Some may remember that I had mentioned I had some exciting news about several things. Well, I figured I should jump on here and share it. The first subject, and most exciting, is Landon's development.
Two days shy of being 9 months old, Landon is now: clapping, following the command to "clap" when someone says "clap your hands" or "yay", waving bye-bye appropriately, getting up on hands and knees to crawl and rocking back and forth (but not quite crawling yet), scooting on his bottom to nearby objects, pulling up on objects and standing on his knees, imitating "clicking" sounds with his tongue, imitating "kissing" noises, feeding himself using a pincer grasp, and finally rolling over well enough in both directions that he changes positions in his crib overnight. He is also starting to experiment more with babbling. He was starting to babble in early Decemeber before his last surgery, but then it just stopped and hadn't picked back up. Lately, we're starting to hear him putting consonant and vowel sounds together again. Yay Landon!
As far as the other exciting news, we are traveling to the St. Louis area on Tuesday to see another new specialist named Dr. Davis. She specializes in using a hollistic approach, a combination of Eastern and Western medicine. In other words, she looks at the big picture and makes sure that the body is balanced and functioning how it should be, but also believes in using Western medicine when and to the extent necessary. She will be conducting a 2 hour interview on Landon's health and medical history and then doing some screening. We will also be doing a stool study in the near future. Several Hirschsprung's parents that I've met in support groups have had success using this type of treatment approach and have been able to turn their child's health around by keeping a balance in the intestines. I'm really excited to meet Dr. Davis and have high hopes that she will be able to do great things for Landon.
I'm hoping that the stool study is going to be the key to solving this complex puzzle, otherwise known as Landon's health. As most already know, Landon went in to cardiac arrest when he was 6 days old from overwhelming infection caused by (Hirschsprung's associated) enterocolitis. The reason that it caught everyone off guard, even when Hirschsprung's had been mentioned as a possibility as to why he was having the issues that he had, is that enterocolitis very, very rarely develops anywhere near as quickly as it did for Landon, and it is even more rare for it to become deadly in such a short period of time. So the question has always been- why Landon? Why did it develop and progress as quickly as it did for him? I've always had a hunch. I went in for an induction on June 28th. I was in labor for over 2 full days- the whole time I was receiving antibiotics every few hours. It is known that antibiotics kill all bacteria in their path- both good and bad. So basically, when you use antibiotics, you wipe out the flora (good bacteria) in your intestines. I believe that for those 2 days that I was in labor with IV antibiotics pumping through my system, Landon's flora was being completely eliminated. Then, once he turned into a birth position where he would no longer descend, Landon had to be delivered via caesarian- also known to disrupt/prevent establishing a balance of flora. And last, but not least, since Landon was too sick to eat from birth and I was never able to produce a single drop of milk from pumping (even with the help of 2 lactation consultants), even though Landon was technically "breastfed" in that he wasn't receiving formula, he actually did not eat anything for the first two weeks of his life. So there was another knock to the balance in his intestines.
Based on everything I've mentioned above, I believe that enterocolitis was practically welcomed into his intestines and had the perfect environment to thrive and quickly take over. In order to manage the enterocolitis, we have had to use the antibiotic Flagyl, to kill the bad bacteria that quickly takes over in Landon's intestines. The only problem with that, is that in the meantime, the Flagyl is also even further preventing Landon from developing flora in his intestines to balance and naturally fight off the chronic/recurrent infections. We already use a very strong, adult strength dose of probiotics to try to replinish the good bacteria in his intestines. Once we do the stool study, we will have specific information on what the levels of both good and bad bacteria are, what types, and what dosages of the specific strands that he needs in order to bring his intestines up to a "normal" point. Then, in May, once we attempt to take him off of the maintanence Flagyl once again, if he has an adequate amount of flora in his intestines, and if we are able to turn his intestines into a very unwelcoming environment for the bad bacteria to grow and take over, we could finally be on a path for a normal life for him without frequent rectal irrigations and antibiotics.
I could be completely wrong about everything, but I'm willing to risk the time and the money to possibly be closer to finding a solution. I will update next week with news about what Dr. Davis has to say. I hope everyone has a Happy Easter!
Thursday, March 28, 2013
Wednesday, March 20, 2013
A Day in the Life
Hello everyone, I hope everything is going well for all of you. Things have been wonderful around here lately. Last week I was off work for Spring Break, and even with a few inconviences in our home (upper respiratory infections, pink eye) it went very well. I finally feel like our life is managable again, for the first time since Landon had his pull-thru surgery in December. Nothing has changed to make it easier, we are just finally getting settled into the routine for all the extra care that Landon requires.
So what exactly is the extra care that Landon requires? Well here is a breakdown:
4:45 AM: Alarm goes off. I get up and put a bottle in the bottle warmer, prepare his antibiotics, make sodium chloride solution for the irrigation, prepare the area, get all supplies ready
5:00 AM: John and Landon get up. John holds Landon while I do the rectal irrigation. It takes 30-45 minutes start to finish because we must continue until the solution runs clear.
5:45 AM: I give Landon his antibiotics and feed him his bottle. Sometimes he falls asleep and goes back to bed. Other times he plays in his bouncer for the rest of the morning.
6:15 AM: I get ready for work, very quickly! I shower the night before and let my hair air dry overnight because I don't have time for anything else. My routine for myself is very minimal. I also get Carson and Austin up and help them brush their teeth, get dressed, and take their vitamins.
7:00 AM: We leave the house. I drop Landon off at my mom's house first, and then Carson and Austin go to daycare.
8:00 AM: I get to work. Lately, Landon is in good enough health that I can focus on my job and the kids that I see. Sometimes Landon has to be brought to my work around 11:30 for an extra irrigation if things aren't going well for any reason.
2:00 PM: Landon takes his second dose of antibiotics for the day. My mom does this when I am at work.
