More calories than Coke?

In the past month or so Ellie has not gained weight as fast as we would like her to, so about two weeks ago we contacted the team and asked for one night of TPN back. That takes us back to 2 nights of D10 and 5 nights of TPN. We are very excited about reducing the number of nights of TPN but only if she continues to grow. Yes, we still weigh her everyday and so we knew that she was not gaining much. This wouldn't have been much of a problem if she had continued to grow lengthwise. But, we also measure her every week or so and so we knew that she had stopped growing.

Ellie at her first pumpkin patch

So, last week we had our normal Short Bowel Clinic day at Children's. They were impressed with her growth, saying most normal 17 month olds gain around 9 grams a day. With the bump in weight after adding the night of TPN back on her average is around 10 grams a day. They were very excited and wanted to take the night of TPN back off.

We had a long talk about what Ellie actually needs from her TPN. She isn't having an issue with hydration so she isn't completely dependent on it for fluid. But, she does stop growing when we reduce nights. This means that she is dependent on it for the calories. So, we had to look at a way to increase her calorie intake so that she doesn't depend on the TPN. So we asked to meet with the nutritionist.

Ellie driving her first tractor(she is blowing me a kiss)

First we increased the calories in her Elecare formula. Now that Ellie is a big girl, she has been bumped up to toddler Elecare, 30 calories per ounce. Still unflovered and foul smelling but a whole lot of calories for our girl to absorb as it passes through.

To give that some perspective, full strength Coca Cola has just shy of 13 calories per ounce. so Ellie is on formula that has just a bit more than twice the calories of a can of Coke. The reason is there will be a fair amount to formula that she eats that she doesn't absorb due to the missing intestine. So no worries we don't have a sugar high spaz for a baby, she seems to be handling the increase just fine.

Ellie and with what she kept calling a fish

We also looked at her solid foods. When we told the nutritionist what we fed Ellie, she said it was way too healthy. She said that we need to increase her calories that come from fat. So instead of Cheerios and pretzels we should be feeding her snack foods like veggie sticks and Ritz crackers. Basically try to keep it healthy but increase the fat. Every short gut kid reacts differently to sugar and to fat, so we just have to try new things and see what happens.

We also looked at her meds. She was on Reglan to help her stomach empty faster, Iron to help with the size of her red blood cells, and Zantac in her TPN to reduce the acid level in her gastric juices. She has had great motility for the past few months so we discontinued the Reglan. This is great because we have heard there are some nasty side effects from Reglan, we haven't seen them yet and we would like to keep it that way.

Ellie with new clothes from Gramma

In the end we decided not to reduce the number of nights of TPN just yet. We wanted to change the calories that she eats and see what happened and if all went well we would take the night of TPN back off. We didn't want to try to change too many things all at once.

It has been five days since we changed everything and she has already gained about a pound. This may be to a bit too much of a jump. We will just have to watch and see if it was a fluke or a trend. It also been five days since we fully discontinued the Reglan and increased her calories and she is doing great. Her output from her ostomy has decreased meaning that we can increase her feeds, either on her milk pump or solids.

She seems to kinda like the Veggie sticks. She hasn't really given the Ritz a chance. We also started adding a bit of olive oil to her baby food and she doesn't mind that and her poops look great! We just have to keep trying new stuff and watch her output to see if she tolerates it. She still isn't allowed to have real milk or any kind of nut, because short gut kids can develop allergies very easily.

Corals at Mystic

The last thing we had done at clinic was a TPA treatment for Ellie's central line. We have had problem drawing blood from it for a while now and just recently it was flushing more slowly. So we did a TPA treatment and it worked like a charm. We have decided that even though the line draws nicely now we will continue to go to the blood lab to do peripheral draws for blood work. The nurse brought up the idea that because only Gib and I are the only ones to ever touch her line, could be one of the big reasons that she not had a line infection in over a year. (please knock on wood now)

So, that is the update. She is doing great and charming us more and more everyday. Oh yeah and we went to Bel Air, MD to visit the Bretkos, Harpers, and the Craigs. It was a blast, and it just left us very excited about the next time. We also went back down to Mystic to visit the aquarium and Ellie had her first play group with the new early intervention. Wow, what a week. Have fun in Detroit Kelly!

Ellie and Kelly

A long overdue update

Ellie is doing amazingly well. Everyone that she meets, both professional and otherwise just can't believe that she has all of the medical issues that she has. She is doing so well, in fact that we often neglect this blog in favor of playing with her or doing other 'normal baby' stuff with her. To us, her daily exploits are quite boring but then a bit of perspective reminds us of how remarkable she really is.

She eats, she sleeps, she grows, she poops, she runs, she talks. All of that normal baby stuff that shouldn't be so remarkable is remarkable since we were told that day that she was born to expect 'significant delays' in her growth and development and reaching developmental milestones. Yeah yeah, delayed.........

