explain to patients the relative risks and benefits of treatment. “Nobody ‘needs’ radiation,” she says, yet adds that it does reduce recurrence risk.

	However, radiation does not reduce metastasis or death rates—those outcomes are very rare with DCIS, and about the same with or without radiation and hormone therapy. Therefore, the benefits of radiation treatment must be balanced against the risks, which include inflammation and redness of the skin, fatigue, and rarely, secondary cancers and cardiac issues.

	One difficulty in advising patients, Hwang says, is that relatively little is understood about the natural course of DCIS and how frequently it progresses to invasive cancer if left alone and closely monitored. “As long as we’ve known about DCIS, we’ve never not treated it,” she says, adding it’s still unclear what would happen if it was routinely left untreated.

	Initially, mastectomy was the standard treatment for DCIS, Julian says. But research showed that lumpectomy combined with radiation provided similar benefits for women, particularly when the area of DCIS was small, he says. And DCIS that’s diagnosed by mammography tends to be small—no more than a few centimeters in size. Moreover, if the DCIS is confined to one region of the breast and not scattered across several regions&mdash;the technical term is quadrants&mdash;the patient would be a good candidate for a lumpectomy, he says. (The size of a woman’s breast also figures into whether lumpectomy is an option.)

	The advantage of lumpectomy, Julian says, is a shorter recovery time than with a mastectomy, with likely less pain and fewer surgical side effects. Moreover, patients retain most of their breast, and thus avoid a reconstruction procedure, he says.

	Still, both Julian and Wapnir say, despite their best counsel, some women with DCIS insist on having a single or even a double mastectomy. These women “feel that they’ve kind of grazed a big monster and that if they are very aggressive, they won’t ever have to face this again,” Wapnir says. “For me, it’s been an astonishing backswing of the pendulum. It’s becoming harder to talk them into lumpectomies.” However, some women may prefer not having to be monitored and subject to the fear of recurrence even if the death risk is minimal.

	Far from leaping to a mastectomy, Walters decided to explore her options, meeting with a second surgeon who agreed to perform a lumpectomy instead. In December 2007, her surgeon removed three areas of tissue from her left breast, with the largest measuring 7 centimeters by 5.5 centimeters by 2 centimeters. Then Walters reached her next decision-making crossroad: whether to undergo radiation.

	One frequently cited analysis, which looked at four DCIS studies, found that undergoing radiation after a lumpectomy cut a woman’s 10-year chance of recurrence by 15.2 percent. Those recurrences were roughly split, with half involving another DCIS and the rest an invasive cancer, according to the analysis, published in 2010 in the 

Journal of the National Cancer Institute Monographs

	Also following surgery, including mastectomy, women are asked to make another choice: whether to take hormone therapy if the DCIS is estrogen-receptor (ER) positive (three-fourths of DCIS lesions are ER-positive, according to one pathology analysis) with a goal of reducing the risk of later developing invasive cancer in either breast.

	In Wapnir’s 2011 analysis, the 15-year risk of an invasive breast cancer emerging was 8.5 percent when women took tamoxifen, an estrogen-receptor blocker, following a lumpectomy and radiation. If they skipped the hormone treatment, it was 10 percent. (Among those women who chose lumpectomy alone, 19.4 percent developed an invasive cancer.)

	But the analysis didn’t find any statistically significant survival benefit to adding either radiation or tamoxifen. This is because a vast majority of recurrences are local—that is, they’re in the breast and are treatable with surgery&mdash;so their impact on mortality is miniscule, if anything.

	Tamoxifen also increases the risk of some uncommon, but potentially serious side effects, including stroke and blood clots. Some women also experience symptoms that adversely affect their quality of life. One study published last year found that 15 percent of women who underwent hormone therapy, including tamoxifen, stopped the drug early because of intolerable side effects, such as hot flashes and blood clots.

	As each woman plots her own path, a key part of this calculation is determining how high her risk of recurrence is in the first place.

	Walters conducted extensive research in that regard, sometimes traveling for consultations and follow-up tests, as well as sending her surgical slides to a renowned pathologist. That physician used a predictive tool, called the Van Nuys Prognostic Index, to estimate that Walters’ recurrence chance following surgery was 4 percent. Thus, adding radiation would reduce her chance of recurrence to 2 percent.

