when your leg just isn’t your leg!?

When Your Leg Just Isn’t When Your Leg Just Isn’t Your Leg!?Your Leg!?

Body Integrity Identity DisorderBody Integrity Identity Disorder

Alison WightonAlison WightonNSW PAR October 2008NSW PAR October 2008

Case Report Mr DOCase Report Mr DO

28 year old Caucasian male with history of 28 year old Caucasian male with history of suicide attempts and requests for suicide attempts and requests for amputation of his right legamputation of his right leg

Transferred to Concord Hospital on Transferred to Concord Hospital on 10/03/2008 from Liverpool Hospital, where 10/03/2008 from Liverpool Hospital, where he had presented with frostbite from he had presented with frostbite from deliberate soaking of his right leg in a deliberate soaking of his right leg in a bucket of ice for five hours.bucket of ice for five hours.

History of Presenting IllnessHistory of Presenting Illness

History of abnormal feelings about the History of abnormal feelings about the right leg since age 4.right leg since age 4.

Possibly related to a TV character with an Possibly related to a TV character with an amputation to cause an attraction to amputation to cause an attraction to amputees?amputees?

Age 7-13 thoughts of leg less urgentAge 7-13 thoughts of leg less urgent Denied any altered sensations, lack of Denied any altered sensations, lack of

coordination, inattention injuries or motor coordination, inattention injuries or motor problems with the leg as a childproblems with the leg as a child


Age 13 - thoughts recurred and the urge to be Age 13 - thoughts recurred and the urge to be rid of the limb became intense rid of the limb became intense

Did not feel his right lower leg was part of himDid not feel his right lower leg was part of him Accidentally tripped down a drain, injuring the Accidentally tripped down a drain, injuring the

right leg in the exact place that he wanted right leg in the exact place that he wanted amputationamputation

Attempted to infect leg by rubbing dirt into the Attempted to infect leg by rubbing dirt into the woundwound

Did not seek medical attentionDid not seek medical attention Day-dreamed of leg falling offDay-dreamed of leg falling off


2006 - deliberately amputated the tip of his 2006 - deliberately amputated the tip of his right middle finger with a knife and right middle finger with a knife and discarded the amputated piecediscarded the amputated piece

This was to suppress his immense This was to suppress his immense devastating feelings with his ‘extra’ legdevastating feelings with his ‘extra’ leg

Managed at Liverpool Hospital with Managed at Liverpool Hospital with antidepressant treatmentantidepressant treatment

Feelings suppressed for short timeFeelings suppressed for short time


Couple initiated research on the internetCouple initiated research on the internet Self diagnosis of Body Integrity Identity Self diagnosis of Body Integrity Identity

Disorder (BIID) late 2007Disorder (BIID) late 2007 Joined online support groups to learn how Joined online support groups to learn how

to deal with the diagnosisto deal with the diagnosis Jan 2008 - Free trip to California arranged Jan 2008 - Free trip to California arranged

by Granada Television for exclusive right by Granada Television for exclusive right to an interview.to an interview.

InvestigationsInvestigations

Jan 2008 - met Dr Ramachandran and Dr Jan 2008 - met Dr Ramachandran and Dr McGeoch at UCSDMcGeoch at UCSD

Tested with MRI brain and magneto-Tested with MRI brain and magneto-encephalographyencephalography

MRI showed an unusually large right MRI showed an unusually large right superior temporal gyrussuperior temporal gyrus

Volumetric analysis of his MRI confirmed Volumetric analysis of his MRI confirmed superior parietal lobule ratio right : left superior parietal lobule ratio right : left of 0.73of 0.73

InvestigationsInvestigations

On magneto-encephalography, touching On magneto-encephalography, touching his right foot produced just primary and his right foot produced just primary and secondary somatosensory activation but secondary somatosensory activation but no activity in the superior right parietal no activity in the superior right parietal lobe.lobe.

Had caloric vestibular stimulationHad caloric vestibular stimulation Partial relief if mirror was placed such that Partial relief if mirror was placed such that

it created illusion that leg was no longer it created illusion that leg was no longer there.there.


26/02/0826/02/08 On returning to Sydney he saw On returning to Sydney he saw

Psychiatrist at Westmead HospitalPsychiatrist at Westmead Hospital He agreed with classic natural history of He agreed with classic natural history of

Body Integrity Identity DisorderBody Integrity Identity Disorder Referral to RPA Hospital for second Referral to RPA Hospital for second

opinionopinion Preliminary discussion with Vascular Preliminary discussion with Vascular

surgeon and Rehabilitation physiciansurgeon and Rehabilitation physician


Unsatisfied with progress trying to seek Unsatisfied with progress trying to seek amputationamputation

Took matter into own hands …….Took matter into own hands …….