A nice update to this stern warning came last week when we enrolled her with a different agency to provide Early intervention services for Ellie in the new town. The new agency folks are just as amazed as the old agency was. At 16 months Ellie is consistently ahead of all of her milestones and tested out at an amazing 26 months for social expression! I guess the Irish bullshit appeared early for her and she was on her game for her evaluation

Her doctors are very pleased with her growth and development and last week gave us the unanimous thumbs up about her progress toward the first goal of losing her TPN and then losing her other hardware. we are a bit shy about peeling away nights of TPN too soon (which will slow her growth if we go too fast) but we are moving in the right direction.

She still has a giant melon which remains a bit of a concern for the lab folks but otherwise she is great and her liver continues to do very well even after a very long time on infant TPN.

And her friends and family are pleased with her as well. This past weekend we finally made the trip to Maryland to visit a bunch of good friends of ours and introduce our kids (all 6 of them) to each other. Even though we had to leave a day early when one kid's bowels decided to erupt, we had a nice short visit and we think that we have dodged the gut bug. We opted for safe rather than sorry, which is our general approach these days since 6 hours in the car with a case of diarrhea is no fun at all.It was nice for us to be able to visit and I think that it was good for all of them to see Ellie in the flesh and see that she really is the super happy giggling little girl that we show on the internet. I'm sure it would be easy to edit out the bad days but (knock on wood) we really haven't had many of those in a long while.

The lack of updates is simply the result of very full schedule and a very happy girl.

When note worthy news happens, hopefully for the better, we will be sure to get it up here, but bear with us while the weather is good and the girl is happy. Denying the girl a tricycle ride in favor of a blog post just doesn't make sense.

Overcoming Short Bowel

In my mind there are many ways to overcome all that comes with being short gut and being the parent of a short gut child. Here are some examples.

1. The intestines can adapt and your child can gradually start tolerating food just like a normal kid. They can eat as much of whatever they want whenever they want.
   
2. Your child can get an intestine or multi organ transplant. There are many issues that come with transplants but in the end your child is no longer "short gut."
3. You can continue to live your life the way you want and not let a little thing like missing organs get in your way and teach your child to do the same. Still try and work everyday at making the intestines that are there do their job better, but do it while living as normally as you can. Hopefully this will lead to the same result as #1.
   

Max Munakata in PARIS!!!!!!

This is Max, he was on our blog last February looking very yellow and not to healthy. Thanks to some amazing parents and Omegaven he is now on VACATION in Paris. Yes, I said vacation. Okay, so his parents are there for work, but how normal to be able to bring their child with them.

Max has extreme long-segment Hirschsprung's which has left him with only 25cm of small bowel. Unless he gets a bowel transplant TPN or some version of fluids will always be a part of his life.

Now some of you out there may think that taking a child with a central line, G-tube, ostomy, and all the medical equipment all the way to Paris is insane and would be hard enough to do with normal a kid. But TPN and all that comes with it is "normal" for short gut parents and kids. So they should be able to live their lives just like all other "normal" people. It is just a different version of "normal". So bring on Paris!

I am so proud of Max's parents and so excited for them. I hope all goes well on their trip and that they come back ready to travel more. With a nice break and much sleep of course.

On the Omegaven news front, there are now 24 Hospitals in the US that are administering Omegaven. There are now 60 patients that are on Omegaven, Ellie was number 23. We also just added a link to the side bar of this blog for the "FDA IND form" that is needed to get your child or yourself approved to use Omegaven.

Gus is doing well. He is came home today with a shaved head and a draining incision on his head. He is doing well, loving the strong antibiotics and riding the pain killer wave. It is nice to have our dog back.

Lumphead

Gus is back in the (semi) capable hands of the vets at Tufts animal hospital today. As he left earlier this week they told us to watch him and watch his lopsided head to make sure that the round side didn't get any bigger or the hollow side any thinner.

By last night our beautiful dog looked like a lab from the right and a mastiff from the left and off he went this morning to be seen by the same doctors who were sure that they had caught his problem earlier this week.

It turns out that while focusing on the positive test for myositis and ignoring the information that Abby gave them about antibiotics helping in the past, the vets had missed the infection that we were concerned about on Monday.

To add insult to injury, the heavy doses of meds that we put him on (prednisone and an immuno-suppression drug) may have opened the door to help the infection along.

So he in back in Grafton today with a swollen head and we are not very happy with his vets.

He will have some tests and probably have surgery to get at the source of the problem. We hope that they will treat the source of the problem and not the symptoms.

I think that having such great rational and logical doctors at Children's for all these months has spoiled us but we have really been baffled by some of the treatment that he has gotten this week. Abby is very good on this stuff but the number of times that she has told the vets how to treat Gus really amazes me.

All I want is my dog back, fetching a ball and letting Ellie climb all over him.

