Jack was born at Mass General on Tuesday, April 13, 2010 at 7:29 am weighing in at 9lbs 7oz and 22 inches long. He was a beautiful healthy baby boy or so we thought.
In the following 24 hours Jack had been very fussy while eating and did not want to eat very much. He then began to spit up green bile and he had not passed his meconium. The doctors started to get concerned and decided to take him to the NICU Wednesday afternoon. They came back to our room later on that evening with a surgeon, Dr. Allan Goldstein. They explained to us that with some testing they found that Jack was backed up and did not have to ability to defecate. Dr. Goldstein then introduced my husband and I to our new world, the world of Hirschsprung’s Disease.
Jack’s colon had been very stretched out and therefore the first surgery, at 16 days old, was a colostomy.
This was to give his colon time to retract back to it’s normal size. Then at 4 months old he had his second surgery, the pull-through. During the next couple of months following we had to use a metal dilator and insert it into Jack’s rear end to keep the newly attached area from healing too tight. We also had to deal with horrific diaper rash because Jack’s rear end hadn’t been exposed to poop for the first 4 months of his life. One of the nurses introduced us to a 3 step system including barrier film, nystatin powder, and isle’s paste. This helped and eventually it healed over the next few weeks.
One of the things the doctor had warned us about is enterocolitis, an inflammation on the colon.
He told us that it is very common for children with Hirschsprung’s Disease to get it. Well, Jack had gotten it 3 times all resulting in hospitalization for about a week or so until they could get the inflammation down. One visit also included a blood transfusion because they noticed that Jack was losing blood internally due to his colon tissue being irritated. These visits occurred over an 8 month period. After the third hospitalization, the doctor had us come in for some other testing to see if there was a stricture, a narrowing at the reattachment site caused by the scar tissue. There was a stricture found and it was decided that Jack would have a third surgery, a re-pull through. Jack did very well after the re-pull through.
We were hospital visit free for just about a year and then Jack started having symptoms of enterocolitis.
We were in the hospital twice in a 14 day period. After the second visit Dr. Goldstein consulted with a GI doctor to see what the next step should be. They suggested Jack come in for a Anorectoal Monometry and a scope of his colon. They explained that they put a balloon into his rear end and test the pressure of the sphincter. If it was too tight they would give him some Botox to loosen up the muscle around the sphincter. The Botox worked a little too well. We were changing Jack’s diaper about 10 times a day for a couple of weeks and was accompanied by regular basis from being backed up. Jack was getting backed up because his stool is not evacuating on its own and at home we had to put a catheter in his rear end and empty out the contents of his colon for him 2-3 times a week. We recently administered another dose of Botox but with only half the dosage. It has worked well so far with slight irritation around Jack’s rear end. He still cannot feel when he has to poop but we have made it apart of our nightly routine that Jack sits and poops on the potty and that is working for Jack right now.
Even though Jack continues to struggle with symptoms of Hirschsprung’s disease you would never know it. He is our little hero and fills our hearts with joy every day!