The Masters

The Masters Golf tournament is this weekend. That means that a year ago, by the sports calendar, our adventure began.

This is where we started. This is day zero before her first-ever surgery, notice no g-tube....

This is where we ended up:

One year ago, before the golf began, I got ready to cook pancakes and Jimmy Dean sausages. It is funny how the details stick around but I guess that the Breakfast of Champions and Pregnant Ladies has a way of staying in your mind.

I had just started cooking while Abby called the Obstetrician to see whether her symptoms-which included the words fluid and leaking and other words that make guys cringe- were a sign of labor.

Abby came in a few minutes later and told me to drop what I was doing and get ready to go to the hospital. The breakfast, incidentally, finally got cooked a few weeks later at the Ronald McDonald House in New Haven.

I ended up watching the Saturday third round from the hospital chair/bed in Rhode Island while Abby was in the early parts of labor. It was pleasant way to spend an afternoon even if it was a little boring for me since a slow labor is very slow.

Little did I know how many days and nights I would spend in a chair exactly like that one in the coming year.

Abby has much different memories of this part of the adventure and tells me that hearing the announcers on TV makes her want to barf. Since I have never felt contractions or had my cervix examined, I can only imagine what she was feeling as I watched the calm green golf on TV.

A long 24 hours later, I watched the Sunday fourth round in the hellish waiting room of the Yale NICU surrounded by dozens of people (link) while I waited for the surgeon to give us news and for Abby’s ambulance to get her to New Haven.

One very long year after that and I am home watching Tiger Woods do his thing from the friendly ass divot in my favorite rotten chair.

It is odd but it is the golf that really started me thinking about the entire last year. I knew that it would be coming but the golf was the trigger. With a milestone like a first birthday coming up I guess we will be thinking about the last year a lot in the coming days and remembering some of the really dark days that we had in the beginning.

Fortunately we have days like today to contrast against the crappy days.

One trip to Stride Rite today to buy real babygirl shoes:

does a lot to overcome the uncontrollable tears as my Mom forced me to eat breakfast in the lobby at Yale on day one.

Likewise, a weekend like we finally had last weekend makes up for the long string of days that I couldn’t physically talk to any of my siblings to explain what was going on and a period where my sister Kate and I avoided each other entirely for about a week because we both knew that we would be blubbering messes. Sitting on Kate's floor with Ellie a week ago made up for lots....

And even though reading a draft of this post made Abby and I cry, writing blog posts like this one makes up for the first one (link) that I couldn’t read to Abby aloud without sobbing in the hospital room at Yale.

As we learn of other short bowel families using this blog as a source to know a little bit about what to expect, I hope that it gives them a glimpse of the fantastic parts that go along with the crappy parts.

If we had known then that Ellie would be upstairs babbling in her sleep right now and the great progress that our little girl has made, those dark times wouldn’t have been nearly as tough.

Clinic Visit

Yesterday was Ellie's monthly clinic visit to see the Short Bowel Team at Children's.

We followed the advice of another trusted short bowel mom and scheduled the last appointment of the day, 4 PM hoping to get here late when people are done with their distractions and can deal with us. We're not sure of this approach but it was just as good as the other times and we didn't spend an entire day at the Hospital as we have for earlier clinic appointments.

They measured her height, weight and head circumference-9.45 kg, 21.70 pounds, 29 inches long, and a giant melon for a head that is filled with Omega-3 brains. In fact, her head is so big (how big is it?) Her head is so big that she needs to wear a size 2T hat to fit into it now. Abby and I both have big heads so she fits right in.

Then we spoke to the doctors. The surgeon, the GI doctor, and the TPN guru. They are very happy with her progress. So happy that they have peeled away another night on TPN from her week. We will now have her on just IV fluids two nights a week. We will need to separate them in the beginning to make sure we don't get a dip in her blood work but two nights of fluids is a lot easier than PN. They even told us that she had a reserve of nutrition. Imagine that, our girl has money in the bank. Or more appropriately fat in her thighs, but I will take it.

Since Ellie is treated by both the Short Bowel Program and her surgeon, it is funny to see the reactions of the same doctors that refused to do the ostomy procedure when they see Ellie's success with an ostomy. There is no skepticism or stern warnings now, just lots of success to go around as her bowel has come down in size, started to move food though, and she is up to round the clock feedings. Sometimes I want to ask them about their feelings on our choice of treatment but for now we will all feel good watching our very pale good sized baby who giggles and plays peek-a-boo with her mom in the hallway.

