49 Weeks in the making

My mother has waited since late April of 2006 to have my whole family together. We tried to get together for Christmas but it was a no-go because of snotty noses.

Today it finally happened.

It didn't seem significant to me until I saw the picture with all of us in it which was a remarkable glimpse of what we should have been doing last summer, fall and winter as we did our time at Children's. But all's well that ends well and we may have to adopt the sign on the wall as our new mantra:

Keep Calm and Carry On.

We had a great time reintroducing Ellie to her cousin Audrey who was born three weeks after Ellie.

Audrey wasn't quite sure of the new baby in her world. My money is on Ellie's precision strikes against Audrey's size.

Our TPN routine has gotten better and tonight's set-up went without a hitch which made us realize that we wish that we had brought supplies for more than one night up here. If this trial run keeps going well we will extend our trips.

It sure is nice to have a lot of hands to watch Ellie and let Abby and I veg a bit. Even for 15-20 minutes it is nice to have some help.

OMEGAVEN!!!!!!!

We picked the tittle for this post because it is what has saved our daughter's life. She was born with "Short Bowel Syndrome" and there-fore at risk of TPN Associated Liver Disease. We are in contact with many other families that have had the same positive experience with Omegaven.

Please! if you are looking for more information contact Dr. Puder at Children's hospital Boston. We also praise Omegaven through out this blog for saving and protecting our short gut daughter's liver. Please feel free to read on, and please contact us if you have any questions. We would love to help in anyway that we can.

Now that Omegaven has saved our daughter's life we feel the need to help save other kids lives.

A good place to look for more information is the short gut wiki, it is also a good place to find general short gut info.
grey.colorado.edu/shortgut/index.php/Omegaven


____________
OFFICIALLY, NOTHING COOL HAPPENED TODAY.

OFFICIALLY.


We leave tomorrow morning for Maine for an overnight stay. It will Ellie's first trip north and my first in over a year. Ellie is very excited, I really think I heard her say "Maine is the best!" right before she went to bed tonight.

To Boston to Boston…

Tomorrow we head to Children’s for a non-medical visit. This visit has two parts and we are looking forward to both of them for different reasons.

In the morning we will OFFICIALLY DO NOTHING COOL.

OFFICIALLY......

In the afternoon we will be going to a memorial service for Carol, one of the great nurses at Children’s who passed away this winter after fighting cancer. Carol’s first day back to work after chemo was our first day at Children’s. She was a great friend and helped us as we went through some really bad stuff making decisions about Ellie’s care. She truly loved Ellie and was an outspoken advocate for her. After her cancer came back last fall and she was not working she would make a point of visiting Ellie when she came to the hospital around her visits to Dana-Farber. Unfortunately, we could not go to her funeral earlier this winter because Ellie was back in the hospital after her prolapsed ostomy.

She loved the tiny bits of progress that Ellie made and would really get a kick out of the smiling happy girl that is at home right now ( Video Link1) learning to walk (Video link2) and the little girl in the pink dress that will be giggling at her memorial service.

We miss her.

New feeding and old friends

Kara and Sophie came over today for a goodbye party for the Delvins as they get ready to move south. Liz, Hank and Ryder are all moving to Tennessee to become good old organic farmers. There are some pictures of the party mixed into this post. Below is Liz and her boy Ryder.
It has been two weeks since our last visit to Children's and since we got to see Ellie's new bowels. We have been changing our approach to her feeding now that her bowels are the normal size. When we first got home she would be on the pump over night a rate of 3cc and hour and during the day we would give her 2 bottles in the morning, put her on the pump for the middle of the day and then give her 2 bottles at night. Our main goal at that time was just to get her bowels working. Dr. Jennings wanted us to really work the bottles to get a "cephalic phase response". That is when the tongue tastes food it starts salivation in the mouth, then the stomach starts emptying and motility increases in the small intestine all in preparation for food. His theory was that through practice we could wake up the digestive system and get it all working better as a whole. Below is Ellie and her new best friend Sophie.
Now we still give her bottles but we don't have to worry about her intestines moving the milk we just do it so that she will remember what eating is like. It has taken us 2 weeks but we managed to get Ellie on the pump for 24 hours a day running at 20cc/hr with a daily total around 500. We did not increase the over all amount that she gets everyday but, she did gain the all the weight that she should have for the time period. That means that by spreading the food out more evenly through the day she was able to absorb more out of her food. That also means that the big 3-4 ounce bottles that we were giving her were just overwhelming her intestines and flowing right out the ostomy before she could absorb anything from the milk.So, for now we are going to stay were we are for food and not increase again, but we are going to start partially plugging her ostomy more often and for longer periods of time. Plugging the ostomy, you might ask how one would be able to do such a thing? Well we use a G-tube. We needed something that was small, thin and soft and a MICKY Button G-tube fits perfectly. We are not hoping to pug it completely just make it a bit harder for fluid to go out the ostomy so more will go down into her colon. When the fluid goes down into her colon her body will be able to reabsorb some of the liquid out of it and there fore increase her overall hydration. The small bowel also has to work a bit harder to push the food down instead of out so this is our way of doing bowel strength training.

