Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 Hi all, Does anyone have a fatty acid oxidation disorder secondary to the mitochondrial disorder? I just got a call from my metabolic doc's NP and she said there were abnormalities on my skin biopsy that are pointing in that direction. They aren't sure what kind of fatty acid problem it is yet, but it may be a medium chain disorder. Hopefully we'll know more next week. Just wondering if anyone else has this as a secondary mito problem--if it means progression of disease and whether there were any changes in daily management? I get all my fat intravenously so I'm not sure I can change my dietary needs too much. I'm seeing the mito doc on Thursday so am trying to get my questions lined up before I go. Thanks! Malisa Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 Hi Malissa, You might want to check out the transcript of Dr. Korson's mito chat that Alice posted recently. In answer to a question about a baby with a partial deficiency in complex I and a suspected fatty acid oxidation disorder (elevated long-chain fatty acids/carnitine deficiency), Dr. Korson said that complex I defects are often associated with fatty acid oxidation impairment. He says the approach to management may not change much if the FAO is confirmed, but it might help point toward changes in vitamin or cofactor therapy. Dr. Naviaux lists riboflavin and Carnitor as the cofactors of choice for FAOs. I can tell you from experience (as a person with both mito and FAO) that both of those cofactors gave me major improvement, though with different groups of symptoms. Years back I interviewed a patient with partial complex I def, partial carnitine def, and partial CPT def. His brother carried one CPT mutation but he did not. In his case, it was not known which defect was primary and which secondary. He responded dramatically to Carnitor. A few months back, I found an article for about a case of complex II deficiency with secondary FAO. That abstract stated that more and more mito cases with secondary FAOs are being reported. That post would be in the archives somewhere. (I posted the entire abstract.) From my rudimentary understanding of the biochemistry involved, I do not believe that the presence of an FAO necessarily means progression. My understanding is that secondary defects are probably present from the start due to biochemical " domino effects. " But I'm guessing that mito science has not confirmed this as fact, given the infancy of the field. I'm sure Dr. Cohen would know. BTW, it is possible for one person to have two primary disorders, one mito, one FAO. But of course, this can only be confirmed by mutation screening. This is all I can offer off the top of my head. Barbara > Hi all, > Does anyone have a fatty acid oxidation disorder secondary to the > mitochondrial disorder? I just got a call from my metabolic doc's NP and she said there > were abnormalities on my skin biopsy that are pointing in that direction. > They aren't sure what kind of fatty acid problem it is yet, but it may be a > medium chain disorder. Hopefully we'll know more next week. > > Just wondering if anyone else has this as a secondary mito problem- -if it > means progression of disease and whether there were any changes in daily > management? I get all my fat intravenously so I'm not sure I can change my dietary > needs too much. > I'm seeing the mito doc on Thursday so am trying to get my questions lined > up before I go. > Thanks! > Malisa > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 Hi Malissa, You might want to check out the transcript of Dr. Korson's mito chat that Alice posted recently. In answer to a question about a baby with a partial deficiency in complex I and a suspected fatty acid oxidation disorder (elevated long-chain fatty acids/carnitine deficiency), Dr. Korson said that complex I defects are often associated with fatty acid oxidation impairment. He says the approach to management may not change much if the FAO is confirmed, but it might help point toward changes in vitamin or cofactor therapy. Dr. Naviaux lists riboflavin and Carnitor as the cofactors of choice for FAOs. I can tell you from experience (as a person with both mito and FAO) that both of those cofactors gave me major improvement, though with different groups of symptoms. Years back I interviewed a patient with partial complex I def, partial carnitine def, and partial CPT def. His brother carried one CPT mutation but he did not. In his case, it was not known which defect was primary and which secondary. He responded dramatically to Carnitor. A few months back, I found an article for about a case of complex II deficiency with secondary FAO. That abstract stated that more and more mito cases with secondary FAOs are being reported. That post would be in the archives somewhere. (I posted the entire abstract.) From my rudimentary understanding of the biochemistry involved, I do not believe that the presence of an FAO necessarily means progression. My understanding is that secondary defects are probably present from the start due to biochemical " domino effects. " But I'm guessing that mito science has not confirmed this as fact, given the infancy of the field. I'm sure Dr. Cohen would know. BTW, it is possible for one person to have two primary disorders, one mito, one FAO. But of course, this can only be confirmed by mutation screening. This is all I can offer off the top of my head. Barbara > Hi all, > Does anyone have a fatty acid oxidation disorder secondary to the > mitochondrial disorder? I just got a call from my metabolic doc's NP and she said there > were abnormalities on my skin biopsy that are pointing in that direction. > They aren't sure what kind of fatty acid problem it is yet, but it may be a > medium chain disorder. Hopefully we'll know more next week. > > Just wondering if anyone else has this as a secondary mito problem- -if it > means progression of disease and whether there were any changes in daily > management? I get all my fat intravenously so I'm not sure I can change my dietary > needs too much. > I'm seeing the mito doc on Thursday so am trying to get my questions lined > up before I go. > Thanks! > Malisa > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 Barbara, The " top of your head " is much better than my " whole " head. Or should that be " hole " head? wheatchild2 wrote: >This is all I can offer off the top of my head. >Barbara > > > > > > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 