Big Day of Appointments

Today I'm home from work with a nasty stomach bug. Fortunately my mom is still watching Landon for me and my step-dad drove Carson and Austin to daycare and so I'm able to stay in solitary confinement to avoid passing this to everyone in the house. I've been out of sick days since December and so it is another unpaid day off, but at least it gives me a chance to update on what all happened yesterday in a timely manner.

First, Landon saw his pediatrician yesterday morning for his 7 month checkup. He is up to 20.5 lbs and 28.5 inches. Not surprisingly, with all of the issues we have had since his surgery he has dropped from the 90th percentile for weight down to the 70th. The good news is that his length has only dropped to the 88th percentile, down from 90th as well, and so he is still growing well despite the setbacks.

Next, we had to address Landon's development. I knew that he was starting to fall behind as he had recently missed all of his 6 month milestones. Already knowing that didn't make it much easier to hear though. When the nurse comes in she evaluates Landon using the scale for typical babies. Preemies have an adjusted scale, as do children with certain conditions such as Down syndrome, but there is no adjusted scale for babies that survived 35 minutes of cardiac arrest and have undergone many hours of general anesthesia and two major surgeries. Landon is still expected to fall on the "typically" developing scale. I had to answer "no" to all of the questions regarding his development.

Having had two other children go through well-baby checks, I can already tell that our pediatrician treats Landon differently. In addition to just looking him over, checking ears, throat, genitals, etc. he also does a much more thorough physical examination. He checks all of Landon's reflexes, puts him into different positions to check his strength, and does several tasks to see firsthand how he is doing. He was concerned with Landon's tone. He said that he is very "floppy" and said that he is going to have to add "developmental delay" and "hypotonia" to Landon's chart. Developmental delay is self explanatory, and I was expecting that, but the hypotonia caught me by surprise. Having worked with the populations that I have, the first thing that came to mind when I heard "hypotonia" is cerebral palsy. It means low muscle tone, and can be synonymous for cerebral palsy. Fortunately we were already heading to St. Louis for our afternoon appointment with neurology, which couldn't have been better timing.

After the unsettling checkup in the morning, the neurology appointment was very reassuring. The neurologist was much less concerned than our pediatrician. She said that he only has mild hypotonia in his extremities, but it is more severe in his trunk. She was encouraged by how alert and social he is. Landon successfully completed several tests with blocks. He struggled with passing them from hand to hand and lifting his arms above his shoulders. He also struggled with sitting up on his own, but for short periods of time he can balance hunched over and he is able to reach and pick up objects while doing so.

Overall, she said that Landon is functioning at a 5 month old level. She wasn't very concerned with his tone, she said that after what he has been through it would be much more concerning if he was hypertonic. She felt that it is likely a combination of everything- long hospital stays, 8+ hours of general anesthesia at a young age, being critically ill when he was a week old- that are causing his delays. Everything she said made sense. If most of his issues are in his trunk, that explains why he was keeping up with his milestones until recently. Sitting up was the first milestone that he missed, and so that goes right along with what she said.

So the next step- what do we do about it. A 2 month delay isn't exactly devastating, especially considering what Landon has been through. After all, this is the child that another member of the neurology team once called "Superman" because he could move his extremities and had his newborn reflexes back- even before they saw a perfect MRI. Just like our other boys, all I want for Landon is to be the best that he can be. So we are going to start physical and occupational therapy. I have contacted early intervention and we are meeting on Tuesday to go over financial and insurance information, and then soon after Landon will be evaluated. If he exhibits a 30% or more delay, he qualifies for services and they will come to our home to work with him. If he doesn't qualify, I have a prescription for OT and PT which I can use to get him therapy privately. The only reason that would not be ideal is due to the fact that we live in the middle of nowhere. The closest place that sees babies is 40 minutes away, and so that would be complicated with my work schedule. But we will do what we have to do. In July we will return to see his neurologist and re-evaluate how he is doing.

