Eat Happy!

Eat Happy!


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Thursday, April 19, 2012

♫♫ Ch-Ch-Ch-Ch-Changes ♫♫

CHOP Follow-Ups
Yesterday we traveled to CHOP for follow-up appointments.  We saw Dr. Blinman first with the GERD clinic.  He initially thought we would need to repair Ian’s hernia after hearing about our in-house stay last week.  After watching Ian for about 10 minutes and seeing how well he was doing... he decided to postpone the surgery.  He told us the older and bigger and stronger he is, the more successful the surgery will be.  The repair will be much more intricate and longer than the initial surgery.  In addition to repairing the hernia and completing the 270 degree Toupet wrap, he first has to undo everything that was initially done.  Since there is more scar tissue this time, it is harder to avoid trauma to the vagus nerve.  Ian already has vagal troubles, so this is very important.
He miraculously told us that corn starch can cause diarrhea if he is getting too much.  I never would have thought this.  Since corn starch makes things thicker, I would have assumed the opposite.  He decreased Ian’s corn starch consumption by about 5-10% to alleviate some of these symptoms.
While admitted to CHOP last week, the formula room was using distilled water to make his food.  This is different from what we do here in Virginia.  Although his dietitian said it is unlikely that the water would cause a problem, it wouldn’t be unheard of.  Since he has been drinking the same water since infancy, it seems strange he would have troubles now.  Well, once Ian was home for 2-3 days, the diarrhea returned!  We switched him to distilled water 3 days ago, and the poops have been much better.
We are hoping that the corn starch decrease and the switch to distilled water will keep things moving in the right direction.  It was their impression, and the impression of his pediatrician today that the recent diarrhea troubles since being discharged from “the Bug,” are diet related and not from the positive Norovirus diagnosis last week.  It is funny sometimes how doctors will diagnose Ian for things that just don’t seem right, only to have another doctor swoop in and figure it all out.  That doctor is usually the GERD clinic surgeon at CHOP.  Unfortunately, when we were admitted, he was out of the country for the week.
Afterwards, we traveled down a few floors to follow-up with GI.  We did this simply as a courtesy and clearance because they saw him as an inpatient.  Dr. Blinman’s group does an excellent job of monitoring and helping Ian with all of his GI issues.  As the GI doctor said “Dr. Blinman does things... (long pause)... differently than the rest of this hospital.”  She finished with saying that he does however help lots of kids.  It seems as though most doctors criticize a lot of Dr. Blinman’s efforts and theories.  Ian has never fit into the cookie-cutter and Dr. Blinman has always had answers for us.
We were able to come home afterwards and spend the night in our own beds.  This morning we were up and running again.  Ian had two appointments today that were also follow-ups.
Virginia Follow-Ups
The first appointment was with the Pulmonologist.  He had a lot of good things to say about Ian, his weight, his progress, his developmental play, amongst other things.  Even though he is a Pulmonologist, he has always been an all inclusive GERD physician as well.  He is very familiar with the GERD and airways relationship as well as systemic motility issues.  He is also very familiar with Nissens and their after-surgery issues.
He had a few suggestions for us.  He moved Ian to only evening Prevacid.  He will still receive the 15mg Solutab, but it will be all at one time at night.  In the morning, we are going to try giving him a small amount of Orange Juice with Calcium and Vitamin D.  Since calcium and iron need an acidic environment to be absorbed, and proton pump inhibitors for GERD suppress all the acid, long term acid suppression medications interfere with minerals, leaving bones to be more brittle and weaker.  We will try the OJ soon and see how it goes.
He was an advocate for the ear tube surgery on 4.26.2012.  He said he would not be surprised at all if once the tubes are placed, Ian begins talking.  With all the chronic fluid in the ears, he mentioned it sounds like always having water in your ears.  Even though he can hear, it is distorted and muffled sounding.  This was an interesting thought and I am hoping it will help.
The Pulmonologist also thinks that with all the motility and GI issues that there is some type of central nervous system component.  This is really no surprised because we know about Ian’s vagus nerve issues.
Our second appointment today was with the Pediatrician.  She was very accepting of all the information and had good things to say.  We all seem to be on the same page now and moving forward.  Tomorrow we begin feeding therapy again on a weekly basis.  This will still be on an outpatient basis.  Dr. Blinman has given us clearance to skip a meal on these days so that Ian will be hungry.  We will only be able to do this on feeding therapy days for a while until he is able to consume enough during those things to make up for more of the calories lost.
We discussed Ian’s vomiting with Dr. Blinman yesterday.  Most of Ian’s vomiting is centered around oral stimulation and eating.  This is most likely due to dysphagia, painful and troubled swallowing.  Nissens are notorious for this.  What happens is he struggles to get food past the Nissen, especially with the hernia present.  It is also painful for his esophagus to push the food and slide past the surgery.  They feel that Ian may soon hit a road block with feeding therapy even when he wants to eat due to this.  Even if you are hungry and want to eat, if it is painful or too difficult, you will stop doing it.  If this occurs, they will need to repair the hernia.
The idea of inpatient feeding therapy programs is still in the back of our minds, but without knowing how the outpatient care will go, and the dysphagia component, it is not appropriate at this time.  An inpatient program would not be successful with a strong dysphagia component.
All in all, we seem to be gathering even more information about Ian every day now.  He is quite the puzzle and always keeps us on the verge of the next adventure.  Tomorrow we begin the feeding therapy adventures.  Keeping my fingers crossed he will at least enjoy some of it!  More to come as the changes keep on rolling in.