All signs are good. If we could just get an angry tooth to finally poke through, we would be in great shape. That will happen any day now.

A Resounding Success

This weekend’s trip was a success on all fronts. We took our PN show on the road, away from the comfort and familiarity of home and the security of the Route 9 to Children’s Parkway and came back without a hitch.

Making our trip even easier was the message from Ellie’s surgeon that we got when we asked him about going away for the weekend:

“Go to Maine – it’s not that far!

I have my cell phone on.

Any crisis and Maine Med is fine – I know the folks there.”

If that doesn’t make you feel bold, nothing will.

Apart from Ellie being exhausted and going to bed tonight at 6:45 she seems to be doing just fine. We will watch her very carefully for the next two or three days to make sure that her cousins didn’t give her a souvenir infection but it seems just fine for now. Sometimes it can take 72 hours for a bug to bloom in her. We felt her forehead a lot today. Lots of false alarms so far.

Abby and I even left Ellie with my family on Sunday and went to lunch for the very first time. We hadn't been away from her since December when the nurse came early one night and we went Christmas shopping.

We are already thinking about the next opportunity to head north how we can make it even easier the next time around and what we need to do to stay for more than one night.

The biggest trick is getting everyone up to speed on the little things that we do all day long as part of Ellie’s preventive care. Particularly interesting is how to teach my brother’s sons who are very precocious 3 and 4 year olds about infections and the need to wash their hands. On one hand, you need to tell them that they need to wash and why but on the other hand, you can’t tell them the full story for fear of scaring them and making Ellie and her tubes seem scary. I think that we reached a happy medium by making hand washing into a game for them.

If only we could Purell the little buggers from head to toe….

The other trick is adapting the routines that we have to a new arena. Simple things like changing an ostomy bag or even a diaper are just a bit more difficult without home court advantage. We are learning the new ways of doing things and fortunately, my sister has a beautiful brand new house that has lot of clean spaces for us to use.

Summer is coming and we have a whole lot of recreating to do to make up for last summer. We have a key to my sister’s house now to go along with the key to my brother’s boat.

It will be tough to stay away.

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.

Taking our Show on the Road

We are getting comfortable at home and have taken the little steps towards being a mobile family- going to the grocery store, going to lunch(buffets are best) and so on but always returned home around 4 PM to get ready for the veening routine. Tonight we started to move toward the final frontier-sleeping away from home.

For a low-risk dress rehearsal we packed up and came the full 6 miles from the safe zone at home to spend the night at Abby’s sister’s house Wayland for the night.

We are a long way from traveling light.

It took six bags to get all of Ellie’s essentials loaded in to the car. Between what we knew she would need (food, TPN, pumps, an IV pole, etc) and what she could possibly need (a new ostomy bag, a new central line dressing, etc) it is a lot of stuff. Add in the normal baby stuff like toys clothes and books and a bed and it is quite a load. Add Gus on top of that and we almost forgot our own clothes and toothbrushes.

I am beginning to see the light about minivans even for us with only a baby and a dog.

Going to Wayland was nice for a test run because it is not so far that we are in a world of trouble if we need something important from home.

Which I did not once but twice this evening.

The first was to pick up cotton balls to fill her ostomy. A simple brain fart on my part to forget a staple of our day. Nothing really urgent and we could have solved it with a trip to CVS.

The second was a bit more important since I screwed up and punctured the only IV bag that we had for the night when I was driving the spike into the bottom on the bag. I simply rammed it through the side of the bag.

Not a big deal when the replacement was 6 miles away, but it was an eye opener for what could have been if we had been three hours away.

Only two return visits to get more supplies and a third scheduled for the morning since Abby was bit spastic in the new environs and blew through our supply of saline syringes while hooking Ellie up tonight.

On the bright side, Ellie is a tough bird who played hard all day:

http://www.youtube.com/watch?v=mruXUHpd-IA

Then she fell asleep on the bed while we set up her PN tonight.

We’ll see what she thinks about sleeping in the Pack n’ Play tonight.

If all goes well we will probably do this again to fine tune the routine. Then it is off to the races......

Nap Time

File this under normal baby stuff that still freaks us out.

Ellie melted last night at 5:00.

Abby was cooking dinner, I was finishing up work for the day and Ellie was in her Exersaucer, playing with toys and watching Abby cook.

