December 14, 2008

Update: Dylan's hanging in there, waiting on surgery

Yesterday was a week since we came home from the hospital, the irrigations are torture but are helping Dylan so much. He is back to his normal weight of 36 lbs (he was a little over 40 when he was sick, probably from the stool backed up for so long). Ive come to realize the irrigations arent so bad as long as I can keep him occupied with something else, and I know its needed so I just have to put on my brave face and talk positive to him. He is truly a big boy, and hes being so brave and happy considering the problems hes had.

Other than that we are preparing for the holidays, the kids are getting really excited for Christmas. And I know "Santa" is gonna bring some good gifts and Dylan will get to take some of them with him to occupy him while hes in the hospital. So as of right now, we are 21 days away from heading out to Cincinnati.....nervous of course, but hoping everything continues to go smooth until then.

Best wishes to you all!

December 10, 2008

It's official-Biopsy shows Dylan still has Hirschsprungs Disease

That dreadful anticipated day has FINALLY arrived. Ive been emailing back and forth with the nurse of Marc Levitt's office, keeping in touch every day to see if the results of the biopsy are back. Sure enough I finally received that email with the results this evening. And here they are:


The biopsy results did come back showing that some part of his bowel is aganglionic, meaning that he does still have Hirschsprung's bowel remaining. So Dr. Levitt can perform the pull through to remove that portion of bowel. The next available date is the first full week of January. Will this work for your family? If so we can go ahead and schedule the surgery and send you an itinerary.I look forward to hearing from you.

I have since emailed back with tons of questions, and asking them to call me. Im so shocked by the fact he did fairly well for 2 years and then WAM we are at the beginning all over again....same diagnosis-same surgery. It's really scare to see your child go through the same thing all over again.....we really thought we were out of the woods, and this goes to show were right back where we started. There's no one to blame, as far as Dylan's surgeon that initially did his first diagnosis and pull-thru, I sill have the most respect for him, he helped us in times that other doctors would'nt even listen to me or take me seriously. I honestly have him to thank this time around, if it wasn't for him taking me seriously this problem would have never been found, although it did take someone else to find it. No one is God, and everyone makes mistakes, thats a common fact. Dylan's first surgeon will always hold a special place in my heart just because he is generous, caring, and really takes the time to listen to the patients parents concerns. Again without him we don't know where Dylan would be at the moment, or what kind of shape he would be in.

So we are all set for surgery on January 6th. I'm doing my best to stay positive although so many things have went wrong. Dylan is now in the care of one of the only specialist that treats this rare disorder, and I have faith that he is in good hands and will be well taken care of.

Stay tuned for more updates, thanks to all that are reading!

Cincinnati Children's Hospital-seeing the specialists in colon disorders

Upon arriving at Cincinnati Children's Hospital from the other hospital in Lexington, I felt a great deal of relief because I knew Dylan was going to be in the best of hands. Dr. Marc Levitt has a wonderful reputation, and from meeting him I can honestly say he has a wonderful bed side manner as a doctor as well.

We first had to register, go through Dylan's history and update them on Dylan's current issues. Once this was done, it was time for an x-ray. Not long after was when we first met "the famous" Dr. Marc Levitt. He went over the x-ray with us and said Dylan still had a blockage, he was not to have anything by mouth foods or liquids and instructed the nurses to start irrigations immediatley. He was the man with a plan, his plan was to get him cleaned out that evening and then the next morning get an x-ray to make sure the obstruction was cleared. If everything was clear on the x-ray he wanted a contrast study done first and then later in the day would take Dylan to the OR to perform yet another rectal biopsy. Dr. Levitt explained to me they had the most qualified professionals that specializes in looking for signs of Hirschsprungs Disease and diagnosing it. So the repeat tests were important.

The irrigations worked well and by Thursday morning we were ready for the contrast study, which to ran smoothly and well. Dr. Levitt came and talked to us after veiwing the results and said he could tell by the contrast study alone, that Dylan very well still had a bad part of bowel left from the previous pull-thru surgery he had in 2006. He explained that indeed there was a stricture 50 cm up, but all 50 cm of the bowel from that stricture down wasn't working like it was suppose to which was indeed a sign that this part of the bowel had Hirschsprungs.

