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Copper Supplementation

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Menkes Disease is a metabolic disorder affecting copper transportation around the body. This results in the accumulation of copper in some organs while others, namely the brain, receive too little.

Copper supplementation aims to provide copper to the deprived organs. The most common and successful form of copper supplementation thus far has been the regular injection of copper salts in Menkes patients. However the impacts of this treatment vary substantially amongst Menkes patients and at this stage cannot be considered a comprehensive cure.

Benefits of Copper Supplementation for Newborns:

Amongst newborns who were diagnosed and began copper treatment before 2 weeks of age there have shown to be promising results with a number achieving successful clinical outcomes, defined as reaching normal or near normal neurodevelopment by the age of 3 years.

The most successful outcomes seemingly occur in newborns who have mutations that allow for some copper transportation (at least 5-10%). In newborns who have mutations that do not allow for such transportation , early diagnosis and treatment has been shown to enhance survival† and reduce seizures in some cases and thus copper treatment should still remain a consideration for these newborns. In a 2008 paper, Dr Kaler reported on 12 newborns who were treated from birth with copper and had a 92% survival rate at 4.6 years, compared with a historical control group of 13% survival rate at 1.8 years of 15 late diagnosis and treatment patients.

In the future, gene therapy to restore the necessary levels of copper transportation in such newborns is the best chance for those who have shown to have mutations that do not allow copper transportation in order to obtain successful clinical outcomes.

Benefits of Copper Supplementation for Older Infants:

While the vast majority of research into the success of copper treatment has centred around newborns, there has been little research into itís success when infants are diagnosed and begin treatment after the first few weeks of life.

However, the limited research into the administration of copper to older infants has not been promising and there have been no reported cases of children in this group reaching major milestones like independent walking, or recovering lost functions once the damage has occurred.

What has been reported in this group is the normalisation of both copper and ceruloplasmin serum levels in the immediate period after treatment began. As well as this, it has been reported that copper administration may assist in preventing or controlling physical seizures. Copper treatment has also been shown to improve muscular tone, motor activity, irritability as well as hair and skin pigmentation in some cases. There is however, no clear evidence that it has any effect on prolonging life span.

Side Effects of Copper Injections:

The possible side effects of copper treatment relate mainly to the use of injections as the administering tool. Discomfort can occur, as can local site reactions and skin infections. However, by utilising very thin gauge insulin needles, the chance of such reactions can be greatly reduced.

Another possible side effect of copper administration can be renal tubal damage which is a known adverse effect of copper overload. Close monitoring by means of regular blood tests and changes in dosage if required greatly minimise the chance of copper overload in the body.

Availability of Copper Compounds:

Copper histidine is an inexpensive and easily manufactured drug, however due to the rare nature of Menkes Disease is not widely available. It is currently manufactured at Westmead Childrenís Hospital, Sydney and Menkes Australia understands that it is also produced at The Royal Childrenís Hospital, Melbourne.

Last Updated on Thursday, 14 June 2012 10:25