PHILADELPHIA — Ed Rendell was Pennsylvania’s governor in 2010 when he made national news by declaring the country “a nation of wusses.”
Two years later his viral rant turned into a book, “A Nation of Wusses: How America’s Leaders Lost the Guts to Make Us Great,” in which he wrote: “We must regain that American spirit, that boldness and courage, that willingness to take on challenges no matter how hard or how great the risk.”
These days, Rendell, 74, seems to be taking his own advice. Last month, he held a press conference to announce he has Parkinson’s disease.
“I was stunned,” he said of the diagnosis, which he received 3 1/2 years ago. “Like all of us, I thought I was indestructible.”
Rendell was reluctant, initially, to speak out about the diagnosis. But he decided to come forward because he wants people to know how beneficial treatment can be.
“My disease is stabilized, progression has stopped, some of the symptoms are better than they were 3 1/2 years ago,” he said. “Ordinary folks who have these symptoms, who get in early, can get these same type of results. … Treatment is available to anybody who has a health care plan.”
Rendell’s sunny outlook represents a change in the national conversation around Parkinson’s disease, which was once marked by dour prognostications.
“There really is a lot of reason for optimism and hope,” said Vanessa Arnedo, director of research partnerships for the Michael J. Fox Foundation for Parkinson’s Research. “There are more symptomatic therapies that are coming to market. In fact, the FDA is reviewing three of those now. And there are over 20 drugs in clinical testing with the hypothesis that they could slow or stop the progression of Parkinson’s disease in its tracks. It’s exciting.”
The pipeline of development for better therapies, Arnedo added, “is really as robust as it ever has been.”
To keep the progress going, the foundation is looking for Ashkenazi Jews, like Rendell, to participate in its landmark observational study, the Parkinson’s Progressive Markers Initiative. PPMI started in 2010 and is the foundation’s single largest investment to date, with 22 industry sponsors and 33 clinical sites worldwide.
“The goal of PPMI is to identify better markers of Parkinson’s disease progression … an objective measure of the disease, whether a diagnosis or how the disease progresses,” she said.
At the moment, there is no blood test for Parkinson’s, and no way for doctors to assess whether the disease is getting worse or better.
“If we were able to have better objective measures of Parkinson’s disease and how it progresses,” she said, “that would actually help accelerate the therapeutic development of treatments that could potentially stop or slow the progression of disease.”
While Parkinson’s is not considered a strongly genetic disease, there is a genetic link in people of certain ethnic backgrounds. In the Ashkenazi Jewish community, a larger percentage of people who have Parkinson’s also have rare genetic mutations that are likely the cause of the disease.
“The prevalence of Parkinson’s disease in general does not seem to be higher among individuals of Ashkenazi Jewish descent,” Arnedo explained. But the LRRK2 mutation, which is the greatest genetic indicator of Parkinson’s, is much more prevalent in the Ashkenazi Jewish population. The PPMI study is also looking at the GBA genetic mutation implicated in Gaucher disease, which has also been linked to Parkinson’s and is prevalent among Ashkenazi Jews.
“When you actually know what’s causing the disease or you know why someone is at risk for Parkinson’s disease,” Arnedo said, “you really already understand a lot more about what’s underlying the biology of the disease. That’s the reason to want to study individuals who have the genetic mutations. Since we know that individuals of Ashkenazi Jewish descent are much more likely to carry the rare genetic mutations, it makes sense that that would be a community that we would want to encourage to get involved in this research.”
Adrienne Berger, a Philadelphia resident active in promoting the arts, is a PPMI study participant.
She was inspired to get involved after she saw how the disease affected both her late father and her brother, but when she learned the study required her to find out if she had specific genetic mutations, she got nervous.
“I really was hesitant because I prefer not to know,” she said.
When she asked the researchers if she could choose not to be informed whether she had the mutations, they said no, as that information would determine her ongoing participation.
“I decided I really wanted to advance the cause, so I was willing to risk the discomfort,” she said. “It was just a cheek swab that I had to send in and I had to answer some questionnaires. … It didn’t demand that much of me. So how could I not do it?”
It turned out that Berger did not have the mutations, which she was happy to know, given her family history.
“It was a big relief to hear I didn’t have it, but the researcher explained that you could still get it when you don’t have them and you don’t have to get it when you do have them.”
For a layperson, it can be hard to understand the connection between the mutations, the disease and Ashkenazi Jewish heritage, which is why headlines like “Are Jews at Risk For Parkinson’s Disease?” and
“Is Parkinson’s a Jewish genetic disease?” appear in Jewish publications. The subject is further complicated by recent research that suggests links between Crohn’s and Parkinson’s in the Jewish population — again, through the LRRK2 mutation.
But for now, Jewish genetic disease testing centers do not screen for LRRK 2.
“Our program is focused on reproductive carrier screening,” said Karen Arnovitz Grinzai, executive director of JScreen. “Gaucher disease (GBA) is on our carrier screening panel, but we do not test for LRRK2 mutations.”
Dor Yeshorim does not test for it either, and it is not included in any list of Jewish genetic diseases. But people concerned about the LRRK2 mutation can find out if they have it through non-reproductive genetic testing services, such as 23andMe, which is how Sergey Brin, the Jewish co-founder of Google whose mother has Parkinson’s, learned that he carried the mutation.
Or people can join the PPMI research study and find out that way, as Berger did. In time, such participation will likely lead to a clearer picture of how this all intersects.
“It is possible that in the future,” said Arnedo, “as researchers are learning more about the underlying disease and what the genetic factors and variants mean, we may actually identify that more of these cases are due to a genetic factor than is currently known today.”
Meanwhile, progress is being made, Rendell noted.
“It’s not a death sentence,” he said. “It doesn’t have to affect the quality of your life. I was out of Philadelphia 15 days of 31. That’s not a sign of someone afflicted with disease.
“That’s someone who has a disease but is coping with it because of treatment, because of therapy.
“It turns out that I wasn’t indestructible; none of us are,” he concluded. “But it turned out that I could be helped. All of us can be.”
To get involved in the PPMI study, visit michaeljfox.org/ppmi for more information.
Liz Spikol is editorial director of Mid-Atlantic Media, which publishes Washington Jewish Week.