Hole in the Wall Gang Camp, Success!!

Ellie loved it!  We went to go pick her up and she was so happy we wished we could have left her there for another week.  There were 7 girls in her cabin ranging in age from 7-9 and a wide range of diagnoses.  She loved all the girls and can't wait to see them next year. From what she told us, it sounded like there was a party almost every day with different themes.  This picture is from the Carnivarty.  (Carnival+Party)

She swam in the pool 2 days, learned archery, caught her first bass, built the Santa Maria out of wood, took cooking classes, completed a ton of craft projects, performed Cotton Eyed Joe dance on stage with her cabin and did a solo demonstration of animal noises for the whole camp.

On the last night she was awarded a cooking award for a banana bread she made while she was there. Then her cabin later that night gave her an award call, Princess Energizer Bunny, for her endless energy.  Which if you have met Ellie, you know this was the perfect award for her.
These are photos of her with some of the counselors.

The medical team was amazing.  They set up her TPN and Omegaven at night, hooked her up, took care of her Omegaven throughout the night, got her line ready for swimming (twice) and had no leaks.  They kept her up to date on all her medications and oral fluid intake and never need PRN hydration.   She had a primary day nurse named Rose that she really bonded with and a night nurse, Kara that she loved as well. 

 

Ellie and Rose

And with the help of the Danielle the Dietary Intern, she tried some new foods and found she loved them.

She had a bed in the Yellow cabin but spent her nights in the Moose lodge, the  lodge that is attached to the clinic.  This is because her Omegaven bottles need to be changed at night.  But she befriended the other girl staying there and they had sleepovers in each other's rooms a few of the nights.  

Ellie's bed in the Moose Lodge.

Over all it was a huge hit and she wants to go back for as many summers as possible.

Will had a great time while she was away.  We tried to keep him as busy as possible and made sure he had a great week too.

It was very odd not having Ellie around.  The first few days we walked around positive that we were forgetting something big.  Nighttime was the hardest time, we couldn't shake the feeling that we should be doing something.   We made it just over 48 hours before we called to the camp to check in on how she was doing. Next year will be easier.

We are incredibly grateful to everyone at Hole in the Wall Gang camp that make Ellie's week so much fun.  Now we leave you with the Hole in the Wall Gang Camp song Ellie taught us.
This camp was good to me! 
This camp was good to me!
Thank you Paul for this Hole in the Wall!
This camp was good to me!

OMEGAVEN!! Welcome

With the article on the front page of the Globe last Friday we have a bunch of new readers. We went from having around 150-200 visits a day to over 900. So we decided to do an quick post to catch everyone up to date. I have linked key words in this post so if you don't know what something is, just click on it.

Ellie before the Omegaven

Ellie was born in with Jejunal Atresia, this left her with 30cm of grossly dilated small bowel coming down from her stomach that ended in a dead end, and then 70% of her colon, that was very small from disuse, coming up from below also ending in a dead end. What happened is that at about 10 weeks into gestation the bowel either got twisted or there was a blood clot, and the bowel lost blood supply and died. It was then reabsorbed into the body. We have been told by the geneticist at CHB that this was a just bad luck and not associated with any genetic disorder.

Ellie post Omegaven

She is was at Children's Hospital Boston for 8 months and now is doing very well. We have been back multiple times since then but not enough to get the name "frequent fliers." She was on Total Parental Nutrition (TPN) and Omegaven for IV nutrition through a central IV line in her chest for the first 32 months of her life. But as of December 1st, 2008 we were able to stop both with the hope that she would be able to sustain her needs from the small amount of bowl that she does have.
She eats 3 meals a day, and the only thing she drinks in Elecare formula. She also is on tube feeds 16 hours a day. This is what the is in the backpack that she wears. The backpack holds a pump and a bag of formula that is pumped into her belly 16 hours a day. Your intestine adapts as it has contact with food, so if it always has contact with food, then it is always adapting. And adaptation is the only way her bowel is going to be able to do the job that a normal child's would.
Ellie had very dilated small bowel that resulted in inability to tolerate food, bacterial overgrowth and a few line infections. So in November 2006 we opted to create an ostomy at the end of her small bowel to allow the back up of food to have a place to go, while keeping the colon attached. This has worked very well and we have seen little to no bacterial overgrowth since. And (knock on wood) no line infections. Click here to see a map of her bowel layout.
We are hoping that sometime this year we will be able to reconnect her bowels and remove the ostomy. Then she will have a central IV line for a short time and a G-tube for as long as she needs it.

