Eat Happy!

Eat Happy!


**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.**

**Resources are listed to support information associated with this BLOG. These resources support copyrights and are permissible. Information presented outside of this BLOG needs to incorporate resource sites to maintain legal status.**

**This BLOG and its information may be shared at no request; photographs will need permission from the blogger.**

Wednesday, January 23, 2013

Before-During-After Air

OK tubie momma engineers....... I have a question........ or more likely........... a challenge!!

Ian has a lot of trouble with gas/air in his belly. It happens all the time. It happens before, during, and after meals. We always make sure to vent him before his meals. Sometimes there is air, and sometimes there is nothing. If there is nothing, this does not mean there won’t be during or after his meals. So......... the trouble comes on how we can vent air out of his belly during and after meals. This sounds like a simply question, but it is far from simplistic.

Farrell Valve Bags are designed to decompress the belly while eating. They allow food, air, and gastric contents to follow out of the body when the stomach is overfilled. it also allows the contents to flow back in as the body allows. The trouble with this is *everything* flows back in: food, gastric content (desired) and the air (undesired).

Farrell Valve Bag

The way the Farrell Valve Bags are designed, there is nothing in the bag before it is used. There is a small one-way valve at the top right of the bag. As contents flow into the bag, it is almost as if a vacuum is created. While the air is allowed to escape at the top of the bag, there is not enough pressure to push the air all the way through the tubing. With food in the tube as well, the pressure of the belly decompressing is not strong enough for the air to pass the food and be released within the bag...... allowing the food to flow back in. As a child or adult who *needs* decompression in the first place, the intake of air along with the acceptance of food is a bad idea.

Farrell Valve Bag

........... essentially, if the air cannot be pushed all the way through the tubing, as the food flows back in....... everything in the tubing does, including the air.

But of course Ian has extenuating circumstance! To make matters worse, he has.......

***** Fundoplication:

Ian has a fundoplication. A fundo is when the top of the stomach (fundus) is pulled upward and wrapped around the base of the esophagus from behind. It is then stitched to the esophagus itself in a various numbers of ways. Ian has a three-quarter wrap, called a Toupet. Many fundos are Nissens, a full 360 degree wrap. Either way, it is very difficult for stomach contents to escape....... which is the purpose of the fundo! Fundos are done in cases of severe reflux when the muscles and lower-esophageal-sphincter is unable to keep contents down. The wrap helps generate that ability because as the stomach contracts to reflux or vomit, the fundo contracts around the base of the stomach, restricting the release of contents into the throat.

***** Delayed Gastric Emptying:

Ian also has delayed gastric emptying (DGE). Many many many times, it is a bad idea to do a fundo if someone has DGE. It creates a “pressure-cooker” type stomach. With no where for contents to escape, everything must wait and be sent through the intestines. Ian can burp or vomit, but he REALLY has to want it, which only happens when he has a stomach bug. Often times, DGE patients will have a fundo with a pyloroplasty. A pyloroplasty is when the pylorus, bottom opening of the stomach is opened more than its original position. It can be cut or dilated. This allows for quicker emptying of the stomach contents when it is overfilled and cannot escape through the fundus. This provides a lot of relief for patients.

Ian's Gastric Emptying Scan 10.2011: He somehow fell asleep!

***** Post-Prandial Hypoglycemia:

Thirdly, Ian has post-prandial hypoglycemia, known as late-dumping syndrome. What happens when a fundo is performed doesn’t only make the fundus-esophageal junction stronger, it changes the shape of the stomach. As the top of the stomach is pulled upward and stretched, it elongates the stomach. In addition to that, the stomach is now smaller because a portion has been used to create the wrap. The stomach is anatomically designed the way it needs to be to push food along. When the stomach changes shape, so does the way it empties. The contractions that push the food along through the stomach are called peristalsis. Peristalsis is not performed the same way after a fundo. 

Messing with the way the stomach empties can cause a multitude of other issues. Ian’s stomach empties slowly, the DGE......... but, when it *does* empty, the food flies out of the stomach very quickly, aka: dumping. As this happens, the blood is flooded with glucose. When glucose enters the bloodstream, insulin is released to take up the unneeded portion and stores it for later. There is something that happens with dumping that signals a LARGE amount of insulin to be released. This “something” is unknown. Ultimately, more insulin is released than needed. The blood runs out of glucose before it runs out of insulin. This creates hypoglycemia (low blood sugar). This hypoglycemia causes sweating, nausea, vomiting. 

