So where did I get to with my last update..ahh yes bees constipation. Although I wouldn’t call it constipation – constipation generally conjures up visions of old men straining on toilets while reading a broadsheet, it’s not something you think of as serious. But unfortunately with Hirschsprung’s disease it is. Constipation for Bee doesn’t mean not pooing regularly it means not pooing at all. It also means being at much higher risk of getting Hirschsprung’s Enterocolitis (a potentially fatal infection).
As a parent there is nothing worse than seeing your child in pain or distress and not being able to help them. This for us reached a critical point at the end of June, we were in a situation where for months we had been sent back from the GPs and consultants with ‘try upping the dose of x or try giving him Y,’ and nothing had worked. He wouldn’t eat, he wouldn’t drink, and when he did he would projectile vomit everything back up.
The symptoms of Hirschsprung’s Enterocolitis are subtle in that they could apply to more than one illness, or you could wrongly downplay them if you looked at them individually rather than stepping back and seeing the picture as a whole. Lethargy, vomiting, fever, diarrhoea, green vomit, distension of the stomach. Bee has had some of these symptoms before from nasty gastro bugs.
So with his lack of eating I was worried and vomiting and distended tummy I was getting quite worried. We couldn’t get his washouts to work properly because all of the equipment we had was from when he was a baby (eating only breastmilk) – to put it bluntly the tubes were just far too small to wash out solid poo with.
I rung his consultant and got nowhere – no one even rung me back. The GP had already told us he was out of his depth and didn’t know what to prescribe next. So I tried the children’s nursing team – I asked them for bigger tubes and explained why. They questioned if our decision to go back on washouts had come from a doctor or not, I explained that we couldn’t get through to anyone and didn’t know what to do. They rung Norwich hospital and found out our surgeon was away. But Norwich hospital were worried about Barney and wanted him to be seen by a local hospital. So off we went to our local hospital, to sit in the children’s A&E ward for 5 hours on a Friday night. By this point we had already managed to flush some poo out of him so had got him a little bit less distended. All they did was take his obs (heart rate, oxygen saturation and temperature) and send us home with a note to go to Norwich the next day – they had no idea about Hirschsprung’s, or Hirschsprung’s’s Enterocolitis so didn’t have the first clue how to help us.
So off we went to Norwich, Barney had perked up massively after we had flushed some poo out of him the night before so we felt it was a bit pointless but hoped we might actually get some proper help from a paediatrician, who actually knew his condition. We did see a paediatric surgeon who felt his tummy, told us it was gas and to wash him out once every four days if we really had to.
Every four days…can you imagine going four days without being able to poo or even pass gas? How bloated and uncomfortable you would be?
We eventually managed to see Barnaby’s surgeon later in the summer. He was shocked when he saw how distended Barnaby’s stomach was and he sent us for an X-ray. The X-ray showed that Barnaby’s bowels were full of poo, and that his rectum was stretched.
I wanted to scream! I had been in touch with Barnaby’s surgeon as soon as he had started getting constipated. I had wanted to keep on top of it…for this exact reason…I didn’t want him to get so constipated that his bowels/rectum got stretched. But yet this is where we had ended up.
His surgeon sent us home with a plan to up his laxatives and put him on a intensive washout/irrigation schedule. With the hope that we could get his rectum to shrink back to a normal size, and then slowly wean him off of the washouts – and hopefully then with all the laxatives he was having he would poo by himself.
I’d like to point out here that getting your child to drink movicol is not an easy thing. I tried everything, mixed it with all sorts of stuff – it tastes like salty water – not something a mostly breastfed 18month old with a sweet tooth wants to drink. So usually I spent the day syringing it into his mouth with a calpol syringe.
During these months I tried a few different things. Like going gluten free – to see if it was maybe a intolerance that was triggering the constipation. I just couldn’t get my head around the fact he had pood like a boss for 9 months and then stopped!
Anyway none of these things worked. Bee still wouldn’t eat, we would both end up in tears at dinner time. I wanted him to eat and thrive but he just had no appetite at all.
Doing washouts isn’t easy on a toddler. They don’t understand what it is or why your doing it. And if they don’t want to comply – then it’s not going to happen. A lot of the time we were lucky – Bee twigged pretty quickly that the washouts made him more comfortable and gave him relief. He would lay and watch Disney films on the iPad while we did it. I’d like to add here washouts are always (at the very least) a two person job, sometimes even more. And I was so lucky my husband was always around to help with these, and if he couldn’t because of work my parents would help me.
We had the normal hiccups – accidentally kicking a jug full of poo over the carpet, getting sprayed in the face, getting poo under your finger nails.
One of the issues we had was getting the right size tubes from our community nursing team, and getting enough of them. As I said in my last post attempting to washout a child who is purely on a liquid diet of breastmilk or formula is totally different from washing out a child on solid foods, which could have chunks and block the tubes.
We had to change our technique, the poo would no longer run out of the tubes with the help of gravity alone. We had to very carefully pull back on the syringe and pull it out. Being soooo careful not to pull hard against anything resistant – as that would mean suctioning in the bowel wall and causing a bleed.
This went on till November, in November he was put under a general anaesthetic to examine him and try and find what was wrong, his surgeon took biopsies to look for a transition zone (area still affected by Hirschsprung’s which is missing ganglion cells that make the bowels work), he also inserted Botox into his sphincter. This is because with HD the sphincter is still affected by HD, and in some people it’s too tight and can’t relax to poo. So the Botox was to relax it.
This procedure however didn’t work – I never really thought it would. The biopsies came back normal. And the Botox just caused him to smear constantly, which made him very sore, but he still couldn’t pass enough poo to empty himself. Botox in HD children is extremely hit and miss, and even if it does work it has to be repeated every six months, as it wears off.
At this point I was despairing. Before we were discharged from the hospital the surgeon came to see us and said that if this didn’t work he wouldn’t know what to try next. That night we stayed in an air bnb near the hospital and I was a ball of anxiety. I was restless and terrified.
When we got home the next day I started asking other parents for recommendations for HD surgeons. We decided we wanted a second opinion… well mostly to just switch doctors entirely.
Our GP said to do this we had to go back and see our surgeon and ask him to organise it. He said he couldn’t do it for us. I felt really awkward about this, it was hugely triggering for my social anxiety. But it was what we needed to do, Bee was still on rectal washouts and the surgeon had no ideas about how to get him pooing. The surgeon was actually fine about referring us to Great Ormond Street – and started the process immediately.
This was in December, and we got our first appointment at Great Ormond Street in early January, as Barnaby’s new surgeon wanted to see him urgently.