Delivery Day

Today marked an unusual milestone for us which might not mean all that much to those folks who don't live by the timing of their weekly infusion delivery. Today we got the (hopefully) last weekly shipment of TPN.

Usually we get two big cardboard boxes delivered each Tuesday morning by our friendly UPS man. On good weeks the delivery comes ahead of the garbage man so we can recycle the boxes that day. In these boxes are all of the things that we need to do Ellie's TPN for the week and a few extras in case we get fat thumbs while setting up her infusion and contaminate an extra set of tubes. Eight bags of fluids (one for each night plus an extra), tubing for TPN and omegaven for the week, syringes etc. All told there are about 150 items in each shipment that need to be opened, checked and stored to make sure that we have what we need. It all goes into one min fridge and big cabinet that Karlene keeps neat and orderly for us which makes the evening set-up much easier:

This made me think of the change that is about to happen as we experiment with life after TPN and how much easier this will be after December 1 and also how much easier it will be to travel with Ellie without TPN.

To give you an idea of what it takes to go on the road, here is the list of things that we need to bring for just an overnight, 80 different items by my count and close to 250 individual items to bring (but I could be missing something). All of the bold things won't be necessary when the TPN turns into hydration.

Zevex Infinity formula Pump and backpack
Charger for Pump
Elecare (1 can for every two days)
Mixing bottles (x3)
Sippy Cups
Zevex Bags (a new bag for each day)
Mic-Key extensions (that connect her pump tube to her g-tube button)
Safety Pins (to pine her tubes to her clothes to keep them from pulling)
Silk Tape (the duct tape of the short gut world)
60 ml syringes for flushing Mic-Key extensions
Small oral syringes for meds
Replacement G-tube kit
Clean Dressings for her g-tube
Bacitracin Ointment (bacteria)
Steroid Cream (swellling)
Nystatin Antifungal cream (fungus)
Q-tips
Lubricant (for reinserting an old g-tube or inserting a new one)
Ostomy bags( 1 for each day)
Ostomy bases(1 for each day)
Ostomy wipes
Cotton balls (lots and lots to absorb the fluid from her ostomy)
Rice Bag for warming ostomy bases and making them pliable
Stoma Powder
No-sting barrier wipes
15-20 wash clothes for bag changes
Curved scissors for cutting holes in ostomy bases
Emesis basins
TPN Pump (CADD Prism)
TPN backpack
TPN bags ( one for each night plus and extra in case*)
Infusion vitamins

10 cc syringes to add vitamins to TPN
IV Zantac
3 cc syringes to add Zantac to IV bag
Portable sharps container
D10 bags
9-volt batteries
Rechargeable battery pack
Omegaven Pump (Curlin)

Omegaven Backpack

Omegaven Pole clamp

Bottles of Omegaven (one for each night plus an extra in case*)

Portable IV Pole

C-cell batteries
TPN Tubing
Omegaven Tubing

Vented Spike adapters (the IV bottle won’t drain unless it is vented. Almost learned this the hard way)

Omegaven Hangers

Y-extension sets (to let two fluids go into one central line)
Positive Pressure Caps
Line covers
Saline Flushes
Heparin Flushes
Alcohol wipes
Hydration Fluid Bags
Hydration tubing
Hydration Pump (CADD Prism)
Central line dressing kits, 2 per week, 1 for scheduled changes and 1 unplanned.
Sorba View dressings for her central line site
Sterile gloves in medium and large
4x4 Tegaderm sheets
Sterile Gauze
IV Clamps
Absorbent Chux Pads ( contraband hospital pee pads)
Replacement bed linens (sometimes even an overnight doesn’t do the trick)
Tylenol
Thermometer
Scissors
Tweezers
Sterile water
Diapers
Overnight diaper for night time (a regular diaper just won’t hold a pound of fluid like an overnight will)
Diaper wipes
Diaper scale (travel version, the hospital grade version stays home)
Glycerin Suppositories
Cutting Board for the clean space for TPN set up
Sanitizing Wipes
“Green Book” (our lime green binder of all of Ellie's daily logs, monthly summaries, lab reports, etc)
Medical history USB Drive
Baby Monitor

YEah, that's about it, I guess. Amazingly we can pack for a week away in about 45 minutes if we need to and somehow manage to get all of this stuff into a car that is smaller than a moving van. In fact since the Pack n’ Play went away a few months ago, we even have extra room in our normal sized car. But we pack knowing that even a little thing from this list will either cause us real problems or even make us come home prematurely. If the tubes don't work, the food doesn't flow and so on. we double and triple check everything...

A good thing is that coming home is always easier than going since most of these things are used and tossed when we are away. But don’t get me started on the amount of waste we make, at least we don’t need a separate garbage bin like some other short bowel families.

Next week's shipment of boxes will be an odd thing for us. Let's hope that the changes go over well with the only person in the room who doesn't really know what is in them. She prefers to hide in the boxes and make snowmen from the styrofoam that insulates the fluids.

Hokey Pokey Ellie

The skirt just keeps on playing that silly silly song!



We think that Ellie might just have it to be Miss America, 2025. But there is no time like the present to get her into training.

Her talent: Dance of course!

Oh and in case you have forgotten, Only 3 more nights of TPN!

NO MORE TPN!!!!

