Angels' Smith trying to strike out degenerative disease
Reliever raising funds for study of Huntington's, which affects his mom and maybe him one day
The real Lee Smith is a strong, intelligent woman with a zest for life and a wholesome disposition. Joe Smith will tell you that, and these days the Angels' reliever will frequently say it to himself.
He needs the occasional reminder, because Huntington's disease has progressively robbed his mother of her true identity.
"You really have to look for certain things," Smith said from his home in Dayton, Ohio. "Those little sparks -- when she laughs, or when she's being sarcastic. You have to look a little extra close, and their personalities will shine through sometimes. But as the disease progresses, it just strips them of that along the way. It's hard."
Huntington's disease is a deadly, neurodegenerative disorder that's inherited within families, causing involuntary movements, physical disability, emotional disturbance and cognitive impairment. Smith's grandmother suffered from Huntington's until her death, his mother has been dealing with it for the better part of a decade, and there's a 50-percent chance he or his sister, 28-year-old Megan Nein, will someday get it, too.
Symptoms typically start at around 40 years old, but Smith -- 31 on March 22 -- isn't worried about that now.
"I can still perform in Major League Baseball, so I'm fine right now and that's good with me," said Smith, who's coming off his first season as the Angels' setup man in which he posted a career-best 1.81 ERA and a career-high 4.53 strikeout-to-walk ratio. "Down the road we'll worry about it, like when we start having kids and stuff like that. But other than that, I don't really worry about it. I worry about my mom."
And that's why Smith has a very specific goal this offseason: Raising $2 million.
He and his fiancée, CBS sideline reporter Allie LaForce, are trying to raise awareness and funds for the disease that affects Smith's mother and more than 30,000 Americans, with another 200,000 or so at risk. They launched the website helpcurehd.com two weeks into October, and they had raised $316,000 as of Nov. 22, nearing 25 percent of their goal.
There's no cure for Huntington's disease, but 100 percent of the proceeds would support an important research study by the Cleveland Clinic's Neurological Institute, which wants to use Deep Brain Stimulation (DBS) -- approved to treat similar involuntary movements for other diseases -- to mitigate Huntington's symptoms.
"You don't realize how many things it actually affects," Smith said. "That's something that can extend her quality of life for another 10 years, so that she doesn't really start getting worse until 66. If you've been around somebody that has it, that's an amazing, amazing difference."
Smith's mother is 56 now, way too young -- and smart and driven and charming -- to go through life so restricted. Her symptoms go back 10 years, starting with the involuntary muscle movements that had her unintentionally slamming doors, pots, dishes and cupboards. She started getting anxious easily and coughing frequently, her mind was racing and her words were slurring. Then came the mood swings.
"It wasn't my mom," Smith said. "My mom is the sweetest person on Earth."
Lee was finally diagnosed with Huntington's in February 2012, and all she could think about was watching her own mother suffer through the conditions she would soon experience.
"She knows exactly what's going to happen," Smith said, "and it scares her to death."
Lee no longer drives. She gets tired easily, the muscle spasms are more frequent and she's worried something may happen on the road. The medications are potent, sometimes making her sleep 12-13 hours a day. Her memory is fading, her motor skills are deteriorating and she has a difficult time doing the activities she loves, from golf to Pilates to cooking.
But Lee, a longtime school teacher, hasn't lost her fight.
"I have my bucket list of things I still want to do, and I don't want people stopping me because they think I'm frail or can't do something," Lee said in a video that's posted on the website -- a video Smith can hardly watch.
"She's a very sweet lady," Smith said, "but in no way is she soft."
Smith's father, Mike, can't help but worry about his two children one day suffering from the same illness, but all he can do is cross his fingers.
"The water is basically under the bridge," he said, "so there's not much we can do at the moment except just go forward and do these type of things so we can find out more about Huntington's -- how we can help people with it and maybe someday cure it. The good news is that neither one of them is showing signs."
If his children do have symptoms, they probably won't manifest themselves for another 10 to 20 years. And by then, Mike hopes a cure will exist. Scientists recently uncovered a major contributor to Huntington's disease, providing them with a new target for drug development and offering up a glimmer of hope.
In the meantime, doctors at the Cleveland Clinic believe DBS can greatly improve the quality of life for those with the disease.
Smith met with them in June, when the Angels visited the Indians, and he was told about how the Food And Drug Administration approved DBS to treat the tremors associated with Parkinson's disease, basically the equivalent of the involuntary muscle movements caused by Huntington's (commonly called chorea). Along the way, DBS also helped with memory loss and depression.
The goal is for the FDA to approve it as a means to treat Huntington's, so patients like Smith's mother won't have to pay out of pocket for a neurological procedure that currently costs hundreds of thousands of dollars.
"I want my mom better; I want her to have her quality of life back and I want this disease to just go away," Smith said. "Whether that happens, it's a slim to none chance. But if we can get this, and it saves other people going through the same stuff, then it's worth it."