Ben's Journey So Far

Benjamin was born on June 11, 2008 by c-section. In hindsight, the elective section was a life saver because Ben's paediatrician was in theatre and had his careful eye on Ben right from the start... this new little baby boy looked like he was all head and no body. A scrawny looking lad at 6lb, 3oz and 47.5cm long. Not a tiny baby by rule, but just looked a little 'runty' somehow.

Ben never passed the meconium that most babies move within the first 24-48 hours of life, but more than that, he wouldn't feed and just seemed to have been born 'sleepy'. In his first 24 hours of life, under the caring eyes of the nurses in Ashford's nursery and the concern of his paed., Ben had a NG (nasal gastric) tube inserted for direct feeding. Unfortunately, this caused vomiting and a bit of bloating in the stomach.

Aspirating the NG tube showed bile in his gut... this was a good indication that he just couldn't tolerate milk.

Ben's doctor came in to tell us that he was going to x-ray his stomach and possibly send him off to the Woman's and Children's Hospital. Once the x-ray showed a blockage in the bowel, we were packed up and sent off! What a scary experience.

Anyone who has had a sick child bundled up and sent off in an ambulance, no matter their age, will tell you that it's one of the most frightening things you will see in your lifetime. Ben went from the safe and comfortable humidi-crib in the quiet nursery at Ashford into a big Mansell Power lifter (a specialist neo natal intensive care crib) with a retrieval team to escort him into the PICU at WCH. No room for Mum and Dad with all that equipment, and for a while there wasn't going to be a bed for me at WCH either. The drive seemed to take forever, and then finding him once we got to the Woman's and Children's felt like the longest, desperate search in my 29 years. The halls seemed dark and long, and I was being pushed in a wheel chair with two flat tyres so it was awfully slow and painful exercise for both Simon and I. No one seemed to know how to get to PICU, finally we found a PSA who happily led us all the way to our little man who we hardly knew.

So there, splayed out on some sort of 'work bench' with monitors and leads which looked like they could control a sophisticated robot, lay our little day old son with a big puffy belly. He looked uncomfortable, but sound asleep. I'd love to tell you what happened next, but it's a bit of a blur. Phrases like 'meconium plug', and 'biopsy', 'highly suspicious of something more serious', 'we'll see what he does' were possibly mentioned, but I couldn't say for sure.

In the next week, Ben was given suction biopsies, x-rays, and ultra sounds. He was then given three dilatation's over three days to help evacuate his bowels and plenty of blood tests and time under UV lights for jaundice. We found the stress levels went down a little for us when Ben was finally moved down to NICU. The care was still intensive, but the mood was a little more relaxed and the nurses had a bit more time to explain what was happening with our boy and why.

 Ben's surgeon, in the first meeting with us, told us that he was in high suspicion that Ben was suffering from Hirschsprung's Disease (HD). The biopsy was negative for this, but it unfortunately also ruled out the notion of the much less serious 'meconium plug' theory.

After just a little over 8 days, which felt more like a month, we were eventually discharged. Ben continued to grunt uncomfortably in his sleep, and look like a little old man. I was never completely happy that his nappies were normal, and at 10 days old we had a check up with Ben's original paediatrician Chris Munt at Ashford between his 'real' appointments. He was nice enough to fit us in because Simon and I weren't confident we had a well baby. Dr Munt agreed and sent us back to the WCH via triage. Our good surgeon was overseas, but his registrar recommended we make an appointment with him as soon as he got back. We did, and Mr Christopher P. Kirby took us very seriously and had Ben back in for a biopsy that same week.

So then we had a diagnosis. Ultra Short Section Hirschsprung's Disease.  HD Info HERE  (This diagnosis is later changed at about 1yr old - more on that later).

The plan of attack, since only a very small amount of bowel was affected, was to give Ben a posterior Rectal Myectomy. Basically, splitting of the muscle that spasms closed. Ben had the surgery at 4 weeks old with a 5 day stay in hospital, and for a while, this surgery was a success.

Ben continued to be frustratingly close to 'normal', but the slightest slip up with the diet and we'd have to work so hard to help him 'disimpact' his bowel. From 6 months old he was on a daily adult dose of laxatives and we we're very strict on what he ate and when.

In April 2009 Mr Kirby decided enough was enough and that he'd go back to basics and do another biopsy in May. So on May 27 Ben was found to have more HD bowel. Approximately 10cm+, rather than the original 3-5cm first suspected.

Why was the biopsy wrong on two accounts??? There are a few theories, but most likely is the theory that doing any surgery on HD bowel before 6 months is a trifle risky as some ganglion cells die off right up to about 6 weeks of age. Ben had his original myectomy at 4 weeks, there's every chance more cells died off after this date.

Always thorough, Mr Kirby had Ben come back in for a higher biopsy to make sure they had tracked every cm of HD bowel. This was done the day before Ben's 1st birthday, and we came home and celebrated his 'parole' from WCH with his very first taste of Ice Cream at Nan's.

So 10cm of HD bowel is just a little bit more than can be treated with an extension of Ben's initial surgery. This meant two things. Both very scary prospects for Simon and I. 

1. Ben would need a stoma made to let his bowel recover from a year of being over stretched, giving it a chance to 'heal'.

2. Ben would need 'pull through' surgery.  Simply put, the HD bowel would need to eventually be cut out, and the good bowel needs to be pulled down and re-sewn. Hence, pulling it through.

So on June 24 Ben had his stoma made, a transverse loop colostomy, and now wears a bag which his dear 3 year old sister calls his 'colostoma'. Since then, once the initial pain of the stoma surgery subsided, Ben has been the best we've ever known him to be. He's a happy little man who's so busy I just know I'm going to be in big trouble once he starts walking! His weight gain is improving and I am sure he's taller. But most of all, he can eat without consequence.  He hasn't refused a meal since June, and sometimes eats more than his Dad! I guess he's had some catching up to do.

Now we wait...

and pray.....

Good technique will only get the surgeon so far, and the rest will be good luck. Sometime in late September or early October Ben will go in for his pull through surgery. Sometime after that he will have his stoma closed/reversed and we'll then see how his 'pipework' handles pressure!