Ellie was in the Boston Globe again!

Make-A-Wish accepting frequent flyer miles

See the whole article at: http://www.bostonglobe.com/lifestyle/travel/2014/05/10/the-tip-make-wish-accepting-frequent-flyer-miles/U5yXAQGSafN1TgRBEvD8hM/story.html

Last fall after our trip we talked to Make A Wish about helping them in anyway we could. Ellie and I went to a basketball game at a local college to help raise awareness and said we would be willing to do more. They asked if they could use some pictures of Ellie for an internal newsletter and we sent them 5-6 photos from the trip saying they could use whatever they wanted if it helped others get a wish too.

 We never thought we would wake up to Ellie in the Globe again.

 If you have miles that you are not planning on using or can't use before they expire, please consider donating them! Once donated, the miles never expire. Participating airlines include Delta, US Airways, and United/Continental. More information at www.massri.wish.org/miles.

Sharing Your Story Can Be Such a Blessing to Someone Else!

I was searching the internet for something else tonight and found this.



It was heart warming to see, and I loved the message.  We hear from families still today that say our blog helped then when they needed it the most.  Even though we don't update nearly as much as we should we keep it up and running just so it can be there to help another family when they need it.  If you have a blog, Facebook page or group that you want to spread the word about just let us know!

A Very Wet Day

Thursday was a wet day, A very wet day. We were up very early to slog through Honolulu traffic to Hanauma Bay, a marine reserve that was set up to conserve and manage a very healthy and very accessible coral reef near Honolulu.  We were told to get there early to make sure that we had a space in the parking lot (Make A Wish made arrangements just in case it was jammed) and we were there for 9:45 AM, along with about a zillion GoPro-toting tourists that really didn't get the concept of don't stand up on the reef.

But fortunately, I had forgoten that Abby Brogan also moonlights as an underwater superhero and proceeded to escort/drag/carry Ellie past the hordes of googins and out into deeper clearer water where the novices didn't dare.  What they found there was nothing short of spectacular.  Turtles, live corals, and fish like Ellie and I had never seen before.

 

 

Ulua, or trevally, cruising the reef.



Ellie showing off the surface dives that she practiced in the pool all summer.

  

Abby the Underwater Superhero showing off on the bottom

One very cool urchin.

A school of convict tangs

One of the many poses that Abby used to escort Ellie around the reef.

 

 

  

All told Ellie and Abby were in the eater for almost three hours with one short break for Elecare and snack. Will, was happy as could be to patrol the shore wade in the warm water and throw pebbles.

Dry Day

Waterproofing Ellie has been very successful for us over the years:  Some Press n' Seal on the CVL cap and two sheets of Tegaderm over the whole site and she is good to go.  but one of the downsides of this is that more than one day of this makes  her skin raw and sore.  So we generally try to have a dry day after every wet day to let her skin recover.

For our first Hawaiian dry day we took off for the center of the island to the Dole Pineapple plantation, a spectacular tourist trap complete with a narrow gauge train, the World's largest maze (like a hedge/corn maze but with hibiscus)  and more pineapple desserts and concoctions than you could imagine.  The kids loved it and we had a blast and we were home early enough to have a rest before Ellie got to do one of the other things on her Wish:  A luau.

The Brogans, via the sea!

And yes, Ellie was on stage more than once.

But this was all a warmup for the coming wet day when Ellie finally got the chance to swim on a coral reef.

Two-fer

Charismatic- (karizˈmatik/)
Adjective. exercising a compelling charm that inspires devotion in others.

 Megafauna- (ˈmegəˌfônə/)

noun .  the large mammals of a particular region, habitat, or geological period.

Monday was a good day.   Especially for a girl with her heart set on seeing big ocean critters.

A spectacular dolphin experience in the morning:

Followed by a Do-It-Yourself turtle safari with Abby in the afternoon:

Yes, Monday was a good day.  

For Will as well: 

The unfiltered early returns

After a quick dry run in the pool this morning, Ellie, Abby and Erin took to the sea to see some fish.  

Here is Ellie's unfiltered words fresh out of the water:

A success.  

Abby had to drag her away from the fish.

What's your Wish?

And We're Off!

Yes, after months of planning and the requisite extra fun that comes with a girl with some extra considerations, we are finally off to fulfill Ellie's Make-A-Wish wish:  to swim with sea turtles and dolphins, see a volcano and go to a luau.  

Yes, if your deduction is up and running, we are off to Hawaii!  Tomorrow morning to be exact, and we couldn't be more excited.