We went ahead with the rectal biopsy that evening, those results would prove whether or not Dr. Levitt's theory was true. After that test and he was woken up, they began letting him have clear liquids. My little guy was so thirsty he drunk 2 of the minature sprite cans in less than an hour. We went back to his room and Dr. Levitt then said he could begin to eat.....and eat he did, that morning he had a donut while waiting for his tray to come up from the cafeteria. He had eggs and sausage and I have never seen a child so happy to eat. He tolerated the food well since his colon was FINALLY empty and free of blockages. He later had some lunch, and the nurse came in and told us the exciting news-WE COULD FINALLY GO HOME! But not until the next day...first we had to learn to do rectal irrigations cause that would Dylan's treatment at home until we returned for his what I now call "re-do pull-thru" on January 6th.

Learning to do the irrigations was easier then I thought. Honestly I didn't think I could do it. But when it comes to keeping your child healthy and safe you will do things you never thought possible or things you never dreamed you would ever have to do for that matter.

After all we were sent home on Saturday the 6th of December on a cold, snowie day. We were glad to finally be heading home, but I was scared of having to go home to the irrigations twice a day, and having to wait for the actual confirmation of the biopsy results. Waiting really makes a parent a nervous wreck. This puts us up to date....right now we are tackling those irrigations twice a day, and although its difficult and I feel like I am torturing my child, I know its all for good reason and needs to be done.

More surgery-hospitalized due to obstruction

This post will put us up to date, thanks to you that have bared with me to get all this typed up. I know the below posts were long, but wanted to share Dylan's history from the beginning so it would all make sense.

Early Thanksgiving morning Dylan woke up at 3am holding to his stomach. When I looked I could tell it was distended and could hear it grumbling. Felt of his head and sure enough he was running a fever. My first thought was UH OH, this could be serious this time around. We had been through this before with re-occuring stomach bug episodes but this time it concerned us more knowing that he had a rare stricture, his bowel movements had slowed down, and we were basically not getting the medical attention that Dylan needed, we were waiting and worrying that he would get sick. Sure enough our worst night mare was coming true. All day Thanksgiving day he didn't eat much, he just wasn't himself. Time passed on, he just had some sips of juice and a bite of his favorite snack which was chicken nuggets, all day long. He went to bed early and was up at around the same time the next morning vomitting. I almost expected this just with the way things were starting out the day before. I tried keeping him hydrated, after several times vomitting through out the day I decided to call his surgeon, his office wasn't in since it was the day after Thanksgiving so I left a message and had him paged so he could call. At his previous office visits he had stressed how important it was to watch for signs of infectons or obstructions and I had that gut feeling that this is exactly what we were dealing with. I waited around after a few hours and felt that I needed to get him some kind of medical help, at this point he was weak and seemed life less, just laying around. I took him to his regular pediatrician, explained the problems he was having and he actually had enough nerve to look at me and say "Well mom, looks like you need to get him to Cincinnati sooner, theres really nothing I can do for him". I believe I probably turned every shade of red because I was so mad that he did nothing for Dylan. So here I was with a sick child and no medical help. I could only pray that this would pass and hopefully was just in fact a stomach bug.

The day went on, Dylan still refused to eat. When he did drink it would just come right back up. His dad and I were really worried at this point. I was so let down by his medical professionals that were suppose to be there for him. FINALLY, at 9 pm his surgeon returned my call, I was so relieved that finally I had someone to help. Turns out his pager had torn up, which is why he was just returning my call. I explained what was going on with Dylan, and how it was a little different than the other times he had these episodes, this time we didn't have any diarreaha and usually when Dylan would take these episodes he would have really foul smelling diarreaha. He then gave me his cell number and told me to keep a close eye on Dylan and try to get him to drink as much fluids as possible, if this was indeed a stomach virus is should subside by morning. I was told to give him a call the next morning to let him know how Dylan was. We prayed through the night that he would start feeling better. It was so heart breaking to watch Dylan, he was unlike his usual self. Usually he was the happiest, most playful toddler and this day he was just so life less. He would lay around mostly, throw up a few times, then try to get up and play but when he did he would stop lean against the wall and hold to his stomach in pain.

