FINALLY!

After being on this roller coaster much longer than I would have liked, I think we have finally turned a corner. Many times we have seen a slight improvement here or there for a few days or even weeks at a time, but never before have we seen such drastic, steady progress.

So I guess I have some back-tracking to do to catch everyone up..

On Monday, the 15th, Landon had his Botox injections. We were told that it would start to work over the next few days, and that in a week if we had not seen any changes then it was not successful. After 2 or 3 days we saw very subtle differences. Landon was passing what appeared to be normal looking stool (which was a first, his stool has always been completely liquid and very undigested looking.) We could tell during the irrigations that the Botox had loosened things up like it was supposed to, but despite the improvements, Landon's bowels were not functioning any better. We still had to do 2 very long irrigations of 3000-4000 mL of sodium chloride and it still took 45 minutes to an hour to get him cleaned out. After waiting the full week, I emailed Dr. Levitt and let him know that we could see changes, but nowhere near to the extent that we had hoped. He forwarded my email to GI and they decided as a team to try something new. They recommended we start a low dose of a stimulate laxative called Senna once a day.

For those that know me, they know how crazy this makes me. I'm a very natural parent. Since having the boys, I stopped using household cleaners containing chemicals, starting buying all organic, steroid, hormone free foods, and the boys use special chemical-free bath products. Carson or Austin neither one take medications on a regular basis, and when they do become sick we don't "over medicate", we only use Ibuprofen if a fever is very high. So you can imagine what a change this has been having a child with a chronic medical condition requiring some form of medication his entire life. Medication has just became something I've had to learn to deal with. I do my best to avoid what I believe to be the more potentially harmful medications in favor of safer alternatives, but at the end of the day there is only so much I can do.

I had to push my worries aside and just deal with the Flagyl, as much as I didn't want to, because without it Landon ended up so sick on his birthday. Laxatives were another area I wasn't thrilled to get into, because it's great if they work, but then you are likely looking at long term use. I researched Senna, and found it to be safe enough that I had no objections. It is something available over the counter, and to my surprise (and delight!) it costs less than $5 for a 12 day supply. We are used to every trip to the pharmacy costing anywhere from $40-$200, so $5 is such a great surprise!

The particular form of Senna that we chose is Exlax chocolate squares. Landon takes one square (15 mg) at bedtime following his irrigation. After starting this, it was like a miracle. I could mistake Landon for one of our other boys when they were this age! His freqency decreased from 10-12 tiny BMs per day to 4-5 large ones. We have used the Senna for almost 2 weeks now and this is continuimg to improve. Yesterday, Landon only had 3 large bowel movements. He stayed clean throughout the morning and afternoon (which also helps his diaper rash issue) and then had his bowel movemnts over the course of the late afternoon and evening.

Since the Botox, we have been tapering off the irrigations and Flagyl. We dropped the morning irrigation and only do one at night, which takes less than 800 mL of solution because Landon is already nearly empty! We are down from 4 doses of Flagyl per day to only one, and next week we are attempting to go off of it altogether. This is the first time in a long time that I have been optimistic that this could work. Even if it doesn't,  we can at least go back to a small maintenance dose which is an improvement from where we've been.

Landon's development is also coming along. He is still struggling with the balance and strength to walk, but has made several other major acheivements. He is eating 100% table food, with only a few dietary restrictions. We introduced some dairy and small amounts of whole wheat, so he is no longer completely dairy or gluten free. We are still avoiding constipating foods, but letting Landon at least try everything he can and then trying to add more laxative foods to balance it out.

Another great accomplishment Landon has made is drinking from a sippy cup. He still takes a few bottles a day to stay hydrated, but he is taking in about half of what he needs through a cup. I'll end this post with a cute picture of what else he has figured out he can do with a sippy cup ;)

Curve Ball

Well, everything has pretty much taken a 180 degree turn since my last update. I received an expected phone call with very unexpected news last Monday. The biopsies of Landon's colon DID contain ganglion cells. Dr. Bernabe said that she wasn't comfortable removing what appears to be good colon that for whatever reason isn't working. She said that she could offer us Botox injections, but wasn't sure if that would help.

