Kennedy's Disease no match for chef's indomitable spirit
It was February 2016 and Chef Daniel Jacobs' future was looking particularly bright.
He and his wife, Kate Riley, had recently purchased their first home together. And it had only been a month or two since he'd signed the lease on his first restaurant, Dandan, a high profile collaboration with friend and colleague Chef Dan Van Rite.
But when progressive muscle weakness necessitated a visit to his doctor's office, he found himself facing a medical blow: the diagnosis of Kennedy's Disease.
Kennedy's is a rare, currently incurable and non-treatable, genetic, neuromuscular disease which is passed along via the X chromosome. The disease typically manifests in males, whereas females typically become carriers who can pass the gene along to their sons or daughters. Although the disease does not typically impact lifespan, its hallmark is muscle weakening and atrophy, which over time can severely impact patients' mobility.
It's estimated that one in 40,000 individuals worldwide have Kennedy's Disease; however, many cases go undiagnosed or are misdiagnosed as ALS, or Lou Gehrig's disease.
In Jacobs' case, the disease manifests in daily pain and weakness in his hands and hip. It has also begun to impact his throat and vocal cords; his voice is sometimes raspy, and he says he sometimes finds it difficult to swallow. He's also experienced symptoms similar to sleep apnea, due to muscles in his throat relaxing too much during sleep.
"I have to be very thoughtful about a lot of things," notes Jacobs. "I have trouble walking up stairs. And it's something I need to think about all the time."
The downside of genetics
And Jacobs isn't the only one who has Kennedy's on his mind. Since his diagnosis, both his brother Mike and 14-month-old niece Rosalie have also tested positive for the disease.
"A lot of the early signs are pretty subtle," Mike says. "I have a few things I've noticed, but wouldn't have thought twice about. Dan had enough things that bothered him that he went to the doctor and got the diagnosis. But that's a relatively rare thing. When I took the test, I didn't want it to be positive, but it was nice to know that both of us were going through it together. And that's the bright side."
It was harder, he says, to think about his daughter Rosalie.
"The silver lining is that women are typically carriers for the disease," he says. "Since it's carried by the X chromosome, 99 percent of women who have it don't have the physical aspect of the disease. But it's sad because at some point I'm going to have a conversation with her. And when she has to make a decision about having kids, it's something she has to deal with."
Dan and Mike Jacobs
That reality became a burden for Jacobs' mother Cathy, who says she couldn't get past the thought that she was responsible for her sons' illness.
"I was devastated," she says. "I couldn't believe it. There was so much guilt. We're not sure which side of the family it came from. I don't have any symptoms, and I'm in my 60s, so there isn't anyone in the family I can go back to to find out if they noticed any symptoms."
Mike says one of the worst things is that there's no treatment available for the disease. "There's really nothing you can do," he says. "And that's the sh*tty part. I take a lot of inspiration from Dan and his attitude, because I'm really not like that. I'm sort of dark and introspective. And from that standpoint, he's been really inspiring. I just try to take it one day at a time. I can't let it dominate my view."
On the other hand, Jacobs says the genetic component of the illness has really allowed him to take a step back and refocus on the bigger picture.
"At this point, I don't have much hope that they'll find treatments for Kennedy's in my lifetime," he acknowledges. "But they might discover something that can help my niece. The benefit has always been about her. If they can find something before she gets into her 20s and 30s, that would be great news."
Coming to terms
For Jacobs, the diagnosis itself was relatively swift. But the symptoms had been brewing for years.
"I remember it was probably 2015, both of us had quit smoking and we started working out regularly," says Jacobs' wife, Kate Riley. "And that September we got a trainer. I was getting stronger and stronger; but he wasn't seeing the same results. In fact, he was noticing that there were certain things he just couldn't do."
Jacobs muscle mass didn't seem to be growing, despite the rigorous training, and he began noticing significant weakness on the right side of his body.
"And then when we moved into our new house, he was having trouble lifting and taking things up the stairs," says Riley. "He didn't have any strength in his hip. At first, we just presumed it was an injury, but after a while, it started to really make him wonder."
When a friend's brother, who also works as a physical therapist, suggested the physical symptoms could be neurological, Jacobs made an appointment to see his sister-in-law, a neurologist at the University of Chicago. There he underwent extensive testing, which ruled out ALS, Parkinson's disease and multiple sclerosis, three ailments Jacobs says were all potential culprits.
In some ways, Jacobs says, the diagnosis of Kennedy's Disease was a relief. "We finally knew what it was, and it's not nearly as life-threatening as some of the other things."
But just before his six-month follow-up visit with the doctor, Jacobs says he began having pain and weakness in his hands. He assumed it was carpal tunnel, a common malady among chefs. But that, too, turned out to be a symptom of the worsening disease. And it hit home.
