Later onset CESD- life goes on... - LAL Solace Patient Community2024-03-29T01:19:23Zhttp://lalsolace.ning.com/forum/topics/later-onset-cesd-life-goes-on?feed=yes&xn_auth=noHi Karen, I am very happy tha…tag:lalsolace.ning.com,2012-04-16:6373865:Comment:112632012-04-16T13:01:01.365ZMelissa Aguilarhttp://lalsolace.ning.com/profile/MelissaAguilar
<p>Hi Karen, I am very happy that your daughter is doing so much better. I also have CESD and it is very scary being diagnosed and just not knowing. I thank my lucky starts for this site and the people on here. I myself am working on the diet and will see what comes of it. As of now I am trying to treat the complictions arising from the CESD.</p>
<p>Sincerely,</p>
<p>Melissa</p>
<p>Hi Karen, I am very happy that your daughter is doing so much better. I also have CESD and it is very scary being diagnosed and just not knowing. I thank my lucky starts for this site and the people on here. I myself am working on the diet and will see what comes of it. As of now I am trying to treat the complictions arising from the CESD.</p>
<p>Sincerely,</p>
<p>Melissa</p> Thank you so much for sharing…tag:lalsolace.ning.com,2011-12-15:6373865:Comment:101062011-12-15T19:30:44.460ZMelissa Aguilarhttp://lalsolace.ning.com/profile/MelissaAguilar
<p>Thank you so much for sharing this information Mary - every little bit helps. I am learning more and more as time passes by.<br></br><br></br><cite>Mary Pruitt said:</cite></p>
<blockquote cite="http://www.lalsolace.org/forum/topics/later-onset-cesd-life-goes-on#6373865Comment4806"><div><div class="xg_user_generated"><p>This answer comes to us from Anthony Porto, a pediatric gastroenterologist and hepatologist at Yale University. </p>
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<p>Patients with Wolman’s and CESD have a low level (as…</p>
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<p>Thank you so much for sharing this information Mary - every little bit helps. I am learning more and more as time passes by.<br/><br/><cite>Mary Pruitt said:</cite></p>
<blockquote cite="http://www.lalsolace.org/forum/topics/later-onset-cesd-life-goes-on#6373865Comment4806"><div><div class="xg_user_generated"><p>This answer comes to us from Anthony Porto, a pediatric gastroenterologist and hepatologist at Yale University. </p>
<p> </p>
<p>Patients with Wolman’s and CESD have a low level (as in CESD) or lack of LAL (as in Wolman’s disease). Loss or decrease in LAL leads to a build-up of fats called triglycerides and cholesterol esters (cholesterol ester is cholesterol which is linked to a fatty acid molecule)in the lysosomes of the cells. Because these fats get ‘stuck’ in the lysosomes, the cells think that it is deficient in cholesterol and begin to make more of it. This leads to a build-up in fat in the liver as more cholesterol is made, and leads to a fatty and enlarged liver. Accumulating fat leads to liver damage and eventually cirrhosis. It is sometimes difficult with imaging studies such as an ultrasound to differentiate a fatty liver from someone who is overweight from someone who has CESD or Wolman’s. Livers of patients with CESD and Wolman’s may have an orange appearance because of the extra fat in the liver. A healthy, normal weight person should not have accumulation of fat and their liver will not have accumulation of fat on imaging studies as there is a balance in fat production, transport and use.</p>
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</blockquote> This answer comes to us from…tag:lalsolace.ning.com,2011-08-05:6373865:Comment:48062011-08-05T20:34:36.438ZMary Pruitthttp://lalsolace.ning.com/profile/MaryPruitt
<p>This answer comes to us from Anthony Porto, a pediatric gastroenterologist and hepatologist at Yale University. </p>
<p> </p>
<p>Patients with Wolman’s and CESD have a low level (as in CESD) or lack of LAL (as in Wolman’s disease). Loss or decrease in LAL leads to a build-up of fats called triglycerides and cholesterol esters (cholesterol ester is cholesterol which is linked to a fatty acid molecule)in the lysosomes of the cells. Because these fats get ‘stuck’ in the lysosomes, the cells…</p>
<p>This answer comes to us from Anthony Porto, a pediatric gastroenterologist and hepatologist at Yale University. </p>
<p> </p>
<p>Patients with Wolman’s and CESD have a low level (as in CESD) or lack of LAL (as in Wolman’s disease). Loss or decrease in LAL leads to a build-up of fats called triglycerides and cholesterol esters (cholesterol ester is cholesterol which is linked to a fatty acid molecule)in the lysosomes of the cells. Because these fats get ‘stuck’ in the lysosomes, the cells think that it is deficient in cholesterol and begin to make more of it. This leads to a build-up in fat in the liver as more cholesterol is made, and leads to a fatty and enlarged liver. Accumulating fat leads to liver damage and eventually cirrhosis. It is sometimes difficult with imaging studies such as an ultrasound to differentiate a fatty liver from someone who is overweight from someone who has CESD or Wolman’s. Livers of patients with CESD and Wolman’s may have an orange appearance because of the extra fat in the liver. A healthy, normal weight person should not have accumulation of fat and their liver will not have accumulation of fat on imaging studies as there is a balance in fat production, transport and use.</p>
<br/> I will forward your message a…tag:lalsolace.ning.com,2011-07-26:6373865:Comment:49032011-07-26T16:56:08.580ZMary Pruitthttp://lalsolace.ning.com/profile/MaryPruitt
I will forward your message and hopefully have an answer for you soon! Thanks for asking such great questions!
I will forward your message and hopefully have an answer for you soon! Thanks for asking such great questions! Hi Mary,Can you ask them anot…tag:lalsolace.ning.com,2011-07-26:6373865:Comment:49022011-07-26T16:14:03.308ZKaren deJonghttp://lalsolace.ning.com/profile/KarendeJong
Hi Mary,<br></br>Can you ask them another question as well? Can you ask them how much we know about Acid Lipase levels? Normal levels are 20-100? Wow, what a range! What is 'normal' for a 2 year old? Would someone who has a level of 15 be diagnosed (or 19?)? Is there a huge difference in the levels of people who are diagnosed earlier or later in life? <br></br>We've been able to get Amy's cholesterol under control with diet with a level of 10. Does that make sense? Will it become more…
Hi Mary,<br/>Can you ask them another question as well? Can you ask them how much we know about Acid Lipase levels? Normal levels are 20-100? Wow, what a range! What is 'normal' for a 2 year old? Would someone who has a level of 15 be diagnosed (or 19?)? Is there a huge difference in the levels of people who are diagnosed earlier or later in life? <br/>We've been able to get Amy's cholesterol under control with diet with a level of 10. Does that make sense? Will it become more difficult to control as she gets older and needs less fats for her body to grow?<br/>Karen <br/><br/>
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<cite>Mary Pruitt said:</cite><br />
<blockquote cite="http://www.lalsolace.org/forum/topics/later-onset-cesd-life-goes-on#6373865Comment4801"><div>Hi Karen! We have been looking for an answer to your question about fat storage in CESD and Wolman's Disease. We are going to be asking a team of medical experts your question very soon! Thank you for your patience:) Mary</div>
</blockquote> Hi Karen! We have been lookin…tag:lalsolace.ning.com,2011-07-23:6373865:Comment:48012011-07-23T14:20:46.537ZMary Pruitthttp://lalsolace.ning.com/profile/MaryPruitt
Hi Karen! We have been looking for an answer to your question about fat storage in CESD and Wolman's Disease. We are going to be asking a team of medical experts your question very soon! Thank you for your patience:) Mary
Hi Karen! We have been looking for an answer to your question about fat storage in CESD and Wolman's Disease. We are going to be asking a team of medical experts your question very soon! Thank you for your patience:) Mary And I am not sure about the i…tag:lalsolace.ning.com,2011-07-13:6373865:Comment:43042011-07-13T15:55:53.506ZMary Pruitthttp://lalsolace.ning.com/profile/MaryPruitt
And I am not sure about the information about Wolman's with fat storage percentages. We were never told about anything like that. Let me look into that. Very good question!!!
And I am not sure about the information about Wolman's with fat storage percentages. We were never told about anything like that. Let me look into that. Very good question!!! Thank you so much for sharing…tag:lalsolace.ning.com,2011-07-13:6373865:Comment:45022011-07-13T15:54:58.976ZMary Pruitthttp://lalsolace.ning.com/profile/MaryPruitt
Thank you so much for sharing! And I am so glad to hear of Amy's improvements...gotta love chubby little ones:)
Thank you so much for sharing! And I am so glad to hear of Amy's improvements...gotta love chubby little ones:) Her doctors talked about her…tag:lalsolace.ning.com,2011-07-12:6373865:Comment:44042011-07-12T23:07:22.485ZKaren deJonghttp://lalsolace.ning.com/profile/KarendeJong
Her doctors talked about her body treating 1 gram of fat like 15 grams. So... she stores 15x the fat? <br/>I am not sure where they came up with those numbers from. Are those kind of #s available for Wolman's?
Her doctors talked about her body treating 1 gram of fat like 15 grams. So... she stores 15x the fat? <br/>I am not sure where they came up with those numbers from. Are those kind of #s available for Wolman's?