The first few weeks Bee was pooing on average 8 times a day. We nailed a system pretty early on that stopped the nappy rash in its path. And we learnt not to change his nappy immediately after a poo – why you ask? Because the first poo was a pre-poo trap! One to lure you in and wait for you to take his nappy off – and then just when you’re vulnerable (mid nappy change) he’d hit us with the real mother load. 

We dealt with poonamis on the regular – a favourite episode of mine was when he decided to drop a ‘burn the babygrow’ korma poonami in the middle of John Lewis.  

Then after a couple of months it started to slow down and he got more or a regular pattern of once or twice a day. Which was a blessing when he started crawling, as just when you think you’ve nailed this nappy changing business they gain the ability to escape you mid change, and you find yourself desperately trying to stop them leaving pooey bum prints on your mums cream carpet.

Weaning was our next challenge – it’s totally normal and understandable for non HD babies to get constipated when they start solids. Remember they have  been on a liquid diet their whole lives! So we started slowly and cautiously weaning him with a few fruits and veggies. The faces and grimaces he made on his first tastes were priceless. And obviously this affected his bowels, and he got a wee bit bunged up. So we started plying him with pears and prunes…except they made the situation worse! They gave him insane gas and when the prune poos finally landed – boy did we know about it. 

But we ploughed on! At 6 months we realised he had some pretty severe allergies (I will go in to detail about those another time) and once we got those sorted we managed to get his poo to a normal colour and finally got him to stop vomiting. 

And we chugged along – his bowel movements changed monthly. One month he’d go every day, the next just three times a week. Then we had two blips, one caused by teething but rectified with some washouts and another caused by a tummy bug, but fixed with some glycerol suppositories. But what mattered was that he was going, and that he wasn’t distended. And this carried on for 9 months.  

And then boom, out of nowhere he stopped. No reason why, no diet change. He just stopped. 

Glycerol suppositories worked for a few weeks. 

Then we tried lactulose – this one didn’t really work for us at all. He’d strain and strain with no relief. 

Then movicol – this worked for a month.

Then Senna – this worked for a week.

And let me tell you, I’m a hopeless optimist. Every time we get prescribed one of these things I imagine comedy style scenes of them being so powerful they have him pooing madly. But nope that’s not the case.

He got to the stage where nothing worked. His surgeon was away. The GP told us this was out of his depth and he didn’t know what else was licensed for someone bees age. Bee ended up basically on hunger strike, with intermittent vomiting episodes and dry nappies in the middle of a heatwave. So we put him back on washouts. Not easy with a then 16month old on solid food, it’s pretty much a complete different technique to washing out a baby on just milk, but after getting bigger tubes and working out which syringes worked best…We got a washout to work! And now it’s like bee knows that this is something which makes him feel better, so he lays there like an angel and watches the iPad while we do it. 

My top tips on preparing for the pull-through surgery.

1. Mentally prepare in advance for a long surgery and when I say long I don’t mean 3hours I mean on average 6 hours (Bees took 8). Know it’s going to be unbearable but take something to do or keep your mind distracted during this time, sudoku/a film/all your boring admin (I sat and rung sky and negotiated a cheaper tv package). Or just find somewhere quiet and go and sleep because you won’t get much sleep on the ward.

2. Depending on the season, pack clothes that are right for the temperature and easy to put your child in. Bee was four months old and it was July so we went for some cute button up short sleeved/short legged onesies from Next & H&M which were easy to put on him as they didn’t have to go over his head with all the tubes and wires he had attached etc.

3. Take baby blankets – the hospital ones are super crispy and starchy. And it’s just nicer when they are cuddled up in their own blankets. Also if you previously swaddled or used sleeping bags – these wont be suitable because of all the wires they have attached. Bee was terrible for kicking his blanket off so I found it worked best if I just put it over his chest and torso and not over the bottom of his legs.

4. Pack lightly the rooms/cubicles aren’t big in hospitals and there is normally one bedside table for stuff and that’s it.

5. Tablets/chargers are essential. Hospital TV is basic and on childrens wards if turns off at 9pm (pre-watershed). We found that the iPad (Poldark’s Cornish tones to be exact) helped amuse bee and get him to sleep. And you can pre-load the iPad with programmes if you download them while on your wifi at home (hosp wifi is slow and unreliable) on apps such as BBC, ITV, Netflix etc. I even downloaded a few episodes of love island to catch up on!