If you are looking for information about short bowel syndrome or short gut, a very good site is the Short Gut Wiki, it was created by short gut parents and patients for anyone with short gut.

If you are looking for more info on Omegaven please look at the Short Bowel Wiki for more information. There are some great stories of some amazing kids on that site. Also you can contact Children's Hospital Boston to learn more about their Omegaven program. The lead surgeon for the program is Dr. Mark Puder he works along with pharmacist Kathy Gura to run the program.
We have met many amazing families through this blog. Please look at the column to the right to visit their blogs, get more info on short gut and our doctors. We also love to meet new short gut families, please feel free to email us (look in column to the right).


We can not say enough about Omegaven, or Dr. Puder, pharmacist Kathy Gura and Ellie's surgeon Dr. Russell Jennings. We give credit to all four for saving our daughter's life. She is an amazing child and we can't wait to see what she will do next.

Clinic Update

Ellie had her first post-op visit to the short bowel clinic (or CAIR-Center for Advanced Intestinal Rehabilitation) yesterday. All went well and eveyone continues to be very happy with her progress.

Ellie continues to grow very well even though she isn't eating or drinking nearly as much as she did before her surgery. Officially she weighed in at her pre-surgery weight yesterday, 12.0 kg (26.4 pounds for you, Gramma). She is 34 1/2 inches long which puts her in the 50th percentile for weight and 80th percentile for height. We have seen a hiccup in her growth after prior surgeries so seeing this fast recovery is a good thing.

Ellie is pooping into her diaper 3-5 times a day now and her ostomy output has slowed significantly since we got aggressive in plugging her ostomy early this week. Even though the CAIR doctors didn't do the surgery and were strongly against it when we opted to change to Dr. Jennings, they are all very happy with the results of her ostomy overall and also the results of her recent revision to her ostomy.

We are going to keep increasing her pump feeding as long as she can tolerate them (no dumping or diarrhea) and see where we can from here. Ultimately we would like to have her colon working and use the ostomy just as a pressure relief valve. That will let us peel away TPN and let her colon do the job of rehydrating her.

The CAIR doctors had a decidedly pessimistic view of Ellie's biopsy results and chose to highlight the presence of old scarring of her liver rather than the miraculous halting of the progression of liver damage that has happened since she went on omegaven. This is in sharp contrast to the opinion of Dr. Puder who tells us that Ellie's biopsy may be the subject of a medical journal article...

The CAIR team continues to stress that we need to get her off of TPN to reduce the risks that come with TPN and the ticking time bomb that is a central line. We agree wholeheartedly that we want Ellie off of TPN but are not going to rush anything.

Ellie put on quite a show for the clinic and we wish that we had brought a camera to record her performance. For a while yesterday she was parading through the clinic in a onesie, a t-shirt and her metallic pink cowboy boots. She loves them and chooses them over all of her other shoes.

A rock star.

Soft and Pink Round 2

Well the results are in and Ellie's biopsy supports all of the great news that we have gotten over the last 21 months about the effects of Omegaven to protect her liver from TPN damage.

The biopsy says, using very technical and scary words that Google says are really bad, that the damage to her liver is the same as her first biopsy that happened just after her liver function tests started to really improve in the summer of 2006. She still has some significant fibrosis in her liver which is a bad thing but it hasn't progressed toward the really bad things that we should have seen many months ago. This good news is further supported by the interpretation of Dr. Puder who noted that this biopsy was missing some of the damage that was seen in the earlier biopsy.

This is a big step for Ellie but also for all of he kids who use Omegaven since this is the first long-term follow up biopsy that they have done on an omegaven kid. Even though all of her liver function tests were good we didn't know for sure what was going on in her liver until now.

Who knows, she may just have her own medical journal article someday to go along with this blog that has chronicled all of her adventures so far.

We sleep a lot better knowing that the damage has been stopped and maybe even reversed a bit.

Ellie is oblivious to all of this and is most interested in her metallic pink cowboy boots that arrived today from her Aunt.

Christmas came almost a month late this year.