Last but not least, is the best part of going back to St. Louis. We were able to see the doctors/nurses/physician assistants/etc. that saved Landon's life. I had written and told them we were coming, with every intention of getting there at least half an hour early to go up and visit. But at the last minute John decided to go in to work, since his sick time is not exactly plentiful right now either, and so we were really rushed to get there by 4:30. We signed in in the waiting room at 4:30 exactly, and before I even sat down one of the doctors taking care of him the night that he coded on the floor was in the waiting room looking for us! I am thoroughly convinced that one of the main reasons that Landon survived was because he had so many people taking care of him that truly cared for him. CPR is very rarely successful after as long of a duration as Landon received it, but they didn't give up. And because they made the split second decision not to give up- even knowing how poor the prognosis surely was- Landon is still here having a wonderful life and making us so proud. Even though I send pictures every few months, it was great to be able to visit. Landon smiled and laughed at everyone. We went up to the 8th floor and saw many people that had been there "that night"- as it is referred to. Although we come to St. Louis often to take the boys for fun days, it is always on the weekend when many of them are off work. The best part of continuing to go in for neurology appointments during the week will be to visit! Since we will be going back in July after Landon's first birthday, we are going to work so hard so that maybe he will be able to walk in and actually say "hi." If early intervention asks what our goal for Landon is- that is it. For him to be able to walk in and greet everyone that saved his life, and to eventually be able to tell them "thank you."

Couldn't wait to share!

I know I usually go weeks without updates, but I couldn't wait to share. Since starting the donor milk at 5:00 am yesterday morning Landon started stooling on his own. My mom texted me a picture of a poopy diaper while I was at work, I'm sure my co-workers think I'm about the strangest person to ever live, but it was such a wonderful sight to see! His belly is the most flat it has ever been in his life. Even when I've thought that it looked good in the past he must have been distended becasue it has never looked this good.

Landon is so much happier and more alert. Struggling to digest his food must have been taking an even bigger toll than I realized. He hadn't rolled over since early December and he rolled 4 times last night. He also did a better job of sitting unsupported- although he is already 6.5 months old he is not yet sitting up on his own. When we did the irrigations yesterday there was hardly any stool that came out at all, he had passed it throughout the day. I know this is gross, but just to paint you a picture, on formula- picture cheese sauce mixed with cottage cheese. On breastmilk- picture water with yellow food coloring with a few tiny flecks.

This is a short update, but I had to share. Once again I'm on my lunch at work and I'm out of time. I hope everyone has a great rest of the week.

Repeating the same experiment over and over and expecting different results is the definition of insanity

Things haven't been great lately. In my last post when I said that the vomitting had resolved, I was wrong. He continued to vomit every 2 or 3 days. His appetite also went way down. Immediately following his surgery he had a huge appetite and ate 36+ ounces of formula a day for about a week. I was excited because he gained back all of the weight he had lost, but since then he has only been eating around 15-20 ounces a day. He was gradually losing weight instead of gaining and about a week before our follow-up appointment he started being unable to pass stool or gas on his own. We were irrigating him 4x per day and that wasn't even enough.

On the 11th we went back up to Cincinnati for his follow-up with Dr. Levitt. One look at his abdominal x-ray and he was sure that Landon had enterocolitis. This was very upsetting because we hadn't had an episode since we started going to Cincinnati when he was 2 months old. So now we're doing a month of antibiotics and continuing the frequent irrigations. On top of all that, Landon was also really tight where they reattached his remaining large intestine to his rectum and so we have to dilate. The smallest rod is the size of a sharpie marker, the largest one is the size of a magic marker. We have to insert them for one minute twice per day. He hates it and screams in pain. It takes one person to hold him still and another person has to do it. It's horrible, but it's what we have to do so there aren't any other options.

