A Disease Stops You Sleeping Until You Die. Never Take the Test.

There is a disease that takes away sleep.

Not some sleep. All of it.

It is called fatal familial insomnia. It is caused by a single change in one gene. That change makes a protein in the brain fold the wrong way. The misfolded protein, called a prion, spreads, damaging the part of the brain that controls sleep.¹

It usually begins in middle age. First, trouble falling asleep. Then trouble staying asleep. Then panic attacks. Strange fears. Hallucinations. Sweating. Racing heart. Then the person stops sleeping almost entirely. They lose weight. They lose the ability to think clearly, to walk, to speak.¹

Most people with the disease die within about a year and a half of the first symptoms.¹

There is no cure.

It is inherited. If one of your parents has the gene, you have a one in two chance of having it too. If you have it, you will almost certainly get the disease.¹

It is one of the rarest diseases in the world. Only a few dozen families are known to carry it.² It was first described by doctors in Bologna, Italy, in 1986, after they studied an Italian family who had been losing members to a strange, sleepless death for generations.¹ One writer traced that family's story back to the 1700s.²

There is a genetic test now. A simple blood draw. It can tell you if you carry the gene, years before you get sick.

Many people in these families choose not to take it.

Giulia Ferro was twenty-nine. She translated books for a living, from English into Italian, at a desk in her apartment in Bologna, often late at night because she liked the quiet.

Her family had a word for the disease. They didn't use the medical name. They called it la veglia. The waking. Like a wake for the dead, where someone sits up all night with the body.

Her great-grandfather had it. Her grandmother had it. Her uncle had it. Her father did not. Her father had taken the test at forty, and it was negative, and he had cried in the car for an hour.

Her mother's side was different. Her mother had never been tested. Her mother was fifty-two, and lately she had been having trouble sleeping.

Giulia had a one in two chance, if her mother had the gene.

The family had a rule. Her grandmother had said it so often that it had become a kind of prayer.

Never take the test. Don't invite it in. Not knowing is the last good night's sleep.

When her grandmother died, Giulia was given her notebook. It was written in the old Venetian dialect, which only Giulia, the translator, could read well.

Most of it was recipes and birthdays. The last pages were from her grandmother's final year. The year of her own veglia.

Third month. I do not sleep. I lie in the dark and I am not tired and I am not rested. I am something else. Like a candle that does not burn down.

Fifth month. The doctors say it is the disease. It is. But I want to write down what my mother told me, and her father told her, because nobody in the clinics will.

When the waking is far along, you are not alone at night.

Someone else is awake with you. They sit at the end of the bed. They are very patient. My mother saw them. My uncle saw them. I see them now. A shape that does not sleep either.

The doctors say it is hallucinations. Maybe. But my mother told me something before she died that I will tell you now, because you will need it.

They come for the ones who know. They come to keep them company. That is why we do not take the test. The ones who never knew went quietly, and they were alone at the end, and it was easier.

The ones who knew had someone waiting for them. Sitting at the end of the bed. For years. Long before the first bad night.