Surgical Update Part 2

I forgot to mention yesterday about what Dr. Jennings said about the status of Ellie's small bowel. He said it looked great! No dilation and it was all very pink with no strictures or adhesions. He said it looked a million times better than it looked last time he was in there. We did not have him measure her bowel, this would only have meant more time in the OR and more trauma for her. We know what we started with (around 30-40cm) and we know it is growing as she grows, now we just need to know what it can develop into.

Ellie also now has a new scar to add to the mix. Dr. Jennings didn't feel that he could reach all of her bowels using the old incision line, so he had to create a new opening. Gib and I were getting nervous about the ability of the old one to handle be opened one more time(it had already been opened 5 times), so we were happy to see the new suture line. The new one is just to the left of her belly button and it is vertical this time.

Ellie is doing very well. She looks much better this morning and is much more aware of her surroundings. The epidural is working beautifully and she is very comfortable. This is aided by the Benadryl that she is on, due to the itching caused by the narcotics in her epidural. With the Benadryl on board she is a very sleepy girl which is fine with us. If all you can do is lay in bed, you might as well be sleeping.

Both Ellie and her father are asleep right now which is good, we all need to catch up on some much needed sleep. Ellie slept for only short periods of time last night and Gib and I are having a hard time relaxing on this new floor. We are still on 10 South but hope to move to 10 East sometime this afternoon.

It is weird being here but not knowing any of the nurses, NPs or support staff. Even more frustrating is being here and having the staff not know us. We are getting some looks of skepticism when we ask questions and make requests. Many times we find that we are getting an answer but not the answer to the question we asked. I guess we also just miss our friends. Luckily we still have our great relationship with the surgical staff to help keep us sane.

We were in our room for about 10 minutes before Dr. Jennings came to check on our girl and then an hour later Dr Puder stopped by for a visit. This morning Dr. Gura stopped by and when rounds came through they were all familiar faces. The rounding surgical staff could not believe how big she was and they were all very happy with her recovery.

We will keep the epidural for the next few days and then see how she is doing. All in all Ellie is doing great and we are happy. Ellie has been asking for “Ga-Ga” (Karlene) and for “Good Dog Gus”. Hopefully she will see them both soon.

Soft and pink

Those are the words that Dr. Jennings used to describe Ellie’s liver. Take a minute to think about that, 20 months on TPN and her liver is “Soft and Pink”. He took a biopsy of both lobes, but said from what he could see and feel, it is in great shape. THANK YOU OMEGAVEN, THANK YOU DRS PUDER&GURA!!!!!

Obviously she is done. We got a call around 1:30 from Dr. Jennings and met him at Au Bon Pain while he got his lunch. He said that the intestines looked great, very little adhesions and that the connection between small and large bowel looked great. The reason food wasn’t going down the colon was that the connection had folded over on it’s self. He straightened everything out and said that is all that was needed.

He did make the ostomy smaller, but only under the skin, so what we see from the surface will look the same. He was going to make the skin opening smaller but said that would have caused a wrinkle in the skin, he was concerned that this would make it hard to keep ostomy bags on. We really appreciate the fore thought, we don’t need any more ostomy bag challenges.

G-tube. He looked at the old track and decided that here was not much he could do. So he installed a new hole. He cut a new hole in the skin but used the same hole in her stomach. It looks great and we are going to try everything we can to keep this one looking good.

Ellie moved to recovery in the PACU at 3:30 and we got to the floor around 5pm. But, we are not on 10 East (8west) there were no beds available. We begged and pleaded but they weren’t willing to kick some one out for us. Shame. So, now we are on 10 South. Same church different pew.

Ellie looks great. She had a very easy recovery with very little pain. She still has the epidural and it seems to be working great. Knock on wood, this is the best recovery she has had so far. It isn’t fun to see so many wires and tubes coming out of her, but they are all helping her feel better. The first time she woke up she looked at me said, “Ma, Ma” and then gave me the sign for the Wiggles. So we pulled out the DVD and we have a very happy girl.

We now can not post on our blog using the hospital’s WIFI, we have to wander with a laptop and find a free signal. We will post more when we can.

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.

Oley

Well, we are back from the Oley conference. It was great. We knew three other families that were there from Children's hospital. The first day we spent the morning in a large group meeting talking about the future medical advances in the area of short gut and for people on TPN. Then in the afternoon Gib and I split up and he went to a talk about Central line care and TPN and I went to one about pre and probiotics. (prebiotics=food for your good bacteria that you already have in your gut. probiotics=adding more good bacteria to your gut)

Gib's sister Beth came along to help with Ellie. Good thing. This was our alternative
Then we both went to a meeting about pediatric issues. It was great, they talked about things we had not really thought of yet. Like, potty training a short gut kid, Tooth and bone development, and puberty. It was eye opening for us, but also good because there were so many parents there and their short gut kids looked great and were running around looking like normal kids. They were just a bit skinnier and had a few tubes.