10/03/08 Took some pain killers before 10/03/08 Took some pain killers before soaking his leg in a bucket of dry ice for 5 hourssoaking his leg in a bucket of dry ice for 5 hours

Presented to Liverpool hospital with (R) LL Presented to Liverpool hospital with (R) LL frostbite injury and self diagnosis of frostbite injury and self diagnosis of Body Body Integrity Identity DisorderIntegrity Identity Disorder

Given morphine for analgesia and CephazolinGiven morphine for analgesia and Cephazolin

Transferred to Concord Hospital for Transferred to Concord Hospital for assessment….assessment….

Past Medical HistoryPast Medical History

MVA 1985-86 ? Skull fractureMVA 1985-86 ? Skull fracture History of migraine headache on and offHistory of migraine headache on and off

MedicationsMedications

Citalopram 20mg daily-for last three Citalopram 20mg daily-for last three monthsmonths

Drug and AlcoholDrug and Alcohol

Drinks average of 10g of alcohol per dayDrinks average of 10g of alcohol per day Up to 100g at a sittingUp to 100g at a sitting 2001-2006 used Cannabis2001-2006 used Cannabis No other illicit drugs and never smoked No other illicit drugs and never smoked

tobaccotobacco

Psychosocial HistoryPsychosocial History

Unemployed, receives parenting pensionUnemployed, receives parenting pension Previously worked in series of low skilled Previously worked in series of low skilled

occupationsoccupations Lives with his Lives with his de facto wife de facto wife and their four and their four

children (12,10,6,4) in a Dept Housing children (12,10,6,4) in a Dept Housing propertyproperty

Partner receives Austudy allowancePartner receives Austudy allowance

ChildhoodChildhood

Parents divorced when he was sevenParents divorced when he was seven Father remarried a woman he did not likeFather remarried a woman he did not like Unstable and complicated upbringingUnstable and complicated upbringing Diagnosed with Attention Deficit Disorder Diagnosed with Attention Deficit Disorder

at age 7at age 7 Short term treatment with AmphetamineShort term treatment with Amphetamine Left school in year 10Left school in year 10

StressorsStressors

1999 - mother murdered by her boyfriend 1999 - mother murdered by her boyfriend by beating her unconscious and then by beating her unconscious and then burning house down with her in it. (19yrs)burning house down with her in it. (19yrs)

2000 - brother got him to unknowingly hold 2000 - brother got him to unknowingly hold stolen goods leading to imprisonmentstolen goods leading to imprisonment

Suicide AttemptsSuicide Attempts

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

Treatment CourseTreatment Course

10/03/0810/03/08 Pain managementPain management Peripheral foot perfusion checks 4/24Peripheral foot perfusion checks 4/24 Probably unlikely to require surgeryProbably unlikely to require surgery Psychiatry consultPsychiatry consult

ImagingImaging

CT Brain-NADCT Brain-NAD CXR under-inflated lungs with bibasal CXR under-inflated lungs with bibasal

collapsecollapse MRI Brain-normalMRI Brain-normal SPECT Brain-normal SPECT Brain-normal

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

LOOK AWAY NOWLOOK AWAY NOW

17/03/200817/03/2008

17/03/200817/03/2008

Blood cultures-gram negative rods in 4/4 Blood cultures-gram negative rods in 4/4 bottlesbottles

Wound-heel pad gangrenousWound-heel pad gangrenous Commenced on Gentamycin and Commenced on Gentamycin and

CeftazidineCeftazidine

OpinionsOpinions

Rehabilitation team (Dr Ross Hawthorne)Rehabilitation team (Dr Ross Hawthorne) Extensive necrosis of heel pad, no benefit Extensive necrosis of heel pad, no benefit

from trying to save the foot or Syme’s from trying to save the foot or Syme’s amputation.amputation.

Supported trans-tibial amputation at the Supported trans-tibial amputation at the level desired by the patient.level desired by the patient.

Burns team supported the medical Burns team supported the medical indication for below knee amputation.indication for below knee amputation.