	Walters ultimately decided to pass on the radiation. She received regular imaging scans without incident, until that tumor was discovered in her healthy breast. The pathologist whom Walters consulted for a second opinion, she says, flatly stated that the cancer was not related to her prior DCIS. “The only thing the DCIS did is put me at higher risk at both breasts, not just the DCIS breast,” she says.

	Radiating the DCIS in the left breast wouldn’t have prevented cancer from developing in the right, Walters points out. But what if she had taken tamoxifen? Her recurrence risk was already low to start with, she responds, adding: “You don’t know what the side effects of tamoxifen are going to be for you until you take it and try it. Women have very different reactions.”

	Hwang strives to walk her patients through a similar personalized calculation, based on the pathology of the DCIS itself, the woman’s age, life circumstances and risk-taking mind-set. That’s the bottom line, Hwang says: “How much of a risk are you comfortable living with? And how much do you feel like you have to do to get that [recurrence] number as close to zero as possible, including mastectomy?”

	The Van Nuys index, which looks at factors such as age, DCIS pathology and margins after surgery, is one of several predictive tools that has been developed, although there’s still no consensus on the best approach, according to the National Cancer Institute.

	One tool, Oncotype DX, aims to take risk analysis a step further by incorporating gene markers. The test analyzes a gene panel to calculate a DCIS score. In a study published last year in the 

, researchers found that the DCIS score in patients who did not receive radiation was significantly associated with the 10-year risk of developing a recurrence, either of DCIS or an invasive cancer. Patients with a low score had a 10.6 percent chance of recurrence within the next decade versus about 26 percent for those with an intermediate or high score.

	Lawrence Solin, the study’s principal investigator, says he uses the score to assist women who are wrestling with their postsurgical options. “It’s a way to stratify patients,” he says. “It’s important to recognize that none of these are perfect predictors. Even a low-risk score doesn’t give her a zero risk of recurrence.”

	Solin, who chairs the radiation oncology department at Einstein Healthcare Network in Philadelphia, acknowledges that postsurgical radiation hasn’t been shown to increase a patient’s long-term survival. Still, the benefit of avoiding recurrences shouldn’t be discounted, he says. “A tumor that comes back, even if it’s local and can be treated with surgery, carries severe costs to the patients,” he says. “There’s tremendous anxiety and an emotional component.”

	But patients also should be filled in on the broader picture, Esserman and Hwang say. Even if DCIS spreads beyond the milk ducts one day and becomes invasive cancer, close screening should catch it early, they say. The rate of five-year survival with localized breast cancer is 98.5 percent, according to the National Cancer Institute. “Are you at risk for something that’s going to be lethal tomorrow?” Esserman asks. “No.”

	So, Esserman and Hwang ask, if a woman’s lesion is low-grade and hormone-positive, why not hit the pause button on surgery? One option that’s now being explored is to offer such women a hormone treatment as a potential prevention measure. (Esserman uses the analogy of prescribing a statin drug to a patient with high cholesterol.)

	Hwang is leading a multisite study, which is recruiting participants, to investigate the impact on DCIS of taking the hormone therapy letrozole before undergoing surgery. After three months of hormone therapy, participants will undergo a magnetic resonance imaging (MRI) scan. If the DCIS hasn’t progressed, the women will continue taking the letrozole for another three months, followed by a second MRI and then surgery.

	The hope is that women on the study will benefit personally if their DCIS shrinks enough to qualify them for a lumpectomy rather than a mastectomy, Hwang says. “But I think the long-term goal also is very important, which is to identify a group of women where the DCIS might go away altogether with hormonal therapy alone,” she says. “That would really help us to get surgery out of the mix for these very good-prognosis DCIS.”

	Some women already are skipping surgery entirely. Mary Jane Lapinski took tamoxifen for three months before surgery in 2003 as part of another study involving Hwang. Her DCIS diminished enough that she could have chosen a lumpectomy.

	But Lapinski decided to continue taking the tamoxifen, bypassing surgery against Hwang’s recommendation. After five years of taking tamoxifen, Lapinski switched to another hormone drug, raloxifene. The 59-year-old, who lives near Washington, D.C., doesn’t consider herself “some kind of daredevil.” She continues receiving regular MRIs and mammograms, and, as Walters did for years, hopes that her brush with near-cancer advances no further.

