In the eye of the beholder Posted Friday, April 8, 2005 by arjuna

Although little is known about eye cancer, some advances are making patients' lives easier

Robin Summerfield
Calgary Herald

Thursday, March 17, 2005


Sarah couldn't focus. Contact lenses didn't help fix the blurriness and her peripheral vision began to go.

Then, during an Easter lunch date with a friend one year ago, her sight failed.

"Half the menu was black," says Sarah, who nonetheless didn't panic that Friday afternoon. "I just felt there was something that wasn't right, but I just didn't know what it was."

The mother of two went directly from lunch to her optometrist. Monday morning, she was at a specialist's office. (At Sarah's request, her name has been changed for this story.)

The specialist found a large mass, about 12 to 15 millimetres deep, in the Calgarian's right eye.

"I was shocked. It was like it was happening to someone else, not me," she says.

"It was life-threatening. I remember he used those words, life-threatening."

The 47-year-old had eye cancer -- an extremely rare form that effects only six people per million per year in Canada.

In Alberta, about 15 to 20 cases are seen each year.

In Sarah's case, the diagnosis was choroidal melanoma -- a form of eye cancer that attacks blood vessels beneath the retina.

But melanomas -- those cancers most commonly associated with sun exposure on the skin -- can occur in various eye sites.

Uveal melanomas can grow on the eyelid, the iris, the film over the eyeball, the choroid (as in Sarah's case) or in the orbit, which is the fatty tissue between the globe and the skull.

Other cancers, including retinoblastoma, epidermid cancers and adenocarcinomas, are also found in the eye.

They start as freckles or spots on the eye and are usually detected during routine eye exams. Freckles on the eye are quite common with one in 100 people developing a spot.

Most remain benign; a few grow cancerous cells. The rarity of eye cancer has left scientists, physicians and researchers with many unanswered questions, despite much investigation.

"We've got a long ways to go. Our overall cure rate hasn't improved over the last 25 years," says Calgary ophthalmologist Dr. John McWhae, considered the city's top eye cancer specialist.

Physicians can save vision "more often than 20 years ago," but we haven't moved forward in curing or preventing eye cancer from spreading, McWhae says.

Alberta's perpetual sunshine means more people here are likely getting eye cancer compared to other places but, "it's hard to prove because the statistics are not there," McWhae says.

Here's what is known: Eye cancer has no known cause or genetic marker; it is more prevalent in blue-eyed, fair-skinned people and in Australians; and if it spreads, an estimated 85 to 99 per cent of the time it will spread via the blood to the liver, where the eye cancer cells find the only hospitable environment in the body in which to grow.

Treatment can range from chemotherapy to radiation to removal of the eye, the lids and other tissue in the area.

Advances are slowly being made in treatment and reconstruction post-eye removal, but there are still many unanswered questions about cause and cure.

Statistics would help to move eye cancer research and understanding forward, but its rarity precludes that, says Dr. Steve Rasmussen, a Calgary-based surgical pathologist with Calgary Laboratory Services.

The epidemiological statistics an expert would need to make confident conclusions about cause are not available, says Rasmussen, who estimates he biopsies about one cancerous eye per month.

"We are just scratching the surface, because there is so much we don't know about cancer," says Rasmussen, also a clinical associate professor at the University of Calgary.

Surgeons removed Sarah's right eye on June 3, 2004, just one month after her fateful Easter lunch.

"It's really hard to explain when you first look at yourself and you have no eye," says Sarah, who works as a business and industry instructor. "It's shocking and devastating, but you also realize you have to move on, too."

She stayed in hospital for three days and recuperated at home for two days.

"I said to myself, 'I'm just going to move on from here,' " says the 48-year-old. "I realized I had to show strength for my kids. I had to say to them and show them, 'Mom's fine.' "

"Fewer and fewer people are having to have their eyes removed because the treatments are becoming much more advanced," says Dr. Paul Finger, an eye cancer specialist at the New York Eye Cancer Center in New York City.

