Behavior problems

The behavior problems, including self-injurious behavior, can also be difficult to eliminate. Effective management of this problem requires three ingredients. The most important of these is prevention by physical restraint. All sharp and dangerous objects must be kept out of reach. The wheelchair should have all sharp edges within reach covered safely. Loose arm ties or elbow splints can be very helpful to control finger biting or hitting. Some children must have their teeth removed if they bite themselves.

The second most important ingredient for controlling self-injurious behaviors is behavior therapy. This can be done at home, but in most cases it is done by professionals who have been trained in behavior therapy. The goal is to focus attention on good behaviors while ignoring bad behaviors. Punishing bad behaviors by shouting or spanking does not work, and may even make the bad behaviors worse. It is also helpful to identify the situations that cause problem behaviors, and then try to avoid these situations.

The third ingredient for controlling self-injurious behaviors is medications. Many different medications have been used to control these behaviors, but they do not work all of the time. Several medications may also be useful in the management of self-injurious behaviors, but none has been evaluated in a controlled clinical trial. Promising possibilities include gabapentin, carbamazepine, diazepam, others. These medications can reduce the frequency of self-injurious behavior, but they rarely eliminate it entirely.

Some physicians also like to use risperdal for self-injurious behavior. Unfortunately, risperdal causes sleepiness and can sometimes make the neuromotor problems worse. If it is used, it is important to verify that improvements in self-injury are not being traded for sedation or worsening of neuromotor function.

Splints

Splints are firm supports that prevent certain types of unwanted movements. The most common ones are attached to the arms to prevent bending of the elbow. By preventing elbow bending, finger biting can be stopped because the child can not get his hand to his mouth. Splints can be obtained from many medical supply stores, such as medi-kid.

Wheelchairs

Most children with Lesch-Nyhan disease need a wheelchair to get around. This wheelchair must be specially designed for safety. Images in this section show an example of a wheelchair expecially designed for Lesch-Nyhan patients. All sharp corners and objects within hand reach must be covered with soft padding, so the child cannot bang or scrape himself. All sharp corners and objects within reach of the feet must be similarly covered.The spokes of the wheels must be covered to prevent the child from inserting his hand when the chair is in motion.

This is an example of a custom made wheelchair of a Lesch-Nyhan patient.
This is an example of a custom made wheelchair of a Lesch-Nyhan patient.

The head rest must be high enough to prevent the child from flinging his head backward and injuring his neck. Side supports for the head may also be required, to prevent him from flinging his head towards the sides.

A soft but sturdy head rest prevents injury to the head and neck and often reduces the urge to fling the head backward.
A soft but sturdy head rest prevents injury to the head and neck and often reduces the urge to fling the head backward.

Straps for the chest may be needed to prevent the child from flinging himself forward out of the wheelchair, or banging his head on things in front of him. Sometimes, arm straps or shields can be used to prevent flinging movements, or other self-injurious behavior such as biting fingers or poking in the eyes. When these straps are adjustable, they can allow a restricted but safe range of movement, preserving functional use of the hands. Straps for the legs might also be helpful to prevent self injury to the legs and feet.

These arm straps prevent flinging movements of the arms and other forms of self-injurious behavior. The shield next to it can be raised to prevent self-injury such as stretching the arms while being pushed through a doorway, or hitting walls or other objects.
These arm straps prevent flinging movements of the arms and other forms of self-injurious behavior. The shield next to it can be raised to prevent self-injury such as stretching the arms while being pushed through a doorway, or hitting walls or other objects.
Straps for the chest can prevent the patient from flinging or falling out of the wheelchair, or banging his head forward or backward.
Straps for the chest can prevent the patient from flinging or falling out of the wheelchair, or banging his head forward or backward.
An example of adjustable arm straps. The length of these straps can be individually adjusted, allowing a restricted but safe range of movement. In this way, functional use of the hand can be preserved, for example to use a cell phone or mp3-player.
An example of adjustable arm straps. The length of these straps can be individually adjusted, allowing a restricted but safe range of movement. In this way, functional use of the hand can be preserved, for example to use a cell phone or mp3-player.

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