I went into the other room and came back to find Ellie in this position:


She had simply run out of gas and fallen asleep in a comfortable position. This wasn't a doze, she was out. So far out that I could pick her up and lay her down in the Pack n' Play in the living room for a 30 minute power nap (but not before taking some pictures, of course):


It is still the 'normal baby' stuff that causes us the most trouble.

Was this normal?
Was her blood sugar low or high?
Had we fed her too much after increasing her rate to 55 ml/hour?

Nope.

Just a sleepy baby.

She rallied at 5:30 and was out for the night at 7:30.

We are well over 500 ml of formula a day these days. Gotta love that pump.

In the groove

We have been home for just over two months now and have gotten our feet under us to the point that both of us are scared that we are getting too comfortable and will slip up because the procedures have become second nature.

We attach and detach her pumps so often that we sometimes forget the gravity of what we are doing and what a tiny slip up could mean for Ellie's health. If you do something twice a day, every day, you are bound to have good days and bad days. We individually were afraid that the bad day may mean a serious slip and when I brought it up this morning, Abby told me that she had the same fear.

This leads us to have moments of anxiety at strange times. For example last might at 2 AM I was absolutley sure that we had forgotten to hook Ellie up to one of her pumps. I had the monitor right next to my head and could hear the pumps going but for some reason knew that I had forgotten something and that the pump was sitting next to us on the floor. I woke Abby up, she knew that I was dreaming and ignored me. I woke her up again and she ignored me. Finally at 3AM she woke up enough to tell me to stop worrying and everything was the way it should be.

It was, I was just being a bit whacko.

It is good to have a co-pilot.

Ellie was up at 5:30 (after sleeping 10 hours straight) this morning and quietly standing in her crib and exploring when Abby went in to check on her.

Standing in her crib. Not crying or looking for us, just having a look around at the world.

We got her up before she could hatch an escape plan and spent the morning in bed:
We hung out until her pumps beeped at 7:30 and we started the rest of our day. She loves watching the Today show with us, and even likes an early morning go-round of SportsCenter...

Did I ever tell you how lucky we are?

Every time we meet or hear about another kid that has short gut or another condition/disease/disorder that requires them to be on TPN, all that Abby and I can say is that we are unbelievably lucky to have stumbled into the great things that are going on at Children’s.

Plain old shit luck.

To think that we were torn for a while at Yale about whether or not to come to Boston.

How different would Ellie’s condition be right now?

Would she have liver disease? Would she be very sick? Would we be wearing the transplant beeper waiting for multiple organs?

The answer to all of those is disturbingly probably, yes.

Instead of the very pale, very happy little girl who eats Cheerios as fast as she can find them: we would be looking at serious liver disease.

Sometimes we forget that there are kids all over the place that are on the ‘old’ Lipid and that there are families that are living through the really bad, scary parts that we were afraid of when we heard about the reality of Ellie’s condition in April.

Liver disease has all sorts of nasty symptoms. All things considered, we got off pretty easy with a bit of jaundice and some yellow eyes.

Shit luck.

We didn’t have inside information or a relative that knew gastroenterology. We simply came to Boston and had this fall into our lap.

But the encouraging thing is that the word about Omegaven is spreading.

I learned last week when I went to the hospital to get meds it is spreading so well that the folks at Children’s can barely keep up with the parade of sick kids that are making the pilgrimage to Longwood Avenue to get on Omegaven.

We know of another family that is at Children’s today from Indiana and hope that their visit goes well.

In the meantime we will keep spreading the gospel of Children’s and Omegaven along with our understanding that this may not be the answer but it is a lot better than any other option.

There aren’t any secrets and we all want our babies to be healthy.

So I will keep ranting about our great luck and great care in Boston until someone tells us to stop.

Knowing the work habits of the folks at Children’s, it probably won’t be them that yell uncle.

More mobile everyday. IT WORKS NOW.

Big fun on a Sunday morning....

I've had bad luck with You Tube so here it is on Google Video.

Good old Google...

Make sure to turn up the volume. My film making skills are blossoming...

http://video.google.com/videoplay?docid=-6227528559150447512

Winter

We finally got a real snow storm this week and this weekend it warmed up enough to take Ellie for a ride in her sled. We bundled her up very well and packed her pump under the covers and off we went:



















Ellie thought it was great fun to ride along in the sled and even thought about falling asleep.

