Eat Happy!

Eat Happy!

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**The information provided in this BLOG is in no way intended to diagnose, treat, or cure any illness. This information should never replace the advice of a doctor. Please use this information as you see fit. This information will pertain differently to each child, each adult, and each family.**

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Saturday, February 22, 2014

Making Tough Decisions

Before I begin this post, I would like to say that we did not receive good news from Ian’s gastric emptying scan. Sadly, we are out of "good" options. At this point, we are looking at experimental treatments and drugs because of his atypical emptying pattern. It is one that is virtually never seen, and leaves doctors asking questions where they just don’t have answers. Believe me when I tell you that by working with four excellent gastroenterology hospitals, and an incredible motility clinic, we have exhausted our great options. We are now left with the investigational and experimental options. We must now make tough decisions about how we will proceed. All of our options are lacking in supportive clinical data and are controversial. Please try and be respectful of our decisions. I am certain they are not for everyone, but we feel confident that we, along with Ian’s team, will assess the case and make the best choice for our family.

I have said it before and I will say it again now, and again at the end of the post..... This is a personal following of our adventures. It is a means of sparking conversation between parent and doctor. It is a way for us to bring forth knowledge that we worked so hard to find and understand, in the hopes that others may not have to work so hard. This blog is not a diagnostic tool, pharmaceutical guide, or “virtual physician.” It is a great honor to share our adventures here, so others can gain support, knowledge, and love, when other searches leave them lacking.

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We have made it home safely from The Children's Hospital of Philadelphia. Ian is all snugly and happy to be back in his bed. To our surprise, we received a phone call from Ian's motility-GI doctor in the car on the way home, at 8:45pm! Very impressed!

We see Dr. Boyle in the Motility department, part of GI, at CHOP. Here is his information:
CHOP Motility: Dr. John Boyle

He has reviewed Ian's tests and was able to provide some good feedback....

1)  Ian's upper GI does not show enough change in the herniated fundoplication to attribute his new symptoms. He does not feel that the fundoplication is the problem. And, not to mention, and all costs, the surgeons don't want to go anywhere near him since a second repair (third fundoplication) may make matters worse due to potential side effects and damage.


2)  Ian's gastric emptying scan showed some seriously atypical results. This was his third gastric emptying scan. First of all, he did empty 51% in the first two hours. This is within normal limits. It was done ON his motility medication, erythromycin. However, between hours two and four, Ian only emptied another 6%. This is highly abnormal.

Gastric Emptying Scan October 2011
Gastric Emptying Scan May 2013
Gastric Emptying Scan February 2014

Erythromycin as a Prokinetic for Infants and Children: PubMed

One reason could be that the erythromycin, a fast acting medication, acts early, but then does nothing for him later. This causes food to "accumulate" over time in his belly, making it harder for him to eat later on. Essentially, if he eats breakfast at 8:00am, there is still 43% of it still in his stomach come 12 noon for lunch. Dr. Boyle, Ian's motility doctor in the GI department at CHOP says that this is a very atypical pattern that they hardly ever see.

When the gastric emptying scan was done last May, Ian emptied 25% in two hours (without any motility meds). The test was not conducted for more hours. This was the first time anyone had done a three and four hour picture. And, the first time being on meds, to see how they were working.

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He did talk to us about a couple options, none of them easy, or fabulous. They include: antispasmodic medication, antispasmodic Botox injections into the pylorus, and bypassing his stomach and feeding into the intestines via a J-tube.

(He did mention Ian was an excellent candidate for domperidone (Motilium.) More on that option in a moment.) ...........

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Option A) Antispasmodics:

We can use an antispasmodic medication to help relax the pylorus, the sphincter valve that releases food from the stomach into the intestines. This would be an oral medication. The theory is that the medication would relax the pyloric valve enough to allow sufficient stomach emptying contractions. It seems backwards, since you need contractions to empty the stomach, but it's true. An "emptying valve" that is trigger happy could be emptying the stomach into the bowels quickly because it isn't functioning properly, or efficiently. By relaxing the pyloric valve, the stomach might be able to regulate it's emptying. This method can also be achieved by injecting Botox into the pylorus. This would allow muscle relaxation that could allow for proper functioning. Dr. Boyle did not seem thrilled with these options but said they might work.