6. Take all the nappy creams with you. Buy loads in advance of the operation. Speak to other HD parents beforehand and see what worked for them and just buy one tube of everything, that way you already have them all and can do a few days trial and error with each till you find a mixture that works.

7. Don’t underestimate how many nappies you will need. Think we went through about 15 a day straight after his operation.

8. Pack your vitamins. Hospital food is shit. Not sure why the hospital catering contractors (fuck you Sodexo) want to kick you while your already down, but the food is appalling whether it’s in the ward of the canteen. Prepare to live on chips and pre-packed sandwiches that will cost you the earth.

9. Get to know the ward, not just the nurses and their names, which will make you feel more comfortable anyway, but the policies. They often have policies for reduced parking fees if your child is on the children’s ward, canteen discounts, free food for breastfeeding mothers – after all every little helps. Also if you speak to the nurses they might let you heat up your own food in their staff microwave, meaning you can eat your own home cooked food or supermarket food – this will save you some money.

10. Pack some toys for your child to play with and make them feel at home.

11. Take slippers for yourself.

12. Prepare for no sleep, between the anxiety of watching and checking on your own child, the noises of the nurses coming and doing obs, the machines beeping (occulizing downstream – which means an IV tube is bent) and the other patients on the ward also doing all of those things – you might get an hour, two if your really lucky. Me and my hubby took it in turns to do nights in the hospital and sleeping in the day at my sisters which was nearby.

13. Don’t be afraid to ask or say no. Someone comes in to do a test on your child – ask what it’s for and why it’s being done, and if they seem to be struggling to do it ask for someone else. We had a senior nurse struggle to take Barney’s blood one day, and it just made it more drawn out and uncomfortable than it needed to be and I wish I had asked for someone else to do it. Also chase them for test results as sometimes things can get lost in the paperwork and you never get informed of the outcomes.

14. You will be in at least a week. Possibly two. Our surgeon prepped us for this beforehand. So we just told ourselves to plan for the worst case scenario of a two week stay, and feel blessed if we were discharged earlier.

15. Your child will be nil by mouth, for how long depends on your surgeon. Ours wanted him to be nil by mouth for a good few days to give the stitches time to heal and to give that area time to rest. This however doesn’t mean your child is getting nothing – they will be on an IV drip with fluids and sugars in to keep them nourished, or a TPN. But as it doesn’t go in their tummies it goes directly into their blood stream they will still get that empty tummy rumbling sensation – unfortunately there’s not a lot you can do about this. If bee got really cranky we gave him sucrose – sugary solution, squirted on to his tongue which distracted him and normally knocked him out. It’s also very handy to distract them when they have uncomfortable things like blood tests.

16. We were finally allowed to feed barney 72hours after the operation. So day four in hospital. They did this by giving him tiny amounts of my expressed breastmilk every few hours, and building up the amounts. Then the next day I was allowed to feed him directly – but only small amounts. And then by day three he was allowed to feed normally. But with the increase in food comes the increase in poo!

17. When will they poo? There is no straight forward answer for this one. Bee had a bowel movement while he was in recovery although I’d imagine it was more caused by some sort of muscle spasm than an actual poo. He started going properly about 48hours after the op! But it varies from child to child and case to case. As they are on the IV fluids their body still produces waste, just not as much as if they were eating properly. Prepare for these poos to smell worse than anything you have ever smelt, and be full of all sorts of blood and gunk from the operation. Nappy changes will also be bloody hard to do, with all the wires and stuff attached to them, and the inability to take them out of the bed. But don’t struggle alone, remember you have loads of nurses and people around to help! With helpful things like inca pads (basically disposable changing mats) which are really helpful as barney used to love to poo when his nappy was off. And get your nappy cream ready. At this point I really was a novice when it came to changing shitty nappies but when you baby starts pooing 15 times a day you catchup fast! I used to get all my kit out first, and squeeze the nappy cream onto cotton wool pads, so it was ready to be applied quickly.

18. Gas! Farts! Trumps! Wind! Another amazing thing your baby may now be able to do! Immediately after the op barney used to do these amazing old man/dad farts that were super duper loud, I just used to grin and laugh and feel amazingly proud.