We are not completely positive that this will work but, it is worth a try. We have a feeling that the G-tube in her ostomy may actually act as a stint and keep the ostomy open and making it easier for food to go out the ostomy. And this is not what we want. But, hey... this is all trial and error so, we just keep on trying new things.
It was great to be able to spend some time with our friends before they left and also fun to see our kids together. We will all miss Liz and her family, but now have reason to go to Tennessee. And Ellie now has a new boyfriend and this one I know will be grow up to be a real southern gentleman. Other good news.. Early intervention came to visit last week to evaluate Ellie for home physical therapy, and..... she got turned down! They said she was at age and even advanced for a 11 month old. Way to go Ellie!

Another one for the books

Last week a doctor in England published a study that reported that kids with a lot of fish in their diets learn faster than kids without because of the omega-3 fatty acids that build their brains. brains:http://news.bbc.co.uk/1/hi/health/6440979.stm

This goes right along with what Dr. Puder had suggested earlier this year.

We really didn't have anything to compare it to any have no idea if she is advanced, but today's tricks made us wonder:

http://video.google.com/videoplay?docid=-6354059401325918517

This may be another case of normal parent things that we are experiencing for the first time, but it was cool anyway.

We think she is super smart.

Play Time

We had another play date today. We went to Jamaica Plain to see Max and Christian. They both are doing very well.

Ellie managed to charm them both and didn't make them cry nearly as much this time. Both of these little boys are tough little troupers that have had to handle a lot for their age.

Max is the boy all the way to the right in the picture. He is 9 months old and from Bolder Colorado. Yea Colorado! He was born with Hirchsprung's disease which has left him with a very short gut. He is here in Boston to get on Omegaven and so far, it's working! All of the numbers that determine liver health have started to head in the right direction. You can tell he is getting better because he is looking better and getting stronger. He wowed us all by showing off his standing legs today.

You can't really tell so much from this picture but, he used to have a strong yellow tint to his skin. This is the result of high levels of bilirubin in the system due to liver damage from the TPN. As Omegaven does it's thing and his liver gets better and better the yellow color will slowly disappear.

Christian is now 3 months post transplant and is doing great. He is doing so well that they are going to send him home to New Jersey in the beginning of April. This is a huge deal because most children are not even out of the hospital 3 months after transplant much less allowed to leave the state. He is a tough little boy that loves to bash things with his head. He and Ellie had matching shiners a few weeks ago, to bad we didn't get a picture. He is such a different kid than the little boy that we met back in August. He is now a strong, solid little boy that likes to point to interesting things and say "WOW".

This is a picture of their play date back in February. I just love the picture so much I had to post it. Colleen aptly titled it "I don't wanna play with girls."

But I think this picture is better and I would like to title it "AW Ellie Farted!"

We have only had play dates with Christian and Max because Ellie's immune system is not as strong as a typical child's. Your intestines are one of the first defenses your body has against bugs from the outside world. If you don't have all of them then your immune system is not as strong. So, we have to be very careful of who she has contact with and let's face it, most kids are snotty messes. So we only have play dates with other children who have medical issues. Because we know that their parents will be very aware of sickness and if they say their kid is healthy, then you know they are.

We are going to miss Christian and his folks when they go home, but at the same time are very excited to see them go. It is a big step going home, Colleen, Christian's mom, has been out here taking care of Christian mostly by her self as Christian's dad works (some one has to make the money and keep the Blue Cross) back in New Jersey. It will be a nice and well deserved break for Colleen who has done an amazing job and her strength still amazes me.

Max will be here for a while because even though the Omegaven is working the doctors in Denver will not let him come home on it. Because it is so new many doctors are hesitant to let their patients use it until it is tested and approved by the FDA. This is heart breaking because of how many kids are out there with livers that are just getting worse and worse. Many doctors, even some here in Boston, are not on board but, that number is dwindling rapidly. Also some of the doctors here that are not on board with Omegaven may have made that choice due to politics, not due to the effectiveness of the drug. And that is a true shame.

Because all short gut babies should look like this.

If you are a parent of a short gut child or know some one that should be on Omegaven, please contact Dr Mark Puder at Children's Hospital in Boston. His contact information is also listed in the information at the top right side of the blog.

