Wednesday, December 31, 2008

Skinny New Years

It has been a month off of TPN. I would love to say we are doing great and everything is pointing towards another month off, but I can't. Oh, what a month. If I had known what this month was going to be like, I would have thought twice about coming off of TPN.

So far we had 3 days of high output that settled down only to be followed by 3 days with no food (NPO) for her bowel study. Which was not fun, but hopefully we will get good results. We got home and were starting to recover from the study when we got hit with a 5 day stomach bug. Which also resulted 6 really fun hours in the ER, blood samples (everything looked good) and stool samples (She tested negative for all the big bad stomach bugs). That was a week and a half ago and she is still only able to handle a half Pedialyte and half elecare mix for her G-tube feeds. And to end the month with a bang, last night she just came down with a doozy of a head and chest cold. Good times, good times.

I am ashamed to say that this month I actually missed TPN. In the past when we had more than 2 weeks when she wasn't able to eat as much as she should, I didn't panic knowing that the TPN/Omegaven would pick up the slack. But that isn't the case any more. Now if she doesn't eat, can't eat or does not tolerate her food; she doesn't get what she needs and that meas weight loss.

We have gone from gaining a pound a month to losing a pound. She is skinny. The pants that were tight around her waist last month are now falling off. It is kinda heart breaking. I know that recovering from an invasive study, stomach bug and cold will take a while and that I should be patient and not jump to any conclusions about what this weight loss means. But it is hard to not get nervous when her clothes look like they are hanging on her.

The worst part is that I know that if this month had been a normal one, she would be doing great. So now we just wait for her to recover from this cold and then wait for her to start gaining weight again.

On the up side, she is looking great! She is charming everyone's socks off up here in Maine and up to her same old tricks. She is very chatty and loves to have long talks, and loves to play hide and seek. Her new favorite obsession is counting all 14 steps of the stair case going up and down each time. She is having a blast playing with her cousins and her grand parents.

Gib and I are looking forward to new year with the possibility of some weight gain, no TPN forever, a reconnection surgery, and Ellie starting pre-school. We wish you all a very happy New Years.

I leave you with a video of Ellie unwrapping her favorite Christmas present.

video

Happy New Years!!

Friday, December 26, 2008

Merry Christmas

The Official 2008 Ellie Brogan Christmas Card Photo

My goodness what a week we have had. It started with a Sunday trip to Children's for cultures and bloodwork (all negative so far), moved quickly into pre Christmas fun at home with a surpisingly chipper little girl.

The holiday adventure moved into phase II yesterdaywhen we headed up the road to Maine for the week to let Ellie and here four cousins in Freeport play for a while. This was the same plan that we had last year that was changed on the road when we heard of a mysterious stomach bug that was making the rounds so we are preparing to go with an understanding that we could be amending our itinerary at any time. Such is life with Gut Girl.

Not a whole lot of time to post much but here are some photos from the week leading up to Christmas. Things are good for now and we are eagerly anticipating the results from the manometry study.


Santa on a Firetruck. a tradition in our town since 1946. At least according to the sign on his sleigh.

Christmas Cookies.


A trampoline for a 2 year old? What could possibly go wrong?

Trampolines make good present opening platforms, too.

Let's play a game!


Mmmm. Ice cream. just a few licks.

Sunday, December 21, 2008

Almost three days

That's how long we lasted this time.

we came home from Children's on Thursday afternoon and made it to Sunday lunch before we had to go back.

Ellie recovered from her study quite well and was raring to go on Friday and Saturday in the first real snow of the year.

We even broke open the gingerbread house and managed to decrate the whole thing without a battle over not eating the candy:


The one thing that we noticed is that the girl who took a suppository to poop before her study was now pooping freely after the study snaked three tubes into her bowels.

We chalked it up to good luck and figured that it would pass by Friday. It slowed a bit Friday but by Saturday we had 8 poops and by Sunday noon we had another 6. That borders on what the doctors call 'stooling out' and we gave a call down the road. Ellie was and is in great spirits (except for her poor bum) but that kind of activity set off alarms for all off us.

As luck would have it the surgeon on-call this weekend was none other that Ellie's surgeon, Dr. Jennings who asked us to come in to the ER for bloodwork (to check her electrolytes) and a couple of stool samples because as he put it rotavirus is every where at Children's these days. So off we went at 35 MPH down the Mass Pike through day three of a very long snowstorn that made me shovel four times....

By 5 we had clean bloodwork results, a sample of ostomy liquid was sent off for the tests and we were heading tothe car when Ellie told us: 'I am pooping, now'. So back they went to give a stool sample and we headed back down and even worse MassPike to get home.

It will take a day or two to get results and if it is rotavirus, we may have a significant change in our holiday plans in the works. We are hping to avoid a Children's Christmas.

Ellie is very excited about Santa.

