Mito Cocktail? Doctor said it has no value!!!!!

[Guest guest]

I would love to show that neuro Gracie's lab work before and after the cocktail. Before she was a sick little girl with a failing liver. Her life expectancy was not too promising. After she is an active almost 2 year old with normal functioning liver. (Though there is permanent damage from BEFORE she was on the cocktail.) This doctor probably just has too high of an ego to admit he does not even know what it is. Hang in there and try to get a mito doc to help your little boy out.

[Guest guest]

I would love to show that neuro Gracie's lab work before and after the cocktail. Before she was a sick little girl with a failing liver. Her life expectancy was not too promising. After she is an active almost 2 year old with normal functioning liver. (Though there is permanent damage from BEFORE she was on the cocktail.) This doctor probably just has too high of an ego to admit he does not even know what it is. Hang in there and try to get a mito doc to help your little boy out.

[Guest guest]

I would love to show that neuro Gracie's lab work before and after the cocktail. Before she was a sick little girl with a failing liver. Her life expectancy was not too promising. After she is an active almost 2 year old with normal functioning liver. (Though there is permanent damage from BEFORE she was on the cocktail.) This doctor probably just has too high of an ego to admit he does not even know what it is. Hang in there and try to get a mito doc to help your little boy out.

[Guest guest]

Our Nuro doctor finally called back this morning. He said the Mito

Cocktail has no value and just a waste of money. That is very

frustrating!!!!! is getting weaker and we really wanted to try

it!!!! What resources can I use to convince the doctor to at least

consider it? What is a good web sight to get on to read about it?

Any help would be great! What I would do for a Mito doc right now!!!

Tamara(Mommy of age 5, unspecific Mito)

[Guest guest]

Tamara,

Oh boy! You should get alot of responses to this one. If it

weren't for the mito cocktail, I am sure my daughter would not be

alive today! Go to the UMDF.org website and go to the resources page

(I think). It has a section called treatment of mito disorders.

Forget you're doc and I would at least start him on the coq10, b

vit, vit e etc. These can all be gotten without a script. Find a new

doc. I have never heard of such a thing. This really ------ me off.

Is this a doc who know's anything at all about mito? I would find a

new doc if I were you. Do not give up, which seems like what he is

saying. Good luck,

Dawn

[Guest guest]

Tamara,

Oh boy! You should get alot of responses to this one. If it

weren't for the mito cocktail, I am sure my daughter would not be

alive today! Go to the UMDF.org website and go to the resources page

(I think). It has a section called treatment of mito disorders.

Forget you're doc and I would at least start him on the coq10, b

vit, vit e etc. These can all be gotten without a script. Find a new

doc. I have never heard of such a thing. This really ------ me off.

Is this a doc who know's anything at all about mito? I would find a

new doc if I were you. Do not give up, which seems like what he is

saying. Good luck,

Dawn

[Guest guest]

Tamara,

Oh boy! You should get alot of responses to this one. If it

weren't for the mito cocktail, I am sure my daughter would not be

alive today! Go to the UMDF.org website and go to the resources page

(I think). It has a section called treatment of mito disorders.

Forget you're doc and I would at least start him on the coq10, b

vit, vit e etc. These can all be gotten without a script. Find a new

doc. I have never heard of such a thing. This really ------ me off.

Is this a doc who know's anything at all about mito? I would find a

new doc if I were you. Do not give up, which seems like what he is

saying. Good luck,

Dawn

[Guest guest]

Tamara,

Here is the info on the umdf website for treatment of mito!

Treatment - At this time, there are no cures for these disorders.

Goals of treatment

(note: goals may never be met)

alleviate symptoms

slow down the progression of the disease

Effectiveness of treatment

varies from patient to patient, depending on the exact disorder and

the severity of the disorder

as a general rule, those with mild disorders tend to respond to

treatment better than those with severe disorders

in some circumstances, the treatment can be tailored specifically to

the patient, and that treatment is effective, whereas in other

circumstance, the treatment is " emperic " , meaning that the treatment

makes sense, but that the benefit of treatment is not obvious or

proven to be effective

treatment will not reverse the damage already sustained, such as

brain malformations

Benefits of Treatment and Effectiveness of Therapies Vary

treatment may be beneficial and noted immediately in some disorders

benefit of treatment may take a few months to notice

benefit of treatment may never be noticed, but the treatment may be

effective in delaying or stopping the progression of the disease

some patients may not benefit from therapy

Key Points to Treatment

Never forget there is standard treatment for some symptoms

(anticonvulsant medication for epilepsy, physical therapy for motor

problems, etc.)

Dietary

Vitamins and supplements

Avoidance of stressful factors

Treatment must be tailored by the patient's physician to meet that

patient's need. Many of these therapies are totally ineffective in

some mitochondrial disorders and would be a waste of time, money and

effort. In some cases, the treatment could be dangerous.

Specific Therapies & Things to Avoid

Dietary Therapy

Many patients, including young children or mentally impaired persons

have already " self-adjusted " their diet, because they know what

foods their body seem to tolerate. The points below are not meant to

be suggested therapies for all patients with OXPHOS disorders, and

some of the points are dangerous for patients with other disorders

(4b could be lethal in pyruvate dehydrogenase deficiency for

example). Do not make any of these dietary changes without

consulting a physician. A dietitian experienced in metabolic

disorders may be helpful.

Avoid fasting. This is perhaps the most important part of the

treatment for most people with metabolic disorders. Fasting

means " not eating " and avoiding fasting means avoid prolonged

periods without a meal (even an overnight " fast " from 8 pm to 8 am

may be dangerous in some patients). This also means that some

patients should not intentionally try to loose weight by decreasing

their food intake. In some patients an unintended fast resulting

from an illness that causes vomiting or loss of appetite (like the

flu) should be hospitalized to ensure continuous nutrition

(intraveneous glucose for example). In order to ensure adequate

frequent nutrition, sometimes a feeding tube needs to be placed in

order for the person to receive feeding at night. In some patients,

awakening them in the middle of the night for a snack can also be

helpful.

Small frequent meals may be better than a typical 3-meal-a-day

routine for some patients.

