Krabbe disease is a rare degenerative condition that affects both the central and peripheral nervous system. A lack of a specific enzyme results in a breakdown in the protective sheath, or myelin, which surrounds nerves in the brain. Krabbe disease is also known as Globoid Cell Leukodystrophy.
Symptoms and characteristics
Symptoms of Krabbe disease generally commence within the first year of life, however, on rare occasions the disease can commence in adolescence or adulthood. The earlier the onset of symptoms of Krabbe disease, the more rapid the progression of symptoms.
For persons with late onset Krabbe disease, symptoms may vary from weakness, or muscle spasticity, where certain muscles are continuously contracted with no deterioration in cognitive function, through to progressive deterioration in both physical and intellectual functioning. A decline in vision and hearing can also occur.
For more information on some of these symptoms and characteristics, follow these links:
Workplace adjustments and solutions
Due to the degenerative nature of the disease, as well as the potential broad range of both intellectual and physical decline, employment related considerations and support for those with late onset Krabbe disease would need to be determined on a case by case basis and reviewed regularly. Some suggestions to assist in the workplace include:
- modifying workstation set up to avoid excessive exertion and conservation of energy
- providing and adjusting an ergonomic chair or stool to a person’s needs, to allow work from a seated rather than standing position or postural rotation
- consider capacity for staff rotation between physically demanding tasks and lighter tasks, as a way of building in breaks without having to stop work
- consider the use of lifting or mechanical aids such as trolleys to minimise lifting requirements
- if mobilising via wheelchair, ensure wheelchair accessibility to all areas of the workplace including kitchen and bathroom amenities, workspace and other doorways or emergency exits
- installation of ramps for independent building access
- automatic door openers and positioning of security pin pads to allow independent door access
- desk modifications to allow for wheelchair clearance and easy access to all items on the desk
- the use of task boards and memory aids or prompts to assist in task completion
- the use of adaptive technology to overcome barrier of vision impairment.
The Employment Assistance Fund provides financial assistance for work related modifications, equipment and services to help people with disability to get employment and perform their work as independently and productively as possible. To determine eligibility, please refer to the full guidelines:
There are specific solutions and adjustments relating to Krabbe disease:
References
Australian Leukodystrophy Support Group Inc. 2011, About Leukodystrophy, Australian Leukodystrophy Support Group Inc., Victoria, viewed 17 December 2011, http://alds.org.au/about-leukodystrophy.
United Leukodystrophy Association 2011, Krabbe Disease, United Leukodystrophy Foundation, Inc., Illinois, viewed 17 December 2011,
http://ulf.org/krabbe-disease.
WebMD 2005-2011, Leukodystrophy, Krabbe's, WebMD, online, viewed 17 December 2011, http://www.webmd.com/brain/leukodystrophy-krabbes.