Mitochondrial Respiratory Chain and Fatty Oxidation Disorder

[Guest guest]

Hi, my name is Tina and I am new to the group. I have a 3year old son who until recently was diagnosed with a Fatty Oxidation Disorder (SCAD to be specific) and have recently been told that he has a respiratory chain disorder with the fatty oxidation being secondary. I was wondering if anyone's child has a dual diagnosis of FOD and Mito. I understand the diets are the total opposite. I have been feeding him and high carb, low fat diet and now I am told that the high carb could actually hurt him. They are running two more test on his skin to see if they can get a clear diagnosis and until then they want me to normalize his fat intake to about 35% and lower his carbs. They tell me the Mito needs to be high fat, low carb. I don't feel comfortable giving him a fat intake of 35% when he is now at around 20-25%. When we had increased to about 30% at one time I did see a change. His urine has a funny smell, the fatty acid (EMA) is increased. For his symptoms now he has muscle weakness, brain calcifications, delayed myelin and loss of myelin, apnea (which is my biggest concern, he is still hooked up to an apnea/heart monitor at 3years old)(Anyone else's child have apnea?) Any suggestions as far as the diet with Mito kids would be helpful also what is the protocol for when they become sick, won't eat, etc. I always had an emergency letter and glucose IV needed to be started is it the same for the respiratory chain disorders?

Thanks for listening

Tina

Mom to 3yrs (Mito/SCAD)

[Guest guest]

You know Darla I get conflicting answers on whether or not the EMA smells in the urine. Some of the doctors say they don't know distinctly that it causing an odor but who knows' maybe it does. I feel it has something to do with it because his urine doesn't smell all the time and when it does is when the EMA level is elevated. I know it's not the carnitor because he just started taking carnitor on the 17th of February. So all the urines I have smelled and checked were before the carnitor was started. I do find it hard to check the smelling now that he is trained, or maybe he just hasn't had a really bad odor since he has been trained. Is your daughter trained? Do you smell it even without being in a diaper. I always worry I will miss the smell and the chance to get his urine tested and know that something is going on now that he is toilet trained. You know along with the apnea has increased heart rate, trouble regulating his body temperature (runs fevers for no apparent reason sometimes only slight but sometimes 105) Do they say that Asenath will be on the monitor her whole life? Well tell her Happy Birthday from me and I'll talk to you soon

Tina

Mommy to AJ (MITO/SCAD)

(unaffected as far as we know)

Mitochondrial Respiratory Chain and Fatty Oxidation Disorder

Hi, my name is Tina and I am new to the group. I have a 3year old son who until recently was diagnosed with a Fatty Oxidation Disorder (SCAD to be specific) and have recently been told that he has a respiratory chain disorder with the fatty oxidation being secondary. I was wondering ! if anyone's child has a dual diagnosis of FOD and Mito. I unders tand the diets are the total opposite. I have been feeding him and high carb, low fat diet and now I am told that the high carb could actually hurt him. They are running two more test on his skin to see if they can get a clear diagnosis and until then they want me to normalize his fat intake to about 35% and lower his carbs. They tell me the Mito needs to be high fat, low carb. I don't feel comfortable giving him a fat intake of 35% when he is now at around 20-25%. When we had increased to about 30% at one time I did see a change. His urine has a funny smell, the fatty acid (EMA) is increased. For his symptoms now he has muscle weakness, brain calcifications, delayed myelin and loss of myelin, apnea (which is my biggest concern, he is still hooked up to an apnea/heart monitor at 3years old)(Anyone else's child have apnea?) Any suggestions as far as the diet with Mito kids would be helpful also what is the protocol for when they! become sick, won't eat, etc. I always had an emergency letter and glucose IV needed to be started is it the same for the respiratory chain disorders?

Thanks for listening

Tina

Mom to 3yrs (Mito/SCAD)Please contact mito-owner with any problems or questions. Please contact mito-owner with any problems or questions.

