A family’s legacy is haunted by a torturous genetic disease.
New York Times science reporter Kolata (Rethinking Thin: The New Science of Weight Loss—the Myths and Realities of Dieting, 2007, etc.) adroitly profiles the plight of the close-knit, rural South Carolina–based Baxley family, hounded by the presence of a “rare, anomalous” neurodegenerative disease. “Abrupt in its onset and unswerving in its course,” the incurable, inherited, and ultimately fatal disease commingles characteristics from both Alzheimer’s and Parkinson’s diseases. Inherited via genetic mutations, the malady renders those afflicted with initial dizziness, followed by severe tremors, facial freezing, mute dementia, and death. Tim Baxley and his three brothers lost their father, Bill, to the disease in the late 1990s after a frustrating cycle of neurologist visits and futile personal fact-finding missions. Throughout the chronicle of the Baxleys and how they scoured their family tree searching for answers, Kolata deftly weaves in the history of kuru disease. She explores the funereal endocannibalistic rituals of Papua New Guinea’s Fore people as both the source of its origins and its outward transmission. American doctor Daniel Carleton Gajdusek performed dogged research on kuru, and he received the Nobel Prize for his work in 1976. Another distinguished researcher, Stanley Prusiner, also won the Nobel in 1997 for his work with associated “mad cow disease,” just as, several years later, Baxley family members began to succumb to kuru one by one. Kolata puts a human face on this incurable, agonizing disease with an affecting combination of neuroscience and anguished anecdotes centered on a loving family at the mercy of an intricate congenital infection. In the concluding section, the story becomes optimistic as the family’s next generation agonizes over the decision to test for the gene mutation but finds hope through a risky, radical profiling procedure that allows for a prenatal diagnosis of at-risk human embryos.
The panic is palpable in Kolata’s moving depiction of a mysterious disease and its frightening consequences.