By HEATHER SALERNO
THE JOURNAL NEWS
(Original publication: May 16, 2004)
On Wednesday, May 19 at 8:00 p.m. Eastern/Pacific time, HBO will premiere Three Sisters: Searching for a Cure, a documentary that chronicles the Estess sisters race to save the life of their sister Jenifer.
Valerie and Meredith Estess had planned a holiday get-together on Dec. 17 at the bedside of their sister, Jenifer. The location was nothing new: Jenifer's bed had been the focal point of family festivities since she'd become paralyzed from the neck down by amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease. This party, however, was not to be. The day before, Valerie and Meredith got the call they'd known would come eventually. Jenifer had died that morning, about six years after being diagnosed with the fatal neuromuscular disease.
Jenifer had defied the odds, living to 40, and beyond the two-to-five-year life expectancy of an ALS patient.
But then, her sisters say, she'd always set her own rules.
"What's so remarkable is that Jenifer was the same within illness as before," Valerie says. "Her life didn't become all about illness. (ALS) provided a new set of challenges, certainly. But she was able to see opportunity even in those circumstances."
Rather than accepting what was essentially a death warrant, Jenifer turned her condition into a cause.
In 1998, she and her two sisters, along with friend Julianne Hoffenberg, created Project ALS, an innovative nonprofit organization that has raised $18 million and gathered teams of international scientists devoted to finding a cure. Valerie, 45, who lives in Greenwich Village with her husband and two children, is the group's director of research. Meredith, 41, a married mother of three from Harrison, is the president. (The sisters also have an older sister, Alison, and a younger brother, Noah.)
Now the sisters hope that Jenifer's story told in an autobiography and an HBO documentary out this week will raise awareness of the devastating disease.
"Tales from the Bed: On Living, Dying and Having It All" (Atria Books, $24), which Valerie co-wrote after nine months of tape-recording Jenifer's recollections, hits bookstores Tuesday. Wednesday night marks the television premiere of "Three Sisters: Searching for Cure," a film by Oscar-winning producer Sheila Nevins.
So far, Project ALS-funded research has led to promising discoveries in gene therapy, stem-cell treatments and drugs that are now approved to treat other ailments and might one day help ALS patients. One study, a gene-therapy technique that has extended the life of laboratory mice with ALS, is scheduled for a human trial early next year.
Though these breakthroughs come too late to save Jenifer, her sisters vow to continue to fight for the 30,000 Americans with ALS who are currently struggling to move, talk and breathe and for the millions who might suffer in generations to come.
Of the current U.S. population, it is estimated that 300,000 people will die of ALS.
Any ALS advances are expected to benefit those with neurological diseases like Parkinson's, Huntington's and Alzheimer's. But unlike those illnesses, ALS never affects mental capabilities.
"Your intelligence, your rational thinking, is intact," Valerie says. "You're imprisoned in your own body."
"You know what's coming next," Meredith adds.
"Three Sisters" splices together the Estess family's home movies, interviews with ALS doctors and an unsparing talk with the sisters in Jenifer's Manhattan apartment eight months before her death. At the time of filming, in April 2003, Jenifer was bedridden and using a breathing machine called a bi-pap, a ventilator that she nicknamed her "Hannibal Lecter mask."
"I very rarely show people what I look like in my mask, but I want to show people what it looks like," she told the filmmakers. "This is where it goes to ALS if it's not stopped."
Jenifer is equally frank in her memoir. She remembers her first time using the ventilator as a moment of clarity a sharp reminder of her fate but a sign that made her even more determined to be an advocate for others.
"Even more than paralysis, the bi-pap represented a point of no return," she writes. "Deep inside I knew the medicine Project ALS was fighting for probably wouldn't come in time for me, but that my friends and I had to keep working."
The movie and book confront the harsh reality of ALS, but the sisters believe that they are inspirational, too.
"It shows that maybe there's hope out there for others who feel bogged down sometimes by circumstances," Valerie says. "By a broken heart, or the fact that they can't pay the phone bill."
"But there's a way to get through it. And there's a way to get through it gracefully and with courage. And that's what Jenifer did, right up until her last moments."
Jenifer Estess was diagnosed with ALS in March 1997, when she was 35.
Her sisters encouraged her to see a doctor after she'd experienced odd symptoms. She'd trip unexpectedly and tire easily, and the muscles in her legs and arms would spasm looking, in Meredith's words, like "waves at Jones Beach."
A visit to a specialist confirmed that Jenifer had the disease. The sisters remember the doctor telling Jenifer that some ALS patients max out their credit cards or take long, expensive trips. Eat all the McDonald's you want, he said.
That's because ALS has no known cure. There's also no known cause and no effective treatment.
And the end does not come easy.
"It robs people of muscle strength ... and it affects the same muscles that affect breathing," says Dr. Jeffrey Rothstein, a medical advisor for Project ALS and director of the Robert Packard Center for ALS Research at Johns Hopkins University.
"And if you can't breathe," Rothstein continues, "you basically suffocate to death."
The Estesses refused to believe that there was no medicine available. One government-approved drug appears to prolong the life of people with ALS, but only for several months. The sisters spent thousands on alternative treatments.