I contacted our nurse at Cincinnati to let her know what was going on and Dr. Levitt emailed me back. He said that he wanted to do a rectal exam under anesthesia with a sigmoidoscopy and possible upper GI endoscopy. We began the process of getting preauthorization from our insurance company and were prepared to wait until at least the end of the month before an opening would be available.

Next came another surprise when the nurse called on Thursday and said that they had an opening on Monday (today.) Then, an hour later called back and asked that we go up the next day for some testing beforehand. Fortunately, my stepdad was able to make the trip with all 3 of the boys and I. We made a round-trip in one day, and so way too much driving with way too many small children. It ending up being worth it to shed some light on the situation.

On Friday, we went down to radiology and had a variation of the contrast study done. They inserted the solution differently and took views from additional angles. Dr. Levitt felt that the narrowing that appeared was inflamation from enterocolitis, not colon that wasn't functioning. To be honest, I'm so exhausted, frustrated, emotional- you name it- that I pushed for doing an ileostomy and getting Landon healthy and his bowels healed. Dr. Levitt respected my wishes, but managed to talk me out of it. He convinced me that trying botox first could be the way to go to see if relaxing Landon's sphincter helps move things along. He left open the possibility that the ileostomy might still happen depending on what condition his colon was in once they took a look inside.

Last night, John, Landon, and I came back up prepared to stay up to a week with the possibility of being able to do everything outpatient and coming back home today. I'm happy to say that everything turned out best case scenario- upper GI endoscopy, sigmoidoscopy, and rectal exam all three were perfect. Dr. Levitt gave Landon 4 botox injections and we were happy to be out the door in under 4 hours. Now we wait a few days to see if the Botox works. If it does, then we will repeat it about every 3 months as long as necessary. If it doesn't work, then we will go back to our discussion on the ileostomy. We are also awaiting results of a some biopsies taken of the small intestine which are expected to be normal. I'll update when I get a chance on how everything goes.

Who, What, When, Where...

Hi everyone. I have a lot of updates. Let me start off with the good news:

Landon is officially a year old now! We celebrated his birthday on Saturday and he was able to enjoy his first cupcake during a Superman themed birthday party.

 

 



What a year it has been! Even though there are still gross motor and feeding issues that are being addressed, he is doing so well for what he has been through. He is getting stronger every day and is doing better at standing on his own and taking steps with a push-toy. The most amazing area of all, no doubt about it, is his communication. His receptive vocabulary is HUGE. This child understands everything. Any time we have a conversation about anything, he is looking around the room at what it is we are referencing. He is also starting to consistently use several words. He calls me "mama", says "hey!" to tell people "hi", and labels his favorite thing of all time- "fish" by saying "fffffsshhhh." What a blessing he has been to watch him grow and learn.

Now for the not-so-good news. Thank God we celebrated his birthday on Saturday, because on his actual birthday on Sunday he became very sick very quickly. He was projective vomitting and became lethargic. We rushed him to the emergency room at St. Louis Children's and they conducted testing and diagnosed him with enterocolitis. He was still taking the full dose of Rifaximin, but it turns out that it was no longer working so well. They started him on IV Flagyl and we noticed improvement after several days.

We were reunited with his surgeon at St. Louis, Dr. Bernabe. It made me realize that there are advantages to being in St. Louis vs. Cincinnati: 1) much shorter drive, 2) our older boys and other family can travel up to visit 3) they more thoroughly know Landon's history and take enterocolitis more seriously, 4) pretty much everyone knows us from his "episode" a year ago today, 5) we are able to visit the doctors that saved Landon's life and express our gratitude and show off how well he is doing.