Monday, April 16, 2012

Home With Answers

Home with Answers
I’m so sorry for the delay, but we did return home from CHOP’s Endocrinology care on Thursday very late in the evening.  With all the mail, laundry, bills, dishes, and unpacking in front of us... I can’t believe we got it all done before work today.  Somewhere in the mix of my “to-do” list was the BLOG update....... but I decided paying the bills might be more important ;)
The last time I updated, Ian has begun his fast at 6:00pm with his final meal.  As the night went on, Ian slept peacefully and never woke up for any of the blood sugar sticks.  The numbers were as follows:
Meal 6-6:48pm....... glucose 89, ketones 0.7
 9:00pm.................... glucose 97, ketones 0.7
 12:25am.................... glucose 83, ketones 0.8
 3:00am..................... glucose 80, ketones 2.2
 6:00am..................... glucose 60 & 59, ketones 4.3
 7:00am..................... glucose 46 & 49, ketones 3.1
The ketones are to rise as the glucose levels drop.  As a refresher, the ketones are a side effect of fat being broken down to supply energy to the brain.  Ideally, ketone tests are negative, but when deprived of glucose in the blood, ketone bodies should appear.  Ian’s ketones elevated, but very quickly.  Theoretically, a child of Ian’s age and weight should be able to fast safety for about 12 hours.  Unfortunately, Ian was only able to fast for about 9 hours before his blood sugar dropped below 70.
This means that Ian has an abbreviated and immature fasting system.  It is diagnosed as Ketotic Hypoglycemia.  Fortunately, this rules out any risk of high insulin levels and/or hyperinsulinism.  Ian’s issues are more centered about a glucose issue as opposed to an insulin issue.  This condition is common in tube fed children.  Since Ian is eating frequently enough and in steady enough levels, his blood sugar remains very level and constant... as a result, his body has not learn to go longer periods of time without food.
They also ran some other tests:
1) Ammonia levels in the blood: for GI bleeding  ***Normal
2) Lactate levels in the blood: for glycogen storage disease  ***Normal
3) Cortisol levels in the blood: for vomiting & diarrhea to rule out pituitary gland malfunction  ***Normal
4) Insulin-like Growth Factor: for pituitary functioning
5) Insulin-like Growth Factor-Binding Protein 3: these binding proteins act as “carrier proteins” for Insulin-like Growth Factor
We are still waiting for any Insulin-like Growth Hormone tests to return.  A positive result on these tests can indicate Glycogen Storage Disease.  We will need to wait and see what the tests show.  So far, I have not heard anything...... I’m hoping no news is good news.
So far, everything has come back with normal results with exception of the fasting length.  Over time, we will be able to improve this time period and lengthen it.  It will be more difficult as we work into feeding therapy more intensely because we will have to watch his blood glucose very carefully.  The best way for Ian to learn to eat is to allow him to be hungry...... without hunger, he has no desire to eat.  However, by skipping meals, Ian’s sugar will fluctuate even more.  It also means that whenever Ian is sick, his blood sugar will roller coaster much more.  He is more prone to hypoglycemia and we now carry a letter in Ian’s traveling medical record.
We must present this letter when he is in the ER because it affects fluids and IV treatment.  Ketotic Hypoglycemia usually resolves by age 7-10 years.  Until then, we will be monitoring it as times arise.  We have been issued a glucometer to test blood glucose levels at home a couple times a day as needed.  Ian doesn’t even flinch or budge when we test at home.  It practically feels like nothing and he is so brave.  We have the Freestyle Lite which requires the least amount of blood on the market and it is fabulous!

We are to follow up with CHOP Endocrinology in 4-6 months.  For the time being, it looks as though it is something that can be managed by us at home, unless emergencies arise.  We are happy to be home, but a little reserved about what the future might hold.  We have ruled out lots of serious complications, but are still faced with some high maintenance, daily concerns that will pan out in time.
We have also entertained the idea of an feeding therapy inpatient program for more intensive treatment.  We will be beginning with Ian’s feeding therapist again in a few weeks and are hoping to make some great strides there.  If not, we will be evaluating the inpatient programs.
Hopefully, we can move forward with the new information we have and build a healthy, happy feeding relationship.  Thank you all for being so patient while I put this together.  Now that Ian is home, there will be no need for daily updates and things can calm down some.... hopefully.... fingers crossed.  I can only take so many waves at a time.
Here is a glimpse of what it was like to leave CHOP.  We have SO. MUCH. STUFF!!!

We will be meeting with Ian's Surgeon and Dietitian this Wednesday to review all the information and set a new plan in place!