The only thing we did notice is that most of the kids that were there had lost their intestines when they were a bit older, not many kids were there that had lost some of their intestines at birth. But then we realized, we (the Omegaven families) are the new group at Oley. Now more and more infants are surviving into childhood because of Omegaven. So many of the meetings were very helpful, but they were all really directed at adults and older children. So now Oley has to recognise this new group that will only get bigger and bigger.

The second day the morning was a large meeting with every one. The guest speakers were Dr. Puder (Mark) and Dr. Gura (Kathy). They were there to talk about Omegaven. I think the message got through. When you say that Children's Hospital Boston used to lose 6-7 kids a year to TPN induced liver damage and now they the lose NONE. That Toronto hospital used to have a near 90-95% mortality rate of children with TPN induced liver damage. Now they have 0%. It is hard to ignore.

After that meeting we all went out for a picnic with four other short gut families, Mark, Kathy, and Denise (the Uber nurse).
Sorry that Gib is blocking out Mark, but he is there. This is from the left Charles and dad, Maggie and mom, Luke and mom, Ellie and dad, Caleb and dad and Kathy and Mark.
This is Gib and Ellie with Dr. Kathy Gura(discovered Omegaven) and Dr. Mark Puder(started and is currently running the Omegaven project). These are the people that are responsible for our happy, healthy baby girl. The only person missing is Dr. Jennings. This is Ellie and Luke on the swings together. It was Ellie's first time on a swing.
Ellie and Maggie playing peek-a-boo.
This is Luke and his mom with Charlie's mom and Charlie playing with Gib. Charlie is the first little boy that was put on Omegaven. They were willing to try anything to save his life. It had only been tested on mice before Charlie, and now he is four years old and his liver is doing better than mine.

If you look back at all the pictures in this post, you will notice that most of these cute kids have back packs on. That is what the whole conference was like. Halfway through the first meeting I realized that many of the adults had little back packs or fanny packs on. Many of the people there were on TPN almost all day and some were on G-tube feeds almost all day.

We had a great time an learned a ton. The biggest take home message we got, is that we are incredibly lucky to be at Children's Hospital Boston. Next week we are making an appointment to talk to Dr. Jennings about a bone density scan, leaky g-tubes, and starting pre and probiotics. Ellie passed out after the picnic. We were in the middle of emptying her ostomy when she fell asleep. Like her shirt?

Ellie's Birthday Cha-cha

I started my birthday with a baby that decided to magically sleep until 7:20am and then Gib let me sleep in even later by getting up with her and Gus. Usually one of us gets up with Ellie and the other feeds and walks Gus. I have been running in the morning with Gus, but it was such a treat to be able to sleep in that I couldn't resist. Sorry Gus.

This birthday was much better than last years, last year Ellie needed her first midnight blood transfusion. This year we spent the day pretty much like every other day, Chasing Ellie. Ellie and I did have one field trip. We went into Boston to Children's hospital to get a weight and length.

The Short Bowel clinic is over booked for the next clinic, so means we wouldn't be able to get in for another four weeks. Ellie was last seen a month ago, and to wait another month is just more that I am ok with. So,I took her in for a quick weight and length just to see how she did over the past month with one less night of PN. The good news is that she grew a half inch, bad news is that, as I suspected and as our nurse's scale was telling us, she is not gaining weight. The picture below is Ellie showing off her big mouth.
I made an appointment for tomorrow morning with her surgeon and called the PN Doctor when we got home and added another night of PN back on. She went from 5 nights of PN to 6 nights of PN. We were very excited for the decrease in PN nights last month, but about that same time Ellie started walking by herself and going up and down the stairs about a million times a day. So, right when she really could have used the extra calories, we took them away. Now we will give them back, give her a month or so and try it again.

It's the short gut cha-cha; two steps forward, one step back and repeat. With the scheduling issues that the Short bowel clinic is having it sounds like we will have Dr. Jennings manage Ellie's care and just check in with the clinic every once and awhile. That works fine with us and they all seem to have a pretty good working relationship so I know Ellie's info will get to everyone it needs to.