3:15 PM: I get off work. I usually have at least one errand to run in Carbondale, the larger town that I work in. Sometimes I pick up produce from the natural foods store near the school where I work, sometimes I have to refill Landon's medicine, or sometimes I pick up milk from one of our wonderful Carbondale-Carterville-Herrin area milk donors. Next I pick up Carson and Austin from daycare.
4:15 PM: I arrive at my mom's house. Either my mom or my stepdad goes out to the car so I don't have to unload the boys. First I review what Landon has eaten for the day and how much. Then whoever stays inside helps me with our second rectal irrigation for the day. I'm spoiled because everything is usually already prepared and set up for me when I get there, saving around 15 minutes.
5:00 PM: I get home with the boys. John is usually gone to the gym for at least another hour, so I unload the boys and get some type of educational television show on for Carson and Austin to keep them out of trouble. Landon sits in his high chair and eats puffs while I prepare dinner.
6:00 PM: I mix Landon's two types of probiotics and his supplements he is taking in apple juice and give it to him. He also eats pureed food around this time.
6-7-ish PM: Somewhere in here Landon takes another bottle.
7:30 PM: Bath time for everyone! Yes, all 3 boys take a bath together in the tub. You would do this too if you saw how much easier it is.
8:00 PM: Carson and Austin get ready for bed and go to sleep. This marks the beginning of preparing to make it through the next day...
8:00-9:00 PM: Thaw donated milk (it is all kept frozen), wash and sterilize bottles, monitor amount of pureed food and prepare more if necessary
9:00 PM: Landon plays in his bouncer outside the bathroom doorway while I take a very quick shower. No time to even think about blow drying my hair, so I prepare it the best I can so that it dries halfway tame.
9:30 PM: Last rectal irrigation for the night. Once again, John holds Landon while I do the dirty work. Then John cleans up while I take Landon out to finish our day.
9:45-10:00 PM: Landon takes his HUGE bedtime bottle. Seriously, during the day he is unable to take more than 4 ounces at a time. But he eats 7-10 ounces for this last bottle of the day. Strange, I know.
10:15-11:00 PM: I hold Landon while he takes his pacifier and falls asleep anywhere within this time range. I usually watch television to stay occupied and whenever he falls asleep I put him in his crib and go to bed myself.
So there you have it. Our friends wonder why we are never able to go anywhere or do anything anymore- that's why. When we do venture out we are on a very strict schedule because a lot of these things just can't be done out in public places. Changing Landon is a great example. Most don't know this, but the stool that Landon passes is extremely acidic due to his intestintal issues. No matter how quickly we change him it always makes an acidic burn which is like diaper rash on steroids, and he has this all the time. The only way to keep this from happening is by completing a nearly 10 minute routine with several products that we have to order. As you can imagine, a public changing table is not the ideal spot for this routine to take place. The people that get in line behind us waiting for the changing table don't understand or appreciate why I am spending so much time changing Landon's diaper.
As you can imagine, weekends are much better than our weekday schedule that I described. For starters, no one in our house gets up until at least 6:30 on the weekends and we start our routine much later. We do have to plan our day around the schedule, but Carson and Austin take a nap during the afternoon anyway and so doing the afternoon irrigation and antibiotics that time just works out well since we are home at that time.
I am going to try to post some exciting updates later this week. Landon is doing some really awesome new things, and we have some exciting news about a new specialist that we are welcoming to the team for Landon's care that we will be traveling to see very soon. So keep checking back for an update hopefully sooner rather than later!
So what exactly is the extra care that Landon requires? Well here is a breakdown:
4:45 AM: Alarm goes off. I get up and put a bottle in the bottle warmer, prepare his antibiotics, make sodium chloride solution for the irrigation, prepare the area, get all supplies ready
5:00 AM: John and Landon get up. John holds Landon while I do the rectal irrigation. It takes 30-45 minutes start to finish because we must continue until the solution runs clear.
5:45 AM: I give Landon his antibiotics and feed him his bottle. Sometimes he falls asleep and goes back to bed. Other times he plays in his bouncer for the rest of the morning.
6:15 AM: I get ready for work, very quickly! I shower the night before and let my hair air dry overnight because I don't have time for anything else. My routine for myself is very minimal. I also get Carson and Austin up and help them brush their teeth, get dressed, and take their vitamins.
7:00 AM: We leave the house. I drop Landon off at my mom's house first, and then Carson and Austin go to daycare.
8:00 AM: I get to work. Lately, Landon is in good enough health that I can focus on my job and the kids that I see. Sometimes Landon has to be brought to my work around 11:30 for an extra irrigation if things aren't going well for any reason.
2:00 PM: Landon takes his second dose of antibiotics for the day. My mom does this when I am at work.
3:15 PM: I get off work. I usually have at least one errand to run in Carbondale, the larger town that I work in. Sometimes I pick up produce from the natural foods store near the school where I work, sometimes I have to refill Landon's medicine, or sometimes I pick up milk from one of our wonderful Carbondale-Carterville-Herrin area milk donors. Next I pick up Carson and Austin from daycare.
4:15 PM: I arrive at my mom's house. Either my mom or my stepdad goes out to the car so I don't have to unload the boys. First I review what Landon has eaten for the day and how much. Then whoever stays inside helps me with our second rectal irrigation for the day. I'm spoiled because everything is usually already prepared and set up for me when I get there, saving around 15 minutes.
5:00 PM: I get home with the boys. John is usually gone to the gym for at least another hour, so I unload the boys and get some type of educational television show on for Carson and Austin to keep them out of trouble. Landon sits in his high chair and eats puffs while I prepare dinner.
6:00 PM: I mix Landon's two types of probiotics and his supplements he is taking in apple juice and give it to him. He also eats pureed food around this time.