	“If [invasive cancer] ever did develop, I would do what I have to do,” Lapinski says. “But when someone is telling you that you have noninvasive cancer, and then they say, ‘Oh, by the way, we’re going to do a mastectomy,’ that makes no sense to me at all.”

	As Walters weighs her options, she distracts herself with time on the tennis court, saying that her energy and optimism have not flagged. “There is just this one stupid problem that I can’t seem to shake,” she quips.

	Once Walters sorts through her treatment options, she plans to update her blog, which she has used to document her search for answers, saying that it’s only right to share this latest installment in her DCIS experience. “That’s one reason women choose more drastic treatments,” she says, “because they don’t want to deal with this ever again.”

Editor’s Note: Read Sandie Walters’ blog at 

	> Patients with DCIS have time to explore treatment options and weigh the risk and benefits. 

	> Patients can reduce their recurrence risk but never eliminate it.

Will suspicious cells remain idle or become aggressive breast cancers?

The DCIS Dilemma

Megan Silianoff described her treatment for ovarian cancer, first in a phone call with the adoption agency in 2011, and then later when she traveled with her husband to attend a group orientation meeting for prospective parents.

She had received the diagnosis the prior year when she was 28. It was a rare form of ovarian cancer, one that’s not typically aggressive, Silianoff says. She underwent surgery, and her Houston oncologist wrote a supportive letter. “She said that that my life expectancy was consistent for anyone else my age, and that she recommended me as a candidate for adoption,” Silianoff says.

The agency’s response was friendly throughout the group orientation gathering, but then roadblocks began to appear. First, her oncologist was asked to write a second letter, with more details about her cancer diagnosis. Then, Silianoff recalls, “they called and said, ‘We got your doctor’s letter. But we need a 100 percent guarantee that your cancer won’t grow back.’ And that’s when I knew they were just yanking us around.”

Such medical certainties, which wouldn’t be required of prospective adoptive parents in other situations, can run afoul of the Americans with Disabilities Act (ADA), says Madelyn Freundlich, a New York City lawyer and social worker who specializes in adoption and foster care issues. Cancer, including a prior history, is considered a federally protected disability, she says.

“The goal is to have people with disabilities be able to fully enjoy services unless the criteria that are being used with respect to their disability can be shown to be necessary to providing, in this case, adoption services,” says Freundlich, who has written several articles about the ADA and adoption and previously served as executive director of the Donaldson Adoption Institute, a New York-based nonprofit organization that focuses on adoption research and policy.

Asking a doctor to provide a medical guarantee is unreasonable, she says, because it wouldn’t be required for applicants without a history of cancer. “There is really no guarantee that any of us are going to live to a certain age,” she adds. “No doctor in his or her right mind is going to start guaranteeing the future health of people.”

Freundlich also suggests that agencies steer clear of requiring cancer survivors to meet specific benchmarks, such as proving that they’ve been cancer free for five years. Cancer behaves differently in each individual, growing at different rates and responding differently to treatment, she says. “That’s really setting arbitrary criteria and doesn’t really look at the individual characteristics of that particular prospective parent.”

Agencies can ask an applicant’s doctor to provide a letter, attesting to current health status and related medical details, without straying into prognostication, Freundlich says. They also can assess other circumstances that might impact the ability of a cancer survivor to parent a child. For example, cancer treatment might have created a financial hardship. Or, “perhaps they’ve physically recovered, but psychologically they are still kind of stuck back in what happened.” But questions related to financial or psychological resiliency would apply to any prospective parent, she says.

Unfortunately, applicants aren’t likely to challenge an agency’s practices on ADA grounds, Freundlich says. “Are they going to risk completely alienating the agency? That’s the really difficult situation, kind of the power differential between the agency and the prospective adoptive parent.”

Silianoff and her husband, Danny, did walk away, losing nearly $1,000 to agency fees and related travel. “We were pissed, but we basically cut our losses,” she says. They moved on, eventually working with an agency in Austin and waiting on pins and needles for that long-anticipated call.

