The book, he hopes, will encourage people to remain curious about possible explanations for family illnesses that can seem unrelated.
“I would love to sit down with my brother and say ‘Hey, this thing [Li-Fraumeni syndrome], why weren’t we paying attention to this? If I could turn back the clock that’s one of the things I would do. I would give my brother a call and say, ‘Hey, could this be us?’”
In 1968, when Ingrassia’s mother died of the breast cancer that had come and gone throughout his childhood, cancer was a bit of a “black box”, he says.
“We just thought ‘this is terrible. It’s bad luck. It’s a little bit rare that she had cancer when she was in her 30s and died at 42 but that happens.’ It was painful but we’re not the only family that had sadnesses.”
More than a decade later, Ingrassia’s youngest sister died from abdominal cancer at 24. Six years later, his other sister died from lung cancer at 32. Subsequently, his brother and his brother’s son succumbed to lung and pancreatic cancer, respectively.
The family did not consider genetic predisposition as a cause until 2015 when the doctor of Ingrassia’s brother suggested he get checked for Li-Fraumeni syndrome - a rare disorder that greatly increases the risk of developing cancer due to mutations in the TP53 gene.
TP53 is basically a “search-and-destroy gene” for potentially cancerous cells in the body, Ingrassia says.
“It looks for them and it either repairs that cell or kills it before it can become cancer ... When it’s mutated, it loses its cancer-fighting power, and the chances of you getting cancer increases dramatically.”
When Ingrassia’s brother tested positive for Li-Fraumeni syndrome, he found it a relief to know what was going on in his body.
Yet Ingrassia - then in his early 60s - was not in such a rush to find out if he too carried this “terrible, terrible mutation” that has a 50% chance of being passed on by a parent.
“I’ve always been a little bit fatalistic, I think, because of seeing my mother and sisters die.”
About a year later though, at his daughter’s request and knowing Li-Fraumeni syndrome can only be passed on by those who have inherited it, he went ahead with the test.
“Sure enough, I was negative. I was clearly the one sibling out of the four, even though it’s a 50-50, chance that [I] did not inherit the mutation which was a great relief, of course, to my children and for their children.”
Although the gene editing technology CRISPR offers some hope that we may be able to repair mutated genes in the future, at the moment there is no way to “fix” a TP53 gene that is pre-programmed to malfunction, Ingrassia says.
For people with such a genetic disorder to get “a fighting chance to have a longer life”, he says extensive annual cancer screening is the only option.
No matter what the cause of cancer - whether genetic or environmental - the chances of treatment being successful are much higher when it is spotted early, Ingrassia says.
Unfortunately, for people with Li-Fraumeni syndrome, even this precaution does not guarantee protection.
“I’ve talked to people who’ve done all the right things, starting with their young children. They’ve done the screening and yet they still get the cancers and sometimes they can’t beat the cancers. So it’s a really tragic syndrome, but there is hope available.”
While writing A Fatal Inheritance, Ingrassia says he had his eye on the calendar, looking out for the date that he would become the longest-living member of his immediate family - 69 years, one month and 10 days.
On this date, to commemorate his family members whose lives were cut short by cancer, he took a ride in a gyrocopter - “a big motorcycle inside a bubble” - on the California coast.
This “very dramatic” experience seemed an unusual but fitting way to celebrate their lives, he says.
“They might have a little bit of a smile on their faces if they saw me doing this.”