6-7-ish PM: Somewhere in here Landon takes another bottle.
7:30 PM: Bath time for everyone! Yes, all 3 boys take a bath together in the tub. You would do this too if you saw how much easier it is.
8:00 PM: Carson and Austin get ready for bed and go to sleep. This marks the beginning of preparing to make it through the next day...
8:00-9:00 PM: Thaw donated milk (it is all kept frozen), wash and sterilize bottles, monitor amount of pureed food and prepare more if necessary
9:00 PM: Landon plays in his bouncer outside the bathroom doorway while I take a very quick shower. No time to even think about blow drying my hair, so I prepare it the best I can so that it dries halfway tame.
9:30 PM: Last rectal irrigation for the night. Once again, John holds Landon while I do the dirty work. Then John cleans up while I take Landon out to finish our day.
9:45-10:00 PM: Landon takes his HUGE bedtime bottle. Seriously, during the day he is unable to take more than 4 ounces at a time. But he eats 7-10 ounces for this last bottle of the day. Strange, I know.
10:15-11:00 PM: I hold Landon while he takes his pacifier and falls asleep anywhere within this time range. I usually watch television to stay occupied and whenever he falls asleep I put him in his crib and go to bed myself.
So there you have it. Our friends wonder why we are never able to go anywhere or do anything anymore- that's why. When we do venture out we are on a very strict schedule because a lot of these things just can't be done out in public places. Changing Landon is a great example. Most don't know this, but the stool that Landon passes is extremely acidic due to his intestintal issues. No matter how quickly we change him it always makes an acidic burn which is like diaper rash on steroids, and he has this all the time. The only way to keep this from happening is by completing a nearly 10 minute routine with several products that we have to order. As you can imagine, a public changing table is not the ideal spot for this routine to take place. The people that get in line behind us waiting for the changing table don't understand or appreciate why I am spending so much time changing Landon's diaper.
As you can imagine, weekends are much better than our weekday schedule that I described. For starters, no one in our house gets up until at least 6:30 on the weekends and we start our routine much later. We do have to plan our day around the schedule, but Carson and Austin take a nap during the afternoon anyway and so doing the afternoon irrigation and antibiotics that time just works out well since we are home at that time.
I am going to try to post some exciting updates later this week. Landon is doing some really awesome new things, and we have some exciting news about a new specialist that we are welcoming to the team for Landon's care that we will be traveling to see very soon. So keep checking back for an update hopefully sooner rather than later!
Saturday, March 9, 2013
FAQ
Hello everyone! Everything is still going well with Landon's health. The maintenance Flagyl and frequent irrigations are keeping everything in check with his health and he has had a very good last few weeks developmentally. He is 8 months old now, and he is officially sitting up 100% independently, as in I can leave the room and go do something and when I return he is still sitting up. Not only that, but he has also learned to scoot in that position to get around. When he's on his stomach he is starting to push up onto his knees and elbows and rock, but he can only hold the position briefly before falling to the ground. He has shown interest in pulling up, but he still isn't strong enough to do it on his own. The best milestone he has hit so far, by far, is learning to clap. He understands the command "clap" or "yay" and starts clapping on his own. I'm concerned that he still isn't really making consonant sounds or starting to babble, but the fact that he is showing that he comprehends certain words is reassuring.
Since I don't have much to update, I thought it would be a good idea to address some of the questions we get frequently. Here are some of the questions we often get:
"What is the problem with Landon's heart?/ Does Landon have a problem with his heart?"
No, Landon does not have any problems with his heart. Although heart defects are the most common cause for going into cardiac arrest, his episode had nothing to do with his heart. Landon went into cardiac arrest from overwhelming infection which was the result of an infection known as enterocolitis related to Hirschsprung's disease. Once Landon was resuscitated, one of the first tests performed was an echocardiogram which showed that there are no problems with his heart.
"What exactly is Hirschsprung's disease?"
See my link above for a full description, but to summarize, it is the failure of ganglion cells- the nerve cells that allow the intestines to move waste material through- to migrate to any extent. The entire large and small intestines can both be affected, only the large, or only part of the large. Never the less, wherever the nerve cells are lacking stool cannot be passed through. This causes stool to back up in the intestines and can cause a variety of serious, life threatening complications. The most common complication is enterocolitis- an infection that only those with Hirschsprung's disease are able to get. Our surgeon, Dr. Levitt, has said that a typical person could go a week or more without passing stool, and although they would be very uncomfortable, they would be fine. A person with Hirschsprung's disease, however, is at risk for developing that infection if they aren't passing stool at least once a day. So it is crucial for patients with Hirschsprung's disease to be kept cleaned out, whether it be with laxatives or rectal irrigations, and then at some point the affected portion of the intestines must be removed.
"Does it affect Landon that he had 1/3 of his large intestine removed? Do you need your large intestine to live?"
This is a complicated question, but technically no, you do not need your large intestine to live. The role of the large intestine is to absorb water and certain nutrients from your stool. If you lose less than half, it generally does not make much of a difference in daily life, other than stool is expected to be looser and more acidic. Once you lose half or more, you run into problems with dehydration and certain measures must be taken. Your small intestine is a whole different ball game. Your small intestine absorbs nutrients. Once you start losing parts of your small intestine you risk short gut syndrome, which means that you are not able to take in adequate nutrition from food. IV nutrition known as TPN can be used to compensate for that problem, but life expectancy and quality of life is affected. Fortunately, Landon still has 2/3 of his large intestine and all of his small intestine, so he really doesn't have it nearly as bad as it could be.
"What is the life expectancy for someone with Hirschsprung's disease?"
For someone only affected to the extent that Landon is, it is typical. Since Landon still has most of his intestines the thing most threatening to him is enterocolitis. He is at the greatest risk from enterocolitis for the next 2 years. The longest duration reported between corrective surgery for Hirschsprung's and a case of enterocolitis is 10 years. So basically, in 10 years he is pretty much out of the woods. He will always have to watch his diet and deal with several other nuisances from less than perfect intestines, but he should live a long life.