A snack before bedtime may be helpful in some patients. This snack

should not be mainly " sugar " , like a candy bar , jello or sweetened

cereal. It is usually best if the snack consists of a complex

carbohydrate. Cornstarch is the best complex carbohydrate, but this

is not very tasty. Theoretically, the best snack would be a homemade

low-sugar rice pudding thickened with a lot of cornstarch. If you

come up with a tasty recipe, let the UMDF know. Pasta, a peanut

butter sandwich, bread and butter, unsweetened cereal (oatmeal) or a

sandwich are acceptable. Many patients benefit by being woken up in

the middle of the night for a small meal and others clearly improve

when a gastrostomy tube is placed for continuous feeds. These final

two suggestions are a small price to pay for health.

a) There are conflicting lines of evidence regarding the use of high

fat meals in patients with electron transport chain disorders. In

patients that seem to gain weight and thrive on a high-fat diet, it

makes sense to continue the treatment. The extra fat can also be in

the form of MCT (medium chain triglyceride oil), which is easier to

metabolize (4c).

In other patients with OXPHOS disorders, reducing fat may be

helpful. This includes reducing added oil, butter, & margarine, and

cutting down on cheese and fatty meats. This recommendation is not

meant to avoid fats altogether. A defect in OXPHOS can create

an " energy backup " , as the respiratory chain cannot handle the flow

of electrons coming into it. This backup may result in the formation

of excess free fatty acids (fats waiting to be burned) , which can

poison the enzyme (adenosine nucleotide translocase) that exchanges

the low-energy ADP located outside the mitochondria for the high-

energy ATP formed at complex v. If you take the approach of limiting

fats, extra effort needs to be made to increase the total

carbohydrate (in the form of complex carbohydrates) in the diet.

c) In some patients (see #4a and #4b above), adding fat in the form

of medium chain triglycerides (MCT), may be helpful. Medium chain

triglycerides of 8 to 10 carbons long are easier to metabolize (turn

into energy) than the longer chain triglycerides (those with 12-18

carbons) because they do not require carnitine to be transported

into the mitochondria. MCT Oil@ is mainly made of 8 and 10 carbon

triglycerides and this type of oil does not occur in nature, but is

made from coconut oil. MCT Oil@ is made by the baby formula company

Mead-. It comes in quart bottles, available by prescription

and runs about $70 a quart. It can be added like oil over pasta and

rice. You can cook with it, but this is a light oil and burns

easily. The special rules are explained in a recipe book that you

can request from the pharmacist. Depending on the situation, a

patient may benefit from a few teaspoons to a few tablespoons a day.

There are oils sold in health food stores called " MCT Oil "

or " medium chain triglyceride oil " . These are much less expensive

($25 per quart), but make sure there is a certified analysis on the

label, stating that the vast majority of the oil is C-8 and C-1 0

(and not C-12 or higher) .

Iron generate free radicals under certain conditions, which is

especially bad in mitochondrial diseases because the free radicals

injure mitochondrial DNA and " poke holes " in the mitochondria,

making a bad problem worse. Therefore, excess iron is theoretically

harmful. In people with mitochondrial disease, there is no routine

need to give supplemental iron, nor is there a reason to eat foods

rich in iron, such as extra red meat, for the purpose of eating

foods rich in iron. This does not mean that the person should not

eat red meat, especially if they enjoy it. There is no reason to

take vitamins with added iron. There is the rare instance when iron

is needed, but this is not common. In addition, vitamin C enhances

the absorption of iron from the intestines, and vitamin C should not

be given around a meal rich in iron. This is important to remember

because some experts feel that vitamin C is a good antioxidant, and

also may be helpful in some disorders of OXPHOS.

---------------------------------------------------------------------

-----------

Avoidance of Toxins

Alcohol has been known to hasten the progression of some

mitochondrial disorders

Cigarette smoke, probably due to the carbon monoxide is known to

hasten the progression of some conditions. Remember that carbon

monoxide kills by inhibiting complex IV of OXPHOS, why make it

worse? Cigarette smoke will make it worse.

MSG (monosodium glutamate) has for years been known to cause

migraine headaches in otherwise healthy individuals, and may trigger

these events in susceptible people with mitochondrial diseases. MSG

is frequently added to Chinese (and other Asian) foods, and is also

found in high levels of dried and canned soups. Read the label and

avoid MSG if there is any sensitivity.

Vitamins and Cofactors

Vitamins and cofactors are compounds that are required in order for

the chemical reactions, which make energy, to run efficiently. By

definition, a cofactor can be made by the body, whereas a vitamin

cannot, and therefore must be eaten. For most people, a regular diet

contains all the vitamins one could possibly need and their bodies

can make as much of any specific cofactor that it needs. For those

with mitochondrial disorders, added vitamins and cofactors may be

useful. The use of supplemental vitamins and cofactors is largely

unproven and their use is therefore controversial in patients with

mitochondrial diseases. For disorders of OXPHOS, coenzyme Q10 is

considered as a generally accepted effective therapy, although it

may not ultimately be effective for an individual patient. Other

treatments may be effective in one disorder but not in others.

Because of the varied nature of mitochondrial diseases some

therapies may be helpful in many, but not in all patients and

therefore cannot be considered as " proven and effective. " Some

treatments should only be undertaken under the specific guidance of

your physician. For specific information about the controversy, as

it relates to your or your child's situation, ask your physician.

Most of these vitamins can be purchased from many sources, including

the drugstore.