Well, in the the immortal words of the late great Jerry Reed, it looks like 'We're gonna do what they say can't be done....'

Yes, as of December 1st 2008, Ellie Brogan is going be OFF OF TPN!!!

(It is okay at this point to stop reading, stand up, and do a little happy No TPN dance. It's like the poop dance, just better.)

Ellie had her first clinic appointment in two months yesterday at Children's. Even though we were anxious about her weight, after a round of nasty colds and mystery ostomy dumps over the last few weeks, she still managed to gain 500 grams (about 1.1 pounds) and grew an inch and half.

'Good growing, kid.'

So after a the usual round of unconventional horse trading, bargaining and negotiation with the doctors pushing to be aggressive and me pushing to be conservative, we worked it out that as of December 1, our weekly shipment of TPN would no longer include TPN and that she would move on to seven nights a week of D10, (10% dextrose solution) to keep her hydrated and give her a tiny amount of sugar. I insisted that we start this after next week's holiday. I just didn't want to run the risk of anything going wrong and then spending the holiday weekend at "The Inn" with a skeleton crew of doctors and nurses piecing things together. No thank you!

This is huge news for a kid with a high ostomy and a small personal victory for us. The doctors that were pushing for these big changes yesterday were the same doctors that we fought with in 2006 because they disagreed on the use of an ostomy to resolve Ellie dilated bowel. They firmly believed that this approach could not work, that she would have constant issues with hydration, mineral balance and skin breakdown. They essentially told us that she wouldn't grow or thrive with an ostomy. And would never come off of TPN.

Have you seen our girl lately?

All of this is nerve wracking for Gib and me. We have seen lots of kids go off TPN and then need it again and had their parents be crushed by this step backwards. We are going into this as another experiment with Ellie. We know that it may not work and she may lose weight and need TPN again. We are cool with that. Really.

We won't be devastated if she needs TPN again. It really won't change our daily routine all that much- we are just down to one IV pump a night instead of the two that we have on TPN nights now. This is all easy to say right now but we'll see we feel if it happens. We also know that when we do reconnect her bowels, she will probably need to go back on TPN while her intestines learn to work together.

Ellie playing with cellebratory ballons from Aunt Kate

One thing happened yesterday as we were leaving clinic that really helped this whole thing sink in. Usual clinic appointments include a visit from Kathy Gura, the pharmacist and Omegaven guru, she will check in on our girl and drop off a large bag full of omegaven for the month ahead. Yesterday she dropped it off as usual and then after the changes to Ellie's TPN were decided, a nurse came back in and grabbed the bag of omegaven saying, "you won't be needing this, I guess."

We are going to have one of our clinical calls with our nutrition team at NutriThrive next week to talk about vitamins, minerals, fats and all the fun stuff that we have to worry about now that she is off of TPN. And now we also need to start looking into fish oil suppliments, because no more TPN also means no more white gold, Omegaven.

All in all we are very excited, and can't believe this is finally happening. But, to keep ourselves grounded we would like to invite you all in embrace a phrase with us:

"She is off of TPN, for now."

YAHOO!!!!!!

What do you say..

A friend sent me this email and I thought I would reply on the blog since we have heard the same question from many, many other parents.

Hi,

I am having a difficult time explaining about my son to people that I don't know. How do you explain when Ellie isn't doing things at the right times, meeting milestones and the obvious stuff like the backpack? I feel like I just don't know what to say to people that I may see again or people that I will never see again. It would be great to have a one liner that explained everything and I know I should have try to keep a positive spin on what I say, but I just don't know what to say. I wondered if you've encountered anything along these lines and might have any suggestions. It's probably a person decision from parent to parent based on their comfort level and such.

Yes, we did and do run into this almost everyday when People see Ellie's backpack. They say, "oh how cute! What do you have in your backpack little girl?" I have always wanted to reply, "Elemental formula, a pump and some scary tubes."

But no, most of the time I smile and explain that actually it contains a feeding pump. And then I explain, with a smile on my face, that she was born missing some of her small intestines. (ok so it was closer to 90%, but people seem to handle "some" better) I also explain that she will need this pump for the next few years and eventually grow out of it. (we hope this is the case, I guess that would be the positive spin you mentioned.)

Some people react really well and seem genuinely interested and will ask good questions. Others end the conversation kinda quickly and move on. I have tried, and am still trying, to learn to not care about the people that get scared. Mostly I try to center the conversation on how well she is doing considering everything she has going on. It does seem that my positive attitude about the whole thing does rub off on people.

I am still surprised by the number of people that when I start explaining what the backpack is, even to complete strangers, how many of those people know other kids and adults that have been on feeding tubes or know some one with an ostomy. The most surprising thing is how many people have been on TPN, on feeds or have an ostomy themselves. This is a whole world that no one talks about until you are living it, but it still shocks me how many people there are out there that have been through some part of what Ellie is going through. The backpack has also been our ticket to the secret club, people see it and know what it is and will chase us down to talk about the pump or commiserate as to what we have to go through. This still throws me off.

Now, that all being said... I do have to admit that sometimes I just hope and pray that I can get in and out of places without people noticing her tubes. And sometimes when people ask what is in the backpack I just smile and say "toys" and keep walking. I don't always have the energy to be upbeat and happy about all of Ellie's stuff. I usually also never mention the Central IV line or the ostomy to strangers that ask about the backpack, I think that would just be too much.