"...But doesn't Landon currently have enterocolitis?"
Yes. It is known that developing enterocolitis before having the corrective surgery for Hirschsprung's puts one at a much greater risk for developing it again. It is believed, although not proven that the severity plays a role. Before Landon was diagnosed with Hirschsprung's he developed entercolitis so severely that it caused his heart to stop. He barely survived- actually, it is a miracle and not able to be explained medically how he did survive after 35 minutes of cardiac arrest. Since then, he has never been able to shake it all the way. He is left with what is known as chronic/recurrent entercolitis. It is under control with a combination of frequent rectal irrigations and antibiotics, but it never goes away. As soon as we attempt to irrigate less frequently or stop the Flagyl he always develops symptoms within 4 days and has to resume treatment.
"When will Landon overcome enterocolitis?"
That's what I would love to know! We're attempting to stop the maintenance meds again in May, and we'll see how that goes. Any time we stop could be "the time" where he is finally able to stay healthy on his own. "The time" is most likely going to be sometime in the next 2 years. Like I mentioned above, the longest duration between the corrective surgery and an episode of enterocolitis is 10 years. So "the time" should definitely be in the next 10 years, excluding some record breaking misfortune. Right now we are mainly concerned with avoiding negative effects from long-term antibiotic use and the enterocolitis becoming resistant.
"Where does Landon receive treatment, you mention both St. Louis and Cincinnati?"
St. Louis is our nearest Children's hospital. That's where Landon was when he went into sudden cardiac arrest. Doctors on the 8 West floor of St. Louis Children's Hospital resuscitated Landon and saved his life. We stayed in St. Louis until Landon was 5 weeks old and he was finally released after his colostomy surgery. We still go to St. Louis for follow-ups with Neurology, and that would be our first choice in hospitals for just about anything. It is a wonderful hospital with great resources for families staying there long term. BUT...
St. Louis only sees around 10 cases of Hirschsprung's disease a year. They divide the cases among surgeons, so each surgeon operates on only 2 cases a year at most. This is typical of most hospitals. Cincinnati Children's Hospital actually has a colorectal center with colorectal surgeons. All they do is operate on cases of Hirschsprung's disease and other colorectal malformations all day every day. Cincinnati is only a 5 hour drive, and so we decided to let them take over Landon's care for everything directly Hirschsprung's related. The top expert in the world on Hirschsprung's disease is Dr. Levitt, and we are fortunate enough to be able to call him Landon's surgeon. He did Landon's pull-thru surgery and determines Landon's plan of care. I thank God every day that we have the resources available to take Landon to the best of the best when it comes to this disease.
"Are Landon's developmental delays related to Hirschsprung's disease?"
No. This is probably the most common misconception surrounding Landon right now. Hirschsprung's disease is purely a disease affecting the intestines. Yes it can present as part of certain syndromes, but Hirschsprung's alone does not affect development. Landon has low muscle tone which is causing his delays. Is it a result of being critically ill at such a young age? Is it the result of spending around 2 months total in hospitals unable to get up and move around? Would he have had it even if none of those thing ever happened? Who knows. Honestly, I don't really care. Why spend time and energy worrying about "why?" Why doesn't change anything. I prefer to devote my energy towards "what do we do about it?" which is where we are right now. Landon sees a physical therapist and is doing very well. He is consistently staying about 2 months behind where we should be developmentally, but not falling further behind.
Hopefully that helps if anyone has had anything they are curious about. I am happy to answer any questions that anyone has or to cite my sources for any of the information I listed above. The reason that Landon ended up as sick as he did was due to lack of awareness. Hirschsprung's disease only affects 1 in 5,000, and so I understand that it's not going to be plastered all over billboards down the interstate, but no one should end up going through what Landon did. Something has to be done to raise awareness. So feel free to ask me anything, anytime, and I will be happy to answer :)
*EDIT*
I am going to add questions that I get. I just got my first :)
"Does Hirschsprung's run in your family or is Landon the first to get it?"
Landon is the first. The genetics of Hirschsprung's are not fully understood, but John Hopkins University is doing a lot of research right now on the topic. John and I, as well as all 3 of our boys have submitted DNA for their research. Anyway, there are several genes that are known to be involved, the most common being the RET gene. Since we are done having children there is no need to pursue genetic conseling or further testing to isolate the specific defect that Landon has, but if we were to have another child he/she would run a 12-14% chance of also having Hirschsprung's. Unfortunately, Landon has a 25% chance of passing it on to his children.
Since I don't have much to update, I thought it would be a good idea to address some of the questions we get frequently. Here are some of the questions we often get:
"What is the problem with Landon's heart?/ Does Landon have a problem with his heart?"
No, Landon does not have any problems with his heart. Although heart defects are the most common cause for going into cardiac arrest, his episode had nothing to do with his heart. Landon went into cardiac arrest from overwhelming infection which was the result of an infection known as enterocolitis related to Hirschsprung's disease. Once Landon was resuscitated, one of the first tests performed was an echocardiogram which showed that there are no problems with his heart.
"What exactly is Hirschsprung's disease?"
See my link above for a full description, but to summarize, it is the failure of ganglion cells- the nerve cells that allow the intestines to move waste material through- to migrate to any extent. The entire large and small intestines can both be affected, only the large, or only part of the large. Never the less, wherever the nerve cells are lacking stool cannot be passed through. This causes stool to back up in the intestines and can cause a variety of serious, life threatening complications. The most common complication is enterocolitis- an infection that only those with Hirschsprung's disease are able to get. Our surgeon, Dr. Levitt, has said that a typical person could go a week or more without passing stool, and although they would be very uncomfortable, they would be fine. A person with Hirschsprung's disease, however, is at risk for developing that infection if they aren't passing stool at least once a day. So it is crucial for patients with Hirschsprung's disease to be kept cleaned out, whether it be with laxatives or rectal irrigations, and then at some point the affected portion of the intestines must be removed.
