Lorenzo's Oil
So, this background doesn't really fit with what I'm going to talk about but I just think it's so cool looking (I stole it from my friend Greg's webpage). This past week in lab we watched the movie Lorenzo's Oil. It is about the disease called adenoleukodystrophy (ALD) which causes a demyelination of the axons that results in rapid decline in motor abilities as well as intellectual abilities, and ultimately death. It only affects males, mostly young boys, and the catch is that it is the mother who passes on the gene to her son. She is the carrier, but it does not affect her.
I didn't quite understand the mechanism behind the disease. I think that the person's body afflicted with it is lacking an enzyme required to break down a certain fatty acid. So there is a build up of this fatty acid....and this is where I get lost, it somehow causes the demyelination of the axons. How does this happen? Since the Schwann cells make myelin in the PNS and oligodendrocytes make myelin in the CNS, how is it that the myelin somehow is destroyed? I would think that it would be regenerated, especially since those glial cells are able to regenerate themselves- they are not like neurons which will never grow back. This is something I would like to know about, if the answer is even known.
Another thing that sparked my interest was when the boy's mind and body started deteriorating the doctors were saying that even if they could stop the progress of the disease, he would still be incredibly brain damaged and would not be able to function normally. My question is, if it is just the myelin around the axons and not the axons themselves that are damaged, then why would brain damage occur? If the myelin could be regenerated around the axons then wouldn't they be able to function as normal?
So...now on to the movie itself...
A major struggle in the movie was between medical science and the patients they are supposed to be treating. This movie had a mission to portray scientists as self-consumed and uncaring which could or could not be true (...ooohhh). It seemed as though the doctors and scientists had a different agenda than the parents of this sick child. A scientist is not the person to talk to about intuition. Of course scientist use intuition all the time to develop hypotheses, but their intuition must be proved over and over again before they will accept it as truth. So when the parents came to these "scientific people" with a possible hope, a possible answer, and wanted to implement it right away with their own child, of course the response they got was "this needs to be tested many times in a controlled situation before it can be administered..." But these parents were losing their child, and they could not wait for this extensive testing to be done. So they took it into their own hands; the scientists can't tell them what they can and cannot feed their child, can they? So they gave him a mixture of two fatty acids, and eventually he seemed to get a bit better. He even started communicating with eye blinks. So was it the fatty acids that helped him? Or was it something else entirely? We won't know because this wasn't done according to the scientific method. But at least his life was made a little better. He was no longer having horrible seizures, and like I said, he could communicate a bit. He was still stuck in a body that wouldn't move, and wouldn't speak....I wonder what he felt inside.