OpinionsOpinions

Vascular teamVascular team Agree with need for amputation, wait until Agree with need for amputation, wait until

necrotic area fully demarcatesnecrotic area fully demarcates Further positive cultures Further positive cultures → → gram positive gram positive

cocci- staph and strepcocci- staph and strep Commenced on VancomycinCommenced on Vancomycin

19/03/200819/03/2008

Heel necrosis worse and malodorousHeel necrosis worse and malodorous Right foot swollen and cellulitic up to mid Right foot swollen and cellulitic up to mid

shinshin Cultures growing Staph aureus, Cultures growing Staph aureus,

Enterococcus and PseudomonasEnterococcus and Pseudomonas Commenced on TazocinCommenced on Tazocin

20/03/200820/03/2008

Calf muscle perfusion scan - non viable Calf muscle perfusion scan - non viable right gastrocnemius muscleright gastrocnemius muscle

21/03/200821/03/2008

Right trans tibial amputationRight trans tibial amputation No post operative complicationsNo post operative complications

The ResultThe Result

Rehabilitation PhaseRehabilitation Phase

Developed Phantom limb painDeveloped Phantom limb pain Treated with Doxepin by Pain team and Treated with Doxepin by Pain team and

patient educated about stump massagepatient educated about stump massage Rigid removable dressing commenced for Rigid removable dressing commenced for

stump managementstump management Progressed well and became independent Progressed well and became independent

with his LL and UL exercises and mobility with his LL and UL exercises and mobility with crutches.with crutches.

Function at DischargeFunction at Discharge

Independent with self care Independent with self care Independent stump careIndependent stump care Independent mobility with crutchesIndependent mobility with crutches Home visit was conducted with OTHome visit was conducted with OT Little equipment required for safe Little equipment required for safe

discharge to Aunt’s house on 17/04/2008discharge to Aunt’s house on 17/04/2008 Prescription for interim prosthesis made Prescription for interim prosthesis made

prior to discharge.prior to discharge.

Attitude Since AmputationAttitude Since Amputation

Feels a weight lifted of his chestFeels a weight lifted of his chest Wants to return to normal life and activitiesWants to return to normal life and activities Feels no longer belongs to the BIID groupFeels no longer belongs to the BIID group States expectations have been metStates expectations have been met Has found acceptance from family Has found acceptance from family

members by explaining BIID as members by explaining BIID as neurological conditionneurological condition

Physiotherapy ProgressionPhysiotherapy Progression

Was quick to progress to independent Was quick to progress to independent mobility with prosthesis unaided.mobility with prosthesis unaided.

Was starting to learn to run, however Was starting to learn to run, however attendance at outpatient physio has been attendance at outpatient physio has been unreliable.unreliable.

Now is happy with current abilities and Now is happy with current abilities and finds he can play with kids at the park etc.finds he can play with kids at the park etc.

Body Integrity Identity Disorder Body Integrity Identity Disorder (BIID)(BIID)

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


The desire for amputation of a healthy limb was The desire for amputation of a healthy limb was first reported in 1785first reported in 1785 (cited in Johnston & Elliott, 2002)

Money et al (1977) used the term Money et al (1977) used the term apotemnophilia (amputation love) to describe apotemnophilia (amputation love) to describe intense and intrusive thoughts to amputate a intense and intrusive thoughts to amputate a lower extremity. These thoughts were related to lower extremity. These thoughts were related to sexual fantasies and sexual arousal. sexual fantasies and sexual arousal.

Sex Res1977;13:115-25)

Description of this disorder was limited to a few Description of this disorder was limited to a few case reports from 1977-2003case reports from 1977-2003


Long standing desire to be an amputeeLong standing desire to be an amputee Rare, mainly menRare, mainly men Often arises around 4 – 5 yrs ageOften arises around 4 – 5 yrs age Often accompanied by sexual arousal but Often accompanied by sexual arousal but

not necessarily primary motivenot necessarily primary motive

Can arise in womenCan arise in women Extremes….Extremes….

BIIDBIID

Patients with this condition have an often Patients with this condition have an often overwhelming desire for an amputation of overwhelming desire for an amputation of a specific limb at a specific level. a specific limb at a specific level.

Such patients are not psychotic or Such patients are not psychotic or delusionaldelusional

Such patients show a left - sided Such patients show a left - sided preponderance for their desired preponderance for their desired amputationamputation

Apotemnophilia and Munchausen’s Apotemnophilia and Munchausen’s Syndrome.Syndrome.