When we first walked into clinic I mentioned who we were and why we were there, and from behind me I hear, "Ellie Brogan? I know Ellie brogan." It was a nice mother with a very cute baby, that was there to see Dr. Puder. She found out about Omegaven from this blog, contacted Dr Puder, traveled across the country, got on Omegaven, and now her daughter is doing much better. The little girl still had some of the yellow skin color that indicates past liver damage, but she was a active, happy, smiling baby, not the normal PN baby. Thanks Omegaven.

I also found out from Dr. Puder that Yale medical, where Ellie was for her first two weeks, is starting to use Omegaven on their short gut babies. Wow, what a difference a year makes, when we were there no one had even heard of Omegaven, and now it is the standard. Great news. Below is Ellie with her book collection, she loves to just sit there and read or pull all the books off the shelves.
We spent my birthday night at my sister's house, for some great Japanese food and birthday fruit tart, my favorite. We have had a birthday bonanza around here, and my sister has been a great birthday host for all of them. Ellie got to play with her cousins and run, yes run, around in their back yard.

We are trying to prep this week for another trip to Maine, but we are aiming for two nights this time. This will be the first time we have done two nights away from home. All it really means is that we have to pack that much more stuff, but it also means we get to relax a bit more when we are there. It takes forever to pack for the trip and forever to pack again once we are there, it will be nice to have a day to relax in between. Oh yeah, and Maine means we have baby sitters! Oooh the possibilities.

Well that is about all that has been going on here, I do have to end with a few more pictures. The first is Ellie's room with... polka dots! I really wanted to put polka dots on Ellie's walls since Ellie never got to see the ones Jess and I painted in her first nursery. But as this is a rental house and I didn't think that our landlord would like me painting on the walls. Then I found stickers for walls, and as you can see I went a bit wild.
And last but not least, another picture of my girl in her tutu. Those of you that knew me as a child I sure were not surprised to see my daughter in a tutu. I can only hope that she will wear hers for as many years as I wore mine.Please look at the previous post for some great video of her before bed antics.

1 Year in Boston

I feel like Gib and I have a bunch of dates coming up that were big milestones last year. But today is probably one of the biggest. Not only is it my nephew's 17th birthday but, it was one year ago today that Gib and I followed Ellie's ambulance up the highway to Boston. If I had known then that in one year Ellie would be doing this well, I would not have been so scared. Below is a picture of my two favorite people, it's scary how much they look alike.
We chose to move Ellie's care to Children's Hospital Boston because they have a short bowel clinic. The idea of going somewhere that truly understood what we were dealing with was very comforting.

We also made the decision to come to Boston because my sister had offered us a place to stay. By this time we had spent over 2 weeks in the Ronald McDonald house, and were just about to loose our minds. The RMH gave us a room within walking distance of the Yale Medical, and that was great. But it was had to live there. It is hard enough to deal with you own emotions and confusion, but it is also very hard to watch other families do the same thing but in VERY different ways. We were really looking forward to staying with family, and enjoying their company and support

The move to Boston had the biggest impact on Ellie's health, because Boston equaled Omegaven. We know short gut children Ellie's age who have required multi-organ transplants to save their lives because they did not have access to Omegaven until it was too late. We came to Children's for the short bowel clinic, but now we will never leave because of Omegaven.

Sometimes I feel like Fresenius should hire me to sell their drug. I would like to stand outside every children's hospital with a banner and a bull horn to let all the parents know about Omegaven and what it can do. I would like to travel back in time to one year ago today to all those parents that we left in the NICU at Yale and convince them to come to Boston with us. It breaks my heart to think about where some of those kids must be today. We have said it time and time again, we credit Omegaven for saving our daughter's life. Thank you Kathy and thank you Dr. Puder.

So, now we have a beautiful healthy little girl. She doesn't eat as much as she should, but she doing well anyway. She is walking fully on her own now, and if I try to help she usually pushes my hand away. As much as we try to keep her clean her favorite place is still the dirty dog bed. She sneaks in there as frequently as she can, past almost any barrier that we erect. We know that she has managed to get in the bed by hearing her giggles and squeals. I found her in there the other day and had to take a picture before I yanked her out.