It is really bothering me that he has enterocolitis again. Since his first episode where we nearly lost him we have always been able to keep it under control when he gets it, but he had gone so long without getting it I thought maybe we were done. And we had been irrigating him several times a day which should have prevented it, so now I'm worried that we no longer are able to keep it under control. We go back for another follow-up on February 8th. He will still be on antibiotics so we expect to find out that the infection is gone, but hopefully it doesn't return.

Either way, once again I feel like we have lost a lot of control. We did all we could and we weren't able to manage it. It is definitely time for a change. I ditched the Culturelle and started Landon on a much stronger probiotic, VSL 3. As far as the poor appetite and weight loss go, there wasn't a lot that we could do. What makes babies gain weight? Fat and increased calories.. which are also constipating. You can't get a child constipated that is already battling dangerous intestinal infections. The stool gives the bacteria even more places to grow and take over. So it's risky to attempt to change formulas, and it might not even help. All I could do is beat myself up over not being able to breastfeed. While we were still in Carbondale I had two lactation consultants helping me with no success, they saw me pump for 30 minutes with nothing to show. The last thing I was told before we left was that due to my blood loss during the c-section (they had to give me 2 units) that my body just might be working on other things. On the 6th day of Landon's life when he went into cardiac arrest in my arms for such a long duration I quit pumping. Who knows what would have happened if I would have kept at it. Once he recovered, he did well on formula with his colostomy and so it wasn't incredibly urgent situation. I knew that breast milk would help him and so I tried the Yaz-Doperidone protocol to re-lactate for 2 months with no success. Once again, I was disappointed, but not a life or death situation because he did well on formula with the colostomy.

Unfortunately, since the pull-thru, his tummy just can't handle it. What options did I have, spend hundreds of dollars on the meds to try the protocol again which would probably have no success, or spend $5 an ounce to buy milk from the milk bank, knowing I could only afford to feed him about 4 ounces a day- and that is with cutting back on some major expenses. After battling with my pride I finally reached out and asked for help on my Facebook page. The response was overwhelming.. it has brought me to tears on several occasions. Enough wonderful ladies have already donated enough to give Landon 16 ounces a day for the next month. Many have commited to pumping the best they can to try to keep giving me more. If I can get 4 people with 4 ounces a day to spare I can continue giving Landon at least half breastmilk. Production can be inconsistent and you never know what is going to happen in life, but I think I've found enough. God bless all of those wonderful people. I know this is going to make such a big different for Landon. This could very well be the key to helping Landon become strong enough to finally kick enterocolitis for good. At the very least, it will be easier to digest and will give his poor intestines a break. It is amazing what people are willing to do to help others. I've given many of our donors a brief description of what happened with Landon and what issues he faces, but they really have no idea how great it is what they are doing for him. For our whole family! It has been incredibly stressful watching him get sick over and over and wondering if there was something I could have done differently to make this stop happening. This could very well be it. Thank you so much if any of you are reading this. There are no words to express my gratitude.
I'm at work right now and my lunch is over, but I will update in a few days. This is day 4 with no vomit, so keep your fingers crossed! I hope things will finally get better. As always, please keep Landon in your prayers.

Post-op Update

Well I'm just now getting around to updating.. things have been busy.

Right after I posted that last update Landon spiked a 103 degree fever, and that was with Tylenol. Then his heartrate climbed into the 200's and was staying there. I, of course, was terrified that he was septic, but they gave him some Motrin and his heartrate went down along with the fever. They assured me that although it is unusal to spike a fever that high following surgery, the fact that his heartrate dropped down along with the fever was a good sign. Fortunately the fever didn't return and that was the end of that scare.

He stooled for the first time 2 days earlier than expected. There was some gas that lingered in his small intestine, though, and so on day 5 post-op he was able to drink Pedialyte. Day 6 he started taking formula again, and day 7 we were discharged. We stayed in Cincinnati and explored for 2 more days, and then day 9 we went home. It was earlier than expected, and we made it home in time for Carson's birthday on the 23rd.

