This is an article that I wrote for Complex Child Magazine back in April. We are working on a new post as well.

Current Information on Omegaven
by Abby Brogan

My daughter Ellie was born in April of 2006. She came a month early, but over all my pregnancy was normal and she was initially doing very well. Things began to change after her first hour. The next 24 hours were something I wouldn’t wish on anyone. It ended with both of us in a new hospital, her recovering from exploratory abdominal surgery, and the very nice surgeon explaining to us that Ellie was born missing 90% of her small bowel and 30% of her colon. After she fully explained what Ellie’s GI system looked like, she started talking about what Ellie’s life was going to be like and what the future could be like. It was a very scary and grim conversation.

Ellie as a baby before Omegaven

Starting TPN

Ellie’s GI tract was not able to absorb enough nutrients to sustain life, so she was placed on IV nutrition support, also called Total Parenteral Nutrition. Total Parenteral Nutrition (TPN) is made up of two main parts. The first part contains all the fluids, vitamins, minerals, amino acids, and medications. The second half of the mixture is the lipid, consisting of a fatty acid that has been broken down enough to be absorbed through the blood stream, and blended into an emulsion that keeps the fats from separating from the fluids. TPN enables an individual to live and grow even though he or she cannot eat.

However, the down side of TPN is that for most individuals on it for an extended period of time, particularly children under two years of age, it will also cause a build-up of fat in the liver causing damage. For these individuals, there is a race to rehabilitate the GI tract and get off TPN before the liver becomes too damaged. If they cannot get off TPN fast enough, they often will be listed for a liver transplant and, if needed, other organs as well. For those ineligible for transplant, liver damage can lead to death.


What is Omegaven?

In 2001, Children’s Hospital Boston started doing research on TPN to find out what part of TPN was causing liver damage. Mark Puder, MD, a surgeon, and Kathleen Gura, PharmD, a pharmacist, collaborated on the research with other doctors at Children's Hospital Boston. Through studies done on mice, they discovered that it was the lipid portion of the TPN that was causing the damage. But the fats contained in lipids are essential for the body, so they needed to find an alternative.

Dr. Gura had previously used Omegaven, a lipid emulsion derived from fish oil that contains omega-3 fatty acids, to treat a patient with a soy allergy and severe fatty acid deficiency. From this experience she suggested they try Omegaven in their trials.

The results were very promising.

In an article written by Children’s Hospital Boston, Dr. Puder explained the results that showed that parenteral nutrition without the fat makes the liver fatty, and by adding the Intralipid, it becomes even more so.¹ "That fat becomes more or less a fuel that burns the liver, causing it to become inflamed....But the Omegaven is an anti-inflammatory lipid that prevents fat build-up in the liver. So if there's no fuel—fat—there's no fire—injury."¹

The results were so promising that on September 26, 2004, the team at Boston Children’s Hospital started the first child on Omegaven. They saw improvements in one week, and a month later the child’s liver function tests were good enough that he was taken off of the transplant list. In most children, during the first month or so the direct bili (liver function test) goes up and then comes down. It is known as the Omegaven roller coaster. But, during this time you get to watch your child turn pink again.

Ellie's bili chart

Some hospitals are using Omegaven in combination with regular vegetable oil-based lipids such as Intralipid. Dr. Gura commented on this, stating, “Based on published papers, it appears that children with PN liver injury do better with Omegaven alone (not mixed with Intralipid). The Diamond paper from Canada actually shows this, although they still like to do the combination until the child fails and then they switch them to plain Omegaven.”²

To date Children’s has treated 166 children with Omegaven and administered over 100,000 doses. Omegaven has helped almost all 166 children, but the team has learned that the faster these children get on Omegaven, the better they do. Using it as a last resort or rescue is not always successful. The damage to the liver is already done.


How to Get Omegaven

Omegaven has not been approved by the FDA to be used broadly. Because of this, it has a Compassionate Use Only status, meaning that it can only be used if it will save the child’s life. To show that the child needs Omegaven, he must have failing liver function tests, including a direct bilirubin number greater than 2 mg/dL, indicating liver damage called cholestasis.

