Adrenoleukodystrophy is Adrenoleukodystrophy Disease Adrenoleukodystrophy Adrenoleukodystrophy is a genetically transferred disease which causes the human body to not be able to breakdown “very-long-chain-fatty-acids’ (VLCFA).
This inability to break down these acids eventually leads to myelin deterioration as well as the deterioration of the nervous system. Although there is no cure for this terrible disease, there are a variety of effective treatments. Adrenoleukodystrophy is a sex-linked trait carried on the X-chromosome and is carried by the mother.
It is only passed on to male children, and every male child that a carrier mother gives birth to has a fifty-fifty chance of acquiring adrenoleukodystrophy. Adrenoleukodystrophy, more commonly called ALD, affects the nervous system. The inability to break down fatty acids causes demyelination of the nerves in the brain, spinal cord, and other areas of the body. Myelin is the insulating sheath around a nerve, and is broken down during demyelination. Once the myelin disappears, nerves will start to short-circuit and stop working. Symptoms can include seizures, tantrums, and mood-swings.
ALD generally occurs in one of three main forms. In the first form, which is also the most common, occurs in young male children, usually before they reach the age of twelve. Although this is the most severe form, causing loss of the function of the brain and nervous system, it has the most available treatments. The second form, which is usually present in adults, is characterized by a gradual loss of nerve function, much like that of multiple sclerosis. It has also been misdiagnosed by some inexperienced or uninformed doctors as MS, which may lend some insight as to the similarities between the two diseases. Unfortunately, there are no “recognized’ treatments for this form of the disease.
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The third form is very similar to Addison’s Disease, in which the VLCFA build up in the adrenal glands, and they lose their function. For this form though, there are treatments available. Basically, there are two forms of treatments for ALD. In one form, treatments are used to prevent ALD from becoming fatal before the symptoms start. In the other form, the treatments are used for children who have already exhibited symptoms of ALD. The first form, know as pro-active treatments, include dietary therapy or administration of the cholesterol-lowering drug, lovastatin.
The dietary therapy method encompasses the use of a low fat diet and consumption of Lorenzo’s Oil. The Lorenzo’s Oil causes the levels of VLCFA to reach a near normal level. It is believed that if these levels remain at normal, their accumulation can be prevented. The lovastatin method works the same way as the Lorenzo’s Oil, only that it is not necessary for it to be used in conjunction with a low fat diet.
The second form of treatment, known as the re-active approach, is used once a child has already begun to exhibit symptoms. It involves a bone marrow transplant. This works because it places in an affected person’s body enough normal tissue to be able to metabolize the VLCFA. This must be executed before the disease has progressed too much, or the transplant will not work, and the person’s death might even be hastened. For the form of the disease similar to Addison’s disease, the therapy is long-term hormone replacement.
Adrenoleukodystrophy is a ravaging disease which can be incredibly devastating to the victims as well as their families. Fortunately, treatments have progressed, and it is possible to prevent the death and many signs of the disease. Currently, there is a research project, known as The Myelin Project, which is working on the ways to possibly remyelinate the nerves, and repair the damage caused by ALD. These promising new insights offer a ray of hope to future sufferers of adrenoleukodystrophy..
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