Although those are all important advantages, number 3 turned out to be the most important. Dr. Bernabe decided to do further testing to see why Landon is continuing to have such a poor outcome following his pull-thru in December. The first test was a lower GI study that was done on Wednesday. It didn't take the radiologist long to see that something was abnormal. Landon had a narrowing in the rectosigmoid region and into the descending and transverse colon, consistent with Hirschsprung's disease before ANY surgery is performed. Dr. Bernabe then came in and explained that although it is rare, sometimes following the pull-thru surgery an area of the colon doesn't receive sufficient blood flow and can actually become aganglionic. We have biopsies from both St. Louis and Cincinnati to confirm that good colon with ganglion cells was pulled through back in December, and so there are no questions about there possibly being a surgical error. So we proceeded with a biopsy, which was done yesterday morning. Dr. Bernabe went in and performed a full-thickenss biopsy under general anesthesia to confirm that a section of Landon's colon is aganglionic again. Landon did very well during the procedure and we were able to come home last night.

So what does this mean for Landon? I've created a diagram to better illustrate what I'm about to explain in hopes that maybe it will be a little less confusing.

 

Here is a colon/large intestine. The area circled in blue has already been removed (15 cm), with the area directly above pulled through and reconnected with Landon's rectum. So the area circled in green is actually stretched and is placed where the area in blue used to be. Fast forward to Wednesday, when we saw the results of the lower GI study. The area in green (another 7 cm) is now narrowed and is not allowing stool to pass. Once removed, Landon's entire descending colon and part of his transverese colon will be gone. This is no longer consistent with short-segment Hirschsprung's disease, resection to this extent occurs with long-segment disease.

 

Those are the facts. Now we get into the opinion of the surgeon. Dr. Bernabe feels that since the ascending colon receives much better blood flow, we may have the greatest chance at a successful surgery if she removes Landon's entire transverse colon and connects his ascending colon. That would leave approximately 25% of his colon in place. That part sounds okay. The downside is that she uses the Soave method. Everyone should do their research on the Duhamel, Soave, and Swenson methods and form their own educated opinion. My opinion is that the Soave method is inferior to the Swenson method and yields a higher complication rate of enterocolitis and re-do operations due to what is known as the "Soave cuff."

 



For that reason, I can't say that I'm comfortable doing the pull-thru in St. Louis. I do feel that St. Louis is better managing Landon's care and is being more thorough, so I asked about the possibility of just going up to Cincinnati for the re-do pull-thru (Swenson method) and then coming back for Dr. Bernabe to manage his care. Dr. Bernabe stated that she would be comfortable with that, and so that is one option on the table.

Another option would be to return to another temporary colostomy. This would be a good option to keep Landon healthy for a while and off of antibiotics, and we would definitely do this in St. Louis. The disadvantages are that the colostomy was not easy to care for, and this would guarantee at least one more additional surgery to reverse it at a later date.

There are a few other options that we are not ready to consider (permanent colostomy, pull-thru with loop ileostomy). So basically what it comes down to is a decision to go back to a colostomy for a while to get Landon healthy and stay out of the hospital for long periods of time, or to go straight to a re-do pull-thru and keep trying to make this work. Both options have advantages and disadvantages that we are weighing. Right now we are deciding what to do, who is going to do it, where it is going to be done, and when it will be done.

Results

As promised, I am finally getting a chance to get on here and update on what happened with our visit to Dr. Davis last week. We found out the results of two tests that we had sent off, a Genova stool study and a Genova nutrition evaluation. Both offered a lot of information- not necessarily good or bad news, just more information as to what is going on with Landon's body.

I'll start off with the results of the stool study. As expected, it showed severe dysbosis. Dysbosis is an overgrowth of bad bacteria and lack or absence of good bacteria within the digestive tract. Even while Landon was on the Flagyl, he still had a severe overgrowth of bad bacteria, and not enough good bacteria (flora) within his intestines to measure. All of that was the news that we were expecting, as Dr. Davis had already been treating Landon with a heavy dose of probiotics and many different herbal supplements for several months now. The news that we weren't necessarily expecting- although also not terribly surprising- was that the stool study also showed a severe overgrowth of several different types of fungus. So now we have added yet another herbal supplement to help eliminate the fungal growth, and if we don't see results within a couple of months she recommends that we do a round of oral Nystatin.

Now on the the nutrition evaluation... it was a train wreck! Landon showed deficiencies across the board of vitamins, minerals, and fats. And since these are things that we know for sure that he is consuming sufficient amounts in his diet, it means that he has malabsorption. The most relevant areas that came up were B vitamins and fats. Dr. Davis prescribed a very high dose of high quality B vitamins and increased his dose of cod liver oil.

