'Goals For Jai' is non profit and managed by a committee to facilitate events and donations for the purpose of raising money for Jai Godbier and awareness of Metachromatic Leukodystrophy (MLD)
Our aim is to meet goals to help Jai and his family to manage his condition and to provide awareness of MLD. Click the "See More" link below to find out about Jai's condition. We have a number of up and coming fundraising events that you can attend to show your support for Jai, (see the events tab under ‘More’ in the navigation links under Jai’s banner). What is Leukodystrophy?
“The leukodystrophies are a group of rare genetic disorders that affect the central nervous system by disrupting the growth or maintenance of the myelin sheath that insulates nerve cells. These disorders are progressive, meaning that they tend to get worse throughout the life of the patient.”
What is Metachromatic Leukodystrophy (MLD)? MLD one of over 40 types of Leukodystrophy diagnosed worldwide.
“Metachromatic leukodystrophy characterised by the accumulation of fats called sulfatides in cells. This accumulation especially affects cells in the nervous system that produce myelin, the substance that insulates and protects nerves. Nerve cells covered by myelin make up a tissue called white matter. Sulfatide accumulation in myelin-producing cells causes progressive destruction of white matter (leukodystrophy) throughout the nervous system, including in the brain and spinal cord (the central nervous system) and the nerves connecting the brain and spinal cord to muscles and sensory cells that detect sensations such as touch, pain, heat, and sound (the peripheral nervous system). There are three known forms of MLD – Late Infantile, Juvenile & Adult
While the prognosis for each of these forms of MLD varies, the most severe and the shortest life expectancy is the Late Infantile diagnosis.”
- Sources: United Leukodystrophy Foundation, Australian Leukodystrophy Support Group, Genetics Home Reference (provided by Specialists)
More about Jai’s diagnosis
Despite Jai’s symptoms commencing outside the “expected” window for the onset of Late Infantile MLD, his specialist medical team in Sydney have diagnosed his MLD as the Late Infantile form. What can Jai expect?
‘After a period of apparently normal growth and development, skills such as walking and speech may begin to deteriorate. Once clinical symptoms become noticeable, they often appear to progress rapidly over a period of several months, with alternating periods of stabilisation and decline.’
Children suffering from Late Infantile MLD eventually become ‘bedridden, unable to speak or feed independently. There may be seizures at this stage, which eventually disappear. Contractures are common and apparently painful. Children with this condition are still able to smile and respond to parents at this stage, but eventually may become blind and largely unresponsive. Swallowing eventually becomes difficult and a feeding tube becomes necessary. With modern treatment and care, the child may survive for 5-10 years…..’
- Sources: United Leukodystrophy Foundation, Australian Leukodystrophy Support Group, Genetics Home Reference (provided by Specialists)
What can we hope for? Mick and Ray; Jai’s parents, have been advised they should expect Jai’s quality of life to diminish significantly over the next 6-9 months. For those that have spent time with Jai in recent months, you will have noticed a rapid decline in his health already. Whilst it is difficult to be certain, the suggestion has been that after this 6-9 month period, Jai’s capacity to function independently will be significantly reduced or impossible. In terms of life expectancy, like many rare disorders, there is no guarantee however the advice received to date is that 3 years is a reasonable expectation. You can support one of our Events or make a donation:
Bank Acct Details:
Commonwealth Bank
Jai David Godbier
BSB: 062 526
Acct: 1013 8278