But I do take a completely different approach when we go somewhere where we will be going many times, like play groups or classes. If I get there early I will introduce my self to each parent and then somewhat quickly segue to explaining all Ellie's stuff. If I get there later and there are a bunch of people there already, I pick Ellie up and introduce her and her backpack and everything to the parents.(might as well shock them all at once) I do this because I want everything to be out in the open, so people will not feel like it is the elephant standing in the corner that they can't talk about. Also, because I want people to know her and once they get to know her, they see past the backpack and the bulge in her shirt from the ostomy.

I also don't want these parents to be afraid of Ellie, afraid of their kids hurting Ellie while playing with her, or have any fear pass on to their kids so that they are afraid playing with her. She is pretty tough, she plays just like any other kid and we secure her tubes so that even if they get tugged everything will be ok. I know she is different, but I want to keep that from her for as long as possible. After a few visits people don't react when her pump beeps or think that it is weird that I need to move to a private room when I empty her ostomy bag. (I still think the contents of her ostomy bag might be too much for outsiders)

Meal times and swimming are still the times when she sticks out as different, but hopefully that will get better with time.

As far as having one sentence that explains it all, good luck, let me know when you figure that out. I will say that it has helped a ton to have this blog. I can just tell people to read the blog and then they catch up on their own and I also don't have to field many questions like....."Oh you must go to the beach a ton, having it right down the street!!" (it is a beach on a lake, and Ellie can not swim in freshwater, not until she looses all extra holes, including the G-tube, so it may be years)

Actually that is one of the main reasons we started the blog, we just couldn't handle telling her story over and over and over and over..... It has also given me a place to vent and to learn to accept everything that is happening with Ellie. Strange as it sounds, it is true.

Ellie taking her dad on a BOAT RIDE!!

Don't Forget to VOTE!!

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Happy Halloween!!

Ellie was very clear this year abut what she wanted to be for Halloween- 'Stellaluna, the fruit bat!' from the great kids book. Incidently this book was a gift from our friend Kelly that used to take care of the bats at Mystic Aquarium. Ellie made her choice of costume when we first discussed Halloween in September and never changed. Abby got to work making a costume and this week we had her debut at the firehouse party, followed by a second act at the town hall with every other 2,3,4,and 5-year old in town:
and the the a final go 'round as a fruit bat last night.


What can I say, our town really likes Halloween and fortunately Ellie's costume was very easy to put on and wear without lots of trouble.

Ellie still doesn't like sweet things except for lollipops so trick or treating was easy for us and we didn't have much of a fight about not eating the candy. She liked ringing doorbells more than the candy. We are pretty sure that this was our last year of distracting her from the mountains of candy that are out there so we will have to work on some strategy for next year to keep her pumpkin empty or filled with things that are good for her and her bowel:A slug of candy could throw her bowel for a loop for a good long while, something that isn't quite worth the trouble. sounds easy but try convincing a 3-year old about this......



Taking her out to walk at night was a new thing for us as well and she did remarkably well with all of the things that were going on around her.

The closest thing we had to a meltdown came after we got home and tried to piece our evening routine together a few hours late.

Overdue Update

We have to apologize for not updating more often but with things moving so smoothly we are just having too much fun to sit down and write about our daily activities. More importantly Ellie has been remarkably stable in the past few weeks and when things are good it makes it seem like there isn't much point in telling everyone that things are good. One email from another parent about their troubles with an SBS toddler reminded us very quickly about how things can go in the toilet just like that so I will take this opportunity to deliver a rave report about the amazing Gut Girl.

With TPN pared down to just 3 nights a week she has just now started to gain weight and grow again. We would have been happy if she held her weight after losing the night of TPN a month ago but her weight is actually up to 14.4 kg and almost 37 inches this week. This places her squarely in the 75th percentile for both height and weight for 30 month old girls. This points to the likelihood that some of that food that goes down the hatch is finally being absorbed- a very good sign since increased absorption is the goal for her. She keeps shoveling meat and noodles and vegetables and when it doesn't come out whole or in a recognizable form, we know that we are on the right track.

We have even dialed down her elecare intake each day to 1000 ml to encourage Ellie to eat solid food instead of drinking liters of formula. To think that just a little while ago we were doing everything we could to get each last ml into her and now we are limiting her formula! Solid food is another goal so this weight gain is a great sign for us.

Ellie had her 30 month evaluation for Early Intervention this past week. It was quite casual for all of us and Ellie did the evaluation tests like they were new fun games. In the end she maxed out all of the tests that were applicable to her and tests at 35 months for everything except things like toilet training (1500 ml of TPN every night sort of shoots that one down), and feeding (we know that she is sort of slow on the feeding skills). Not to brag but I'd like to send this report to the doctor who told us that she would be delayed. Delayed my ass.

The upside of this is Ellie likely won't need a lot of Special Ed services (like PT, OT, or speech) when she goes to preschool. The downside of this is that since she won't need services, the town won't pay for her preschool. I'll pay that tab happily if it means that she has beaten the odds for hospital kids. We are going to make sure that her medical disability is well documented because we can't predict what is coming next week let alone next year and want to make sure that accommodations will be made if, for some reason, she misses school or if the reconnection surgery changes her situation like it possibly could.

