![fatal familial insomnia fatal familial insomnia](https://i.ytimg.com/vi/nVjdfBOq6d4/hqdefault.jpg)
![fatal familial insomnia fatal familial insomnia](https://www.dirjournal.com/info/wp-content/uploads/2012/09/FFI-1.jpg)
FFI baffled investigators for years, because certain symptoms resemble encephalitis, end-stage alcoholism, and dementia, among other conditions (1). More children, however, also means more potential FFI cases- a tough paradox for the I-function to work through.
#Fatal familial insomnia free
Paradoxically, it is in the parents' best interest to have more children, in order to ensure that at least some live FFI free into old age. Because it does not strike until middle age, however, parents who may be carriers must make difficult decisions about childbirth. If this disease occurred in childhood, natural selection would have long ago done away with it. Here the first questions of the I-function, and the first paradoxes of the disease, appear. If parents have such a high chance of passing on the disease, one might ask, why don't they simply choose not to have children? The answer: FFI, unlike many other fatal genetic conditions, doesn't appear until the victim is middle-aged, and tests to see whether parents carry the FFI mutation only recently became available (1). But every other aspect of the disease is wildly unpredictable, forcing family members to make difficult decisions. Pierluigi Gambetti, one of the discoverers of the condition known as fatal familial insomnia (FFI), claims that it is "the worst disease you can get." (5) Given the vast number of diseases in the world, Gambetti's claim seems farfetched at first glance, maybe even selfish who wouldn't want to take credit for discovering one of the worst diseases in the world? But a quick overview of the disease presents solid evidence in favor of the claim- and some interesting insights about the many tasks of the I-function.įFI has been discovered in only 28 families worldwide it is an autosomal dominant gene mutation, meaning that a parent with the disease has a fifty percent chance of passing it on to his or her child (2).