Get more from the Web. FREE MSN Explorer download : http://explorer.msn.com

[Guest guest]

You know Darla I get conflicting answers on whether or not the EMA smells in the urine. Some of the doctors say they don't know distinctly that it causing an odor but who knows' maybe it does. I feel it has something to do with it because his urine doesn't smell all the time and when it does is when the EMA level is elevated. I know it's not the carnitor because he just started taking carnitor on the 17th of February. So all the urines I have smelled and checked were before the carnitor was started. I do find it hard to check the smelling now that he is trained, or maybe he just hasn't had a really bad odor since he has been trained. Is your daughter trained? Do you smell it even without being in a diaper. I always worry I will miss the smell and the chance to get his urine tested and know that something is going on now that he is toilet trained. You know along with the apnea has increased heart rate, trouble regulating his body temperature (runs fevers for no apparent reason sometimes only slight but sometimes 105) Do they say that Asenath will be on the monitor her whole life? Well tell her Happy Birthday from me and I'll talk to you soon

Tina

Mommy to AJ (MITO/SCAD)

(unaffected as far as we know)

Mitochondrial Respiratory Chain and Fatty Oxidation Disorder

Hi, my name is Tina and I am new to the group. I have a 3year old son who until recently was diagnosed with a Fatty Oxidation Disorder (SCAD to be specific) and have recently been told that he has a respiratory chain disorder with the fatty oxidation being secondary. I was wondering ! if anyone's child has a dual diagnosis of FOD and Mito. I unders tand the diets are the total opposite. I have been feeding him and high carb, low fat diet and now I am told that the high carb could actually hurt him. They are running two more test on his skin to see if they can get a clear diagnosis and until then they want me to normalize his fat intake to about 35% and lower his carbs. They tell me the Mito needs to be high fat, low carb. I don't feel comfortable giving him a fat intake of 35% when he is now at around 20-25%. When we had increased to about 30% at one time I did see a change. His urine has a funny smell, the fatty acid (EMA) is increased. For his symptoms now he has muscle weakness, brain calcifications, delayed myelin and loss of myelin, apnea (which is my biggest concern, he is still hooked up to an apnea/heart monitor at 3years old)(Anyone else's child have apnea?) Any suggestions as far as the diet with Mito kids would be helpful also what is the protocol for when they! become sick, won't eat, etc. I always had an emergency letter and glucose IV needed to be started is it the same for the respiratory chain disorders?

Thanks for listening

Tina

Mom to 3yrs (Mito/SCAD)Please contact mito-owner with any problems or questions. Please contact mito-owner with any problems or questions.

Get more from the Web. FREE MSN Explorer download : http://explorer.msn.com

[Guest guest]

You know Darla I get conflicting answers on whether or not the EMA smells in the urine. Some of the doctors say they don't know distinctly that it causing an odor but who knows' maybe it does. I feel it has something to do with it because his urine doesn't smell all the time and when it does is when the EMA level is elevated. I know it's not the carnitor because he just started taking carnitor on the 17th of February. So all the urines I have smelled and checked were before the carnitor was started. I do find it hard to check the smelling now that he is trained, or maybe he just hasn't had a really bad odor since he has been trained. Is your daughter trained? Do you smell it even without being in a diaper. I always worry I will miss the smell and the chance to get his urine tested and know that something is going on now that he is toilet trained. You know along with the apnea has increased heart rate, trouble regulating his body temperature (runs fevers for no apparent reason sometimes only slight but sometimes 105) Do they say that Asenath will be on the monitor her whole life? Well tell her Happy Birthday from me and I'll talk to you soon

Tina

Mommy to AJ (MITO/SCAD)

(unaffected as far as we know)

Mitochondrial Respiratory Chain and Fatty Oxidation Disorder

Hi, my name is Tina and I am new to the group. I have a 3year old son who until recently was diagnosed with a Fatty Oxidation Disorder (SCAD to be specific) and have recently been told that he has a respiratory chain disorder with the fatty oxidation being secondary. I was wondering ! if anyone's child has a dual diagnosis of FOD and Mito. I unders tand the diets are the total opposite. I have been feeding him and high carb, low fat diet and now I am told that the high carb could actually hurt him. They are running two more test on his skin to see if they can get a clear diagnosis and until then they want me to normalize his fat intake to about 35% and lower his carbs. They tell me the Mito needs to be high fat, low carb. I don't feel comfortable giving him a fat intake of 35% when he is now at around 20-25%. When we had increased to about 30% at one time I did see a change. His urine has a funny smell, the fatty acid (EMA) is increased. For his symptoms now he has muscle weakness, brain calcifications, delayed myelin and loss of myelin, apnea (which is my biggest concern, he is still hooked up to an apnea/heart monitor at 3years old)(Anyone else's child have apnea?) Any suggestions as far as the diet with Mito kids would be helpful also what is the protocol for when they! become sick, won't eat, etc. I always had an emergency letter and glucose IV needed to be started is it the same for the respiratory chain disorders?

Thanks for listening

Tina

Mom to 3yrs (Mito/SCAD)Please contact mito-owner with any problems or questions. Please contact mito-owner with any problems or questions.

Get more from the Web. FREE MSN Explorer download : http://explorer.msn.com