"Does it affect Landon that he had 1/3 of his large intestine removed? Do you need your large intestine to live?"
This is a complicated question, but technically no, you do not need your large intestine to live. The role of the large intestine is to absorb water and certain nutrients from your stool. If you lose less than half, it generally does not make much of a difference in daily life, other than stool is expected to be looser and more acidic. Once you lose half or more, you run into problems with dehydration and certain measures must be taken. Your small intestine is a whole different ball game. Your small intestine absorbs nutrients. Once you start losing parts of your small intestine you risk short gut syndrome, which means that you are not able to take in adequate nutrition from food. IV nutrition known as TPN can be used to compensate for that problem, but life expectancy and quality of life is affected. Fortunately, Landon still has 2/3 of his large intestine and all of his small intestine, so he really doesn't have it nearly as bad as it could be.
"What is the life expectancy for someone with Hirschsprung's disease?"
For someone only affected to the extent that Landon is, it is typical. Since Landon still has most of his intestines the thing most threatening to him is enterocolitis. He is at the greatest risk from enterocolitis for the next 2 years. The longest duration reported between corrective surgery for Hirschsprung's and a case of enterocolitis is 10 years. So basically, in 10 years he is pretty much out of the woods. He will always have to watch his diet and deal with several other nuisances from less than perfect intestines, but he should live a long life.
"...But doesn't Landon currently have enterocolitis?"
Yes. It is known that developing enterocolitis before having the corrective surgery for Hirschsprung's puts one at a much greater risk for developing it again. It is believed, although not proven that the severity plays a role. Before Landon was diagnosed with Hirschsprung's he developed entercolitis so severely that it caused his heart to stop. He barely survived- actually, it is a miracle and not able to be explained medically how he did survive after 35 minutes of cardiac arrest. Since then, he has never been able to shake it all the way. He is left with what is known as chronic/recurrent entercolitis. It is under control with a combination of frequent rectal irrigations and antibiotics, but it never goes away. As soon as we attempt to irrigate less frequently or stop the Flagyl he always develops symptoms within 4 days and has to resume treatment.
"When will Landon overcome enterocolitis?"
That's what I would love to know! We're attempting to stop the maintenance meds again in May, and we'll see how that goes. Any time we stop could be "the time" where he is finally able to stay healthy on his own. "The time" is most likely going to be sometime in the next 2 years. Like I mentioned above, the longest duration between the corrective surgery and an episode of enterocolitis is 10 years. So "the time" should definitely be in the next 10 years, excluding some record breaking misfortune. Right now we are mainly concerned with avoiding negative effects from long-term antibiotic use and the enterocolitis becoming resistant.
"Where does Landon receive treatment, you mention both St. Louis and Cincinnati?"
St. Louis is our nearest Children's hospital. That's where Landon was when he went into sudden cardiac arrest. Doctors on the 8 West floor of St. Louis Children's Hospital resuscitated Landon and saved his life. We stayed in St. Louis until Landon was 5 weeks old and he was finally released after his colostomy surgery. We still go to St. Louis for follow-ups with Neurology, and that would be our first choice in hospitals for just about anything. It is a wonderful hospital with great resources for families staying there long term. BUT...
St. Louis only sees around 10 cases of Hirschsprung's disease a year. They divide the cases among surgeons, so each surgeon operates on only 2 cases a year at most. This is typical of most hospitals. Cincinnati Children's Hospital actually has a colorectal center with colorectal surgeons. All they do is operate on cases of Hirschsprung's disease and other colorectal malformations all day every day. Cincinnati is only a 5 hour drive, and so we decided to let them take over Landon's care for everything directly Hirschsprung's related. The top expert in the world on Hirschsprung's disease is Dr. Levitt, and we are fortunate enough to be able to call him Landon's surgeon. He did Landon's pull-thru surgery and determines Landon's plan of care. I thank God every day that we have the resources available to take Landon to the best of the best when it comes to this disease.
"Are Landon's developmental delays related to Hirschsprung's disease?"
No. This is probably the most common misconception surrounding Landon right now. Hirschsprung's disease is purely a disease affecting the intestines. Yes it can present as part of certain syndromes, but Hirschsprung's alone does not affect development. Landon has low muscle tone which is causing his delays. Is it a result of being critically ill at such a young age? Is it the result of spending around 2 months total in hospitals unable to get up and move around? Would he have had it even if none of those thing ever happened? Who knows. Honestly, I don't really care. Why spend time and energy worrying about "why?" Why doesn't change anything. I prefer to devote my energy towards "what do we do about it?" which is where we are right now. Landon sees a physical therapist and is doing very well. He is consistently staying about 2 months behind where we should be developmentally, but not falling further behind.
Hopefully that helps if anyone has had anything they are curious about. I am happy to answer any questions that anyone has or to cite my sources for any of the information I listed above. The reason that Landon ended up as sick as he did was due to lack of awareness. Hirschsprung's disease only affects 1 in 5,000, and so I understand that it's not going to be plastered all over billboards down the interstate, but no one should end up going through what Landon did. Something has to be done to raise awareness. So feel free to ask me anything, anytime, and I will be happy to answer :)
*EDIT*
I am going to add questions that I get. I just got my first :)
"Does Hirschsprung's run in your family or is Landon the first to get it?"
