Manage: Behavior Management
This page explores the challenging behaviors with Lesch-Nyhan in detail and provides effective management strategies to support those affected by LNS, aiming to improve their quality of life and overall well-being.
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“Ignore the Behaviors, not the individual”
Overview
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.
Categories of Self-Injury
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.
Recognition of self-injurious behaviors is the first necessary step to inform and manage the 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.
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.
Examples: False accusations, suicidal thoughts.
Intervention: Recognize the pattern of false accusations and manage accordingly.
Management Strategies
Selective Ignoring with Redirection (SIwR)
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 Principles:
There must never be evidence – in the behavior or actions of the caregiver – of punishment, however subtle.
It must be immediately implemented after recognizing a self-injurious behavior.
Use by everyone across all environments until the behavior is extinguished.
The redirection component allows for altering the behavior without calling attention to it.
Protective Device
Application: Used judiciously to prevent self-injury.
Adjustment: Consider readjustment based on the individual’s needs.
Example Scenarios
In a Classroom: If an individual curses or spits at a teacher, the teacher should continue the lesson without responding to the behavior.
During Medical Exams: Address self-injurious actions with minimal attention and without verbal response.
Dental Care
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.
FAQs
What does it mean that a caregiver must not “give a verbal or physical recognition of the LN behavior”?
When redirecting to another topic, there should be no facial expressions of disapproval, no physical retreat, or any reassurance such as “That’s O.K.”
What to do if harm appears imminent?
If a hand comes out of a protective device and the child starts to self-injure, intervene unemotionally and without verbal response.
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.