Manage: Tools and Equipment

Please note: The information provided in this section is for educational purposes only and is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.

Suggested First Steps

Health Tips

Therapies

Tools & Equipment

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Behavior Management

“Ignore the Behaviors, not the individual”

Protective devices/safeguarding equipment by setting, including therapist assigned to the task. Ongoing monitoring of devices required on a daily basis. Backup must be available. Material to cover padded devices must be friction free to avoid self-injury from rubbing.

Equipment may need additional considerations and modifications to prevent SIB’s. Parents often innovate out of necessity, crafting solutions using items like pool noodles, foam, padding, yoga mats, and towels to ensure the safety of their children.

Classic Lesch Nyhan

BED

Arm Devices • PT

Leg Devices • MD

Chest harness

Protective padding

Pelvic belt

BATHROOM / BATHING/TOILETING

Tub Protective padding •PT

Arm devices • OT

Leg devices

Custom shower trolley

Gary Eddey, MD, ScM(HYG), Kelly  Henry, MSW

MEAL PREP

Positioning of head and neck • ST/dysphagia

Consistency of liquids  • MD/aspiration

IF GASTROSTOMY TUBE PRESENT

Abdominal protection • MD

STROLLER

Arm Devices • PT

Leg Devices • OT

WHEELCHAIR

Arm devices •. MD

Leg devices • PT

Chest Harness • OT

Headrest modifications oversized  • Rehab

Padding • WC Vendor

Safety Cover for seatbelt

Use of protective devices or safeguarding equipment

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

Augmentative and Alternative Communication (AAC)

Augmentative and Alternative Communication (AAC) refers to methods of communication used to help those who are unable to use verbal speech to communicate effectively. AAC encompasses a wide range of communication methods, from basic gestures and sign language to more complex technology-based systems. The primary goal of AAC is to facilitate expressive and receptive communication for individuals with speech and language impairments.

No-tech / low-tech options:

  • Gestures and facial expressions

  • Pointing to photos, pictures, or written words

High-tech options:

  • Communication Apps on iPad and Tablets

  • Speech generated devices

  • Eye Gaze communication

Protective Devices

  • Protective devices will be necessary to protect your child from self-injury.

  • Elbow immobilizer

  • Wrist immobilizer

  • Padding on wheelchair

  • Leg restraints

  • Mouth guards

  • Helmets

Durable Medical Equipment (DME)

Durable medical equipment will be necessary for comfort, safety, and mobility. Here’s a list of common equipment for used for Lesch-Nyhan.

Equipment Modifications

The following equipment is essential to assisting with Lesch-Nyhan.

  • Wheelchair:

    • A wheelchair is essential for a child living with LND. A wheelchair comes in many sizes with a variety of supports. A headrest, 5-point harness, lateral supports and leg restraints will be necessary for safety.

    • Consult with your child’s physical therapist and a Durable Medical Equipment (DME) provider to determine the appropriate supports.

  • Stander:

    • A stander is a frame that holds the patient in various positions, including upright, prone, and supine. Getting in a standing position improves bone density, supports skeletal development, enhances head control, supports proper hip alignment to name a few.

  • Gait Trainer:

    • Children with LNS have no independent mobility due to the lack of overall muscle control and severity of the motor disorder. A gait trainer offers a variety of supports, which will allow your child to move freely.

      • Like wheelchairs, a gait trainer will provides wheel-assisted mobility.

        • Improves independence

        • Assists with balance and mobility

        • Builds muscle strength

  • Bath/Shower Chair

    • Bath and Shower Chairs will be necessary for safety as your child grows. These specialized chairs will offer the appropriate head support and 5 point harnesses necessary for safe bathing. Speak with your child’s physical therapist and local DME provider to discuss options.

  • Foot & Arm Bracing

    • AFO (Ankle Foot Orthosis) - Supports weak muscles and helps to reduce spasticity. Assist in keeping feet and ankles in appropriate alignment.

    • Elbow Immobilizer - Helps to prevent self-injury by immobilizing arm.

  • Footwear:

    • BILLY Footwear incorporates zippers that go along the side of the shoes and around the toe, allowing the upper of each shoe to open and fold over completely. This allows the wearer to step into the shoe while wearing their AFO, then just zip it closed.

  • EazyHold:

    • Soft silicone that attaches to many regular items and helps children and adults grip the item more securely.

  • Elbow Immobilizers:

    • Most commonly used to prevent the hand from getting to the face during times of self-injury.

Equipment may need additional considerations and modifications to prevent Self-Injurious Behaviors. Caregivers frequently use the following:

  • Pool noodles

  • Foam padding

  • Yoga Mats

  • Towels

  • Extension on headrests

Daily use