In the beginning, patients had to transfer to Boston to get Omegaven therapy. Now, thanks to the passionate outreach by Puder and Gura to the medical community, it has been used by almost every free-standing children’s hospital in the United States. Physicians can also apply to be able to administer Omegaven themselves, and the Boston Omegaven team has gone so far as to publish their therapy instructions with the Oley Foundation, a support group for those who rely on TPN.

Each year, more and more insurance companies are paying for Omegaven enabling patients to be treated locally and not requiring them to travel to Boston. The FDA now allows billing of insurance companies upon request. Approval, however, isn’t mandatory, and insurance companies are not required to pay for it. For a list of the hospitals that have used Omegaven and the insurance companies that have covered it, visit the Short Gut Wiki.

Making Omegaven available to everyone and covered by insurance will continue to be an issue until it is approved by the FDA for use in all people receiving TPN. Currently, Fresenius Kabi AG, the manufacturer of Omegaven, is working with Children’s Hospital Boston to get the necessary data for a new drug application submission to the FDA. The process to get a drug approved in the US is lengthy. There was hope in the beginning that additional animal testing would not have to be done or could be avoided; however, these tests were ultimately required and are currently underway.

Children’s Hospital Boston has not seen any adverse effects of the use of Omegaven, but they are monitoring all their children with long-term blood work and other follow up examinations.


Success!

The child that received the first dose as an infant in 2004 is still on TPN and Omegaven at age seven, goes to school and has shown no adverse effects from the TPN that likely would have destroyed his liver just a few years ago.

A recent picture of Ellie

My daughter Ellie was the 23rd child to receive Omegaven, and has now been on it for almost five years. She is doing well, growing and thriving. Both my husband and are so thankful for Omegaven, and the doctors that brought it to our attention. I will not let myself imagine where she would be without it.


Thanks to Mark Puder, MD, and Kathleen Gura, PharmD, for their personal assistance in providing information for this article.

Abby lives in the Boston area with her husband and daughter Ellie. Ellie was born in 2006, missing 90% of her small bowel and 30% of her colon due to Jejunal Atrisia. Ellie is diagnosed as having Short Bowel Syndrome. Ellie has had multiple surgeries since birth and is on tube feeds and Home Parenteral Nutrition (HPEN). Abby and her family have faced the many challenges that come along with having a child with tubes, yet lead a remarkably active lifestyle. Abby is also a consumer advocate for ThriveRx. Please feel free to contact Abby at
1-774-270-3425 or via email at abrogan@ThriveRx.net.

---

¹http://www.childrenshospital.org/dream/dream_fall06/fishing_for_the_right_solution.html See also http://pediatrics.aappublications.org/cgi/content/full/118/1/e197
²Personal communication with Dr. Gura. The study she cites is Diamond IR, Sterescu A, Pencharz PB, Kim JH, Wales PW. Changing the paradigm: omegaven for the treatment of liver failure in pediatric short bowel syndrome. J Pediatr Gastroenterol Nutr. 2009;48(2):209-15.

Trouble for Omegaven in Maryland

Gib just learned that there is a bill before the Maryland legislature that will prohibit the manufacture, distribution or sale of any products made from Atlantic Menhaden, the tiny smelly fish that we have been told is the primary source for Omegaven's omega-3 oils. HB1142, as it is known, is scheduled for a hearing on March 16. It is very vague but could mean the end of omega-3 supplements, medications, possible research in Maryland unless they can be shown to be derived from non-Menhaden sources.

http://house.state.md.us/2011rs/bills/hb/hb1142f.pdf

We are not fans of the commercial menhaden industry and believe their fishing should come under more stringent control to look out for the oceans and the Chesapeake Bay, but this approach could have real consequences for people, both old and young in Maryland and beyond.

