No One Will Talk About Anal Cancer

A recent campaign photo from the HPV and Anal Cancer Foundation. HPV, or human papillomavirus, causes 90 percent of anal cancer cases, as well as cancers of many other areas of the body. Rosie Hardy/The HPV and Anal Cancer Foundation

Paulette Crowther said she had colon cancer, but she didn't. As she fought for her life, only her children and closest family friends knew her real diagnosis: Stage IV anal cancer. "She didn't even want to say the words anal cancer out loud, let alone talk about it. Colon cancer seemed more normal to her," says Crowther's daughter, Justine Almada. Crowther was diagnosed after a routine colonoscopy picked up a lesion on her anus that was later determined to be squamous cell carcinoma. Further tests showed the cancer had spread to her lymphatic system. In 2010, just two years after the initial diagnosis, she died at the age of 53.

Diagnosed in just over 8,000 people each year in the U.S., anal cancer is a rare disease, but its incidence has doubled over the past three decades and continues to rise at about 2 percent each year. Anyone can get anal cancer, but Crowther's diagnosis was a shock since none of the typical risk factors—having multiple sex partners, having anal intercourse, being HIV-positive or having received an organ transplant—applied to her. She felt fine and had no signs or symptoms leading up to her tests. But much more than surprise, Crowther felt embarrassment. "She had a lot of shame and fear of assumptions people would make—that they would think she had done something to bring on her cancer," says Almada.

Dr. Cathy Eng, an oncologist who specializes in treating anal cancer at the University of Texas MD Anderson Cancer Center in Houston, says Crowther's response is common. "There's a real stigma with anal cancer that doesn't exist with other cancers," she says. Attaching societal stigma to those suffering certain cancers is nothing new. Breast cancer, now celebrated loudly with all things pink, was once only spoken of in hushed tones—it was considered revolutionary in 1974 when then–first lady Betty Ford openly discussed her diagnosis and radical mastectomy. People with colorectal cancers and testicular cancers faced similar social shame, until Katie Couric made colorectal a household word after getting a colonoscopy on morning television and Lance Armstrong went public with his fight.

Anal cancer faces a "double hit" when it comes to prejudice, says Eng. "First, because it's associated with a body part that is already taboo. And second, because it's associated with sexual activity." The prejudice against anal cancer, coupled with its rarity, may make those affected slower to respond to early symptoms, such as itching or bleeding.

"I thought I had a hemorrhoid. I was not exactly in a hurry to have someone check out my hemorrhoid," says Michele Longabaugh, who was diagnosed anal cancer in 2010. Longabaugh has experienced her fair share of raised eyebrows from people when she discloses her diagnosis. "If I had breast cancer, I'd have all the support in the world. With anal cancer, you get the feeling people want you to justify your sex life for them," she says. Almada says her mother's fear of judgment left her feeling alone and isolated. Almada and her siblings were determined to find other patients who might understand what she was going through—but their search revealed no one.

More than 90 percent of cases of anal cancer are caused by human papillomavirus, or HPV, the same virus that causes the majority of cancers of the cervix, vulva, vagina, penis and oropharynx (the middle part of the throat). HPV is passed from one person to another through intimate skin-to-skin contact, most often via vaginal intercourse, anal intercourse or oral sex. An estimated 79 million people in the U.S.—about one in four people—currently have at least one strain of HPV, and most people will be infected with HPV at some point during their lifetime. Of the 14 million new cases of HPV that occur every year, most clear up without requiring treatment. In fact, many people infected with HPV never even know it. But persistent HPV infection leads to 27,000 cancer diagnoses each year, including more than 5,000 cases of anal cancer. Dr. Joel Palefsky, who founded and heads the Anal Neoplasia Clinic at the University of California, San Francisco, says those numbers could be reduced to almost zero through vaccination for HPV infection.

It's been 10 years since the introduction of the HPV vaccine, and today the Centers for Disease Control and Prevention recommends routine vaccination for both girls and boys starting at age 11 or 12. "The goal is to vaccinate kids before they have a chance of becoming sexually active to protect them from developing HPV-related cancers that occur later in life," says Palefsky. But most parents take a pass: Only 40 percent of girls and 21 percent of boys in the U.S. are fully inoculated with the HPV vaccine. There are many reasons for low uptake, but a recent study has shown that a major factor is the pediatricians and family care doctors not strongly recommending the vaccine to parents, many of whom were uncomfortable talking about it. Doctors' hesitancy contributes to ambivalence among parents, many of whom already have concerns about the safety of the vaccines or that it will encourage promiscuity—concerns Palefsky says have "been proved many times over to be unfounded."