This type of treatment would be similar to treating visceral hyperalgesia (organ pain). We are using gabapentin now for Ian’s visceral hyperalgesia, but, Levsin, and antispasmodic medication, reduces pylorus spasms and other visceral pain, like the kind Ian has. His gabapentin is not an antispasmodic medication.

Oral Antispasmodics:

Complexchild.com: Functional Abdominal Pain and Visceral Hyperalgesia: Why does my tummy hurt so much?

Complexchild.com: Solutions for Retching (antispasmodic medications and Botox)

Complexchild.com: Medications for Reflux and other GI problems (Levsin as antispasmodic)

Botox:

Gastroparesis: a review of current and emerging treatment options

Clinical trial: a randomized-controlled crossover study of intrapyloric injection of botulinum toxin in gastroparesis

Botox for Gastroparesis

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Option B) J-tube: (or GJ Tube)

A G-tube, also known as a Gastrostomy Tube, is what Ian has now. It feeds directly into the stomach, bypassing his mouth and esophagus entirely. A J-tube, also known as a Jejunal Tube, feeds directly into the Jejunum, the middle part of the small intestines. (The first part is called the duodenum. As the stomach churns out the food substance through the pylorus, it is deposited into the duodenum.) J-tubes can come as a GJ (gastro-jejunal tube) with both ports. These ports can be used individually, or in conjunction with one another for feeding, decompression, and medications.


G-Tubes


GJ-Tubes

Jejunum feeding bypasses the mouth, esophagus, and stomach entirely. Feeding the intestines is very different from feeding the stomach. It entails much stricter regimens and efforts. It is not often a #1 choice.  J-tube meals are not as simple as "feeding him whatever he doesn't eat by mouth through the tube." Without constant and strict J-tube feeding monitoring, you have no way of knowing what is in the intestines before you feed it. This is not to say the J-tube feeding would keep Ian from eating by mouth ever again.  I am pretty sure he would still having oral eating opportunities.  Dr. Boyle does not like the option of a J-tube right now either.

J-Tube Resources:

CHOP: J-tube

Feeding tubes

Feeding tube awareness: type of tubes

Types of tubes

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Option C) Domperidone:

He did mention that there is another drug called Motilium (generic name is domperidone). Domperidone is the #1 motility and antiemetic (anti-nausea) medication everywhere in the world, but the USA. It is not FDA approved here for various reasons. One is lack of research and another is the risk of cardiac side effects. There are a few clinics in the country who can prescribe his medication under an "investigational new drug" (IND) status from the FDA. The Children's Hospital of Philadelphia is not one of them. They have been working on there IND, but it is not approved at this time.

There is a special form used for doctors to apply for an IND. This was one I found online

This medication is incredibly effective and has the same therapeutic effects as the ever so well-known Reglan. However, since Reglan crosses the blood-brain barrier, it has a risks of neurological side effects. Ian, of course, experienced these side effects, and can not tolerate Reglan. Without crossing the barrier, domperidone is able to provide excellent GI success without the risk of neurological side effects. Dr. Boyle said that Ian is an excellent candidate for this drug. He feels that with his motility and nausea symptoms, and past medicinal history, that he would do well.

Since CHOP is not able to prescribe domperidone, we cannot get the medication from them. It would need to come from a doctor with an IND. And then, you have to find a pharmacy to dispense it for you. Most of them come from Canada and Europe, but he mentioned there is one in Arizona now. Dr. Boyle did his pediatric internship and pediatric residency Boston Hospital. He knows the motility doctors there and feels as though they may be able to help us get on the right track with domperidone......Who has an IND. His colleges there have been using the medication for quite some time. Actually, when Dr. Boyle was working there, he prescribed the medication also. Since he moved to CHOP, who doesn't have an IND, he has not been permitted to prescribe it.