These supplemental compounds can serve two functions:

POSSIBLY ENHANCE ENZYME FUNCTION AND RESULT IN IMPROVED EFFICIENCY

OF ENERGY GENERATION

SERVE AS ANTIOXIDANTS, WHICH MAY SLOW THE PROGRESSION OF THE DISEASE

---------------------------------------------------------------------

-----------

Vitamins and Supplements That May be Helpful

Consult your physician before starting any of the following possible

treatments

First Tier Supplements

Supplement

Dose Range

CoQ10

5 – 15 mg/kg/day

Levo-carnitine

(Carnitor)

Variable, starting dose of 30 mg/kg/day, typical maximum of 100

mg/kg/day

Riboflavin (B2)

100 – 400 mg a day

Second Tier Supplement

Supplement

Dose Range

Acetyl-L-Carnitine

250 – 1000 mg per day

Thiamine (B1)

50 – 100 mg a day

Nicotinamide (B3)

50 – 100 mg a day

Vitamin E

200 – 400 IU; 1 – 3 times a day

Vitamin C

100 – 500 mg; 1 – 3 times a day

Lipoic Acid

(a -lipoate)

60 – 200 mg; 3 times a day

Selenium

25 – 50 micrograms a day

b -carotene

10,000 IU; every other day to daily

Biotin

2.5 – 10 mg a day

Folic Acid

1 – 10 mg a day

---------------------------------------------------------------------

-----------

Medication, Minerals, Vitamins and Substrates that May be Helpful

Any use of the following medications, minerals, vitamins and

substrates MUST BE made only under a physician's direction

Supplement

Dose Range

Calcium

Variable

Magnesium

Variable

Phosphorus

Variable

Succinate

6 gm per day

Creatine

5 gm bid after initial load (adults)

Uridine

To be determined

Citrates

Variable

Prednisone

Variable

Vitamin K3 5-30 mg per day

---------------------------------------------------------------------

-----------

Avoidance of Physiologic " Stress "

Physiologic stress is triggered by external factors that may result

in worsening the metabolic situation, which may result in

temporary , or in sometimes, permanent worsening of the condition.

It is impossible to avoid all physiologic stressful conditions, so

one should not attempt to do so. However, recognizing what may be

stressful for patients allows one to adjust the lifestyle. Many

patients and their parents have already identified these stresses,

despite not knowing why the stresses were important, and avoid them.

Cold Stress is extremely important. Thermal regulation (temperature

control) is not always normal in people with mitochondrial diseases

and exposure to cold can result in severe heat loss and trigger an

energy crisis. When going out into the cold, all exposed body parts

should be covered, and exposure to extreme cold should be avoided

for anything more than a short period. Over bundling can be a

problem too (see below).

Heat Stress can be a problem in some people. This is especially true

of those with an inability to sweat normally. Heat exhaustion and

heat stroke may occur on hot days. It is typical for parents to

describe that their child seems to " wilt " in situations like hot

classrooms or direct sunlight, whereas the other children function

normally. Light clothing is important. Patients should avoid direct

sunlight on hot days and stay indoors if it is too warm outside. An

air-conditioned environment may be needed.

Starvation – avoid fasting.

Lack of sleep may possibly be harmful.

Except where noted, the above excerpts were taken, with permission,

from Mitochondrial Cytopathies: A Primer written by Dr. Bruce Cohen,

MD

---------------------------------------------------------------------

-----------

No Frames Navigation

[information Center] [Library] [Patient Registry] [Chapters &

Groups] [Events & Activities] [For Healthcare Professionals]

[Research & Grants] [Personal Journeys] [Resources] [About UMDF]

[Contact Us] [Join UMDF] [Make A Donation] [HOME]

Return to Frames Navigation

[Guest guest]

Tamara,

Here is the info on the umdf website for treatment of mito!

Treatment - At this time, there are no cures for these disorders.

Goals of treatment

(note: goals may never be met)

alleviate symptoms

slow down the progression of the disease

Effectiveness of treatment

varies from patient to patient, depending on the exact disorder and

the severity of the disorder

as a general rule, those with mild disorders tend to respond to

treatment better than those with severe disorders

in some circumstances, the treatment can be tailored specifically to

the patient, and that treatment is effective, whereas in other

circumstance, the treatment is " emperic " , meaning that the treatment

makes sense, but that the benefit of treatment is not obvious or

proven to be effective

treatment will not reverse the damage already sustained, such as

brain malformations

Benefits of Treatment and Effectiveness of Therapies Vary

treatment may be beneficial and noted immediately in some disorders

benefit of treatment may take a few months to notice

benefit of treatment may never be noticed, but the treatment may be

effective in delaying or stopping the progression of the disease

some patients may not benefit from therapy

Key Points to Treatment

Never forget there is standard treatment for some symptoms

(anticonvulsant medication for epilepsy, physical therapy for motor

problems, etc.)

Dietary

Vitamins and supplements

Avoidance of stressful factors

Treatment must be tailored by the patient's physician to meet that

patient's need. Many of these therapies are totally ineffective in

some mitochondrial disorders and would be a waste of time, money and

effort. In some cases, the treatment could be dangerous.

Specific Therapies & Things to Avoid

Dietary Therapy

Many patients, including young children or mentally impaired persons

have already " self-adjusted " their diet, because they know what

foods their body seem to tolerate. The points below are not meant to

be suggested therapies for all patients with OXPHOS disorders, and

some of the points are dangerous for patients with other disorders

(4b could be lethal in pyruvate dehydrogenase deficiency for

example). Do not make any of these dietary changes without

consulting a physician. A dietitian experienced in metabolic

disorders may be helpful.

Avoid fasting. This is perhaps the most important part of the

treatment for most people with metabolic disorders. Fasting

means " not eating " and avoiding fasting means avoid prolonged

periods without a meal (even an overnight " fast " from 8 pm to 8 am

may be dangerous in some patients). This also means that some

patients should not intentionally try to loose weight by decreasing

their food intake. In some patients an unintended fast resulting

from an illness that causes vomiting or loss of appetite (like the

flu) should be hospitalized to ensure continuous nutrition

(intraveneous glucose for example). In order to ensure adequate

frequent nutrition, sometimes a feeding tube needs to be placed in

order for the person to receive feeding at night. In some patients,

awakening them in the middle of the night for a snack can also be

helpful.

Small frequent meals may be better than a typical 3-meal-a-day

routine for some patients.

A snack before bedtime may be helpful in some patients. This snack

should not be mainly " sugar " , like a candy bar , jello or sweetened

cereal. It is usually best if the snack consists of a complex

carbohydrate. Cornstarch is the best complex carbohydrate, but this

is not very tasty. Theoretically, the best snack would be a homemade

low-sugar rice pudding thickened with a lot of cornstarch. If you

come up with a tasty recipe, let the UMDF know. Pasta, a peanut

butter sandwich, bread and butter, unsweetened cereal (oatmeal) or a

sandwich are acceptable. Many patients benefit by being woken up in

the middle of the night for a small meal and others clearly improve

when a gastrostomy tube is placed for continuous feeds. These final

two suggestions are a small price to pay for health.

a) There are conflicting lines of evidence regarding the use of high

fat meals in patients with electron transport chain disorders. In

patients that seem to gain weight and thrive on a high-fat diet, it

makes sense to continue the treatment. The extra fat can also be in

the form of MCT (medium chain triglyceride oil), which is easier to

metabolize (4c).