"When we found out it was just a progression of Kennedy's, I was incredibly bummed," says Jacobs. "And it took me a few days to break out of that. But out of that came the idea to host a dim sum event to raise money for the Kennedy's Disease Foundation. It's one thing we, as chefs are great at: raising money for charity. And I knew I had to do something."
(PHOTO: Kate Bonamasso)
The struggle is real
Riley, who works as a ceramics artist, says the days since Jacobs' diagnosis have been filled with both good and bad. Among the good, she says, is the amount of time they're able to spend together.
"Our life is so different now," she says. "He needs more physical downtime to recuperate from long days at the restaurant. I've always been more of an introvert, and he's more of an extrovert. So I love it that we spend more time together at home."
On the other hand, the need for better health insurance – a luxury for many in the hospitality industry – is a factor that sits above her head like a black cloud.
"It takes a lot of money to maintain his health," she says."Right now we have high-deductible free-market health insurance. And we can't go on like this. It's one of the things that has been stressing me out … and now he has a pre-existing condition. It's frustrating. And I hate that. They're thoughts that are running through my head constantly. We're both very lucky because we're able to do what we love. But it makes me question whether or not I need to find a 'real' job."
Jacobs says his day-to-day has changed fairly dramatically over the past year.
"I really like prep, so I try to do what I can," says Jacobs. "But I don't break down fish anymore. I can't break down lobsters. It just aggravates things. I've also had to change the way I work out. Lifting weights and running are out the window. Mostly now I do low impact activities like bike riding."
Tasks so many of us take for granted – such as opening jars – are often performed by others in the kitchen, including pastry chef Jaceleen Latin-Monagle.
It's a simple action that Chef Paul Zerkel of Goodkind says made the news of Jacobs' diagnosis hit home. "It didn't hit me hard until about a month ago," he says. "He asked my wife, Lisa, to open a jar for him. And I was like 'Wow.'
"He's always prided himself on doing all the little monotonous things that he doesn't have to do. And there's a lot of humility in that. So, losing his hands is a big deal. And I think he'll miss that the most. It's the time when he comes up with his dishes, his inspiration."
Dandan co-owner Chef Dan Van Rite says he takes care of the tedious prep at the restaurant on weekdays while Jacobs handles the office work. It's a trade-off that gives Jacobs a break from the physically taxing kitchen tasks so that he can assist with dinner service.
"We've struck a good balance in how we work together." Van Rite says. "Every day is different. If it comes down to it that he's in the office all the time, we'll make it work. And if he needs help, I'm there for him."
And that phrase, "I'm there for him," rings out over and over as you speak to Jacobs' family and friends.
"As his best friend, I can't just sit back and watch everyone feel sorry for him," says Zerkel. "He knows that I think about him and care about him a lot. But I try to keep it lighter and remind him that everything is going to be OK."
"I made him a promise when he first found out that I would do anything I can to help him out – except helping him wipe his ass – I'll never do that," he laughs. "I always joke that I'll carry him around when he gets really bad. I'm the jester in the corner who tries to keep him laughing."
Jacobs says their friendship has been a boon.
"I don't need Paul to be sappy with me," he says. "I don't want to be serious about it. There's plenty of time for that later on. For now, I love the fact that we can joke around about it. I need that part of it. There's enough to worry about in the day to day."
Cathy Jacobs says she's grateful her son has such an amazing support system.
"I'm really happy that he has a wife like Kate," she says. "She's a rock, and she's there for him. There's so many people. I love Chicago. But, one of the things I love is the community in Milwaukee. The whole chef community and people in the industry are really loyal to one another. The support that they come forward with, and the way they stand up for one another. It's really great."
Stubborn as sh*t
"He's stubborn as sh*t, so he's not going to let this get him down," says Zerkel when I ask him what he's observed about Jacobs over the past few months. "He's going to keep going as long as he can."
And that's always been a part of who Jacobs is. He's loud. He's sometimes brash. And he's determined.
"I think, of the two boys, Dan is a little more emotional," says Cathy. "He's just so positive, and he works so hard for whatever he wants to get. As a result, he's facing this head on. He's accepted it and is going to move forward and do whatever he can. I'm so proud."
Riley says it's all about looking forward.
"We opened a restaurant this year. We bought our first house this year. And we got this diagnosis," says Riley. "It's a lot. But we're not going to let this take over. That's no way to live. So we're both doing what we love. And we're going to keep doing it until we can't anymore."
Jacobs agrees wholeheartedly.
"This is something I need to deal with for the rest of my life," he says. "And I've come to terms with the fact that I'm eventually not going to be able to do certain things. But I can't fret about it. I'm a Cubs fan. And we're optimists. Every year we go into spring training thinking: 'This is the year!' And that's the story of my life."
"Days that are good – when I don't have pain – are a blessing," he goes on. "And I want to live life and do the things I love. I want to cook for as long as I can. I want to ride my bike and play with my dog. I want to go skiing before I can't do it anymore."
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