We will start the process of transition to the school department in the coming months and hope that things go as well as we have been told to expect from our local schools. When I spoke to the early childhood coordinator a few weeks ago about Ellie, it was funny when she told me 'we've had kids with nurses, kids with ostomies, and kids with feeding tubes.'

'Ever had one with all three and a central line?'

'Um, I can't say that we have.'

'Yeah she's got the trifecta. But you wouldn't know it unless you opened the hood.'

She was stunned. I guess most parents don't talk about their kids like racehorses or hot rods.

So onward and upward.

Still no word on scheduling the coveted manometry study. that will hopefully give us the green light to reconnect her. There is only one GI doctor at Children's who does the study and he is a very busy guy.

Also no word from the geneticist about Ellie's tests. Apparently there are some studies that link Ellie's condition to a genetic trait and before we sign up for another little one, we want to make sure that we aren't playing against a stacked deck.

I am off to Washington for a long overdue visit to the Oceana mother ship. Let's hope that this trip is easier than the last time I flew away for work.

The backpack is back.

As it is with all good experiments, sometimes you don't get the results you want. It seems that Ellie needs more time on the enteral backpack than we hoped. About three weeks ago Ellie started showing signs of low hydration around 4 pm everyday. All of her other numbers looked good, but she was still looking dry. So, we added 2 more hours of pump time to each day; on at 8pm and off 10am. As of last week she has also stopped gaining weight too. Then this past Wednesday we decided we needed to add two more hours back on. So now she goes on the enteral pump at 8pm and off at 12 noon.

Now remember at this time we are also running the experiment of one less night of TPN (we are at 3 nights of TPN and 4 of hydration). I know, I know we increased the variables so now we don't know if her lack of weigh gain is due increased time off of the enteral pump of if it is due to less TPN. We are really hoping it is the enteral pump. We are willing to add as many hours of enteral pump time as possible if it means we don't have to add the night of TPN back on.

We haven't been to clinic in almost a month and we are a bit nervous about how she is doing. Fortunately we have had the clinical reviews with NutriThrive. About every two weeks we have a conference call with her nutritionist, clinical nurse and pharmacist to review her progress and see if we need to change anything. This is great, because I really need/want the peace of mind that I get from knowing that some one else is watching her just as closely as we are. During our last call we reviewed her status and they were also concerned about her lack of weight gain. Upping the hours on the enteral pump seemed like the next step and they gave us some great ideas for new food options.

So, the backpack is back. She still has free time from 12-8pm everyday and luckily the backpack doesn't slow her down. And other than all that she is doing pretty well. We keep wondering how she is handling the reduced TPN and really blood work is going to be the best indicator so we are going to schedule a clinic for sooner rather than later. The trace elements and fats are what is concerning me the most now. We will see...

Just a quick shout out some of our friends.
Carter: Stay on that cannula, you and your parents have been through enough in the past week. We have been watching your ups and downs are so excited to see things are getting better. You are one amazing kid.

Eithene: We are thinking of you and hoping that they find a solution that will work for now and for the long term. I'm so sorry for everything you have had to go through just to get a port! We are thinking thoughts of tough veins and quick recoveries.

Ryan: Congrats on your first night off of TPN!! YAHOO!!!!! We are so excited and are keeping our fingers crossed for you and your folks!

Pants

As it gets colder this fall we are really appreciating Ellie wearing pants with a waist. A little thing for most people but when she was smaller pants weren't an option since they would go right over her ostomy and make the bag fall off. Overalls or a dress with Baby Legs were good for a while but were tight on her legs and would leave rings when she wore them last year.

This year she is big enough that the ostomy bag rides under the waistband and doesn't dislodge anything. A big change for us and a new world for Ellie, who thinks that dropping trow in the living room is great fun...


Fortunately her grandmother has been supporting this new bit of fun and Ellie has all sorts of fashinable styles for this fall. There is always a onesie underneath to pin her central line and keep her from playing with her plumbing but pant make life a lot easier and may even lead us towards the potty again after a nine monnth layoff.

Feeding Gut Girl

What happens when you ask Ellie to smile and open her eyes at the same time....

Our ongoing challenge is to move Ellie forward towards eating like a typical two year old and getting away from the IV and tube feeds as much as we can. Tube feeds have their purpose but they aren't our goal for Ellie so we keep looking forward toward real food for her. To get from point A to an oral feeding kid takes a bit of work.

We have heard from other parents asking about what she eats and how so here is the rundown of the day for Ellie's feeding. All of this should come with the caveat that we 'cheat' a bit because of her ostomy- if we feed her something that gives her gas, or diarrhea, its effect is minimized because the ostomy is a great escape valve for both of these things. This will all change significantly when she is reconnected and has to deal with gas and other side effects of a varied diet.

Her feeding day is quite regimented and begins with her coming off the evening IV around 8 in the morning. The overnight combination of IV and g-tube feeding at 40 ml/keep her happy overnight and she still sleeps between 10 and 12 hours each night. Who says tube feeds are all bad?

As soon as she comes off the IV we give her a glycerin suppository to help her poop which usually works like magic. We'd like her to poop on her own but it just doesn't happen so most mornings we 'clear the decks' as soon as we can and open up room for more food. If we don't do this, we can be sure that her ostomy output will be increased for the day- if there isnt anywhere for the food to go it goes out the ostomy along with a lot of fluid. Remarkably her poops have good consistency, something that isn't all that common for kids with short bowel.