Barbara, The " top of your head " is much better than my " whole " head. Or should that be " hole " head? wheatchild2 wrote: >This is all I can offer off the top of my head. >Barbara > > > > > > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 Barbara, The " top of your head " is much better than my " whole " head. Or should that be " hole " head? wheatchild2 wrote: >This is all I can offer off the top of my head. >Barbara > > > > > > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 I'm sitting here laughing because that was exactly what I was thinking. Alice wheatchild2 wrote: >This is all I can offer off the top of my head. >Barbara> Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 I'm sitting here laughing because that was exactly what I was thinking. Alice wheatchild2 wrote: >This is all I can offer off the top of my head. >Barbara> Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 I'm sitting here laughing because that was exactly what I was thinking. Alice wheatchild2 wrote: >This is all I can offer off the top of my head. >Barbara> Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 Malisa, We have this with and I know a few other online with this with their children (Zach and Sam and Caden and Carsen) so it is not uncommon. Most of what we do is what you do already because of your blood sugar issues. We never let her be in a fasting state. This includes a tube feed in the middle of the night. IV fluids with any vomiting or diarrhea. low fat diet. Let me know if I can help. It sound like they are finally finding some answers to your questions! I hate to say congrats, that sounds so stupid...how about, it's about time! Dawn Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 Malisa, We have this with and I know a few other online with this with their children (Zach and Sam and Caden and Carsen) so it is not uncommon. Most of what we do is what you do already because of your blood sugar issues. We never let her be in a fasting state. This includes a tube feed in the middle of the night. IV fluids with any vomiting or diarrhea. low fat diet. Let me know if I can help. It sound like they are finally finding some answers to your questions! I hate to say congrats, that sounds so stupid...how about, it's about time! Dawn Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 Malisa, We have this with and I know a few other online with this with their children (Zach and Sam and Caden and Carsen) so it is not uncommon. Most of what we do is what you do already because of your blood sugar issues. We never let her be in a fasting state. This includes a tube feed in the middle of the night. IV fluids with any vomiting or diarrhea. low fat diet. Let me know if I can help. It sound like they are finally finding some answers to your questions! I hate to say congrats, that sounds so stupid...how about, it's about time! Dawn Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 me too! Re: Re: Fatty acid issues & mito > > I'm sitting here laughing because that was exactly what I was thinking. > > Alice > > > The " top of your head " is much better than my " whole " head. Or should > that be " hole " head? > > > > > wheatchild2 wrote: > > >This is all I can offer off the top of my head. > >Barbara> > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 me too! Re: Re: Fatty acid issues & mito > > I'm sitting here laughing because that was exactly what I was thinking. > > Alice > > > The " top of your head " is much better than my " whole " head. Or should > that be " hole " head? > > > > > wheatchild2 wrote: > > >This is all I can offer off the top of my head. > >Barbara> > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 me too! Re: Re: Fatty acid issues & mito > > I'm sitting here laughing because that was exactly what I was thinking. > > Alice > > > The " top of your head " is much better than my " whole " head. Or should > that be " hole " head? > > > > > wheatchild2 wrote: > > >This is all I can offer off the top of my head. > >Barbara> > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 Good info here too: http://www.fodsupport.org/ Dawn Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 Good info here too: http://www.fodsupport.org/ Dawn Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 Good info here too: http://www.fodsupport.org/ Dawn Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 You know me Alice, always trying, but never quite getting there! Te he! A ADAMS wrote: >I'm sitting here laughing because that was exactly what I was thinking. > >Alice > > >The " top of your head " is much better than my " whole " head. Or should >that be " hole " head? > > > > >wheatchild2 wrote: > > > >>This is all I can offer off the top of my head. >>Barbara> >> >> > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 You know me Alice, always trying, but never quite getting there! Te he! A ADAMS wrote: >I'm sitting here laughing because that was exactly what I was thinking. > >Alice > > >The " top of your head " is much better than my " whole " head. Or should >that be " hole " head? > > > > >wheatchild2 wrote: > > > >>This is all I can offer off the top of my head. >>Barbara> >> >> > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 You know me Alice, always trying, but never quite getting there! Te he! A ADAMS wrote: >I'm sitting here laughing because that was exactly what I was thinking. > >Alice > > >The " top of your head " is much better than my " whole " head. Or should >that be " hole " head? > > > > >wheatchild2 wrote: > > > >>This is all I can offer off the top of my head. >>Barbara> >> >> > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 We have this diagnosis too. We also have a familiar diagnosis of a Complex I, III and IV issue. Alice Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 We have this diagnosis too. We also have a familiar diagnosis of a Complex I, III and IV issue. Alice Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 We have this diagnosis too. We also have a familiar diagnosis of a Complex I, III and IV issue. Alice Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 17, 2004 Report Share Posted September 17, 2004 Ah well there's plenty of holes in this head too.....just ask my family! B > > >This is all I can offer off the top of my head. > >Barbara > > > > > > > > > > > > > > > > Quote Link to comment Share on other sites More sharing options...
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