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.