All told we were outside for about half an hour of fresh air which was good for all of us and gave us another glimpse of what things are like for parents who don't know TPN from TP or the difference between a G-tube and a GJ tube.

Ellie's Boyfriends

Ellie got a chance to go visit her boyfriend Christian, and pick up a new one, when we had our first ever play date yesterday. We went into Jamaica Plain and visited Christian and another little boy named Max. Christian is doing great post transplant and Max is here from Denver to get on Omegaven.

It was great to see both Christian and Colleen and Christian looks like a new little boy. First thing he did was push all the toys away from Ellie and then started hitting him self in the head with a couple of other toys. Such a boy!



Ellie got even though she later tried to use Christian as a stepping stool. He didn't really like that. He didn't like that, but they did have a good time exploring the great range of toys that Christian has.

Ellie does have one major problem though. She scares all the boys away.







When she gets really excited she lets out an incredibly loud squeal. It was this squeal that made all the boys cry. I don't think Christian or Max minded having Ellie come to visit, they just wished she would have been a bit more quiet.

Max is 8 months old and has been on Omegaven for 3 weeks now. It usually takes around 30 days for Omegaven to do it's thing, so they have a bit longer to wait. He is very cute hopefully we will get to see more of them before they go home.

I hope the boys had as much fun as Ellie did, she fell asleep almost as soon as she got in the car and took a nice long nap when we got home. It was great to see Ellie interact with other kids, we may need to work on her manners for next time. She just has to remember that you play with the boys not climb on them. Maybe next time Christian can teach Ellie how to say "ball" or how to do his great monkey impression.

All Reglan is not created equal

Since Ellie left the hospital she has been on a whole string of medications to help her gut do more of what it is supposed to do.

These are not your average drugs and can’t come from the CVS or Walgreens down the street. Instead, the have to be ‘compounded’ by a special kind of compounding pharmacy. This is usually the older independent pharmacies that you may not even notice in your town.

A couple around here still call themselves apothecaries, a term that I thought went away with Romeo and Juliet, phrenology, and leeches.

Apparently I was wrong.

When we left Children’s in December we got Ellie prescriptions filled at the apothecary that the hospital knew that is close by the hospital. It is a tiny place in the basement of a building with beakers and vats of stuff cooking. It is a bit of a pain to get to, but it had the drugs that Ellie needed and made them quickly for us so we got her meds there to start. It also has all sorts of people getting their custom meds which is a good sign.

With two return visits to Children’s, that original 30 day supply managed to last us almost 2 months.

This past weekend the Reglan ran low and to save a trip to Brookline for a refill we called the small independent pharmacy here in Wellesley to ask about getting it refilled.

Sure , they had it and could fill it for us. Great. We don’t have to go to Brookline to get her meds, just to the pharmacy next to the grocery store.

Some meds, some dinner and I’ll be home.

A great plan which worked just fine until Abby tried to give Ellie her evening Reglan-Ellie wanted no parts of it and spit half of it out before barfing the rest onto her bib.

Something wasn’t right with the smell of the liquid and the taste was off as well. It tasted like Chocolate/Vanilla instead of baby fruit. We stopped right there and waited for morning.

Reglan is a nice drug but we stop and start it when things seem odd.

We called the pharmacist at Children’s the next morning and found out that all Reglan isn’t the same. Most likely what she got was the adult version that had the same dosage but also had a lot of alcohol and sugar in it- two things that are no-nos for short gut kids and can cause all sorts of other issues.

Strike One.

On to pharmacy number two, in Needham which mixed up a batch of ‘alcohol free’ Reglan with ‘just a tiny bit of sugar in it for the watermelon flavor’.

Ellie took it.

But then the fireworks started.

Instead of pooping 2-3 times a day, Ellie has pooped six times for the past two days and was just grumpy in the night.

Strike Two. Pooping six times in a day brings a certain Johnny Cash song to mind…..

Poor little girl.

We’re not sure if the new version of Reglan is the culprit but we stopped it yesterday and her poops are back in order.

Her poor sore bum seems to better, too. We have really good hospital diaper rash cream, powder and ointment which takes care of the rash very quickly. How we will ever go back to plain ol' desitin is a mystery.

Abby is off to Boston today to get the good stuff directly from the pharmacist Children’s to tide us over until the next prescription is due.

We’ll be driving to Brookline for meds from now on.

Love is a burning thing
and it makes a fiery ring….