Unfortunately, the 23rd turned out to be more eventful than I would have liked. Landon started vomitting violently and having awful diarrhea the consistency of water. We were afraid of enterocolitis and couldn't irrigate him that soon after surgery and so we had no choice but to bring him back to Cincinnati. My dad went with me so that John could stay here and give the other boys a halfway normal Christmas. When we got there, everything was a mess. They hadn't put through the orders for Landon to be admitted like we had discussed so we ended up waiting in the ER waiting room for 4 hours. This was while Landon was getting severely dehydrated, not to mention coughed and sneezed all over by tons of kids with the flu, RSV, and God knows what.

Finally they saw him in the ER and got him admitted. As I expected, getting the IV in was horrible because he had gotten so dehydrated. They had to dig, and dig, and dig.. at least 10 different times. It was a horrible thing to watch just wishing they could do it to me instead.

They did a full work-up, labs, x-rays, stool culture, and everything was fine and so we were able to leave Christmas eve night. We later found out that the stool cultire came back indeterminent for c-diff and so they wanted to treat him with Flagyl as a precaution, otherwise assuming it was just a nasty GI bug.

A few days later, the same thing started all over again with the violent vomitting and diarrhea. Another hospitalization resulted, and everything looked fine besides his electrolyte balance being off from being dehydrated. The stool culture was repeated, and this time negative for c-diff which meant either he never had it or it cleared up extremely fast.

We went back home, and he then he would be fine for about 2 days and then vomit again. The diarrhea continued for around 10 days before it finally cleared up. The vomitting has only recently resolved. They are hypothesizing that due to the Hirschsprung's and a recent surgery on his intestines he just hung on to a GI bug way worse than a typical baby would. I was worried that he lost nearly 2 lbs. between the surgery and illness, but in only a week he has gained a pound of it back and so I'm sure a month from now there will be no evidence that any of this ever happened.

We go back next Friday for a follow-up and to find out if we have to start doing dilations. If you don't know what those are, I'll give you a hint: it is what it sounds like. Dilating his... you know.. the area he had surgery on. It won't be pleasant. I left out of this story a huge piece regarding what ended up happening with Endocrine in Cincinnati for his MEN-2 testing, it was a huge battle and a long story. I will add that sometime later just to provide some entertainment. I hope that everyone had a great Christmas and New Years.

Post-op

Long time no update! I can't believe I didn't update before Landon had his surgery but I have been so busy that I didn't get a chance. Everything went well.. now let me back track-

Saturday we left to come up here around noon. For some reason we have been having bag leaks about every 6 hours for the past couple of weeks even though I do everything the same every time. I have my suspicions that about half of them are defective.. But anyway, even though we put a fresh one on before we left we didn't even make it to Louisville, KY (3 hrs away) before he was leaking all over the place. We stopped there to clean him up and walked around their mall (a really nice mall, by the way!) and then we were back on the road.

Two hours later we made it to Cincinnati. Our first pleasant surprise was our hotel. I had booked our room through the hospital and had looked at their price range chart and selected the cheapest one I could find within 10 miles. Even though we had gotten a discount, you don't usually get much for $50 a night and so I was prepared for the worst. It was actually one of the nicest hotels/rooms I've ever stayed in, and we usually spend $120-$150 a night on rooms! It had an indoor pool and was walking distance to tons of shopping and restaurants, plus due to unusual weather it was 60 degrees out and so we were able to take advantage of that and explore the area. Fortunately I had already booked the same hotel for the few days following Landon's release from the hospital and so we can look forward to returning.

Sunday morning we came in and Landon was admitted to the hospital. He was put on a clear liquids diet and they inserted an NG tube to give him GoLytely, which should really be called go violently since he kept blowing off bags every 15 minutes no matter how often we emptied them. His bowels got cleaned out and then we went down for surgery yesterday morning. His surgery was scheduled for 11, but having been through surgeries in the past it was no surprise that they were just taking him back at 12:30. It took an hour to prep him and then 3 hours for the surgery, as expected.

