Hirschsprung's disease

thisplaceisstupid

is anyone familiar with this in their LO? let me know. thanks!

snairic102

thisplaceisstupid said:

is anyone familiar with this in their LO? let me know. thanks!

Lurker here, but you rarely see anyone asking about Hirschsprung's on TB so I thought I'd jump in.  My 20 month DD has Hirschsprung's.  She was diagnosed when she was 4 months old and is doing great.  I'm happy to answer any specific questions.

ToastieSimons

What would you like to know?  My DS1 doesn't have it but we had to go through lots of testing for it, all up until the biopsy and endoscopy.

Lizzybizzy80

My son has Hirschsprung's. He's 18 months and had his pull through surgery when he was 10 days old. He's doing great now. Happy to answer any questions I can either here or pm.

thisplaceisstupid

snairic102ToastieSimons,
@Lizzybizzy80

well my daughter is 4.5 and was born a health 8.5lb baby but has always been slow to gain weight and always has chronic constipation. she hasnt gained any weight in a year and recently started getting full even quicker than normal so i took her in to get things figured out. we go to a GI early next month. her x-ray showed lots of stool in her colon. but the few things my child does is fruits, veggies, and is already on a miralax regimen and has been one and off for years. i just feel like something may be causing the constipation, but of course i dont know.
so i googled, and it does seem like a its a disease that shows up in infancy but can still occur in toddlers and children.
anyways, what kinds of symptoms did your kiddos have? and what were the treatments? all her bloodwork at her regular pedi came back normal, if that makes a difference..
tyia!

Lizzybizzy80

I think it's worth looking into and going to the GI, good luck! It could be that she has a milder case but still something you want to confirm or rule out as you try to resolve.

My son was 8lbs 13 oz and looked perfectly healthy but didn't pass any meconium/stool for 48 hours after he was born which is a big red flag. He had difficulty nursing and was spitting up and gagging. He had a glycerin enema and was taken to the NICU for irrigations and X-rays. The barium enema xray showed the stool backing up and swelling his intestine. A small portion at the end wasn't functioning or allowing anything to pass.

When he was 6 days old we were transferred to the NICU at the Children's hospital where he had a rectal biopsy and lots of tests/bloodwork. They confirmed it was Hirshsprungs and would need surgery. He had an IV and picc line and was not allowed to eat anything until a week or so after his surgery. The surgery was laparoscopic and they pulled through the colon so that the affected portion would be bypassed. They took biopsies during surgery to see how much was affected so they didn't take too much or too little. They said the affected portion was about 10 cm.

We had to watch him closely for signs of constipation/illness that first year, and still do, to make sure he isn't developing enterocolitis. We had to do dilations at home with plastic tubes of increasing size to make sure the scar tissue didn't tighten him up. He had one stricture that the surgeon fixed easily during a follow up. He is prone to horrible diaper rash so we use Ilex/aquaphor at night and triple paste/aquaphor during the day for every change. We haven't had any major issues but I'm a bit nervous about potty training. We haven't had to change his diet but may try probiotics if we have issues.

thisplaceisstupid

Lizzybizzy80
poor baby! that seems so scary especially when they are so tiny! did they say if he woud have any issues long term?
if my lo does have it, it definitely would be a milder case but we shall see. we dont go for a couple more weeks. in the meantime i have to give her the glycerin liquid suppositories to maintain until then at least its not a full out enema but im sure that means nothing to her!
thanks for the info! i appreciate it.

snairic102

Sorry your LO is having struggles. I think it's worth asking the doctor about hirschsprungs, though as you said, it's more common for it to be diagnosed at an earlier age because the kids usually get really sick. Milder cases can go undiagnosed for longer though.

Our story is very similar to all that @Lizzybizzy80 said... Like her son, my daughter didn't pass meconium within 48 hours of birth.  I think that's the biggest red flag for HD.  She was transferred to the NICU, they did a few enemas, and she started pooping on her own so was released after a few days.  She showed no more symptoms until 3 months or so.  At that point she stopped having BMs regularly, her belly started being super distended all the time, and she stopped gaining weight.  She would only poop if stimulated (like if we took a rectal temp).  

We were lucky that her pediatrician thought of HD (based mainly on her having not passed meconium after she was born and now showing these signs) and referred her to back to the pediatric surgeon that had seen her in the NICU.  They did two biopsies -- first a suction biopsy, which was inconclusive, and then a full thickness biopsy which confirmed she had HD.  After that they did a barium enema to try to see how much colon was affected.  Then she had her laparoscopic pull through surgery to remove about 6 inches of colon.  We did dilations afterward for a few months, but life is pretty normal now.  She has no food restrictions as of now.  We try to keep her on a high fiber diet, encourage lots of fluids, and intervene with prune juice or Mirilax if she seems like she's getting backed up.  We were also told and have read that potty training can be difficult.

The biopsy is the only way to confirm a diagnosis, and that would involve general anesthesia for an older child.  There are lots of less invasive tests they can do to see if HD looks likely.  One thing to keep in mind maybe, is that I have heard that GIs are more likely to push laxative regiments for chronic constipation.  If you can get a referral to a pediatric surgeon, they may 

be a better person to talk to about HD or any other structural causes for the constipation.

Good luck!  

Lizzybizzy80

That's gotta be rough, we haven't had to do any suppositories

They don't think he will have long term issues. He definitely hasn't had any problem with constipation since the surgery, he may poop more often than most. Google was not my friend while we were in the hospital but I couldn't stop looking. I think some kids post-op have trouble with smearing or knowing when they have to go poop. We haven't tried any potty training but he definitely knows when he needs to go because he'll rush to a corner of the room and start grunting. His surgeon said this was a good sign. hope you have some answers soon!