DS just diagnosed with Hirschsprungs disease

[pudseypie]

Please can someone share positive stories of Hirschsprungs disease? Ds who is almost 2 has just been diagnosed this morning after waiting nearly 5 weeks for his biopsy results. He's had problems since birth, but on the scale of Hirschsprungs must not be the most severe form as we manage it with medicine although he's had a few hospital admissions. What happens next? Has anyone been through this?
Got to wait for appt now to go back to see surgeons at the childrens hospital just want to know what to expect next. Am still in bit of state of shock even though I knew it might be diagnosed.

[funkypigeon]

Sorry to hear this, I have no experience of this although I've been pushing for DS (5) to be tested for it. Can you give me a brief rundown of what your DS' symptoms are?

We've had constipation and bowel control issues for 3 years...

[pudseypie]

He struggled to pass much meconium at birth and vomited bright green bile, was jaundiced and wouldn't feed. Was in hdu and surgical paediatric ward for 12 days until he started to open his bowels himself. Since then he's been has episodes of severe constipation which have led to hospitalisation for bowel washouts and enemas. It was so severe once his bowel almost twisted. Day to day symptoms are being bloated, regular constipation and trouble passing wind and vomiting when they do get constipated. Ds has a lot of laxatives and we manage his diet closely so he does ok at the moment and hasn't had an enema since end of May. More common in boys than girls apparently. Is your dc under the care of a paediatrician?

[funkypigeon]

Oh dear...sounds awful. Well, yes he is for both bowels and asthma. Our situation is different though, he didn't have the problems your DS had at birth, but was constipated from around 1 yo. From then we've had endless sachets of movicol, lactulose and they made him go around 8-10 a day at its worst.

He wasn't looking like he had any control of his bowels until about 3 months ago when things started to improve with toilet training. We watch him though as he holds it in, gets bloated and his tummy always looks big. A lot of wind as well. We are also controlling his diet carefully, but he can go a week sometimes without having a poo.

Paeds rather dismissive at the moment and prefer to just palm off with laxatives, but I really don't think it's getting to the root of the problem. So frustrating though. I wish you all the best with your DC.

[pudseypie]

Something we get told all the time is that constipation is the most common reason for a child to see a paediatrician but what's not normal is being hospitalised for it. Hirschsprungs normally shows itself fairly soon after birth from what ive read so I would be hopeful that your ds doesn't have it. My ds was first checked for coeliac disease and food allergies as my db is coeliac. Has your ds been tested for anything like that?

[minkersmum]

I used to look after a little girl born with hirschsprungs. She had a 'pull through' operation as a baby as the rectum wasn't formed. She was managed with laxatives, occasionally weaned off for a break and yakults were quite effective for a while.
She would have bouts of being bunged up and often required enemas at hospital. Often preceeded by days of leakage. Managing diet was a huge factor in how well she was.
Despite all the pain and hassle of hirschsprungs she was a delightful, spirited, bright little girl who has grown into a successful beautiful young woman.

[pudseypie]

Thanks nice to hear a positive story minkersmum. I expect he'll have to have a pull through operation but just want to know what happens after surgery as he grows up, will he need another op and still have any problems? Anyone else know?

[DeputyDeputyChiefOfStaff]

Pudseypie, I don't know in any great detail but I recently found out that an acquaintance had hirschprungs, and you wouldn't know it now. I don't know what he went through as a baby/child, but he seems very healthy now and has children, a demanding job etc.

Funky pigeon, has your dc with asthma and bowel problems been tested for cf?

[pudseypie]

Thanks Deputy. Am hoping it's only one round of surgery but i am worried he'll have some degree of constipation all his life and need further surgery as he grows up.

[pudseypie]

Bump. Someone, anyone have firsthand experience of this?!

[rachmini]

My DS has Hirschsprungs Disease, he was diagnosed within a few days of birth and had the Soave pullthrough done at 16 days. He was also re-admitted a few weeks later and was operated on for bowel adhesions. He is now 2 ands half and continues to do well with regards to his Hirschsprungs. He has half a sachet of movicol a day and that seems to work for us at the moment.
Please feel free to ask any questions.

[pudseypie]

Hi rachmini, good to hear your ds is doing well. Is his short or long segment? I'm finding it really hard to find out what to expect after surgery, such as will he have bowel problems all his life and will he need any further treatment, continuous medicine etc? I'm waiting for an appt to see the surgeons again and I guess, get him booked in for surgery. We've waited a long time for the diagnosis as he had a suction biopsy at birth which came back negative but still showed symptoms so I didn't expect him to be suddenly diagnosed with it. I trust the hospital completely with the surgery, they have saved him twice already, but just worried about how HD will affect the rest of his life.