19. Sleep patterns. Before barney had his operation he used to sleep through the night, for us unfortunately I think his operation and the trauma/unfamiliarity and messed up routine of being in the hospital clashed with the four month sleep regression and from then on he has only slept through a handful of times. But being in an unfamiliar noisy environment with observations being done through the night really does mess up their sleep patterns and routines.

Up till recently it’s been a bit too raw for me to write about Barneys operation, particularly because he’s been doing so well, I didn’t want to dwell on such a shit time.

But here we go:

The night before his operation we washed Barney out (for what would hopefully be the last time) packed the car up and then travelled to Norwich to stay with one of my sisters (she lives very close to the hospital and has been an absolute saint! She put us up at her house and looked after us really well during the whole ordeal, even though she was heavily pregnant and on maternity leave and should really have just had her feet up). Saying goodbye to family members before we left for the operation was much more emotional than I expected. 

Barney slept through the night like an angel, and I woke him at 3am for his last feed before the operation. He wasn’t allowed milk after 4am, and then could just have sips of water until 6am. 

They had booked the theatre for the whole day because the operation can be quite long, and he had a morning admission time.

Being the angel he is, he went back to sleep after his feed and we had to wake him to take him to the hospital at 6.30 because he normally slept till 8.

I didn’t sleep, I worried and twisted and turned in bed, with an anxious knot balled up in my stomach churning away.

After a quick pump to keep my milk supply going we headed to the hospital. We were put in the High Dependency Room, right next to the nurses station, with another baby and the nurses got us and Bee ready for surgery.

The morning seemed really long while we waited for him to go to surgery, getting in lots of cuddles and making him laugh. I didn’t want him to pick up on our anxiety before he went, I wanted him to be happy and settled, so we smiled and played with him as much as we could.

We saw the anesthetist and the surgeon (our consultant) for any last minute questions and then we went off. We carried Barney through the long hospital corridors up to the theatre. I gave barney a kiss and a cuddle and his daddy took him in to the theatre. I had been warned by friends that some babies fight the anesthetic and it’s upsetting for parents (particularly mums) to see them have it, so we had agreed that hubby would take him in. I sat in the family room next to the theatre and just sobbed. When my husband joined me we were briefed on what would happen next, we were given a pager and told we would be buzzed towards the end of surgery.

Barney went into theatre at 9am. We went back to my sisters house, had breakfast and tried to just busy and distract ourselves. The hospital had told us that the pagers reach as far as Norwich town centre, and they advise parents to go home or to work or shopping, just anything to keep themselves distracted.

We knew the surgery would be at least 6 hours but we headed back to the hospital at about midday anyway, not wanting to be far just in case.

At 3.30 we were finally paged to go to the theatre, we waited another thirty mins and the surgeon came out to tell us the surgery had gone well. It was only Short Segment (SSHD) and he hadn’t needed a colostomy bag, and had only lost a tiny bit of blood.

He said they would now be finishing up and setting up the epidural which would be his pain relief for the next few days.

It was another hour and a half until we saw him in recovery. He was asleep, with machines and people buzzing all around him. He had IV fluids and antibiotics, heart monitors, a catheter and the epidural so we couldn’t pick him up. They wheeled us back to his room and he started to perk up, that first night he was quite spaced out, staring in to the distance a lot. They managed his pain well though and there was only one instance where they hadn’t given him his paracetamol in time that he became very distressed.

The next day you could see he was doing well, he was alert and kicking his legs, he was grumpy as he was hungry and wanted a cuddle, but otherwise doing really well. He even smiled at the consultants and nurses. 

Barney had his operation on Monday and was nil by mouth until the Thursday. Wednesday night was the worst night because that’s when he was really bothered by the hunger. And at this point we still couldn’t pick him up because of all the tubes and wires. So the nurses would give us some sucrose (sugary watery solution) to squirt in his mouth which was usually enough to settle him for a bit so he could sleep. 

Because we couldn’t pick him up we had to be more innovative in ways to keep him entertained and comforted. He loved cuddling his bumble bee toy! We played with him doing funny voices and acting out little scenes. We also learned that barney loves the telly, so we would pull that over the bed so he could watch it. (Although because it’s a children’s ward the TVs stop working at 9pm!). He particularly liked watching Poldark with me when I stayed over night on the ward – must be those lush Cornish accents.