Poop Dance!!
(pay no attention to the mess, Abby has her knee 'scoped on Monday and we are still in recovery mode)

With a path cleared for food we wait and by 9 or 9:30 her blood sugar has come down from the IV to make her hungry for breakfast. This hunger drive makes it a lot easier to feed her and keeps meals fun instead of a battle. Breakfast is usually some kind of eggs (scrambled, hard-boiled, or fried and mostly whites) a slice or two of avocado and maybe a slice of ham along with her first cup of elecare for the day, about 100ml (3+ ounces). We don't tell her how foul it is and she keeps guzzling it, a fair arrangement.

Around 10 she comes off her G-tube pump. Since midnight she will have gotten about 3-400 ml of elecare through the tube which brings her to 4-500 ml for the day so far. Now we have a free kid until bed time.

'Ellie free. No milk bag!'

At 11 she gets another 100 ml of elecare. 5-600 for the day.

between 12 and 1 it is time for lunch. Lunch means some cold chicken, ham or turkey, some cooked vegetables like peas, broccoli, green beans, or asparagus and some plain noodles or noodles cooked in broth. She also likes soup and will drink broth, which the nutritionists tell us is great for her. And another 100 ml of elecare. 6-700 for the day.

Mmm. Double fisted Broth.

Nap time runs from 2 or so until 4 and ends with a second round of glycerin. Another poop around 4 and we are ready for a snack or third meal of the day along with another dose of elecare. 7-800 ml.

Dinner is a repeat of lunch. Meat, noodles, vegetables, and a final round of elecare that brings us up to around 900 ml for the day. Usually she a version of whatever we are eating, but if it isn't an Ellie friendly meal, then she eats her own food. And these days a bit or dessert- a couple of berries or a shared all fruit popsicle.

What's you name little girl?

Then it is off to do the TPN routine, hook Ellie up to her g-tube pump and TPN by 8. After a round of stories she is usually asleep by 8:30.

Then we reset pumps at midnight, add up totals for the day and other than changing a very wet diaper at 4 AM because of the huge volume of TPN that it our day until we start it all over again tomorrow.

All hooked up and ready for bed

All told she will have had around 1100 ml of elecare, three real sit down meals, one heavy snack and two poops, with the associated poop dances to celebrate each one:

This schedule seems like it might take the fun out of the day but really we manage to have lots of fun in between all of these pit stops and the periodic feedings, dresisng and ostomy changes don't really get in the way of things. She is so good at being the patient these days that they are remarkably quick and we get on out way to having fun:

Looking Behind the Curtain

This past week I (Abby) got a chance to go to the Pediatric Intestinal Failure and Rehabilitation Symposium in Pittsburgh. It was a three day event, the first day is geared toward parents and caregivers, and professionals in the field. The second two days are geared toward professionals but, parents were welcome to attend.

The first day was nice with some good information and it was fun to see the parents that I had talked to on line but never met. Also good to get a chance to meet their kids that I had heard so much about. I arrived on the first day I ran into almost all of the staff from our short bowel clinic and a bunch of nurses and NPs from our floor at Boston Children's. The second day was very interesting in that almost every short bowel GI or surgeon I have ever heard of, both here and abroad, were in attendance. Some of the talks were really good, Dr. Puder gave a great presentation about Omegaven. This week the 100th kid will go on Omegaven.

Really, the second and third days were an opportunity to pull back the curtain on the wizard and see how doctors really are at an event like this. The whole event was very interesting and since the majority of the attendees were professionals most of the presentations were geared to professionals. I thought this would be my opportunity to hear some really mind blowing new ideas and ground breaking presentations, but no. I felt like there was some good info but I also felt that most of the speakers were afraid to propose any ideas or techniques that were "out of the box" for fear that another professional might disagree with them. I felt like almost every professional was dancing on egg shells during their talks.

This may have been because some of the doctors that were in attendance arrived at the event with an idea of how they felt about, Omegaven, Bacterial overgrowth, and feeding techniques, etc.. and no matter how good the other doctors presentations were, they were not going to change their minds. It was almost like they were able to filter out anything that didn't agree with their perspective. That was very frustrating to see and a bit disappointing.

All this being said, there were some great presentations and I met some great parents and met some great doctors. I even met some great short bowel doctors from international hospitals like Paris. After having met these people, I would have no problem traveling abroad to certain countries with Ellie.

Interesting things I did learn:

1. In Paris they rarely feed by tube feedings. They follow the natural physiology of the human body and feed by mouth as soon and as often as possible. This is also the reason that they don't like to do tube feedings at night.
   
2. We need to get Ellie involved in her care ASAP. We need to tell her all about her CVL, pumps, feeds and all of her supplies and explain what she can and can not do with them. This comes from a nurse that specializes in the transition from pediatric to adult care that told horror stories of 3 year old waking up in the middle of the night and unhooking TPN/enteral feeds, turning off pumps, finding syringes and accessing their own line in the middle of the night (that last one made me want to barf) and a few other things that made my me want to run out of the room and call Gib immediately.
   
3. There are many, many different types of motility drugs out there that all target different parts of the digestive system.
   
