Behavior Management
Living with Lesch-Nyhan Syndrome affects more than movement and physical health — it can also influence behavior in unique and sometimes challenging ways. From self-injurious behaviors to emotional and social differences, each person’s experience is different.
This page is designed to help families, caregivers, and community members understand what to expect, recognize patterns, and respond with compassion and support. We share practical strategies, tools, and insights to help manage behaviors safely and respectfully, always keeping the focus on the individual’s well-being and quality of life.
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Teeth Removal
“Ignore the Behaviors, not the individual”
Overview
Self-injury can be one of the most recognizable and challenging parts of Classic Lesch-Nyhan Syndrome. While similar behaviors can appear in other conditions, people with Lesch-Nyhan often experience them in unique and complex ways. These behaviors are not intentional or within the person’s control—they are part of how the condition affects the brain and nervous system.
Because of this, punishment or negative behavioral approaches can make things worse, while calm, compassionate, and supportive strategies are much more effective. Living with Lesch-Nyhan affects many areas of life, so individuals benefit most from ongoing, coordinated care that addresses physical health, emotional well-being, and behavioral support throughout their lives.
Individuals with Classic LND both require and desire the use of protective devices. The goals for the use of devices are to:
1) provide comfort to the patient and
2) prevent self-injury and irreversible harm.
3) Safe participation in physical activities (i.e. Special Olympics).
They do not want to hurt themselves, or others, and know that they will if allowed to do so.
Individuals with Classic LND become anxious and fearful when left unprotected.
Damage to the mouth and face from teeth is a central form of self-injury; it does not respond adequately to the use of oral devices, and the standard of care is to remove the teeth as soon as this pattern of injury is identified. The devastation that can occur to the face using the teeth to self-injure warrant an alert to physicians.
The Joint Commission accepts that in LND protective devices are enabling because they prevent self-injury and reduce stress and the fear of self-injury. The Joint Commission and the Center for Medicare and Medicaid Services (CMS) have exempted these safeguarding devices from their standards regarding the writing of PRN orders for restraints. Because these devices look like restraints, the exception to the standard/rules was written into the Joint Commission manuals, but only for individuals with Lesch Nyhan syndrome. Hence, this standard of care has been adopted by every state CMS office. School systems concerned with the use of these devices should be directed to the CMS and Joint Commission regulations and standards. With time individuals with LND may learn to direct the application of their own protective devices.
To find out about Joint Commision Regulations adopted by your state on protective devices Google CMS (your State) “exceptions to the restraint rule” Lesch Nyhan.
Recognition of self-injurious behaviors is the first necessary step to inform and manage provision of care. To facilitate the identification of the range of behaviors we describe nine categories of self-injury. Although the movement disorder of dystonia is discussed, it is important to note that generalized dystonia, in combination with normal strength, will impact both the frequency and severity of self-injury.
Categories of Self-Injurious Behaviors and Management Stratigies
Gary Eddey, MD ScM(HYG), Kelly Henry, MSW
Self-injury is a hallmark feature of Lesch Nyhan disease and constitutes the most dramatic part of the behavioral phenotype. Although self-injury occurs in other intellectual disabilities, individuals with LND exhibit an unrelenting and diverse form of self-injury not otherwise observed. What also sets it apart is a dramatic increase in self injurious behaviors when punishment is used as a component of behavioral modification interventions. In addition to behaviors that impact every facet of their lives, Lesch-Nyhan disease is a complex multisystem disability that requires ongoing care throughout the lifespan.
Physical Self-Injury
The individual uses their body to self-injure.
Examples: Eye poking, scratching (fear of scratching is worrisome to patients), closing off airway to induce hypoxia.
Note: Self-injury using teeth warrants its own category, described below.
Self-Injury Using Objects
The individual uses an object in the environment to self-injure. This occurs early – one to two years – and continues through the lifespan.
Examples: Hands and fingers inserted in spokes of a moving wheelchair, rubbing head and ears against unprotected headrests, head banging, extending head, arms, hands, legs, and feet as the individual is wheeled through a doorway.
Protection: Essential devices are needed to prevent this category of self-injury.
If a hand comes out of a protective device and the child immediately starts to put their finger into the eye socket, it must be stopped. But it must be done with only minimal necessary attention and without a verbal response.