He didn't stop vomitting through the night, by this point he had nothing left to throw up. I called the surgeon and he told me to bring him to the hospital where he practices which was 3 hours away (yes very sad when you have to travel that far to get medical care). When we arrived at the hospital the doctor had already let them know we were coming and left orders for Dylan. We had blood work, x-ray and was started on IV fluids. Later that evening Dylan's doctor came in reviewed the x-ray and blood work. The blood work didn't show any signs of infection which was absolutely a great start, but then came the bad news, the x-ray showed 3 dilated loops in the bowels which was a sign of an obstruction. He was taken up to the floor and admitted, started on a clear liquid diet and the doctor planned on trying enemas, and then possibly a rectal biopsy to confirm if in fact it was just the stricture causing problems or if it could indeed be more Hirschsprungs left behind from the first surgery. This rectal biopsy was suggested to Dylan's surgeon by Dr. Levitt in Cincinnati, he thinks if a child is still having problems after a pull-thru surgery that this test should be done to make sure there isn't any diseased bowel left. The nurses started with enemas, it did work, which relieved alot of stool and of course pressure off of his stomach. The next day he was taken to OR and had a rectal biopsy done. His dad and I waited nervously for the results, in the mean time the doctor explained to us, that if the results came back and showed that it was just the stricture that he wasn't gonna wait to send him to Dr. Levitt that he would just do the operation himself since he felt this was causing Dylan to be so sick. We kept our fingers crossed, hoping that indeed it was just this stricture and it could be taken out and then my little Dylan could be on the road to recovery. We were certain this would be the case, we really had it in our heads that these results would show this was the only problem. We decided while Dylan was resting and feeling ok, and his grandparents were there by his bed side that we would run home (3 hours away) pack some clothes up since most likely Dylan's surgery would be the next day so we knew we would need more clothes cause we would be there a week or so after the surgery.

While at home, I got the call from the surgeon, my heart racing and my voice shakey I answered the phone as calmly as I could. Sure enough I was overwhelmed with the results, the biopsy showed more confusion, not the results the doctor wanted at all. These results showed the oppostite of what he wanted to see, he was hoping to see good ganglion cells and nerver fibers, which would mean he would be able to go ahead and remove the stricture and Dylan would be fixed. Instead they showed no nerve fibers, which he then explained to me could be Neural Intestinal Dysplasia. These nerve fibers are the nerves that send messages to the brain that the colon is full and indeed needs to empty which signals the ganglion cells to do their job and start moving stool through the large intestine and then out the rectum......So here we were with yet another problem and possible different diagnosis than Hirschsprungs Disease.

Dylan's doctor then decided it was a good idea to call in the "big shots" at Cincinnati, he knew he could not deal with yet another rare problem, and felt that Dr. Levitt would be the only one to take care of this problem for Dylan.

We talked with Dylan's doctor more about the results, and his hopes of Dr. Levitt being able to help Dylan once we got there. He had us transferred the next morning. We were off to see yet another doctor thinking we were in route of finding out the problem and getting it fixed...what we didn't know is that once arriving at Cincinnati Children's Hospital and seeing Dr. Levitt, we were going to get a completely different opinion and a whole other diagnosis.

The start of something else, yet another problem

Dylan conquered his first surgery like a pro, he did have some problems a few months afterwards with entercolitis and scar tissue. After several months of check up visits for dilations and eventually having a balloon dilaton in OR to stretch out the rectum to get rid of scar tissue, Dylan was finally on the road to a successful recovery, or so we thought anyways.

Dylan began struggling with having too many bowel movements a day, it was almost like having diarreaha on a daily basis. He then struck ill with stomach virus episodes, usually these symptoms would only last a day or so but they were terrible. Finally after dealing with these problems on and off for a year and a half we saw his surgeon again in August of this year. I was determined at this point to do anything to help straighten out Dylan's bowel habits. At this point he was two years old and potty training wasn't anywhere in the picture due to his bowel movement being so loose and so often. Dylan's surgeon finally realized what were dealing with, we described all the episodes of the stomach virus symptoms and the fact that Dylan had never had a formed bowel movement. Dylan's surgeon felt that since Dylan was 2 1\2 years post surgery that his bowels should be formed and slowing down by this point. He suggested trying a dairy free diet for a week to see if maybe Dylan had an allergy to milk products and call him with the results. We tried this, there was no change in the bowel movements. He then felt it was nescessary to do a colonoscopy, this would show him the entire colon and identify any problems if there were any.

(fast forward to September)
As parents we wondered if this test was really needed, we wondered if we were putting him through this for nothing but then realized its better to know if there is a problem then to let things get worse. We had the test done, while waiting, I was really positive we would get good news and maybe start on some type of meds to bulk up the stool or even maybe try a bowel management program. Dylan's surgeon came out with pictures in hand, his first words were "Well, I'm glad we decided to go ahead with this colonoscopy. I've found the problem." My heart then sunk into my stomach, here I was being so postive that nothing was wrong and that I was probably over reacting al; along. Turns out his doctor couldn't even complete the colonoscopy, he entered the rectum with the scope and could only get 50 cm into the large intestine before running into an adhesion, possibly more scar tissue but this time it was even more rare, never before had he seen these type of results this high up in the colon. He explained he got a biopsy from this area and was testing to see if this was possibly Inflammatory bowel disease or if it was indeed a rare ischemic stricture, which is a blood vessel that is damaged and dead, which causes scar tissue to build up and no oxygen to get to that part of the intestines.

