Hirschsprung's Disease Weaning and General Advice Needed

[LudwigVanBeetroot]

Hello

(Regular MNetter but have namechanged).
My 5 month old son was diagnosed with Short Segment Hirschsprung's Disease (ie. only the last 2 cm or so of his colon is affected) soon after he was born after failure to pass meconium. So far we have avoided surgery and he mostly poos regularly for himself (although they can be explosive), doesn't appear to suffer from constipation and is thriving. We occasionally administer rectal washouts but recently these have become far fewer. We see a consultant every month or so and have been on a wait and see what happens til we wean scenario. Obviously, we are aware that surgery is likely - but so far we have been incredibly lucky.

I have a few questions.

1. I can only seem to find info/blogs/threads on the net for those children who have HD and had surgery. I know of nobody else in our situation and if there is anybody on here with similar experience, please say hello because I would dearly love to hear from you if your child has not had surgery but has been diagnosed with HD. I'm keen to hear how you managed it and how they are doing.
   

1. I will start weaning my boy in the next month or so. So far he has been EBF, and I will be continuing to BF because of the natural laxative effects. If you weaned your child before (if any) surgery, what foods would you recommend we avoid or focus on?
   

It's really lonely out there when your child has a rare disease. There doesn't seem to be heaps of research, and given that it is a poo issue, people don't rush forward to discuss it. I've read the existing threads on here but they haven't been updated in a while, and it also strikes me that the children concerned have had HD to a much more serious extent.

Thanks for reading thus far. As I said, any info much appreciated, and if you have gone through this (especially the diagnosis so soon after birth) my heart goes out to you because it was very traumatic for us as a family.

Love
Ludwig Van Beetroot

[PUMBA]

Hi my little one doesn't have sshd but has had a lot of gastro surgery due to dysmotility .... It is difficult waiting to see what will happen sometimes I think the waiting is the worst part..

[fancyfilly]

hi there, my daughter has suspected short seg hd which wasnt even noticed until she was 6 weeks! she passed meconium and seemed to have no problems with pooing. she always seemed to have a distended abdomen though and always seemed to be crying and unsettled. by 6 weeks her stomache was really swollen and her bowel movements were explosive!.
i took her to the docs numerous times and eventually got sent to a childrens unit at the local hospital where i was constantly told that she had colic, heartburn , reflux ect. i just knew it was more that because she was ebf for a start and none of the treatment for the above eased her discomfort.
eventually ,we took her to a different hospital and straight away they took xrays which showed a massivley swollen lower bowel and what they described as a ragged rectum. we were transfered to the big childrens hospital in manchester where she had all sorts of tests including a rectal biopsy. before they did this however they gave her a wash out which seemed to provide almost instant relife from the pain of the built up gas.
she now has two wash outs a day whilst waiting for an anal manometry. i really understand how you feel about the whole weaning thing! isabella is now 5 months and still not sleeping through because she always seems to be hungry! she started showing an interest in food the past few weeks so i decided to try her with small quantities of baby rice once a day....big mistake!!! omg she last had a proper poo, which was foul!, on the sunday (sorry for the detail lol) and by the wednesday still hadnt had another bm! after speaking to the district nurse ,i started giving her her washouts with a few mls of olive oil in which helped a tiny bit but in a week she has had only two smears of poo in her nappies!
she had an appointment with the consultant yesterday who said to give her two oily washouts a day for the next week until i see him again. he also said not to bother giving her any food at all and just offer her as much milk or water as she likes. (she is bf in mornings and overnight with formula in the afternoons) i just feel really sorry for her because she is obviously interested in food now!
your so right about the lack of info for pre op hd , especially short segment. im just going to do as im told for the meantime as i dont want to cause her any more discomfort. so sorry for the essay lol just thought id share my experience so far with you lol
let me know how you go on with your weaning situation best of luck!