Gus

Well the saga continues. To give you an idea of what we have been dealing with, I need to give you the history of the past month. About a month ago we noticed Gus was acting funny. He was sleeping all the time and didn't want to play fetch. Now he is a lab, so he should want to play fetch even when he is dead. So, off the vet we went. The vet decided that he had an infection in his jaw caused by something that he was chewing on (grass or a stick). The infection probably moved under the muscle up to around his eye and to the hinge of his jaw (that is where it is in a dog). So he was put on antibiotics to treat the abscess.After 10 days of antibiotics he was great, playing fetch with Ellie and playing with his soccer ball just like he used to. Then we get a call from the vet. It ends up that Gus has tested positive for an autoimmune disorder called masticatory myositis. Basically his body is attacking the muscles of his face and jaw because they have the same protein signature as a bacteria. The treatment is a long term, decreasing dose of steriods. So we started him on steroids. And the side effects of steroids, among other things, is increased hunger. Bring on the corn cobs!

Gus post surgery with his C collar

So, then we have the exploratory abdominal surgery to try to remove the corn cobs. Gus spent three days and nights at the hospital and got a big shaved belly with 12 stitches. We had to stop the steriods because they are known to inhibit healing. Then last Wednesday we noticed that he was sleeping all the time again and couldn't even eat Ellie's carrots that she was feeds him on the sly. We called the vet clinic and got the ok to start the steroids again.

Gus's first day with us.

Ok, so this brings us to Monday. He was in bad shape, would not leave my side and could barely eat his food. A lab that can't eat is a sad dog. Back to the vet. They start thinking that it is the abscess again and want to do another exploratory surgery of his face that afternoon to find the abscess. I decided that Gus has been explored enough, so we packed up and headed to TUFTS veterinary hosptial.He was admitted as an emergency case and is kept over night. He was seen by two regular vets, three students, and two neurologists. I was able to pick him up last night. They think that it is still just the autoimmune disorder. They have him on a much larger dose of steroids, (hide under lock and key all food for the next month or so) and an immune suppressant. (nice word for chemo)The problem is that they just think it might be the immune disorder. We are now playing my least favorite game called, wait and see. If it is the autoimmune disorder then he will slowly get better. If it is the abscess then it will get really bad very quickly because his immune system will not be able to fight it.So for now, we wait and watch Ellie play with her best friend. When we got home last night Ellie spent the rest of the evening chasing Gus around yelling, "Ga, Ga, Ga!" and then tackling/hugging him every time he stopped moving. We miss him when he isn't here and get nervous as to what he has in store in the future. We are actually hoping for the autoimmune disorder, which would mean no more surgery. Luckily Ellie has been doing great through all of this.

Via the Sea

We finally got Ellie on the ocean this weekend. We took a break from our work in progress of a house and went to to Maine to see my family for a few days. Traveling is very easy for us now and we have worked out most of the kinks in the logistics which makes it a lot more fun for all of us.

We have been trying to get Ellie out for a boat ride since this spring but with naps and ostomy changes we never had things line up to get her on the water.

This weekend's major goal was to get floating and Saturday morning we cut ourselves loose for a short ride around the harbor where I spent my summers a kid. Fortunately my brother has lots of life jackets for his kids and had one that was small enough for Ellie.

Mom, what is this thing and why can't I clap my hands?

She took a little while to get used to the new surroundings:

You want me to get on that?

But after a few minutes and with the company of her best friend Gus she was ready to float:

Oh, this isn't so bad.

Our whole tour lasted 20 minutes but by the end she had been all over the boat and even stood with me to drive a bit:

A successful mission to circumnavigate Pound of Tea Island (over my left shoulder in the picture above) and we were back on the dock.

She insisted on walking us up the runway. She already walks up and down the docks better than her grandfather.

Day at the farm

We had a good weekend last week, we worked on the house, planted our gardens and went back to the farm with the petting zoo.
Ellie had a great time. She seemed to really understand that those things in the pens were actually animals. She really like the chickens and had a good time petting the donkey. But, try as we might we couldn't get her to stop calling the pigs "Gus." They were some pretty big pink and black pigs, I don't know how they reminded her of Gus, other than that they too might eat anything and everything.I like this picture, because I think it is actually talking about her hair and not the goats that live in the pen.Gus is healing well from his surgery, and can't wait till next Monday when he gets his stitches out so he can swim again. All in all the house is starting to look like a home, especially now that we have a kitchen sink and counter tops. Ellie is doing very well. She is handling the three nights off of TPN well. She seems to have stalled again with weight gain, but she is still growing. We had to go buy new shoes, real shoes not crocs, yesterday. We are trying new foods, adding a fourth meal in and trying to keep her eating as much a possible. Mainly she eats baby food mixed with rice cereal but we are trying some bits of our food when ever we can.
Her favorites are avocado, banana, pickles, pretzels, and carrots. She likes almost any baby food we give her, we just have to be careful and make sure it is something that a short gut kid can tolerate. We mix almost everything with rice cereal so that it will stay in her colon for a longer period of time, so she can get more liquid from the food. We try to have bananas or apples in her diet at least once a day also. Peaches and peas are the only thing that we have seen a negative reaction too. Negative as in too much ostomy output. So we just keep offering her foods and hope for the best.
Ellie has also started to really pick up on the sign language. Not as much as other kids that we know that are signing super stars, but Ellie can get her point across. Last week we were reading a book Christian gave her and she surprised me by giving the sign for fish. Growing up in this house we knew that we were going to see the word Fish sooner or later from her.