Thursday, April 12, 2012

Glucose and Fasting

I am pretty tired tonight so this won’t be a long post...  yea right, I always say that!  The morning began with Ian’s Glucose Tolerance Test.  He was given just shy of 2 ounces of the sugary sweet drink through his G tube... the same drink used during the same test in pregnancy.  Ten minutes later, after lots of coughing and gagging, Ian promptly threw it up :(
Not long after, the Attending Doctor, Dr. Palladino and his Fellows trailed to say we were going to run the test again.  I told them he was going to throw it up again and asked why.  Apparently, Ian’s higher blood sugar of 154 , up from 84, was not enough to trigger the nausea.  He said it was probably just the drink itself because it is so sicken-ly sweet.  This time, we infused the 2 ounces over 20 minutes through his pump.  This time, although a little coughing, Ian kept it down.  Ian’s sugars were perfect through the test and showed no signs of dumping syndrome, go figure.  Lactate, Insulin Series, and C.peptide were also drawn.  These results are not back yet.
Baseline Glucose: 84
45 minutes after: 102
60 minutes after: 119
90 minutes after: 101
120 minutes after: 87
180 minutes after: 92
Not too long after the completion of this test, Ian did another Glucose Tolerance Test with his formula recipe, only without the corn starch to help him buffer his sugars.  Once again, Ian passed with flying colors and had not trouble with dumping.
Baseline Glucose: 92
30 minutes after: 110
60 minutes after: 83
90 minutes after: 80
120 minutes after: 85
180 minutes after: 89 (ketones 0.7)
The completion on this test essentially began the fasting test..... the final 180 minute blood draw marks the initial testing of the fasting test.  Ian then was able to have a regular 6:00pm meal, withholding the corn starch again.  Corn starch is meant to help sugars and could skew the results of the testing.  It took 4 hours for a Fellow to come and explain that to me.  A little annoyed, but glad to finally understand some decisions, even though the Formula Glucose Tolerance Test was already over.
So far, Ian ate at 6:00pm...... once he was done, sugars begin testing every 3 hours.  His 9:00pm test showed excellent values of glucose level 97 and ketones 0.7.  His 12:00am test showed values of glucose at 83 and ketones at 0.8.  The ketones should continue to rise as glucose falls.  Once ketones reach 2.5, two glucose stick tests in a row, the test is over because Ian’s sugars are doing what they should.  My previous post talks about ketones, and their importance in the test and in the body.
Dr. Palladino explained a few outcomes of the Fasting Test.
  1. Ian’s glucose falls, but his body increases ketone bodies form fat metabolism for the brain energy and the test continues for long enough, about 16 hours or so and everything is deemed “normal.”
  2. Ian’s glucose falls, but his body once again increases ketone bodies from fat metabolism for the brain energy....... however, this happens quickly and Ian’s body is unable to fast for extended, “normal,” periods of time.  This is called and Immature Fasting System and happens frequently with tube fed children.  Their bodies simply are not used to having to fast because they are continually being fed and keeping sugar levels even.
  3. Ian’s glucose falls, but ketone bodies are NOT created because fat is NOT being metabolized...... a dose of Glucagon is given to breakdown liver glycogen into glucose for energy, but nothing happens.  this once again shows signs of an Immature Fasting System.
  4. Ian’s glucose falls, and again, ketone bodies are NOT created because fat is not being metabolized....... a dose of Glucagon is given to breakdown liver glycogen into glucose for energy, and Ian releases a large dose of glucose.  This signals that there is too much insulin and there is a problem.
Option 1 is the ideal scenario.  Options 2 and 3 create a mild issue that can be resolved over time and growth.  Option 4 signals a medical problem that then needs further attention.  There are other possible outcomes that can signal other medical reasons for Hypoglycemia.  I am not sure what these are, but if those arise, I will keep everyone updated....... Come on OPTION 1!!!
Another sticky subject is the difference between GI, Endocrine, and Dr. Blinman’s Reflux Clinic when it comes to theories and treatment options.  Dr. Blinman tends to think outside the box and is often criticized for his treatments and opinions.  I can tell by speaking GI and Endocrine about dumping and reflux and other concerns with Ian’s history that there is tension there.  They do NOT all agree on how to best treat things.
Bottom line, we have been very happy with the treatment from Dr. Blinman.... and although not initially tested, we believe his diagnosis of Late Dumping Syndrome to be accurate.  The Endocrine Fellows believe Ian does not, and did not have LAte DUmping Syndrome.  While we have always been told by Dr. Blinman that children can outgrow and move on from this, Endocrine doctors are telling us Late Dumping Syndrome is permanent and really never goes away.
I know the food recipe, corn starch included, can also help with reflux, and vomiting, but I firmly believe its has helped Ian with his blood sugars....... even if today’s tests did not show it.  We will see what Dr. Blinman has to say next week when we take all the information to him for review and decision making.  Robin, ian’s dietitian was in today to review some of the things with us.  I could tell she did not agree with some of the reasons for Endo’s tests, but hoping that the information will prove useful next week.  As the Fasting Test continues overnight, we are G tubing Ian some special Sodium Chloride solution here and there overnight to keep him hydrated.  I will let everyone know tomorrow how he is doing.
He was s brave today since he needed a big Iv for blood draws every 30 minutes for the Glucose Tolerance Tests.  This kept him from being stuck to draw blood every time.  Since Ian’s veins are small, they had a hard time drawing blood.  There was lots of pouty kips and crying....... hoping the Fasting Test glucose sticks will be better for him.  He doesn’t even flinch now when they stick him for a glucose test.
On a funny note, Ian has learned where the buttons for the bed are.  He now raises and lowers the head and food of the bed and thinks it is hysterical when mommy goes up and down!!  He always knows how to keep us smiling!  he has been lifted from “room arrest” by infection control and is busy running up and down the halls flirting with nursing students and getting a whole new set of girlfriends in Philadelphia.  He’s such a ladies’ man!  That cute smile, dimples, and blue eyes go a looooooooooooog way!