Family members came to visit, my mum and dad came every day and my sister would cook food and bring it to the hospital for us. This was such an amazing support for us because being on the ward is horrible. You have new medical people come round every day and ask the same tedious questions, and run tests and do observations. They come at the most inconvenient times – like when he’s asleep. Or they forget to come at all and don’t silence machines when they have finished and the machines beep away and wake up the lovely sleeping baby (who it’s hard to get to fall asleep as you can’t feed or hold him). So having family round was lovely, it gave us new faces to talk to, some respite and some well needed emotional support. 

Barney started having bowel movements on Tuesday night! I can’t tell you (and you probably will never understand it unless your child has HD) how fantastic it feels when your child poos. Even if they smell like the worst thing on earth (which they did straight after the op). But it’s still bloody amazing! Oh and he also started trumping away! Farting like a trooper!

By Thursday they said Barney could start eating again. They kept him nil by mouth so that his bowel would have time to heal, with as little as possible passing though it. He had been getting what his body needed through an IV drip – straight into his blood. They would only let us feed him tiny amounts of expressed breast milk every three hours by bottle on the first day, 20mls, then 40mls, then 60mls (then 60mls after that). This is because the doctor didn’t want his bottom to be overwhelmed by nappy rash when it started working again. 

But as we introduced food, boy, did he start pooing! It was non stop explosive poos! The only way I can describe it is like a comedy fart noise, super loud and wet. But again I celebrated everyone,  i didn’t care if they were in the middle of the night, I still celebrated them. The only awkward bit was changing his nappy before they took the epidural and catheter out. Especially because Barney has a new party trick – while I’m cleaning up one poo he likes to squeeze out another one – so in the hospital this meant a change of bed sheets and pissed off health care assistants.

Barney was discharged on Saturday morning and we were advised to stay in the local area for a while just to be on the safe side, so we were near the hospital. So we stayed with my sister for another week. The hospital discharged us with some medi-honey (nappy rash cream) and some paracetamol for his pain. I have gone into some detail about nappy rash in another blog post and why that particular cream is useless!

On the day he was discharged they took his dressings off and we finally saw his wound. It was a shock, we had been told he would have keyhole surgery but his scar was the size of my csection scar just positioned more to the left. But we discussed this with the doctors who explained that they sometimes find they can do a better job if they do it that way rather than keyhole. (Also 7 months on his scar is so well healed and faded you can barely see it at all).

But we had our boy! He was out of the hospital! And his bum worked!!!! And he was doing amazingly well!

Since then we have had a few bumps in the road, weaning, constipation, nappy rash, severe allergic reactions, asthma, eczema, gastroenteritis…. but we have only had to wash him out a handful of times since his operation! And he bounced back from the whole thing so quickly! 

After a pull through operation one thing parents will have to face during their child’s recovery is nappy rash.

Because of the numerous HD Facebook groups I’m on, I was ready for this. And I’m incredibly thankful to the parents on those groups for being so open about the struggles they were having with nappy rash and what they were found working and what wasn’t, as this really helped get us prepared before the operation. It also helped us prep for the scale of nappy rash, which is much worse than the nappy rash a non HD child would get.

Here’s a few things we did that worked for us.

1. Slightly odd…  

Firstly a few months before the operation our surgeon suggested we do something slightly unusual in our daily routine with Barney, he stressed that he has no idea if it worked, but that he had another parent also trying it. He told us to get some of Barney’s poo (which at this point we were getting out via the twice daily washouts) and rub it on his bum. The idea being to expose his bum to some poo beforehand, to hopefully get the skin used to it and for it to start toughening up.       
So everyday when we did the washouts and when we first put the tube in, before putting in any saline at all, we were sometimes able to get out some poo. We then got this in a nappy ready to smear and leave on his bum for a few hours after the washout. Now, I have to say, rubbing poo onto your child’s bum is a weird experience – it’s the absolute opposite of what your instincts are to do

2. A good nappy!

Secondly I had read parents saying that the nappies they were using were exacerbating the nappy rash, because the nappy would get stuck to the skin alongside the poo, and cause the skin to come off when the nappy was removed. So when we put the poo on Barney’s bum everyday we tried out different nappies to see how they reacted. Unfortunately I found that the big two brands we used really got stuck to his skin, that was Pampers and Mamia nappies. 