One small step for Ellie, One great big pain in the ass for us

http://www.youtube.com/watch?v=XlWx3QeCW_Q

After a Valentine's Day wardrobe change because of a leaky G-tube (who else would have more than one outfit for today?), Ellie decided to stop messing around and start standing up for us and took a few steps today.

These are steps two and three.

It took us a second to find the camera.

Note the multitasking as she blows raspberries as she stands and steps.

We are in for a big change and a lot of Ellie chasing....

Happy Valentine's Day

Just a quick note to wish all our friends and family a happy Valentine's Day.






Ellie is having a great day watching the snow/rain fall. We have had such a mild winter that she has never really had a chance to see that much snow. We are hoping that the snow will stick around long enough for her to try out her new sled.
She is spending the day seeing how far she can get on the first floor and trying this new thing called standing up and letting go. Her current record is about 30 seconds. She gets really excited and we get a little nervous. But she is definitely having a good time.
Thank you to all our friends, family and complete stangers out there who have been following Ellie's story over the past year. We have felt very loved and supported. I know Ellie would like to give you all a big wet open mouthed kiss. Thank you and happy Valentine's Day! XOXO

One night down, Six to go

We made it through our first night without PN without any noticeable side effects.

It was a little bit nerve-wracking for us since it required new plumbing of her IV lines to run just one fluid in but we figured it out and made it through just fine.

We also managed to deal with our pessimism that is attached to new things with her care. We were both sure that Ellie's night off of PN would be accompanied by a trip to the ER for some unknown reason. It is just the way things seem to work with Gut Girl sometimes

Fortunately, we had a smooth night without a hiccup.

A night off of PN wasn't all that much different than a night on PN. Instead of PN with its amino acids and vitamins, Ellie was hooked up to a steady stream of 'D5', a solution of 5% dextrose and saline at her normal PN time. This kept her hydrated and kept her blood sugar normal.

Regardless of the small difference, it is a big step and represents a step towards hopefully losing the PN all together at some point.

It sure would be nice to lose the central line and not have a huge infection liability sitting in the line of fire just under her nose:

If you look closely you can see bananas, sweet potatoes, cheerios and rice cereal...

That will come in time as we get her to eat more and more.

She hits 400 ml most days. 500 shouldn't be that hard by next Thursday.

Ads

You may have noticed the square ad to the right and the blinking ad at the bottom.

These aren't accidental or part of a big plot to show lots of ads all over the internet. As a blog, you can make $$ selling ad space but that's not what this blog is for.

Instead, that are links to an organization that supports organ donation, something that has become near to us and some of the families that we know and we wanted to spread the word about how important this is.

Clinic Visit

Ellie had her periodic clinic visit today at Children’s. Abby took her while I went to New Hampshire for the third day of interminable fisheries management meetings.

In addition to charming everyone in the place with her smile, laugh and walking tricks as they did laps around the clinic, Ellie also had a bang up day with her tests which is great.

It was a meeting of the Omegaven families club today with old and new families there to see the docs. Abby met lots of families that were only names on the internet or email and even met a family that learned about Omegaven through this blog that we didn’t even know about. The community of families that has assembled around Boston is really something and growing by the day.

Please come to Boston for the Fish Oil....

Her weight is up. Up 320 grams from the last visit which is more than the 280 that the nutritionists and GI docs had hoped for. She still is in the 50^th and 75^th percentiles for weight and height respectively but ahead of where they want her to be. She now weighs 8.75 kilograms or 19.25 pounds. Quite a load that is giving Abby quite a set of arms.

As promised, we have reduced her TPN again. This time, in addition to reducing the overall volume, we are getting one whole night a week without TPN. She will still get fluids by IV on those nights since her colon isn’t fully attached to absorb fluids but no TPN.

The weaning continues and will continue as long as we can keep increasing her feeds. The new goal is to be at 500 ml per day when we go back in a month. Since we have already gone past 500, this shouldn’t be a problem. Shouldn’t but you never know.

We are still hesitant to increase too fast and Ellie will have an upper GI dye study done to check on the progress of her bowel and it hopefully shrinks.

We got word from the docs that we are doing well with foods and got the okay to add new foods to her diet. As long as it doesn’t have lactose in it, we are good to experiment which should be fun and let Ellie exercise the tooth that is trying to come in her top front.

She is happy and growing and absorbing lots of the food that passes through her ostomy.

Lots of folks are very happy with the way things are going.