At 4:30 we were called in to one of the conference rooms to talk to Dr. Levitt and find out the specifics. The first thing that we found out was that the part of his colon used in the ostomy was in fact good. He said there was a tight band around the ostomy and thought maybe that was why it had not been emptying like it should causing Landon to keep getting enterocolitis. That turned out to be great news because it meant less colon to lose. Landon was able to keep two-thirds which is more than was expected. Once you lose more than half you run into problems with dehydration and so it was good that Landon was able to keep so much.

Everything has been going well so far, but Landon is in a lot of pain. He had a tap block which wore off around 3 AM and ever since has been pretty sore. He is getting IV pain medication and Valium. He has an NG tube in suctioning his stomach, and currently has a nasal canula for extra oxygen which should be gone by the end of the day. We were warned that today will be the worst day for Landon as far as pain goes, so we are trying to keep him resting. After making it through today we will wait for him to have his first bowel movement sometime between tomorrow and Friday and then they will do an x-ray to be sure that he emptied his bowels. If everything looks good with that then he will begin eating again, which is the first step working our way towards leaving the hospital.

The only thing that has not gone well so far was the plan for genetic testing. Babies with Hirschsprung's often have RET gene defects, one of which is known as Multiple Endocrine Neoplasia Type 2 (MEN-2). Just under 5% of children with Hirschsprung's have that specific defect, which causes malignant tumors on the thyroid and adrenal glands. If the defect is discovered then there are steps that can be taken to minimize the risk of cancer, but it is not currently standard practice to perform genetics testing on Hirschsprung's disease patients. I had requested that the vials of blood be taken during surgery to avoid extra sticks and blood draws and that was the plan, but for whatever reason Endocrine decided not to do it without telling me. They are coming by any time to talk to me about the tests and I assume to try to tell me that they are not necessary, but I want this testing done and now I'm frustrated that Landon has to have extra sticks because they randomly decided to deviate from the plan and didn't feel the need to talk to me about it first.

I'll update again soon and let everyone know how Landon does. Here is a picture of him being his normal happy self right before going back for surgery.

Phone Appointment

Hello again! Today I had a phone appointment with Dr. Levitt, Landon's colorectal surgeon in Cincinnati. This was my first time speaking to him aside from exchanging a couple of emails nearly 2 months ago when Landon was staying so sick and I contacted him for a second opinion. I was pretty intimidated by the thought of speaking to him, he is pretty much a "celebrity" in the Hirschsprung's community. People travel from all over the country- even other countries to see him because he is by far the most experienced surgeon when it comes to Hirschsprung's. Anyway, I guess I expected him to be arrogent, and to my surprise he was very down-to-earth and friendly. He didn't make me feel like the crazy, over-protective mother that I am.

Rather than typing a long paragraph that's difficult to follow, I'll list the questions I asked and his responses:

Me: After reviewing Landon's records, what is your opinion on the reason that Landon went into cardiac arrest on day 6 of life?
Dr. L: It is hard to say with the information that is provided and without seeing him around the time that happened. There are several types of complications that can arise from Hirschsprung's associated enterocolitis (HAEC) that could have led to that event.
Me: So you do think it was related to HAEC, though?
Dr. L: Yes
Me: That leads to my next question- all the literature available states that HAEC is nearly impossible to get while a leveling colostomy is in place. On the other hand, it is fairly common after the pull-thru procedure. Since Landon has had so many issues with the colostomy when the risk is so low it makes me nervous for what will happen when the risk is significantly higher....
Dr. L: I suspect that there is a problem with the colostomy, that the passageway is too narrow to allow him to empty completely. I would expect improvement after the pull-thru, you may or may not have to continue doing irrigations.
Me: The irrigations have been very effective, doing them 2-3 times a day has kept him healthy. But I'm concerned with how quickly he accumulates bacteria. Is there any danger of bacteria going into his blood stream when you open up his large intestines for surgery?
Dr. L: As long as the proper procedures are followed through before the surgery, no. (Referring to giving him nothing besides clear liquids the day before and doing frequent irrigations in the hospital before surgery)
Me: Which type of pull-thru procdure will you be doing?
Dr. L: Swenson. In my experience it has shown to be the cleanest approach and the best way to be sure that none of the affected colon is left behind.
Me: Landon will be having the surgery right at 5.5 months, but he is currently approaching 4 months and getting to the point where he could start solids. Do you have any recommendations on whether it would be better to start solids and get him used to them while he still has the colostomy or if it would be better to hold off?
Dr. L: Either is fine. You can do the same as with your older children, it shouldn't make a difference.

