So WTF is hirschsprungs right? And how did we come to find ourselves here…

Before Barnaby was born I was tested for Group B Strep. For anybody who doesnt know about it it’s a normal harmless strep virus that lives in the gut, however it can be passed to your baby during labour and could cause dangerous complications if it did. The NHS don’t routinely screen for this bacteria in pregnancy as women can test positive for carrying it and then also become negative in a short space of time – all before they go in to labour. However you can pay to have it done privately – and they send you a really simple testing kit – so this is what I did.

I tested positive. This isn’t really an issue when you have a csection unless your waters have broken beforehand, as the baby isn’t coming out of your foof so should manage to avoid the Group B Strep bacteria.

However when Bee was born his breathing was really fast. So they put him on IV antibiotics – which are given via a cannula (tube straight into a vein in the foot or hand). Digression: Cannulas are gross! Super gross! I’m not squeamish but seriously they ick me out! Barney was really good though and took his like a pro. Top tip if they give one of these to your child, make sure they wrap it up otherwise a small bump and they come out and they bleed a lot. (One doctor recently told us a fairy dies every time a cannula is knocked out). Back to the story: they decided to keep us in the hospital for a few days to keep an eye on him and give him a full course of antibiotics while they waited for his test results.

He wasn’t feeding very much, I couldn’t get him to latch and he was very sleepy. But for all I knew that could be normal newborn baby behaviour. However when he was about 36hours old he started vomiting green bile. It was about 3am in the morning and as he wouldn’t feed from me the midwives had convinced me to let them give him formula. They were doing this in the hallway with the lights on to wake him up. I was woken by a midwife who asked me to come in the hallway, she was stood with a doctor and Barnaby. They told me they urgently had to take him to the neonatal ward as he was vomiting green. I was petrified. I called my husband in hysterics. The midwives took me to neonatal so I could see that he was being well looked after and stable, they calmed me down and sent me back to bed to rest.

The next morning when I woke I wasn’t as freaked out as you think. I was convinced he would be fine and it would just be something to do with the Group B Strep virus which he was already being treated for.

Once my husband arrived at the hospital we went up to neonatal, the paediatric doctor informed us that they thought Barney had some sort of blockage in his gut – they had done an X-ray and could see it was all inflamed and swollen. As he hasn’t pood since he had been born and was vomiting green bile, they basically thought he was backed up all the way up to his stomach. They told us as soon as a transport team was available we would be taken to a hospital with a bigger neonatal ward.

While we were sat with Barney, who was in an incubator, another baby in the room went into cardiac arrest. We were rushed out of the room while they did compressions to save its life, it was an awful and truly shocking thing to see and really brought home the fragility of life and the gravity of our situation.

We had to wait all day for the transport team, with them finally arriving at 5pm. The midwives had worked quickly that morning to discharge me – as we thought the transport team was arriving imminently. That day felt endless. One of the longest of my life. For the first time ever I didn’t google anything – I was too terrified.

I tried to stay positive. I sat next to Barney’s incubator and told him everything we were going to do in the future, the places I would take him and the adventures we would have. He was hooked up to lots of monitors and had a tube in his nose draining his stomach – I had made this beautiful little person, who I hadn’t had the chance to get to know yet, and now we couldn’t hold him or help him as it was in other people’s hands, we just had to wait.

To begin with I was told I couldn’t go in the ambulance with Barnaby when they transported him but thankfully they had a change of heart and I was allowed. I waddled along the hospital hallways behind the ambulance transport team who were moving at high (non c-section) speed. I wasn’t allowed to get in the back of the ambulance with him so instead I had to climb up the steps in the front – not an easy thing when you have just had your stomach cut open. FYI 48hours after a csection you still don’t feel great – I felt like a tank transporter had driven back and forth over my stomach.

Before we had left frimley park hospital they doctors told us they thought at the new hospital (Southampton) they would do a barium enema and another X-ray to find out where the blockage was. However when we got there they rushed him straight into the intensive care nursery and did a rectal washout on him. This involves putting a small tube (catheter or nasal gastric feeding tube) up his bum and flushing the poo out with saline. A crazily large amount of poo came out. We celebrated we rang friends and family shouting “it’s fine it was just poo” then we went back in to see him and were met by the on duty surgical consultant. She informed us that Barnaby most likely had Hirschsprungs disease, and they would continue washing him out and keep him Nil by mouth but if that didn’t work to decompress his stomach they would fit a stoma. She never once explained to us what Hirschsprungs disease was or what it meant for Barney, luckily my husband knew what a stoma was but I was clueless. She abruptly asked me why I had gone pale – I wanted to scream “well lady 48 hours ago I gave birth to my son, I’m still floating on oral morphine and now you are telling me he has a serious health condition that I know nothing about?!!!”