Luckily my sister had seen an advert for a smaller brand of nappies sold online, Beaming Babies Biodegradeable nappies – the brand claims to have a lot less chemicals in the nappies (one of the main causes of nappy rash). So we brought some of these and gave them a go! They are fantastic nappies, they are made differently so don’t have the same sort of mesh material as normal nappies and so don’t get stuck to the skin with the poo! We stock piled these before the operation. The only downside being they are a lot more pricey than your standard nappy!

I had also heard very similar good things about reusable cloth nappies, however because we didn’t know exactly how many times a day he would be pooing after the op (some kids go a few times an hour) we thought it best to put these on hold. 

However now we are a few months down the line and his bottom has got more of a regular pattern to it, we do use reusables. It can be a bit overwhelming because there are so many brands and types of reusables, so I’d advise you to seek out your local cloth nappy library/group and ask them to show you a demo kit and talk through it all.

Also as soon as you mention them to an older relative they will just start telling you negatives about when they used them, because that’s all they had, and what a pain they are. Just ignore them! They are so easy to use nowadays, no fancy folding or pinning. Just Velcro/poppers and a cloth liner to soak it all up. They also come in some super cute patterns.

We don’t use cloth all the time – however we have a routine of using them in the mornings and more often if he has nappy rash. I think they are a lot more breathable for their skin when they have nappy rash, so I always think it helps.

3. Air it out!

This is so important! Think about when you get a cut on your finger and you put a plaster on it constantly for a few days, when you take the plaster off it’s all moist and shrivelled like a cocktail sausage. You need to give your little ones nappy free time every day. Once we were discharged from hospital we did 30mins to an hour every day. This is so you can get some air to the skin, let it breathe, dry out and heal. To help with this we used inka pads given to us by the hospital, or nappy training mattress protector sheets, or puppy pads. If your baby is small you can cut these in half or quarters so you get more out of them. 

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4. The right cream

Finding the right cream that works for your child sadly isn’t a straight forward thing, and it may take a few weeks to get the right one, and the only way to do it is trial and error.

In the hospital we were given medihoney to use, although this didn’t work for us, and in my opinion is pretty naff as it’s not thick at all so barely creates a barrier, let alone a nice waterproof one.

Yellow metanium cream – this stuff is pretty good and has worked for us, but it doesn’t always, it depends on the cause of the nappy rash (teething, frequent poos etc). And because it’s so waterproof it’s a bit of a pain when you do want to get it off, so don’t get it on your hands!

Orabase – available on prescription or online, this stuff is amazing and always seems to work for us. It forms an ultra thick layer that’s very waterproof and really protects the skin.
Ilex – available on prescription. This is the strong, last resort type stuff, we have found if combined with orabase it does work. We did one layer ilex, one layer orabase.

Corn starch – not one I have tried, but I have heard lots of people say it’s fantastic, and works well when added to the nappy creams or applied as a layer on top.

Epiderm ointment – haven’t tried this but have also heard it works on nappy rash.

Desitin (purple) – an American nappy cream, we have tried this and despite the weird smell, it does work on milder nappy rash.

Vaseline/petroleum jelly – whichever cream you pick i would recommend adding a layer of petroleum jelly for babies on top. It works to make the cream thicker and protect the skin.

5. Method

How you clean their bottoms is just as important as the cream you use. Prior to his surgery we used water wipes, which are pretty sensitive, but afterwards we didn’t even want to risk these so we just used water and cotton wool. I brought a travel bottle for cosmetics and filled it with water, we spray this directly on his skin and just wipe with the cotton wool. This worked for ages, however with a more recent persistent bout of nappy rash it didn’t work. We live in a particularly hard water area, and I think the water can be almost too drying on their skin, so I have changed to organic coconut oil and cotton wool now. (Top tip use cotton wool pads, the cotton wool balls just get fluff stuck everywhere!).

I have read some parents say they wash their child each time they poo, this is fab if your at home or have a bedea.

I have also heard some use a hair dryer on a low setting to dry baby bums and set the nappy cream. (Haven’t tried this myself).