Last but not least, was another surprise. We have been battling a strange rash all over Landon's back and stomach for a couple of months. When I brought him in to the doctor they diagnosed him with contact dermatitis and prescribed steroid ointment. The cream cleared it up, but after a few weeks it came back. Once again we applied the ointment and it cleared up, but it kept coming back. We couldn't figure out what was causing this. The usual instigators- laundry determent, fabric softeners, bath products- were out of the question because we already don't use products containing chemicals/dyes/perfumes. After we returned from Myrtle Beach he had the worst outbreak yet. I showed it to Dr. Davis and she was confident that it is eczema. She said that often it can be a reaction to wheat or dairy. The mystery was instantly solved. Although Landon doesn't consume wheat or dairy on a daily or even weekly basis, I am guilty of giving him small bites of these things occasionally. While we were in Myrtle Beach our whole family ate terribly, including Landon. Although I'm not sure yet if it is dairy or wheat that is triggering the reaction, now that we have completely eliminated both things again the eczema has cleared up. 

That sums up our visit with Dr. Davis. Otherwise, everything is still going well. We got our situation with our insurance company resolved so that we can affordibly get the Rifaximin. We actually only have to pay a $40 co-pay for a 10 day supply, which is the same that we were paying for the Flagyl. The only difference is that the Flagyl only costs $40, and so our insurance company was not having to pay a penny. Now, they are having to pay over $800 for their part of a 10 day supply, so $2400 a month. I expect that it will only be a matter of time before they try to deny coverage or force him to move to a different antibiotic although it is working so well. So hopefully next time we try to take him off antibiotics in August it will finally be successful.

As some already know, Landon is having his first birthday next Sunday! It is such a miracle that he is still here after all that he has been through and gets to celebrate something that so many people take for granted every year. He is really doing well with some things- like crawling, pulling up on furniture, waving hi/bye, understanding basic language, and finally babbling and starting to try to say words. Other areas are not so great. His low muscle tone is becoming more of an issue again. He can't stand on his own or cruise on furniture without his legs giving out on him. He is also having a lot of trouble with eating. He gags and chokes on everything that is not completely pureed. We will be bringing both of those issues up at his next appointment coming up, but I'd imagine we will be contacting Early Intervention again to re-evaluate and possibly start back up therapy. We will also be going for his one-year follow-up with neurology soon, but their office is so backed up with appointments that weren't able to get in until the middle of August.

I hope everyone is having as good of a summer as we are so far! Landon is such a little fish and loves to swim already. I've enjoyed the first month of staying home with the boys so much already and can't wait for the next two months!

Start of a Great Summer

Hi everyone. I apologize for the lack of updates, things have been chaotic around here! We just returned from our first vacation as a family of 5. Landon had so much fun seeing the ocean for the first time and crawling in the sand. We managed to bring all of his irrigation supplies, supplements, and medications and stayed on track with everything the whole week we were gone. I will write a longer update later this week to update on the new medication he started and what we find out at a follow-up visit with Dr. Davis on Wednesday. Meanwhile, I will post some cute pictures of Landon enjoying the beach.