Tumours are divided into three sizes and treated accordingly. A mass of three millimetres or less is considered small and can be treated with radiation. A mass of three to 10 millimetres is considered medium sized and can be treated with radiation or surgery. However, a mass larger than 10 millimetres means the eye must be removed.

Eye plaque radiation therapy, the implantation of a disc impregnated with low level radiation that targets the tumour during radiation treatment, while simultaneously shielding the healthy eye, is one procedure. Chemotherapy can also be administered orally, intravenously or topically as eye drops. (However, using chemotherapy is not often used to treat eye cancer, says McWhae.)

"Very few tumours have to be removed these days because we are finding them smaller and smaller," says Finger.

So what can people do to protect themselves? Physicians agree sunglasses can't hurt and regular eye exams will help catch the disease sooner.

Sarah took off the bandages within days and lived in sunglasses until she was fitted with a prosthetic eye in Vancouver on July 20, 2005, just six weeks after her surgery.

Doctors stitched a coral implant -- a piece of coral wrapped in sclera (the white of the eye) from the eye bank -- into her eye cavity and connected the muscles. Her new eye is a custom-made, removable half globe that sits snugly inside the coral implant with the help of suction.

The eye, a striking green hazel, perfectly matches the colour of her left eye. Unless Sarah pops it out, which she does once a month to clean it, one would be hard pressed to know it's not real. The only hint the eye isn't normal: it stays fixed, looking straight ahead, but that could be explained away as a lazy eye.

Her top and bottom lids close naturally around her artificial eye, completing the illusion.

She is looking to have another surgery that would permanently screw the half globe into the coral implant and create "extreme movement," she says. If successful, both eyes would move in sync.

Calgarian Roxana Anderson's eyes will likely never move in sync, despite advances in science.

Twenty years ago, she lost her right eye, the lid and much of the surrounding tissue with meibomian cell carcinoma, a cancer that attacked her eye lid. She was left with a 11/2 -inch wide gaping hole in her face.

"It was socially isolating and emotionally devastating," says Anderson, who later joined About Face, an international support group for people with facial differences.

Her first prosthetic was held in place with contact cement, it often jutted out a half inch from her face, took 40 minutes to get in place and she constantly worried it would pop out. So she avoided going out.

"It wasn't worth the effort," says Anderson, now 56.

In 1991, she found a more natural answer at the Craniofacial Osseointegration & Maxillofacial Prosthetic Rehabilitation Unit inside Edmonton's Misericordia Community Hospital.

Using surgery, implants, facial and oral prosthetics, surgeons and specialists rebuild and repair patients with head and neck defects caused by cancer, infections, trauma or birth defects.

They build ears, eyes, brows, noses, jaws and cheeks.

The goal is perfect-looking, or as near to natural-looking, prosthetics as possible.

"If someone had it and you walked by them on the street, unless you stopped and looked, you be hard pressed to know," says prosthodontist Dr. Johan Wolfaardt, the unit's executive director.

In Anderson's case, titanium posts implanted in bone anchor the implant to her face.

Doctors have built her an eye, a bit of cheek and brow using silicon. Her implant includes freckles, wrinkles and extra hair at her brow that match her existing eyebrow.

"There's a lot of artistic work that goes into these prosthetics," she says.

Physicians and researchers have more than artistry in mind when it comes to vision.

"In the long term, and this may sound like science fiction, we will be looking to restore vision," says Wolfaardt, from Banff, where he attended an international conference of specialists in the field.

Next month, Sarah completes her masters in education in adult and higher learning at the U of C. She is thinking about going for her doctorate. She is an avid reader, still drives and loves to downhill ski.

"I just felt that I wasn't going to let it change my life, take over my life, or defeat me."

Cancer, says Sarah pointedly, "doesn't have to end your life."

rsummerfield@theherald.canwest.com
© The Calgary Herald 2005