4. I got an education in line care after meeting a 15 year old boy who is on TPN and doesn't dress his line, swims in lakes, ocean, pools and rarely changes his cap. He is healthy and has had his line for over two years now. It made me flinch when he showed me his undressed line during lunch, but I guess it just goes to show that there are many different ways to skin a cat.
   
5. That some GI docs don't treat bacterial over growth mainly because there still isn't a truly good way to test for it.
   
6. The last thing I learned is that there are some very smart surgeons/GI docs out there. I we weren't so amazed and happy with our current suregon and team we would feel lucky to have them take a look at Ellie. And then there are others... that I would purposely drive past their hospital in favor of another.
   

All in all I'm glad I went and will probably go to the next one 2 years from now. It would have been nice if all the doctors that were in attendance were a bit more open minded, but.. what are you going to do. I think Gib is actually surprised that I didn't see that coming.

Our version of Penicillin?

Friday morning we had what now seems like a fortunate error (at the time, it was an 'oh, shit!' moment)

Ellie has been on an enteral (formula) pump for about 20 hours a day for the last six months, working her way up to getting about 40 cc's (about 1 1/3 ounces) of formula every hour. This is a big step for the girl that used to tolerate only 1-2 cc's an hour. Each night we have taken her off the pump from 4 to 8 to give her a break from the backpack, give us some time with a 'free' girl, and also try and build her appetite for dinner each night. It worked well and the trickle of food was good for her bowel.

At our clinic appointment on Thursday the doctors suggested a slow transition to more time off the pump and more solid foods to complement her formula total but suggested that we stop increasing her pumped formula because tube feeds isn't the end goal for her.


In the confusion of a clinic visit on Thursday we forgot to plug Ellie's pump in and at breakfast on Friday the battery died. This forced us to give her Elecare by mouth for a few hours that morning while the pump charged. At noon when it was time hook her back up to the pump, we noticed a very interesting thing: her ostomy was behaving very well for us and she had a great appetite for lunch, which is usually a hit or miss meal. Since we were on pace to get a respectable daily total of formula into her for the day, we made the decision to stick with this approach for the day and see where it went. In the past big amounts of formula have flushed through her so fast that they weren't worth anything, but this time around, for some reason they seemed to be working.

The end results were great. She took all of her formula for the day, had a good ostomy numbers for the day and ate four good meals (we have added a 4th meal between lunch and dinner).we hooked up her enteral pump at 8 with her TPN for the overnight infusion and we had a breakthrough on our hands- a orally feeding girl who only had the pump at night!

I guess good things happen by accident sometimes.

Since Friday went so well we gave it a shot on Saturday as we went to zoo in Boston. We simply gave her cups of formula every few hours instead of letting the pump work.

Ellie loves sippy cups and likes them even more with straws. She gulped her stinky Elecare and we were in business.



This change was more dramatic for Abby and I than Ellie. It is a strange thing that is hard to describe but something that we both noticed during our trip- we suddenly had a kid who looked 'typical'-without tubes. We have gone from having a kid that used to get comments like, 'Oh my. That child is so sick that she needs tubes!' (no kidding) and now we don't have anything more than a lump for her ostomy.


It was/is sort of like losing your Sick Kid Club membership. With Ellie wearing abackpack with the scary white tube it automatically made Ellie one of the 'sickest' kids in the place which made is sorts of kindred spirits with any parent that had kids that were 'atypical'. All of a sudden we had a bubbly little girl running through the zoo. For the first time in a long time, at first glance, she had absolutely nothing in common with other kids with medical issues.

At one point, we emptied an ostomy out in the open (as we did in the Boston Public Garden this summer) because there wasn't a bathroom nearby as we normally do. Abby and I agreed that a little part of us wanted to get our membership back.

We kept going with this approach through the day today to see if we were seeing a new trend or a fluke but things are still moving in the right direction. Some things have popped up that we have noticed but overall it is a great change.

For example, Ellie gets truly hungry for the first time in her life now and we can see her energy level decrease when she needs formula and increase with a fresh dose of Elecare. we have also noticed an increase in her appetite through the day which is great since solid food seems to do great things for her bowel and poops.

And a two hump camel makes a two-hump poop....

Her dinner was so good tonight that we all shared a Popsicle:

So much for the dislike of sweet things. I guess this is what it is like to have a typical two year old, huh?

So onward we go with the experiment. We know that this may be a passing thing and it could all go away tomorrow but for now this progress is great.

I'll have to forget things more often. Just nothing to do with her TPN or Central Line. Those are still terrifying enough to keep us vigilant and on script. No ad libbing with sterile techniques.....

Children's Check-in

Ellie had another clinic appointment at Children’s today. It was another smashing success for her and for us.

She continues to grow in length (36.5 inches) and weight (13.8 kg/30.25 pounds) and although her weight wasn’t above 14 kg like we had seen at home in recent weeks, it was later in the day than we normally weigh her.

They are happy with her progress (duh, just look at her) but are very happy with her numbers. If you add up the calories she gets from her Elecare and the calories she gets from her solid food, it takes her well past the 50/50 mark between food and TPN.

Things are so good that we have peeled away another night of TPN from her schedule down the three nights each week with four nights of hydration (just a dilute sugar/salt solution).

That’s right, she is off TPN more than she is on it now! Amazing considering she still has an ostomy and we had been waiting to get her reconnected before progressing very far toward weaning.