Munchausen's patient is obsessed with self Munchausen's patient is obsessed with self inducing symptoms repetitively for the sake of inducing symptoms repetitively for the sake of being a patient where as an apotemnophile is being a patient where as an apotemnophile is supposedly satisfied with just one amputationsupposedly satisfied with just one amputation

Apotemnophiles need only one medical Apotemnophiles need only one medical intervention that leaves them with obvious intervention that leaves them with obvious stigma of disability which will permanently satisfy stigma of disability which will permanently satisfy their need for love and attention.their need for love and attention.

Factitious Disability DisorderFactitious Disability Disorder

Bruno 1997- divided this disorder into 3 Bruno 1997- divided this disorder into 3 subsetssubsets Devotees Devotees Pretenders Pretenders Wannabes Wannabes

DevoteesDevotees

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

PretendersPretenders

Pretenders are non-disabled people who Pretenders are non-disabled people who live as if they have a disability. live as if they have a disability.

Pretender paraplegics can confine Pretender paraplegics can confine themselves to their chairs full time and themselves to their chairs full time and never walk. never walk.

The pretender amputee has more difficulty The pretender amputee has more difficulty trying to be an amputee and feels trying to be an amputee and feels frustrated and dissatisfied.frustrated and dissatisfied.

WannabesWannabes

Wannabes are usually non-disabled Wannabes are usually non-disabled individuals that want to become someone individuals that want to become someone with a physical disability. with a physical disability.

See themselves in bodies that are not fully See themselves in bodies that are not fully functioning. functioning.

They have difficulty finding identity.They have difficulty finding identity.

BIIDBIID

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

The ResultsThe Results

90% had education beyond high school90% had education beyond high school 65% were currently employed. 65% were currently employed. 27% had surgical or self inflicted 27% had surgical or self inflicted

amputationamputation 17% had major limb amputation and two 17% had major limb amputation and two

thirds had used methods that put thirds had used methods that put themselves at high riskthemselves at high risk


He found that 15% of wannabes identified He found that 15% of wannabes identified sexual arousal as a reason for amputation, 63% sexual arousal as a reason for amputation, 63% wanted to be restored to their "true identity" and wanted to be restored to their "true identity" and 37% said the limb "felt different".37% said the limb "felt different".

Thirteen percent said the limb didn't feel like Thirteen percent said the limb didn't feel like their own and six people had tried to perform their own and six people had tried to perform their own amputation, including using a their own amputation, including using a chainsaw.chainsaw.

87% reported being sexually attracted to other 87% reported being sexually attracted to other amputees.amputees.

Desired Location for AmputationDesired Location for Amputation

95% wanted an amputation of major limb95% wanted an amputation of major limb 92% wanted above knee amputation92% wanted above knee amputation 55% wanted left sided amputation55% wanted left sided amputation In 77% the site of desired amputation was In 77% the site of desired amputation was

fixed since it started in childhood.fixed since it started in childhood.


Most felt the somatosensory perception of the Most felt the somatosensory perception of the limb did not differ from that of their other limbs.limb did not differ from that of their other limbs.

65% had onset prior to age 8; and 98% had 65% had onset prior to age 8; and 98% had onset by age 16 years.onset by age 16 years.

Majority reported exposure to an amputee in Majority reported exposure to an amputee in childhood.childhood.

44% of First’s subjects reported that their desire 44% of First’s subjects reported that their desire interfered with social functioning, occupational interfered with social functioning, occupational functioning, or leisure activities.functioning, or leisure activities.

Co-morbid PsychopathologyCo-morbid Psychopathology

Three quarters reported having had Three quarters reported having had psychiatric condition sometime in their psychiatric condition sometime in their lives.lives.

Most commonly depression, anxiety and Most commonly depression, anxiety and somatoform disorder.somatoform disorder.

Treatment EfficacyTreatment Efficacy

65% had psychotherapy, for none of the 65% had psychotherapy, for none of the subjects it reduced the desire for subjects it reduced the desire for amputationamputation

40% were treated with psychotropic 40% were treated with psychotropic medications - no appreciable effect from medications - no appreciable effect from the medication on the desire for the medication on the desire for amputationamputation

12% patients had amputation at their 12% patients had amputation at their desired leveldesired level

Causes of BIIDCauses of BIID

There is no one single causal factor for the There is no one single causal factor for the development of BIID.development of BIID.

One theory states that a child, upon seeing an One theory states that a child, upon seeing an amputee, may amputee, may imprintimprint his or her psyche, and his or her psyche, and the child adopts this body image as an "ideal".the child adopts this body image as an "ideal".