A new chapter, a new set of rules

Now that we know that Ellie's bowel is not dilated and food passes through easily and quickly, we need to slow things down to make sure that she gets the most out of the food as it passes through.


Maximizing the contact time between the food will hopefully decrease the amount of food that comes out of her ostomy which will also help her hydration since she won't be losing those fluids. We were encouraged that she was drinking 100ml bottles last week but now realize that they may have flushed through her like a toilet without any real benefit because the food was going through so fast.

Over the last four days we have changed our approach to feeding and now give Ellie her food over a long period of time. Instead of getting the 500 ml into her in as little pump time as possible we now have her on the pump almost all of the time. We had her up to 60ml per hour last week which had her on the pump for only a few hours each day. Now we have her at 30 ml per hour for 12-14 hours a day. It is a pain to have the pump on so much but that's a minor hassle compared to the other things we deal with.

Such is life with a short bowel.

This new approach has shown mixed results but some signs are showing that she is absorbing more and passing more down her colon as well.

We are trying to up her nighttime feedings as well:
For some reason her body shuts down at might and won't handle more than 5 or so ml per hour. If we can boost her nighttime tolerance it will add food to her system and boost her ability and adaptation. We'll see what sleeping Ellie can do. Right now we are at whopping 8 ml per hour at night. it all adds up, though.

We have also been working on the next step towards losing an ostomy as well- plugging her ostomy periodically to urge food to divert down into her colon instead of out the ostomy. This involves inserting a soft silicone plug into her ostomy for a few hours each day. We're not sure if this is doing much but her output goes down significantly when she is plugged so it must be doing something right.

What we are realizing is that we are back into the land of very slow progress again and may make improvements a single ml at a time.

Onward and upward hopefully with minimal slides downward.

A New Chapter

Get ready.. this is great, good and kinda scary new territory we are heading into.

Today we had an appointment with Ellie's surgeon, Dr Jennings. It was just a check up visit to see how she was doing and how our treatment plan was working. In order for us to have clear understanding of the state of affairs we needed to get a map of the new intestine layout with the ostomy and also get a better understanding of her current motility(ability to move food through the intestines). So early this afternoon before our appointment with Dr. Jennings we went to Radiology to get an Upper GI(UGI) study done.
A UGI study is done by placing Ellie under a fluoroscope machine and then injecting very thick white liquid barium into her stomach via her G-tube. Then we sit back and watch a live x-ray of her intestines to see #1 how fast the stomach moves the barium out into the intestines #2 how fast the intestines move the food through them (a measure of her motility) and on to the colon or out to the ostomy bag and #3 to see if her grossly dilated small bowel has gotten more or less dilated since creating the ostomy.

To give you an idea of how these things have gone in the past, we inject the barium into her stomach, and sit there looking at the x-ray of her belly at watch nothing happen. Because nothing happens we are sent away with instructions to return every half hour. Our return visit is usually better, the barium has moved into the intestines but, not very far and there is still a good portion of the barium in the stomach. This is usually when the radiologist looks at Ellie's small bowel and says, "Yup those are some grossly dilated bowels." We end up returning to radiology about 3-4 more times and the whole process would take 4-6 hours. Needless to say we never really looked forward UGIs because the news was always that things were the same or had gotten worse.

So back to today. They inject the barium and Gib and I watch.... her stomach move the barium out into the intestines. Then we watch the intestines start to move the barium through. We are shocked and very excited. The path looks good with no kinks or puddles. This is GREAT! Her stomach and intestines are acting the way they should, a huge change from previous studies. As we are cleaning Ellie up we look down and see barium coming out of her ostomy. The radiologist quickly puts Ellie back under the scope and we see that the intestines have moved the barium all the way through and you can even see some barium in her ostomy bag.
Now this may look like a large while blotch, but in reality the large white oval at the top right is her stomach, and all the hazy squiggles below and to the left are the 50 or so cm of small bowel that she has, on the lower right you can see her ostomy it looks like a light gray dough nut. The blotches of white all the way to the right is the barium in her ostomy bag.

So just as we are rapping up the study, I ask the radiologist how the bowels themselves look. She says "normal." Gib and I just kinda nod and accept that they are the same. Then it dawns on us.. Normal? I ask her to clarify does she mean normal for Ellie (ugly and dilated) or just NORMAL 11 month old bowels, short of course but other than that...NORMAL? "Oh, yeah they look like what you would expect a normal 11 month old intestines to look like." I think we asked her that same question using different wording about 10 times, we wanted to make sure we were hearing what we were hearing.

Ellie's small bowel is like it has never been before, undilated. She was born with dilated bowels that just got worse over time, and now.. normal. These bowels before her ostomy were in places over an inch in diameter. And now they are.. NORMAL. Sorry, I keep repeating the same word, normal, but it is a word that we didn't think, normal, we could hear in relation to Ellie's bowel for a very long time, if ever, normal. The fact that the diameter of her bowels is now normal would account for the increased motility.