Landon is the first. The genetics of Hirschsprung's are not fully understood, but John Hopkins University is doing a lot of research right now on the topic. John and I, as well as all 3 of our boys have submitted DNA for their research. Anyway, there are several genes that are known to be involved, the most common being the RET gene. Since we are done having children there is no need to pursue genetic conseling or further testing to isolate the specific defect that Landon has, but if we were to have another child he/she would run a 12-14% chance of also having Hirschsprung's. Unfortunately, Landon has a 25% chance of passing it on to his children.
Saturday, February 23, 2013
Quick Update
Not much to update, everything is going about the same.. but I do have some good news to share! Landon had his last check-up with his pediatrician at the end of January, and then a week and a half later went up to see his colorectal surgeon in the beginning of February- as some may remember. He had not gained weight between the appointments and so I was beginning to get concerned. On Tuesday, I took him to get the rest of his 6 month shots (he's still running behind from not being able to receive vacinations while he was so sick the first few months of life) and he has gained a whole pound in less than 3 weeks! Other than that, not much is new, so I thought I would add some new photos.
Monday, February 18, 2013
Maintenance
Well, the plan we made at our follow-up in Cincinnati was short-lived. As I mentioned before, we discontinued the Flagyl and dropped to 2 irrigations a day. By Monday, just 3 days later, Landon seemed off- excessively fussy and slightly poorer appetite- and so I began watching him closely. Then on Tuesday morning he refused to eat altogether and projective vomited at about 6 in the morning. Fortunately I kept what was left of his Flagyl and so I was able to get him back on it right away. I called Cincinnati and spoke with his nurse and she confirmed that I had done the right thing in starting him back on the Flagyl, and she recommended we do 4 irrigations a day until symptoms disappear and then go back up to 3 a day. She later called back and instructed us to fill a prescription for 25 mg of Flagyl per dose (his regular dose is 37.5 mg) and said that Dr. Levitt wanted us to continue the lower dose as a maintenance medication for the next 3 months.
In a way, I'm relieved that we don't have to worry about Landon getting sick again for a while. He will be 10.5 months old by the time we discontinue the maintenance meds. He will be much bigger and stronger, plus that will be more time that he has been on donated breast milk gaining antibodies which are making his immune system stronger. The downside is that I really hate him having to take Flagyl. Studies done on the safety of it have shown that it is carcinogenic in both mice and rats. It has been shown not to be carcinogenic in hamsters, which is good, but who knows which rodent reacted more closely to how a human baby would. Plus there are risks with long-term use of any antibiotic, such as developing c.difficile (c.diff) or creating resistant strains of bacteria. So hopefully he can successfully go off the Flagyl in May when we try again. I have done some more research and have decided to start Landon on grapefruit seed extract, to see if that will help. I also want to discuss trying sodium cromoglycate. There was a study done in Finland in 2001 on using sodium cromoglycate to aid with management of recurrent and chronic enterocolitis. Even though the sample size was small, only 5 participants, 3 of them responded favorably, and so it might be worth a try.
Last week we finished all 3 evaluations for Early Intervention. The physical therapist came first, which was the therapist I was the most interested in seeing since Landon's delays are mostly gross motor. She agreed that he has hypotonia, and classified it as being a moderate degree. When you pull him up from laying on his back he has what is known as "head lag" which means that he isn't strong enough to lift his head up with his body. His joints are also hypermobile, which means that they flex more than they should because he lacks the strength to form resistance. She showed me several exercises to do with him to help him make progress towards what he should be doing. She also explained that because of the hypotonia, when he attempts to make progress towards any gross motor milestone he is at a lower starting point than a typical baby. He has to work much harder at everything, which will result in greater fatigue. He also will need more time to meet his milestones, and so he probably won't crawl or walk on time. But she reassured us that when 5 year olds are playing on the playground they aren't going around comparing the age at which they walked, and so Landon will be fine long-term, he just may not be a star athlete.
The occupational therapist came next, and she was impressed with Landon's abilities. He is functioning pretty close to age appropriately with regard to his fine motor skills. The developmental therapist also thought that he looked good, with the exception of his gross motor skills. We are meeting on Thursday to discuss what the plan will be. Landon will either go ahead and start physical therapy, or the physical therapist will come back to re-evaluate him in 2 months. He was slightly below the criteria of a 30% delay, but the physical therapist was confident that in 2 months when there are more milestones to hit, that he will miss them and then meet the criteria. Either way we have exercises to do at home for the time-being, which is what I was hoping for. So here's to hoping for an uneventful few months full of lots of progress with Landon's gross motor skills!
In a way, I'm relieved that we don't have to worry about Landon getting sick again for a while. He will be 10.5 months old by the time we discontinue the maintenance meds. He will be much bigger and stronger, plus that will be more time that he has been on donated breast milk gaining antibodies which are making his immune system stronger. The downside is that I really hate him having to take Flagyl. Studies done on the safety of it have shown that it is carcinogenic in both mice and rats. It has been shown not to be carcinogenic in hamsters, which is good, but who knows which rodent reacted more closely to how a human baby would. Plus there are risks with long-term use of any antibiotic, such as developing c.difficile (c.diff) or creating resistant strains of bacteria. So hopefully he can successfully go off the Flagyl in May when we try again. I have done some more research and have decided to start Landon on grapefruit seed extract, to see if that will help. I also want to discuss trying sodium cromoglycate. There was a study done in Finland in 2001 on using sodium cromoglycate to aid with management of recurrent and chronic enterocolitis. Even though the sample size was small, only 5 participants, 3 of them responded favorably, and so it might be worth a try.
