How to live when you have a 50% chance of developing early onset dementia

It’s just heartbreak after heartbreak after heartbreak.’ An American woman named Barb is speaking to the writer Robert Kolker. She is talking about her pain as she witnesses members of her family succumb to early onset dementia. Everyone knows about Alzheimer’s disease, not so many people understand that there are many other forms of dementia.

Barb’s relatives have frontotemporal dementia (FTD), a disease that attacks parts of the brain controlling personality and behaviour. Typically, it begins to affect people in middle age. In Kolker’s words, they ‘lose the ability to earn an income, look after their children, do household chores, or take care of their hygiene’. A particular genetic mutation means that Barb’s family members have a 50/50 chance of contracting FTD.

Barb’s mother Jean had the disease. One of Barb’s sisters, Christy, was in her 30s when she began to display the first signs. By her 40s, she was losing her previous self. In years to come, sisters Jenny and Mary would fall prey to FTD, as would her brother Dutch.

Some of the manifestations of their disease verged on black comedy. When Cynthia, Christy’s daughter, brought a boyfriend home, her mother regaled him with fantasies of how David Hasselhoff had made Cynthia pregnant.

The more serious symptoms were much more distressing. When police entered Christy’s home for a welfare check, they found her sitting alone amidst mountains of dirty clothes and maggot-ridden cans of fruit.

A rare condition such as FTD does not attract the attention of pharmaceutical companies as Alzheimer’s does. There are not enough patients to make it worthwhile. What research has come up with is a test to discover who has the genetic mutation and who has not. Barb took a long time to decide if she would take the test. She is free of the mutation but what is worse? Having it, or watching loved ones lose themselves because of it. Kolker proves the ideal chronicler for a story that demands sensitive telling. An intensely moving, humane study of a family forced to deal with an unimaginably cruel fate.