Tuesday, April 10, 2012

Ritz to Railroad Car

Today was a very uneventful day..... almost a wasted day in my opinion.  Since the Endoscopy and FlexSig were cancelled for today (Norovirus would have skewed any results)... we were simply “observed” by GI for the day.  Ian was a model patient and perfectly behaved for having to be under “room arrest” again all day.
We started to day a little early at 6:00am.  Ian’s connection had come loose sometime in the morning and he fed the bed again!!  He was lying in a puddle of formula and gastric juices, icky!!  Somehow or another, he managed to sleep through it.  This is unusual for him.  The nurse noticed when she came in to turn off his feeding pump...... which I ad already done when the alarm went off.  As a feeding tube mommy, you quickly learn to do the things yourself so you can set up, turn off, take down, and manage any errors on the feeding pumps.  Nurses are busy people and sometimes alarms happen at the worst times!  I usually always disconnect him and lock his feeding tube extension whenever he finishes eating in the early am hours, but for some reason I didn’t this time....... of course it would be the time he was soaked!!
In the early morning, about 8:00am, they came to test Ian’s Cortisol levels again.  Since the phlebotomist is on the floor between 4:00-5:00am, they drew too early yesterday.  Cortisol peaks around 8:00am or so.  They levels were about 11, normal.  Fortunately, all the endocrine tests have come back normal so far.
The rest of the day was spent watching The Wiggles, Sesame Street, Sprout TV, and Baby Einstein DVDs.  I can sing all those songs in my head.  I have to admit that I never get tired of LadyBug Picnic!  Some, like the ones I watched on Sesame, are simply classics.
As he afternoon rolled by and Ian awoke form his second two hour nap of the day, we were discharged from GI and transferred to Endocrinology.  There was an initial concern of whether we could even see Endo due to the Norovirus.  After speaking with the residents and finding out that Ian has not shown any Nororvirus symptoms, such as diarrhea, since Friday, Dr. Palladino accepted him as an inpatient now.
As we packed up all of our belongings......... being 3 hours away form home, traveling to a hospital with a toddler is like packing for a month’s vacation....... we left our Ritz Carlton suite and traveled down the hall.  Little did we know that the Railroad Car was waiting for us.  I now know why everyone is always talking about how wonderful the doctors are at CHOP, because a lot of the rooms don’t have anything good to say about them.
Endocrinology is a smaller unit, with mild-severe patients which are sometimes hospitalized for longer periods of time.  I’m not sure why, but there are only 4 private rooms in this unit.  These rooms are dedicated to children with respiratory contractable illnesses who need isolation.  As a result, we were placed into a double occupancy room.  I didn’t even know hospitals had these anymore until our stay at Johns Hopkins last year.  (Ian was in a Quad room with two other babies and a 6 year old for 2 days, with curtain partitions....... talk about HIPPA violations!)
My initial concern was “How are we going to occupy a 19 months old under room arrest for 3 days or more in a 12X12 space, with two adults?”  My second concern was “Why is he sharing a room if he is contagious and cannot leave the room?”  The answer form the nursing staff was because he simply needs to be 3 feet away form contact.  Now, I’m sorry in advance if I offend anyone..... but seriously, why is a child under room arrest if he is rooming with another child who doesn’t have the same illness?
Ian is only 9 months old and he has already run over to the other boy’s bed 3-4 times and touched tons of things!  I can only chase after him so fast... and I cannot do it time, after time, after time, after time.  This is a less than ideal set-up.  I spoke with a nurse about a private room and it will be at least 2 days for on to open up.... however, if a respiratory illness admits, we will be deferred again.
If I were the little boy’s parents next us, I would be more than upset that Ian was in the room with my son if Ian is “sick enough” to not be allowed to leave the room.  It just seems OFF to me.  Infectious Disease is going to come and meet with us tomorrow hopefully about a release of confinement.  Typical hospital protocol is “contact precautions” until 2 weeks after symptoms disappear.  But........ the virus is usually a 48 hour thing, 4 weeks at most....... Ian has had it for 3 months!  Sorry guys, but I seriously doubt he is still shedding the virus after over 2,500 hours if it is typically a 48 hour bug with a possible 2 weeks, only 336 hour, shedding period.  I am not getting my hopes up and I don’t think it’ll happen.  Infectious Disease may decide that since Ian is not experiencing symptoms and has had the virus for a large extended period of time, to lift the restrictions.
The room has no room for a rocking chair, so Ian and I snuggled in bed and sang songs until he fell asleep.  I have done this more times than any mother should ever have to do.  Naturally, there are mothers who have done it way more than myself, but trust me, no matter how many times you do it....... it is always hard, it is always sad, and it does NOT get any easier.  It doesn’t matter how many tubes, wires, or machines are there........ it isn’t home, it isn’t there bed, and they don’t understand.
Fortunately, Ian is such a super-trooper that he seems to do it all effortlessly.  He seems unfazed at this point that the room is 12X12 and that he cannot leave.  He simply enjoys visiting his roommate and crawling into the large wardrobe closet in the room.  He keeps our spirits up and continues to make us smile.