You can also buy specific nappy cream brushes, random I know. But they look like a mini silicone spatula and they help you to apply a really thick layer of cream evenly, without getting any on your hands. 

6. Speed

Timing is important, you have to change these nappies as quickly as you can. And straight after the op you may find your child is going several times an hour, and then again during the nappy changes. This includes through the night. But because the poo can be acidic the best thing is to just get it off them as soon as you can. Watch out for this on long car journeys aswell. 

P.s If you have any other tips or advice to help parents battle nappy rash please let me know and I will add them on to the list.

Also FYI the main image is for comedic value, also because I just watched Wonder Woman 😀

Disclaimer: I am not a doctor – seriously, although…I may have watched a lot of episodes of embarrassing bodies thats not a recognised medical qualification. This is my understanding of the condition – the condition simplified. 

Who/what/why?

Basically when a baby is developing in the womb one of the first things to develop is its digestive system. Starting with the mouth and throat – the cells reproduce creating the stomach and keep going to produce the small intestine and then the large intestine. Crucial to making the intestines work are nerve cells called ganglion cells. These nerve cells tell the muscles in our intestines to relax and let stool pass through. Without these our intestine is permanently contracted and stool can’t get through (like a tunnel that’s caved in). In Hirschsprung’s disease these ganglion cells are missing from part of the intestine: there are a different names for the condition depending on how much of the bowel is missing the cells:

Ultra Short segment Hirschsprung’s disease (USHD).

Short segment Hirschsprungs disease (SSHD).

Long segment Hirschsprung’s disease (LSHD).

Total colonic Hirschsprung’s disease (TCHD) this is all of the large intestine and some of the small intestine. 

Why: When we found out he had the condition I felt sick, sick to my very core, questioning was it something I had done? Was it my fault? I had worked too hard when I was pregnant, got stressed too frequently, eaten badly, not gone over speed humps slow enough, broke sharply in the car, forgotten to take my vitamins. But they aren’t sure why the condition develops, some believe it could be something hereditary, a faulty gene, some researchers feel there is a link between a family history of both thyroid and digestion/GI problems. But importantly they don’t think it’s anything the mother did when she was pregnant and at the moment it isn’t something they can prevent from developing.

Symptoms: The condition is usually picked up shortly after a child is born when they dont have a bowel movement. Other symptoms that often come along side this are bilious (green) vomiting, a distended (sticky out and swollen and hard) stomach, lack of appetite and lethargy (sleepy). However the condition can be diagnosed in older children who have been having bowel movements but suffer from extreme constipation. 

Diagnosis: The condition is usually diagnosed by a rectal suction biopsy (RSB). A special tool is used that sucks a few cm of the bowel nearest the anus in and takes a few snips out for testing in the lab. These biopsys are then sent off to a pathologist who cuts them in to super fine slices and stains them and looks for ganglion cells – or more specifically looks for them not being there. This is supposed to be a very definitive method of diagnosing the condition so it has a ‘gold standard‘. This is done while the child is awake and not under general anaesthetic.

Barney had three of these done in two different hospitals – expect some bleeding (just a bit nothing too heavy) for 24hours or so after this is done. Generally the child will also be uncomfortable because after one of these is done you can’t do a rectal washout for 24hours. But supposedly the procedure itself isn’t painful, as there isn’t the right nerve endings there or it to be painful. One doctor described it as being similar to biting the inside of your cheek.

If this test is inconclusive a strip biopsy can be done. This involves removing a bigger strip/piece of the intestine to send to the lab. This is done under general anaesthetic as it’s more invasive. (From speaking to parents – I have known these to be done because when the RSB takes its biopsies it misses the section that is missing ganglion cells. As the section that’s missing them is so close to the anus). 
Other means used to help identify the condition are X-rays and a barium enema. X-rays by themselves show how full the intestine is – and these are like enlarged/swollen loops. The barium enema X-ray is slightly different as contrast material containing barium is put into the anus and then an X-ray done – the barium then means the large intestine can then be seen clearer on an X-ray and they can see the affected segments. However these two usually aren’t considered enough on there own (without a biopsy) to give a diagnosis of hirschsprungs.

