Dave Dubin is a rock star. That is, he is a rock star among people who are three-time survivors of colorectal and kidney cancer, who started a foundation, who are calendar models, and who are also passionate about soccer … Dave has hereditary nonpolyposis colorectal cancer (HNPCC), also known as Lynch syndrome, an inherited malignancy of the digestive tract. Lynch syndrome mutation carriers have a substantially higher risk of developing cancers of the colon, rectum, endometrium, stomach, ovary, ureter, renal pelvis, brain, small bowel, hepatobiliary tract, and pancreas.
Some persons with Lynch syndrome never develop a cancer; however, most have one or more cancers during their lifetimes, with colorectal cancer (CRC) being the most common malignancy.1,2 If there are multiple cases of CRC on one side of a family, either husband or wife, Lynch syndrome may be the cause. There is also some evidence that the colorectal cancer seen in Lynch syndrome may occur more often on the right side of the body. The disease is also often found at a younger age than among the general population.
The Amsterdam Criteria, the original diagnostic criteria for Lynch syndrome, have been modified. Now referred to as the Amsterdam II Criteria or the Modified Amsterdam Criteria, the revised criteria include the multiple types of cancers that run in Lynch syndrome families.3
• The patient must have three or more relatives with a type of cancer related to Lynch syndrome. One relative must be a parent, sibling, or child of the other two.
• Types of cancers include colorectal cancer, endometrial cancer, small bowel, ureter, or renal pelvis cancer; possibly ovarian cancer.
• There must be a cancer diagnosis in at least two generations of the family, with one or more diagnoses in a person younger than 50 years.
• Familial adenomatous polyposis (FAP) is ruled out as the cause4
According to recent studies, probands (first identified persons affected with the disorder among other family members) of families fulfilling the Amsterdam criteria carry a high risk of rectal cancer and of metachronous colorectal cancer. These studies advise total proctocolectomy, or total colectomy and a 1-year interval of proctoscopic surveillance when a high risk of rectal cancer can be predicted.1
GRANDFATHER, FATHER, SON …
Dubin is all too aware that the disease is hereditary, with the diagnosis coming at a younger age in each subsequent generation. His grandfather was 67 years old when his CRC was diagnosed; his father was 46 years old. Dubin first found out he had the disease when he was 29 years old. He no longer felt indestructible, as most young people do. He felt as though he had been “smacked in the face by a sudden sense of vulnerability.”
The chemotherapy added to the reality. Although it was tolerable at first, after 6 months of treatment, he felt totally defenseless. He was weak, in a constant state of nausea, and had to force himself to eat.
As difficult as it was to know that he had the same serious disease as his father and grandfather, the knowledge also was inspiring. His grandfather survived into his 80s and his father was still alive in his 70s; if they could do it, there was no reason why he could not as well. They were examples of what he could accomplish.
Dubin had recently become a father when his first cancer was diagnosed, and both he and his wife, Robin, quickly decided that they could deal with the disease and move on. He is an avid amateur soccer player, and has been since he was 5 years old. He was not going to stop, and his fellow athletes would not let him.
“The guys I play soccer with are tough. They had no sympathy for me whatsoever. They were expecting me out on the field for games, which was a good thing for me. People react to this differently. There are those who feel doom and gloom. There may be anger. Some people may be confused and feel a heightened sense of vulnerability. And then of course there are those, like me, whose attitude is, ‘Okay, we need to deal with this. We need to embrace it and figure out how to fix it. We have to get past it, and if we cannot get past it what are our options?'”
CANCER FREE? REALLY?
Dubin lived with the belief that he was free of cancer for 10 years, until he went to donate blood and blood tests indicated he was not. His iron count was low, and he realized that there was something more wrong than just the cancer. Dubin was 40 years old and keeping pace with a full schedule of work, volunteering, and other activities. By this time he and his wife had three sons. He was still playing soccer. It had been a year since his last colonoscopy (he undergoes screening annually). He had no symptoms whatsoever.
Dubin and his wife underwent genetic testing and learned about Lynch disease. At that time, he was not worried about surviving another episode of CRC. But when he developed kidney cancer 2 years later, he thought, “‘Great. I’m a breeding ground for cancers!’ My oncologist says her job is to catch them early. She tells me with almost certainty that I am going to have more in the future.”
Dubin copes by maintaining an attitude and a sense of humor. When he goes for a follow-up, colonoscopy, or mammography, he has smiles and terrible jokes for everyone. His behavior helps him and the oncology staff working with him. They pick up on his attitude, even if it is a façade. As Dubin explains, “No one likes to be put under anesthesia or to know they are going to be unconscious for whatever number of hours or minutes. Ultimately you may not wake up, or you may wake up with something else. When I go in for a colonoscopy every year, even though I have no symptoms I could theoretically come out with something else. It is a game of Russian Roulette, to an extent.”
Dubin volunteers for many events and fundraisers to raise awareness of CRC. He was Mr. January in the 2012 Colondar, a fundraising calendar produced by The Colon Club, a nonprofit organization that works to raise awareness of colon cancer and provide support for patients and survivors. Colondar models have struck poses revealing their abdominal scars and sharing their survivors’ stories for a number of years. These women and men, all of whom must be younger than 50 years to qualify as colondar models, send an important message that anyone of any age can battle CRC.
This soccer fanatic recently started his own foundation, AliveAndKickn’, whose mission is to raise funds to increase awareness and prevention of CRC through early detection using screening and genetic testing. In October 2012, the first of many national AliveAndKickathons was held, in partnership with regional cancer centers and professional soccer teams. For 24 hours anyone of any age, sex, or ability could come on the field and play an hour of soccer. Details and photos of the first event, partnered with the John Theurer Cancer Center of Hackensack University Medical Center in Hackensack, New Jersey, are available at www.aliveandkickn.org. More AliveAndKickathons are scheduled throughout the country.
Dubin explains that his ultimate goal is to make AliveAndKickn’ a foundation that people want to be associated with regardless of whether they have had CRC. The disease is the second leading cancer killer in the United States, so awareness and early detection are essential to survival. ONA
Bette Weinstein Kaplan is a medical writer based in Tenafly, New Jersey.
1. Cirillo L, Urso ED, Parrinello G, et al. High risk of rectal cancer and of metachronous colorectal cancer in probands of families fulfilling the Amsterdam Criteria [published online ahead of print September 10, 2012]. Ann Surg; 2012.
2. Steinhagen E, Moore HG, Lee-Kong SA, et al. Patients with colorectal and renal cell carcinoma diagnoses appear to be at risk for additional malignancies [published online ahead of print September 28, 2012]. Clin Colorectal Cancer. doi:10.1016/j.clcc.2012.07.004.
3. Pinar G, Ayhan A. Carcinomas associated with Lynch syndrome: a family history. Int Surg. 2011;96(4):286-290.
4. Lynch syndrome. Cancer.net. http://www.cancer.net/cancer-types/lynch-syndrome. Accessed November 18, 2012.