Self-Injury Using Teeth
Often the first obvious sign of self-injury is the use of teeth to bite fingertips, lips, and inside cheeks to disfigure the face.
Intervention: Immediate removal of teeth is the standard of care in at least 50% of individuals with LND.
Indirect or Outward Aggression
These behaviors are directed against caregivers, but strangers are often the target.
Examples: Aggressive swearing at caregivers to elicit a negative response; cultural, sexist, and racist comments depending on the setting.
Intervention: Early identification and use of Selective Ignoring with Redirection (SIwR).
Self-Sabotage
Active and passive behaviors to prevent the accomplishment of goals.
Examples: Refusing to attend an event after purchasing tickets to a favorite event, willfully performing poorly on tests, head butting the physician during a physical exam, making false statements to strangers.
Manipulative Behaviors
Behaviors that manipulate caregivers to do things the individual with LND knows are not in their best interest.
Examples: Convincing caregivers that protective equipment is not needed, stopping communication between caregivers and other important figures.
Intervention: Addressing triangulation and ensuring consistent communication.
Diversion and Disruption
Verbal attempts to focus on a complaint intended to pull caregivers into finding a solution for an irrelevant issue.
Examples: Making false complaints to avoid tasks, claiming to have a bomb or a broken arm.
Intervention: SIwR to extinguish behavior.
Vomiting and Spitting
A form of self-injury using the body to harm oneself.
Intervention: SIwR to manage these common forms of self-injury.
Lying and Exaggeration
“Lying” in individuals with LND has been described since the 1960s. This behavior is often misinterpreted and is better thought of as a form of over-exaggeration rather than lying. Mostly benign, except for false accusations of abuse. Accusations must be investigated, however a pattern of false accusations is readily identified. Suicidal thoughts almost universally fall into this form of LN behaviors.
Examples: False accusations, suicidal thoughts.
Intervention: Recognize the pattern of false accusations and manage accordingly.
Over or Under Exaggeration - to avoid
To avoid an activity or medical appointment patient exaggerates complaints so they don’t have to go. In the case of over exaggeration, this leads to more frequent medical interventions and tests. The “Red Herring” in the medical history of unsuspecting physicians often leads to an iatrogenic form of self-injury. There can also be under exaggeration of symptoms which can lead to more significant medical consequences even death.
After recognition of a self-injurious behavior the caregiver must immediately implement Selective Ignoring and Redirection and consider, if necessary, the application or readjustment of protective devices. Selective Ignoring with Redirection is always appropriate, whereas protective devices as an intervention must be implemented judiciously.
Identification of a LN behavior must never be followed by a verbal or physical recognition by the caregiver – or bystander – of the Lesch Nyhan behavior in question. Intervention proceeds after a determination of whether if harm appears imminent. If no harm, then redirection should be used. When the behavior is completely ignored and there is no negative consequence raised to the behavior, then the behavior will lose its self-injurious value and decrease
“Ignore the LN Behavior not the individual”
Selective ignoring with redirection refers to the intentional act of choosing to ignore certain stimuli, information, or distractions while redirecting attention or focus toward a specific target or task. It involves filtering out irrelevant elements to concentrate on what is considered more important or relevant in a given context. • Essential to the method: There must never be evidence – in the behavior or actions of the caregiver – of punishment, however subtle.
Steps to Implement Selective Ignoring with Redirection
After recognition of a self-injurious behavior the caregiver must immediately implement SIwR and consider, if necessary, the application or readjustment of protective devices. Selective Ignoring with Redirection is always appropriate, whereas protective devices as an intervention must be implemented judiciously.
There are three components to Selective Ignoring with Redirection. It must be immediately implemented after a LN behavior is observed. It must be used by everyone across all environments until the behavior is extinguished. The use of the redirection component allows for altering the behavior without calling attention to the behavior.
The challenges of this intervention are several. All family members and members of health care and educational teams must utilize the technique. There must never be a negative consequence to any LN behavior, and it has long been recognized that not showing a negative response to the behaviors is not easy to accomplish.
“If a hand comes out of a protective device and the child immediately starts to put his/her finger into the eye socket it must be stopped. But it must be done with only minimal necessary attention and without a verbal response.”
The following provides examples how LN behaviors can be ignored without calling attention to the maladaptive behavior. The use of periodic seminars focusing on examples of this technique implemented during activities of daily living will facilitate it’s use among all who interact with the individual.