We are very happy here in Boston and are puzzled by what our life could have been like if Ellie was born with everything intact. We would have continued on as normal and we would have taken everything for granted. We never would have known how great it is to raise a child close to family. We never would have the chance to see how great our friends and families are in a time of crisis. We never would have gotten the chance to build new relationships and grow much closer with many members of our families. We never would have known how great a city Boston is and how much fun it is to explore Wellesley with a jogging stroller or baby backpack. And even though I miss my job, I never would have known how great it is to be able to spend everyday watching your child discover her world, the dog bed or the one kitchen cabinet that is filled with baby friendly Tupperware.

I can't help but think that maybe this was just meant to be.

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.

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.

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.

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.

Livers, Old and New

Hello Abby here.

As some of you may remember a while back we wrote about a little boy that we met at Children's receiving a Liver, small bowel and partial pancreas transplant. Many of you have been asking how he is doing so here is an update.

He is doing very well. He is out of the ICU and has settled into to spend a while learning how to use a full set of intestines and healthy liver. Amazingly enough he was able to keep the 18 cm of small bowel that he had so now he has more intestine than most people. He is having normal baby poop and his parents are looking forward to being able to feed him anything he wants, even junk food.

His new liver is doing well. There were some signs of partial rejection early on, but everything seems to be getting better. His blood numbers that indicate liver health look better than they have looked in a long time.

He still has a long way to go but is definitely on the up swing from where he was, and that is great news. Life post transplant can be hard but, it is what this little boy needed. At this point his has almost fully recovered from the surgery and now his body is just struggling to learn how to use its new parts.

His parents were able to meet with the pathologist and get a look at Christian's old liver. Now this may be a bit gross for some of you out there but below is a link to a video of the liver and what the pathologist said. I find it fascinating and one of the best visual aids as to what doctors mean when they say "liver damage."

This is your liver:














This is your liver on TPN:

http://video.google.com/videoplay?docid=-2822532247311576098&pr=goog-sl

I thank Christians parents for posting the video so that we could put it on this blog. I hope that other short gut parents are able to see this video and get a better understanding of what TPN does. Christian is a very strong little boy, once again he is my proof that these kids make us adults look like wimps.

We hope and pray that by using Omegaven and not intralipid Ellie's liver will not end up looking like this. She has had two liver biopsies in the time that she was in the hospital and both times they said there was mild scarring but no inflammation. Their theory is that the scarring, since it is not getting worse, could have happened in the beginning when she wasn't on Omegaven. They also said that its appearance was normal and not green.

We also realize that Ellie was only the 23rd infant to go on Omegaven and that there has been no true testing done, so we realize that she will not be out of the woods until she is off of TPN.

Sepsis

Ellie is doing very well today. She is alert and awake and looking around when she is awake, but sleeps a more than usual during the night and during the day. Hey if you had an infection like she does, you'd sleep a lot, too.


One of the most amazing things is the look on the doctors' faces when they come to look at her every day and discuss the treatment for her infection, which we have narrowed down to being a gram negative rod infection of her central line and also her blood itself. One of the nastier of all infections.


'Wow, she looks great. Not what we would expect."

That is quickly followed by: "why are her lips so blue?" The medicine that treats the thrush in her mouth dyes her mouth and her clothes a nice purple color. If only it weren't purple. I've got a daughter who looks like she borrows A-Rod's lip gloss!!!!

This prompted me to ask Ellie's Omegaven doctor this afternoon whether this was a great side effect of her fish oil treatment.

He told us that with Omegaven kids, their body's reaction to the infection is not as severe as kids on other lipid sources. It is the body's reaction which makes these infections have high mortality rates and that they generally Omegaven kids haven't gotten as sick as other kids.

Yes, the infection that she has has mortality rates associated with it. Somewhere between 25 and 30% of all cases from what the doctors tell us. That includes the kids who become very very sick and go to the NICU. Still this is an eye opener for us and very scary to think about.

Fortunately, knock on wood, Ellie's infection was caught very early (a blood culture was in the works even before a fever came on) and she is doing well on the cocktail of antibiotics.

Thanks, it seems, in part to a nice foundation of menhaden squeezings and a very alert doctor who watches ellie's blood work like a hawk.

Nice catch, Dr. Puder!

Did I ever tell you how lucky you are?

There is a Dr. Suess kind of book with this title and I tried to read it to Ellie the other day and just couldn't because every time I read about the person's unfortunate events, I found myself thinking about Ellie's situation and how unlucky we are (but not like Hurley on Lost, fortunately).