We waited impatiently and nervously for these results. It was quite scary considering if it was indeed an ischemic stricture Dylan would need another surgery to remove this part of the intestines. His surgeon had admitted himself that he had never treated a case of this before, and he had been a surgeon for 25 years. But of course, being the most rare diagnosis and so uncommon it was indeed the results of the biopsy, this specimen showed this part of the bowel was dead. Dylan's doctor then began setting up for us to see Dr. Marc Levitt in Cincinnati. I had researched Hirschsprung's Disease so much that I had remembered reading about Dr. Levitt and the fact that he was a pro at treating Hirschsprung's Disease, he had done several surgeries, world wide for this rare disorder and help establish the only Colorectal Center for children in the United States. This man dedicated his life to children who have defication disorders. I was very confident that he could fix Dylan's problems.

At this point we were playing the wait and see game. Dylan had a few test done and we were in the process of conversing with Dr. Levitt's office to set up a consult and more test so he could diagnose the problem himself. Like I said, we played the waiting game for the most part, very nerve racking from a parents perspective to just wait things out when your child is possibly sick with a rare problem. At this point Dylan's bowel movements had slowed down to every few days and this was signs of a partial obstruction. We were still in disbelief of the new problems Dylan was diagnosed with. We stayed positive and waited to hear from the medical professionals in hopes that they could get Dylan taken care of before the problem turned into anything more serious.

Part 2 of Dylan's Journey with Hirschsprung's Disease-The corrective surgery

After finally getting the diagnosis of Hirschsprung's Disease, the next step for Dylan was a corrective surgery called a pull-thru. His surgeon described this as removing the diseased bowel that didnt have ganglion cells (that are in charge of pushing the stool through the colon and out of the rectum) then reconnecting the good bowel to the rectum so we could get rid of the colostomy and hopefully Dylan would be a normal child again.

We were skeptical but had confidence in Dylan's surgeon. We scheduled and went ahead with the 4 hour surgery. Recovery was difficult the first few days. Of course he was in pain and very uncomfortable, which is the one thing any parent hates to see. He spent a week in the hospital recovering and began to tolerate feeds and finally had his first bowel movement. Sounds crazy but I can honestly say I've never been so happy in my life to finally change a soiled diaper. I always said some parents take these things for granted, I, on the other hand cherished it because at one point we didn't think this would ever be possible.

Dylan at this point was 6 months old, he was thriving, growing and just a happy baby. A few weeks after surgery he got sick with an enterocolitis episode and was hospitalized for a few days, then required dilations due to scar tissue building up from surgery. After a few months of struggling we finally got our boy back on track and was thrilled that he was on his way to living a normal life just like other kids his age. His life was ordinary, just a developing infant slowly turning into a young toddler and we enjoyed every minute.

At his first birthday we celebrated with joy, because at one point we didn't know if we would see this event. He had several check ups with the surgeon and we discussed his bowel patterns at this stage. At this point his stools were soft and he had several bowel movements a day. The doctor seemed confident though that things would improve and that we just needed to give his intestines a year to heal and work properly. So we waited, time was on our side, and only time would tell.

December 9, 2008

Dylan's journey from birth with Hirschsprung's Disease

All expecting parents have the vision and hopes of having a healthy baby. On the day that Dylan Chase was born (March 27, 2006) he was perfect and seemed as healthy as any other newborn baby. He was a healthy weight of 8 lbs and 7 ozs and 20 inches long. His father and I were ecstatic to have a healthy newborn baby boy, or so we thought.

Day two of Dylan's life began to turn from healthy and normal to odd and uncomforting. The nurses couldn't get him to eat and here he was not even 24 hours old and hadn't passed meconium, which is the plug that holds in the stool while the baby is in the mother's uterus. The nurse had came to me and explained that if Dylan didn't have a bowel movement by the time he was 24 hours old they would have to move him to the NICU. I was a little concerned, but in all honesty I was clueless at the problems that were yet to come, my thoughts were "he has too poop, all babies poop". But that was not the case at all.

We enjoyed our time with Dylan, holding him, cuddling our newly born baby. We never put much thought to the possibilities of him going into the NICU. We went on as though everything was normal and looked forward to taking our new baby boy home the following day. Little did we know that wasn't going to happen.

