When Your Leg Just Isnt Your Leg Body

When Your Leg Just Isn’t Your Leg!? Body Integrity Identity Disorder Alison Wighton NSW PAR October 2008

Case Report Mr DO Ø 28 year old Caucasian male with history of suicide attempts and requests for amputation of his right leg Ø Transferred to Concord Hospital on 10/03/2008 from Liverpool Hospital, where he had presented with frostbite from deliberate soaking of his right leg in a bucket of ice for five hours.

History of Presenting Illness Ø History of abnormal feelings about the right leg since age 4. Ø Possibly related to a TV character with an amputation to cause an attraction to amputees? Ø Age 7 -13 thoughts of leg less urgent Ø Denied any altered sensations, lack of coordination, inattention injuries or motor problems with the leg as a child

History of Presenting Illness Age 13 - thoughts recurred and the urge to be rid of the limb became intense Ø Did not feel his right lower leg was part of him Ø Accidentally tripped down a drain, injuring the right leg in the exact place that he wanted amputation Ø Attempted to infect leg by rubbing dirt into the wound Ø Did not seek medical attention Ø Day-dreamed of leg falling off Ø

History of Presenting Illness Ø 2006 - deliberately amputated the tip of his right middle finger with a knife and discarded the amputated piece Ø This was to suppress his immense devastating feelings with his ‘extra’ leg Ø Managed at Liverpool Hospital with antidepressant treatment Ø Feelings suppressed for short time

History of Presenting Illness Ø Couple initiated research on the internet Ø Self diagnosis of Body Integrity Identity Disorder (BIID) late 2007 Ø Joined online support groups to learn how to deal with the diagnosis Ø Jan 2008 - Free trip to California arranged by Granada Television for exclusive right to an interview.

Investigations Ø Jan 2008 - met Dr Ramachandran and Dr Mc. Geoch at UCSD Ø Tested with MRI brain and magnetoencephalography Ø MRI showed an unusually large right superior temporal gyrus Ø Volumetric analysis of his MRI confirmed superior parietal lobule ratio right : left of 0. 73

Investigations Ø On magneto-encephalography, touching his right foot produced just primary and secondary somatosensory activation but no activity in the superior right parietal lobe. Ø Had caloric vestibular stimulation Ø Partial relief if mirror was placed such that it created illusion that leg was no longer there.

History of Presenting Illness Ø 26/02/08 Ø On returning to Sydney he saw Psychiatrist at Westmead Hospital Ø He agreed with classic natural history of Body Integrity Identity Disorder Ø Referral to RPA Hospital for second opinion Ø Preliminary discussion with Vascular surgeon and Rehabilitation physician

History of Presenting Illness Ø Unsatisfied with progress trying to seek amputation Ø Took matter into own hands …….

History of Presenting Illness 10/03/08 Took some pain killers before soaking his leg in a bucket of dry ice for 5 hours Ø Presented to Liverpool hospital with (R) LL frostbite injury and self diagnosis of Body Integrity Identity Disorder Ø Ø Given morphine for analgesia and Cephazolin Ø Transferred to Concord Hospital for assessment….

Past Medical History Ø MVA 1985 -86 ? Skull fracture Ø History of migraine headache on and off

Medications Ø Citalopram 20 mg daily-for last three months

Drug and Alcohol Ø Drinks average of 10 g of alcohol per day Ø Up to 100 g at a sitting Ø 2001 -2006 used Cannabis Ø No other illicit drugs and never smoked tobacco

Psychosocial History Ø Unemployed, receives parenting pension Ø Previously worked in series of low skilled occupations Ø Lives with his de facto wife and their four children (12, 10, 6, 4) in a Dept Housing property Ø Partner receives Austudy allowance

Childhood Ø Parents divorced when he was seven Ø Father remarried a woman he did not like Ø Unstable and complicated upbringing Ø Diagnosed with Attention Deficit Disorder at age 7 Ø Short term treatment with Amphetamine Ø Left school in year 10

Stressors Ø 1999 - mother murdered by her boyfriend by beating her unconscious and then burning house down with her in it. (19 yrs) Ø 2000 - brother got him to unknowingly hold stolen goods leading to imprisonment

Suicide Attempts Ø 1999 -attempted cutting his wrist in response to mother’s death.