In other patients with OXPHOS disorders, reducing fat may be

helpful. This includes reducing added oil, butter, & margarine, and

cutting down on cheese and fatty meats. This recommendation is not

meant to avoid fats altogether. A defect in OXPHOS can create

an " energy backup " , as the respiratory chain cannot handle the flow

of electrons coming into it. This backup may result in the formation

of excess free fatty acids (fats waiting to be burned) , which can

poison the enzyme (adenosine nucleotide translocase) that exchanges

the low-energy ADP located outside the mitochondria for the high-

energy ATP formed at complex v. If you take the approach of limiting

fats, extra effort needs to be made to increase the total

carbohydrate (in the form of complex carbohydrates) in the diet.

c) In some patients (see #4a and #4b above), adding fat in the form

of medium chain triglycerides (MCT), may be helpful. Medium chain

triglycerides of 8 to 10 carbons long are easier to metabolize (turn

into energy) than the longer chain triglycerides (those with 12-18

carbons) because they do not require carnitine to be transported

into the mitochondria. MCT Oil@ is mainly made of 8 and 10 carbon

triglycerides and this type of oil does not occur in nature, but is

made from coconut oil. MCT Oil@ is made by the baby formula company

Mead-. It comes in quart bottles, available by prescription

and runs about $70 a quart. It can be added like oil over pasta and

rice. You can cook with it, but this is a light oil and burns

easily. The special rules are explained in a recipe book that you

can request from the pharmacist. Depending on the situation, a

patient may benefit from a few teaspoons to a few tablespoons a day.

There are oils sold in health food stores called " MCT Oil "

or " medium chain triglyceride oil " . These are much less expensive

($25 per quart), but make sure there is a certified analysis on the

label, stating that the vast majority of the oil is C-8 and C-1 0

(and not C-12 or higher) .

Iron generate free radicals under certain conditions, which is

especially bad in mitochondrial diseases because the free radicals

injure mitochondrial DNA and " poke holes " in the mitochondria,

making a bad problem worse. Therefore, excess iron is theoretically

harmful. In people with mitochondrial disease, there is no routine

need to give supplemental iron, nor is there a reason to eat foods

rich in iron, such as extra red meat, for the purpose of eating

foods rich in iron. This does not mean that the person should not

eat red meat, especially if they enjoy it. There is no reason to

take vitamins with added iron. There is the rare instance when iron

is needed, but this is not common. In addition, vitamin C enhances

the absorption of iron from the intestines, and vitamin C should not

be given around a meal rich in iron. This is important to remember

because some experts feel that vitamin C is a good antioxidant, and

also may be helpful in some disorders of OXPHOS.

---------------------------------------------------------------------

-----------

Avoidance of Toxins

Alcohol has been known to hasten the progression of some

mitochondrial disorders

Cigarette smoke, probably due to the carbon monoxide is known to

hasten the progression of some conditions. Remember that carbon

monoxide kills by inhibiting complex IV of OXPHOS, why make it

worse? Cigarette smoke will make it worse.

MSG (monosodium glutamate) has for years been known to cause

migraine headaches in otherwise healthy individuals, and may trigger

these events in susceptible people with mitochondrial diseases. MSG

is frequently added to Chinese (and other Asian) foods, and is also

found in high levels of dried and canned soups. Read the label and

avoid MSG if there is any sensitivity.

Vitamins and Cofactors

Vitamins and cofactors are compounds that are required in order for

the chemical reactions, which make energy, to run efficiently. By

definition, a cofactor can be made by the body, whereas a vitamin

cannot, and therefore must be eaten. For most people, a regular diet

contains all the vitamins one could possibly need and their bodies

can make as much of any specific cofactor that it needs. For those

with mitochondrial disorders, added vitamins and cofactors may be

useful. The use of supplemental vitamins and cofactors is largely

unproven and their use is therefore controversial in patients with

mitochondrial diseases. For disorders of OXPHOS, coenzyme Q10 is

considered as a generally accepted effective therapy, although it

may not ultimately be effective for an individual patient. Other

treatments may be effective in one disorder but not in others.

Because of the varied nature of mitochondrial diseases some

therapies may be helpful in many, but not in all patients and

therefore cannot be considered as " proven and effective. " Some

treatments should only be undertaken under the specific guidance of

your physician. For specific information about the controversy, as

it relates to your or your child's situation, ask your physician.

Most of these vitamins can be purchased from many sources, including

the drugstore.

These supplemental compounds can serve two functions:

POSSIBLY ENHANCE ENZYME FUNCTION AND RESULT IN IMPROVED EFFICIENCY

OF ENERGY GENERATION

SERVE AS ANTIOXIDANTS, WHICH MAY SLOW THE PROGRESSION OF THE DISEASE

---------------------------------------------------------------------

-----------

Vitamins and Supplements That May be Helpful

Consult your physician before starting any of the following possible

treatments

First Tier Supplements

Supplement

Dose Range

CoQ10

5 – 15 mg/kg/day

Levo-carnitine

(Carnitor)

Variable, starting dose of 30 mg/kg/day, typical maximum of 100

mg/kg/day

Riboflavin (B2)

100 – 400 mg a day

Second Tier Supplement

Supplement

Dose Range

Acetyl-L-Carnitine

250 – 1000 mg per day

Thiamine (B1)

50 – 100 mg a day

Nicotinamide (B3)

50 – 100 mg a day

Vitamin E

200 – 400 IU; 1 – 3 times a day

Vitamin C

100 – 500 mg; 1 – 3 times a day

Lipoic Acid

(a -lipoate)

60 – 200 mg; 3 times a day

Selenium

25 – 50 micrograms a day

b -carotene

10,000 IU; every other day to daily

Biotin

2.5 – 10 mg a day

Folic Acid

1 – 10 mg a day

---------------------------------------------------------------------

-----------

Medication, Minerals, Vitamins and Substrates that May be Helpful

Any use of the following medications, minerals, vitamins and

substrates MUST BE made only under a physician's direction

Supplement

Dose Range

Calcium

Variable

Magnesium

Variable

Phosphorus

Variable

Succinate

6 gm per day

Creatine

5 gm bid after initial load (adults)

Uridine

To be determined

Citrates

Variable

Prednisone

Variable

Vitamin K3 5-30 mg per day

---------------------------------------------------------------------

-----------

Avoidance of Physiologic " Stress "

Physiologic stress is triggered by external factors that may result

in worsening the metabolic situation, which may result in

temporary , or in sometimes, permanent worsening of the condition.