It arrived a few days before their daughter, Macy, was born in September of 2012. The proud mother describes her curly haired daughter as a “happy little baby,” passionate about Elmo and blowing bubbles. Silianoff, now 32, continues to get ultrasounds every three months and is cancer-free. Last year, she delivered her first book: 

99 Problems but a Baby Ain't One: A Memoir about Cancer, Adoption and My Love for Jay-Z.

Survivors wanting to adopt may be protected from discrimination by the American Disabilities Act. Learn what you need to know.

On the Web: Adopting After Cancer

Fertility preservation, such as storing frozen sperm or embryos, remains the best way to maximize a cancer patient’s chance of conceiving after treatment, says Mitchell Rosen, a reproductive endocrinologist who directs the fertility preservation program at the University of California, San Francisco. But some patients may not have that option due to the urgent time frame to tackle the cancer. Also, insurance coverage for fertility preservation can be complicated by varying state laws and other provisions.

Chemotherapy can damage a woman’s eggs, Rosen says. In some cases, a woman’s menstrual cycle shuts down permanently. A study Rosen was involved in, that published in 2012 in the journal Cancer, found that 3 to 10 percent of 620 women experienced acute ovarian failure after chemotherapy for malignancies including leukemia, Hodgkin lymphoma, non-Hodgkin lymphoma, breast and gastrointestinal cancers.

Some women initially feel as though they are in menopause, including enduring hot flashes, but then their menstrual cycle returns. Citing a 2013 study in the 

Journal of Cancer Therapeutics & Research

, Rosen says that the majority of women experience a return of the menstrual cycle within a year, but in 9 percent of cases, the cycle comes back after a year.

Since some eggs might have been damaged by treatment, female patients might be advised to wait a year before trying to get pregnant, Rosen says. Those women also may face a time crunch, though, as some treatments can trigger earlier menopause—even if their menstrual cycle returns for a stretch.

Men worried about their fertility can get a sperm count. In women, tests can quantify remaining eggs. Above all, speed is of the essence, and Rosen’s own study shows that women can become pregnant despite drug toxicities.

Overall, 19 percent of the 620 women surveyed did report giving birth. Those diagnosed with Hodgkin lymphoma and gastrointestinal cancers were most likely, with 24 percent and 22 percent respectively, followed by women with non-Hodgkin lymphoma (21 percent), leukemia (17 percent) and breast cancer (9 percent).

Nature can still spring some surprises&mdash;even when the toxic effects of cancer treatments have seemingly closed the fertility door for some men and women.

Biologic Surprises: Pregnancy After Cancer

If Emily Beck hadn’t consulted a fertility specialist, many more months might have passed before she learned about the 10-centimeter tumor in one of her ovaries.

At the time, the 35-year-old Philadelphia attorney and her husband had been trying to get pregnant for more than a year when they sought help from a physician and underwent the related tests. A vaginal ultrasound in November 2007 identified a malignancy, leading to removal of her cancerous ovary and fallopian tube, followed by six cycles of paclitaxel and cisplatin over the course of 18 weeks.

Initially, Beck held on to hope that she could still one day bear her own child, and her doctor left her uterus and other ovary intact pending the outcome of further tests. When the pathologist confirmed stage 3 ovarian cancer, as well as stage 1 endometrial cancer, Beck underwent a second surgery to remove her remaining ovary and uterus. Given the severity of her diagnosis, time was scarce and steps such as freezing embryos were simply off the table, she says.

“There was no longer any pretense that I might be able to become pregnant and have my own biological child,” she says. “That’s when the first wave of that mourning process began.”

Beck, now 41, describes those bleak days during a rare window of quiet. Her 2-year-old son, Earl, naps nearby, likely dreaming of trains and the melodies of “Yellow Submarine,” his Beatles fixation of the moment. “He’s insanely gorgeous,” she says, her laughter bubbling up. “We have serious problems.”

For some patients, a cancer diagnosis might close off the option of one day conceiving a child, because of the surgery, drugs and other treatments involved. Choosing adoption is another path to parenthood, one that can be relatively easy—or filled with hurdles&mdash;depending on the agency and its policies and how the survivor’s future is described by the oncologist.

