A rare condition called body integrity identity disorder (BIID) is back in the news after a North Carolina woman who claims to have the ailment shared her story in hopes of bringing more attention to it.
People with BIID feel, from early in life, that they should be disabled.
In most cases they experience one of their limbs as foreign and are driven to have it amputated. One man whose case is recorded in the scientific literature shot himself in the leg to force its amputation.
Jewel Shuping, 30, wanted to be blind from childhood. In an interview with Bancroft TV, she described pretending to be sightless and wearing thick dark glasses.
By age 20, she could read Braille fluently. At age 21, she blinded herself with drain cleaner — she says with the help of a psychologist. She’s now living happily as a blind woman, according to her own account.
Cases like Shuping’s provoke strong reactions. Are people with BIID psychotic? Are they just desperate for attention? What forms of care can doctors ethically provide?
These were the questions at the forefront of Dr. Michael First’s mind when he began to research BIID. First as a professor of clinical psychiatry at Columbia University and a prominent researcher on the condition — he pioneered the diagnosis “body integrity identity disorder” — and has a current patient who wants to be quadriplegic.
First had never heard of BIID when a BBC reporter called him in the late 1990s to ask about it. Dumbfounded, he began researching the condition.
Woman Who Blinded Herself with Drain Cleaner Brings Attention to Unusual Condition
People with body integrity identity disorder long to be disabled from early childhood. They may not be as crazy as you think, experts say.
Most of what First and the public know about BIID can be traced back to a single doctor, a Scottish surgeon named Dr. Robert Smith, who amputated the healthy limbs of two patients before the hospital where he performed the surgeries shut him down.
Smith’s first patient arrived with a thick stack of psychiatric evaluations that determined he was not psychotic. He was professionally employed and arrived with his wife.
He told the doctor that he feared he would attempt to amputate his leg himself if the doctor didn’t help him. He did not want to because he knew it was dangerous and he did not want to die.
Smith felt that refusing to operate presented greater danger to the patient.
The debate over Smith’s actions is still smoldering in the bioethics community. But after nearly two decades of subsequent research into BIID, something of a patient profile has begun to emerge.
(It should be said, though, that the number of people worldwide who are believed to have some form of BIID is so small that research on the condition is inconclusive.)
Researchers have found that BIID has a neurological basis. Patients have differences in the part of their brains that maps the body and says "This is me."
“We have reported, within the scientific community, that BIID has some neural correlates, so the condition, in a way, must be considered a ‘neurological condition,’ said Peter Brugger, a Swiss neuropsychologist at University Hospital Zurich who studies BIID.
Brain scans of people exhibiting BIID symptoms have shown unusual activity in the part of the brain that maps our awareness of our body. The area of the brain that tells most people “this is my left arm,” for example, may be disconnected from that limb, creating the sense that it does not belong.
Both Brugger and First point out that the scans don’t mean BIID is a neurological disease. They may be showing the neurological effects of the disorder, which often leads people to not use the offending body part, First said.
Brugger also made the case that detectable differences in the scans do not make BIID an illness. Brain scans of avid readers would likely look different from those who know how to read but rarely do, he said.
“This does not mean that [passionate], even compulsive, reading would be a brain disease,” Brugger noted.
Brain architecture clearly isn’t the whole story.
“If it was completely neurological, then we’d expect this condition to be fixed,” First said. “You’d be born with ‘I don’t want to have a left leg,’ and then always the left leg. But people sometimes switch locations over time in life.”
But much of the research into BIID has failed to turn up as much mental instability as one might initially expect from patients who blind themselves with drain cleaner or attempt to freeze-dry their limbs.
Patients are often “troubled,” First acknowledged. But his and other studies conclude that the psychological distress patients exhibit is more likely the result than the cause of their sense that their body isn’t as it should be.
“They understand that this is strange. They can’t explain it, but they feel incredibly compelled to have their body be the way they think it should be,” said First. The desire “totally preoccupies their life. It’s really all-consuming.”
First also found that those with BIID aren’t just seeking the attention that comes with being disabled.
“When I first started this investigation, I had all these ideas that that’s what it was,” he admitted. What he found was far different, and even mundane.
“A lot of people feel like they want to fade into the woodwork and just be a disabled person like everybody else,” First said.
Whatever the cause, there’s no easy answer to the question of how to treat people with BIID.
Most agree that harm reduction is the best approach, beginning with psychotherapy. Talk doesn’t cure patients, but it may help them manage their feelings. Psychiatric medications may also help.
Lifestyle modification may be an option as well. First’s patient, for example, lives publically in a wheelchair, although he is not physically disabled.
First does not endorse nor fully rule out whether voluntary amputation should ever be an option. But Arthur Caplan, Ph.D., director of the Division of Medical Ethics at the New York University Medical Center, expressed no such doubt.
What Can Doctors Do?
“I think doctors and psychologists cannot set up to maim somebody or harm them. You’ve got to try to get them to treatment, I don’t care whether they appear competent or not,” he said.
A doctor who carried out a theoretically unnecessary amputation in order to prevent his or her patient from doing it themselves with potentially fatal results would fall outside of the widely accepted scope of medically ethical practice, Caplan said.
“There are a lot of conditions like anorexia where there’s a core of people who just don’t respond [to treatment],” he said “You don’t give up, you keep trying. That’s all you can do. You don’t indulge it.”
It’s clear that if a counselor helped Shuping blind herself, that doctor acted in violation of both professional ethics and the law. Counselors are not medical doctors and drain cleaner is not a medically approved treatment for any condition.
“I’m personally very dubious that she could find a psychologist to do this,” First said. His hunch is that she’s saying that so that “it looks more like it was sanctioned by a healthcare professional. I think it’s more likely she found some friend.”
One media report backs First’s hunch. In an earlier version of the story, Shuping recounts administering the drain cleaner without help.
To try to grapple with this strange condition, many point to transgender people as a comparison. From an early age, individuals experience a compelling desire to change their bodies.
“Go back to the '50s when people wanted to have sex reassignment, and originally there was this idea you can just do therapy and get them to accept they’re male. But nowadays we would never do that,” First said.
Hormone therapy and surgery are now widely accepted treatments.
But Caplan saw a clear difference between surgery to change sexual characteristics and surgery to induce disability.
Gender surgery “may change who you are, but you’re not lessening what you can do in a functional way. You may actually be adding things if you have sex or function better,” he said. “But blinding, amputation, cutting yourself, scarring yourself — these are all things that hurt you. They limit you. That’s the difference for me.”
Could attitudes on BIID shift towards acceptance in coming years? That will depend on scientific research, and on people like Shuping who share their stories.
“Now that BIID…begins to be known in the general population, including medical practitioners, its real frequency and range becomes apparent,” Brugger said. “There are many more among us who have a strong desire to change bodily appearance and functionality. They just didn't dare to have their ‘coming out.’”
But even Shuping isn’t ready to hold a pride parade for others like her.
“This is not a choice, it’s a need based on a disorder of the brain,” she said. “Don’t go blind the way I did. I know there is a need but perhaps someday there will be treatment for it.”