What is the life expectancy of a person with Adrenoleukodystrophy?

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please tell me what is the life expectancy of a person with Adrenoleukodystrophy. please also cite sources. thanks a lot! =)
Without the wonderful finding of the parents of Lorenzo the life expectancy was terrible. But they came up with Lorenzo's oil to slow the brain's deteriations down. It was a miracle.

http://jnnp.bmj.com/cgi/content/extract/67/3/279  (+ info)

How can adrenoleukodystrophy be treated? Can a person die from it?

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I have a science project due soon and i would like to know if anyone knew anything about adrenoleukodystrophy. Please tell me anything you know about the disease and any links to any websites that you may know of. Thanks!!
Ever hear of the 1993 movie "Lorenzo's Oil"? The movie is about a boy named Lorenzo Odone and he has ALD. Rent it and watch it. If you have Netflix, watch it on your PC.

There is a group of genetic disorders called leukodystrophies and Adrenokleukodystrophy (ALD) is one of them. Adrenoleukodystrophy is a rare, inherited metabolic disorder. The myelin sheath on nerve fibers in the brain is destroyed and the adrenal gland degenerates. This leads to progressive neurological disability and, ultimately, death. People with ALD accumulate high levels of saturated, very long chain fatty acids in their brain and adrenal cortex because the fatty acids are not broken down by an enzyme as they normally are in others without ALD.

There are two subtypes of ALD. The more common form is X-linked ALD, or X-ALD, (abnormal gene located on the X-chromosome) and the less common (and milder form) is adult-onset ALD.

Adrenal hormones are part of the treatment. Other treatment is symptom-targeted and supportive -- physical therapy, psychological support, special education. A mix of oleic acid and erucic acid (Lorenzo's Oil) applied to boys with X-ALD can reduce or delay the appearance of symptoms. BMT (bone marrow transplant) can help boys who have early evidence of X-ALD. Docosahexanoic acid (DHA) given orally may help infants and children with neonatal ALD.

The prognosis is poor. This disease is progressive neurological deterioration. Progressive...that's the key word. Death usually occurs within 1 to 10 years after the symptoms appear.

This is your project and you are the one who is supposed to do the research for it. I am confident that you'll find many websites about this condition if you look for them. Run a search for what you need to know, like symptoms, genetics, treatment, whatever.  (+ info)

What is the life expectancy of a person with adrenoleukodystrophy?

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And dont tell me what it used to be ! I want to know what it is now.
Its going to depend on how good the treatment plan is.

An expert researcher/consultant will provide better treatment than an expert consultant (who is always busy and not up to speed with latest developments).

Often times; prognosis is well underestimated.

As every individual tumour is different in genetic makeup; it is not possible to accurately specify a prognosis. For instance: 2 patients might be diagnosed with adrenoleukodystrophy and given the same prognosis; but one patients tumour maybe far more aggressive than another. Tumours develop in a particular location in the body; and that's how they are categorized, but that has no bearing upon tumour aggressiveness.

Doctors may use immunohistochemistry and the study of cell morphology to guesstimate the progression of the tumours development..

In future: Patients will have their tumours genetically analyzed so that the exact mutations in the tumour will be identified allowing for specific treatment and more accurate prognosis. Some doctors have utilized this approach and had good result, but it is few and far between.

One must have a very proactive approach to ones own treatment, to push for the best advice and best treatment option. Always get a second and third opinion, that has been my experience.  (+ info)

This is about Adrenoleukodystrophy: What are the factors can affect the build up of very long fatty acids?

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What I understand is that in ALD, LCOFA is the cause of the demyelination... Now what I don't know is what are the factors that could cause this long chain of fatty acid build up? Any answers please for my case..
I think you mean VLFCAs (Very-Long-chain fatty acids).

The reason these build up in the brain is that ALD patients cannot shorten fats longer than 24 carbon atoms (the ALD protein that is used in this process doesn't work or has diminished functionality).

And these fats then get integrated in structures (like myeline) where they cause problems (in ALD myeline is not correctly formed due to the overabundance of VLFCAs).

If you have more specific questions about this process please ask them and I'll do my best to answer them.  (+ info)

How would you support a patient that has Adrenoleukodystrophy and their family?

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Not sure  (+ info)

I work in a nursing home. There is a boy there who as adrenoleukodystrophy.?

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He cannot talk, walk, eat by mouth, control bowels/bladder, and as a tracheotomy. I always wonder with him if his cognitive abilities are intact. Does anyone know about this disease? Would he still understand everything going on around him? Thanks
A rapidly progressive X-linked genetic disorder - affecting only boys - usually between the ages of four and ten, adrenoleukodystrophy (ALD) is a member of a group of diseases called leukodystrophies, that cause damage to the myelin sheath of nerve cells. This is the insulating envelope of myelin that surrounds the core of a nerve fibre or axon and facilitates the transmission of nerve impulses. There are three basic forms of ALD: childhood, adult-onset, and neonatal. The childhood form of the disease is the classical form and is the most severe. Childhood ALD is progressive and usually leads to total disability or death. Prognosis for childhood and neonatal ALD patients is poor because of the progressive myelin degeneration. Death usually occurs between one and ten years after onset of symptoms. There is a test for cognitive ability associated with this disorder which is done using Magnetic resonance spectroscopy. The details are technical and I will not discuss them here, except to state that each patient has different results and therefore there is no way of knowing the answer to your question without the assistance of the test.
I add a link which has some details of this disorder

http://www.betterhealth.vic.gov.
au/bhcv2/bhcArticles.nsf/pages/
Leukodystrophy?OpenDocument

Hope this helps
matador 89  (+ info)

how many people are suffering from adrenoleukodystrophy?

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ok so in bio we watched Lorenzo's Oil the movie and now we hvae to write this paper and i need to know how many people are suffering from it... i tried searching everything =[ but maybe you guys can help? its also called ALD

thanks a bunch!
also how much does the lorenzos oil research cost?
It affects approximately 1 in 20,000 to 1 in 50,000 individuals from all races.

This is a link to some URLs that can answer any of your questions:
http://www.ninds.nih.gov/disorders/adrenoleukodystrophy/adrenoleukodystrophy.htm#What_research_is_being_done  (+ info)

What is the current treatment for Adrenoleukodystrophy, or ALD?

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Subject of film 'Lorenzo's Oil' dies at 30
http://news.yahoo.com/s/ap/20080531/ap_en_mo/obit_lorenzo_odone

The man whose parents' battle to save him from a nerve disease ALD was depicted in the movie "Lorenzo's Oil" died Friday at his home in Virginia, having lived more than 20 years longer than doctors had predicted.
The best answer I found is at the link below:

It is technical but readable.  (+ info)

what percentage of people in the united states have adrenoleukodystrophy?

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  (+ info)

does anyone know about a gentic diease called adrenoleukodystrophy diease?

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i kno the baics about it but can anyone go into detail
has oneone kno someone that has had it

i have to right a paper
but i really just wanna kno more i read about it its the saddest thingk ever
Rent the video Lorenzo's Oil - its based on a true story of someone with this disease.  (+ info)

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