The day went on, still no bowel movement and still not feeding at all. That evening a doctor from the NICU came in to talk to us, he explained that Dylan should have already had a bowel movement, should be eating, so he was gonna take him to NICU to keep a closer eye on him. By this time I was concerned, but hoped things would turn around on their own over night. That evening the specialist then returned explaining if Dylan didn't have a bowel movement by the next day (meaning he would be 48 hours old) they would have to send him to UK Children's Hospital. This indeed opened my eyes that something was wrong with my baby, the question left was what is it and can it be fixed.

Waiting over night was worrying, I called the NICU several times (annoying them I'm sure) asking if Dylan had yet had a bowel movement. All through the night I got the same dreadful answer "no, nothing". After finally getting some sleep I was woken by the specialist once again, this time saying they did x-rays and there was dilated loops in Dylan's large intestine. Of course I had no idea what that meant, and questioned him with everything that came to mind. He then told me that the signs and symptoms Dylan had was pointing to a rare disorder of the large intestine called Hirschsprung's Disease. I didn't have to hear anymore before bursting into tears, the thoughts running through my head were indescribable, "disease, how did he get a disease, did I pass something on to him, did I cause this". The scariest moment in my life, I had no idea what it was, if it would cause my baby to die, if it was curable, would he be sick for the rest of his life. I had so many unanswered questions. Even the specialist couldn't answer all the questions I had, he, being a specialist still didn't know much about it himself. The only thing he could tell me was sometimes it can be treated with a colostomy. He stressed how important it was to get him to UK and get this diagnosed which was something a pediatric surgeon had to do, and there were no pediatric surgeons at the hospital we were currently at. Dylan left that evening by helicopter, our family cried as we watched the helicopter leave. There were so many thoughts and feelings as they flew away with our baby...all we could do is pray that God would watch over him until we could get to the other hospital to be with him. I prayed for God not to let anything happen to my baby not without his mommy there to hold him, and to reassure him everything would be ok.

After arriving at the new hospital Dylan was on antibiotics and immediately the new doctors, specialist, and surgeon began testing. But we were still not out of the woods, but thankfully at a hospital that was more educated and had the abilities to care of such a rare disorder and sick baby. The first day there, they mainly did test, x-rays, and enemas to try to get Dylan to have a bowel movement. By day two Dylans colon had had enough, while out getting something to eat and then back to the hotel to rest up and shower, we rang the hospital to check on him. I will never forget the nurse telling me "Mrs. Johnson, the doctor has been trying to reach you" she then put the doctor on the phone. Seems while we were gone, Dylan had become very sick. They performed another x-ray and found that his bowels had perforated (torn apart) and he was rushed to emergency surgery. Of course we ran straight to the hospital and into the surgery waiting room. After what seemed like eternity, we saw Dylan being wheeled by in his crib, the surgeon then came and spoke to us. He began telling us that he had to fix a tear in the bowels and that it was still not known what caused the tear. At this point we didn't have the for sure diagnosis of Hirschsprung's Disease and had been told it could be a few other things such as a dead part of the bowel that didn't develop right.

It was a long road to recovery, two weeks to be exact. Since Dylan was a small baby, and had such a big wound from surgery, when he cried his wound burst open. Instead of fixing it the surgeon thought it was best to leave it alone, but to keep it clean and covered. Dylan began to tolerate feedings, and began stooling out of his stoma (the part of the intestine brought through the skin to make the colostomy so his body could dispose of stool). He was in fact improving. Finally after two weeks and five days (and learning to dress and care for his wound) my baby finally got to go home from the hospital for the first time. He was so little almost like a preemie, he had lost weight with the surgery and not being able to feed. We took him home and began nursing him to health and helping him get use to life away from the hospital.

Dylan did fine, growing, learning, and developing like a normal infant. His colostomy was alot for me to take care of, he couldn't wear colostomy bags for a month or so due to his wound needing cleaned and dressed everytime he would have a bowel movement. I did it with pride though, knowing this would be temporary and we just had to have the colostomy long enough for him to grow and gain weight so he could have his corrective surgery which is called a pull-thru, to reconnect the good intestines. In the mean time we traveled back and forth for check ups with his surgeon in Lexington who performed several biopsies. We finally got a diagnosis of Hirschsprung's Disease in June, which was a sigh of relief that it wasn't anything else but still we knew there was gonna be another surgery which was scary to think about.

~This concludes Part 1 of Dylan's journey with Hirschsprung's Disease~

This story will continue in sections because it is so long and I only have so much free time with having 3 kids at wishes to you that are reading.