Newborn w/ HD questions -

Hirschsprung's Disease
#1

Hello everyone. I’m new here. I’m a 35 year old new dad. My son has HD and is in the 10% that has total colon HD. Last Thursday he had his appendix removed and a colastomy bag added.

I spoke to his doctor today and he told me in 5 or 6 months they will do the second surgury and my son will most likely lose all his “left intestines” I assume he means large intestines thats what my sons mother told me. I’m sorry this is all over whelming and Im not a doctor and don’t understand everything the doctor says.

He explained that my son has a good chance of survival and he hopes that with the next surgury he will be able to fix my son so that he can go to the bathroom normally but he might have to have a bag for the rest of his life.

Can someone help explain this to me. Im so scared and I dont want to lose my baby.

I was born with HD but it wasn’t nearly as bad as my son has it. I’m fine with no problems. I just feel so guilty that he got this from me and I feel so helpless.

#2

Hi,

I have had HD for 37 years and have to give myself enemas to stay regular.
As I get older it is a little harder to manage. It just takes so much time and
effort to do. I am trying now to get surgery, and I am 55 years old. I also
managed my diet for years, and used stool softeners, and laxatives. However,
it seems the bowel cooperates less as I get older. I wish you and your son
well. I understand that surgery is the best cure. Paulette

************************************** See what’s new at http://www.aol.com

#3

Good day Sir,

I am 42 years old dad of 12 years of boy. My boy suffered an intestine
perforation just after birth, 2 times operated but biopsy results were fine
and left as is.
But at the age 5, he had a hard blockage and biopsy, barium tests and some
more tests were repeated and diagnosed as HD (last 3 inches of intestine)
but against all practices, his doctor decided not to remove this part
because he was doing well during that 5 years period.
He is 12 now, and with a little bit controlled diet and one spoonful of
virgin-olive oil before going sleep, he is doing normal.

As far as I understood you had HD yourself, but you are fine. I was
wondering if is there any other sample that is not operated and living under
normal condition.
Please share your experience on this respect
Thanks in advance

Lutfu
From Istanbul-Turkey

From: Americancyco [mailto:hirschsprungs-cpt6175@lists.careplace.com]
Sent: Wednesday, October 03, 2007 8:47 AM
To: lutfusavaskan@altinbas.com
Subject: [hirschsprungs] New born w/ HD

#4

Sir, My daughter has surgury (pull-through) at 4 months old… They removed all of the bad intestine. She had no problems afterwards and has been a very healthy happy girl. Now she is 4 years old and undergoing belly aches consistently. But they have not said it is related at this point undergoing some testing. Good Luck. We went through Childrens Hospital Boston Mass with a wonderful surgeon. Good day. Beth

#5

lutfusavaskan2004 asked about my having surgury,

I had surgury when I was a baby, a couple duing my first year and some of my intestines removed. But it wasn’t as much as my son is going to have.

I wish i could help you with astory of not having surgury but I can’t sorry.

#6

Many thanks,
Best wishes for your child, I will pray for you

Lutfu SAVASKAN

-----Original Message-----
From: Americancyco [mailto:hirschsprungs-cpt6175@lists.careplace.com]
Sent: Wednesday, October 03, 2007 3:12 PM
To: lutfusavaskan@transal.com.tr
Subject: Re: [hirschsprungs] New born w/ HD

#7

First of all your son is so handsome, congrats on his birth. My daughter Danielle is five with total colonic hd. She had her bag untill she was ten months old and did a pull-thru. The first two years was the roughest for us. Now she has very good bowell control and does well with meds to thicken her bm’s. I had a therapist come in emed…to keep her up developmentally and found ways to keep her hydrated. We also ended up with a feeding tube to use to increase feeding and add liquids to her. I loved the tube once in and she still uses it today. It was one of the best moves we made. The road you on now is going to be long but their is hope at the end of the tunnell. Yahoo has a coupld of great support groups that may help you. We have a small one set up for just total colonic parents. Feel free to look us up. We will be glad to help you on your journey. Feel free to ask me any questions. Gen

#8

First of all, congratulations on your new baby!

I want to encourage you to take a look at my family’s story. My 11 year old son has Total Colonic HD just as your new son, as well as my daughter. We’ve had many struggles over the years, but all in all, this has shaped mine and my child’s life so beautifully that we can encouage others with out story.

DO NOT feel that you are responsible for your child’s diagnosis. We are all created with a purpose.

Your son has a fabulous chance with all of the new procedures and techniques now. Most people would never know that my children are different in any way.