It is impossible to avoid all physiologic stressful conditions, so

one should not attempt to do so. However, recognizing what may be

stressful for patients allows one to adjust the lifestyle. Many

patients and their parents have already identified these stresses,

despite not knowing why the stresses were important, and avoid them.

Cold Stress is extremely important. Thermal regulation (temperature

control) is not always normal in people with mitochondrial diseases

and exposure to cold can result in severe heat loss and trigger an

energy crisis. When going out into the cold, all exposed body parts

should be covered, and exposure to extreme cold should be avoided

for anything more than a short period. Over bundling can be a

problem too (see below).

Heat Stress can be a problem in some people. This is especially true

of those with an inability to sweat normally. Heat exhaustion and

heat stroke may occur on hot days. It is typical for parents to

describe that their child seems to " wilt " in situations like hot

classrooms or direct sunlight, whereas the other children function

normally. Light clothing is important. Patients should avoid direct

sunlight on hot days and stay indoors if it is too warm outside. An

air-conditioned environment may be needed.

Starvation – avoid fasting.

Lack of sleep may possibly be harmful.

Except where noted, the above excerpts were taken, with permission,

from Mitochondrial Cytopathies: A Primer written by Dr. Bruce Cohen,

MD

---------------------------------------------------------------------

-----------

No Frames Navigation

[information Center] [Library] [Patient Registry] [Chapters &

Groups] [Events & Activities] [For Healthcare Professionals]

[Research & Grants] [Personal Journeys] [Resources] [About UMDF]

[Contact Us] [Join UMDF] [Make A Donation] [HOME]

Return to Frames Navigation

[Guest guest]

Tamara,

Here is the info on the umdf website for treatment of mito!

Treatment - At this time, there are no cures for these disorders.

Goals of treatment

(note: goals may never be met)

alleviate symptoms

slow down the progression of the disease

Effectiveness of treatment

varies from patient to patient, depending on the exact disorder and

the severity of the disorder

as a general rule, those with mild disorders tend to respond to

treatment better than those with severe disorders

in some circumstances, the treatment can be tailored specifically to

the patient, and that treatment is effective, whereas in other

circumstance, the treatment is " emperic " , meaning that the treatment

makes sense, but that the benefit of treatment is not obvious or

proven to be effective

treatment will not reverse the damage already sustained, such as

brain malformations

Benefits of Treatment and Effectiveness of Therapies Vary

treatment may be beneficial and noted immediately in some disorders

benefit of treatment may take a few months to notice

benefit of treatment may never be noticed, but the treatment may be

effective in delaying or stopping the progression of the disease

some patients may not benefit from therapy

Key Points to Treatment

Never forget there is standard treatment for some symptoms

(anticonvulsant medication for epilepsy, physical therapy for motor

problems, etc.)

Dietary

Vitamins and supplements

Avoidance of stressful factors

Treatment must be tailored by the patient's physician to meet that

patient's need. Many of these therapies are totally ineffective in

some mitochondrial disorders and would be a waste of time, money and

effort. In some cases, the treatment could be dangerous.

Specific Therapies & Things to Avoid

Dietary Therapy

Many patients, including young children or mentally impaired persons

have already " self-adjusted " their diet, because they know what

foods their body seem to tolerate. The points below are not meant to

be suggested therapies for all patients with OXPHOS disorders, and

some of the points are dangerous for patients with other disorders

(4b could be lethal in pyruvate dehydrogenase deficiency for

example). Do not make any of these dietary changes without

consulting a physician. A dietitian experienced in metabolic

disorders may be helpful.

Avoid fasting. This is perhaps the most important part of the

treatment for most people with metabolic disorders. Fasting

means " not eating " and avoiding fasting means avoid prolonged

periods without a meal (even an overnight " fast " from 8 pm to 8 am

may be dangerous in some patients). This also means that some

patients should not intentionally try to loose weight by decreasing

their food intake. In some patients an unintended fast resulting

from an illness that causes vomiting or loss of appetite (like the

flu) should be hospitalized to ensure continuous nutrition

(intraveneous glucose for example). In order to ensure adequate

frequent nutrition, sometimes a feeding tube needs to be placed in

order for the person to receive feeding at night. In some patients,

awakening them in the middle of the night for a snack can also be

helpful.

Small frequent meals may be better than a typical 3-meal-a-day

routine for some patients.

A snack before bedtime may be helpful in some patients. This snack

should not be mainly " sugar " , like a candy bar , jello or sweetened

cereal. It is usually best if the snack consists of a complex

carbohydrate. Cornstarch is the best complex carbohydrate, but this

is not very tasty. Theoretically, the best snack would be a homemade

low-sugar rice pudding thickened with a lot of cornstarch. If you

come up with a tasty recipe, let the UMDF know. Pasta, a peanut

butter sandwich, bread and butter, unsweetened cereal (oatmeal) or a

sandwich are acceptable. Many patients benefit by being woken up in

the middle of the night for a small meal and others clearly improve

when a gastrostomy tube is placed for continuous feeds. These final

two suggestions are a small price to pay for health.

a) There are conflicting lines of evidence regarding the use of high

fat meals in patients with electron transport chain disorders. In

patients that seem to gain weight and thrive on a high-fat diet, it

makes sense to continue the treatment. The extra fat can also be in

the form of MCT (medium chain triglyceride oil), which is easier to

metabolize (4c).