Treatment Course 10/03/08 Ø Pain management Ø Peripheral foot perfusion checks 4/24 Ø Probably unlikely to require surgery Ø Psychiatry consult

Imaging Ø CT Brain-NAD Ø CXR under-inflated lungs with bibasal collapse Ø MRI Brain-normal Ø SPECT Brain-normal

If you’re not good with blood and all things a bit yucky…… LOOK AWAY NOW

17/03/2008

17/03/2008 Ø Blood cultures-gram negative rods in 4/4 bottles Ø Wound-heel pad gangrenous Ø Commenced on Gentamycin and Ceftazidine

Opinions Rehabilitation team (Dr Ross Hawthorne) Ø Extensive necrosis of heel pad, no benefit from trying to save the foot or Syme’s amputation. Ø Supported trans-tibial amputation at the level desired by the patient. Ø Burns team supported the medical indication for below knee amputation.

Opinions Vascular team Ø Agree with need for amputation, wait until necrotic area fully demarcates Ø Further positive cultures → gram positive cocci- staph and strep Ø Commenced on Vancomycin

19/03/2008 Ø Heel necrosis worse and malodorous Ø Right foot swollen and cellulitic up to mid shin Ø Cultures growing Staph aureus, Enterococcus and Pseudomonas Ø Commenced on Tazocin

20/03/2008 Ø Calf muscle perfusion scan - non viable right gastrocnemius muscle

21/03/2008 Ø Right trans tibial amputation Ø No post operative complications

The Result

Rehabilitation Phase Ø Developed Phantom limb pain Ø Treated with Doxepin by Pain team and patient educated about stump massage Ø Rigid removable dressing commenced for stump management Ø Progressed well and became independent with his LL and UL exercises and mobility with crutches.

Function at Discharge Ø Independent with self care Ø Independent stump care Ø Independent mobility with crutches Ø Home visit was conducted with OT Ø Little equipment required for safe discharge to Aunt’s house on 17/04/2008 Ø Prescription for interim prosthesis made prior to discharge.

Attitude Since Amputation Ø Feels a weight lifted of his chest Ø Wants to return to normal life and activities Ø Feels no longer belongs to the BIID group Ø States expectations have been met Ø Has found acceptance from family members by explaining BIID as neurological condition

Physiotherapy Progression Ø Was quick to progress to independent mobility with prosthesis unaided. Ø Was starting to learn to run, however attendance at outpatient physio has been unreliable. Ø Now is happy with current abilities and finds he can play with kids at the park etc.

Body Integrity Identity Disorder (BIID) Ø Apotemnophilia, or body integrity identity disorder (BIID), is characterized by a feeling of mismatch between the internal feeling of how one’s body should be and the physical reality of how it actually is.

Body Integrity Identity Disorder (BIID) Ø The desire for amputation of a healthy limb was first reported in 1785 (cited in Johnston & Elliott, 2002) Ø Money et al (1977) used the term apotemnophilia (amputation love) to describe intense and intrusive thoughts to amputate a lower extremity. These thoughts were related to sexual fantasies and sexual arousal. Sex Res 1977; 13: 115 -25) Ø Description of this disorder was limited to a few case reports from 1977 -2003

Body Integrity Identity Disorder (BIID) Ø Long standing desire to be an amputee Ø Rare, mainly men Ø Often arises around 4 – 5 yrs age Ø Often accompanied by sexual arousal but not necessarily primary motive Ø Can arise in women Ø Extremes….

BIID Ø Patients with this condition have an often overwhelming desire for an amputation of a specific limb at a specific level. Ø Such patients are not psychotic or delusional Ø Such patients show a left - sided preponderance for their desired amputation

Apotemnophilia and Munchausen’s Syndrome. Ø Munchausen's patient is obsessed with self inducing symptoms repetitively for the sake of being a patient where as an apotemnophile is supposedly satisfied with just one amputation Ø Apotemnophiles need only one medical intervention that leaves them with obvious stigma of disability which will permanently satisfy their need for love and attention.

Factitious Disability Disorder Ø Bruno 1997 - divided this disorder into 3 subsets l l l Devotees Pretenders Wannabes

Devotees Ø Devotees are non disabled people who are sexually attracted to people with disabilities, typically those with mobility impairments and amputees

Pretenders Ø Pretenders are non-disabled people who live as if they have a disability. Ø Pretender paraplegics can confine themselves to their chairs full time and never walk. Ø The pretender amputee has more difficulty trying to be an amputee and feels frustrated and dissatisfied.

Wannabes Ø Wannabes are usually non-disabled individuals that want to become someone with a physical disability. Ø See themselves in bodies that are not fully functioning. Ø They have difficulty finding identity.

BIID Ø The first person to use the term BIID was US psychiatrist Associate Professor Michael First from Columbia University, who interviewed 52 ‘wannabes’ as part of a recent study.

