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Pamela Munster's story: Cancer in the family


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Checked Special populations: People with pancreatic cancer

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In the Saturday essay of The Washington Post, Dr. Pamela Munster recounts her family's history with cancer associated with a mutation in the BRCA2 gene. She details her father's extraordinary journey with pancreatic cancer, one of the most aggressive and deadly cancers. (11/27/18)

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ARTICLE AT A GLANCE

This article is about:

a personal story about a father and daughter with an inherited BRCA2 mutation.

Doctors are people too

Dr. Pamela Munster is a breast cancer oncologist at University of California, San Francisco. When she was diagnosed with breast cancer at age 48, she found that she carried a BRCA2 mutation. Women carrying BRCA2 mutations have a 45-56% risk of breast cancer and 23-31% risk of ovarian cancer by age 70. Dr. Munster had a double mastectomy as part of her treatment.

She notes:

"Despite my years as a practicing oncologist—specializing in breast cancer, of all things—I was not prepared for it to come to me."

A family history from her father's side

BRCA mutations may be inherited from either side of the family with the same impact - increased risk of breast and ovarian cancer. In Pamela Munster's family, her paternal grandmother Gertrud was diagnosed with breast cancer at 65. Gertrud survived her breast cancer and was diagnosed with pancreatic cancer which lead to her death in her 80's. Pamela's father voiced words of concern at her surgery they proved prophetic:

"This is just not right...This should be me, not you! You should not have to go through this at your age."

When her otherwise healthy 78-year-old father Norbert complained of stomach pains, she became concerned. She recognized that stomach pains as a potential symptom of pancreatic cancer. Knowing that she had inherited her BRCA2 mutation from Norbert, she realized he was also at increased risk of BRCA2-related cancers including pancreatic cancer. Norbert was indeed diagnosed with pancreatic cancer in 2013.

Pancreatic cancer screening guidelines

NCCN guidelines reccommend screening for hereditary cancer for all individuals with pancreatic cancer, regardless of age of diagnosis or family history. Up to 10% of people with pancreatic cancer will have a mutation in a hereditary cancer gene that may inform their treatment and prevention decisions and those of their family. This includes mutations in BRCA2, PALB2, p16, STK11, ATM, PRSS1 and the Lynch syndrome genes (MLH1, MSH2, MSH6, and PMS2).

People who inherit a BRCA2 mutation account for 5 to 17 % of all hereditary pancreatic cancers. The NCCN gives no specific screening guidelines for previvors with a BRCA2 mutation, but it does notes that "screening may be individualized based on cancers observed" in the family and that investigational screening protocols should be considered.

Pancreatic cancer is the fourth most common cause of cancer death. Five year survival rates are low—less than 9%—in part due to these cancers being found late. Pancreatic cancer has been called a "silent disease" because symptoms, which mimic those of more common diseases, are often not recognized until the cancer has advanced. Symptoms include: jaundice (a yellowing of the skin and eyes), belly or back pain, unintended weight loss and poor appetite, bloating, nausea and vomiting, stool changes, changes in diabetes, and blood clots in veins.

The International Cancer of the Pancreas Screening (CAPS) consortium published a consensus paper in 2013 indicating that pancreatic screening is justified for BRCA2 carriers and other high-risk people with one or more affected first-degree relatives (i.e. parent, sibling and/or child).

As part of their provisional clinical opinion on pancreatic cancer, the American Society of Clinical Oncology (ASCO) indicates that:

 "Benefits and limitations of pancreatic cancer screening should be discussed with individuals whose family history meets criteria for FPC [familial pancreatic cancer] and/or genetic susceptibility to pancreatic cancer."

Coping with BRCA2 pancreatic cancer

At the time of his pancreatic cancer diagnosis, Norbert's doctor stated that surgery was not an option due to his age and the size of his tumor. Dr. Munster, her father's self-stated "advocate and protector" asked his doctor whether treatment would be an option if they could successfully shrink the tumor. She said to her father:

"The odds are against you...But knowing you, I think you would rather go down in flames than not try at all."

They proceeded with aggressive chemotherapy knowing that side effects might be difficult to tolerate and that the treatment might not work. This tradeoff between the hope of treatment and the decrease in quality of life while enduring treatment is one that is balanced differently by each individual. Fortunately, Norbert's tumor responded to chemotherapy and in eight weeks was reduced to half its original size when it was surgically removed.