In other patients with OXPHOS disorders, reducing fat may be

helpful. This includes reducing added oil, butter, & margarine, and

cutting down on cheese and fatty meats. This recommendation is not

meant to avoid fats altogether. A defect in OXPHOS can create

an " energy backup " , as the respiratory chain cannot handle the flow

of electrons coming into it. This backup may result in the formation

of excess free fatty acids (fats waiting to be burned) , which can

poison the enzyme (adenosine nucleotide translocase) that exchanges

the low-energy ADP located outside the mitochondria for the high-

energy ATP formed at complex v. If you take the approach of limiting

fats, extra effort needs to be made to increase the total

carbohydrate (in the form of complex carbohydrates) in the diet.

c) In some patients (see #4a and #4b above), adding fat in the form

of medium chain triglycerides (MCT), may be helpful. Medium chain

triglycerides of 8 to 10 carbons long are easier to metabolize (turn

into energy) than the longer chain triglycerides (those with 12-18

carbons) because they do not require carnitine to be transported

into the mitochondria. MCT Oil@ is mainly made of 8 and 10 carbon

triglycerides and this type of oil does not occur in nature, but is

made from coconut oil. MCT Oil@ is made by the baby formula company

Mead-. It comes in quart bottles, available by prescription

and runs about $70 a quart. It can be added like oil over pasta and

rice. You can cook with it, but this is a light oil and burns

easily. The special rules are explained in a recipe book that you

can request from the pharmacist. Depending on the situation, a

patient may benefit from a few teaspoons to a few tablespoons a day.

There are oils sold in health food stores called " MCT Oil "

or " medium chain triglyceride oil " . These are much less expensive

($25 per quart), but make sure there is a certified analysis on the

label, stating that the vast majority of the oil is C-8 and C-1 0

(and not C-12 or higher) .

Iron generate free radicals under certain conditions, which is

especially bad in mitochondrial diseases because the free radicals

injure mitochondrial DNA and " poke holes " in the mitochondria,

making a bad problem worse. Therefore, excess iron is theoretically

harmful. In people with mitochondrial disease, there is no routine

need to give supplemental iron, nor is there a reason to eat foods

rich in iron, such as extra red meat, for the purpose of eating

foods rich in iron. This does not mean that the person should not

eat red meat, especially if they enjoy it. There is no reason to

take vitamins with added iron. There is the rare instance when iron

is needed, but this is not common. In addition, vitamin C enhances

the absorption of iron from the intestines, and vitamin C should not

be given around a meal rich in iron. This is important to remember

because some experts feel that vitamin C is a good antioxidant, and

also may be helpful in some disorders of OXPHOS.

---------------------------------------------------------------------

-----------

Avoidance of Toxins

Alcohol has been known to hasten the progression of some

mitochondrial disorders

Cigarette smoke, probably due to the carbon monoxide is known to

hasten the progression of some conditions. Remember that carbon

monoxide kills by inhibiting complex IV of OXPHOS, why make it

worse? Cigarette smoke will make it worse.

MSG (monosodium glutamate) has for years been known to cause

migraine headaches in otherwise healthy individuals, and may trigger

these events in susceptible people with mitochondrial diseases. MSG

is frequently added to Chinese (and other Asian) foods, and is also

found in high levels of dried and canned soups. Read the label and

avoid MSG if there is any sensitivity.

Vitamins and Cofactors

Vitamins and cofactors are compounds that are required in order for

the chemical reactions, which make energy, to run efficiently. By

definition, a cofactor can be made by the body, whereas a vitamin

cannot, and therefore must be eaten. For most people, a regular diet

contains all the vitamins one could possibly need and their bodies

can make as much of any specific cofactor that it needs. For those

with mitochondrial disorders, added vitamins and cofactors may be

useful. The use of supplemental vitamins and cofactors is largely

unproven and their use is therefore controversial in patients with

mitochondrial diseases. For disorders of OXPHOS, coenzyme Q10 is

considered as a generally accepted effective therapy, although it

may not ultimately be effective for an individual patient. Other

treatments may be effective in one disorder but not in others.

Because of the varied nature of mitochondrial diseases some

therapies may be helpful in many, but not in all patients and

therefore cannot be considered as " proven and effective. " Some

treatments should only be undertaken under the specific guidance of

your physician. For specific information about the controversy, as

it relates to your or your child's situation, ask your physician.

Most of these vitamins can be purchased from many sources, including

the drugstore.

These supplemental compounds can serve two functions:

POSSIBLY ENHANCE ENZYME FUNCTION AND RESULT IN IMPROVED EFFICIENCY

OF ENERGY GENERATION

SERVE AS ANTIOXIDANTS, WHICH MAY SLOW THE PROGRESSION OF THE DISEASE

---------------------------------------------------------------------

-----------

Vitamins and Supplements That May be Helpful

Consult your physician before starting any of the following possible

treatments

First Tier Supplements

Supplement

Dose Range

CoQ10

5 – 15 mg/kg/day

Levo-carnitine

(Carnitor)

Variable, starting dose of 30 mg/kg/day, typical maximum of 100

mg/kg/day

Riboflavin (B2)

100 – 400 mg a day

Second Tier Supplement

Supplement

Dose Range

Acetyl-L-Carnitine

250 – 1000 mg per day

Thiamine (B1)

50 – 100 mg a day

Nicotinamide (B3)

50 – 100 mg a day

Vitamin E

200 – 400 IU; 1 – 3 times a day

Vitamin C

100 – 500 mg; 1 – 3 times a day

Lipoic Acid

(a -lipoate)

60 – 200 mg; 3 times a day

Selenium

25 – 50 micrograms a day

b -carotene

10,000 IU; every other day to daily

Biotin

2.5 – 10 mg a day

Folic Acid

1 – 10 mg a day

---------------------------------------------------------------------

-----------

Medication, Minerals, Vitamins and Substrates that May be Helpful

Any use of the following medications, minerals, vitamins and

substrates MUST BE made only under a physician's direction

Supplement

Dose Range

Calcium

Variable

Magnesium

Variable

Phosphorus

Variable

Succinate

6 gm per day

Creatine

5 gm bid after initial load (adults)

Uridine

To be determined

Citrates

Variable

Prednisone

Variable

Vitamin K3 5-30 mg per day

---------------------------------------------------------------------

-----------

Avoidance of Physiologic " Stress "

Physiologic stress is triggered by external factors that may result

in worsening the metabolic situation, which may result in

temporary , or in sometimes, permanent worsening of the condition.