Mobile. With an anchor

Ellie is getting more mobile by the day. She migrates across the room from furniture to furniture and will leave the room every now and then as she walks along the wall. ehr coolest trick is that she will now stand for 5 or so seconds without holding onto anything, long enough to look at us as if to say, 'isn't this the coolest thing?'.

We even lost her this evening when she migrated to the far side of the table with both of us in the kitchen. Her hiccup gave her away.

One of the sad and fortunate things of Gut Girl learning to walk is that her milk pump sits on the floor. She gets up and moves but unless we move her pump backpack, the tube to her G-tube gets taught and she will stop like a boat on an anchor. The tube is attached to her clothes by a safety pin so it doesn’t pull on her belly.

As she gets better on her feet we have been trying the backpack on for size and to see how well she does. We are concerned that it might not be a good thing to put a backpack on an 18+ pound infant and have only done it for limited times until the PT folks tells whether it is okay or not.

She loves the freedom of movement and loves to explore with her new wall walking trick.

Who would have thunk in April that she would do this in February? Every day's fun is extra cool when you think about how far we have come.

Gut Drugs.

Ellie is on a string of drugs every day to help her deal with her limited plumbing. Off and on, she has been on these and some others forever.

Reglan (Metoclopramide if you are our generic friendly insurance company) is a drug that helps Ellie move things down the pipe and through. Her motility has been pretty bad in the past and Reglan may help to boost this. This is one of the standard list of short gut drugs that seemingly every short gut kid is on. Some parents question whether this is necessary but it seems to do some good for Ellie. She gets this four times a day, either by mouth during the day (‘p.o.’ as the docs say) or through her G-tube when she is sleeping.

Actigal (Ursodiol) This is bile salt that is found in bears. No kidding. But, instead of finding Yogi and milking his gall bladder, they synthesize it.Bile salts help you digest fats in your intestine. Since Ellie doesn’t have full function in her intestines we give her this to give her digestion a boost. It also has good effects for her liver. Another of the cocktail of short gut drugs that every kid gets. She gets this twice a day either by mouth or by G-tube.

Iron- For some reason Ellie is anemic. To boost her iron levels she gets an iron supplement twice a day. We have just started this and will keep her on it until the measure of her iron ‘hematocrit’ comes back up to normal levels. She gets this twice a day p.o or in the g tube.

All of that brings the grand total to 8 times a day that we have to draw up the drugs in the tiny little syringe and pump in into her. 8 times that we have to remember to do this. Checklists and alarm clocks help, but we miss them every now and then. We are getting better. Hopefully they will help help get better at digesting and absorbing.

Playtime

Playing is a lot more fun at home than in the hospital.

Ellie gets to play with Mom:

Whoa! Everything is upside down!!!

And play with Gus:

This place smells like dog farts!

Up up and away

We are settling into a routine again after our time in the hospital and have adjusted to adding Ellie's wound care to our list of tasks each day. It is remarkably calm around here while being very busy at the same time.


The wound where her suture line opened a few weeks ago is healing nicely and getting narrower and shallower each day. Hopefully it will heal completely by next week's clinic visit which will take that off of her list of care items.

Using a combination of bottles and the pump we have exceeded 300 ml every day this week and on some days even gone over 400 ml. During the day we can feed her at 50ml/hr which adds up quickly and we have to stop her feeds early on most days when she reaches the 400 ml mark. We don't want to overfill the small bowel that we are trying to shrink and until we get an upper GI study done on Thursday to get a status of her bowel, we are going to stop at 400 each day.

Ellie is loving food by mouth and will chew through a dozen cheerios in a short time. Bananas and apple are also good. Rice cereal needs some banana in it to get her to eat it. It is encouraging to have her eat.

She is very close to walking for us and has started to let go of the table for longer periods. She will also migrate around the room. Floor to table to couch to table to wall to dog bed. Last night she took herself for a walk and went into the next room while Abby and I watched.

Last night Abby walked in for a middle of the night check and found Ellie sitting up in bed looking at her. apparently they were each surpised by this one-Abby because Ellie only rarely sits up by herself, and Ellie because she wasn't quite sure of what she had done. We are very glad that we have the covers on her IV lines as she gets more mobile I ahve nightmars of the lines getting tangled around her neck.

We got good news yesterday that a little boy that we know from Children's who had a multivisceral transplant a little while ago had his central line and G-J tube removed this week, leaving him with just a g-tube. Great news.