How a warning label nearly wiped out a deadly childhood brain disease
In 1980 American doctors reported 555 cases of Reye syndrome, a rare, fast-moving illness that swells the brain and damages the liver in children recovering from flu or chickenpox. Then health agencies warned against giving children aspirin. By 1994 the count had dropped to about two cases a year, one of medicine's cleanest turnarounds.
Reye syndrome typically begins just as a child seems to be getting over a viral infection. Vomiting, personality changes and confusion can give way to seizures and coma. The liver is harmed, yet jaundice is usually absent, and blood tests reveal high ammonia, low sugar and slow clotting. Between 20 and 40 percent of those affected die, and roughly a third of survivors are left with significant brain damage. The serious effects appear to stem from injured mitochondria, the energy units inside cells, at least in the liver.
Australian pathologist Douglas Reye, with colleagues Graeme Morgan and Jim Baral, published the first detailed description in The Lancet in 1963, though cases may have been reported as early as 1929. The following year George Johnson's team described similar illness after an influenza B outbreak, which is why some call it Reye-Johnson syndrome. The aspirin connection came from epidemiology: in 1979 Karen Starko's study in Phoenix, Arizona found the first statistically significant link, soon confirmed in Ohio and Michigan.
Action followed step by step. The US Centers for Disease Control began cautioning doctors and parents in 1980, the Surgeon General issued an advisory in 1982, and the FDA required warning labels in 1986; notably, cases were already falling before the labels arrived. Britain issued its own warnings in June 1986, and incidence among under-12s fell from 0.63 to 0.11 per 100,000. A French survey of 1995 to 1996 found nine definite cases, eight exposed to aspirin.
The story has a twist. No animal model has ever shown aspirin causing the syndrome, and as genetic testing spread in the 1980s, many cases once labelled Reye turned out to be inherited metabolic disorders. A study of 49 survivors found most had such conditions, especially a fatty-acid processing defect called MCAD deficiency. Treatment remains supportive, with mannitol sometimes used to reduce brain swelling. Adult cases are rare and usually resolve fully within two weeks.
Source: Reye syndrome
