Health and Fitness

Her grandmother, mother and several aunts died of cancer

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Sunrise, May 10, 2022 – Cheri Pribyl, right, and her daughter Rachel, 25. Cheri’s grandmother, mother and several aunts died of either colon cancer or breast cancer before they turned 65. Cheri had a genetic test done at Cleveland Clinic Florida and learned she has a genetic mutation known as Lynch syndrome, which increases the risk of colon, breast, uterine and stomach cancers. Her two daughters have the same genetic mutation.

jiglesias@elnuevoherald.com

Cheri Pribyl thought her family’s high incidence of cancer was caused by toxic sludge dumped at a site around her mother’s New York neighborhood: Love Canal.

Pribyl’s grandmother, mother and several aunts all died of either colon cancer or breast cancer before they turned 65, and many of her mother’s neighbors who lived around the contaminant-filled landfill in Niagara Falls, New York, also became ill.

“They had a lot of neighbors and they themselves were sick with different cancers, all five siblings, and they attributed it to living near the dumpsite on Love Canal,” said Pribyl, now 57. “My mom’s best friend, in 1972, died from leukemia. She had many miscarriages, and the one child she did have died.”

From 1942 — the year Pribyl’s mother, Christine McCabe, was born — until 1953, Hooker Chemical Co. dumped 21,800 tons of toxic chemicals into the 16-acre landfill, a 1981 New York State Dept. of Health report said.

“Hooker covered those barrels with earth and then sold the land to the city’s Board of Education — for a dollar — and the city built an elementary school and a playground directly on top of it,” said Keith O’Brien, author of “Paradise Falls: The True Story of an Environmental Catastrophe.”

In the late ‘70s and early ‘80s, the federal government closed the site, remediated it and relocated the families. Congress passed a law in 1980 that established the federal Superfund program to clean up hazardous waste sites.

“There were roughly one thousand families living in the neighborhood,” O’Brien told the Herald.

Pribyl’s family was among those living there before moving to Broward County in the ‘60s.

Family’s cancer background led to test

Pribyl’s belief that her family’s cancer was caused by Love Canal changed after she went to Cleveland Clinic Florida in Weston in 2018 to get a routine mammogram.

“I had gone and had my mammogram,” said Pribyl, a Broward County school district employee, who described how her medical history form raised eyebrows. “The technician at Cleveland Clinic said, ‘Wow, you seem to have a lot of cancer especially on your maternal side. Have you ever been genetically tested?’”

She had not.

The technician suggested Pribyl undergo genetic testing, and follow up with a genetic counselor.

“I just spit into a vial, and they sent it off,” Pribyl said, explaining a procedure similar to what some people do when they trace their ancestral lineage. “When it came back with Lynch, that was like wow. They said we had the PMS2 gene.”

Lynch syndrome diagnosed

Pribyl’s genetics test showed she had Lynch syndrome, an inherited cancer that stems from mutations in the MLH1, MSH2, MSH6, PMS2 or EPCAM genes.

People diagnosed with Lynch syndrome have a much higher risk of developing colon cancer, and also are at increased risk of developing uterine, stomach, liver, kidney, brain and certain types of skin cancers, according to the Centers for Disease Control and Prevention. They also have a higher risk of contracting these cancers at a younger age (before 50), the CDC says.

Lynch syndrome is also one of the most common inherited cancer syndromes, with estimates saying that 1 in 300 people may carry one of the genetic mutations, according to the American Society of Clinical Oncology.

“Lynch syndrome is a condition that causes an increased risk for cancer to run in the family,” said Sara Rhode, a genetic counselor at Cleveland Clinic Florida Naples who worked with Pribyl. “It is quite common, actually, about 1 in 280 people have Lynch syndrome.”

However, most people with Lynch syndrome are unaware that they have it, Rhode said, because they have not undergone genetic testing.

The syndrome was named after Dr. Henry T. Lynch, a 6-foot-5 former professional boxer who pounded opponents as “Hammerin’ Hank.”

Risk of cancers increase

Lynch initially sought to unlock the genetic roots of schizophrenia. But about 50 years ago, his focus turned to whether cancer was caused by exposure to environmental toxins.

Many cancers, Lynch said, were hereditary. Upon his death, in 1991, the Lancet called Lynch a “leading early figure in cancer genetics.”

“If someone has Lynch syndrome, their risk for certain cancers can be increased,” Rhode said. “For example, the risk for colon cancer can be somewhere between 10 and 74 percent lifetime risk.”

One of the challenges with Lynch syndrome is that there are five different genes associated with it, Rhode said. And each gene presents a different level of cancer risk.

“There is anywhere from 10 to 14 different types of cancers that can be associated with Lynch syndrome,” Rhode said.

While there is no cure for Lynch syndrome, Rhode said there are some preventive measures that can be taken, including starting colonoscopies at an earlier age and at greater frequencies.

“Finding a polyp and removing it prevents a colon cancer from developing,” said Rhode, who said those interested in obtaining a genetic test should first consult with their primary care provider.

Pribyl credits the doctors at Cleveland Clinic Florida for saving the life of her 25-year-old daughter Rachel, when they removed an aggressive polyp in her colon when she was 23. Her oldest daughter, Rebecca, 29, also has Lynch syndrome and has been getting colonoscopies annually without any findings.

“If you have it [Lynch syndrome], they said there is a 50 percent chance your offspring may have it also,” Rhode said. “It’s good information to have because these are preventative cancers.”

Pribyl is now on a mission to raise awareness about Lynch syndrome and potentially help save more lives.

Genetic counseling

For information about genetic counseling, contact:

Cleveland Clinic Florida in Weston: (954) 659-5000 or my.clevelandclinic.org





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