When we told him we were highly interested in the domperidone, he said he would email his colleagues in Boston to see what we could do to fast-track us. He is going to send Ian's case history and testing information to see if they concur with his candidacy for the medication. He did tell us that if we can get the prescription, he could do the monitoring from CHOP. Thee would be frequent blood tests and EKG to monitor levels and heart rhythms. He told us that if we could get our hands on the medication, he could guide us on how to dose, and how to use it.

He did tell us that the medication is prescription only. I may chose to argue that. It seems as though there are online pharmacies where you can purchase the medication without a license. I'm not saying that we are jumping on that wagon, it just seems strange that he would say that if it seems so easy to buy for yourself. According to the internet, domperidone is prescription only in Australia and Canada. It is also available over the counter in the UK, Italy, China, and other Asian and European countries. Tom and I still feel that working through an IND facility is the safest way to go.

This is a site where the medication can be purchased without a prescription.  It ships from Vanuatu, a small island in the South Pacific region. I know of many families using this site. It is popular for many who are unable to obtain the medication in the States, but can be managed by a knowledgeable doctor. I have also heard that it should be used with caution as it may not contain the same level of active ingredient, or may contain higher levels of unsafe ingredients. I am be no means supporting or refuting this pharmacy.... simply putting it out there as knowledge. Please remember that the resources in this blog are purely for information and investigational purposes.

Tom and I have been interested in domperidone for quite some time. Back in May, when we first started being officially treated for test-diagnosed motility problems, we asked about the medication. We were placed on the Bethanechol since there is no IND. Fortunately, Ian did very well and has been ever since. Sadly, the medications don't seem to be working sufficiently anymore.

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So, for now, our plan is to see how Ian does the next week with rearranging his medications. We are going to continue the erythromycin and Bethanechol, but stagger them. He will receive his erythromycin before breakfast, lunch, and dinner. We will then give him the Bethanechol before his new late morning and late afternoon snacks. The theory is that the Bethanechol will be coming in about two hours or so after his last meal. This will hopefully offer him some emptying effects right before he eats again. Dr. Boyle would like to see how is goes while he is getting in touch with his colleagues in Boston.

General Knowledge for domperidone:

Wiki domperidone

Janssen pharmacies: domperidone

The role of the vagal pathway and gastric dopamine in the gastroparesis of rats after a 6-hydroxydopamine microinjection in the substantia nigra. (mentions the vagus nerve plays an important role in peripheral gastric motility disorder.)

Domperidone: review of pharmacology and clinical applications in gastroenterology

Advantages:

The Advantages of domperidone

Domperidone review, legal matters, and side effects

Disadvantages:

Domperidone and ventricular arrhythmia or sudden cardiac death: a population-based case-control study in the Netherlands

Domperidone and long QT syndrome

AHA: Domperidone Should Not Be Considered a No-Risk Alternative to Cisapride in the Treatment of Gastrointestinal Motility Disorders

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Causes:

Ian was recently sick. Gastric emptying issues can be exacerbated by illness. However, Ian began having problems three weeks before he was sick. It doesn't seem likely that this would be a result of illnesses, but it could play a role. That being said, illness induced motility complications don't simply go away as an illness fades away. They can hang around for months, years, or even be permanent. So, even if it is illness-induced, there is no guarantee it will improve.



Another possible cause is that Ian's medications simply aren't not working anymore. There is a medicinal effect called tachyphylaxis. Tachyphylaxis is when there is a sudden decrease in response to a medication. This can be from time, or a decrease in the number of receptors available for which the medication to act upon. Raising the dosage can potentially restore the effect, but may not be permanent. With Ian being on the high side of his medications, increasing the doses is not necessarily a good options. Worth mentioning though, Ian's first two hours of the emptying study (where he had taken the erythromycin) were almost normal. So, the erythromycin he is taking for motility seems to be working. It just does seem to be working week enough, long enough. I have no good explanation for why this is a problem now, but not before.

