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Hi Sunny

Here is a summary of things to avoid, put together by one of our

members from information gathered at one of the mito conferences. I

have some of the original articles that I can send as an attachment

privately.

laurie

MEDICATIONS CONTRAINDICATED IN MITOCHONDRIAL DIESASE

On page 269 of the Syllabus for the International Conference on Mito

Disease, 1997 you will find the following recommendations:

" Treatment remains largely symptomatic and does not significantly

alter the course of the disease. It includes avoidance of drugs and

procedures known to have a detrimental effect, symptomatic treatments,

supplementation with co-factors, prevention of oxygen radical damage

to mitochondrial membranes and dietary recommendations. "

" It is advisable to avoid Sodium Valproate and barbiturates which

inhibit the respiratory chain and have occasionally been shown to

precipitate hepatic failure in respiratory enzyme deficient children.

Tetracyclines and chloramphenical should be avoided as well, as they

inhibit protein synthesis. "

As well certain types of anaesthesia are also contraindicated. There

is a wonderful and comprehensive article dealing with the safety of

anaesthesia in mitochondrial cytopathy patients in the October 98

issue

of the United Mitochondrial Disease Federation newsletter, as well as

at the UMDF site.

It is not always possible to avoid these drugs but at least if you are

informed ahead of time and the possibility of an alternative exists,

you can make an informed decision. There is no doubt that some folks

with

mitochondrial cytopathy have used these drugs uneventfully, although

the potential is still there. Be an informed consumer!

Cohen and Shoffner lists about anaesthetic agents.

Barbiturates - Inhibits complex I activity at high levels

Benzodiazepines - Inhibits adenosine nucleotide translocase

Propofol and/or lipid carrier - Inhibits mitochondrial function

Halothane - Increased risk for heart rhythm disturbance

Nitrous Oxide - Neurotoxic, possibly by increasing nitric oxide

production, which inhibits cisacotinase and iron-containing electron

transport enzymes: affecting energy production

Non-depolarizing Agents - Increased sensitivity to the paralytic

effects and prolonged responses reported

Local Anaesthetics - Bupivacaine uncouples oxidation and phosphorylation

Should never use Lactated Ringer's solution (Ringer's Lactate) because

it contains lactic acid.

Depolarizing agents (Succinylcholine) bothers some people.

****************************

Valproate or Depakote (Valproic Acid) is an anti-convulsant. It is

known to be a potential toxin to those with either mitochondrial

disorders or metabolic disease. It can cause severe hepatoxicity or

liver failure in susceptible persons.

Phenobarbital is another potential mitochondrial toxin that can also

be dangerous to those with mitochondrial cytopathy. It is wise to

always ask your pharmacist for the generic name of any drug being

prescribed for someone with mitochondrial cytopathy.

Hormonal Birth Control

Aminoglycosides are ototoxic for *some forms of Mitochondrial

Cytopathies - a specific genetic variation ( Mitochondrial 12S and 16S

ribosomal RNA - mutation of A to G in the 12S rRNA at position 1555)*

{Hutchins, Cortopassi 1994}. Aminoglycosides are *some* of the

antibiotics which end in " _______mycin " . Ototoxic means that these

drugs can cause hearing loss or deafness. ASK YOUR PHARMACIST AND

PRESCRIBING DOCTOR.

Ergotamines due to the impact on hepatic function.

Neuroleptic drugs such as Haloperidol, Chlorpromazine, and Thiothixene.

Lipitor should not be used by someone with Mitochondrial disease as it

depletes CoQ10 from the cells.

Steroids can be used but should be used only when absolutely necessary

and this should be a very low dosage and not for an extended period of

time.

Friendly alternatives (?)

Lamictal seems to be being used successfully. Topomax also appears to

be mitochondrial-friendly for intractable seizures.

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