I don’t want to overload you with information so I’m going to leave it at this. The link to our website is in the signature below.
Best wishes.

Warmly,
Lisa Busha
HMDSN
lisa@hirschsprungs.infomailto:lisa@hirschsprungs.info
mom to Tyler (11) TCHD, NID, Ileostomy
and Emily (7) LSHD, NID, Colectomy, Duhamel Pullthrough and Miralax dependant
www.hdkids.orghttp://www.hdkids.org/
----- Original Message -----
From: Americancycomailto:hirschsprungs-cpt6175@lists.careplace.com
To: bushafamily@msn.commailto:bushafamily@msn.com
Sent: Tuesday, October 02, 2007 10:47 PM
Subject: [hirschsprungs] New born w/ HD

#9

Lutfu,
I have a almost-3-year-old with HD in just the last inches of her intestine and she has not had surgery. We have been able to control her symptoms with diet, and very occasionally miralax. We watch her diet very closely and things seem to work out. She still gets constipation sometimes, but so far we haven’t had any major problems. This seems better than having her go through the surgery.
I haven’t ever talked to other parents who have been able to control it with just diet, so it is nice to hear of another out there. :slight_smile:

Good luck to you and your son!

#10

Hello Sir,
I am new too, this is my first email to respond to, but I was moved by your emotions. I have been there, or in the same vicinity anyway.
I am the mom to a 4 1/2 yr. old boy, diagnosed with HD shortly after birth. His is not total, but he did have about 3 feet of his intestines removed. He went thru 2 colosotmy surgeries (the first pull-thru did not work because all of the affected intestine was not removed)–this is VERY unusual. I think most kids have a colostomy for 8-12 months, then they will go back in to remove how ever much intestine is affected, then they will re-attatch his remaining intestine–then it is a wait and see situation. It takes a while, maybe a week or more for thier system to start working again while recovering. If it works (meaning he starts pooping), then you manage it from there. There is ALOT of trail and error, but you begin to be an expert on your child’s patterns.
If it doesnt work, then that is when you may be looking at a colostomy again. We thought for awhile we would have a colostomy bag forever–we (my husband and I)were able to accept it, finally. Kids are so accepting and adaptable. They only believe something is wrong with them or different if we teach them that is the case.
Even tho this is not the road we would pick for our sons, I have to say to you I have met several kids with this diagnosis. NONE of them blame thier parents. They have all been the most caring, confident, compassionate children I have ever seen. Your son is very lucky to have you for a dad. With you involved, he will accept who he was made to be and he will be awsome at it.

The first 3 years were very rocky for us, but we are very good now. The things I wished someone would have told me when my son was born…
TRUST YOUR GUT INSTINCT–no one knows your son as well as you will
ASK QUESTIONS until you understand what is going on with your son–you will need the information to help him
WRITE DOWN WHAT THE DR. SAYS–you will not remember it otherwise
and most importantly, love your son the way he is–without guilt. You could not have known this would happen, he will only love and accept himself as much as you do.
Best wishes to you and I will keep you and your son in my prayers.

PS My son now loves to show off his belly scar to his friends–he is the only one with a smiley face on his tummy.

Tonya, mom to Cole

----- Original Message ----
From: Americancyco hirschsprungs-cpt6175@lists.careplace.com
To: tonya_d_stewart@yahoo.com
Sent: Tuesday, October 2, 2007 10:47:03 PM
Subject: [hirschsprungs] New born w/ HD

#11

This is a ? for those who have HD and managing w/o an operation. My son has severe constipation that is managed by diet and laxatives. He had 2 biopsies in the past month. 1st one showed he had short HD; 2nd one higher up showed normaility. Surgeon said he is functional and that it wont be fixed w/ surgery. The thing is he still doesnt really say or diagnose that our son has HD or not. He said there is a chance that he might have gone into the transition zone it would show abnormality or have really,really short HD. He said he did see the transition zone and went above it, but there is a chance that it could have still been in the zone. I was just wondering how those biopsies came out to figure out how many inches. My son’s biopsy abnormaility was a few centimeters so does that mean only a few centimeters are technicaly messed up. Also my son cant or will not poop anything that has form. it has to be peanutbutter consistency or less. He cries and whines and sometimes strains for an hour or so when it is thicker or almost formed. Is that consistient w/ HD. Does it hurt. He has had the same symptoms since 4 months of age, he is 2yrs old now.