If you would like to have your opinion heard on this, especially if you live in Maryland, letters can be addressed to:

ENVIRONMENTAL MATTERS COMMITTEE
Delegate Maggie McIntosh, Chair
Delegate James E. Malone Jr., Vice Chair

Room 250, House Office Building, Annapolis, MD 21401-1991

Not Heavy Enough

We had clinic today with Dr. Lo's TPN clinic. Ellie had only gained just a tiny bit and had grown an inch since last Nov. The problem is in December she got sick, got admitted, had really bad SBBO and lost a ton of weight. So the fact that she is up from November's weight is good news to us. But when they did the body mass evaluation her numbers were down. So she is growing but needs more calories. They want us to get those calories enterally not through more TPN. So we are going to push more elecare by mouth and turn up the overnight enteral pump. We are also going to try going up in calories per ounce of formula.

They also talked about prepping to get her off of TPN/hydration fluids. To do this we are decreasing the amount of every overnight hydration bag from 1200mls to 1000mls. We have clinic again in 2 weeks and if she is doing well we will decrease more, if not then we go back up. First they said they wanted to leave every night at 1200mls and then do one night at 600mls. The idea of that made me gulp. So we compromised with a decrease to 1000mls.

All in all, they were happy with how she was doing, just not as impressed as I was. So now we try new stuff and hope for a big weight gain over the next few weeks. We're in for big fun now!!!

Think Heavy & Remembering the Beginning.

Ellie has been doing really well lately. She is eating like a horse and we have been able to keep better control over her bouts of overgrowth. From what we learned in the last Maximize Health webinar, protein is key for Ellie. Protein is digested in your Jejunum and the only small bowel that Ellie has is her Jejunum. So we have kicked it up a notch in the protein department. We have started offering, low fat ground turkey, low fat ground beef, poached chicken and some vegetarian options. Eggs apparently at a perfect protein and Ellie is loving her eggs right now. She has been very happy with our diet modifications.

So, we are keeping our fingers crossed that at our TPN clinic tomorrow we will hear good news. We always tell Ellie to think heavy thoughts before she steps on the scale at clinic. And tomorrow she better be thinking HEAVY. We are only on 3 nights of TPN and we want OFFFFFFFFFFFFFF!

Ellie has already come off of TPN once. When she was 2 and half years old she was off for 6 months. Then we did the reconnection surgery and now almost 2 years later we are hoping to get off again. Keep your fingers crossed for us!!

Getting this close to getting off of TPN has made us think about when we went on. I have also been talking to some parents that are right at the beginning of this roller coaster ride. I have forgotten how scary and uncertain things were after Ellie was born. William has been very easy and it has only pointed out to us how hard things really were. When Ellie was born there was very little information out there about Short Bowel, TPN or Omegaven. Many times we felt like we were flying blind. We did get hope when Ellie was a month old, a family that used to live in AZ send us a picture of their son that was short bowel. He looked fantastic. It made us realize that how things were now was not how they were going to end up.

Now there are many resources out there for parents and adults with short bowel. Over the next few weeks I am going to update the links we post on this blog to make sure that all the resources are listed. I also want to update our blog list. Most of the kidos listed are around Ellie's age, give or take a year. I would love to add some blogs/caringbridge sites for more kids and even some for adults. If you have any suggestions for resources or would like your blog added please either leave a comment on here or email me abbybrogan@hotmail.com.

The next topic for Maximize Health is fats in the short bowel diet and how to avoid fatty acid deficiency. This will be very important if we get off of TPN!!

Nutrition for Short Gut

We have struggled with Ellie's nutrition for a while and found that there were very few resources out there to help. It is a balancing act of getting her to eat and gettine her off of TPN,...good diet=less TPN.

We have a good nutritionist at Boston Children's but we only see her once every 2 months or if something is wrong. We have a great nutritionist through ThriveRx but what I have always wanted was a book or guide that we could keep on hand. Something that we can also share with our family and Ellie's nurse to help them understand the intricacies of Ellie's diet.

Finally we got what we needed. ThriveRx has created an online short bowel diet guide called Maximize Health. Each month they release a new topic, so far they have covered Diet overview, hydration, Carbohydrates and the next module is going to be on protein. The educational modules are available on line and then they host a webinar on the second Tuesday of each month led by the author of the modules. She gives a better explanation of the content of the modules and then at the end she answers any questions that you might have.