It is impossible to avoid all physiologic stressful conditions, so

one should not attempt to do so. However, recognizing what may be

stressful for patients allows one to adjust the lifestyle. Many

patients and their parents have already identified these stresses,

despite not knowing why the stresses were important, and avoid them.

Cold Stress is extremely important. Thermal regulation (temperature

control) is not always normal in people with mitochondrial diseases

and exposure to cold can result in severe heat loss and trigger an

energy crisis. When going out into the cold, all exposed body parts

should be covered, and exposure to extreme cold should be avoided

for anything more than a short period. Over bundling can be a

problem too (see below).

Heat Stress can be a problem in some people. This is especially true

of those with an inability to sweat normally. Heat exhaustion and

heat stroke may occur on hot days. It is typical for parents to

describe that their child seems to " wilt " in situations like hot

classrooms or direct sunlight, whereas the other children function

normally. Light clothing is important. Patients should avoid direct

sunlight on hot days and stay indoors if it is too warm outside. An

air-conditioned environment may be needed.

Starvation – avoid fasting.

Lack of sleep may possibly be harmful.

Except where noted, the above excerpts were taken, with permission,

from Mitochondrial Cytopathies: A Primer written by Dr. Bruce Cohen,

MD

---------------------------------------------------------------------

-----------

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[Guest guest]

Have you seen the Woman's Day article on mito? It is in the Apr 15th

issue with the Easter eggs on the cover. The little boy featured in

the article responded very well to the cocktail. His story sounded so

much like my Evan's. Before starting the cocktail,

Evan's fatigue was so severe that he couldn't play at the park without

literally falling asleep after a short time. His loss of muscle tone

and coordination was huge. He went from being a very robust three year

old to looking as if he had CP when he was 6 or 7. He was no longer

able to tie his shoes or write legibly. He was ataxic and very

weak.

When Dr. Kelley saw the photos he was amazed. Evan's local neuro (not

his current neuro) said that not all kids are strong, and he felt that

Evan's poor results on strength testing might have been from lack of

effort (which he attributed to Evan's ASD diagnosis). Evan's energy

level is so much better now, and his weight gain has been good for the

past two years after starting carnitor, nighttime carb loading, and IV

glutathione. We do have to be very

careful when Evan is sick though. I thought he was OK when we

increased

his

carnitor and pushed gatorade, but it has taken him a

whole

month to bounce back from strep throat. He was very off

neurologically. I think that I will push for IV support next time. I

read

somewhere that each

degree rise in temperature above 98.6 increases energy demands by about

10 percent. It is easy to see how our kids run into problems quickly

with only moderate fevers.

[Guest guest]

Have you seen the Woman's Day article on mito? It is in the Apr 15th

issue with the Easter eggs on the cover. The little boy featured in

the article responded very well to the cocktail. His story sounded so

much like my Evan's. Before starting the cocktail,

Evan's fatigue was so severe that he couldn't play at the park without

literally falling asleep after a short time. His loss of muscle tone

and coordination was huge. He went from being a very robust three year

old to looking as if he had CP when he was 6 or 7. He was no longer

able to tie his shoes or write legibly. He was ataxic and very

weak.

When Dr. Kelley saw the photos he was amazed. Evan's local neuro (not

his current neuro) said that not all kids are strong, and he felt that

Evan's poor results on strength testing might have been from lack of

effort (which he attributed to Evan's ASD diagnosis). Evan's energy

level is so much better now, and his weight gain has been good for the

past two years after starting carnitor, nighttime carb loading, and IV

glutathione. We do have to be very

careful when Evan is sick though. I thought he was OK when we

increased

his

carnitor and pushed gatorade, but it has taken him a

whole

month to bounce back from strep throat. He was very off

neurologically. I think that I will push for IV support next time. I

read

somewhere that each

degree rise in temperature above 98.6 increases energy demands by about

10 percent. It is easy to see how our kids run into problems quickly

with only moderate fevers.

[Guest guest]

Have you seen the Woman's Day article on mito? It is in the Apr 15th

issue with the Easter eggs on the cover. The little boy featured in

the article responded very well to the cocktail. His story sounded so

much like my Evan's. Before starting the cocktail,

Evan's fatigue was so severe that he couldn't play at the park without

literally falling asleep after a short time. His loss of muscle tone

and coordination was huge. He went from being a very robust three year

old to looking as if he had CP when he was 6 or 7. He was no longer

able to tie his shoes or write legibly. He was ataxic and very

weak.

When Dr. Kelley saw the photos he was amazed. Evan's local neuro (not

his current neuro) said that not all kids are strong, and he felt that

Evan's poor results on strength testing might have been from lack of

effort (which he attributed to Evan's ASD diagnosis). Evan's energy

level is so much better now, and his weight gain has been good for the

past two years after starting carnitor, nighttime carb loading, and IV

glutathione. We do have to be very

careful when Evan is sick though. I thought he was OK when we

increased

his

carnitor and pushed gatorade, but it has taken him a

whole

month to bounce back from strep throat. He was very off

neurologically. I think that I will push for IV support next time. I

read

somewhere that each

degree rise in temperature above 98.6 increases energy demands by about

10 percent. It is easy to see how our kids run into problems quickly

with only moderate fevers.

[Guest guest]

Just to let you know that 2 of the 3 drugs

in the Mito Cocktail are over the counter vitamins! You can try them yourself.

I would run this by at least one or your doctors to get the correct dose.

(Co-Q10 and Vitamin B-2)

Sue

Mito Cocktail?

Doctor said it has no value!!!!!

Our Nuro doctor finally called back this

morning. He said the Mito

Cocktail has no value and just a waste of

money. That is very

frustrating!!!!! is getting weaker and

we really wanted to try

it!!!! What resources can I use to convince

the doctor to at least

consider it? What is a good web sight to get

on to read about it?

Any help would be great! What I would do for

a Mito doc right now!!!

Tamara(Mommy of age 5, unspecific Mito)

Please contact

mito-owner with any problems or questions.

[Guest guest]

Just to let you know that 2 of the 3 drugs

in the Mito Cocktail are over the counter vitamins! You can try them yourself.