Beck’s journey to motherhood was fairly straightforward, due in large part to a supportive adoption agency. But her story is by no means universal. Some survivors encounter additional layers of resistance and complexity as they strive to convince agencies or other adoption facilitators that they’re well-suited—and healthy enough&mdash;to provide a good, long-term home for a child.

Northwestern University’s Oncofertility Consortium conducted a survey of adoption agencies and created an online list of roughly a dozen “cancerfriendly” agencies, after survivors reported “a lot of hurdles” in the adoption process, says Teresa Woodruff, a researcher and the consortium’s director. In some cases, women would be rejected outright based on their cancer histories. “I was quite shocked by that,” she says.

As with any adoption, there are no guarantees, and the agency might have requirements, such as a doctor’s letter, Woodruff says. “But at least there would be no barrier to walking in the door.”

Regardless of medical circumstances, adoption can be a costly and time-consuming process. Nearly twothirds of domestic adoptions involving a newborn take at least six months and cost an average of about $35,000 to $40,000, according to a 2012-2013 survey of more than 1,100 families conducted by 

International adoptions cost at least as much, and the process can take a few years, depending on the country. Moreover, they can be particularly difficult for cancer survivors because some countries have health-related restrictions, says Kris Faasse, director of adoption services at Bethany Christian Services, one of the agencies identified by the consortium as being receptive to cancer survivors.

Beck and her husband, Mike, first approached the Open Arms Adoption Network, which took her cancer history in stride. The agency’s director, Meredith Rose, estimates that the agency has worked with about 10 cancer survivors in recent years, typically requesting a letter from the individual’s doctor, as they would in any circumstances involving a medical or mental health condition, she says.

In their biographies, which are included in the adoption portfolio, cancer survivors are encouraged to reflect on how the diagnosis and treatment made them more resilient, as individuals and as a couple, Rose says. “I would say that many birth parents are drawn to [cancer survivors’ resilience]. It makes them feel even more comfortable that this family is strong, and they’ve weathered something so enormous, and they are so ready to be parents.”

But not all adoptive parents are so warmly embraced. At least one adoption agency states on its website that individuals must be cancer-free for five years before being considered for a domestic adoption. In addition, those pursuing adoption will sometimes complain that the requirements or restrictions aren’t explained, including within the same agency, says Kairol Rosenthal, author of Everything Changes: The Insider’s Guide to Cancer in Your 20s and 30s. “So, is it then just up to the person who is interviewing you? How are these decisions being made? There is so much uncertainty.”

Attitudes within adoption agencies do appear to be improving, as it’s better understood that many survivors can experience a full life span, says Kristin Smith, a patient navigator with the Oncofertility Consortium. But she also describes how an agency was dropped from the consortium’s list after it balked at working with a woman who previously had ovarian cancer.

Despite the fact that her oncologist had given the OK, “they felt like her life expectancy wasn’t what they had wanted,” Smith says. “This patient was devastated.”

Ensuring that a cancer survivor is prepared to become a parent is part of the screening process, just as it is with any applicant, Faasse says. Once an applicant’s doctor confirms that the cancer history doesn’t pose a parenting issue, the agency delves into other logistics, such as the couple’s plans for child care, access to health insurance, a broader support network and a contingency plan if the cancer should recur.

Also, given that the adoption agency is responsible for the child, all prospective parents are asked, regardless of their prior medical history: What would happen if you got sick?

Applicants who have recently finished cancer treatment need to be honest with themselves and the agency about their emotional and physical capacity, Faasse says. “I probably would ask them, ‘What will happen if you are picked tomorrow? Are you ready to raise a newborn? Do you have enough emotional reserves to really meet the needs of a child?’ ”

When Beck returned to work less than a month after finishing treatment, she threw herself into researching agencies and the adoption process. But, she now says, she gave herself “a colossal panic attack” and backed off, realizing that she was pushing herself too hard in her drive to resume her pre-cancer life plans. The couple put adoption on hold for two years.

Writing in her biography for the adoption, Beck didn’t mince words about that daunting stretch in 2008, briefly detailing her diagnosis, two surgeries and chemotherapy. “I lost my fertility, my hair, a good bit of weight and all sense of security,” she wrote. She also highlighted how the experience forged stronger connections with friends, within her, and led to new interests, such as rock climbing.