I have begun to take Ellie's progress back from her yellow months for granted and don't think about it as such a big deal. I guess that I never saw her fail like so many other parents of short gut babies have and never had the despair of not having anything to do to save her liver.

We really are very very lucky to be here and have Ellie doing so well.

Today, the Boston Globe started to expand the luck and published a neat article on the snake, er, fish oil that Ellie is on. It explains the good results that they have seen so far and the need for large trials to get more kids on the juice.

http://www.boston.com/news/globe/health_science/articles/2006/07/03/how_fish_oil_may_have_saved_babies_lives/

Hopefully this this will help some other babies that aren't as lucky as Ellie is.

Her liver numbers keep dropping and her Bilirubins are now "normal", .7 and 1.2.

The Short Gut Hokey Pokey

And the dance continues.

As soon as the doctors told us that they were encouraged by Eleanor's progress and ability to move forward with her feeding (from a whopping 1 ml/hour to 2 ml/hour), she goes and decides to start spitting up her food along with some nice green bile. Not a lot. Not even enough to need to change her clothes.

All engine stop. Circle the wagons.

Bing bang boom, in one hour this afternoon we had a new x-ray or her belly (all signs are good down there), a culture of her blood, a count of the cells in her blood ('CBC' is a Complete Blood Bount if you ever wondered what they were saying on ER) and a fresh new prescription for some baddass antribiotics (Vancomycin) to nip any infection of her central line in the bud.

Damn.

So much for the great progress. Now we stop and wait to see what the deal is with today's problems.

No food. Just some prime Ellie watching. Which, on the bright side, is going very well. She is up and smiling, cooing, and looking around all day and is very active. These are good signs that may show that whatever infection she may have is early in its progression and we hope that we can deal with this and kepp on the food wagon some time soon.

Another bright note for today- Ellie's blood test today was an opportunity to get another bilirubin test to see how her fish oil feeding is going. Her numbers continue to go down. 1.6 is her new number. down from 4.4, a few weeks ago and 2.3 on Sunday. the graph shows how she has progressed with omegaven. The graph of other kids would be a line upward, towards a transplant, hopefully Ellie won't go that way.

Menhaden Matter, In a New Sort of Way

Right now, Eleanor is being fed with a Total Perenteral Nutrition (TPN) system. This consists of a bag of fluids that looks like Lemon-Lime Gatorade and a fat white syringe of fats that are injected into her Peripherally Inserted Central Catheter, or PICC line in her right arm. This supplies her with almost everything that she needs to grow and develop. TPN is one of theose miracles of modern medicine that wasn't around 30 years ago that saves lots of lives, both infant and adult.

One of the quirks about TPN is that when infants are on it for an extended period of time (which varies from kid to kid), there is a negative effect on the kid's liver, eventually causing cirrohsis over time and necessitating a transplant of one, two or more organs. Nobody knows why the TPN causes this damage in kids but it is an accepted fact of the feeding through TPN that once you start it, you are in a bit of a race to get the kid off of it before the liver damage happens.

Curiously, and unbeknownst to us before we came to Children's, there is a doctor on the staff who is working on the million dollar TPN mystery question and has an innovative new treatment that may better better for kids than the traditional TPN.

Traditional TPN uses soybean oil as the fat. In a curious turn of events for Abby and I who have spent our lives chasing and working with fish, the doctor here is trying using oil from menhaden, a nasty little oily fish that most people use for bait or grind up into fish meal for use in dogfood, as a substitute for the soy bean fat. It turns out that the Omega-3 oils that are in the fish oil are anti-inflammatories and may decrease the ill-effects of TPN in infants and small kids. So far the doctor has had either positive results that reduce the ill-effects or null results that have no effect either way compared with soy oil.

Abby and I spoke with the doctor yesterday and asked a million questions of him and agreed that this is the best thing for her since the name of the game in her treatment is time. We hope that fish oil wil give her the most time to teach her broken intestine to be a super intestine.

Besides, if there were ever a sign of something that we need to do, having a fish show up in a hospital just may be it.

We recieved word today that Ellie has been approved by the FDA to be a participant in this study and will begin her fish eating life with menhaden. Abby and I will continue our fish eating life tonight with a round of sushi.

No menhaden on the menu. We hope.