Last week we finished all 3 evaluations for Early Intervention. The physical therapist came first, which was the therapist I was the most interested in seeing since Landon's delays are mostly gross motor. She agreed that he has hypotonia, and classified it as being a moderate degree. When you pull him up from laying on his back he has what is known as "head lag" which means that he isn't strong enough to lift his head up with his body. His joints are also hypermobile, which means that they flex more than they should because he lacks the strength to form resistance. She showed me several exercises to do with him to help him make progress towards what he should be doing. She also explained that because of the hypotonia, when he attempts to make progress towards any gross motor milestone he is at a lower starting point than a typical baby. He has to work much harder at everything, which will result in greater fatigue. He also will need more time to meet his milestones, and so he probably won't crawl or walk on time. But she reassured us that when 5 year olds are playing on the playground they aren't going around comparing the age at which they walked, and so Landon will be fine long-term, he just may not be a star athlete.
The occupational therapist came next, and she was impressed with Landon's abilities. He is functioning pretty close to age appropriately with regard to his fine motor skills. The developmental therapist also thought that he looked good, with the exception of his gross motor skills. We are meeting on Thursday to discuss what the plan will be. Landon will either go ahead and start physical therapy, or the physical therapist will come back to re-evaluate him in 2 months. He was slightly below the criteria of a 30% delay, but the physical therapist was confident that in 2 months when there are more milestones to hit, that he will miss them and then meet the criteria. Either way we have exercises to do at home for the time-being, which is what I was hoping for. So here's to hoping for an uneventful few months full of lots of progress with Landon's gross motor skills!
Monday, February 11, 2013
Finding a balance
Thursday night we headed back to Cincinnati for Landon's follow-up with Dr. Levitt Friday morning. Just like last time, we went in and did an x-ray first, and then went up to discuss what it showed and what has changed since our last visit. The x-ray looked much better than his last. His small intestines are no longer dilated and there were no signs of enterocolitis. Dr. Levitt also did a rectal examination and Landon's sphincter is much better as well, which means that the dilations were successful.
We are continuing the dilations at least twice a day for maintenance, but also can do them as needed to help stimulate the area to move things along. We stopped the Flagyl all at once, but left with a presciption for half the dose just in case he doesn't tolerate the change. Dr. Levitt also set up a plan for tapering off the irrigations. He felt that the reason that Landon is not going on his own is because we are keeping him flushed out so often. The irrigations empty him out, and when we are doing it 3 times a day he isn't getting the chance to accumulate enough stool to feel the urge to push. The only problem with that is that Landon is still extremely prone to enterocolitis, and if things sit around for too long he gets an infection. So we have to find a balance between letting things accumulate enough that he learns to use those muscles and push, but not letting things sit around long enough to cause an infection. Dr. Levitt had us drop an irrigation, and so we are already down to two a day and will continue this for two weeks. At that point we will go down to once a day, and continue that for two weeks as well. If that goes well, then we will try to stop irrigating altogether. If at any point he starts showing signs of enterocolitis then we have to go back up to 3 irrigations a day, and then start the process of tapering off again. So we will see how that goes.
Landon is still doing well with starting solids. He has done so well that we have been able to introduce a new food every 3 or 4 days. So far all of the foods we have tried have been a success. Like I mentioned before, we only do foods from the Cincinnati "Laxative Diet" list. I make all of Landon's food which is a new adventure for me. Two of the most common culprits for causing constipation- bananas and apples- are in just about every baby food on the market. Every single combination food that I can find contains one or the other. Fruit and vegetable skins are also known to cause problems, and so when I make his food I'm able to strain everything and know that it does not contain skins. Those were the main reasons that I decided to make his food, so that he could eat a variety of foods and not be as limited by his diet restrictions. So far everything is going well with that, and I have a feeling it will get easier with time.
This afternoon Landon is having his first evaluation with Early Intervention. The physical therapist will be coming to our house to see what she thinks. On Thursday the developmental therapist will be coming, and we are still waiting on the occupational therapist to contact us, which I would imagine will be sometime this week. Landon has actually made a lot of progress over the past week with sitting up unsupported. He is sitting up without propping with his hands and holding it for 5-10 seconds, sometimes longer. He also does well sitting in his high chair at the table, but he isn't strong enough to eat in that position- he leans forward too much and food spills out of his mouth. I have to sit him in either his car seat or bouncer in order to feed him, which works at home, but it is yet another obstacle which keeps us from being able to leave the house often or for long periods of time.
Well, lunch is over for me. I will update soon on how the EI evaluations go. I hope everyone has a great week!
We are continuing the dilations at least twice a day for maintenance, but also can do them as needed to help stimulate the area to move things along. We stopped the Flagyl all at once, but left with a presciption for half the dose just in case he doesn't tolerate the change. Dr. Levitt also set up a plan for tapering off the irrigations. He felt that the reason that Landon is not going on his own is because we are keeping him flushed out so often. The irrigations empty him out, and when we are doing it 3 times a day he isn't getting the chance to accumulate enough stool to feel the urge to push. The only problem with that is that Landon is still extremely prone to enterocolitis, and if things sit around for too long he gets an infection. So we have to find a balance between letting things accumulate enough that he learns to use those muscles and push, but not letting things sit around long enough to cause an infection. Dr. Levitt had us drop an irrigation, and so we are already down to two a day and will continue this for two weeks. At that point we will go down to once a day, and continue that for two weeks as well. If that goes well, then we will try to stop irrigating altogether. If at any point he starts showing signs of enterocolitis then we have to go back up to 3 irrigations a day, and then start the process of tapering off again. So we will see how that goes.
Landon is still doing well with starting solids. He has done so well that we have been able to introduce a new food every 3 or 4 days. So far all of the foods we have tried have been a success. Like I mentioned before, we only do foods from the Cincinnati "Laxative Diet" list. I make all of Landon's food which is a new adventure for me. Two of the most common culprits for causing constipation- bananas and apples- are in just about every baby food on the market. Every single combination food that I can find contains one or the other. Fruit and vegetable skins are also known to cause problems, and so when I make his food I'm able to strain everything and know that it does not contain skins. Those were the main reasons that I decided to make his food, so that he could eat a variety of foods and not be as limited by his diet restrictions. So far everything is going well with that, and I have a feeling it will get easier with time.