Fourteen months after the couple started working with Open Arms, the couple met at a local diner with the agency director, a social worker and a 19-year-old woman who was nine months pregnant. “She had not had an easy life by any stretch and was very wise and sensitive in a lot of ways,” Beck says. “She was a pretty keen young lady.”

The teen credited Beck’s cancer history, among other factors, for having brought them together that day, which was just nine days before Earl was born.

“She said, ‘I just feel like after everything you’ve been through, you really deserve this. You deserve to be happy,’ ” Beck recalls. “That’s when we all started crying. It’s really a moment I’ll never forget.”

Northwestern University’s Oncofertility Consortium wants to expand its list of adoption agencies that are receptive to working with cancer survivors. Contact them with suggestions at 

Building a family after cancer treatment can be a productive challenge.

The Adoption Option

In the nearly nine years since he received a diagnosis of stage 4 head and neck cancer, Ed Steger has experienced a sea change in his ability to access his medical records online.

During the first years following his 2005 diagnosis, as he weathered a battery of treatments that included three major surgeries, 36 radiation treatments and seemingly endless cycles of chemotherapy, the medical records office became a routine stop during each visit to Houston’s MD Anderson Cancer Center. The paperwork accumulated, filling three hefty binders, which he no longer needs to reference.

Instead, Steger can tap a few computer keys and review everything from lab and imaging test results to the notes dictated by his doctors following each visit. These days, after the radiologist calls Steger to discuss his latest round of imaging scans, the 62-year-old Houston resident further reassures himself by reviewing the news online.

“I want to make sure that everything is how I heard it,” says Steger, who’s been cancer-free since late 2007. “It’s kind of a check and balance that I have.”

Once a rarity, this level of online access, through what’s typically dubbed a patient portal, has become more common in the last few years. Increasingly hospitals and doctors’ offices are adopting these portals, protected by passwords and other security measures, so patients can do more than simply schedule their next appointment. Depending on the facility, patients can request a medication refill, check test results or sift through the nitty-gritty of their physician notes. Steger recounts how he’s even downloaded key details of his medical record to help resolve an insurance coverage dispute.

“At times, it’s a really good thing,” says Mikkael Sekeres, a physician who directs the leukemia program at Cleveland Clinic in Cleveland. “I’ve had patients who emailed or texted nurses with pictures of a rash to say, ‘What should I do about this?’”

But some clinicians also worry about unintended consequences for patients with serious illnesses such as cancer, most notably that their untrained view of a test result or physician comment could cause undue anxiety.

“Lab results and radiology results can be indicators of new disease or progression of disease,” says Elizabeth Rodriguez, a nurse leader at New York’s Memorial Sloan-Kettering Cancer Center who has been closely involved with its portal rollout. “That’s different from going online to look at your cholesterol tests.”

With most patient sites, users can view, via a secure online door or portal, their personal medical information from the comfort of a computer or mobile device. Roughly 50 percent of U.S. hospitals already deploy some degree of portal technology, although, in some cases, patient access is still being developed, according to a recent report by global consultant Frost & Sullivan.

The portal can help bridge the miles between doctors or family members. Patients might be able to provide caregivers access to their portal, to keep them in the loop.

[Read how to keep your medical information secure]

Another advantage when the doctor’s notes are available: patients can review the summary after an information-packed and sometimes emotional visit, says Tom Feeley, the physician who heads up MD Anderson’s Institute for Cancer Care Innovation and collaborated on the portal’s development. “A lot of our patients have told us that what they really value [about] being able to go back and read the doctor’s note is to be able to reinterpret what they heard with what someone has written down and really process things,” he says.

The type of information cancer facilities make available and the time involved in getting it posted online can vary significantly. MD Anderson holds all results—lab work, imaging and pathology reports&mdash;for seven days, giving clinicians the opportunity to discuss any worrisome findings with the patients first, Feeley says. Similarly, Cleveland Clinic’s Taussig Cancer Institute gives physicians time to discuss information with patients before posting, with the maximum delay being two business days for lab work, 10 for imaging scans and 20 for pathology results.

