Fatal Familial Insomnia: Yes, Sleeplessness Can Kill

Like so many other Americans, I've suffered from insomnia for most of my adult life. Like so many others, I've spent many nights staring at a computer screen, a ceiling, or the insides of my eyelids for hours on end, desperately hoping that my I-need-to-sleep instinct would eventually kick in. On occasion, I've gone as much as two or three days with no sleep. We insomnia sufferers know how exhausting and maddening it is to do without sleep-- but, for some unfortunate individuals, insomnia leads to hundreds of sleepless nights until it actually becomes fatal.

According to Daniel Max, author of "The Family that Couldn't Sleep," there have only been about 100 cases of fatal familial insomnia-- a condition that causes people to suddenly and completely lose their ability to sleep. The first recorded case appeared in Venice in 1765, when a renowned doctor came to Campo Santi Apostoli, reporting a complete inability to sleep. Over the course of a single hellish year of total sleeplessness, he gradually became insane, then mute, then paralyzed. Two months after he entered total paralysis, he finally passed away.

Today, the descendants of the deceased insomniac, along with members of 39 other families afflicted with fatal familial insomnia, suffer a similar fate. As a genetically dominant trait, familial fatal insomnia afflicts half of the children of people with the disorder. There is no effective treatment for it. Even with the advent of modern medicine, fatal familial insomnia is just as terminal and unbearable as it was at the time of its discovery in 1765.

People with fatal familial insomnia first start showing symptoms some time between age 35 and age 60. Most sufferers are about 50 years old when they first develop the disease. It begins with fairly typical insomnia symptoms: difficulty sleeping, waking up during the night, and perhaps anxiety during the day. This gradually worsens and, by the time the stage has run its course after four months, the fatal familial insomnia sufferer begins to experience a severe decline in mental health.

The next stage involves the loss of sanity, and usually lasts from the fourth to ninth month of the disease's progression. Unable to sleep for more than a few minutes at a time, the person with familial fatal insomnia enters a state in which he is neither completely asleep or completely awake. Dreams and visions leak into his waking life as hallucinations. He has severe panic attacks and anxiety. As the stage ends, the patient is no longer able to communicate normally with other people.

During the ninth to twelfth month of the disease's course, fatal familial insomnia causes the victim to completely lose his ability to sleep. During this stage, even large doses of sedatives can not provide him with any relief. The afflicted person rapidly loses a significant amount of body weight and becomes emaciated. He can not function or communicate normally and is likely to experience heart problems. His symptoms become similar to Alzheimer's and other forms of dementia.

About a year after person with fatal familial insomnia develops symptoms of the disease, he becomes totally mute and unresponsive. His heart and lungs fail gradually. In general, the person can't eat normal meals, may experience choking, and won't respond to commands or communication from other people. In the months following this stage, the person essentially becomes paralyzed, ultimately dying 12-36 months after the disease's initial onset.

This rare disease's greatest mystery-- and tragedy-- its its complete untreatability. Even when injected with sedatives, people suffering from fatal familial insomnia are unable to find any relief from their horrific struggles. Perhaps, in the future, stem cell research and other scientific innovations will reveal a key to unlocking the cure for these unfortunate individuals. Until that time, I'll continue counting my blessings: an occasional anxious, sleepless night no longer looks so upsetting.

David Max's poignant revelation illuminates this mysterious, deadly disease.