- In episode three of Hulu’s TV show, The Act, Gypsy Rose and Dee Dee Blanchard attend a cosplay convention.
- There, Dee Dee meets a man whose son suffered from (and later died of) Batten disease.
- Batten disease is a rare, fatal genetic disease that mainly affects children.
On Wednesday’s episode of The Act, Hulu’s newest (incredibly addicting) TV show, Gypsy Rose Blanchard’s health issues weren’t on full display—instead, it focused on one specific event: a cosplay convention.
But, while Gypsy (played by actress Joey King) was still at the forefront of the episode, aptly named “Two Wolverines,” her mom Dee Dee (played by Patricia Arquette) got her own separate storyline, and it involved a man who showed interest in her.
At the convention, Dee Dee catches the eye of Russ (played by Dean Norris)—one of the aforementioned two “Wolverines” (a.k.a., they dressed up as the comic book character, Wolverine). Russ strikes up a conversation with Dee Dee, clearly trying to find out more about her—but Dee Dee, though intrigued, isn’t playing along.
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When Dee Dee says she’s at the convention because it’s something Gypsy likes, Russ asks, “Well, what do you like?” When Dee Dee answers again with more of what Gypsy likes, Russ asks again: “What about you” to which Dee Dee replies “I don’t understand.” Finally, Russ says it seems like Dee Dee has “layers” to her—and that he’d like to find out more about them. Dee Dee, clearly flustered, excuses herself.
When the two meet again later on, Russ tells Dee Dee he didn’t mean to scare her off earlier—and explains that he’s been in a similar situation. “I lost my boy when he was 13,” Russ said. “It was Batten disease; he was in a chair at the end, so I understand how your kid could be your everything.”
And then Russ offers her some advice: “You gotta cut out a little piece of the world for yourself…you can lose yourself in all the giving.” Dee Dee, of course, thanks him and heads off to give Gypsy more medication.
Wait just a minute. I’ve never heard of Batten disease…what is it?
So, Batten disease is kind of an umbrella term—it covers a broad class of rare, fatal inherited disorders of the nervous system, also known as neuronal ceroid lipofuscinoses (NCLs), according to the National Institute of Neurological Disorders and Stroke (NINDS).
The NINDS says an estimated two to four out of every 100,000 children in the U.S. are affected by Batten disease, though many more individuals may be carriers.
Each form of the disease is caused by a mutation in a specific gene, which essentially interferes with the body on a cellular level, specifically a cell’s ability to recycle certain molecules. (FYI: In a process called autophagy, your body’s cells essentially clean house, getting rid of molecules that are no good and reusing ones that are.)
Most forms of Batten disease begin in childhood, per the NINDS. Common symptoms for most forms of Batten disease include vision loss, seizures, dementia, and abnormal movements. According to the NINDS, children with Batten disease eventually become “blind, wheelchair bound, bedridden, unable to communicate, and lose all cognitive functions.” Children with Batten disease also have a decreased life expectancy.
There’s no known treatment or cure for any form of Batten disease, but some medications, along with physical therapy, can help symptoms and allow those with the disease to remain functional for a longer time.
Clearly, the disease is devastating. It’s unclear whether Russ was an actual part of Dee Dee’s life or just part of the show, but regardless, it really makes Dee Dee look…extremely insensitive, don’t you agree?
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