Memorial Sloan-Kettering provides lab work immediately but builds in a lag of three full business days for imaging results, Rodriguez says. “Our aim is to show the patients as much of their medical record in real time as possible,” she adds, noting that, after all, it’s the patient’s information. The time frame, she continues, was carefully considered to balance the needs of patients with the concerns of clinicians, who were worried that imaging results might require more explanation.

Consider a patient whose lung cancer is in remission who reads a radiologist’s report that describes the detection of a new 1-centimeter nodule on a recent scan, she says. The patient could overreact, convinced that the cancer has recurred. “It may have,” Rodriguez says. “It may not have. It could be something as simple as the patient’s positioning during the scan. It could be an infectious process and not related to the cancer.”

Still, because some lab results are never even communicated to many patients, the Department of Health and Human Services issued a ruling in February that requires clinical laboratories to provide patients direct access to their own test results within 30 days of receiving a request.

As portals become more common, clinicians are envisioning other uses—benefitting both doctors and patients. Cynthia Tankersley, a nurse and clinical director of survivorship services at the West Clinic in Memphis, Tenn., says patients have been involved in an upgrade of its portal that includes a 90-item symptom survey, the results of which can help physicians identify a patient’s most significant concerns and allow discussion of sensitive subjects, such as sexual dysfunction and other psychosocial issues. Caregivers, who are often best positioned to monitor any changes in symptoms over time, can assist patients with the survey, which can be easily accessed through the portal or on various mobile devices.

I think most people respect boundaries with clinicians. I worry more that patients will be emailing serious medical questions that really deserve a phone call.

MD Anderson also intends to use its portal technology to more closely monitor not only symptoms, but also treatment results, Feeley says.

For example, information obtained through the portal could help clinicians better ascertain a man’s ability to return to normal sexual function after surgery for prostate cancer or a woman’s level of satisfaction with breast reconstruction.

As portals proliferate, one worry is that patients will become too dependent on interacting with pixels rather than people. Consider email, which should be strictly confined to nonurgent situations, Sekeres says. “I think most people respect boundaries with clinicians,” he adds. “I worry more that patients will be emailing serious medical questions that really deserve a phone call.”

Patients also should consider their own emotional stamina before delving too deeply into their online information, the leukemia specialist adds. As it happens, Sekeres doesn’t check his own test results through the Cleveland Clinic’s portal. “I’d rather discuss it with my doctor and have it be placed in context.”

Portal services give patients access to medical records, but some test results could be misinterpreted.

That reticence extended even to a potential medical crisis this past year, when an ultrasound of his heart initially appeared to show evidence of an earlier, silent heart attack. In an essay Sekeres wrote for The New York Times, he describes walking in his patients’ shoes as he coped with the stress of waiting for days until further tests determined that the first scan was technically flawed, and his heart was given the all-clear.

Did Sekeres sign on to the portal while suffering through that medical limbo? Nope, he says. He waited until he could speak with his cardiologist.

Online access to your medical records can be a valuable asset, but shouldn't replace doctor-patient communication.

Making the Most Out of Your Patient Portal

Patients who access their medical records on their physician's portal with a mobile device should do so with caution, security experts say. Carelessness can expose patient data, says Adam Greene, a Washington, D.C., attorney who specializes in health information privacy and security.

Greene and Angela Dinh Rose, a director at the Chicago-based American Health Information Management Association, offer these suggestions for protecting portal information.

Pick strong passwords for both portal access and the electronic devices where the information is viewed or downloaded, ideally ones with a mix of letters and numbers. Check if the mobile devices limit the number of log-in attempts before the user is locked out, Greene says. With some mobile devices, setting a password automatically triggers encryption of the information the device contains, he says. Verify if it does so.

 To further protect the password, strictly limit the number of family members who have access to that information, Greene says. Even better, some portals allow patients to assign access to delegates, essentially sub-accounts that family members could use, once they set up their own password.

 When downloading information from the portal, think about where it’s being stored. One option is to save medical records to an external flash drive rather than on the device itself, Rose says. Encrypted flash drives can be purchased or software to encrypt an existing drive can be located online, she says. And by all means, wipe those aging phones or computer devices clean before sending them out with the trash.

“These have become very expensive disposable devices,” Greene says. “You oftentimes will upgrade your phone after a few years and don’t necessarily take very good care of the data on your old phone.”

Keep your electronic medical records as secure as your paper records.