Like many cancer patients, Jennifer Giesel has side effects from treatment.
There’s the neuropathy in her hands, a holdover from chemo. There’s jaw stiffness from her multiple surgeries: an emergency intubation when she couldn’t breathe due to the golf ball-sized tumor on her larynx and two follow-up surgeries to remove the cancer. And then there’s hypothyroidism and xerostomia, or dry mouth, a result of the 35 radiation treatments that beat back the cancer but destroyed her salivary glands and thyroid.
“I went to my primary care doctor a couple of times and mentioned the side effects,” said the 41-year-old laryngeal cancer patient from Cleveland, who was diagnosed two years ago. “She was great but she didn’t seem too knowledgeable about what I was telling her. She was like, ‘Oh really?’ It was more like she was learning from me.”
Patients like Giesel should have an easier time communicating their unique treatment side effects to health care providers with the recent release of new head-and-neck cancer survivorship guidelines. Created by a team of experts in oncology, primary care, dentistry, psychology, speech pathology, physical therapy and rehabilitation (with input from patients and nurses), the guidelines are designed to help primary care physicians and other health practitioners without expertise in head-and-neck cancer better understand the common side effects resulting from its treatment. The goal is that they’ll then be able to better make referrals or offer a holistic plan for patients to get the support they need.
“Head-and-neck cancer survivors can have enormous aftereffects from the disease and treatment by virtue of the location of the primary tumor,” said Dr. Gary Lyman, a public health researcher with Fred Hutchinson Cancer Research Center who helped create the guidelines. “There are functional interruptions, like losing the ability to talk, eat or taste. And some of the surgeries can be disfiguring.
“I’m really glad the American Cancer Society decided to take this on,” he said. “These guidelines are sorely needed, long overdue and will serve cancer patients who are incredibly affected — both physically and emotionally.”
Currently, there are more than 430,000 head-and-neck cancer, or HNC, survivors in the U.S., accounting for around 3 percent of the cancer patient population.
As with many other cancers, HNC is an umbrella term for a number of different malignancies, including cancers that develop in or around the mouth, tongue, throat, nose, sinuses or larynx. Brain, thyroid and esophageal cancer are not considered head-and-neck cancers.
HNC has traditionally been linked to tobacco and alcohol use, and about 75 percent of HNC are related to these risk factors. Increasingly, though, human papillomavirus, or HPV, is causing a significant number of head-and-neck cancers (another reason why the HPV vaccine is such an important prevention tool).
An isolating group of diseases
For some patients with HNC, there can be a certain amount of stigma and isolation, due to its association with drinking and smoking. Treatment can also isolate patients since it sometimes mars a person’s appearance or alters their speech.
Some patients, literally, have no voice.
HNC’s complicated nature — it’s not one disease but several, all of which behave and respond to treatment differently — also results in very small patient populations, which can hinder research.
“Head-and-neck cancer patients have historically been somewhat ignored,” said Lyman, an oncologist with Seattle Cancer Care Alliance, Fred Hutch’s treatment arm. “Many view this as a lifestyle-associated cancer, like lung cancer, heavily influenced by tobacco exposure and [drinking] alcohol to excess. And people may have difficulty dealing with the appearance of some of the more severely affected patients.”
It’s a sentiment echoed by Dr. Eduardo Méndez, a Fred Hutch clinical researcher and head-and-neck cancer surgeon at SCCA.
“It’s in a location that affects your appearance, it affects your ability to speak and to swallow, and those are all things that you need to interact with others,” he said. “It can have an effect of shutting you down from the rest of society. Even the treatment for head-and-neck cancer can have consequences that affect those very same things that the tumor was affecting — swallowing, speech, appearance.”
Not surprisingly, many HNC survivors suffer from depression and/or body image and self-esteem issues after diagnosis and treatment.
“I struggle with body image issues every day,” said Beci Steelman, a 42-year-old court clerk from Bushnell, Illinois, who went through radiation and eight surgeries, including a total right maxillectomy (a surgery of the upper jaw), after being diagnosed with a rare head and neck tumor in 2010.