Tomorrow, Ian will actually have the Glucose Tolerance Test.  Originally, we were told this wouldn’t need to be done since the treatment for the diagnosed, by never tested, late dumping syndrome was working just fine.  However, I believe Dr. Palladino felt it was worth while to observe.  Seeing as though he is the #1 Pediatric Endocrinologist in the country at the #1 Pediatric Endocrinology Program/ Hospital in the country, I thought we should follow his guidance.  This will happen either tomorrow morning or in the afternoon.  After another feeding or two, the fasting test will begin in the evening.  This way, Ian can sleep for some of the test.  After 24 hours of not eating, I can only imagine the crank-pot he is going to be.  I’d be pretty pissed too if someone took away all my food.
Ian was without food for 36 hours back in January, but was still receiving IV dextrose (sugars) along with hydration fluids.  This time, he will receive hydration fluids, but of course, no sugars........ since the test is designed to look at blood sugars, and the ability to buffer your own sugar.
After speaking with the Endocrinology resident this afternoon, I have a better understanding of the test, possible outcomes, and what they all mean.  As the test begins, Ian’s blood sugar is taking with a finger or toe stick.  He then has a regular meal, via his G tube.  After this blood sugars are tested every three hours or so until his sugar drops to below 70.  Seventy is typically the lowest “normal” blood glucose level.  Then, as his sugars drop into the 60s, they will start testing every hour.  Testing begins at 30 minutes intervals once it drops below 60.
As it turns out Ian should be able to fast for 24-36 hours if everything is “normal.”  What happens during the test follows like this...........
****As fasting takes place, glucose is in the blood stream as blood sugars to provide energy for cells and life.  As fasting continues, since no food is going in, glucose levels begin to decline.  This is because insulin is taking up glucose and converting it to glycogen to be stored in the liver for reserves, and because less sugar (food) is going in.
****As blood sugar levels drop lower and lower, the liver begins breaking down glycogen into smaller chain sugars, called glucose, to be used as energy for cells.  In the meantime, the brain is unable to use these types of sugars, so ketones are created.  Ketones are energy reserves broken down from fat cells.  This type of energy is for the brain.
Hence, the presence of ketones is a GOOD thing, as well as stable blood sugars for a longer period of time.  If the blood sugar drops more rapidly, it shows a decrease in the ability to break down glucose from glycogen reserves.  This can be the result of too much insulin.  The excess insulin continues to take up glucose to be converted into glycogen for later, instead of STOPPING so the glucose can remain in the bloodstream for energy.
If ketones are not created, a dose of glucagon is administered.  Glucagon is a hormone produced by the pancreas as well.  It is given to help elevate low blood sugar.  Ideally, if no ketones are present, and glucagon is administered, the blood sugar will not respond...... an immediate response to glucagon is an indicator for Hyperinsulinism.  "Normal" babies and children will NOT react to glucagon and require "regular feeding to bring up blood glucose levels.  Very rarely, individuals do not respond to glucagon that still have troubles.  Those specific medical conditions are unknown to me.  What is significant is, if no ketones are present, and Ian DOES respond to the glucagon, that means he had too much insulin.
Now, how those results are interpreted are also unknown to me.  We will be asking doctors tomorrow about whatever our outcome means and how it plays a role, if any, in his future.
A few things can stop the test:
  1. Ian’s blood sugar drops to 50
  2. Ketones in his blood (sugar sources from broken down fats when glucose is unavailable) appear at a high enough level..... which is unknown to be
The whole idea and system of Endocrinology has never been a strong point of mine.  I remember in all of my medical classes that this, and Embryology, were always the most difficult to follow.  As I speak with friends, family, and doctors, I am doing LOADS of research from my laptop in hospital lounges.  I never take anything at face value and always want to know WHY something works, HOW it works, and WHAT causes it and helps make it better.  Telling me a diagnosis or treatment is never enough to satisfy me.  Call it a hunger for knowledge, the determination of a mother, or the stubbornness of a patient...... I don’t care.  maybe it’s all three!
I have included some links to endocrine testing in my previous post and listed in the resources section below if interested.
I don’t know if I will ever understand how Ian is as strong as he is.  All I know is that I am thankful for his cheery-attitude (lots of us could stand to learn form that) and eagerness to be a normal little boy.  I don’t know if it is simply because his little body keeps on trying and trying, or if it is because it gets a strong determination and stubbornness from all his experiences.  Maybe it is both...... either way, his smiles light up my day.  I have never seen a little boy get such enjoyment out of the tiny things in life.  he certainly never takes ANYTHING for granted, and I believe we should all try and do the same.