For us the Carbohydrate module was very informative as this is 80% of Ellie's diet. It changed the way we looked at her food and made us be much more aware of what she is eating. I am very excited about the module on protein since we know this is very important for Ellie but we are always struggling to get enough in to her.

The best part about this education program is that it is free to everyone. If you are interested just visit ThriveRx.net You have to register for the program, this will give you access to all the past modules, and recordings of past webinars and get you an invitation to upcoming webinars.

If you have questions about this email me at abbybrogan@hotmail.com.

They are also hosting a webinar next week on TPN stability called. TPN Compatibility and Stability: What’s the big deal? it s on February 4th @ 12:00 noon EST.

Presenter: Sheila Pedapati, Pharm.D.

She will talk about:

• Verification of IV compounds and supplies
• Patient counseling regarding medications, supplies, and pump use
• TPN compounding and different types of pumps
• Working closely with nurse liaisons to optimally manage patient care

IF you are interested in this one email: info@thriverx.net.

2010 A YEAR IN REVIEW

Again we have let too much time pass between posts. Posting once in 6 months is not OK. Rather than try to catch up with a quick update. I am just going to do a review of the year.

2010 A YEAR IN REVIEW

We started the year with the huge bummer of Gus getting diagnosed with cancer. Then moved on to the excitement of Ellie’s first solid poops. In mid January we had a new refrigerator delivered and installed. The next morning we woke up to a flooded basement, and flood damaged kitchen. This led to a nice little kitchen and basement renovation that we have always wanted to do, Thanks Sears!!. During all this we also found out I was finally pregnant with baby number 2. I was rocked by ragging morning sickness, but I would have put up with anything, we were so excited to be pregnant again. I think this made loosing Gus in April a bit easier to deal with.

We struggled with hydration for Ellie through the end of the spring into the beginning of summer. We finally got things under control just in time for the Oley conference in NY. This kicked off a great summer with Ellie taking to swimming like a fish. We spent a week on the Cape with my family.

Ellie was on the beach and in the water everyday. We managed to get our routine for covering her dressing down so well that she went the whole week on the beach, and in the water, without her dressing ever getting wet. She had such a blast she has been talking about going back ever since.

The summer ended with Ellie starting school again. She started five days a week and we struggled all fall to keep her in all 5 days. She stays home if anyone in her class or the class next to hers has a stomach bug or if she is feeling punky. We work with her school to create a letter that goes out to all the parents explaining Ellie’s issues and why following the school’s sick policy is so important to her. This worked well last year and so far has worked well this year. The best part is it really helped other parents be able to us about Ellie.

We spent the fall apple picking, going to local farms, exploring Boston and enjoying the cooler weather.

I loved the last part of my pregnancy and loved making it all the way to my due date. My pregnancy with William was much easier and I felt much better thought out the whole thing. This just made us realize how sick Ellie was in utero and how much of a toll that took on me. William was a breeze in comparison.

At the center of the hedge Maze

William was born via C-section because I went into labor but never progressed. We called the ball a bit earlier with William than we did with Ellie and that made my recovery much faster with William.

Ellie was a giraffe for Halloween and managed to cover most of the neighborhood with her friends.

She doesn't like chocolate so that made the candy easier to deal with. And after the first few days she forgot that she had candy and that made it easier for Gib and I to finish it off.

I took the rest of the year off from work and enjoyed the rest of the fall and holiday season at home with my kids. I am still getting used to saying “kids”.

Through out the fall we struggled with Ellie’s short bowel bacterial overgrowth (SBBO) , so much so that the intervals between outbreaks got to be shorter than 1 week. We made our rounds through different drugs trying out Flagyl, Vanco and Cipro without really making any progress. We got to the point where we were giving her an extra 1000 mls of hydration fluids each day and still not keeping up with what she was loosing through her loose stools. This finally came to a head in December when we lost control of Ellie’s hydration and had to have her admitted to Children’s.

She spent 5 days in the hospital getting hydrated, doing bowel prep and getting a scope of her bowels to see if we could see anything that was wrong. We were also able to sample some of the fluids that they found in her bowels and culture it see what bacteria we were dealing with. They typed it out and then tested different antibiotics on it and found that Bactrum was the new antibiotic that we should try.