Tachyphylaxis

There are no other easy causes to throw out. It is unknown as to why this has now become a problem. Maybe it is because he is eating more as he is getting bigger and is taking in more calories. Maybe it is because of illness. Maybe it is from tachyphylaxis. Maybe it's all of them. But, the bottom line is we need a new therapy regimen that can help relieve his symptoms.

Ian is starting to show food aversions again. This is something he hasn't done since last May when we struggled with what seems like the same type of problem. The last thing we want is for him to start making negative food associations again. Overcoming those is so much harder than teaching new foods. Old habits die hard. And they die even harder the second and third time. This would be the third aversion issue for Ian.

Photo from last May, 2013.
Taken after feeding clinic, when motility issues became a major problem.
Ian developed serious aversions again and we were able to overcome them.
It was the second time, and took much more effort.


Ian has always been a happy and smiling child who seems to be bright and cheerful through it all. This time around, he seems tired, and saddened. While he still happy, feeding times have become "un-fun" for him. When he vomits, he says things like "I'm sorry, I didn't mean to" and "mommy, I got sick" (with a sad face). It makes me so sad to know we are back here again. But, Tom and I feel very confident that we are in the right place, with the right people. It is just so hard to be patient long enough to get what you need. When you are watching your child cry, vomit, and throw up food everyday, waiting becomes the hardest thing. But, right now, all we can do is rearrange Ian's medications, wait to hear from Dr. Boyle, and hope for a fast-tracked appointment with Boston to get some new medicinal therapy.

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Here are some Motility resources for organizations:

International Foundation for Functional Gastrointestinal Disorders

International Foundation for Functional Gastrointestinal Disorders- Motility /

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Here are a few pictures from Ian during his Upper GI series and his Gastric Emptying Scan. He finds ways to make hospital gowns look so cute!

Upper GI:





Gastric Emptying Scan:




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And now I'll say it again...........

We know that this is a controversial medication and that everyone will not agree with our decision. Please know that we are essentially out of "good" options. Pediatric motility is a field with lots of unknowns and not many good medicinal choices. Research is being done all the time to help foster new ideas, testing, and therapies. CHOP and Boston are two of the best hospital in the country and we feel incredibly blessed to be within driving distance of both.

Please also remember, as with all the information in this blog, that each child, each family, each doctor, and each set of treatment options will be different. This blog is a way for us to share our story, in hopes that others will feel inspired. I aim to supply resources spanning various treatment options for parent education. I hope that parents can then be more educated to ask the right questions, and bring therapies to the forefront that might otherwise go undiscussed. I find ruling out inappropriate options can be as emotional therapeutic as finding good ones. I hope that by doing this, parents and physician can work together to provide the best health, happiness, and life for their children. 

Thank you for following our journey!

Tuesday, February 18, 2014

Upper GI and Hernia Update

Ian's upper GI series (swallow study) went very well. They started with him drinking the liquid through a straw. His swallowing looked great, and it passed the fundoplication well. (This was liquid only) Then they inserted barium in through the G-tube in order to check the integrity of the fundo. There was no reflux. The liquid passes put of his stomach very well.

Tom and I have seen eight of these Upper GIs now and are pretty familiar with what we are seeing. There is a hernia, the same one from last May. It does not appear to be any larger. The Dr. will review it later today. I'm not sure if we will hear from him.

If it is decided the hernia is the problem, I'm not sure what our options are. Ian has already had one slipped-twisted-herniated fundoplication. There was probable vagus nerve damage with the initial fundo. When it was repaired last July, there was the possibility of more vagus nerve irritation. It is impossible to know unless major damage is present (which here was not). Each fundo has a higher risk of failure. This would be the third one. It isn't as simple as a "fix" like undoing and then redoing. There is tons of scar tissue, making the nerves hard to see and navigate. Surgeons avoid continual repairs as frequently as they can.