[rachmini]

My DS has short segment HD, not sure exactly how much was removed. From the information I have read different children with HD all have their unique issues depending on the severity of the disease. Common issues are nappy rash and constipation, we use Ilex cream with a layer of Vaseline over the top for nappy rash and movicol for constipation. Another complication is enterocolitis. There are many support groups, www.Champsappeal.co.uk and www.hirschsprungs.info these forums are a wonderful support network and a useful source of information.
I hope ur appointment comes soon, there's nothing worse than waiting. We have learned to take one day at a time. Please keep asking questions and I will share what I know. X

[pudseypie]

Thanks for the reply. Good to speak to someone at last who has gone through it! The diagnosis explains why ds has always suffered with severe nappy rash. Have read about the Ilex cream but is it hard to get on prescription? The best over counter nappy cream I found was in Ireland and I get it shipped over now but prescription will be cheaper if I can get it now we have a diagnosis. Since having the pull through has your ds had any enemas or wash outs or has he been ok? My ds is on 2 sachets of movicol a day plus sodium picosulphate 2/3 times a wk which seems to work ok but he still has the odd day like today where he hasn't gone yet and I start freaking out!

[rachmini]

We buy Ilex from Oakmed uk, think we pay about £13 per tube, which is a lot but to us is worth every penny. It is not available on prescription (typical)!!
Since his pull through we have not had to give washouts or enemas, we r lucky!
If your family is like ours, then poo is the top topic of conversation! Hope u see some shortly!!

[pudseypie]

Oh yes poo is our specialist subject too! He's finally done a small poo and ive given him 2 satsumas so fingers crossed the rest comes soon! Good to hear your ds has been fine since the pull through, my ds hasn't had an enema since end of May which is longest he's gone without, am hoping with a pull through it might end them all together. Can't believe you can't get the cream on prescription makes the one I get sent over cheap in comparison. Thanks for all the advice

[rachmini]

Let's cross fingers that the surgery goes well and there will b no need for enemas! Can imagine that as they get older the more they will resist!
My DSaid 2 and a half is not walking and has been diagnosed as having low muscle tone and hypermobile joints as well as Global Developmental Delay. Aside from this he is an extremely happy boy and is progressing (at his own pace!)
It's all go!!

[pudseypie]

Sounds like you have a lot to deal with but you have a very content little boy! My ds already resists the enemas, he says 'uh oh' when he sees the hospital and runs away from the matron as he recognises her from doing his enemas before.
If it's any help the nappy cream I get from Ireland is Caldease ointment or Morhulin cream. Got both in Tesco and Boots over there, just on the shelf. Pretty thick stuff but it literally heals his bottom overnight. Worth trying if anyone you know goes over and can pick you up a tube to try. I'd tried everything here, both prescription and off the shelf and ds bottom would just bleed.

[rachmini]

Quite a bit to deal with but we've got used to all his appointments now. He's had numerous tests done including an MRI but everything is clear, we r now awaiting genetic results to see if there is a reason behind his GDD and low tone, we may never get a diagnosis. He's improving on a daily basis so we r happy.
Many thanks for the info on the creams, I will definitely get someone to pick them up for us if they r going over to Ireland.

[pudseypie]

Glad he's improving. Must be stressful though waiting for the results? I had to wait nearly 5 weeks for the hirschsprungs results and it drove me mad. It's strange though how you get used to all the check ups and get to know the staff on the wards.

[rachmini]

sorry for the late reply, it's been a busy few days!
we have waited for soo many results and have decided that whatever they find we will still treat him the same as we have been. The consultant did tell us they may never find a diagnosis for his delay.
I read over the weekend that Ilex will be available on prescription in the new year!!!! yippee!!!!

[pudseypie]

Whatever they find with your ds as long as he's a happy little boy that's all that's important and it sounds like he is. Good news about the Ilex cream!

We are still battling with the hospital to get an appt for a follow up to find out what they intend to do or even the type of hirschsprungs he has! Am so frustrated, he had his biopsy 6 weeks ago and I still don't know what type he has or what happens next. Surgeon's secretary was so unhelpful and rude yesterday we ended up making a complaint. Think we have fallen down a gap between the 2 hospitals we deal with but seeing his local paediatrician nxt week so will see if he can help.

[rachmini]

Good news today, we had a letter off the geneticist to say that the results were normal!! He is still part of a developmental delay genetic study but so far so good!
I really hope u get an appointment shortly, there's nothing worse than not knowing what is happening
especially to ur child! 6 weeks is such a long time to wait. It's been such a long road for u to get a diagnosis and now you've got one it's totally understandable that u want to get an appointment to sort out the next stage. x

[pudseypie]

Oh that's fantastic! I hope he continues to improve for you, sounds like he's doing really well now. Will he build up muscle tone as well for walking?

[rachmini]

really pleased!!
my Ds has physiotherapy, rebound (trampolining), hydrotherapy aswell as OT and speech and language! all this input has certainly made a massive difference! the rebound therapy is working well for his muscle tone and he is weight bearing a lot more!

[pudseypie]

So pleased all the therapy is working for your ds! It must give you a lot of hope to see hin progressing so well.

Just found out the reason the hospital are so slow is that they've sent the biopsy sample onto Great Ormond St hospital for a second opinion as it was positive for hirschsprungs which is standard procedure apparently, they just didn't tell us that!

We have Irish friends going home for Xmas who will pick us up a bulk load of the nappy cream that works for us, if you'd like to try a tube pm me your address and I'll pop a tube of it in the post to you in the new year.