David's Hirschsprung's Story - Part II

You can read about the first part of our journey with Hirschsprung's here.

Picking up where I left off, we scheduled the biopsy to look for evidence of Hirschsprung's Disease for June 8 and I tried not to think about it too much beforehand.  The procedure involves scraping a very tiny piece of tissue from the colon that is then tested for the presence or absence of ganglion cells.  Ganglion cells are nerve cells in the intestines responsible for moving things along.  Hirschsprung's Disease is the result of a person not developing ganglion cells in part of or all of their large intestine.  If ganglion cells are present, the patient likely does not have Hirschsprung's.  If not, then the patient is determined to have Hirschsprung's or needs to be tested further.

The necessity of sedation during the biopsy made things a bit more complicated.  The procedure itself is only 15 minutes and painless but with the anesthesia needed for it to happen, it was an all morning event.  No food past midnight (no nursing past 2 a.m.), a long wait in the GI surgical suite, the procedure itself, wake-up, and then recovery.  I have to say the staff on this floor on this day were wonderful.  It was so comforting that Brian and I could hold David, walk him into the surgery room ourselves, and be right at David's side while they gave him the anesthesia and then he was out.  I will admit it is an incredibly horrible feeling to watch your precious little one's eyes roll back as the gas works and seeing firsthand how powerful medicine can be (and yes, I did burst into tears as we left the room), but I would MUCH rather be there with him, experiencing it with him and reassuring him, than not.  We were allowed to wait right outside where the biopsy took place and as soon as they were done, they wheeled him out and we could see him and sit with him.  The recovery nurse was wonderful and as soon as David began to stir a little bit later, I picked him up and nursed him.  The wake-up was vastly different than what they had prepared me for...screaming, thrashing, etc.  I am sure that was because we were right there and I could nurse him and comfort him immediately.  Once he was fully awake, they checked him quickly and we were able to leave.  From the time he was done with the biopsy, it was only about an hour until we left the hospital.

About 2 weeks later, we FINALLY got our appointment for the follow-up and results.  Why they can't or won't tell you these things over the phone is maddening!  When the doctor finally came in, he gave us the verdict: David's biopsy showed no ganglion cells and therefore it was suspected that he did, in fact, have Hirschsprung's.  Silly as it sounds, I was not ready for that.  Everyone had made it sound like it was so unlikely and I was the one who was pushing for the test but still it took me by surprise.  The satisfaction of proving a mother's intuition right was lost for the realization that this was a big deal.  This wasn't just something we could ignore and hope would correct itself on its own.  It wasn't something I could fix with a special diet or vitamin.  The doctor gave us the names of some of the surgeons at Children's Hospital and I was told I needed to call and schedule a consult and a full-thickness biopsy to confirm the Hirschsprung's diagnosis.  I left the office, cried a bit and then started to figure out with Brian what the plan would be.

We met with Dr. Glick at Children's Hospital in Buffalo.  We talked to an intern for a while who took our history and checked David (we didn't even know if it was Dr. Glick at the time...they're much too busy to fool around with things like introductions and such).  Dr. Glick came into the room and asked us some brief questions.  He was quite certain that David was "just constipated like 98% of the other kids who are sent to me for Hirschsprung's".  This was with the positive biopsy results in his file.  We were told we needed to schedule David for a barium enema and then the full-thickness biopsy.  I was prepared for him to not have the greatest bedside manner because the referring doctor had warned of us of such, but it was still startling to see how rushed we were with our questions and we left after scheduling those tests, unsure of what in the world was even going to be done during those tests.  It was up to us to actually learn what those tests are because he wasn't going to be bothered by explaining them.

The barium enema was a week or so later. I was incredibly nervous.  I knew I couldn't be in the room for the xrays and learned that morning that they would take about 10 minutes to complete.  I didn't know if I could handle him being strapped to the table while they inserted the barium and took the pictures that they needed.  Thank God for several wonderful technicians who were so so good with him.  I waited right outside the room and could hear them talking with him and working with him and he didn't cry once.  I had prepared him beforehand as best you can a two year old.  I told him how they would be taking pictures of his tummy and that he would get to lay on the table and see the big camera, etc.  I know it wasn't an enjoyable experience for him, but thanks be to God, it was so much better than I thought.  We were able to go home soon after.

The next biopsy was scheduled for July 7.  We felt like this would be pretty similar to the first biopsy.  After all, they were basically doing the same thing, just taking a slightly larger and deeper sample to test for Ganglion cells.  We had to do the fast again and this time we couldn't get him scheduled until noon.  NOON.  Because apparently that was when our doctor got the O.R.  We had to go to the surgical floor this time because we were now working with the resident surgeon.  The wait was long and hard but thankfully, the surgical suite waiting room is well stocked with lots of toys.  The process was more complicated than the last time...first see this nurse, then get your gown on, back to the waiting room, come see this nurse, now you can come downstairs to the holding room, now talk to the anesthesiologist, etc.  Once we were down in the holding room, things got very difficult very fast.  