I don't know what our options will be if the herniated fundo is causing the problems. Even if it has not gotten worse, it could be causing problems. Last time, Ian dealt with his hernia for six months before it caused enough problems for repair. (Remember, they absolutely want to avoid repairs whenever possible.)

Assuming we don't get a call canceling the test tomorrow, we will do the gastric emptying scan tomorrow at 11:30. That will check his stomach motility and see if he is hanging on to food too long in his belly, causing him to fill quickly, and keeping his stomach from accommodating the amount of food normally needed.

Ian will need to eat as much of one scrambled egg, bread, and juice as possible in ten minutes. Three-quarters of the egg is our major goal. We are going to make him *really* hungry and hopefully be able to use some ketchup for the egg if they will let us. Ian still loves the novelty of dipping things in juices / ketchup / gravy / sauces.

Here are the comparison upper GIs.



Saturday, February 15, 2014

Feeding Troubles?

Could it be swallowing troubles, Illness, Behaviors, Reflux, Visceral Hyperalgesia? or all of the above? We have been having quite a bit of trouble with Ian lately when it comes to feeding and pooping. Just as a little background...

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About a month ago, we took an unexpected one week trip to Ohio for a family emergency. Ian started having chewing and swallowing issues. We thought they were purely behavioral, until recently when we figured out that he began clearing his throat very frequently at the same time. Prior to making this connection, we went back to the original rewards protocol. We saw improvement for about three days. It had been going on for about ten days before this three-day improvement. While we were in Ohio, the throat clearing and chewing-swallowing refusal was not frequently resulting in vomiting... mostly retching and gagging.

The restart of the trouble, after the three-day improvement, was about one week before he got sick. These troubles resulted in four vomits the week before he caught the cold. This is when the chewing and swallowing refusal began resulting in frequent vomiting. He got sick on Feb. 6th. The cold seemed to be mostly respiratory, lots of coughing. We were doing his nebs and also doing his hydrocortisone. He's appetite was practically nothing. We did the best we could to get his calories in, averaging about 700 a day. He was meeting his fluid needs and getting about 36 oz a day. He was not experiencing any diarrhea of fevers at this time.

He started showing some improvement with his cold after a few days, and even had two days where he ate over 1000 calories. But then, he started back up again, with diarrhea this time and very little coughing. He had been sick for one week when the diarrhea began. Still no fevers.

The chewing and swallowing refusals do not seem to be linked to a particular time of day, or special meal. He seems to refuse and have trouble, all the time. He is even rejecting purees at times. I don't know how to tell if it is a behavioral issue going terribly wrong that is being made worse by an illness, the simple fact that he is sick, or a more physical concern because it started back when he began clearing his throat and wasn't even sick. We had previously decided with his feeding team that it was a behavioral issue because it began when we went out of town. That was the first time things had gone awry like this and it was the first time he had left home for that long of a time and had so much disruption to his schedule.

Yesterday, we decided enough was enough... we would simply take away food, give him 24 hours of Pedialyte and fluids and give his gut a rest. We hoped that would give his system enough time to reboot. The pediatrician agreed, but said if he wanted a few snacks, to let him have them. Well, go figure, the kid was really hungry! He had some Cheerios and a donut. He also begged for pizza at dinner and ate it like crazy. He was very interested in food when we weren't pushing meals and protocol....... but, he wasn't getting structured foods or meals. So, he didn't really have 24 hours of Pedialyte, but had a great day. There was no refusals, retching, or vomiting.

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This morning, he once again showed interested in food. He ate a whole bag of Goldfish crackers and some cereal with milk. A couple hours later we gave him his applesauce and Fruit-Eze. This was his first structured meal in the chair since the food removal Friday morning. He did great through the whole meal, until the very end. He promptly gagged on the last two bites. Afterwards, he began retching and threw up his breakfast. I don't know whether to think it was behavioral (since this was his first meal back in the chair) or physical (because we were determining the amount of food he was getting and not deciding for himself). If it is physical, we are concerned it could be the wrap or a swallowing/reflux issue. I have noticed that he usually talks about needing to poop around these vomiting and retching issues.