We did a week of Bactrum right before Christmas and it finally stopped the cycle of SBBO that we had been seeing. We also finally started probiotics (VSL3) after talking to another family that has been using them for years. Our team at Children’s was against this decision but they understood why we wanted to try them. And so far so good, it has been just under a month since we saw any signs of SBBO. (knock on wood now)

Christmas was great, we even got some snow to make it a true white Christmas.

We spent the week following Christmas and New Years in Maine with Gib’s family. We had a blast and Ellie loved seeing everyone up there.

So, now we have started the New Year. Things are good so far. In comparison to last year it has been an uneventful year so far. We did make the decision to take Ellie out of school for two days a week; we are hoping this will give her a chance to recover from her fall. William also started part time daycare last week. I think that was harder on me than it was on him.

All in all William has been so easy and laid back that if we could afford it I would be pregnant again. It has been great for Gib and I to have the chance to be typical parents. There is nothing like a very high maintenance baby to make a typical baby fell like a cake walk. Although we did have to remind our pediatrician to treat us like first time parents because we have no clue what to do with a baby that is under 8 months old and at home. All the typical kid stuff throws us for a loop. Things like… eating and pooping, those are things we didn’t have to deal with in Ellie’s first few months.

Today we are spending the day inside watching a true Nor’easter dump close to 2 feet of snow on our house. We have been out playing in it once already today and will head out again as soon as our mittens dry out. We promise to make more of an effort to keep this blog up to date this year. I know you have heard that before, but this time we really, really promise!

William Pics #2

Here are some more pictures of William. We were discharged from the hospital yesterday. We have all been recovering trying to sleep as much as possible. Right now William is spending all his time eating and sleeping. Now here are some more pics of William and his family.

William and his Aunt Jennifer

What the house looked like when we got home. Thanks Sara, Jennifer, Karlene and Ellie!

Karlene, Ellie and William

William in a blanket that his Aunt Karen knit

William and his sister

William Gilbert

Ellie got a little brother this weekend:

William Gilbert Brogan arrived at 2:47 AM on Sunday. 8 pounds 11 oz. and perfect.

The question now is how long will it take us to get used to a typical kid, doing typical things. Poop, eating, and sleeping are all new for us and force us to admit to the nurses that we are not quite first time parents.

Abby and I both look to clear IV lines when we pick him up.

Ellie wanted us to know that he shouldn't be afraid about dressing changes and wondered where his ostomy bag was because that's what babies have.

How different it would have been to have the complicated kid come second.

Saved by a fish, Swims like a fish.

It has been a very long while since we have reported Ellie’s progress and I thought that it would be good to bring this blog up to date with some info about how the girl is doing. In a word, fantastically. So well in fact that we have very little time to sit down and write about it.

But more specifically, now that we have the time here are the newest developments.

We have had a pretty rough Spring. Ellie’s Bacterial Overgrowth (caused when the bad bugs flux up into her small bowel from her colon) made her gassy, and vomit a lot while taking her appetite and dehydrating her all at the same time.

No fun for anyone. All to often it leads to a funny/awkward scene at the mall with me standing over a puddle of short gut barf trying to flag down a janitor before someone slips and falls as Abby and Ellie run to the bathroom before the second wave hits....

It looks like four courses of heavy antibiotics including seven-day courses of Flagyl, followed by Cipro, followed by Vancomycin, and another course of Vancomycin over an eight week period this the spring has finally done the trick and knocked down the carpet of bugs.

We treated the effect and then hunted down the cause. We finally thought of her overnight hydration. In the end of March we decreased her nightly IV infusion from 750 to 500mls. Not a big change but big enough. Two weeks after this change is when the overgrowth reared its ugly head. The fact that it wouldn't go away meant that we had to find the cause. And about two weeks ago we finally did. Bump her back up to 700mls a night, and Presto-Change-O, SHE"S BACK!!!

Ellie’s appetite is back now and she is doing quite well eating three meals every day, asking for snacks in between. If she had her way, she would eat nothing but macaroni with olive oil and wheat thins. Her mother and I do everything we can to get protein into her diet and she is doing well with scrambled eggs, ham, chicken, and Boca sausages.