I would run this by at least one or your doctors to get the correct dose.

(Co-Q10 and Vitamin B-2)

Sue

Mito Cocktail?

Doctor said it has no value!!!!!

Our Nuro doctor finally called back this

morning. He said the Mito

Cocktail has no value and just a waste of

money. That is very

frustrating!!!!! is getting weaker and

we really wanted to try

it!!!! What resources can I use to convince

the doctor to at least

consider it? What is a good web sight to get

on to read about it?

Any help would be great! What I would do for

a Mito doc right now!!!

Tamara(Mommy of age 5, unspecific Mito)

Please contact

mito-owner with any problems or questions.

[Guest guest]

Just to let you know that 2 of the 3 drugs

in the Mito Cocktail are over the counter vitamins! You can try them yourself.

I would run this by at least one or your doctors to get the correct dose.

(Co-Q10 and Vitamin B-2)

Sue

Mito Cocktail?

Doctor said it has no value!!!!!

Our Nuro doctor finally called back this

morning. He said the Mito

Cocktail has no value and just a waste of

money. That is very

frustrating!!!!! is getting weaker and

we really wanted to try

it!!!! What resources can I use to convince

the doctor to at least

consider it? What is a good web sight to get

on to read about it?

Any help would be great! What I would do for

a Mito doc right now!!!

Tamara(Mommy of age 5, unspecific Mito)

Please contact

mito-owner with any problems or questions.

[Guest guest]

I agree with Dawn. Find a new doc. The one thing my neuro said was to take

Leah off all supplements and start her back slowly on one and then take her

off and try another to see which one benefited her the best. But we

(including him)decided not to do that because of the gains she had made at

that time. Our metabolic doc said that they aren't sure which supplements

work the best with each child, but it doesn't hurt to try. We have seen

great things with Leah. I guess it is similiar to us taking vitamins and

other supplements. we could try and get them through eating but we can't;

look how they want us to take calicum supplements for woman because we may

not get it through our milk and a few other products. Our children with mito

can't get all of their vitamins from the food they eat because some have

difficulty eating and won't get all the proper energy supplements that way.

I guess I would like to know why he says that. Why does he think it's a

waste of money? Do not give up is right. Do your research and find out what

approiate doses to give your child and try. You may be surprised and prove

that doctor wrong.

Nerenhausen

mom to Leah

thefiveofus02 wrote:

> Tamara,

>

> Oh boy! You should get alot of responses to this one. If it

> weren't for the mito cocktail, I am sure my daughter would not be

> alive today! Go to the UMDF.org website and go to the resources page

> (I think). It has a section called treatment of mito disorders.

> Forget you're doc and I would at least start him on the coq10, b

> vit, vit e etc. These can all be gotten without a script. Find a new

> doc. I have never heard of such a thing. This really ------ me off.

> Is this a doc who know's anything at all about mito? I would find a

> new doc if I were you. Do not give up, which seems like what he is

> saying. Good luck,

>

> Dawn

>

> Please contact mito-owner with any problems or questions.

>

[Guest guest]

I agree with Dawn. Find a new doc. The one thing my neuro said was to take

Leah off all supplements and start her back slowly on one and then take her

off and try another to see which one benefited her the best. But we

(including him)decided not to do that because of the gains she had made at

that time. Our metabolic doc said that they aren't sure which supplements

work the best with each child, but it doesn't hurt to try. We have seen

great things with Leah. I guess it is similiar to us taking vitamins and

other supplements. we could try and get them through eating but we can't;

look how they want us to take calicum supplements for woman because we may

not get it through our milk and a few other products. Our children with mito

can't get all of their vitamins from the food they eat because some have

difficulty eating and won't get all the proper energy supplements that way.

I guess I would like to know why he says that. Why does he think it's a

waste of money? Do not give up is right. Do your research and find out what

approiate doses to give your child and try. You may be surprised and prove

that doctor wrong.

Nerenhausen

mom to Leah

thefiveofus02 wrote:

> Tamara,

>

> Oh boy! You should get alot of responses to this one. If it

> weren't for the mito cocktail, I am sure my daughter would not be

> alive today! Go to the UMDF.org website and go to the resources page

> (I think). It has a section called treatment of mito disorders.

> Forget you're doc and I would at least start him on the coq10, b

> vit, vit e etc. These can all be gotten without a script. Find a new

> doc. I have never heard of such a thing. This really ------ me off.

> Is this a doc who know's anything at all about mito? I would find a

> new doc if I were you. Do not give up, which seems like what he is

> saying. Good luck,

>

> Dawn

>

> Please contact mito-owner with any problems or questions.

>

[Guest guest]

I agree with Dawn. Find a new doc. The one thing my neuro said was to take

Leah off all supplements and start her back slowly on one and then take her

off and try another to see which one benefited her the best. But we

(including him)decided not to do that because of the gains she had made at

that time. Our metabolic doc said that they aren't sure which supplements

work the best with each child, but it doesn't hurt to try. We have seen

great things with Leah. I guess it is similiar to us taking vitamins and

other supplements. we could try and get them through eating but we can't;

look how they want us to take calicum supplements for woman because we may

not get it through our milk and a few other products. Our children with mito

can't get all of their vitamins from the food they eat because some have

difficulty eating and won't get all the proper energy supplements that way.

I guess I would like to know why he says that. Why does he think it's a

waste of money? Do not give up is right. Do your research and find out what

approiate doses to give your child and try. You may be surprised and prove

that doctor wrong.

Nerenhausen

mom to Leah

thefiveofus02 wrote:

> Tamara,

>

> Oh boy! You should get alot of responses to this one. If it

> weren't for the mito cocktail, I am sure my daughter would not be

> alive today! Go to the UMDF.org website and go to the resources page

> (I think). It has a section called treatment of mito disorders.

> Forget you're doc and I would at least start him on the coq10, b

> vit, vit e etc. These can all be gotten without a script. Find a new

> doc. I have never heard of such a thing. This really ------ me off.

> Is this a doc who know's anything at all about mito? I would find a

> new doc if I were you. Do not give up, which seems like what he is

> saying. Good luck,

>

> Dawn

>

> Please contact mito-owner with any problems or questions.

>