We opted for the ostomy because her motility was so bad that food was pooling in her intestines and causing bacterial overgrowth and many other issues. That is no longer an issue, she now has very good motility. So, Ellie has done it, her bowels are working, the only problem is they are working a bit too well. The whole study took about 15 minutes. That is too fast for food to move through the intestines. Too much motility=New territory. This is the problem that most short gut children have, and now we are part of the club.

So we go to meet with Dr. Jennings and we are very excited and nervous because this is not what we thought we were going to be talking about today. He is very happy about the improvements in her bowels and quickly starts talking about what this means. It is too early to reconnect her bowels, there is to high of a risk that they will re-dilate. But what we will do is start getting her bowels ready to be reconnected and try to feed her in a way that she will be able to do the most with the food she is given.

About three times a day we will start putting a device into the ostomy to make it harder for food to go out of the ostomy. Because her colon is still attached this will force more of the food to go down her ostomy. This should slow the progress down a bit because the intestines have to push harder to get the food though the colon.

Along with this we are going to change our feeding approach. We will keep the total amount of milk that she takes in the same just spread it out more over the course of the day so the intestines have a better chance of absorbing the food. Ellie has been taking 4 ounce bottles in the morning and night, and while these are great we have now seen that they will only stay in her for about 20 minutes. So she actually got very little from them. We will still try to increase but maybe not as quickly.

This is going to be a work in progress as it always is with short gut kids. It maybe that we have to start Ellie on some anti-motility drugs if the above techniques don't work. But we will cross that bridge when we come to it. Gib and I are very excited to be heading into this new territory, and very nervous at the same time. We were really ready to hear more of the same today, and were thrown for a loop by what we did hear.

While our world was rocked today, Ellie was no worse for wear and was happy to be back home at the end of the day with her favorite toy, Gus.

Freaking out the Normals

Taking Ellie out in public with her gear can be an interesting experience. She looks very healthy and giggles and screams like an almost-11-month old baby should. Without the pump and tube this usually gets a smile from people in the grocery store or restaurant.

Happy babies do that (even with the shiner under her eye):

There is something about the white tube hanging from her crotch that makes people turn into idiots.

Today for example, we went to lunch here in Wellesley. Abby, Ellie, my sister Beth and I. As we went in to the diner, Abby went in and I got Ellie out of the stroller along with her little black backpack and the white tube attached to her.

When Abby went in ahead of us, she ran into a woman who was staring out the window at us, telling her friend,

“Look, that baby has an IV. I wonder what is wrong with that baby? Look! Look ! She’s coming in here. There must be something really wrong with that baby…..”

Needless to say the woman shut up and looked sheepish when I walked in with Gut Girl and she realized Abby was that baby's mother .

Abby and I have noticed this pattern too many times to count. At the mall at the store, in restaurants.

We have even given it a name- Freaking Out the Normals.

You can follow their eyes in a pattern- Eye contact with me, look at Ellie, see her tube, and look away as fast as possible. Run away.

I have to admit I honestly don’t care about what anyone thinks and have stopped trying to cover it up at all.

Sometimes, I even let the tube hang extra long just to mess with people.

I guess it comes down to the fact that we are proud of our girl and everything that she has been through. Right now her pump is part of who she is and if anyone can’t deal with that, well you know what they can do with it.

We have decided that we won’t flaunt her tubes at Baby’s R’ Us anymore. Expectant parents have enough to worry about without a flesh and blood illustration of what could be.

Denial isn’t just a river in Egypt.

Road Trip!

On Tuesday of this week I truly took the show on the road.


I loaded up Ellie and the two of us drove down to Mystic for the day. We left Gib at home, who used the peace and quiet to get some work done. It is about an hour and half drive both ways. And Ellie did very well.

She managed to sleep the whole way down and then fell asleep in her stroller at the aquarium and slept the whole way home. She loved the fish and spent most of her time screaming at them. She showed off her walking skills and charmed everyone in the place. I have to thank Jess for being nice enough to be our photographer for the day, since I forgot my camera.
For Ellie I'm sure it was like meeting a giant fan club, everyone recognized her and was very excited to see her. She got some great practice at being the center of the universe. It was a mile stone for me, I have been dreaming of bringing my girl back to Mystic for a long time now. She was supposed to come back there 11 month ago, but this was good enough. It was nice to see friendly faces and to see all the animals. It was odd to be there and not be involved in everything, but very freeing at the same time.

It was a big day for the both of us, it was first test of how she does for a longer car ride, and her the first trip to see where she was supposed to have grown up. We had a great time and will definitely be going back.