We will be here for at least 3 more days...... I will keep everyone posted!  Thanks again for all the support.  In the words of a friend...... our little ones who try so hard and go through medical ordeals that no child should have to undergo should have a TV game show entitled..... “Are You Tougher Than A  Toddler?”  Thanks Jason! :)

Monday, April 9, 2012


Upper GI Series, Endocrine Blood draw and visit, Bacteria/Virus results, Occupational Therapy, Endocrine Fasting test, GERD clinic Dietitian visit, Button upgrade
Endocrine Blood Draw
This morning began early with Endocrine blood draws at 4:30am.  Thankfully Ian went right back to sleep.  They took several vials of blood to test for:
Ammonia levels in the blood
for GI bleeding
Lactate levels in the blood
for glycogen storage disease
Cortisol levels in the blood
for vomiting and diarrhea to rule out pituitary gland malfunction
Insulin-like Growth Factor
for pituitary functioning
Insulin-like Growth Factor-Binding Protein 3
these binding proteins act as “carrier proteins” for Insulin-like Growth Factor
The ammonia and lactate tests came back negative today.  The cortisol showed to be low; however, this tests it to be done between about 7:00 and 9:00am.  The blood was taken at 4:30am, hence the levels were too low.  Cortisol peaks in the bloodstream around 7:00-9:00am.  They will take this again tomorrow morning to make sure all levels are normal.  The two Growth Factor tests will take a few days to return with results.
Upper GI Series
Not to long after this, about 10:00am, we traveled to radiology for the Upper GI Series using fluoroscopy.  This is the 6th study Ian has had, it was nothing new to us.  After putting about 4 ounces of barium through the G-tube, Ian’s Nissen was shown to be “in-tact” and functioning.  The Radiologist was about to see the hernia, but there had been no change since January.  This is good news.  We heard later on from the Dietitian that surgery is indicated when the hernia begins to cause trouble.  It may, or may not, be causing trouble now.  We visit with our CHOP surgeon who manages Ian’s feeding routine and Late Dumping Syndrome next week.
Thankful that the Nissen hadn’t “busted” open, we returned to the room.  Still, we didn’t know what was causing all the vomiting, or the blood in the stool.  Hopefully, the surgeon will be able to shine some light on the future of the Nissen next week.  This week he, OF COURSE, is out of the country and unreachable.  His dietitian has been our liaison there and she is wonderful!
Occupational Therapy
An OT came to visit today to asses Ian and do an overall evaluation.  She, as expected, found Ian to be at expected, normal, levels and said he is doing very well.  She encouraged us to do sensory play and push the sensory boundaries with him to give him more exposure.  We can do this at home, as we have been.
GERD Clinic Dietitian Visit
Robin is Ian’s Dietitian here at CHOP.  She works with the surgeon, Dr. Blinman, running the GERD clinic.  Lots of these patients, such as Ian, are children who have had Nissens and such that continue to have problems.  Dr. Blinman and his team are very “outside-the-box” thinkers and their techniques are criticized all over.  I can tell you though, Ian has always been an “outside-the-box” kid, and they have been the first ones to help us when it comes to GI reflux in a LOOOOOOONG time.
Most of their kids revolve around Nissens that have created dumping issues as a side effect, Ian included.  They have a long list of things they move along to find each kid’s “Happy Place.”  The feeding routine, recipe, including the additive of Microlipid, Pectin, and Cornstarch are all theories of their program.  The Microlipid is designed to give longer-chain fats to the diet to allow Ian more time to break them down without the fats “flying” through his system during the “dump.”  This elevated blood sugar would in turn, trigger large insulin releases.  The pectin is literally canning pectin.  This is added to slow it down in the intestines and allow for more time as well.  The cornstarch is added, both for calories, and for extra carbohydrates for him to break down..... avoiding more simple-sugars.
talks a little about fats and cornstarch to help “buffer” blood sugar levels
talks about Microlipid, long chain triglycerides, as a way to delay gastric emptying and prevent “dumping.”
talks about pectin and how it slows glucose uptake in the blood
Ian’s surgeon’s article on the frontier of post-prandial hypoglycemia, late dumping syndrome
While most of what I just talked about is a re-hash of previous discussions, I feel like with everything going on, it is important to understand why Ian is on the diet he is on, especially since a lot of GI doctors do not agree with this treatment.  I’m not sure why, but I get the feeling form a lot of doctors while we are here that they feel it is very “experimental.”
Robin and I discussed again why what we are doing has helped and she reassured me that Dr. Blinman would discuss any vomiting, Nissen, feeding issues when we see him next week.
Bacteria/Virus Test Results
All of Ian’s stool tests are finally back.  All the of bacteria, including c.diff (for the 7th time) have come back negative.  The viral testing showed something different.  Ian has test positive for Norovirus!  While norovirus is responsible for about 80% of gastroenteritis, it is not usually tested for, unsure why... but back in January when he had THE BUG, they did not test for this.  Ian’s months of diarrhea and recent increase in vomiting have been linked to this virus and the idea that he has had it for three months.
After speaking with several of the doctors, they have never known anyone to test positive or hold-on the norovirus for that long..... 4 weeks was the maximum.  This is what we most likely ALL HAD back in January.  The virus usually runs a 1-3 days course and has a 1-2 day incubation period.  The symptoms are vomiting, nausea, headache, joint aches, fatigue, and watery diarrhea.

