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!

3 comments:

  1. My heart goes out to you all. Ive been on dom for dge prescribed by my gi at the time and it was compounded at a local pharmacy. I also ordered online to help my supply years later. I hope it helps him and he starts to feel better. You seem to have covered all options, best wishes.

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  2. Our son had a gjtube. It is a very easy thing to place and goes directly into the gtube hole, meaning no added surgeries or risk of side effects from medication. There are so many kids who have them these days and do wonderfully with them. I wish you well in your decision. Sounds like you have some good options and great doctors to direct you.

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    1. I also know of many children who have them and do great. They are a wonderful choice for many. Our family is certainly keeping that option on the table, we are just trying some others first. Thank you for bringing a positive light to the GJ/J tubes. Many families are afraid of them and I think lots of parents feel that they have hit the bottom when they get them for their children...... which they most certainly have not.

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