[fancyfilly]

oh yeah my little girl is now 20 weeks when writing this so maybe you could give me some info as your post was in feb lol only just noticed the date of your post :)

[stressedmum2]

Hi my ds is 3 has short segment hd found out when he didnt pass meconium at birth and obstructed his bowel. Had pull through op at Sheffield at 6 weeks took about 5 cm of bowel made such a difference to him. Still struggling with issues such as potty training which is not happening at moment. The problem we find with food is that too much fibre doesn't seem to be very good on his bowel weetabix is hell on him bottom. He also suffers really badly with nappy rash which I think is very common in hd. It is really difficult to find out what to except with this as nobody seems to know how things will go and people don't seem to understand how difficult a condition it Is to manage. It is good to hear from somebody in same boat as put few posts on here but not much come back.

[nickinoo]

my friends llittle boy who is 5 days old has just been diagnosed with this, obviously early days and not sure exactly how much of the bowel has been infected yet. He is going to have keyhole to assess. He is not big enough to have any operations yet so she has been told she has to give him washouts 4 times a day ( he is still in hospital at the moment but when he comes home)

She is understandably devastated and is very unsure as what the future hold.

Any information anyone can give will be highly appreciated

[LudwigVanBeetroot]

Hi All

LONG POST ALERT!

I noticed this thread on the front page a few days, wrote a long detailed post and then my computer promptly deleted it! I'm back now and hopefully this post will work. I was meaning to come back and let you know how we're getting on but have been busy with work and baby.

Our situation has taken an interesting turn. I don't want to give anyone false hope as every situation is so different with HD and it is of paramount importance that you follow exactly what your health professionals advise. But I thought I should update, and nickinoo I really feel for where your friend is at right now, that was us ten months ago and I remember only too well that shock and fear so close to just having given birth.

When I posted we were thinking about weaning. Shortly before we started feeding DS solids, we had an appointment with our regular consultant. We had been advised that we might be looking at surgery around 6-9 months, and it would either be pull through in one stage or rectal myectomy. Whether it was a busy day or just a blip, but during our appointment it became apparent that the consultant had confused our son's case with another more serious one, and started talking about far more invasive surgery than he had mentioned previously. We were telling him our child was pooing pretty much every day and washouts (which were advised every 24 hours unless our child had a spontaneous bowel movement) were becoming less and less frequent. It was as if he (doc) simply wasn't listening. We came away from the appointment and immediately sought a second opinion from a more senior consultant at the same hospital, but in his private practice.

The difference has been extraordinary. He was already aware of our son's case and said to us that because the initial biopsy (not done under anaesthetic) was inconclusive it didn't necessarily mean our son had HD, and that around 85% of newborns admitted for failure to pass meconium in the first 48 hours do not have anything as serious as HD. He told us to suspend washouts, feed our child sensibly - ie, avoiding the four main constipators of Banana, Rice, Applesauce and Toast to start with - and let our boy learn to poo without intervention. He did of course advise this with reminding us sternly about doing a washout if baby showed any of the signs of entercolitis, something we had been watching for from day one. After four days DS started to poo every day, sometimes up to three or four times a day, and that was in March. We haven't looked back since. He occasionally goes a few days without pooing, but we give him a little bit of lactulose most days as advised by our consultant and he now eats EVERYTHING, even rice, all sorts. I am still breastfeeding him, but he has the odd bit of formula on days where I am working and haven't been able to express.

We haven't ruled out having another biopsy done - under anaesthetic - should DS ever get seriously constipated but at the moment it's not on the horizon. So it looks as if DS doesn't have HD or if he does, it is ultra ultra short segment that doesn't seem to be a major problem.

I must stress again that this is our particular experience and we follow our consultant's instructions to the letter. But I wanted to post an update because we were distraught in the first few months after our son's birth, dreading surgery we saw looming ahead, worried about what to feed him, whether it would affect his growth, and it hasn't been like that at all. But one of the great things about the NHS is how seriously they take anything involving babies, and I will always always be grateful that our son received incredible care at such a critical point. Still, I am extremely glad we got another opinion and really pushed to be listened to. It has made a huge difference.

I hope you and your little ones are all getting along ok.