Top this off with a liter of Elecare and you have a recipe for a growing girl. In the last two weeks, she has put on almost two pounds of the weight that she lost since getting overgrowth in the early part of the spring.

With all of the food and fluid going in, you might ask about her bowel habits. First off, she is very proud of the fact hat she has been wearing big girl underpants for a long time now. Each day she starts out by taking on the persona of the Disney Princess on her drawers and most nights she can even make it through the night with the TPN pump going with only one call for help to carry her pumps into the bathroom for her.

Accidents are quite rare and most days see between one and three poops of varying consistencies*. If she gets backed up, she gets nauseated and uncomfortable, an indication of how ‘short’ she really is but we have learned to recognize these signs and get her bowels to clear and make room below.

*This is the part that she will crucify me for writing when she is 14. Sorry, Ellie, it’s public service, I guess J

Unfortunately our bumpy spring has slowed our doctor’s ambitious plans to have her off IV fluids by the fall and in fact, we had to increase her TPN this spring to keep her weight up. Right now she is on 4 nights of TPN a week with Omegaven, and just D10 on the other nights. It is working to keep her weight increasing and also keeps her well hydrated even when it is hot. Those are good things right now.

We have TPN clinic in a few weeks and will likely get advice to wean a night of TPN. If the magic scale at Children’s shows her weight holding its own, I am all for it.

Another exciting update is that our little swimmer has been improving rapidly in her aunt’s pool over the last few weeks and now swims, with just her bubble on her back, from one end of the pool to the other and rarely needs help. We have had a lot of practice and are getting quite good at waterproofing her Central Line to the point that everything is dry when we are finished.

Our Recipe for success in pictures:

1) Wrap Press n’ Seal tightly around the CVL cap making sure to cover the threads of where the cap screws onto the line.

2) Coil the line plus cap over the dressing

3) Cover the coiled line and dressing with a large piece of tegaderm so that nothing is sticking out, and voila, you are waterproof and submersible.

More often than not everything under the tegaderm is dry and we just pull off the tegaderm and leave the dressing. If the dressing is wet we change it ASAP but, because the cap is covered with press-n-seal it stays dry. That be said, more often than not we change the cap after a swim day. The risk is just not worth it.

Ellie swimming has been great for her, but even better for us. Imagine two marine biologist having a child who's life is saved by fish but cannot swim. Finding a way for Ellie to swim and seeing her love it as much as her parents do is, priceless.

The final bit of news is Ellie’s first big girl haircut. This weekend her long hair became too much to manage and we got a bit aggressive with the scissors. The result is our four year old who seems to be channeling her inner Natalie Portman in the The Professional:

I’m trying to avoid the mental image of her telling me, “I've decided what to do with my life. I wanna be a cleaner.”

HE DID IT, 13.1 miles with Short Gut!!!

Dave Vermette ran a half marathon yesterday. Remember this is the man that is missing most of his small bowel due to Crohn's Disease and he is on TPN.

Check out his blog for pictures: http://thriverx.wordpress.com/

One picture you will not see is this one of his jersey. Check out the third name down. I have been saying that Dave is an inspiration to me and will be an inspiration to Ellie when she is older. But it seems that Ellie has been an inspiration to Dave the whole time.

It is so good to see someone defying the odds and refusing to settle for a life that the doctors told him he would have to live. The training was not easy, and not always fun. But the smile on his face yesterday was amazing.

I also think some of the other runners were a bit jealous of the IV bag he hooked up to after the race.

GO DAVE, GO!!!!!!!!!!!!

NSTAR walk for Children's hospital

We just finished the NSTAR Walk for Boston Children's Hospital. We walked 2 miles with team Blaise! The team was made up of Blaise and her family, and Faith and her mom, and some great friend's of Blaise's. As a team we raised just over 3,200$. No to shabby.