The Results Ø 90% had education beyond high school Ø 65% were currently employed. Ø 27% had surgical or self inflicted amputation Ø 17% had major limb amputation and two thirds had used methods that put themselves at high risk

The Results He found that 15% of wannabes identified sexual arousal as a reason for amputation, 63% wanted to be restored to their "true identity" and 37% said the limb "felt different". Ø Thirteen percent said the limb didn't feel like their own and six people had tried to perform their own amputation, including using a chainsaw. Ø 87% reported being sexually attracted to other amputees. Ø

Desired Location for Amputation Ø 95% wanted an amputation of major limb Ø 92% wanted above knee amputation Ø 55% wanted left sided amputation Ø In 77% the site of desired amputation was fixed since it started in childhood.

The Results Most felt the somatosensory perception of the limb did not differ from that of their other limbs. Ø 65% had onset prior to age 8; and 98% had onset by age 16 years. Ø Majority reported exposure to an amputee in childhood. Ø 44% of First’s subjects reported that their desire interfered with social functioning, occupational functioning, or leisure activities. Ø

Co-morbid Psychopathology Ø Three quarters reported having had psychiatric condition sometime in their lives. Ø Most commonly depression, anxiety and somatoform disorder.

Treatment Efficacy Ø 65% had psychotherapy, for none of the subjects it reduced the desire for amputation Ø 40% were treated with psychotropic medications - no appreciable effect from the medication on the desire for amputation Ø 12% patients had amputation at their desired level

Causes of BIID Ø There is no one single causal factor for the development of BIID. Ø One theory states that a child, upon seeing an amputee, may imprint his or her psyche, and the child adopts this body image as an "ideal". Ø Another popular theory suggests that a child who feels unloved may believe that becoming an amputee will attract the sympathy and love he or she needs.

Biological Theory Ø BIID is a neuro-psychological condition in which there is an anomaly in the cerebral cortex relating to the limbs. It could be conceptualized as a congenital form of somatoparaphrenia, a condition that often follows a stroke affecting the parietal lobe Ø Possibility of genetic basis

Research shows most of the BIID population had experienced a significant childhood event. Ø Can show up as early as 4 or 5 years old. Ø Typically no change in the desire for amputation. Ø Ø Participants who received amputation reported after amputation, they feel better than ever and lose the desire for further amputation.

Extreme Measures Ø Because most surgeons refuse to amputate a healthy limb, some people with BIID go to extreme measures to get rid the limb. l l Paying for surgery “under the table” Homemade devices Using ice, train tracks, electric saws, etc. At home “accidents”

Treatment Ø Medication such as antidepressants help little but can treat concurrent conditions such as depression Ø Most sufferers gain little help from psychiatric and psychological therapy, it helps to control the desire rather than to abolish it.

Mirror Feedback Treatment During therapy the patients are instructed to use the mirror in a way that the mirror image produces an illusion of one absent limb. Ø This technique is be used to convey the visual illusion to the patient that his arm has been amputated or is missing. Ø This might provide a sort of ‘‘dress-rehearsal’’ for the amputation and may de-sensitise and eliminate the desire. Ø

Vestibular Caloric Stimulation Cold caloric irrigation, temporarily ameliorates the symptoms of somatoparaphrenia. Ø As per researchers cold-water caloric irrigation to, at least temporarily, alleviate these patients’ intense desire for an amputation. Ø Such a reduction of symptom intensity in BIID sufferers post irrigation would be suggestive of a similar aetiology. Ø Perhaps with repeated irrigations BIID patients might come to accept the rejected limb into their body image; Ø

Ethics of Amputation Ø Tim Bayne et al came up with three arguments for allowing self-demand amputation of healthy limbs: l Harm Minimization l Autonomy l Therapy

Harm Minimisation Ø Given that many patients will go ahead with amputations in any case, and risk extensive injury or death in doing so, it might be argued that surgeons should accede to the requests, at least of those patients who they judge are likely to take matters into their own hands.

Autonomy Ø An individual’s conception of his or her good should be respected in medical decision-making contexts. Ø Where a wannabe has a long-standing and informed request for amputation, it therefore seems permissible for a surgeon to act on this request.

Therapy The argument rests on four premises: Ø (i) wannabes endure serious suffering as a result of their condition; Ø (ii) amputation will — or is likely to — secure Ø relief from this suffering; Ø (iii) this relief cannot be secured by less drastic means; Ø (iv) securing relief from this suffering is worth the cost of amputation.

What do you think? ? ?

With thanks to Dr Veena Rayker for her assistance in preparing this presentation.