Sometimes he goes, and other times, he sits on the potty and tries but never goes. I don't know whether it is stalling, or a bowel comfort issue. The stool consistency doesn't seem to be the issue, but we are wondering if maybe he is experiencing bowel discomfort when eating with his gastro-colonic reflex. And potentially not being able to cope with his bowel sensation during eating and also during bowel movements. We aren't sure if increasing his gabapentin might help alleviate any of these symptoms and provide him an increase his desire to eat foods during structured meal times.

Here are our guesses as to the cause of the troubles:

1) A behavioral issue with swallowing while away due to schedule and environmental disruptions... where he had improvement with protocol when returning home... but then started having problems again with his body's sense of an oncoming illness. And in that case, we would now still be having GI issues for a while after his recovery?

2) A physical issue independent of travel and behaviors, because throat clearing began at the same time? (fundoplication problem or motility issue)

3) A bowel discomfort, visceral hyperalgesia problem since he seems to be mention pooping several times that he has trouble. Although, it doesn't seem to be based on if he has pooped before eating and he doesn't always poop when he says he needs to (but then again, is that because there is a bowel comfort issue to begin with?)

I believe an option #4 of being purely behavioral is untrue at this time. There seems to be too many other components to base his troubles now on behaviors alone.

That seems to sum things up. Right now, we are looking to physical issues (illness or reflux / fundoplication / motility). This seems to be a similar type of trouble we had before his motility and visceral hyperalgesia were under control.

I have a call into the Richmond Feeding Clinic and the GI department at CHOP, but did not hear from them yesterday. I don't know whether his Eryped or Bethanechol would benefit from an increase or not. He does have a gastric emptying scan scheduled for March 14th. Also, this doesn't seem to be the same trouble we had recently, where increasing the Benadryl and Periatcin helped. He does not seem to be struggling with mucus unless it is the mucus he creates vagal-ly when retching.

We are concerned about his troubles and he recent illness in terms of weight loss. While we don't know for sure, we are fairly certain he is losing weight. Please let me know your thoughts and ideas!

Monday, February 3, 2014

US News Best Children's Hospitals of 2013-2014

I know a lot of mommies (and daddies) looking for great medical programs for their children.  This list is certainly not comprehensive, but gives a good look at departments, both in the top ten and throughout the list.  If one hospital is not suiting your needs, make sure to check out the list.  There is someone out there to help every child.  Unfortunately, finding them proves to be one of the hardest parts!

This TOP TEN list is certainly not complete.  make sure to look at the individual lists of specialties.  Some are TOP TEN rank in specialities but not overall.... making them highly valuable in their areas of expertise.  However, it should go without saying that each hospital will not fit each family.  Every child and family is different and providers should be evaluated on an individual basis.


US News Best Children's Hospital of 2013-2014


#1  The Children's Hospital of Philadelphia:
http://www.chop.edu/

#2  Boston Children's Hospital:
http://www.childrenshospital.org/

#3  Cincinnati Children's Hospital:
http://www.cincinnatichildrens.org/default/

#4  Texas Children's Hospital:
http://www.texaschildrens.org/

#5  Children's Hospital Los Angeles:
www.chla.org

#6  St Louis Children's Hospital:
http://www.stlouischildrens.org/

#7  Children's Hospital of Colorado:
http://www.childrenscolorado.org/

#8  Ann and Robert H Lurie Children's Hospital of Chicago:
https://www.luriechildrens.org/en-us/Pages/index.aspx

#9  Johns Hopkins Children's Center:
http://www.hopkinschildrens.org/index.aspx

#10  Children's Hospital of Pittsburg:
http://www.chp.edu/CHP/Home