Daniel DeFabio, is dad to Lucas, who had the rare disease Menkes Syndrome. Here he shares his thoughts on Knox Goes Away

Written by Daniel DeFabio, The Disorder Channel

Before we even see the title graphic in the film “Knox Goes Away”, the main character gets a diagnosis of Creutzfeldt-Jakob disease. John Knox is played by Michael Keaton, who also directed the film. The cast includes James Marsden, Marcia Gay Harden, and Al Pacino.

Creutzfeldt-Jakob disease (CJD) is an extremely rare disease that can cause memory loss and personality changes within months. As Knox’s doctor tells him, the speed of progression is incredibly fast. There is no treatment.  “You have weeks, not months. I suggest you look into a care facility.”

And so, the film begins a narrative structure divided by a countdown of title cards that read: Week 1, Week 2 and so on.

Like almost anyone recently diagnosed with a rare disease Knox begins to do his own research. He’s shown to be an intellectual with a PhD and an avid reader generally.  And if you catch the reference to the sacrifice made in “Tale of Two Cities” you’ll get a bit of foreshadowing for the plot twist to come. Early on we see him reading the section on CJD in “A Complete Guide to Neurology”. In a nice moment of dark humor later in the film, he literally weaponises that heavy medical text to defend himself.

But Knox’ research does not include following his doctor’s advice of looking into care facilities.  It becomes clear he has built up an impressive nest egg throughout his career, so the issue doesn’t seem to be one of affording care. On the other hand, who of us would want to burn down our life savings on medical costs? As we find out by the film’s final twist his plan for “going away” relies on a very different type of care facility for his final days. And while not too much is made in the film of this point, it may well be a bit of a dig on the US health care system’s costs.

Creutzfeldt-Jakob disease is terrible for anyone to go through. It is fatal with a very fast deterioration. But for Knox there are some added complications which increase the film’s drama. Knox is a hitman for hire, and his deadly skills do not mix well with declining mental abilities. When he makes mistakes, they can be deadly.

The notion of “Going Away” is repeated several times and we’re meant to pick up on the different interpretations of that phrase. Knox himself uses it ambiguously to let some people think he means traveling away or retiring. He also uses related phrases like “I’m slipping” and “I’m cashing out”. Each one working on at least two levels of interpretation. But of course, the deepest level the phrase evokes is to mean the essence of a personality, so embedded in our brains, is diminishing. Is there a point when the core of our identity is gone?

I was glad to see CJD used as more than the gimmick that propels a story we’ve seen before of a hitman losing his mental abilities.  Instead, unique aspects of a deadly and rapidly progressing rare disease infuse the story with elements many of us in the rare disease space might relate to.

Themes around isolation: the detached loner lifestyle may work well for a hitman, but it does not work for someone who suddenly needs a caregiver.

Themes around legacy: Facing the imminent end of his life, Knox’s chief concern centers on what he can leave behind and how to best position his family and loved ones after he is gone.

The theme of “Why me?”: It’s an interesting twist to see a man you’d probably have to judge as unredeemable, if not outright evil, as he questions: does he deserve this dire illness?  Of course, no one deserves to get a rare disease and suffer from it. But this film makes us check some value judgments we may carry. It’s easy to think of “good people” not deserving to suffer. It’s harder to figure out if a merciless killer like Knox deserves just as much sympathy. Does he need to redeem himself to be worthy of empathy? Can a man like him redeem himself?

The film offers a brief moment of sympathy through shared experience, when Detective Emily Ikari who is investigating Knox (played by Suzy Nakamura) realises they have both needed the help of the same neurologist. It’s just a second glance she gives him, but it communicates the notion of “we may not be so different after all” in a way far less conventional than the speech we often get in movies from a villain to a hero before their final face off.

It won’t surprise you that a central part of the plot in this film is Knox feeling he has one last job he needs to pull off before his condition robs him of his abilities. What may surprise you is who the “victim” of that one job turns out to be.

“Knox Goes Away” is streaming on HBO Max.

If you prefer your films on Creutzfeldt-Jakob disease to be documentaries, we have one for you streaming free on The Disorder Channel. The 2014 film “One in a Million: a CJD Documentary” by Trevor Baierl is a ninety-nine-minute examination of the terrible toll of this disease and the state of research for treatments. 

Trevor is a veteran producer of reality TV programs including “Wahlburgers” and “Alaska: The Last Frontier”. Due to CJD being listed on his grandfather’s death certificate, he knew there was a family history of CJD, and when his mother developed symptoms he began to capture some of his last moments with her on camera. We watch as his mom declines and finds it harder to recall details and answer what should be easy questions.

The film takes its title from the prevalence of this disease. Each year one in one million people will become symptomatic with CJD. In the US that means 300 people per year.

CJD can produce not only dementia, but radical mood and personality alterations.  One of the hardest moments for Trevor was telling his mom he loved her, to hear her (very much out of character) scream back at him “I don’t love you.” He tells us it was “Like somebody had taken over her body,” as if it were a possession.

With the genetic version of CJD each child of an affected person has a 50% chance of having CJD themselves. As the film goes on Trevor enrolls himself in a study at UCSF meant to assess the earliest symptoms and warning signs that a person has CJD. He undergoes a series of cognitive tests. In this process he is offered the chance for the genetic test that will tell him if he too has CJD.

But Trevor doesn’t limit this film to his own story and his mom’s. Among those interviewed is Anita Jenkins. When Anita was told her mom’s diagnosis with CJD could mean she has just weeks left, Anita took two months medical leave to be there with her mom. She watched as her mom lost the ability to walk, to prepare her meals, to care for herself, to use the bathroom and by the end her ability to speak.

Unlike most rare diseases, CJD has a variant –sporadic CJD– which is transmitted by contact. But even then, only by ingesting or infusing infected brain tissue or nervous system tissues. Due to a misunderstanding around this aspect of the disease, the funeral home told Anita it was impossible to offer an open casket service for her mom.  Only by enlisting help from the CJD Foundation was Anita able to convince the funeral director that an open casket was a safe option.

Another remarkable interview in the film is with Sonia Vallabh Minikel. She lost her mother at age 52 to fatal familial insomnia which like CJD is a prion disease. Sonia knew she faced the 50% chance that the disease would affect her too. She resolved to get the genetic test. The results revealed a genetic variant and very high likelihood she’d develop a prion disease.  This news would upend anyone’s life, but Sonia and her husband Eric made some remarkable changes as a result. They each quit their careers in 2012 to take new jobs in research labs and enroll as PhD students in biology at Harvard Medical School in order to research prion diseases.

The film ends with Trevor returning to UCSF one year after he first visited there, to learn the results of his genetic test.  Either result would have been a dramatic moment, and Trevor ends his film quite abruptly once the answer is revealed. In that spirit this review will end equally abruptly: Trevor’s test was positive. He will develop CJD.

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