A lot of people I talk to about A Sleeping Country ask if Fatal Familial Insomnia is a real disorder. They’re wondering if it actually exists, or if Marnich invented it for the play. I was able to find an article about FFI by Pierluigi Gambetti, MD. I found it at the Merck Pharmaceuticals website because if I were Julia and thought I had that is the first place I would look, especially since she’s been trying to take so many different medications to combat it.
“Fatal familial insomnia is a rare prion disease that interferes with sleep and leads to deterioration of mental and motor functions,” Gambetti writes. “Death occurs within a few months to a few years.” Fatal familial insomnia is inherited “due to a specific mutation in the PrPc gene,” Gambetti says. But there is more than one type of fatal insomnia. According to Gambetti, fatal insomnia “can also occur spontaneously, without a genetic mutation. This form is called sporadic fatal insomnia.” Both types of this insomnia affect the thalamus, which is the part of the brain that influences sleep (Gambetti). “The disease usually begins between the ages of 40 and 60 but may begin in a person’s late 30s,” Gambetti says. It starts out with the patient possibly having “minor difficulties falling asleep and occasional muscle twitching, spasms, and stiffness.” Eventually, the patient can no longer sleep. “Occasionally, the sleep signs are difficult to detect,” Gambetti said. “Other changes include a rapid heart rate and dementia. Death usually occurs about 7 to 36 months after symptoms begin.” In order to receive a diagnosis of FFI, the patient should have the typical symptoms as well as a family history of the disease. Genetic testing can confirm this history. Gambetti ends the article by saying, “No treatment is available.”
So we can see why Julia is willing to travel all the way to Italy to find a sufferer of FFI who may be a relative: if there is no treatment for her insomnia and it will kill her, she needs to know. She tells her fiancée, “I have something that’s killing me and that’s not going away and not getting any better and it’s driving us both crazy. I’m sick of having it and I’m sick of bothering you with it. I’m sick of it all. […] I won’t do anything drastic. […] Until I go to Italy. To Venice. To the source. Who is currently Isabella Orsini. I just found her online. She’s sort of the home base for Fatal Familial Insomnia. ‘FFI’” (Marnich, A Sleeping Country, 25-26).
That FFI is a real disease is a little disturbing to me, but I’m glad Marnich is writing about something real real. True, there are some things in A Sleeping Country that are odd, such as the unorthodox psychiatrist Julia visits, but the fact that Marnich is actually writing about something real that could happen makes her play more credible. It tells me that Marnich knows what she is doing, and that she will research things that happen in her work. It also tells me that she won’t sacrifice accuracy in order to tell a story. These are good methods to incorporate into my own researching and writing processes.
Works Cited
Gambetti, Pierluigi. “Fatal Familial Insomnia”. The Merck Manuals Online Medical
Library Home Edition for Patients and Caregivers. January 2007. Web. October, 2010.
Marnich, Melanie. A Sleeping Country. New York: Dramatists Play Service, 2010.
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