This virus is common among “close settings” such as dorms, camps, nursing homes, and...... wait for it...... HOSPITALS!  Most likely, Ian picked this up when hospitalized for the nosebleed in January, or when visiting CHOP as outpatient to see Dr. Blinman for feeding clinic or nosebleed/ hernia follow-up.
The bleeding is most likely coming from colitis.
The fact that Ian has had the diarrhea for three months certainly explains that his colon is “unhappy.”  Unfortunately, there is nothing the doctors can do for it and it will need to run its course.  The treatment is only for symptoms, such as dehydration and hypoglycemia.... none of which Ian is experiencing.  He does not seem to be in pain and is not losing weight.  In fact, he is gaining weight!  He seems happy and jovial even though he finally threw up today for them.
We have had one bloody diarrhea diaper since being here and one vomit.... go figure.  At least they have been able to see something.  So there isn’t much they can do at this point other than to monitor for symptoms to worsen and cause problems with hydration.  Unfortunately, this also means Ian is still under “room arrest.”  Norovirus is highly contagious.  I suppose since we all have had it, there is less risk of us catching it, again.
Endocrine Fasting Test
Hopefully tomorrow we can be discharge from GI’s care and moved to Endocrine’s floor for the insulin and blood glucose testing.  We may or may not have to change rooms for that.  Ian’s IV was able to come out tonight.  The IV needed for the endo test needs to be a new one that has never had fluids flushed through it, and needs to be able to draw blood.  They were going to need to give him a new one anyway.  He may or may not need a second one, depending on fluids.  Hopefully, the saline hydration fluids can be given through his G-tube and not through a second IV.
This is an example of the testing Ian will have.  It test for elevated insulin and possible insulinomas that might throw off blood sugars.  Thankfully, the Metabolism screening for Fatty Acid Quantification that was done included LCHAD... which is a fatty acid metabolism defect that can sometimes be passed recessively through HELLP syndrome, which occurred while I was pregnant.
The fasting test will watch to see what Ian’s blood sugar does when essentially starved, to make sure he can use reserves to help buffer and provide glucose to stabilize his own blood sugar.  If I understand correctly, this will indicate if there is a genetic insulin problem that is contributing to Ian’s blood sugar hypoglycemia.
LCHAS deficiency
If all of this provides normal results, there is essentially no risk of hyperinsulinism problems.  Fortunately, CHOP is the #1 program in the country and the only Endocrinology Attending Physician is ranked #1 in the country as well.  If there is a problem, they will find it!
Once Endo has finished with Ian, we will be able to go home.  His fasting test will most likely be 18-36 hours.  He may not be given a glucagon stimulation once the blood sugars drop.  They will also be drawing blood almost every hour for glucose testing and to look for ketones.
Glucagon Testing
Ketone Testing
We are hoping Ian’s only insulin problems are the post-prandial hypoglycemia from Late Dumping Syndrome that were exacerbated by the gastroenteritis, creating what is called ketotic hypoglycemia.
Ketotic Hypoglycemia’s first episode can be attributed to severe gastroenteritis infection, which Ian clearly had, or may still have.
Button Upgrade
Finally, earlier today, the G tube Nurse Practitioners came in to “size” Ian for a new button.  His AMT MIMI ONE is currently a 12 French, 1.2cm size.  We have been getting some leakage and irritation.... a sign that the button might be too small.  Ian now ha a longer button, 1.5cm.  The French, or diameter if you will, of the button is the same.  Essentially, his button is longer, but not any bigger around, so the hole does not need to be stretched or dilated to fit the new size.