I was hysterical, well except that’s an understatement. There isn’t a word for what I was. Inconsolable. Devastated. Those words aren’t enough.
I rung my sister, she knew about Hirschsprungs disease as she had done postdoctoral research into it with one of Americas leading surgeons at Harvard university. She explained the condition, how it is diagnosed (by rectal suction biopsy) and the outlook.

The first night we were given a room on the ward, it had a bed and a breast pump for me and a chair for my husband. After that we were given a room in Ronald McDonald house. (This was a lifesaver as the cost of commuting or staying at a hotel in Southampton would have been astronomical.) 

I don’t think I have ever felt as upset as I did that first night. I wish they could have given me something to calm me down. After speaking to my sister I had gone back on the ward to be with Barnaby and his notes were out. I read them and was abruptly told off by his nurse – who said they were confidential and I would have to request them first. I can’t remember exactly what i said to her but I don’t think I was polite.

But it’s an awful feeling – I was desperately worried about my child but completely utterly helpless to help him. We couldn’t cuddle him or care for him at all. He was laying in a cot in the intensive care nursery in just a nappy, with strange hospital blankets on him and a cannula in his hand – a drip giving him fluids and antibiotics, heart and blood pressure monitors on his chest and feet and a NG (nasal gastric) tube in his nose draining any green bile from his stomach, being looked after by strangers. That first week he slept a lot, barely even opened his eyes – I was desperate to see them open, for him to be awake so we could get to know him.

My husband and I deal with things like this in very different ways. I google – I need to know everything to prepare myself for every possible outcome, every term, every treatment, every side effect – all of it. I feel helpless otherwise talking to the doctors.
We spent 12 long days in Southampton hospital neonatal unit, and the worst part of those days was the lack of a diagnosis, the unbearable weight of not knowing anything.

We were admitted on the Thursday and Bee was immediately nil by mouth and put on the fluid and antibiotic drips, and 9 hourly rectal washouts – by Sunday they were happy with how he was doing (that he was decompressed and his stomach no longer distended) and started feeding him again. He had unfortunately lost a lot of weight at this point as a result of being nil by mouth, I don’t know his lowest weight but I know he lost more than a lb.

By Monday when they were happy that what was going in one end was coming out the other (being washed out) I was finally allowed to breastfeed him directly. And on the Wednesday they did the first rectal suction biopsy.

The rectal suction biopsy is the gold standard method for hirschsprungs diagnosis. It involves a specialised gun type machine which sucks the first few cm of the bowel in and takes very small slices out to be examined by the lab. They told us it would take 5 working days for the lab to come back to us with the test results.

The lab would cut the samples into lots of thin slices, stain them different colours and look for ganglion cells. The absence of ganglion cells is the defining feature of Hirschsprungs disease. Ganglion cells tell the nerves to relax and allow the bowel to open and stool and gas to pass through, without them the bowel is permanently contracted and the stool and gas unable to be expelled from the body. 

Bizarrely despite it taking 5 days for the results of the biopsies to come back not an hour after they had done them a surgical sister took it upon herself to sit me down and convince me barney had the condition. She even presented me with a pre-laminated alert card personalised for him which said ‘this child has Hirschsprung’s disease’ on it. I know we all cope with things differently but I still don’t understand why she did it as we didn’t have any test results for a definitive diagnosis at that point.

The first biopsies it turned out were inconclusive – we found this out on the Friday. They weren’t deep enough so there weren’t enough layers of cells for the pathologists in the lab to see what they needed to. After a lot of chasing from myself more were done the following monday.

Over that weekend the doctors and nurses had begun preparing us for life at home with Barney. They explained that as Barney had this condition when he was better and stable we would be discharged and able to take him home but that we would have to perform his medical treatments (washouts/irrigations) ourselves and bring him back a few months later for an operation. So they began training us to do his washouts once a day. We had already watched them do so many on him at this point that we weren’t scared – we knew it would be straight forward enough to learn.

Digression: hospitals at the weekend are not places you want to be. This is because there is just a skeleton crew of over worked medical professionals working. Things like repeat tests and test results don’t happen, they have to wait till Monday. Because for some bizarre reason we feel that medical problems are a 9-5 monday to Friday thing. Even the maternity ward – who I had been told could help me out and keep an eye on how I was doing – told me off as they had no staff and one midwife there to look after several labouring ladies!