But there isn't anyhting we can do about that one. we'll wait and see.

Wednesday, December 17, 2008

Back up and at 'em

At 5 PM tonight Ellie's adventure with manometry finally came to an end with a successfully completed full test and 10 hours of squiggles for the doctors to look at, analyze and interpret. We should have results in a week or two but the doctors very careful not to give us any preliminary results in such a technical test.

The reason is that the test produced a lot of these computer records (10 hours worth): Each line is a separate sensor in a manometry catheter. The top 5 or 6 are her stomach, the next 5-6 are her small bowel and the rest are her colon. Although these are lines are relatively flat, we watched as she finally pooped out the test solution late this afternoon and can tell you that the squiggles really dance when her bowels move.

Whether they are doing the right dance is the big question.

A little after 5 the doctor came in, removed the catheters from her colon, ostomy and g-tube hole, replaced the g-tube button with a fresh new mic-key button and we were done.

Ellie was ready for a walk around the floor:
By about 6:45 we had Ellie back in her bed and Abby was doing her best to keep Ellie from doing 'exercises' in her bed when I left:

Post Test, Wednesday 6:30 PM

She had Cheerios and elecare for dinner. We will come home tomorrow morning and spend the next day or so feeling our way along and trying get her back up to full meals as quickly as possible and hope that the little girl that we brought into pre-op yesterday is back in full force.

They sure look like the same kid:

Pre-Op, Tuesday, 1:00 PM

Getting better all the time

Things have settled down significantly since this morning and we are nearing the end of her 12-hour study.

Since lunch time Ellie has started to eat, shown good signs as food moves through her gut and has finally settled in for a nap.

Because of her vomiting it looks like she will need to stay another night just to make sure that things are working. We had anticipated this and Abby even packed for two nights.

We haven't heard results yet and probably won't for some time so we will wait to give the verdict until the pros give us the official opinion.

We have no idea when that will be.

So much for the smooth sailing

Well it isn't like we weren't warned about the down sides of a manometry study. And just to keep the reputation up, Ellie's gut has decided that it doesn't like having extra tubes in it and has started to vomit for us. IT started last night with some small spit ups and has progressed overnight to a full-on Exorcist/Stand By Me barf-o-rama by 9 this morning.





Abby is on her third set of borrowed scrubs. If we could only get Ellie to stop watching TV while she barfs we would be a lot better but I guess those Brogan genes are overpowering. Oooh, magic picture box......

This is very curious to us and to the GI/motility doctor but he las learned quickly that Ellie's gut is far from typical and had a few 'oh, really' moments this mrong when we told him new things about her history.

The thought right now is that the catheters that are in her bowels are blocking her bowels and things are backing up until she gets full and decides to unleash a wave of green bilious vomit on us. The color and barfing are eerily similar to the night she was born and all of those months where things were piling up in her dilated small bowel.

The doctor is concerned about this and may pull the plug on the test early if things don't get better soon. We hope that we can peel away tubes on by one to keep the colonic test, which is the real goal of this study for us.

More to come. This fancy wing of Children's has a laptop in each room so we can post from here. The hospital even updated their internet security to allow blogger to work. Finally.

Tuesday, December 16, 2008

Day 1 Update

All is well at Children's.

In fact, all is better than we thought going into the day.

Everything went as we expected and at about 5 this afternoon we were summoned into the recovery room by the GI specialist who is running Ellie's studies this week. He came to find us and tell us that she wanted her mom. Now.

So we went in to the recovery room and got her settled and learned the following things from the GI/bowel motility doctor in his words:

'She has most of her colon'. No kidding. the same kids who we assumed had only about 70% of her colon now has most of her colon. Considering that in some cases the colon can take on some of the function of her missing small bowel, this is great news and makes us even more optimistic about her gut adaptation.

'Her bowel looks good' Ah, words that we love to hear. Abby and I had tried to avoid thinking about what the doctor would see on the inside but hearing pink and good looking is always a good thing. That doesn't mean that it is working perfectly but on appearance it seems that there aren't any areas of dead or sick bowel in the length of her remaining small bowel.

'Some twists and turns at the top of her colon' Maybe the answer to the mystery of Ellie's lazy pooping. If there are kinks or turns or some other strange configuration of her colon up abouve, it might slow down the progression of stool through her colon.

All of these good words made our day but the proof will be tomorrow when the second half of her test gets under way. In addition to scoping her bowel the doctor left a bunch of new tubes in Ellie that will measure her bowel function tomorrow morning. Right now she has a catheter tube in the lower end of her colon, a catheter tube in her ostomy going up into her small bowel, a catheter going in her g-tube hole going through her stomach to her small bowel, a tube in her g-tube hole that works as a vent and a feeding port, and a central line. A helluva lot of tubes for little girl but she is still pretty dopey from the anesthesia so she has been pretty good about it so far.