“You can see that my eye looks like someone’s pulling it halfway down my cheek,” she said. ”My mom and I just call it my googly eye and joke that I have ‘really good face days’ and others that are just ‘face days.’ Clearly something’s not right. When I smile, you can see a bit of metal from the obturator, this weird rubbery dental piece that plugs the hole in the roof of my mouth. Some days I just feel like I’m so ugly.”
Holistic approach benefits patients
There is good news with these cancers: most patients are diagnosed with HNC in its early, most curable stages.
“The majority will be completely functional and normal [after treatment],” said Dr. Christina Rodriguez, the medical oncologist who oversees the majority of HNC patient care at SCCA.
According to the National Comprehensive Cancer Network, around 80 to 90 percent of early stage patients (stage 1 and 2) go into remission after receiving surgery or radiation. Advanced stage patients (stage 3 and 4) receive more aggressive treatment and have lower cure rates, with the exception of patients with HPV-related head-and-neck cancers. Their 5-year cure rates are close to 90 percent.
But even those who go into remission may have to contend with a constellation of difficult side effects.
The head and neck area is “like a fine-tuned machine,” said Dr. Keith Eaton, a medical oncologist at SCCA and Fred Hutch who specializes in lung cancer and HNC. “There are so many dedicated structures that we can’t do without. If you get rid of half your liver, not a problem. If your epiglottis doesn’t work, you aspirate.”
In addition to trouble with swallowing and speech, stiffness in the jaw and problems with shoulder and neck mobility, HNC patients can be left with hypothyroidism, hearing loss, taste issues, periodontitis and lymphedema, the swelling that comes after lymph nodes are surgically removed, a common step in cancer treatment. Because of this complexity, patients need a holistic approach, said Méndez.
Steelman’s cancer extended to the orbital floor of her right eye which meant she had to undergo extensive surgery to her face including the removal of four back teeth, an incision to the roof of her mouth and the shortening of a jaw muscle.
“They got the tumor out and then put me back together,” she said. “I feel like Humpty Dumpty.”
She now wears a prosthetic (which requires daily maintenance) and has had injectable fillers to help with the atrophy around her right eye (an implant in the area became infected and had to be removed). She’s lost hearing in her right ear, her speech is sometimes “a little marble-y,” she has dry mouth from damage to her salivary glands and her jaw will not open as wide as it once did.
Steelman tapped a number of specialists to help her deal with these issues, including an otolaryngologist (ear, nose and throat doctor), speech pathologist, a prosthodontist (an expert in the restoration and replacement of teeth) and a plastic surgeon.
“You have to be your own advocate,” she said. “You learn that very quickly.”
Get help early
Physical therapists, speech pathologists, dietitians and providers with expertise in palliative and pain care (also called supportive care) can improve survivors’ quality of life enormously, especially when therapy is started early.
“Careful — and early — attention to side effects and treatment-related complications can help optimize survivors’ quality of life,” said Eaton, the SCCA oncologist.
Dr. Elisabeth Tomere, a physical therapist at SCCA, said she and her colleagues prescribe exercises that help patients regain strength, range of motion and tissue flexibility that surgery and/or radiation may have diminished. Some patients, for instance, need help building up their trapezius muscle to improve shoulder function they have lost after neck surgery. Others need to learn movements that strengthen the front of their necks and the muscles needed to maintain posture.
Patients with lymphedema in the face and neck — a common side effect from HNC treatments — can also benefit from early intervention by a physical therapist, said Tomere.
“These issues are all helpful to address as quickly as possible so they’re not ongoing,” she said, adding that it may take up to two years for patients to mentally and physically recover from treatment.
“We try to give people a realistic timeline,” she said.
The new ACS guidelines should help providers without expertise in head-and-neck cancers find the right specialists for their patients, she said.
Cancer physical therapy, while new, is becoming more standard. Both the American Physical Therapy Association and the Lymphology Association of North America allow providers or patients to search for specialized physical therapists near them — a boon to primary care providers who are not “connected to that world,” said Tomere.