We have known for a long time that Ellie would likely qualify for a wish and almost a year ago we started the process to apply.  As we expected she was quickly approved and then it was just a question of timing and the coming week is just perfect.

So at 5:30 tomorrow morning the magic limo arrives and we (Abby, Ellie, Will, myself and Ellie's nurse, Erin) are off to Oahu.  Ellie is armed with a sleeve of tegaderm to waterproof her CVL, a fresh snorkel to keep up her skills from the pool this summer and a new underwater camera.  She has learned her Hawaiian fish names and even used Humuhumunukunukapuaa as a special second grade spelling word this week.

We will be sure to take lots of pictures and are going to dust off this old blog to make updates along the way. 

It should be quite an adventure.  And if we get more than we bargained for, a doctor that used be with the Children's Short Bowel Program works in Honolulu.

Updates. Good news, Great news, Not-so great news and other things

I'm not sure if it is the the facebookification of the world, the onset of the Age of Will who is now 2 1/2 ,  Ellie hitting her stride as a school-aged kid, some other factor or a combination of a bunch of these but we have been seriously negligent in updating this blog for a significant amount on time.

So I will try to update all fronts here with the latest and greatest from Broganland.

Ellie:  Ellie is great. 

A happy soon-to-be seven year old, she goes to first grade every morning on the bus with her nurse/consigliere and loves first grade. Two different rounds of show-and-tell with her class have made her medical issues more of a fascination for her classmates than a problem.  She routinely gets supplemental IV hydration mid-day without missing a beat or a call home.  She just gets off the bus some days with a IV backpack. Abby and I just look at each other and thank the Commonwealth of Massachusetts for good nursing.

Ellie's school couldn't be more accommodating.  Everyone from the Principal to the nurse and her amazing teacher are great for us and do what it takes to keep Ellie going.  Even the food service people are bending over backward to include Ellie in school lunch by sending us their recipes and preparing special meals for Ellie so she can go through the lunchline.  Just like everyone else. 

We got great advice when we were moving-our town is truly where you want to be if you have a kid, especially a kid with some issues.

Some very cool news:  Because of her diagnosis, Ellie was approved this Spring for a wish from Make-A-Wish!  We have known for a while that it was  a possibility because of her central line and other factors so I sent an inquiry thinking that it would take a while to get things rolling. Boy was I wrong! Within a week, we had a full approval and a home visit scheduled and it looks like Ellie the fish/science geek wants to go to Hawaii to see sea turtles and volcanoes.  We are looking at timing and details but it should be sometime before next winter.

Some not-so-cool news:  Ellie has been off of TPN for more than two years now, but in the past six months her growth has stalled when it should be moving steadily upward.  We have tried a lot of tricks but we are heading in on Monday to restart TPN and give her the boost that she needs.  We will be at Children's for 3-5 days next week getting her restarted and watching for a side effect of restarting TPN called Refeeding Syndrome.  As we have experienced in the past, if the shit hits the fan, it is much better to be at Children's than at home. So we will go in on Monday, hunker down with all sorts of distractions and wait between frequent blood tests until it is time to go home.  This isn't a big mental setback for Abby and me.  Ellie hasn't made progress toward life without IV bags in a long time so our feeling is to get her what she needs and add some muscle and height.  Adding TPN adds another few minutes to our day and adds one pump to our arsenal but is not a significant change for us.

In Puder We Trust. One of our chief concerns in restarting TPN was what lipid Ellie would use.  The strict Omegaven protocol prohibits kids that have been off for more than a year from restarting Omegaven.  Which meant that we would be looking at Intralipid, as Ellie's lipid source- the same stuff that whacked her liver when she was an infant.  She is older now so the effects of Intralipid would likely not be as severe as a few years ago but there is that risk and her liver is already damaged so we were anxious.  Fortunately,  if there is one person who was more concerned about this than Abby and me, it was Dr. Puder at Children's who got Ellie on Omegaven back in 2006.  He went to work and within days of the nutrition team scheduling her admission, he had persuaded the FDA to give Ellie special approval to restart regular infusions of menhaden squeezings. 

She will not spend a single night on Intralipid. 

Ellie's compromised liver and her parents both did the same little dance that Carol the nurse did when we got the news that Ellie was approved in 2006. 

Omegaven Saves Lives.  