The higher the carbohydrate load, the worse the response. Using more complex carbs helps delay the digestion process and postpose glucose drops. When the large amount of insulin is released, it does not happen all at once. It continues to do this in order to “take-care-of” all the glucose in the bloodstream. The body responds to the administration of glucose, raising the blood sugar, and restoring the balance. 

***** Hypersensitive Vagal Response: 

The vagal response is the one that can bring on gagging and retching. When the vagus nerve is *triggered* by a stimulus (vomiting, food or mucus in the throat, bearing down for a bowel movement) gagging and retching occur. Vomiting can be an end result. Ian’s vagus nerve is very sensitive and can be triggered by lying down after a meal. It is hypersensitive from the fundo. The nerve runs through the area and is *disrupted* during surgery.

Vagus nerve passing through the top of the stomach

When the fundo contracts to keep in stomach contents, it irritates the vagus nerve. As the food sloshes around and near his esophagus, he begins to retch and gag, strongly! It is also triggered by sitting up too quickly after a meal, as the stomach is compressed when it is full. Because of this, we are often unable to lie Ian down during a meal to vent him. We certainly are not able to lie him down after a meal, with a full belly, for venting. 

Here are a few images of the vagus nerve and its pathway through the throat and innervation of the stomach. There is much more detail than what is needed.... but it shows the placement, the emetic (vomit) reflex that is triggered, and swallowing process.

Vagus nerve passing from the brain, through the neck, and into the stomach

How the Vagus nerve affect the upper GI tract

Image of the swallowing phases, which can irritate the Vagus nerve

An *extremely* over-exagerated picture!!!
However, it does show that the emetic (vomiting) reflex in the brain is directly linked to the Vagus nerve.
The cytotoxic chemo is irrelevant to our discussion.  "Vagal afferents" means "sensing."



As a recap, Ian has a lot of air in his belly before, during, and after meals. He is unable to release air through burping because of the fundo. His belly also doesn’t emptying properly due to DGE. He holds food in his stomach for a long time and releases it all at once, creating dumping and hypoglycemia...... all of these interacting with a hyperactive Vagus nerve.  Ian’s hypoglycemia and dumping are *relieved* by complex carbs and slow feedings. Throughout the entire digestion process, air is created through the stomach and intestines. THIS is the air we need to escape during and after his meals. Farrell Valve Bags are insufficient in allowing the air to escape.

Here is the challenge....... We need a way for this air to escape, other than venting him before, multiple times during, and after his meals, allowing for the free flow of food into the stomach, while feeding him slowly by his feeding pump.

Historical attempts include using the Farrell Valve Bags and periodic venting. We tried placing the bag at higher and lower levels to try and mess with gravity and pressure. I even cut a hole in the bag in different sizes and at various levels in hopes of relieving some pressure within the bag. The air was still unable to escape. Then I tried rearranging the tubing so that gravity pulled in multiple ways....... still no luck. After an hour in the bathroom with a feeding bag, backpack, tubing, Farrell Valve Bag, pump, syringes, and some blue food coloring in water for visual, I was unsuccessful...... and messy. Thankfully I had the end of the tubing extension in the bathtub to minimize the mess.

Ideally, it would be great if could somehow rig a 60mL syringe in a manner that the food would neither spill out nor be overflowed from too much decompression. It might be unrealistic to expect this result with all of Ian’s issues........ including the one that I cannot get a two year old to sit and do something while he eats....... I’ll keep trying on that one!

60 mL syringe attached to some tubing for venting

Without an evolutionary idea, we will continue taking our chances with lying him down before, during, and after meals for periodic venting.

OK creative mommies........ GO!

Thursday, January 17, 2013

Preschool Scholastic book fair

We just took Ian shopping tonight at his school book fair to help raise money for the school's books.

He got some awesome new books!

He even got to play with Clifford!!!!

Growth Assessments

Ian recently had a consultation in Manhattan for Russell-Silver Syndrome. While I want to write about that, I discovered that what I was writing had no basic foundation without talking about growth in general first. So, here goes..............


When Ian was born, he was 930 grams, which is .93kg. This equates to two pounds and one ounce. To give you a frame of reference, an average baby born at 40 weeks, is typically around seven pounds and nine ounces, give or take some. It equates to about 3500grams, or 3.5kg. However, a baby born at 29 weeks, such as Ian is estimated to weigh two pounds eight ounces or so. This is 1153 grams or 1.153kg. Clearly, Ian was smaller, even for being born at 29 weeks.

Ian, One day old

The same can be said of length. A full term baby of 40 weeks would be expected to be around 20 inches, which comes to around 51cm. A 29 weeker would average 15 inches or so. That is just over 38.5cm. Ian was 34cm, which is about one-tenth shy of 13.5 inches. Once again, notably smaller.