I tried like the time before to make sure I could be there right when David woke and that I could nurse him right away.  The surgeon laughed at me and said "that's not going to happen here."  The anesthesiologist began talking about his breathing tube...wait?  Breathing tube?  WHAT breathing tube??  For a 15 minute procedure?  The surgeon then told us it would be longer than that (despite what we had been told previously).  The anesthesiologist told us that he would do 'his best' to do the least amount of anesthesia necessary.  We weren't convinced.  Dr. Glick told us that "everyone gets treated the same here. If we don't, then we make mistakes."  Really?  So, you don't look at the patient and his or her needs...you treat the child having major heart surgery the same way you treat the baby going in for a simple biopsy.  That is incedibly logical, right?  Dr. Glick was so incredibly callous that I was in tears.  I think the one medical student felt sorry for me and she came back to check on how we were doing.  I asked her to never become like him.  That whole waiting time is now just a painful blur on that difficult day.  They took David away and he wasn't thrilled but he wasn't too upset either.  The idea of him being with strangers who would then have to put him under and I couldn't be there to reassure him was killing me.  The wait felt like forever.  I was in tears for much of it and paced for the rest.  After about half an hour or so, Dr. Glick came back out and said everything went fine and that he should be waking in a few minutes.  He explained a bit about what to expect and went into a little bit about his barium enema results.  Apparently, his whole colon was very distended from dealing with the Hirschsprung's for so long.  It was so hard to concentrate when all I wanted to do was run throught those ugly surgical doors and hold my baby.

Dr. Glick left us and we waited for the okay that David was waking and that we could come see him.  And we waited.  About 15 minutes later the two students came out and said that it should just be a little bit.  So we waited some more.  A full hour after we spoke to Dr. Glick, STILL no one had come to get us.  I was more than nervous.  Brian finally went in and asked about David and suddenly it was just a few minutes and Brian was able to go see him.  We switched off a few minutes later and I could finally go in and hold my little boy.  He was so groggy and his throat was so scratchy.  Apparently, they had put in the breathing tube, despite it not being at all necessary.  He just wanted to keep sleeping (after all, not only had he just had anesthesia, it was his normal nap time!) but we needed him awake to make sure that all was okay and so we could bring him upstairs and ultimately home.  I held him and tried to help him stay awake while gently pleading with the nurse to send us upstairs.  During that time, she casually mentioned how she had administered some morphine to him.  Huh?  Morphine?  For what??   The doctor had assured me that this procedure would cause no pain (there are no pain nerve cells in the intestines).  The only thing that could have been bothering him was the scratchy throat caused by the unnecessary breathing tube...but even that certainly wasn't worthy of MORPHINE.  Her response was "well, I read that he had a full-thickness biopsy and that sounds like it could be painful, so I gave him some morphine."  REALLY?? So he wasn't really in pain, you just THOUGHT he might possibly be so you gave him a powerful drug??  Obviously, I get a little riled up about it.  No wonder the poor kid couldn't keep his eyes open.  I was livid that we were not even asked about that and the helplessness of having others make huge (to us) decisions was so frustrating.

Thankfully, we were sent upstairs soon and I just carried my little boy in my arms.  It took a while and several nurses checking him but FINALLY we were able to get the go-ahead to get him dressed and take him home.  It was such an exhausting and horrible day and all we wanted was to be home.  I can't even imagine if he had been there for more serious reasons and I thank God that we were able to get out of there when we did, despite the fact that it was hours after we were expecting to leave.  There were certainly many parents and children there dealing with things far greater than we were.  We scooped up our baby and got out of there as quickly as we could, picking up the older boys on the way home (and yes, that involved me bursting into tears when I saw my sister who had been watching them for me).  What an awful day.  David perked up a bit that evening but was ready for bed early.  His throat stayed scratchy through the night and for much of the next day but other than that, he was just fine from then on.

We made the follow-up appointment for the next week, assured by Dr. Glick that the results would be in by then.  They were and Dr. Glick came in and told us that yes, again the biopsy had revealed no ganglion cells and therefore, David did have Hirschsprung's Disease.  He told us that he would like to try to handle this a bit differently than normal.  His idea was that if David had short segment Hirschsprung's then possibly his body would respond to easier treatment.  This would involve Botox injections around his sphincter.  If that relaxed him enough to be able to go on his own, then Dr. Glick would perform a sphincterotomy, basically a small cut in the sphincter muscle.  The Botox trial would last about three months and would need to be repeated just to be certain.  He also said that despite the fact that injecting the Botox would only take about five minutes, we would need to go through the whole surgical suite process through the hospital.  ("Because I need my lights".  Of course.)  He didn't describe the sphincterotomy too much and we left knowing that we had a lot to research and think about before making any decisions.  He did schedule us to come in to learn how to do bowel irrigations.  Apparently this is pretty standard for Hirschsprung's children and is done on a daily (or more) basis to (supposedly) lessen the risk for colitis and keep them cleaned out.

Brian and I left the appointment and spent the next week or so learning about the various methods of treating Hirschsprung's.  I knew I wanted a second opinion and to see another surgeon.  There was no way I could deal with Dr. Glick being responsible for my little boy unless it was absolutely necessary.  And I wasn't so sure about the process he was encouraging to treat David.  Brian and I learned about the standard treatment for Hirschsprung's which is pull-through surgery.  The affected part of the colon is removed and the healthy part is reattached in the hopes that the child will then be able to function normally.  I felt lost and tried the best I could to find other options for his care.  We debated between Strong in Rochester, Pittsburgh and Cleveland, the places near us that were known for pediatric surgery.  We eventually were led by our pediatrician to try Strong Hospital in Rochester, NY and she gave us the name for Dr. Pegoli, the chair of general pediatric surgery for the hospital.  I made an appointment there and we waited to see what his thoughts would be.

Wow...have to stop again.  This is sooo much longer than I thought it would be but I will continue on soon with the third (and hopefully last) part of this story of ours!

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