Only 40 percent of girls and 21 percent of boys in the U.S. are fully inoculated with the HPV vaccine. One reason for the low uptake is doctors’ hesitancy, which can contribute to ambivalence among parents. Jessica Rinaldi/Reuters

"What they should be worried about is their child getting cancer. It's a no-brainer," says Palefsky. "If you're not recommending the HPV vaccine to both girls and boys, you are not doing your job as a doctor."

As a young woman, Crowther had an abnormal Pap smear during her annual pelvic exam. The routine test, in which a sample of cervical cells are examined under a microscope to detect cellular abnormalities, indicted cervical dysplasia, a condition caused by HPV infection, and which often precedes cervical cancer. The lesions were treated with cryotherapy—frozen by liquid nitrogen and then allowed to naturally slough off. Afterward, Crowther's Pap smears were all normal. But back then, doctors didn't know that cervical dysplasia upped Crowther's risk for anal cancer, so she was never examined for any signs of the condition.

There is no clear medical consensus on screening for anal cancer, but some doctors advocate screening for those who are at high risk, through an anal Pap smear. If precancerous or cancerous cells are found, Palefsky says, they can then monitored or removed at their earliest, most treatable stages. "We believe that detecting and removing HPV-damaged cells should be standard of care for the prevention of anal cancer, but we haven't yet established the evidence for this yet. To put this in perspective, no one knew that cervical cancer was preventable before the use of Pap smears became widespread in the 1960s either," says Palefsky.

Palefsky is lead investigator of the Anchor study, the first clinical trial to study the role of screening for anal cancer. Funded with an $89 million investment from the National Institutes of Health, the eight-year study will investigate whether screening for and treating certain HPV-related abnormalities reduces their risk of developing anal cancer. A nationwide network of 15 hospitals and clinics will enroll more than 5,000 HIV-infected men and women—a population that is 80 times more likely to develop anal cancer—into the Anchor study.

Crowther's family believes the study will prove that screening works. "We think our mom would still be here today if she had received proper screening," says Almada. Anal cancer, when caught early enough, can sometimes be cured with surgery. As it grows, it is usually treated with a combination of chemotherapy and high-dose radiation that 26-year anal cancer survivor Richard Goldman calls "brutal." "I am still managing many of the long-term side effects of my treatment. Colon spasms. Nerve damage. It never ends," he says. But even Goldman is relatively lucky; if cancer spreads to surrounding lymph nodes, the five-year survival plummets to 60 percent, and the illness no longer considered curable. And once it has spread to distant organs, only about one in five patients lives longer than five more years.

With the exception of advances that have made the radiation therapy more precise, the treatment for anal cancer has remained unchanged since Goldman was diagnosed in 1989. Almada attributes the lack of progress to "systemic stigma." "When my mom was diagnosed with anal cancer, there were no new treatments for the disease and none in clinical trials either. What other cancers are using the same treatments for 40 years?" she says.

Three months after their mother passed away, Almada and her siblings established the HPV and Anal Cancer Foundation to raise awareness of the connection between HPV and anal cancer, the importance of HPV vaccination and screening in cancer prevention, and to push for research into new anal cancer treatments. The foundation has, for example, provided grant funding to advance research into powerful new immunotherapies, which rally the body's own immune system to find and destroy cancerous cells without damaging healthy tissue.

One such grant supported Eng's early research into a potential treatment for anal cancer that has spread beyond the anal canal and no longer responds to standard care. Recently published results from her study show that the drug, an immunotherapy called nivolumab already approved to treat other forms of advanced cancers like Hodgkin lymphoma and non–small cell lung cancer, could work: Of the 37 patients in the trial, two had tumors that disappeared and seven had tumors that shrunk considerably. In another 17 patients, nivolumab kept tumors from growing any larger.

The trial was at capacity within months, which Eng says speaks to the "unmet need" of anal cancer patients. "We have been using the same old chemotherapy and radiation since the 1970s, says Eng. "This is a real step forward in a cancer where there have not been many steps forward."