PHILADELPHIA — For two decades, car-crash victim Rom Houben lay paralyzed and voiceless in what his doctors thought was a vegetative state. He appeared to be awake, but showed no signs of awareness.
But three years ago, a hi-tech brain scan revealed Houben was, in fact, fully conscious and alert inside a paralyzed body — a condition known as “locked-in syndrome” or “pseudocoma.”
Now, 23 years after the near-fatal accident, with the help of an aide holding his hand, the former martial arts enthusiast from Belgium is tapping out messages on a computer screen, one letter at a time, telling the world about his “new birth.”
National Catholic Bioethics Center ethicist Father Tadeusz Pacholczyk, who trained as a neuroscientist at Yale, called the case “fascinating simply in terms of the question of misdiagnoses.”
“Is there a tendency to write people off too quickly?” Father Pacholczyk asked. “The doctor who was in charge of this case commented that a person suspected of being in a permanent vegetative state should be diagnosed at least 10 times.”
“The vegetative state is one of the least understood and most ethically troublesome conditions in modern medicine,” University of Cambridge brain scientists wrote in the Sept. 8, 2006, issue of Science.
Answering journalists’ questions via his computer keyboard, Houben, 46, revealed that years of being trapped inside his own body with no way to communicate left him feeling “alone, lonely, frustrated, but also blessed with my family.”
“Someone had thrown away the key forever,” Houben wrote in an article published in a magazine put out by the care center where he lives, 50 miles east of Brussels. “I was lying there ... day in, day out. I heard. I saw. I felt, but only deep inside, hidden from everyone, but ... myself.”
The neuroscientist who caught the misdiagnosis is Steven Laureys of the Coma Science Group at Belgium’s Liège University. He and colleagues have done research that shows Houben is far from alone. When Laureys’ team used state-of-the-art technology to examine 44 patients diagnosed to be in persistent vegetative state, they unearthed an astounding fact — 18 of the patients (41%) showed signs of awareness.
It can take years before aware and sensitive patients, locked in immobile bodies, are recognized as being conscious. But once a locked-in patient becomes medically stable and is given appropriate medical care, Laureys observed, “life expectancy increases to several decades.”
Houben’s story “is frightening because we’re hard-pressed to understand just how incredible an ordeal it must have been for him,” said Christopher Tollefsen, an ethicist and professor of philosophy at the University of South Carolina. “But it’s also inspiring. It’s like a Helen Keller story, really.”
Tollefsen noted this remarkable case suggests that “a lot of the problems that patients with severe disabilities face are our problems: They’re problems with our own ability to creatively and imaginatively engage with these patients and to offer them opportunities to pursue their human well-being.”
The story is so incredible that skeptics suggest it’s not Houben, but his assistant, who’s really tapping out the words — a process known as “facilitated communication.”
“It’s Ouija board stuff,” said University of Pennsylvania bioethics professor Arthur Caplan, after watching a video of Houben and his speech therapist tapping out messages. “It’s been discredited time and again when people look at it. It’s usually the person who is doing the pointing who is doing the messages.”
Even Laureys was skeptical at first. So he put Houben to the test. “I showed [Rom] objects when I was alone in the room, and then, later, with his aide, [and] he was able to give the right answers,” Laureys told The Times of London. “It is true.”
Summing up the facilitated-communication dispute, Father Pacholczyk said, “There’s evidence to suggest that the communication is real, but it doesn’t appear to be definitively established. Further studies will likely be published to resolve the issue.”
No one doubts Houben was misdiagnosed, though. What’s worse, misdiagnosis of consciousness in cases like this can “lead to grave consequences, especially in end-of-life decision making,” Laureys wrote in a chapter published in Progress in Brain Research and available free online.
Many doctors lack the training required to detect subtle differences in brain consciousness. Whether a patient is in a coma, a persistent vegetative state or a fully conscious locked-in state is often little more than a guess.
“This is an area in which many physicians are not well trained and do not perform well,” said Dr. James Bernat, professor of neurology at Dartmouth. “It requires a certain finesse, a certain skill in training to be able to detect evidence that someone who is very poorly responsive is actually aware.”
Bernat said the finding that 41% of persistent vegetative-state patients are misdiagnosed sounds high, in his experience. Although he does not doubt the rigor of Laureys’ study, Bernat said he’s not sure the Belgian findings can be generalized to other patients in other settings.
A Life Not Worth Living?
Over the years, one person never stopped believing Houben was alert and conscious: his mother, Fina. Refusing to accept the word of his doctors, she took her son to the United State five times for tests. It was she who finally got in touch with Laureys and requested the tests that revealed the truth.
“I was always convinced,” she told an Associated Press reporter. When the breakthrough finally came, she was vindicated.
Of all the extraordinary details in Houben’s story, the one involving his mother is most typical. A recent survey of 44 locked-in patients revealed it was usually a family member (not the doctor) who first realized the patient was conscious and could communicate via eye movements.
It’s hard to imagine a physical disability more cruel than the inability to speak and to move. And yet, in the face of such suffering, many locked-in patients show remarkable courage and lead inspiring lives.
One well-known case was that of Elle magazine editor Jean-Dominique Bauby. In December 1995, the 43-year-old editor suffered a massive stroke. Several weeks later, he emerged from a coma to find himself in a locked-in state able to move only one eyelid.
Determined to show the world his disability hadn’t hampered his spirit, he wrote an entire book, choosing letters by blinking his left eyelid. He also founded the French Association of Locked-In Syndrome to help patients with this condition and their families. Bauby died of septic shock only weeks before his book The Diving Bell and the Butterfly became a best seller.
Since its creation in March 1997, the Association of Locked-In Syndrome has registered hundreds of locked-in patients in France and given researchers a database that offers insights into their lives.
A 2003 study at the University of Michigan in Ann Arbor found that most locked-in persons continued to remain active through eye and facial movements. Listed activities included watching TV and movies, listening to music and books on tape, using the computer, teaching, vocational training, and going to school, the beach, bars and family vacation homes. Among those suffering from locked-in syndrome, no deaths could be attributed to euthanasia, and no locked-in person had a “do not resuscitate” status.
One locked-in attorney uses Morse code eye blinks to provide legal opinions and keeps up with colleagues through fax and e-mail. Another locked-in patient taught math and spelling to third graders using a mouth stick to trigger an electronic voice device.
A 1999 study done at Santa Clara Valley Medical Center in San Jose, Calif., reported that 95% of locked-in survivors surveyed were “glad to be alive.”
Houben would undoubtedly concur. “I shall never forget the day when they discovered what was wrong with me — it was my second birth,” he told reporters. “I want to read, talk with my friends via the computer and enjoy my life now that people know I’m not dead.”
Houben’s story “just proves that some people who are diagnosed as being in persistent vegetative states are incorrectly diagnosed,” Dartmouth’s Bernat reiterated. “It means that doctors who apply that diagnosis need to apply it with great care and not make mistakes.”
Sue Ellin Browder writes from Ukiah, California.