by Julianne Dunn Herzog
It never occurred to me that I would be writing the name Angelina Jolie on the same page as the world famous cancer physician/scientist Henry T. Lynch, M.D., chair of the Department of Preventive Medicine and Public Health at Creighton University School of Medicine in Omaha. Nor did I think that Kenneth Cowan, M.D., Ph.D., breast oncologist and director of the Eppley Cancer Institute for Research in Cancer and the Fred & Pamela Buffett Cancer Center of the University of Nebraska Medical Center, also in Omaha, would end up on that same page.
But that is the way it is with cancer, the great leveler, the Emperor of All Maladies described by Siddhartha Mukherjee in his Pulitzer Prize-winning book of the same name.
Dr. Mukherjee tells the 4000-year story of cancer from the Persian Queen Atossa, who commanded her servant to cut off her malignant breast to the last patient of the book, Germaine, who fought a six-year fight against gastrointestinal cancer. Neither knew each other but both fought the same silent enemy as did all the patients and doctors and families in between and going forward.
Cancer grants no favors, honors no exemptions and affects individuals without regard to fame or fortune.
Which brings us back to Angelina Jolie. In early 2013, this famous actress, fiance of Brad Pitt and mother of six children, announced that she had undergone a double prophylactic mastectomy because she learned that she had a hereditary mutation on the BRCA1 gene. She was tested because her mother died from ovarian cancer.
Although more common in the Ashkenazi Jewish population, non-Jewish persons can be affected by hereditary cancer. Ms. Jolie’s ethnic background was not publicly discussed, so it is unknown whether or not she has Jewish origins.
But there was no uncertainty in the response of Ashkenazi Jewish women to the disclosure of her genetic testing and her subsequent action in response to the results. Dr. Cowan said that women were calling oncology centers across the country in a panic, demanding to be put on a surgical schedule for prophylactic mastectomy and hysterectomy because they, too, were worried about the risk of breast and ovarian cancer.
Dr. Cowan said that there should be no such thing as an “emergency” prophylactic mastectomy or hysterectomy due to BRCA1 or 2 gene mutation. These are not women who have cancer. These are women who are Jewish, Ashkenazi, and now aware of a possible risk of hereditary cancer.
The awareness of risk is good. The panic is not.
For most, there is plenty of time to consider the options available for a comprehensive risk assessment by a specialist to define their risk of developing cancer and to develop a plan to manage that risk.
Educating oneself about the danger signs is the first step. A recent article in the genetics section of the July/August 2013 Moment magazine set forth the signs of a possible hereditary cancer:
If any member of a family had any of the following:
• Ashkenazi Jewish heritage and breast cancer before age
• Breast cancer at age 50 or younger;
• Breast cancer in both breasts at any age;
• Both breast and ovarian cancer;
• Male breast cancer;
• “Triple negative” breast cancer; and
• Ovarian or fallopian tube cancer at any age.
and/or more than one relative on the same side of the family with any of these cancers:
• Breast cancer;
• Ovarian or fallopian tube cancer;
• Prostate cancer;
• Pancreatic cancer;
If any of the above apply to you and you are worried, then you can do several things, Dr. Cowan said, to reduce your risk of getting breast or ovarian cancer:
Lifestyle changes: Get your weight under control for your age, height and body build; If you drink alcohol, restrict your drinking to one drink per day; Don’t smoke.
Screening: Breast self-exam monthly: If you don’t know how to do that, ask your primary care doctor or nurse; Annual screening mammogram; If there is a family history, then a mammogram 5 years before the onset of cancer of the first family member and then yearly; MRI as recommended by the physician.
If you are still worried and need to know more about the risk you are facing and the best course of action, your next stop could be an oncologist or a genetics specialist for a complete family and personal history to identify risk.
You can find an oncologist at either Creighton University or UNMC; you can find a genetics specialist at the Hereditary Cancer Center at Creighton University where you will see Dr. Lynch and genetics nurse specialist Carrie Snyder, APRN-CNS, MSN, APNG. Families of this community have long been a part of the extended family hereditary cancer groups at Creighton.
What will happen there is a thorough family history and what is called a “kindred” history. If you have ever seen a geneology map done by someone for a hobby, well, that is what you will be doing with the added information as to whether or not any member of the family had cancer.
This family and kindred history is your lifeline in more ways than one. Analysis of this data can give the genetics specialist the information necessary for you to select treatment options with your physician.
Back in 1970, when he first researched the phenomena of cancer clusters, Dr. Lynch did not receive the worldwide respect and fame which he enjoys today. His theory of environmental causation of cancer stood then-commonly accepted theories of virus causation on their collective heads. His application to present a paper on environmental cluster cancers at a conference in 1971 was declined. When he added hereditary familial cancers to his research targets, many in the close-knit world of cancer research wrote him off and ignored him.
He stayed the course. Now, at the age of 85, he is a world famous physician/scientist who is credited by his peers with not only changing the world of cancer research, prevention and care but also sharing his findings with other researchers. Although he has spent the bulk of his career at Creighton University, his work has influenced two generations of cancer research and treatment all over the world.
The Hereditary Cancer Center has compiled the basic data and records on 1,473 extended familial cancer families affecting more than 30,000 persons, said Dr. Lynch. Of those family groups, close to 200 have the BRCA 1 or 2 gene mutations. The accuracy and reliability of this core information leads other scientists to build upon his findings either in separate research or in collaborative projects such as the one Dr. Lynch and Dr. San Ming Wang, a molecular geneticist at UNMC are now planning.
One of those, Mary-Clare King, Ph.D., now at the University of Washington but formerly at the University of California, Berkeley, is credited with the discovery of the gene BRCA 1 in the 1990s. Dr. King named the genes after Berkeley, CA, where she was working at the time. She echoed Dr. Lynch’s perception of being ignored by mainstream cancer researchers who espoused a “viral” theory of cancer transmission. Change of attitude in science comes slowly.
Advances in cancer genetic knowledge seem to have cleared the way to present action. Even if a person tests positive for the mutation, there are effective treatments short of organ removal, Dr. Cowan said. However, if a person chooses organ removal, then he or she needs to know where to go, who to go to, what surgery is available and who pays.
Next week you will read about non-surgical treatments from oncologists; about surgical removal and reconstructive surgery from general and plastic surgeons, and about the information-gathering and decision-making process from women in our own community who had to make tough decisions based upon the information available to them at the time.