Extract from Continual Raving by Janet R. Gilsdorf

‘Helen Keller reading braille’. Public Domain via Wikimedia Commons

Continual Raving: A History of Meningitis and the People Who Conquered It tells the gripping, human story of how meningitis was defeated by a series of sequential, serendipitous discoveries by multiple scientists in the 19th and 20th centuries. Taken straight from the book, this extract explores whether it was meningitis that left the writer, lecturer, and political activist, Helen Keller, deaf and blind.

Before the advent of antibiotics, meningitis was a dreadful infection by any standard; many of its victims were young children, and almost all died, succumbing to the disease from days to 6 weeks, or sometimes longer, after the onset of their illness. Incredibly, patients occasion­ally survived but were often left with varying degrees of neurologic damage.

In the winter of 1882, when she was 19 months old, the writer, lecturer, and political activist Helen Keller suffered a devastating illness. In her words, from her autobiography: “In the dreary month of February came the illness which closed my eyes and ears and plunged me into the unconsciousness of a new- born baby.” Her illness was said by her doctors to be “congestion of the stomach and brain.” The disease that left Helen deaf and blind, and yet intellectually intact, was consistent with what was then known as “brain fever,” a term commonly used in nineteenth-century literature. It had emerged as a replacement for the word phrensy and referred to inflammation of the brain. The symptoms were described as “a vehement pyrexia [fever], a violent, deep- seated headache, a redness turgescence [swelling] of the face and eyes, an impatience of light or noise, a constant watching, and a delirium impetuous and furious.”

Before the advent of antibiotics, meningitis was a dreadful infection by any standard; many of its victims were young children, and almost all died, succumbing to the disease from days to 6 weeks, or sometimes longer, after the onset of their illness. Incredibly, patients occasionally survived but were often left with varying degrees of neurologic damage.

Brain fever was thought to always be associated with “marked in­flammations of membranous parts” of the brain, and the no­menclature is consistent with the common use of the word fever at that time, often meaning a form of disease, such as typhoid fever, yellow fever, scarlet fever, rheumatic fever, or breakbone fever, rather than an elevated body temperature.

Many citations that discuss Helen Keller’s illness declared, without attribution, that historians and physicians believed she had scarlet fever or meningitis or less likely diagnoses such as typhoid fever, rubella, or encephalitis. Scarlet fever, which has been readily recognized by sore throat, and a prominent rash; at the end of the infection, the skin often peels, similar to a snake shedding its skin. Waves of this illness swept through the United States from 1840 to 1877, with high mor­tality rates, but by 1882, the year of Helen Keller’s illness, both cases and deaths were in decline.

In her book, Helen Keller states that during her illness, “I . . . turned my eyes, so dry and hot, to the wall, away from the once- loved light.” Such light sensitivity was a characteristic of meningitis as clearly described a century earlier in patients with “dropsy of the brain.” When her doctors spoke of “congestion of the stomach,” they may have referred to vomiting, a common symptom of meningitis. And by “congestion of the brain,” her doctors may have speculated, based on what they knew from patients who died of meningitis, that she had “congestion” of the fluid in her cerebral ventricles secondary to inflammation of the meninges. At that time, congestion of the brain would have been a pathologic observation only made at autopsy.

The disease that left Helen deaf and blind, and yet intellectually intact was consistent with what was then known as “brain fever,” a term commonly used in 19th century literature. It had emerged as a replacement for the word “phrensy,” and referred to inflammation of the brain.

If Keller had meningitis, which bacteria would have caused it? Before antibiotic treatment became available, meningitis caused by pneumo­cocci was uniformly fatal. The mortality rates from H. influenzae meningitis were very high (97%) and from meningococcal meningitis moderately high (60%– 80%). Among survivors of both influenzal, as Rivers noted, and meningococcal meningitis, a few patients with deaf­ness or blindness were reported.

Could Helen Keller have had both meningitis and scarlet fever? Scarlet fever is occasionally, but rarely, associated with the development of meningitis and is usually the result of spread of group A streptococci (the bacteria that cause scarlet fever) to the meninges from an infected middle ear. Reports in the 1930s of over 17,000 cases of scarlet fever re­corded only 19 cases of meningitis, and one of these patients recovered, with no neurologic sequelae. An extensive review of scarlet fever in 1904 revealed no evidence of partial or total deafness as sequelae, and the review made no mention of blindness. On the other hand, streptococci were reported as the cause of less than 1%, 4%, and 7% of all meningitis cases.

Indeed, considering Helen Keller’s young age at the time of her illness, her symptoms of prolonged fever and light sensitivity, and her specific neurologic sequelae (complete visual and hearing loss), she likely had bacterial meningitis. Scarlet fever itself isn’t associated with deaf- blindness and would have easily been diagnosed by her physician. Thus, the evidence suggests she suffered from meningo­coccal or, possibly, H. influenzae meningitis (or, less likely, group A streptococcal meningitis) and was one of the fortunate, rare, survivors.

Janet R. Gilsdorf, MD, is the Robert P. Kelch Research Professor Emerita at the University of Michigan, where she cares for children with complex infectious diseases and formerly directed the Haemophilus influenzae research laboratory. Her scientific endeavors involve elucidating the epidemiology of bacterial infections, exploring how bacteria cause infections, and developing vaccines to prevent ear infections in children.