This afternoon Landon is having his first evaluation with Early Intervention. The physical therapist will be coming to our house to see what she thinks. On Thursday the developmental therapist will be coming, and we are still waiting on the occupational therapist to contact us, which I would imagine will be sometime this week. Landon has actually made a lot of progress over the past week with sitting up unsupported. He is sitting up without propping with his hands and holding it for 5-10 seconds, sometimes longer. He also does well sitting in his high chair at the table, but he isn't strong enough to eat in that position- he leans forward too much and food spills out of his mouth. I have to sit him in either his car seat or bouncer in order to feed him, which works at home, but it is yet another obstacle which keeps us from being able to leave the house often or for long periods of time.
Well, lunch is over for me. I will update soon on how the EI evaluations go. I hope everyone has a great week!
Tuesday, February 5, 2013
A little bit of everything
If you made it over here, then I see that you found that I moved Landon's Caring Bridge. Since we only have the internet on my phone, this makes it easier for me to update more often since Blogspot offers a mobile template. Also, I like the comment feature on the posts in case there is anything anyone would like to share.
Landon is doing okay lately, not really good or bad either one. He is still eating better, and probably gaining weight and so that part is good. We have introduced two different solids so far. Even though we will only be using foods selected from Cincinnati's 'Laxative Diet' list, I was still paranoid about food causing constipation. For that reason, we started with prunes. They may have had a little bit too much of a laxative effect, as they ran through him in less than 2 hours. I know that is good that he was able to pass them on his own, but I'm unsure if he was able to gain any nutrition or calories in that short of a transit period. Next was green beans, and last night was the first time that he tried them. He had an inconsolable period of crying from 9-10 last night, but that is something that happens from time to time anyway and so it is too soon to tell if it was from the food. I just fed him green beans again about 30 minutes ago and so by tonight I will have an answer.
Earlier this afternoon I met with the service coordinator from Early Intervention. We were assigned therapists for developmental, occupational, and physical therapy that will be contacting me to come seperately to evaluate Landon. If he exhibits a 30% or more delay in two areas then he will be eligible to receive therapy in our home for only $30 a month. If he doesn't qualify, then I will use his prescription for OT and PT and get him therapy privately, but that means more time off work to get him there and it will probably be much more expensive.
On Thursday night we are leaving to go back up to Cincinnati for another follow-up. They are doing a repeat abdominal x-ray and discussing what has happened since our last visit. We will be discontinuing the antibiotic that Landon has been taking for the last month, but I'm not sure if we will be stopping right away or doing it gradually. I also want to discuss why Landon is having so many issues with the affected portion of his intestines removed. It is normal to have some issues post-op, but not this many and to this extent. When he still had his ostomy he also struggled with enterocolitis, which is very rare. So I feel like there is something more going on. I have been doing a lot of research on chronic/recurrent enterocolitis and the outcomes. I found an old post from several years back from a parent that said that her daughter struggled with issues like that for 5 years before they discovered she had damaged connections from her small to large intestines. That got me thinking about an incidental finding in Landon's upper and lower GI studies done in July. We discovered that he had a mobile cecum. It isn't exactly rare, approximately 15% of us have a mobile cecum and have no idea. It isn't known to cause problems in the general population, but who knows if it causes problems when it exists along with Hirschsprung's. If anyone knows, it would be Dr. Levitt and so I plan on bringing it up. I'll try to update this weekend on what we find out in Cincinnati.
Landon is doing okay lately, not really good or bad either one. He is still eating better, and probably gaining weight and so that part is good. We have introduced two different solids so far. Even though we will only be using foods selected from Cincinnati's 'Laxative Diet' list, I was still paranoid about food causing constipation. For that reason, we started with prunes. They may have had a little bit too much of a laxative effect, as they ran through him in less than 2 hours. I know that is good that he was able to pass them on his own, but I'm unsure if he was able to gain any nutrition or calories in that short of a transit period. Next was green beans, and last night was the first time that he tried them. He had an inconsolable period of crying from 9-10 last night, but that is something that happens from time to time anyway and so it is too soon to tell if it was from the food. I just fed him green beans again about 30 minutes ago and so by tonight I will have an answer.
Earlier this afternoon I met with the service coordinator from Early Intervention. We were assigned therapists for developmental, occupational, and physical therapy that will be contacting me to come seperately to evaluate Landon. If he exhibits a 30% or more delay in two areas then he will be eligible to receive therapy in our home for only $30 a month. If he doesn't qualify, then I will use his prescription for OT and PT and get him therapy privately, but that means more time off work to get him there and it will probably be much more expensive.
On Thursday night we are leaving to go back up to Cincinnati for another follow-up. They are doing a repeat abdominal x-ray and discussing what has happened since our last visit. We will be discontinuing the antibiotic that Landon has been taking for the last month, but I'm not sure if we will be stopping right away or doing it gradually. I also want to discuss why Landon is having so many issues with the affected portion of his intestines removed. It is normal to have some issues post-op, but not this many and to this extent. When he still had his ostomy he also struggled with enterocolitis, which is very rare. So I feel like there is something more going on. I have been doing a lot of research on chronic/recurrent enterocolitis and the outcomes. I found an old post from several years back from a parent that said that her daughter struggled with issues like that for 5 years before they discovered she had damaged connections from her small to large intestines. That got me thinking about an incidental finding in Landon's upper and lower GI studies done in July. We discovered that he had a mobile cecum. It isn't exactly rare, approximately 15% of us have a mobile cecum and have no idea. It isn't known to cause problems in the general population, but who knows if it causes problems when it exists along with Hirschsprung's. If anyone knows, it would be Dr. Levitt and so I plan on bringing it up. I'll try to update this weekend on what we find out in Cincinnati.
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