That concluded our conversation. I feel reassured that everything will be okay and much more prepared to go do the surgery now. I can't believe it is barely more than a month away. At least now when we have bad days with the ostomy (bag falls off in public and leaks, huge messes, bags won't stick, etc.) there is light at the end of the tunnel. If Landon's colostomy was functioning like it was supposed to and kept enterocolitis away I really don't think I would be willing to do the pull-thru yet. I would do it eventually since it is a quality of life issue for him, but not yet, not at 5 months. As difficult and expensive as the colostomy care has proven to be, I would do anything for him to just get to be healthy for a while without uncomfortable interventions. Who knows, maybe that day will come sooner than expected after the pull-thru. That child has done nothing but beat the odds so far, why would he stop now?

Save the Date

We officially have the date for Landon's pull-thru/colostomy reversal surgery- Dec. 10th. We will drive up there on the 8th and stay in a hotel, and then first thing the morning of the 9th he gets admitted and placed on a clear liquid diet with frequent irrigations through the colostomy and his rectum. The surgery is sometime mid morning on the 10th, and then he stays in the hospital 7-10 days before discharge. After discharge they recommend we stay in Cincinnati a couple more days, and so again we will stay in a hotel and explore what there is to do in Cincinnati. I'm hoping to be home by the 23rd, which is Carson's (our oldest son) birthday. In case we aren't back in time we are having his birthday party on the 1st before we leave. It will be sad if we don't make it back for his actual birthday, but that was the way the scheduling had to happen. Aside from all of the other reasons that Landon needs prayers for his bowels to wake up and for him to have a quick recovery, that is another reason. So please keep him in your prayers until then.

On October 25th I have a phone appointment with Dr. Levitt, the surgeon doing Landon's procedure. I'm asking a long list of specific questions about everything. One concern I have is that anesthesiology wants to do a phone consult with me on Dec. 5th, just 3 days before our scheduled departure to Cincinnati. They are concerned with Landon's history of a previous cardiac arrest. Landon did go through over 4 hours of general anesthesia for his colostomy surgery and did fine when he was only 2.5 weeks old, but that was considered a life saving surgery and so they were more lenient on the conditions they would perform the surgery in. This surgery isn't absolutely necessary, yes Landon is doing perfectly, yes he could live his whole life with a colostomy, but this is a quality of life issue. I'm hoping to gain insight from Dr. Levitt on how likely he feels that it is that anesthesiology will either shoot this down or scare me out of doing it by discussing risks. I'm pretty nervous and anxious for both phone consultations.

Landon is still doing great. Still off antibiotics, still no infections. We are still doing irrigations 2-3 times per day, but we haven't tried to stop. Doing them is manageable and so it isn't worth the risk to stop doing them for experimental purposes. He is still gaining weight like crazy. All 3 of our boys had these matching shirts, but Landon's was in size 6-9 months. I put the other 2 boys in theirs, but figured Landon's would be huge on him. My mom was over at my house and insisted that I try it on him anyway and it fit! He's only 3.5 months old and it actuually fit! He is right on track with the size his brothers were at the same age, despite everything he has been through. Not to mention that most Hirschsprung's babies have some degree of difficutly absorbing nutrients and growing. We have been blessed 100 times for every unfortunate event that has happened and I am so thankful. I will probably update next after the phone appointment next week and let everyone know how it goes and what Dr. Levitt has to say.