Tumors of BRCA carriers are more sensitive to some chemotherapy treatments

The responsiveness of Norbert's tumor to chemotherapy, while surprising, may reflect the biology of the BRCA2 mutation. Because cells with BRCA mutations lack functional BRCA protein, their ability to repair DNA damage, including damage in tumor cells that is caused by chemotherapy, is impaired. This poor DNA repair can also lead to other mutations which contribute to increased cancer risk in people with BRCA mutations.

The advent of new treatments like PARP inhibitors for maintenance of BRCA cancers holds promise. PARP inhibitors work by inhibiting a protein that is involved in DNA repair, and in doing so, may help to kill cancer cells. PARP inhibitors are currently approved for treatment of metastatic breast and ovarian cancer but not yet for pancreatic cancers. Several ongoing clinical trials are enrolling participants to test the usefulness of PARP inhibitors (Lynparza, Rubraca, Veliparib, and Niraparib) for treating pancreatic cancer .

The story is not over

Five years after his diagnosis, Norbert is still alive. However, his pancreatic cancer returned two years following his surgery. He underwent more chemotherapy and radiation treatments and is now living with metastatic cancer.

As Dr. Munster points out:

"Metastatic cancer is an ongoing battle. It requires steadfast support, resources, stamina, and, most of all, courage and hope. There are endless setbacks and bad days. But seeing my father emerge from his struggle, my own blue eyes meeting his, I am proud. We are now connected by more than blood and DNA."

She notes that her father's life long habits of exercise and good diet were important. While she does not suggest that these lifestyle choices impacted his cancer, his good physical health before chemotherapy did mean that he was in a better state to endure the difficulties of cancer treatment.

For more about Pamela and Norbert Munster's cancer journey, you can read Dr. Munster's book Twisting Fate: My Journey with BRCA - From Breast Cancer Doctor to Patient, and Back.

What does this mean for me?

If you have a mutation in BRCA2, PALB2, p16, STK11, ATM, PRSS1 or one of the Lynch syndrome genes (MLH1, MSH2, MSH6, PMS2), you have an increased risk of pancreatic cancer. While the NCCN guidelines does not have guidelines for pancreatic screening, they do suggest exploring investigational options (e.g. endoscopic ultrasound). Knowing the signs and symptoms of pancreatic cancer may be useful: symptoms include: jaundice (a yellowing of the skin and eyes), belly or back pain, unintended weight loss and poor appetite, bloating, nausea and vomiting, stool changes, changes in diabetes, and blood clots.

If you have had pancreatic cancer, NCCN guidelines indicate that you may want to have genetic screening for hereditary cancer gene mutations. Knowledge of being a mutation carrier may inform your health care providers treatment decisions and be useful information for your family members.

Questions to ask your health care provider

How does my mutation status impact my risk of pancreatic cancer and other cancers?

With my mutation status or family history, is it appropriate to consider pancreatic cancer screening?

As a pancreatic cancer patient, am I eligible for genetic testing for hereditary cancer genes?

What are the risks and benefits of genetic counseling and testing for me?

Are there any clinical trials that I qualify for?

The clinical trials listed below are currently enrolling pancreatic cancer patients. With FORCE's research study tool you can search the national clinical trials database. For the general research study search tool, click here. For pancreatic cancer trials, click here.

Trial of PARP inhibitor Lynparza (olaparib) for metastatic pancreatic cancerLynparza has been approved by the FDA of advanced ovarian cancer in women with BRCA or PALB2 mutations who have failed chemotherapy.

Trial of PARP inhibitor Rubraca (rucaparib) for BRCA1,2 or PALB2 mutation carriers with pancreatic cancer. Rubraca and Niraparib are currently approved for treatment of ovarian, fallopian tube or primary peritoneal cancers.

A phase II trial of PARP inhibitor niraparib for pancreatic cancer.

Phase II trials of the new, not as yet FDA-approved PARP inhibitor Velaparib.

An investigational phase 1 trial of a new platinum chemotherapy drug for breast, ovarian, pancreatic or prostate tumors

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Posted 11/27/18

References

Munster P, "My Father's Fight Against the Breast-Cancer Gene." The Wall Street Journal. September 28, 2018.


Munster P. Twisting Fate: My Journey with BRCA—from Breast Cancer Doctor to Patient and Back".

Related Information and Resources

American Cancer Society: Pancreatic Cancer

FORCE Information: Genes associated with hereditary cancers

FORCE Information: Pancreatic cancer (in HBOC)

FORCE XRAYS category: Cancer treatment

FORCE XRAYS category: Family and Caregivers

FORCE XRAYS category: Genetic testing

FORCE XRAYS category: Risk for other cancers

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