#12

Your story sounds very similar to ours. My daughter is almost 3 now. She was
consitpated from the day she was born, and most of the doctors told us it
was “normal”. When she was 4 months old she had a giant hemhroid that left
her a skin tag. She was finally biopsied and I had the same confusion. The
doctor was not very clear about where the transition zone ended and the HD
began. And it took months for us to actually get a confirmed HD diagnosis.
He finally gave us a very vague statement that it was “only the final
inches”, I couldn’t ever get him to commit to an exact length, but I’m not
sure if an exact length is even important.
My daughter’s HD is managed by diet. It is hard because toddlers can be very
picky eaters, or some days she won’t want to eat at all. She is very easily
constipated and, like your son, she cannot pass anything that is hard or
"normal". She only passes softer BM’s, usually about the consistancy of
peanut butter. If she has anything larger or harder, she struggles for hours
or even days and we have to use other means to help her (stool softner or
supository), and it seems to hurt her, which is difficult to see your child
go through. She won’t let anyone near her when she is having a BM, she
usually hides behind the couch under a blanket. She also tells us all of the
time that “it hurts to go poop”.
The best thing I have found is prune juice. i dispise the use of unnatural
interventions. I mix the prune juice with apple juice to make it taste
better, and she hasn’t ever complained. I also have her drink a lot of water
throughout the day. I use only whole grains and she eats a lot of veggies
and fruits. I have to watch her diet very closely. I am always looking for
good diet tips.
My heart goes out to you and your son and I hope I can be of some help. It
is a very frustrating process not being able to determine exactly what is
wrong and how to help your little one feel better.

Janae

On 10/3/07, desgei hirschsprungs-cpt6175@lists.careplace.com wrote:

#13

When my daughter was diagnosed with the disease. They told us only at surgury would they actually know how much needed to be removed. They had to remove, biopsy to see if more needed to be removed, remove, biopsy, ect, ect. Beth

desgei hirschsprungs-cpt6175@lists.careplace.com wrote:

#14

Jmhwales, Thankyou so much for replying. Only changing the diet never worked for us, the meds is what makes a difference. When he was a baby I would give him prunes and prune juice and it did not make enough difference. My major concern now is that he started out at 1/4 cap (@ 5 mo.) of miralax and now at 1 1/3 daily. Every few to several months we have to up his dose and every month we have to clean him out to almost start over again w/ completely loose stools, and than again when it becomes to thick for him to handle. My son is not on a complete strict diet, I just give him food w/ 100% whole grains and fresh fruit (doesnt like veggies). I give him a banana every day since about 1 1/2 yrs and now started giving small piece of chese every few days. Can that affect him? Tried only soy milk for 1 1/2 months and no change (3mnths ago). The surgeon is also giving us a hard time cause he also thinks that it might be behavior. Which makes me wonder if my son has a phobia to solid poo. Trying to figure this is so messed up when docs dont have answers. They also think that his colon maybe is extremely slow. I am frazzled cause I dont want him on any meds, but it seems like there is no other way. I really hate that there is no definite diagnosis cause it make me wonder well maybe it is behavior. I dont like this surgeon but still happy that he is willing to still help out, so far. Also I dont know much about Miralax, if it was behavior wouldnt my son have complete diareah w/ 22g of miralax and woldnt get thicker as time went on, cause he doesnt have diareah. Checking out info on the internet I found out that the doctors dont really know much about probable issues w/ the colon, I sure hope they start doing more research on it.

#15

My boy, before his surgery would strain all the time and cry all the time. He was able to poop some and then we did suppositories the rest of the time, before surgery. This def. sounds like it could be HD to me and I would get another surgeon, he doesn’t sound sure of much and with dealing with intestinal problems you need confidence and clarity to come from the one who will be doing the cutting if it needs to be done. My son to this day hurts down there after he poos, he hates to be wiped…
Truly,
Mary

----- Original Message ----
From: desgei hirschsprungs-cpt6175@lists.careplace.com
To: marycoody22@yahoo.com
Sent: Wednesday, October 3, 2007 3:13:29 PM
Subject: Re: [hirschsprungs] New born w/ HD