Complications: 

Enterocolitis: The condition is usually picked up shortly after the child is born. However if it isn’t it can be fatal. This is because children with this condition can develop an infection called hirschsprungs enterocolitis. Hirschsprungs enterocolitis – for a child without hirschsprungs might just be a tummy bug like norovirus, however for someone with this condition it’s much more serious. Before the pull through operation (when the child can’t poo) – unlike a non HD child who would get diarrhoea and vomiting and manage to clear the bugs out of their body by vomiting and having bowel movements – a HD child can’t do the diarrhoea part. So the bacteria multiplies in their gut. This can be fatal if not picked up and treated early enough because of two things, either the child becomes so dehydrated or the infection goes into their blood stream and they get scepticimia. Symptoms to look out for are lethargy, off their food, diarrhoea, vomiting, distended abdomen, fever, foul smelling bowel movements. If you suspect hirschsprungs enterocolitis – you must take your child straight to A&E as soon as possible, it can become extremely dangerous in a matter of hours. Hospitals will treat the child with IV antibiotics (flagyl also known as metronidazole), nil by mouth and rectal washouts. They aren’t sure why but even after children have had the corrective procedure (pull through operation) they are still very prone to getting this infection, so unfortunately it’s something you have to keen an eye on for years. So I avoid friends and family with tummy bugs like the plague!

Stoma: Lots of children have to have a temporary stoma fitted. This is when part of the intestine is brought out through an incision in the stomach and the poo is collected in a bag. As far as I understand these are fitted for two reasons:

1. They cannot decompress the child enough with rectal washouts. So a stoma is fitted – usually if this is the case this will be done at a few days old. This is reversed at a later date when the child is older, usually after the pull through operation has been performed and had time to heal.

2. When they perform the pull through operation they don’t feel the blood supply to the area is good enough – so they fit a stoma to allow that area of the bowel to rest and heal. 

From what I have heard from parents there is lots of good support from specialists nurses available if your child has one of these. You are trained while still in the hospital to do the bag changes. Downsides are things like skin nearest the stoma breaking down – as it’s abdomen skin it’s not used to fecal matter and lots of creams and powders have to be used to stop this. Bags splitting/breaking. Babies pulling bags off. But I have also heard positives from parents – no need to do washouts which can be more stressful and time consuming for parent and child, much easier to monitor and measure their output, also no severe nappy rash on their bottoms.

Treatment:

The condition is treated with a Stoma or daily rectal washouts until a pull through operation is performed. The pull through operation can be done varying ways – key hole, open surgery, or through the anus. And there are three ways of doing the actual pull through itself – Duhamel, Swenson and Soave. 

The operation is a long one, Barneys took almost 8 hours. But they seem to average about 6. It’s split into two or three parts.

Part One: the surgeon going in and doing biopsies inside the large intestine to map out the areas missing the cells. They do a lot more biopsies then they have done before, at say every few cm. Once they have done this (a process which takes a few hours as they do the biopsies then wait for the lab (who are on standby) to give results) they then understand how much of the intestine has been affected by the condition.

Part two: Now they know how much is affected they then they find the completely healthy part of the bowel (which has ganglion cells) and pull this down to the rectum. So the poo can now get from higher up in the intestine to the rectum without getting stuck in a section that’s missing cells. The different methods Swenson, Soave and Duhamel all do this pull through in slightly different ways. (Advice: do your research into these and their success rates and which you feel will be the best for your child, your surgeon will probably specialise in a particular one but if you feel strongly you can always change hospitals).

Part three: if they haven’t been able to decompress the child with washouts they may fit a stoma. Also if they do this operation and the area where the pull through is doesn’t have a good blood supply they will fit a stoma – this works by diverting the poo so it comes out of the stoma in the abdomen, allowing the bowel and pull through time to heal and recover with poo passing through it. 

Transition zone: this is an area of the bowel above the part which is missing cells – this area has some cells but may not have 100% of the cells. Pull through operations may need to be redone if the surgeon has pulled down part of the transition zone as opposed to completely healthy bowel.

Complications: infections, scar tissue, bleeding, failed pull throughs, nappy rash.

After the operation: Bee was lucky he had SSHD and there was no need for a stoma. His recovery was also straight forward so we were discharged after 5 days, but told to stay in the local area. Children are kept nil by mouth and given fluid drips for the first few days to give their bowels time to rest and heal. Bee had his op on Monday and was finally allowed to eat by Thursday. But some children are kept nil by mouth for longer and fitted with a tpo line to feed them. We were warned his hospital stay could have been up to two weeks.

