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.

Christian's Re-birthday

1 year and 2 days ago a little boy we met at Children's hospital got a liver, small bowel and partial pancreas transplant.

It is hard to believe that this is the same little boy that we met in August of 2006.
Here is a link to watch a great video of Christian's re-birthday party.
video.google.com/videoplay?docid=-2702633552182627672&pr=goog-sl

You can see for yourself how great he looks. That video warms my heart and makes a bit choked up every time I watch it. Congratulation's to Christian for one great year and many many more to come and to his amazing parents and their big strong hearts.

Everyone please, please become organ and tissue donor today, as your holiday gift to the many little kids in the world like Christian. If you are already a donor, thank you, thank you, thank you.

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.

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


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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.

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.

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.