In my opinion, it is much easier to see numbers when they are listed separately.

40 weeks 7 lbs. 9 oz.
29 weeks 2 lbs. 8 oz.
Ian 2 lbs. 1 oz.

40 weeks 20 in.
29 weeks 15 in.
Ian 13.5 in.

*These figures are estimates based on averaging various site’s numbers. The values for the “averages” and “guidelines” are not set in stone for taken from a perfect medical profile.

Because of Ian’s weight and length at birth, he fits into several “growth” categories, all having their own sales. Ian is Small for Gestational Age (SGA), Extremely Low Birth Weight (ELBW), and Intrauterine Growth Restriction (IUGR). While all revolving around being “smaller” than expected, they all have their different meanings and references.

IUGR and SGA are very easily confused.

IUGR refers to “something” that is causing the baby to not grow as they should. It signifies poor growth in the womb and means the baby is below the 10th percentile. IUGR typically signifies some type of pathology...... something isn’t going right and is causing the condition. SGA on the other hand describes ANY baby who is below the 3rd percentile. SGA babies can be IUGR babies, but not all of them are. SGA could simply mean that you grew a small baby. Both conditions can be identified during pregnancy. SGA babies come with their own sets of issues and concerns. Ian embodies quite a few of them.

The MAGIC Foundation has excellent information on these conditions.

Certain indicators and/or health conditions can bring on IUGR. Some contributors to IUGR are: decreased oxygen availability and nutrition due to high altitudes, multiples, problems with the placenta, preeclampsia and/or eclampsia. Chromosomal abnormalities and infections can also cause IUGR. Risk factors can include alcohol abuse, drugs, smoking and poor nutrition. However, clotting disorders, high blood pressure, heart and kidney disease are also considered.

Me, 28 weeks pregnant, 7 days before Ian was born

IUGR can be either symmetrical or asymmetrical. Symmetrical implies the baby is proportionately small all over. Asymmetrical IUGR means that the baby is undernourished and is focusing all of it’s growth on vital organs, such as the brain and heart. Some of the liver, muscles, and fat are sacrificed to redirect growth. The head is of normal size. Asymmetrical SGA babies usually have small abdominal circumference from smaller livers, thin skin from lack of fat, and skinny limbs from reduced muscle mass.

It was noted that Ian was experiencing IUGR at the level two anatomy ultrasound at 20 weeks. The previous ultrasounds were six, ten, and twelve weeks...... all showing no evidence of IUGR. Ian’s IUGR was asymmetrical. His head always measured above the 50th percentile, but his abdominal circumference and femur length were almost always below the 20th percentile. As the weeks went by, Ian was monitored more frequently by ultrasound. As my HELLP syndrome gradually came into existence, Ian’s ultrasounds showed decreased growth. IUGR can lead to fetal mortality if the brain is compromised. Ultimately, as my HELLP became severe, in addition to maternal mortality risks, Ian’s risk was great as well.

It wasn’t until recently that this all began to come into the light. As we discussed beginning growth hormones based on IUGR and SGA diagnoses, the terms came into the light a little bit more. As an IUGR and SGA baby...... and child...... Ian’s organs and his stature are compromised. Growth hormones will give him an extra chance for his body to do more than it can on his own. He will begin his growth hormones on Friday.

SGA children typically catch up in growth by age two. About 10% of children do not make-up for this . Ian, of course, is in this group. For most of these children, growth hormones can help accelerate that growth. Unfortunately, the IUGR and SGA keep Ian from growing in all areas, not just stature. Ian’s lungs, digestive tract, and other organs will hopefully be able to grow and mature the way they should. Since Ian has chronic lung disease, any extra help is worth a try.

This wan’t an easy decision for us to make. While we would love for him to have the added assistance and extra height, it is a nightly injection with a needle. As a two year old, Ian is *very* tolerant of what needs to be done. He will even sleep through his glucose and ketone checks. Originally, Tom and I believed the growth hormone “pens” were the same type of sharp, quick stick. Once we started learning more about them, we discovered it was an open needle that needed to remain under his skin for at least six seconds while administering the dose. I do believe over time he will become accustom to the routine.

As a dental hygienist, I am quite familiar with needles and injections. The higher the “gauge” number, the thinner the needle. As a point of reference...

Tongue piercing jewelry barbells are usually 10-14 gauge

Belly button piercing jewelry is typically 14-16 gauge

Ear piercing guns use 20 gauge needles

Dental needles typically run 27-30 gauge

Most glucometer lancets are 28 gauge

Ian’s growth hormone needles are 32 gauge. Hopefully, this will be easier for him to get used to since he can already sleep through the 28 gauge.