Nappy Rash – we were warned by our surgeon to expect nappy rash after the operation. I know what your thinking what’s the big deal right – but I’m talking no skin, completely raw horrific nappy rash. Our surgeon was blunt – he used the word raw about 6 times and told us Bee would likely scream every time he went to the toilet. Part of this is because they have never had poo on their bums before. The other part is because their poo is acidic and finally the pure frequency of bowel movements doesn’t help. We created a strategy based on things I had heard from other HD parents to combat this (but that will have to be a whole blog post on its own).

Long term – the outlook: this varies from child to child depending on severity of the condition and how much of the bowel has been affected, and how successful the surgery is but generally from the research I have read they have either got the condition under control or learnt how to manage it enough that by their late teens it shouldn’t be lowering their quality of life. 

Some children have the pull through operation and never have a problem again, others suffer with constipation and 30% can be varying degrees of incontinent. Some children also have problems caused by scarring, sutures and muscle spasms. They also are still susceptible to Hirschsprung’s enterocolitis for years after the operation – the jury is out on why this is – as after all, if they can have bowel movements and have healthy bowels they shouldn’t be more susceptible to infection.  

Oh and again not to play it down at all, the nappy rash can be a horrendous problem that parents have to battle for a really long time too.

Technically it’s the early hours of Saturday as I write this. And the wildest part of my Friday night was getting a takeaway Nando’s (and I didn’t even get the peri-peri chips) and then getting in the jacuzzi with my mum.

Since I have had Barney most of the time I don’t know what day of the week it is – I can sometimes work it out by if eastenders is on. But god help me if it’s a bank holiday as I have no clue.

It’s like time has gone really fast since Barney was born but also really slow. I can’t remember what I did in my life before him, who or how I was and what I did with all my time. But now it’s like wow he’s suddenly 16 weeks old – it’s flown by. And I really get that cheesy phrase “treasure every moment”, cos they are only this small once and you won’t get this time again: when they can’t crawl – so you don’t have to worry about them sneaking off when your not looking, and you can still enforce cuddles. And they can’t speak so they can’t scream no at you and tell you they are too old to wear matching mother and son dungarees. 

On Monday Barnaby has his operation. It’s called a pull through operation. They will remove the part of his bowel which is missing ganglion cells (ganglion cells make his muscles relax and the bowel open so stool can pass through). It’s not a small operation – from speaking to other parents with children with the condition it seems to average about six hours.

You can do a lot in six hours: Watch two lord of the rings films or six episodes of love island. Have a spa day. Shop till you drop. Make a lasagne from scratch. Paint a room. Knit a hat. Have 4 REM cycles. 

The surgeon has warned us of all the risks of the operation. Of course I want to know all these but I’m also now completely petrified. It’s the most bizarre thing as I have been wanting him to have this operation since he was born and been eagerly waiting for a date. It’s the next step in his recovery. Wishing time to go quicker so he could have the operation but now I wish I hadn’t.

People ask me if it will just be the one op and it will be fixed. I wish I could say yes. But I can’t – we just don’t know. At this stage we still don’t know how much of his bowel is effected, or what condition it’s in. We don’t know if he will need a stoma. And we don’t know what his recovery will be like in the short or long term. Again it’s all about time. 

I can hypothesis about it. And i do:

“On Monday we found stool at ….cm so that means …..”

“The gas is always trapped at ….cm so that means this….”

“He pood by himself for three days in a row so surely that means this….”

But I don’t want to get my hopes up. I have read medical research papers and they don’t go into that much detail pre-op about the condition. I have scoured the internet for stories. But i think I have confirmation bias so I’m just looking for things to back up my theory.

The not knowing and the absolute lack of control and complete helplessness of the current situation is breaking me. That’s why at 1.20am I am writing this. Instead of catching up on some serious Zzzzzz’s. 

And on Monday I will be wishing time away again. Wishing for the operation to be over. Wishing to be reunited with my child. Wishing to know the results. 

And then his recovery will be time focussed too. How long will they keep him nil by mouth? How long till he does his first poo? How long till he’s well enough and we can take him home?