Hirschsprungs Family Connections

Bailey was born on 22 April 2012 at 11.32pm in Guernsey. All seemed well and he had his first feed of breastmilk. However, it soon became apparent that something was not right as he was constantly being sick and was uninterested in feeding. Because he was born by c section initially we were not too concerned as I had my first child by emergency c section also and I was reassured that the lack of feeding was normal due to the amount of medication I had been taking (epidural etc).

 

The next day Bailey was still not feeding and had not yet passed his first poo. On the advice of the midwives I went on to express milk for Bailey and we also tried him with a cup of formula, but again the whole lot came back up (all over the auxiliary nurse!). I am sure you can imagine how stressful it was not to be able to get any food into your new-born and although I was aware of mucus issues with C Section babies I had some maternal instinct telling me something else was going on.

 

By the third day all Bailey was doing was sleeping so he was referred to the hospital paediatrician who immediately whisked him into the Special Care Baby Unit for more checks. It turned out that Bailey was severely dehydrated and had a distended tummy. A scan of his bowel was sent to paediatric surgeons at Southampton General Hospital for review and they asked that Bailey be transferred that afternoon, so the paediatrician and a paediatric nurse all jumped in the air ambulance with Bailey and I and we were all whisked away to the Paediatric Intensive Care Unit at Southampton General Hospital.

 

As I am sure you can imagine there were all sorts of worries going through mine and my husband's minds. I was upset about having to leave my three year old and husband behind at short notice but we had to get to the bottom of what was wrong with Bailey so I had to be strong. The paediatric registrar was brilliant and explained that the doctors in Southampton suspected Bailey had something called Hirschsprung's Disease (something I had never heard of before). He explained that this disease only affects 1 in 5000 babies and was more common in boys. He also advised that they would need to undertake a rectal biopsy in order to determine whether Bailey definitely had this disease.

 

Two biopsies and almost two weeks later the biopsy result said “suggestive of Hirschsprungs Disease but inconclusive” and it was only when they took him to theatre for the keyhole surgery that the condition was formally diagnosed. The surgery (called a "pull through") removed part of his bowel which was not functioning properly. The last part of his large intestine was missing the nerve cells so could not interpret the signals that his bowel was receiving so the it was unable to "push" the stool along, this had created a blockage in his colon. While waiting for his surgery, Bailey was having rectal washouts once or twice daily in order to keep him healthy.

 

When the day of the surgery arrived it was one of the worst and most anxious days of my life (luckily my husband and daughter had come to stay for two weeks by this point so I had some emotional support and I had my daughter to look after to take my mind off of the worries). I can't even imagine what was going through my husband's mind at the time but we both had to be strong for both Bailey and our daughter Tia.

 

Thankfully the surgery went really well and they only removed 6 inches of Bailey's bowel (he was diagnosed with short segment Hirschsprung's Disease). Bailey then spent an hour in the PICU and was then transferred to the neonatal unit of the Princess Anne Hospital. He spent several days with fluids, pain relief and antibiotics into a cannula, it was really hard seeing him hooked up to all of the wires and machinery and feeling helpless, however I HAD to remain strong for him and the rest of the family. I continued to express milk in order to keep my supply going to be able to feed him after he had recovered from surgery.

 

Finally about four days post-surgery he was allowed to breastfeed again as had started pooping all by himself! Never did I think I could be so excited to see poo! Only a parent of a child with bowel problems (Hirschsprung's in particular) could relate to how excited my husband and I were! The surgical team had advised that he would need to remain in hospital for five to seven days post-surgery, he did very well and they let us go home on the fifth day.

 

Bailey is now 3 years old and has regular check-ups in Southampton. He is prone to getting dehydrated and it is likely that he will take longer to potty train (he is showing no interest at present). Then of course there is always the risk of him developing Enterocolitis. I believe that choosing to breastfeed Bailey for over 2 years has definitely helped him with his condition due to the natural laxative effect of the milk.

 

My husband and I just take each day as it comes with Bailey and his bowels. He recently had a Botox injection and we have been battling with managing his increased output and sore bottom. Bailey is always cheeky and definitely a character! Who knows what the next few months will bring (more Botox or constipation problems again), doesn't really matter as this is part of HD and we just deal with it as best as we can.