"Business as Usual"
"Business as Usual"
“Business as Usual”
It is essential that the caregiver/educator must act as if the behaviors are not happening and continue to conduct “business” as usual.
Example : If in the middle of an individual reading lesson the person with LND curses or spits at the teacher, the teacher, must continue the lesson without any response to the behaviors.
Identification of a LN behavior must never be followed by a verbal or physical recognition by the caregiver – or bystander – of the Lesch Nyhan behavior in question. Intervention proceeds after a determination of whether if harm appears imminent. If no harm, then redirection should be used. When the behavior is completely ignored and there is no negative consequence raised to the behavior, then the behavior will lose its self-injurious value and decrease.
Important
There are times when it is impossible to ignore a LN behavior because of impending harm. If a hand comes out of a protective device and the child immediately starts to put his/her finger into the eye socket it must be stopped. But it must be done with only minimal necessary attention and without a verbal response. It’s important that the caregiver must intervene as unemotionally as possible.
Self-injury is a hallmark feature of Classic Lesch-Nyhan disease and constitutes the most dramatic part of the behavioral phenotype. Although self-injury occurs in other intellectual disabilities, individuals with LND exhibit an unrelenting and diverse form of self-injury not otherwise observed. What also sets it apart is a dramatic increase in self-injurious behaviors when punishment is used as a component of behavioral modification interventions. In addition to behaviors that impact every facet of their lives, Lesch-Nyhan is a complex multisystem disability that requires ongoing care throughout the lifespan.
Biting
In Classic LN more than half end up having some teeth removed, providing relief to both the patient and their family by preventing self-biting. While tooth extraction has proven beneficial in these cases, it's not a one-size-fits-all solution. Keeping all teeth is possible when biting is minimal or infrequent. Consultation with a dental surgeon is crucial if urgent tooth extraction is needed. We discourage prolonged use of intricate dental devices for long-term care in this patient population due to self injury and choking hazards.
“Identifying a dentist can be difficult but don’t give up!”
FAQs
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Redirection can be overt, such as discussing their favorite sports team; or redirection can be subtle, such as in continuing with a lesson in the classroom.
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When redirecting to another topic and ignoring the LN behavior there must not be facial expressions of disapproval, no physical retreat, or any reassurance such as "That's O.K."
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If a hand comes out of a protective device and the child starts to self-injure, intervene unemotionally and without verbal response.
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In Classic LN more than half end up having some teeth removed, providing relief to both the patient and their family by preventing self-biting. While tooth extraction has proven beneficial in these cases, it's not a one-size-fits-all solution. Keeping all teeth is possible when biting is minimal or infrequent. Consultation with a dental/maxillofacial surgeon is crucial if urgent tooth extraction is needed. We discourage prolonged use of intricate dental devices for long-term care in this patient population due to self injury and choking hazards.
Tooth Extraction: Often necessary to prevent self-biting, providing relief to the patient and their family. Study
Consultation: Essential with a dental/maxillofacial surgeon for urgent cases.
Long-Term Care: Prolonged use of intricate dental devices is discouraged due to self-injury and choking hazards.
Essential to the method
There must never be evidence – in the behavior or actions of the caregiver – of punishment, however subtle.
(This work is based on the work of Anderson and Ernst and developed by Zumwoff and refined by Henry.)
Published Studies
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Modes and patterns of self-mutilation in persons with Lesch-Nyhan disease
Kenneth L Robey 1, John F Reck, Karen D Giacomini, Gabor Barabas, Gary E Eddey
Affiliations Expand
PMID: 12613772
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Behavioral aspects of Lesch-Nyhan disease and its variants
David J Schretlen et al. Dev Med Child Neurol. 2005 Oct
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Self-injury in Lesch-Nyhan disease
L T Anderson et al. J Autism Dev Disord. 1994 Feb.
If your child has recently been diagnosed with Lesch-Nyhan, it's important to understand the signs and symptoms associated with the condition. This information can help you better understand the individual’s needs and advocate for appropriate care.
Children and adults with Lesch-Nyhan may experience a variety of symptoms, including self-injurious behavior, neurological problems, motor difficulties, kidney stones, and gout. However, it's important to remember that each individual's experience with the condition will be unique.
If you have any questions or concerns about your child's or adult’s symptoms, please talk to your doctor. They can help you understand the individual’s specific needs and provide appropriate care.