Endocrinologists vary greatly on the recommended starting dose. While I won’t be posting his starter dose, I can tell you that the doses are typically based on milligrams per kilogram, per week (mg/kg/wk). The pens have dose markers on them so you can “Set” the dose needed. Each pen contains a certain amount of milligrams. Ian’s pens will be 10mg each. As each dose is administered, it is simply subtracted from the total in the pen, 10mg. When the pen is empty, another pen picks up where the previous left off.

They make the pens super cool so kids, teens, and adults simply are stabbing themselves with blue pens. Ian’s pens are norditropin by Novo Nordisk.

They even have stickers and charms you put on the pens to make them more fun.

norditropin FlexPro pens

norditropin Starter Kit

Example of some pens stickers

Example of a pen charm
The kit comes with a backpack, carrying case, and cooler to help make traveling easier. Ian already has a diaper bag, medicine bag, nebulizer backpack, and an overnight bag when we travel. Tom and I are thinking we might be able to combine the growth hormone backpack into one of the other..... but since they are all full to the brim, it doesn’t look good.

We are becoming quite the pack mules. I am feeling as though I need a hand truck to transport everything. While we might not need *every* thing and *every* moment, you can’t really go to the store without it in the car. That would be the time he yanks out his G tube, has an asthma attack, or becomes hypoglycemic.

Back on track...........

In addition to IUGR and SGA, there is another classifying growth category. Low birth weights fall into three tiers. Ian was born in the third tier, weighing only 930g.

Low Birth Weight babies (LBW) <2500g <2.5kg

Very Low Birth Weight babies (VLBW) <1500g <1.5kg

Extremely Low Birth Weight babies (ELBW) <1000g <1.0kg

The two main causes for low birth weights are IUGR and preterm births. Ian was subject to both of these.... and was even small for a 29 weeker, moving him down into the ELBW category.

While Ian falls into all three of the lower, or poorer, groups, he always finds a way to defy the odds. He has been diagnosed as IUGR, SGA, and is classified as ELBW. Many children who fall into these three groups have multiple medical conditions. We are EXTREMELY fortunate to have as healthy of a child as we do. Everything becomes relative. While we may never blend into the “normal” (whatever that is) but we are also very blessed for Ian to have the health he does. We are a very blessed family and are thankful everyday for all the progress and milestones. We have a truly happy little boy that we love very much.

*Information in the posting is from numerous pages which are listed in the Endocrine resources section since they pertain to growth.

Happy Holidays

As 2012 came to a close, we were blessed to have Ian well for Christmas and New Years. He was home for about 10 days after his RSV visit for the holidays. Christmas eve brought snow to northern Virginia. Too bad it only lasted a few hours. We were able to take Ian out for a little bit before it all melted. This was his first experience with snow. it was fairly uneventful seeing as though there was barely enough snow to cover the ground. I think his favorite part was watching the snow fall. He had a great view from the bathroom window on the third floor of our town house.

Shortly after the snow stopped, the rain washed it all away. Thankfully, Ian and Tom were able to play a little on the deck before it was all gone. Once he was asleep for the night, we piled all his presents from Santa under the tree. The next morning, he was excited to find his stocking filled with all sorts of goodies. We had a great morning of sitting by the tree watching smiles kiss his face all over. He played with blocks, cars, and farm animals.

We had a great day with family. As usual, the time went by way too fast and Christmas was over. It felt especially short this year since we didn’t have much time before to decorate and get excited. It was sweet to see Ian having fun and doing a great job ripping paper off of presents. Ian did receive a doctor’s kit for Christmas from his MiMi. He knows how to use all the pieces. The kit has a stethoscope, blood pressure cuff, intra-oral mirror, scissors, an otoscope, syringe, and a beeper. He actually looks pretty darn cute using the kit.

We can only hope that 2013 will bring healthier times for our little boy. He is doing well right now and has a lot of great things coming up in the new year. He will be enrolled in the eight week day-patient program at The Children’s Hospital of Richmond. He also starts growth hormone therapy in January under the diagnosis of Small For Gestational Age and for Intrauterine Growth Restriction. He also start speech therapy with the schools and will be visiting them twice a week. We have a lot to look forward to in 2013!

As always, I hold a special place in my heart for the souls of children who flew away with angel wings this year. Whether it be children who have learned far more than they should ever have to about life, sickness, pain, and loss..... or those ripped away during horrific violence..... the loss of a child leaves a void which is never filled. Before opening our gifts on Christmas day, we took a moment of silence to remember those children and their families.