Just an update

Hi everyone! First of all I would like to apologize for the lack of updates. Our oldest two boys have started gymnastics and one of them has also started t-ball and so things have been busy lately. Since my last update we finished the 3 month course of maintenance Flagyl and tried to take Landon off of it again. Unfortunately, it was not successful. I reluctantly called Cincinnati, knowing that they would want to put him back on another long course of Flagyl. I explained that although I understand that this is necessary in order to keep Landon alive, at the same time I'm uncomfortable with the damage that it is causing. Flagyl is known to cause neuropathy with long-term use and is also anticipated to be a known carcinogen. Landon has already been on it for 8 months total which is longer than I would have liked.
To my surprise, they actually had an alternative available. Dr. Levitt prescribed a new antibiotic called Rifaximin. Rifaximin is a broad-spectrum antibiotic that is not absorbed into the blood stream, meaning that it only affects the intestines- the location of the problem. It is a much safer drug for long-term use with no known adverse effects. So like myself, you are probably wondering why this drug isn't using more commonly as an alternative to the Flagyl. The answer: cost. I just spent over $800 for a 3 month supply of this medication. Just like any other situation we have encountered, our insurance does not want to cover it because like the Flagyl, it is compounded. Cincinnati is currently working to get them to authorize it so I can be partially reimbursed for the cost, but it is still up in the air. So unfortunately, this 3 month course could be the only course that we are able to afford if our insurance won't cover the cost.
We are still using all of our natural supplements and will be returning to St. Louis soon to find out the results of the stool study that was done. We will also possibly be returning to Cincinnati in another few months depending on the results of an abdominal x-ray that will be scheduled. Dr. Levitt has mentioned doing some more invasive testing to try to find the source of the issues that Landon is having, including a colonoscopy. I just wish that we could find an answer as to why he is having chronic infections to the extent that he is. Even Dr. Levitt, who sees the most complicated cases of Hirschsprung's disease in the world, is beginning to sound a little less confident.

Exciting News

Hello everyone! I'm happy to say I have all kinds of exciting news to share. First of all, we have a crawler! Three days after he turned 9 months old, Landon took off crawling. He loves to chase his brothers and to explore the house. He especially loves going in rooms that he is not supposed to be in (kitchen, bathroom...). Time to bring back out the baby gates!

Next is an update on the visit with Dr. Davis. She took a detailed health history and recommended that we do a nutrition urine analysis and Genova stool study. Both have been submitted, but we don't have results yet. We will be going back up for a follow-up visit at the end of May to discuss the results and if and how it will affect the treatment plan. To make a long story short, she agreed with me on the major points. She also felt that the flora in Landon's intestines is severely disrupted (which the stool study should confirm how and to what degree) and also that if we don't change the plan nothing will change trying to take him off of the Flagyl again in mid-May.

So we now have what I wanted- a new plan. Dr. Davis agreed to work with Dr. Levitt's recommendations from Cincinnati with continuing the maintanence Flagyl until the middle of May and then trying to wean off of it. She also put Landon on a lot of supplements.

 

Here is what all Landon is taking daily:

 

Digestive Enzymes- Gastro Calm 4 caps daily
Sacro B. 3 caps daily (over 9 billion CFUs)
Artic Cod Liver Oil 2 tsp. daily
HMF Replete Probiotics 1 packet daily (150 billion CFUs)
TruFiber 1 1/2 scoops daily
Gen Intrinsic 20 drops daily
Artemesia Int 40 drops daily
RegenRX Small Intestine 40 drops daily

 

The dosages are divided up so that he is taking 1/3 of the daily dose of all of the above in the morning, afternoon, and evening. I want to add a disclaimer that these are super high doses well above what most adults are recommended to take and that no one should give these doses to a child (or especially a baby!) without first consulting a doctor. But with that said, all of these supplements are working very well. Landon is staying cleaned out and it has drastically shortened the duration and amount of sodium chloride solution that we are using each irrigation. We are experimenting now with dropping the afternoon irrigation and only doing one in the morning and one in the evening. Keep your fingers crossed that this is successful because it would make life a lot easier, especially now that the boys have started gymnastics and Carson is starting t-ball.

 

A lot of people have asked how it's going with the donated milk. It is going really well! We have 5 ladies that have donated on a regular basis, and many others that have donated large quantities. Even those that have donated a few spare bags have helped tremendously. We have enough coming in that for over a month now Landon has not had to have formula. He eats pureed fruits and veggies and about 30 ounces of milk per day. If we stay on track with bringing in the milk, I'm really comfortable that we are going to make it until he is 12 months. If we could go beyond that it would be wonderful, but 12 months old is the goal. I'm not sure yet what he will drink once he transitions to something new. Almond milk or coconut milk seem to be the top contenders, but neither provide very many calories- which could be problematic with Landon's off and on struggle to gain weight.

My older boys are refusing to nap and terrorizing my mom's house as we speak, and so this is going to have to be all that I post for now. I hope everyone is able to get out and enjoy the weather now that it finally feels like spring!