Dietitians can play a key role, too, since many HNC patients struggle to eat. Treatments can cause dry mouth, taste changes or make chewing difficult. Food can become unappetizing or difficult to ingest.
“There’s an emotional component. Food becomes medicine,” said Linda Kasser, an SCCA dietitian and specialist in oncology nutrition. Patients must eat to keep their weight up, “but it can become exhausting … Sometimes they need to force themselves to eat. They feel pressured, which can contribute to family tensions and even food aversions.”
Dietitians can offer approaches to help patients maintain their weight and strength, from using new cooking strategies to make food more palatable to recommending temporary feeding tubes inserted into the stomach that help patients avoid the pain of chewing and swallowing altogether. They also help alleviate patients’ worries about food and separate “nutrition fallacy from fact,” said Kasser.
Not surprisingly, communication is strongly emphasized in the guidelines.
“We wanted to make sure that there is open communication between the providers and caregivers,” said Lyman. “That there’s a care plan that the patient understands and the caregiver understands. All the different specialists involved in the care should be on the same page.”
The new guidelines also emphasize lifestyle choices that will help to reduce the risk of HNC recurrence and secondary cancers: smoking cessation, limiting use of alcohol, regular exercise and good oral hygiene.
Exciting new research
Chemotherapy, radiation and surgery remain the standard of care for HNC — and drive many of the side effects covered by the new ACS care guidelines — but recent advances are making researchers like Méndez very optimistic for future care.
Thanks to advances in genomics, researchers now know that the mutations found in head and neck tumors vary widely.
“One size will not fit all,” said Méndez. “Treatment will have to be individualized.”
Méndez is leading efforts at Fred Hutch to develop tailored therapies based on the cancer’s genomic mutations, zeroing in on cancer cells’ “Achilles heels” — molecular pathways that tumor cells rely on to survive but that normal cells can do without. The approach is already paying dividends: Méndez is currently leading a clinical trial of a drug he and his team identified that exploits a vulnerability unique to head and neck tumors missing a key gene called p53.
“Once we understand the genotype driving tumor growth, strategies [for treatment] can become more targeted, more effective and less toxic,” he said.
New robotic-assisted surgery has also transformed the procedure for certain patients with tumors in the larynx and at the base of the tongue, allowing surgeons to perform fewer incisions and better preserve functions like swallowing and speech, he said.
Immunotherapy also looks like a very promising path to better HNC treatments.
“New immunotherapy drugs are getting FDA approval for head and neck cancer,” said Méndez. “I think in the next few years we will see it moving to a first-line therapy. It’s a very exciting time for head and neck cancer.”
For patients like Steelman and Giesel, that’s great news.
“I had a social worker who helped me get through the thick of [treatment], but nobody talked about what it would be like when treatment was over,” said Giesel, who had to teach herself how to swallow food a new way (she no longer has an epiglottis). “I thought I’d be returned to myself and I’d be fine, but it was not like that in any way.”
These new guidelines, she said, will help patients like her get the help they truly need.
“Primary care doctors need to know about the physical and emotional effects,” she said. ”I have a lot of good support and know how to ask for help, but I can’t imagine how [patients] who don’t know how to ask for help explain how they’re feeling.”
Do you or someone you love have a head-and-neck cancer? Join the conversation about treatment challenges and how the new guidelines might help on our Facebook page.
Diane Mapes is a staff writer at Fred Hutchinson Cancer Research Center. She has written extensively about health issues for NBC News, TODAY, CNN, MSN, Seattle Magazine and other publications. A breast cancer survivor and patient advocate, she writes the breast cancer blog doublewhammied.com and tweets @double_whammied. Reach her at email@example.com.
Sabrina Richards is a staff writer at Fred Hutchinson Cancer Research Center. She has written about scientific research and the environment for The Scientist and OnEarth Magazine. She has a Ph.D. in immunology from the University of Washington, an M.A. in journalism and an advanced certificate from the Science, Health and Environmental Reporting Program at New York University. Reach her at firstname.lastname@example.org.