We will continue to ramp up her food intake as much as we can while keeping her Elecare about the same for now, about 1200 ml every day. Since she eats well for us, the thought is to peel away formula and make her hungrier for real food, which is better for her both short term and long-term.

And on top of that they don't want to see her at clinic again for three months. Christmas time.

Yes, we have more months on the pumps. But we are in a solid routine for TPN and have lived with it since Ellie came home. Besides, we would miss our morning quality time waiting for the pumps to beep:

The highlight of the day was Ellie seeing her surgeon, Dr. Jennings from across the room, running to him and giving him a high five like she had seen him yesterday.

The hospital is still a fun adventure for her. Bringing Karlene along for clinic makes it a lot more fun.

We also got our orders from one of the Short Bowel team about how to handle Ellie and her new cousin Jack who arrived from Ethiopia earlier this month. Since gut bugs are a big deal for us, we wanted to make sure that she was safe to go near him and that he was screened for all of the necessary bugs before we went to Mainee the next time. As it turns out one of the short bowel GI docs is also an African gut bug doctor for a few weeks every year. Her told us that as long as the stool samples were clear and the blood tests for hepatitis were clear (gotta protect that liver) we are good to unite the family. So we wait for the third stool sample to come back as clean as the first two and then we start making plans to introduce Ellie to Baby Jack.

Woohoo!

That means another shot at a boat ride for Ellie:

And her very own algae lab:

Finally, Ellie wants to say happy birthday to her Gramma:

The Hair

Just for the record.
This crazy curly hair

Is this long.

Oh yeah, in the first picture.. she is doing her own charting. Ya know, it gets to a point where you just gotta put these kids to work.


Side note: Jack and his mom arrive TOMORROW from Africa!!!!!!! Or as Ellie says, "baby Jack coming HOME!"

One, Two, Three, Four, Five

Ellie has been learning to count in the last few months, usually regaling us with 'one, two, eight!'.

In the past week she has moved on and now gets to about seven most of the time, and for some reason skips her favorite number EIGHT!

Last night she decided to pair her new counting with a her version of hide and go seek. She brought her laundry hamper into the living room and proceeded to count her way to hiding:

Going, Going......

You can't see me, can you?

Here I am!

This is all great fun and will be until she really decides to hide on us. She is a pretty quiet girl when she is playing by herself so we need to work on getting her to answer to us when we ask.

20 Calories per Ounce

This is the calorie level that we were at in May of last year. We then increased to 25 cal then a few months later we went to 30 cal. She seemed to tolerate the 25 cal, but never really did that well at 30 cal. We had about 4 months of high output that we tried to blame on everything else. But then this February we decreased back to 25 cal and she started to do better. This takes us to this May, and our hospitalization for a GI bug. We were discharged on 20 cal with instructions to increase the calories over the next few weeks.

We never did increase. Ellie’s output was lower than we had seen in half a year, her urine production went way up, we were able to double the rate on her enteral pump and she started to have a real interest in more solid food. We knew she was doing well but we were sure we were going to get our hands slapped at clinic for not increasing.

But, they were so excited by her progress they said don’t change a thing. Our nutritionist said that because Ellie is getting less calories from her formula her body is now making the effort to get what it needs from her solid food. Typical kids move from formula/breast milk to whole milk (18 calories per ounce) at the same time they start to really eat larger amounts of solid food.

This seems to be what Ellie is doing. So now Gib and I have been desperately trying to figure out what and how much a typical child should eat. What seems to work for Ellie is a diet centered around protein and vegetables. She also gets a small amount of carbs and fruit. The only fruit she gets is small amounts of the high fiber fruits and only fresh; bananas, blue berries, blackberries, and her favorite.. strawberries. The only thing we don’t give her much of is dairy; she still does not tolerate it very well. But, snacks are always a struggle; we are always searching for good snacks. If anyone has a good ideas please let us know.

We are very excited by her progress but, almost everyday we want to kick ourselves for not trying this earlier. It is really hard to look back at everything we have had to go through over the past year+, and wonder what we could have avoided by decreasing her calories sooner or by never increasing in the first place.

Live and learn I guess, right? Right?

It is blue berry season here, so here are some shots of our last two trips to the patch.

Ellie and one of the many blue berries she ate that day.

Ellie and Daddy checking out the apples on the way to the patch.

Ellie getting a piggy back ride back to the car.

Guess What we did yesterday!!!

Yes, a last year we did a post about how we have decided not to let Ellie swim. But.. now she is two and that central line is not going anywhere any time soon. So, let the girl swim! Yesterday we loaded up and when to my sister's house for a short swim.
It was not a decision that we came to easily. We have been debating the idea for about two months. We have let her wade in the ocean but we were still very careful that her central line never got wet. When I was at Oley last month, I was asking everyone and anyone that that had a central line what they did when they swim. And I got pretty much the same answer. Cover everything as best as you can, and then change the dressing and cap when you are done.

She never really swam but she did leave the steps and walk around with us. Next time we will do it during the hottest part of the day and bring lots and lots of toys.

We also checked with Children's before we attempted this, and they ok'ed it. With the right precautions for her line, she is allowed to swim in clean ocean water and in a private pool that we know the owner of, so we know that water is clean. My sister's pool fits that bill and Ellie had a blast, so we will be back.