Ellie and Faith

We couldn't help but think of the last time we walked and how different Ellie was this time. Other than the fact that she is 3 older. Differences: No ostomy, potty trained, finishing her first year of pre-school, off of TPN and back on, and she is starting to read. Gib and I hoped during the last walk that 3 year later she would not still be on TPN, but hey, things are pretty good and we are not complaining. Ellie had a great time this time and walked a fair amount of the walk. She really liked looking at all the duck and geese along the way. She also loved all the after race festivities. Like getting her face painted, getting metal for finishing and the Blue's Clues moon bounce. She also love collecting all the swag from the vendors.

It was also fun to catch up with Blaise's parents and Faith's mom. We had dinner on Friday with everyone and as always it is so nice to talk to people that have been through what you have been through. We hadn't meet Blaise's parents in person before, we had emailed and talked on the phone but that is it. We also hadn't seen Faith's mom since right before they went home last year after coming out here for Omegaven. It was amazing how big both girls were and how great they are both doing.

During the walk today we ran into a bunch of the nurses from 10 East and the child life specialist. It was good to see them outside of the hospital. I don't think we say thank you to them all enough. They all always do so much for us to make our stays at the "inn" as pleasant as possible.

To all the nurses on 10 East and Lakeisha (the toy lady as Ellie calls her) .....Thank you, Thank you, Thank you.

It was a vary fun day followed by Ellie not taking a nap

Short Bowel Hydration Hints

I am posting information about a Webinar that ThriveRx is hosting next Friday, June 4th at noon. I just saw a trial run of the talk and it was fantastic. I really wish I had seen it about 3 years ago. The talk is free and open to anyone. It centers on oral hydration tips but it would work for those without short bowel as well.

“Hydration Hints for the

Short Bowel Consumer”

Presented by Maria Karimbakas, RD

June 4^th at 12 noon

Topics to be discussed:

v Causes of Dehydration

v Signs and Symptoms of Dehydration

v Treatment Options

v Preventing De-Hydration

v Review of Oral Re-hydration Solutions

Maria Karimbakas is a registered dietitian. She has worked at Brigham and Women’s Hospital in Boston, Nutritional Restart Center in Hopkinton, MA, and currently she works as a consultant for ThriveRx. Maria has co-authored several abstracts and papers on the subject of diet and SBS.

You may access this program by phone only or via the web.

To register for this event please email Abby Brogan at abrogan@thriverx.net.

2 Hours 20 min

Another record for Ellie. Fastest UGI since she was reconnected. Very good news. They saw a bit of dilation but not blockages or narrowings. We were very happy and said, "ok what IS wrong then?" We are brainstorming and trying to come up with some ideas. This is the part of Short Gut I don't love, the guessing part. Since no two short gut kids are the same, you just have to try everything until you find something that works. UGH.

UGI

Yes, that means Upper GI study. About a month ago Ellie started having all her normal signs of overgrowth.

1. Stinky poops and gas
2. Increased gas
3. Decreased hunger drive
4. Yellow colored very loose stool.
5. Vomiting in the late afternoon/before dinner.

So we called CHB and got her on a week of Flagyl. No change. Maybe we targeted the wrong bug. Start one week of Cipro. No big change but a bit better. Ellie's nurse comes down the stomach bug and Ellie starts vomiting more frequently. We chalk it up to the bug and wait it out. Last week we finally called CHB, squeezed in to a last minute clinic appointment. She lost just over a pound in the past 2 months. They started her on a week of Vanco and added a night of TPN. We talked to Dr. Jennings and he said a UGI might be in order, and Gib and I begged to try everything else first.

Now it is a week later, all her signs of overgrowth are gone, but she is still vomiting. UGH.

So this afternoon we are headed to CHB for an UGI. I hate these. Ellie doesn't mind them too much, they do not involve a blood draw or a shot, so she is happy about that. But the worst part is getting the thick white barium out of her after the study. This one will be different because I cannot be in the room with her for this one, pregnancy and radiation do not mix. We have talked about it a bunch last night and today so I think it will be ok.

We call the UGI cave that they put you in a camera. So we bring our camera, Ellie takes pictures of the camera, the room, the tecs and us and then the "camera" takes pictures of her belly. It worked pretty well last year, so we should be good this year. Knock on wood. We got a KUB x-ray last week and Ellie ran into the room, stepped on the lever to lower the table, hopped on, laid down, pulled up her shirt, lowered her pants to expose her belly and yelled "say cheese!" . You should have seen the technician's face.