#16

We have had similar issues with miralax. Our son had pull through at 6 wks.
By 7 months he had quit going on his own and was started on miralax. We
started at 17g and gradually moved up to 34g. (he is now 11 months old)
After a couple of good weeks we suddenly had all water - only one time a day
but it was all water. We have played with the dose over the last couple of
weeks but either we get diarrhea or no poop. He has an appt to see Dr.
Levitt Oct 17 and he said to start doing daily enemas. He does not
routinely prescribe miralax. Thus far the enemas have been very good for
him. We haven’t had any distention and we get one good poop per enema and
no small “leakage” poop in between. I have been trying to get the surgeon
or the GI to give us another course of treatment for awhile but they are
diehard miralax people. I’m not wild about miralax - its basically
antifreeze and they know that in normal people without constipation the
miralax is completely expelled from the body but in constipation they
believe some of it is retained. Its not approved for pediatric or long term
use. Honestly they don’t know what it does to a person if it is left in the
body or where it goes. I am hoping dr Levitt will be able to give us a
better option. We are currently using babylax - liquid suppositories. It
really is trial and error and some doctors don’t want to try other things
which is frustrating when the one thing they suggest isn’t working well. I
hope you find a doctor that is willing to work with you. Oh…bananas are
constipating. As far as cheese - some say its constipating others say high
fat dairy acts as a laxative…whatever works. The amount you are giving
him probably doesn’t matter anyways.

Susan

----- Original Message -----
From: “desgei” hirschsprungs-cpt6175@lists.careplace.com
To: xians@charter.net
Sent: Wednesday, October 03, 2007 5:47 PM
Subject: Re: [hirschsprungs] New born w/ HD

#17

I just want to thank everybody who wrote me or answered one of my questions. This is all still over whelming for me but I’m doing better right now. I can’t begin to tell you ho much it helps to have you people to talk to.

I never even heard of TCHD before yesterday, I thought HD was bad enough (but I turned out fine so I ha high hopes for my son. I still have high hopes but it was a shock to learn all of this)

Just want to say thank you all again.

#18

Did they do a barium enema? What type of biopsy was done? Full thickness
or suction? Full thickness is the definitive -but they have to be under GA
for it. The biopsies do not tell you how many inches unless they take
multiple ones going up higher and higher until they find ganglion cells.
They can guess from the barium enema based on the transition zone but it is
just a guess.

Typically with HD you have explosive poops or diarrhea, or lots of little
poops and the symptoms should be there from birth. I don’t know about the
consisitency. I would find another doctor or have another pathologist
review the slides. Make sure they are using a pediatric pathologist…I
have been told that that makes a difference.

I’m not sure what you mean by he is functional. If it is HD then surgery
would take out the part of the bowel that is not working - ideally fixing
the problem. I would seek another opinion.

Susan
----- Original Message -----
From: “desgei” hirschsprungs-cpt6175@lists.careplace.com
To: xians@charter.net
Sent: Wednesday, October 03, 2007 4:13 PM
Subject: Re: [hirschsprungs] New born w/ HD

#19

They did do a barium enema and it did show negative, but it also showed that his whole colon seemed enlarged. They did full thickness biopsy (surgery) 2x’s, 1st one was inconlusive cause there was a part that had no ganglions and the rest all diminished. They had to do a 2nd higher up and that showed ganglion cells. My son seemed to have no problems w/ pooping, but from newborn untill he basicaly stopped @ 4 months (when I added formula) they were always explosive and he only went after every meal (none in between at all) and he never ever got sick or seemed to have problems.
I am also confused about functional too, cause the internet uses that term for a kid that got constipated and is now withholding cause they are scared to go, but they say for an infant or pre potty training that they should be done with it in a few months and not have to up the doses and keep cleaning them out. I believe the docs are saying functional cause the laxatives are working. On the internet there is not much out there that causes constipation. My son also has Waardenburg, and hirschsprung’s can be part of the syndrome. If it is HD than surgery should fix the issue, but I think the doc doesnt know what to say cause it would be such a tiny amount that would be affected and maybe surgery wouldnt fix it or he thinks that something else is going on. I emailed Dr. Levitt about 2 weeks ago and he did say that he can help my son. My husband and I are really discussing going there but that would be going across the country for us. I guess on my other post I was asking and wondering if it is possible to have it be so short on the biopsy but be considered to still have it. This surgeon beats around the bush for almost every question we have w/ nothing definitive.

To: desgei@hotmail.com> From: hirschsprungs-cpt6175@lists.careplace.com> Subject: Re: [hirschsprungs] New born w/ HD> Date: Wed, 3 Oct 2007 22:33:50 -0400> >

#20

My daughter at 4 months had a barium and it was still unsure…but showed something to question and that is when they moved onto a biopsy. She had the surgury pull-through right away…they said it is something you should’nt wait on. It could be dangerous…waste is toxic. We had a wonderful surgeon Dr. Fishman out of childrens Hospital Boston, Mass. Excellent Doctor!!! Explained things on a parent level so as we could understand this whole thing. Best of luck. Beth

desgei hirschsprungs-cpt6175@lists.careplace.com wrote: