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Dr. Andrew Rynne
MD
Dr. Andrew Rynne

Family Physician

Exp 50 years

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Suffering From Hirschsprungs Disease. Stomach Never Feel Full.Not Gaining Weight. Any Treatment?

i have hirschsprungs disiese, i was born with it and i am not on TPN i was when i was younger and Vanderbilt does not no of any other person that has it and was born with it that is not, i am only 5ft 5 and way maximum of 120 pounds. im 17 years old male and constantly hungry i out eat everyone i know and letterly never feel up. Things like eating a whole bag of bread does not even feel me and i can not gain weight for anything, i curently take alot of vitamans. I have tried weight gainers but, cant drink anything that even taste like milk i cant even make my self drink milk total whitch could be a problem with me gaining weight but i take calcium pills and at least eat one yogurt a day. Please give me some tips! PLease!!!!!! and i dont have a email at this time but if you could please give me a call or something my number is 615-542-3396.... really hope that you can help i am tired of being small and can not find any help in Nashville,Tn Vanderbilt at all. My little brother also had the same thing as me and past away but had a feeding tube and was on tpn and was still very small. Another question have you ever herd of someone having A child with hirshsprngs another kid thats fine then another kid with hirsprings? .
Wed, 13 Nov 2013
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Ayurveda Specialist 's  Response
**
1. since in Hirschsprung's disease [HD] there are no ganglion nerve bodies in the colon thus it cannot move out stool from colon to anus resulting in chronic constipation and intestinal obstruction [causing proximal section of bowel to become distended with feces, sometimes called as congenital aganglionic megacolon]
2. since it is from childhood, thus hopefully doctors would have gone through baseline investigations like barium X-ray, manometry, biopsy to diagnose the problem, and if presently the problem is interfering with your day to day living you can ask for treatment options like Ostomy Surgery or pull-through procedure.
3. because of TPN (Total Parenteral Nutrition) which has to be cycled intravenously, because of the risk of dehydration and lack of weight gain due to the high losses of stomal output from their ostomies , [ probable reason of death of your brother], there is a very restricted diet [consult with a Dietitian regarding the same]
4. other major concern will be high growth of bacteria due to building up of feces, which may cause serious infections [enterocolitis] if the problem is not treated.
5. since there is associated intestinal obstruction which causes constipation which leads to vomiting and thereby loss of weight [ which can be a reason of not gaining weight]
6. since there is loss of appetite in people suffering with HD, while you are feeling constantly hungry, this means you have to be evaluated by a Gastroenterologist for any other morbid pathology.
7. Hirschsprung disease appears to have a dominant pattern of inheritance, which means one copy of the altered gene in each cell may be sufficient to cause the disorder. The inheritance is considered to have incomplete penetrance because not everyone who inherits the altered gene from a parent develops Hirschsprung disease. [reason of your last query].
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Suffering From Hirschsprungs Disease. Stomach Never Feel Full.Not Gaining Weight. Any Treatment?

** 1. since in Hirschsprung s disease [HD] there are no ganglion nerve bodies in the colon thus it cannot move out stool from colon to anus resulting in chronic constipation and intestinal obstruction [causing proximal section of bowel to become distended with feces, sometimes called as congenital aganglionic megacolon] 2. since it is from childhood, thus hopefully doctors would have gone through baseline investigations like barium X-ray, manometry, biopsy to diagnose the problem, and if presently the problem is interfering with your day to day living you can ask for treatment options like Ostomy Surgery or pull-through procedure. 3. because of TPN (Total Parenteral Nutrition) which has to be cycled intravenously, because of the risk of dehydration and lack of weight gain due to the high losses of stomal output from their ostomies , [ probable reason of death of your brother], there is a very restricted diet [consult with a Dietitian regarding the same] 4. other major concern will be high growth of bacteria due to building up of feces, which may cause serious infections [enterocolitis] if the problem is not treated. 5. since there is associated intestinal obstruction which causes constipation which leads to vomiting and thereby loss of weight [ which can be a reason of not gaining weight] 6. since there is loss of appetite in people suffering with HD, while you are feeling constantly hungry, this means you have to be evaluated by a Gastroenterologist for any other morbid pathology. 7. Hirschsprung disease appears to have a dominant pattern of inheritance, which means one copy of the altered gene in each cell may be sufficient to cause the disorder. The inheritance is considered to have incomplete penetrance because not everyone who inherits the altered gene from a parent develops Hirschsprung disease. [reason of your last query].