Now of course through all this Ellie has been her normal bubbly self. She is still going to pre-school, up to Maine for her grandfather's 70's birthday, to ballet, to swimming lessons and building the most awesome Lego castles. She doesn't like the vomiting part, but she has over all been pretty good.

Both Gib and I are not sure it is an obstruction that is causing the problems but we seem to have looked at everything else. I really don't want to see one today, but on the other hand I want answers as to what is wrong. Again we are heading into summer with GI issues. We lost most of our summer last year to GI issues and surgeries. I would love it if we could not have that happen again. I would like to worry about what we are going to do for fun and how we are going to fit it all in, not about being inpatient again. And I really don't want to have to tell Ellie that she needs another surgery, she remembers too much from last summer.

Sorry, no pictures on this post. I will post more later. And we will update you on anything that they find during the study.

Inspiration

I have been helping my friend Dave write his blog about training for the Boston Half Marathon. What makes this is so incredible is that he only has 20cm of Small bowel. He works full time, is an avid advocate for others in his situation, and oh yeah.... he is going to run 13.1 miles this June.

I am perfectly healthy and that task seems daunting. But it has been really fun helping Dave and to see his progress. But I have to say the best part is seeing his determination and refusal to just sit back and be an observer.

Whenever I get sad about the things Ellie might not be able to do when she is older, I just check out Dave's blog and I feel much better.

Thanks Dave for the inspiration.

Dave's Blog: No Guts, All the Glory

4 YEARS OLD!!

Happy 4th birthday Ellie!!

I can't believe that she is 4. That means she is much closer to being 5 and the next thing I know she will be 10 and then 20 and......

I am so not ready for my baby girl to grow up.

We are having a quiet celebration here tonight and then a blow out birthday party with her preschool class. We have rented a bounce house and are ready for the madness.

We check the weather forecast about a million times a day, and so far we have sun. So keep your fingers crossed for sun!!!

Ellie has requested cupcakes again this year, so I will be baking all afternoon. She is decorating cookies with her class today at school. It is amazing to me how good dairy free, nut free, egg free cookies and cupcakes can taste.

#2

We got our first look at #2 today. The high end ultrasound was way better than the one that the previous OB used.

Maybe that's why they missed the diagnosis completely! Either that or they just weren't very good.

#2 looks great, and is quite active even though not big enough for Abby to feel the activity.

We will miss you Gus

We had to say goodbye to the Wonder dog yesterday. He was great and we will miss him.

Drumroll, Please.

Ellie has some breaking news that she would like to share:

http://www.youtube.com/watch?v=bW40R-_iQrw

Swimming

This winter Ellie started swimming lessons at our local YMCA.

How, you might ask, is the same girl that didn't take baths is now swimming at the Y?

The answer lies somewhere in the land of technology, calculated risk and a healthy dose of normalcy.

The technological side came with our fine-tuning of a way to keep Ellie's central line cap and dressing dry even in a pool. We started experimenting last summer in Abby's sister's pool and had some miserable failures but since we have gotten much better and have a waterproof system that works very well.

It starts with a piece of Press n' Seal from the supermarket. We wrap that around her cap and press it to seal it. Water against the cap is the big risk for infections and s it does sealing food in your fridge.

Once the cap is sealed, we take a piece of tegaderm, a very sticky, waterproof film that comes from the medical supply company and cover her whole line, dressing and cap under a canopy of plastic.

Voila, we are waterproof!

We think that the biggest thing is that now Ellie's chest is finally big enough to fit a sheet of tegaderm onto her chest without any folds. Folds are bad and let water in. That isn't to say that the solution is fool-proof and we are quick to come home and change her dressing and cap before her next IV infusion but it reduces the risk and lets us get her doing things that other almost-four-year-old kids do.

It was time get her wet and this does the trick nicely even if Abby and I both keep personal 72 hour clocks after she gets wet to watch for central line infections.

We can now focus on getting her to let go of the pool wall by summer.