Sunday, April 8, 2012


Easter at CHOP turned out to be almost as fun as Easter at home.  We made sure to bring Ian’s Easter basket to the hospital when we left Friday evening.  It was filled with goodies for him.  He had fun playing with his new toys, and of course, the Easter grass.  He was filled with smiles and laughter again... such a blessing.
Family was also here to visit today.  All 4 grandparents were helping us celebrate the holiday.  Even the hospital brought Ian a small blue bunny stuffed animal for the occasion.  Ian especially enjoyed tossing the plastic eggs off the hospital bed and onto the tile floor in his room.  He watched some new DVDs and enjoyed some new books.
As the day went on, he kept up his good spirits.  His new favorite toy is the cushion that comes off the back of the sleeper couch.  It is long and narrow.  Since it has the feel and texture of the foam padded toy cushions kids play with, he loves it.  He stands on top of it since it is only about 5 inches tall or so and then wiggles on it.  He looks like he is surfing.
This wiggle hips dance he does now to most music he hears is too cute.  He’s our own little Elvis.  He also makes fishy mouths now and is beginning to blow kisses.  He is waving when everyone leaves.  When the medical professionals of any kind enter the room, he is always suspicious of them, but is never reluctant to wave when they leave... almost to say “Bye bye, don’t let the door hit you on the way out lady!”
Today was not a very medical eventful day... which was kinda nice since it was a special Easter day.  The GI doctors are still planning on the Upper GI series tomorrow sometime.  He will be an add-on, most likely in the morning time frame, nothing definite yet.  When he is done eating at 4am, they will begin his fast.  He will need a certain amount of time without food before the study is done.  IV fluids will start when the fasting starts.
The Upper GI series will commence with the swallowing of barium..... this is always a chore, because Ian doesn’t drink, or swallow, without a fight MOST of the time.  (Although he ate another french fry today)  Once he swallows, the barium shows up on a radiographic video image on a screen.  You can then see it move through his digestive system as white fluid on the monitor.  This will show movement, such as reflux, and also areas of anatomical defect, such as his hiatal Nissen hernia.  Depending on what they see, an Endoscopy or Colonoscopy will most likely be done on Tuesday.  He will need more fasting and anesthesia for that procedure.
Back to today... we also received another consult from Enodcrinology.  This time, and attending physician came.  Yesterday, without my knowing, a fellow came.  Not that she was perfectly capable of handling the case, but I could tell the attending was here because he did not agree with some of her thoughts and recommendations.  He believes that there is no sense in running an Oral Glucose Tolerance Test, glucose drinking test.  If Ian DOES have post-prandial hypoglycemia, or late dumping syndrome, and it was treated and is working, then he doesn’t see the point in testing for it.  He recommendation is to continue “treating” the late dumping syndrome and if Ian begins to have trouble, then do the test.
However, he did offer to us the fasting test to see if Ian can stabilizes his own blood sugar without food.  We accepted this offer.  However, it take special staffing and blood draws.  Because of this, it will be done after GI is done, and on the Endocrine floor.  That way Endocrine nursing staff and medical teams are there to carry out the procedure.  Ian will need to fast for about 24 hours.
The only other medical information from today is blood sugar testing.  Ian ate his 3pm meal, and then the testing began.  Sugar was tested immediately after finishing the feeding, and the every 30 minutes again for three hours.  This is the same test that was run yesterday.  The Endo doctor wanted it repeated.  Ian’s post-prandial (after-eating) blood sugars were:
Day 1: Yesterday (Saturday)
Immediately after eating..... not taken
30 minutes after eating....... 106
60 minutes after eating....... 86
90 minutes after eating....... 89
120 minutes after eating..... 88
150 minutes after eating..... 89
180 minutes after eating..... 90
Day 2: Today (Sunday)
Immediately after eating..... 102
30 minutes after eating....... 99
60 minutes after eating....... 102
90 minutes after eating....... 105
120 minutes after eating..... 100
150 minutes after eating..... 92
180 minutes after eating..... 99
Back in January, when the current feeding regimen was tested before sending us home:
Fasting............................ 108
Immediately after............ 149
30 minutes after eating... 82
60 minutes after eating... 77
90 minutes after eating... 91
Once we get the GI issues (Vomiting, diarrhea, blood, and mucus) resolved, we can move on to Endo issues if need be.
We are still waiting for the Feeding/ Swallowing center consult.  That will be a weekday, sometime soon.  Most doctors here continue to agree Ian needs an intensive therapy program, especially after seeing him gag and retch after seeing and touching foods.
We finally collected enough poop to send off a sample for cultures and testing.  We are still confined to the room until those return with negative c.diff results.  If, for some reason, this... the 7th c.diff test should come back positive, who knows what happens then?  They are not expecting a positive result, but hospital policy will not allow Ian to leave without a negative result.
While collecting the remaining poop sample, Ian decided to give the most normal looking poop ever this morning!  Thanks Ian :(  I made sure to notify the doctors that this is NOT what it looks like, ever!!  Regardless, he did go again this afternoon, pretty normal as well.... at least one of those tested negative for blood presence.  Once again, showing that blood is not always in the diarrhea, just about half the time, like we mentioned.  I was corrected and told that the fecal blood test was POSITIVE for yesterday’s stool.  Originally, we were told it was negative..... but we visibly saw the blood in the diaper.  We were told today it was most definitely positive.
The previous post from today included some pictures of Ian this morning with his Basket along with a few cute videos.  He continues to amaze me at how brave and “relaxed” he seems to be..... relaxed for a kid in a hospital, of course.  I will make sure to keep everyone posted on the day’s events tomorrow.  We are all curious to see what the Nissen is doing!!!
A few studies favoring Toupet over Nissen procedures when it comes to decreased side effects, such as dumping and dysphagia (difficulty swallowing)