Rectal irrigations/washouts are not something I thought I would ever do, and admittedly my husband is much better at doing them than I am. I panic when Barney cries and wriggles and then I can’t concentrate and carry on. One person guides the tube into his bum, while the other person pours 20ml of saline in to a syringe tube attached to the end of the tube every 5cm further in it goes. The second person also does a tummy massage (to get it all moving) and the saline then washes out all the poo and trapped gas. Whilst doing this ideally you have a third person to hold the poo jug, mop up wees and put dummies back in his mouth. Sounds easy – well the older Barney gets the harder it is – the more he’s aware of his surroundings and the more wriggly he gets, the more he kicks off, cries and tries to leap frog away, or just kick the poo jug over!

I know what your thinking it sounds like an enema/colonic irrigation – they used to be all the rage for socialites on a detox/cleanse. But the difference is with a rectal washout you go in at set lengths (5, 10, 15cm) and do a 20ml saline wash at each one, moving further in each time (we would wash up to 45cm) ensuring the fluid you put in and any stool comes back.

Top tips (for anyone having to do these):

* Ryles catheters have more holes so work way better than ng tubes, and they are clear so you can see when you have found poo (a blockage etc.) and need to wash more.

* Dummy dips – use a dummy dipped in breast milk or formula to calm a crying child.

* If that doesn’t work use a bottle of milk or heck the breast itself. I have contorted myself and bent over meter high tables to feed my hungry upset child while we washed him out.

* Play music – distracts the child, amuses the adults. We would put on Spotify playlists of film soundtracks and play guess the film – my husband is scarily good at this game.

* iPad/iPhone with nursery rhyme videos – amuses the child.

* Use kitchen roll – Barney loves to wee during a washout.

* Bend the child’s knees up towards their chest (this is the poo pumper – baby yoga move) this helps expel gas. But don’t try this until they have had the six week check up and had their hips checked by a GP.

* There is a spot on the child’s left hand side just under their ribs if you massage here it really really helps to get things moving.

*Don’t do it straight after a feed as the massage will make them vomit.

*Don’t do it when the child is really hungry, they will just be more agitated.

Anyway once the hospital was satisfied we could do these washouts  adequately they finally said we could go home.

On the day we were discharged we finally met Barneys consultant. Not the on call one we had met when he was admitted, the actual Gastro-intestinal (GI) specialist. He came and chatted to us and answered our questions and finally actually explained the condition!

Thank god for sisters or I would have gone mad waiting 12 days for someone to explain it to me. My sister had very kindly come the first weekend we were in hospital to see us and meet Barney. And talk us through his condition. For some reason this had really got the on call surgical consultant’s backup – when my sister gave me informed questions to ask I was told by the consultant – “don’t believe everything you read on google” to which I replied my sister has told me about the condition, “who is she?” and “what does she do?”she  demanded. “Who does she work for?” I was interrogated – had I stumbled into a wormhole and woken up in North Korea? I honestly don’t think they were used to patients actually asking informed questions and saying anything other than yes, so they got pissed off at us!

The GI consultant  told us they were 99% sure barney had the condition. And he told us that the first biopsys we had been told were inconclusive were actually showing that he was missing ganglion cells – but they still weren’t 100%. Why they couldn’t have told us that days earlier when they found out – I have no idea!

And then we left. Hopped/skipped/galloped out of that fucking place. As fast as we bloody could.

We had to go back three days later for his formal test results from the second biopsy. They were conclusive – he had Hirschsprungs – I knew by this point he did. So I didn’t cry.

I had had two weeks of crying and sobbing in hospital rooms next to my son in intensive care. I had to look at the positives – he was feeling better, we had him at home, he was feeding well. I decided then that I had to try and get through this by thinking about it step by step. And just think about the next step – rather than 5 steps ahead.

Step 1: get his care transferred to a better hospital with a more specialist doctor, close to family for support

Step 2: get all his medical supplies setup to be delivered to our new home.

Step 3: meet the new consultant.

Step 4: get a surgery date.

Step 5: get more biopsies (this step was unexpected).

Step 6: get Barney MRSA free (again another unexpected step – but a debacle I will explain another day).

Step 7: get through the operation.

Step 8: get through the recovery.

Step 9: I don’t know…..it’s all dependent on how he copes and recovers following his operation. (Weaning, vaccinations, potty training, constipation, incontinence, hirschsprungs enterocolitis are all possible hurdles).

P.S Although as you can probably tell from this post, I wasn’t overly ecstatic at the manners or level of professionalism of all of the people we encountered on our journey, I will always be extremely grateful to the midwives, nurses and doctors at Frimley Park Hospital who identified Barnaby had this condition and by doing so saved his life.

They thankfully worked out quickly that something was wrong and started treating him. I have spoken to many parents who were not as lucky as us, who were instead discharged and sent home and had much more complicated (almost fatal) journeys as a result.