They bought a battery-powered muscle stimulator advertised on the Internet. They hired a healer who'd supposedly helped patients by the laying on of hands.
Twice a week, Meredith and Valerie drove Jenifer to Long Island from Meredith's home in Harrison the town where the girls grew up to meet with a psychic masseuse who would "rearrange her cells."
Nothing worked. Yet, as Valerie scoffs, "everyone took Mastercard."
About eight months after Jenifer's diagnosis, the night before Thanksgiving, the sisters and Hoffenberg sat around Meredith's dining-room table. Scientists working on ALS were smart, they agreed, but it didn't seem as if their search for a cure was urgent. Valerie wondered why doctors couldn't be forced to come up with a more immediate plan of action, "kind of like the Manhattan Project."
Brainstorming more, they realized that their professional backgrounds gave them an edge that could make a difference. Jenifer and Hoffenberg were former producers with Naked Angels, a New York theater company whose members included Matthew Broderick, Sarah Jessica Parker, Gina Gershon and Marisa Tomei. Valerie was an advertising copywriter, and Meredith worked in fashion merchandising.
After a few calls to some high-placed pals, Project ALS held its first benefit, in June 1998 at New York's Roseland Ballroom, and raised $500,000, with the help of host Ben Stiller. By 1999, their star-studded galas attended by A-listers like Katie Couric, Hillary Rodham Clinton, Brad Pitt, Jennifer Aniston and George Clooney were raising more than $1 million in one night.
But lots of celebrity-supported charities can raise that kind of cash. Where Project ALS makes its mark, experts say, is in the way the organization distributes that money. Before Project ALS, most of the disease's support groups were focused on patient services, not research. And the research tended to be piecemeal: Doctors worked on individual projects at separate institutions, rarely pooling information and often competing with each other when applying for grants.
Project ALS launched what Hoffenberg calls the "blackmail" approach: Promise wads of money to the world's leading scientists, but only if they agree to work together and share research data with the organization.
"If you're not going to collaborate, you're not going to get the cash. That's it," says Meredith.
Project ALS moves fast. Doctors receive grants within weeks of submitting an explanatory letter about their ideas (which Valerie vets with the group's medical advisors and board of directors).
By comparison, an application to the National Institutes of Health is 50 pages long and takes at least a month to fill out. Doctors can wait up to nine months for a decision.
"(Project ALS) saves years' worth of research time," says Rothstein. "And they spearhead novel ideas."
Meredith and Valerie eventually quit their jobs to work full-time for Project ALS.
Michael Manganiello, senior vice president of the Christopher Reeve Paralysis Foundation, met the sisters in 2000, when Reeve and Jenifer testified before Congress in support of federal funding for stem-cell research. Manganiello remembers being impressed by Valerie and Meredith's devotion to their sister.
"They put their lives on hold for her, but not really, because it created new lives for them," Manganiello says. "I doubt there's one ounce of regret, except for the loss of Jenifer, for the way they've lived their lives. It's only made them better people."
Hoffenberg adds: "ALS strengthened a bond that was already incredible ... They were like one person."
The sisters' closeness goes back to a childhood marked by their parents' divorce when the girls were teens. Their father left their mother for another woman, and he had little to do with the family after that.
The three girls made a pact to stick together, no matter what.
"There are defining moments in life where you can either step up to the plate or go back to bed," Valerie says. "And I think my sisters and I learned that if you step up to the plate, you can really get through anything."
Valerie and Meredith knew Jenifer's health was failing, but to them, her death was sudden.
"Jenifer dying was a huge surprise, a shock," Valerie says.
"It was not supposed to happen," Meredith says.
Jenifer's family and friends were realistic, but they had never lost hope.
"We thought we'd find that maverick working on an experiment, give him a million dollars and have a cure for Jen," says Hoffenberg of Project ALS's early days. "Maybe we were kidding ourselves, but we really thought that."
Meredith says that their dream was for Jenifer to become one of the first ALS patients enrolled in the gene-therapy clinical trial scheduled for next year.
Now the study is "Jen's legacy," she says.
Jenifer herself may have known she didn't have much time left. As best she could, she'd started to prepare her loved ones.
As the documentary highlights, she had long late-night conversations with one of her round-the-clock nurses, worrying about how her sisters would cope when she was gone.
She made a point of telling her young nieces and nephews how much "Aunt Jen Jen" loved them.
And just months before she died, she insisted that Hoffenberg who'd been her roommate for five years move to her own apartment.
"From the moment we moved in together, she felt protective of me, and I think she didn't want me there at the end," Hoffenberg says. "Deep down, maybe she knew it was coming."
Jenifer was also committed to the documentary and the memoir.
"It was a story about sisters, and a story about how love conquers all," Valerie says. "Even if she died, we would still go on."
"It's kind of like 'It's a Wonderful Life,' " Meredith adds. "Without Jen, none of this would be happening. Not this movement in science, not this galvanization of the entertainment community. Not her sisters' deep, deep commitment to making a difference in their world, and in the lives of their children."
In the end, she says, "it's all about the love."