Another popular theory suggests that a child Another popular theory suggests that a child who feels unloved may believe that becoming who feels unloved may believe that becoming an amputee will attract the sympathy and love an amputee will attract the sympathy and love he or she needs.he or she needs.

Biological TheoryBiological Theory

BIID is a neuro-psychological condition in BIID is a neuro-psychological condition in which there is an anomaly in the cerebral which there is an anomaly in the cerebral cortex relating to the limbs. It could be cortex relating to the limbs. It could be conceptualized as a conceptualized as a congenitalcongenital form of form of somatoparaphreniasomatoparaphrenia, a condition that often , a condition that often follows a stroke affecting the parietal lobe follows a stroke affecting the parietal lobe

Possibility of genetic basisPossibility of genetic basis

Research shows most of the BIID population Research shows most of the BIID population had experienced a significant childhood event. had experienced a significant childhood event.

Can show up as early as 4 or 5 years old. Can show up as early as 4 or 5 years old. Typically no change in the desire for amputation. Typically no change in the desire for amputation.

Participants who received amputation reported Participants who received amputation reported after amputation, they feel better than ever and after amputation, they feel better than ever and lose the desire for further amputation.lose the desire for further amputation.

Extreme MeasuresExtreme Measures

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

TreatmentTreatment

Medication such as antidepressants help Medication such as antidepressants help little but can treat concurrent conditions little but can treat concurrent conditions such as depressionsuch as depression

Most sufferers gain little help from Most sufferers gain little help from psychiatric and psychological therapy, it psychiatric and psychological therapy, it helps to control the desire rather than to helps to control the desire rather than to abolish it.abolish it.

Mirror Feedback TreatmentMirror Feedback Treatment

During the therapy the patients are instructed to During the therapy the patients are instructed to use the mirror in a way that the mirror image use the mirror in a way that the mirror image produces an illusion of one absent limb. produces an illusion of one absent limb.

This technique is be used to convey the visual This technique is be used to convey the visual illusion to the patient that his arm has been illusion to the patient that his arm has been amputated or is missing. amputated or is missing.

This might provide a sort of ‘‘dress-rehearsal’’ for This might provide a sort of ‘‘dress-rehearsal’’ for the amputation and may de-sensitise and the amputation and may de-sensitise and eliminate the desire.eliminate the desire.

Vestibular Caloric StimulationVestibular Caloric Stimulation Cold caloric irrigation, temporarily ameliorates Cold caloric irrigation, temporarily ameliorates

the symptoms of somatoparaphrenia. the symptoms of somatoparaphrenia. As per researchers cold-water caloric irrigation As per researchers cold-water caloric irrigation

to, at least temporarily, alleviate these patients’ to, at least temporarily, alleviate these patients’ intense desire for an amputation.intense desire for an amputation.

Such a reduction of symptom intensity in BIID Such a reduction of symptom intensity in BIID sufferers post irrigation would be suggestive of a sufferers post irrigation would be suggestive of a similar aetiology.similar aetiology.

Perhaps with repeated irrigations BIID patients Perhaps with repeated irrigations BIID patients might come to accept the rejected limb into their might come to accept the rejected limb into their body image;body image;

Ethics of AmputationEthics of Amputation

Tim Bayne et al came up withTim Bayne et al came up with three three arguments for allowing self-demand arguments for allowing self-demand amputation of healthy limbs:amputation of healthy limbs: Harm MinimizationHarm Minimization AutonomyAutonomy TherapyTherapy

Harm MinimisationHarm Minimisation

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

AutonomyAutonomy

An individual’s conception of his or her An individual’s conception of his or her good should be respected in medical good should be respected in medical decision-making contexts. decision-making contexts.

Where a wannabe has a long-standing Where a wannabe has a long-standing and informed request for amputation, it and informed request for amputation, it therefore seems permissible for a surgeon therefore seems permissible for a surgeon to act on this request.to act on this request.

TherapyTherapy

The argument rests on four premises: The argument rests on four premises: (i) wannabes endure serious suffering as a result (i) wannabes endure serious suffering as a result

of their condition; of their condition; (ii) amputation will — or is likely to — secure(ii) amputation will — or is likely to — secure relief from this suffering; relief from this suffering; (iii) this relief cannot be secured by less drastic (iii) this relief cannot be secured by less drastic

means;means; (iv) securing relief from this suffering is worth the (iv) securing relief from this suffering is worth the

cost of amputation.cost of amputation.

What do you think???What do you think???

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

presentation.presentation.

when your leg just isn’t your leg!?

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