Will:  What to say about Will?  2 1/2 and everything you would imagine from a boy that age. Trucks, boats, cars, Richard Scarry and a vocabulary that is growing exponentially along with the length of his sentences. He is wonderful and oves his sister.  The other night Ellie's baby monitor stopped working in the middle of the nightand her pump alarmed when the battery died.  Will heard this and started yelling to us,"Mom.  Dad.  Ellie pump Beeeeeeping!  Ellie Pump Beeping." 

He is awesome.

We think that he will miss Ellie when we go to Children's next week.

Here's to hoping for an uneventful visit to 300 Longwood next week.

America's Test Kitchen

Ellie is now 6 years old.   Over all she is doing well and is a very happy active little girl.  But she has been dealing with Short Bowel Syndrome and all that comes with it for 6 years.  Normal for her is not normal for most kids.  Her normal involves monthly blood draws, lots of doctors and often not feeling well.  Because of this we have started considering looking into a Make a Wish trip for her.   

Before Ellie was born I thought that Make a Wish was for dying children.  One final wish.  But I was wrong.  These kids have to deal with so much.  Much more than any child should have to deal with and on top of that, they have to grow up, make friends, go to school and learn how to live their life.   We have realized that Make a Wish is for children who are dealing with a serious medical condition and now deserve a break.  They deserve to receive something special for putting up with all their medical issues. 

So we have been considering this for Ellie but, we haven't done anything yet.  After a bad few weeks we were talking to Ellie about maybe doing something special for her.  We asked her if she could go anywhere, where would she want to go?  Ellie thought and thought and said, "America's Test Kitchen."

For a little girl who has a very limited diet and often has a belly that just doesn't feel good.  She loves to watch America's Test Kitchen, read Cook's Illustrated and cook.  She knows everyone on the show and talks about them like they are family.  So Gib sent America's Test Kitchen an email, a really good email.

Today Ellie and I toured America's Test Kitchen.

We walked in and while we were waiting for the tour to start Christopher Kimball walked by and Ellie fell out of her chair.  He walked by again and met Ellie.  I'm sorry to say that I was not on the ball and did not get a picture.  I was so taken by watching Ellie completely lose her mind.  After Chris, as Ellie calls him, left we started our tour.

We started in their cookbook library containing over 3,000 cookbooks.  Then we saw where they photograph their food for their cookbooks.  They use real food and were talking all about who was going to get the meat loaf or the chicken for lunch. Nothing I have ever cooked looked as good as those plates of food.  Then we moved on to the kitchens.  As we walked in the door Julia Collin-Davison walked out and said, "You must be Ellie!"

 Ellie has said many times that Julia was her favorite person on the show.  We were told that Chris might be there but they weren't sure about Julia.  Julia WAS there and gave Ellie a tour of all the kitchens, explained some of the tests that were being run and showed us all the equipment they use.  Ellie was in heaven.

 All the pots and pans they use for filming and photographs.  Notice how clean they are!

Test kitchen staff testing cooking grains in a pressure cooker.

 Ellie and Julia on the stage where they do all the TV shows.  

 All the plates that they put food on, they wrap all clean plates in saran wrap to keep them clean.

5 Hams waiting for tests

We had a great time and didn't want to leave.  Thank you to all the staff at America's Test Kitchen for taking time out of your day for Ellie.  Thank you especially to Shannon, Julia and Chris.  

Ellie update:

Ellie has been doing well.  We have had our ups and downs.  We had some hick ups last fall but over all she has been doing well. She is still off of TPN but still on IV hydration of D15 every night.  We stopped over night feeds early last spring due to a bad round of Small Bowel Bacterial overgrowth.  That fixed the problem but it still comes back every 1-2 months for a week or so.  We are going to restart them in the next few weeks.  

We also tried a new formula but the verdict is still out on that.  We had to go back to good old Elecare for a bit but we will try the new formula again in the next week.   

Other big news is that Ellie finally gained and grew this summer.  She has been holding steady on both for most of the past year.  It was brutal to watch her numbers go across the charts not up over the past year.  But at our last clinic visit she had a nice jump up in weight and height.    

All in all we are doing well. 

Can't forget Will:  

Happy Summer!!

A little bit of Congressional Help, Please

We have been getting scary news (and here)from around the world of TPN over the last few weeks: some of the important ingredients in TPN and the supplies that go with it are in critically short supply, as in there is NONE to be found anywhere. This isn't such a big deal for Ellie right now since she is still on just D10 overnight, but if she should need to go back on TPN, there are key elements that would need to be rationed or omitted from her mixture like calcium, magnesium and other trace elements that typical people get through their complete diets.