RAIN and friends

For those of you in New England this is old news, but we have had a bit a rain this past week. Three days of never ending rain and thunder storms. We had to introduce Ellie to THUNDER and explain that we could not make it stop just because she asked. We explained that thunder was good because thunder meant that we would have puddles to jump in, one of Ellie's favorite past times. Below is a frame by frame of what we did for about a hour each of the three days.

1

23

We all had a very good time. Ellie and her best friend Ka (Karlene) got to even play in the mud. Ellie is still doing well. The new big girl bed is great, and a challenge. She is sleeping well, but I still am having nightmares of her getting out of bed, walking into our room and leaving her pumps and her CVL back in her room. Right now we have her pumps in backpacks at the foot of her bed, but we think we will either lower her IV pole and teacher her how to roll it or put her pumps in her shopping cart. For now we are hoping that we can convince her not to get out of bed without us. (I know, I know, Fat chance!)

Today we finally had some good weather and got a chance to see some friends from out of town. The Solomons flew in for clinic and stayed for a extended visit. We spent the afternoon at the aquarium and then had lunch in the Quincy Market area. It was a blast and really fun to get the kids together. I'm hoping that Ellie will have a strong base of short gut friends when she grows up. I hope that these kids will be her support system throughout her life. She and Zack got along great. They many long conversations, that only they understood.

Zack and Ellie at the aquarium

This is a picture of two very tired kids after lunch. We had a great time, and hope to do it again next time they are in town.

I leave you all with a picture. To those of you that know what she is touching... scary picture hu? You should have been there when she tried to kiss it.

Yeah it's fake, but it still gives me the willies.

Look at the big girl

We used the vacation as the end of our time with Ellie in a crib and went cold turkey when we got home with her newest fascination-

A Big Girl Bed!This has been in the works for a while now ever since Ellie started to show interest in climbing out of her crib. As soon as she started climbing Abby and I have had nightmares of Ellie jumping out of her crib but leaving her pumps on the wrong side of the crib. Putting the crib against the wall made things better but it was just time to get her into the next bed.

So now we are having entirely new nightmares about Ellie falling out of bed or deciding to go for a walk but leaving her pumps behind.

So far she has been great at staying in her bed for us and her pumps are off the pole in at the foot of her bed to make them more flexible but we know that this won't last for long.

So for now one or both of us jump whenever we hear her make a noise. Sort of like the first nights home with her.

Speaking of which, Tyler April, Ellie's cousin who was born May 23rd at 28 weeks (2 pounds 13.8 ounces), went home today just north of 5 1/2 pounds. Thinking of those first few nights at home makes me want to throw up a little bit. We know that Ben and Sharron will do well with him and that home will do well with him as well. Congratulations.

"Congratulations"

Ellie had her first clinic appointment since March today and that was the common comment from every single doctor, nurse, and specialist that saw her or discussed her progress. Every single person that we met said this to us like we had won something or that we were at the end of something which seemed a bit weird to me. I wanted to stop and tell them that we aren't even close to the finish line even though she looks so good and is so happy and healthy.

The numbers:

• She is still growing well 75th percentile for height and weight. her wardrobe of outgrown clothes told us this a long time ago but it is nice to hear it officially.
  
• She has progressed on her formula and solid food eating . Right now her pump is set to 37 ml/hour and all told she gets just about a liter (1000 ml) of elecare each day. On top of that she eats three good meals a day and some snacks. This was all great news to the nutritionist and got a solid wow when reviewed Ellie's diet with us.
• All of this means that we are losing a night of TPN next week and we are back to 4 nights a week of TPN and three of dextrose. We are keeping the increased volume in there for the summer to keep her hydrated but the calories will continue to be pared down.
• Her labs all look good which means that her nutrition is good and she is well balanced. We like a well balanced kid.

What's next:

• We are still waiting for the bowel motility study that was cancelled in May when Ellie got admitted with the gut bug. The Dr. who does the study is very busy and we got sent to the back of the line when she cancelled. We are going to push to get this done and gets some kind of an answer about why it takes glycerin to make her poop.
• If all goes well witht he study (called manometry) we will probably think about reconnection sometime this fall. Yeah, yeah. heard that one before. No hurry here to get it done. we'd rather wait than rush into it and have to reverse course. We're getting good at ostomy changes anyway.

So the take home message is keep doing what we are doing and bring her back in a month or so to see about progressing towards more TPN-free nights.

I think that they just like seeing her dance down the hallway.

Great White.

So we made it out to Martha's Vineyard and we are staying near south beach. Ellie has only been splashing in the one of the ponds, because we discovered that she is not a fan of big crashing waves. She says " no, no, no" and then points to the car and says " go car home, now." So tomorrow we will try a much calmer beach and see what she thinks.

We have been in the surf a few times and the beach we are staying at is the beach that they sited the Great White at two days ago. Yeah, a 20 foot great white and yes we have been in the water. As our friend Dan likes to say, "if you get eaten by a shark, then it was just your time to go."

This is the link to the story if you are interested. http://news.bostonherald.com/news/regional/general/view/2008_07_12_The_Great_shark_hunt:_We_look_for_the_beast_off_Martha_s_Vineyard_/srvc=home&position=0

I promise there will be pictures of Ellie in the water soon.