The cause of this is uncertain but two primary reasons have been suggested: one, that there aren't enough raw materials available to make the additives and two, that these very important drugs just aren't profitable for the drug companies and they have made a cold business decision to stop making the drugs or have slowed the production down.

If they were making widgets or car parts, this would be one thing, but we are talking about really important things that will have real consequences for thousands of people around the country including typical people who are in the hospital, need antibiotics or chemotherapy.

So what is there to do about this other than worry and hope?

One simple thing that we can all do is let our representatives in Washington know about the issue and the very real consequences of letting this problem continue. There are things that even a gridlocked Congress can do to urge and nudge the FDA and the drug companies to fix this problem but Congress won't act unless they hear from their constituents, who vote and are vocal.

A simple letter to your member of Congress and Senators still has power. Find them here, and write them a simple letter telling them who you are, how you or your child are or will be affected by drug shortages and ask for their help. These people have connections and do solve problems for voters every day. Ask them to help and also ask them to consider sponsoring the bills that have been introduced to increase oversight over this emerging problem. Also, if your Congressman is on this list or or Senator is on this list, they sit on an important committee or subcommittee and could be even more influential in getting answers and solving this mess.

For all of the kids and adults with short bowel and the range of other conditions that require the more than 150 medications that are currently in short supply, we thank you for your help.

Ellie got the go-ahead this week to go off of TPN.

Again.

Yes, starting with next week’s shipment, she will only be getting fluids without nutritional additives and without Omegaven.

ONE MORE TIME!!!(click on me) listen while you read: NO MORE TPN!!!

Two and a half years ago she went off of TPN for the first time. She was growing enough in both in height and weight for her team to think she could stop her TPN(total parenteral nutrition, IV nutrition). She was off of TPN for 6 months that time.

Ellie's 5th Birthday Party (she covers her ears when people sing)

Before her surgery to lose her ostomy, we put her back on to get her ready and fatten her up for surgery and keep her weight up during the recovery time while her reconnected bowel learned how to behave. That strategy worked and she did very well and for the next 2 plus years. But, she has done well....ON TPN.

The problem is that TPN even with Omegaven, is hard on her body. We trust that her liver is protected but we never know for sure. Because of this we have been trying and trying for 2 years to get her off again. We have been trying different things on and off to wean her from the calories and fats that come with TPN. She has been growing on and off for over 2 years. But not enough and not consistently. Which sucked. She would put on weight and then a round of bacterial overgrowth would kick her back down to more days of TPN.

This Wednesday we went in for clinic with Dr. Lo (the TPN guru at Children’s) and Ellie had gained enough weight and grown enough for the team to feel that it was time for her to come offof IV nutrition again. She will still be on IV hydration 7 nights a week, but no IV nutrition.

Those of you that have met Ellie have seen how she runs from place to place, swims all summer, dances from room to room and lives life on her toes. The heat of summer is not the time to decrease her IV hydration.

So we are now in a two month trial period away from the complex land of TPN. As with all things short gut we understand that TPN may come back to Ellie if she needs it, but for now this significant step is great and makes us think about the terrifying possibility of a night without the whirr of an IV pump coming through the monitor.

We have a busy summer planned for Ellie. Complete with a Lego camp with 100,000 Legos. We have discovered that soccer is probably not a good option for Ellie. We signed Ellie up for soccer every Saturday afternoon. And after 2 sessions we agreed that soccer is not the sport for Ellie-Here we work all day everyday to get her to gain weight and then we sign her up for something that is just one giant calorie burn. We would watch her run around for over an hour struggling to keep up with the other kids, and then it occurred to us...

WHAT WERE WE THINKING?????

So no more soccer.

For now Ellie will continue to dance ballet, go to school (Kindergarten starts in September), take swimming lessons (at the Y!) and be a very typical 5 year old. And well, I am pretty happy about that. In the future, I think that she will rock T-ball as a left field daisy picker.

About her brother Will: He is an eating machine! He is eating, growing in to a tank of a baby. I was watching him chew the other day and realized that what I was watching took us 6+ months of feeding therapy with Ellie. It was a thing of beauty. I was mesmerized:

He has also decided that crawling is for sissies. I honestly think he started pulling himself to standing and cruising because we told him that Ellie started cruising at 7 months. A Month behind his sister Will started pulling himself up to standing and walking. Bring on the crash helmet.

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.

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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.