Tomorrow's tests will take a long time and begin before breakfast for most people. I have to go to bed to get GUs fed and happy before going in for a 6AM start time.

We hope to have some new answers by this time tomorrow and who knows, maybe a plan for the next surgical step....

Monday, December 15, 2008

Let the games begin

This afternoon marked the beginning of a very long few days with a very different girl than the last time we had to take her in for bowel studies.

No matter how old she is, the doctors need a clear bowel to look around so we have to take steps to clean everything out before they go a lookin'. So at 1 this afternoon Ellie had a good lunch with Karlene. Noodles, asparagus and chunks of cold chicken breast. They had a picnic in our living room as a special treat with ellie doing gymnastics between courses.

I swear Ellie knew something was up because, by our estimate that is it for her until Wednesday afternoon. She is off of solid food, off of formula and onto pedialyte until tomorrow morning. Then it is nothing for a long while until this whole thing is over on Wednesday and we have to begin the task of getting her back on formula and food.

To add insult to injury we also had to give her an enema tonight and another in the morning to help clean things out. What is is that the wode-mouthed frog says? Ooooh.

Poor little girl.

We will see how she does tomorrow morning when she wakes up and will likely be hungry. We will try and distract her by doing fun things until 11 when we leave for the hospital but after a while she will want food and tell us, like she did tonight: 'I hungry. eat dinner.'

It is especially tough to deny her food when we have worked so hard to get her to eat over the past few years. I sincerely hope that this isn't an event than sets us back months on her feeding skills and drive.

Who knows we may take the plastic off of the candy cane by Christmas. All she wants is one lick. Really.

The bags of distraction are packed and we are ready to set up camp again at CHB. I wonder what they would think if we brought the dress-up clothes to make Ellie fancy:
If we can simply avoid Au Bon Pain. I hate that place and their lousy coffee. It tastes like hospital to me.

We will be laptop-less for this adventure so updates will come late tomorrow night when I get home.

Who knows, we may have lots of good news to show for the hard work.

Wednesday, December 10, 2008

Manometry

Next week we take the next step in the mystery of Ellie's Bowel when we head to Children's for a few days of tests. Ideally the tests will show us what the inside of her bowels look like, how well her gut adaptation is going physiologically, and also how well her bowels are working.

She will have two studies done over the course of two days. On Tuesday afternoon, after being food and formula-free since midnight (NPO/NPG as they call it) she will have a endoscopy of her bowels. Surprisingly, this simple look inside hasn't happened yet in her colon, we are hoping it will tell us some things about what is going on inside. She will be under general anesthesia for this and the plan is to scope her from the top down, from the bottom up and maybe from the middle, as well.

Then after the 'scope they will snake a tube from her stomach, all the way through her small and large bowel or maybe one from above and one from below. This setup will be left in over night and then first thing on Wednesday morning, Ellie will have an intestinal manometry study done. The tube that is in her intestines is connected to a small pump, which pushes water slowly through the tube and into intestine. As the intestine contracts and tightens around the tube, it stops water flow in the small holes in the tube. Each hole has a pressure sensor (manometer) attached to it and the whole thing is attached to a fancy computer so each contraction is recorded and measured and showing a pattern of activity. This study which takes eight hours of sitting in a bed attached to a fancy machine will show us how well the muscles in her bowel contract, whether the waves are coordinated, and also if the small and large bowels are in sync with one another or dancing to separate tunes.

All of these tests will hopefully answer the question of why Ellie still needs help to poop every day. If there is an obstruction or a 'pseudo-obstruction' that slows things down we could work to fix that. A lot of kids with multiple short bowel surgeries will develop scar tissue called adhesions that can constrict or kink the bowels.

The other thing that we hope to learn is whether things are coordinated enough to think about reconnecting her bowels and getting rid of her ostomy. Our concern is if we connect her small bowel to a large bowel that is blocked or partially blocked it will create a log jam and possible re-dilate her bowel, something that we have used an ostomy to fix for more than two years.

This week we are moving towards a total lockdown with Ellie since her much-anticipated intestinal studies are scheduled for next week and we had to delay this same study last spring because of a relatively simple GI bug. It would be really frustrating to get this close again and have to postpone it because of a cold or a stomach bug so we are keeping a tight hold on the reigns until next week. They do very few of these studies each week and a delay might mean another six months.

So no gymnastics this week, except in the living room.

The plan is to go in around lunchtime on Tuesday and be home for dinner on Wednesday but all things considered, we are planning for the possibility/likliehood of staying Wednesday night as well. Something about pumping a lot of water into her bowel sounds like it may be tough to bounce back from and an extra night at the Inn may be needed.

So we are collecting our DVD's for the 8 hour test to keep us all entertained and debating the old crib/cage versus a big girl hospital bed. Thankfully Ellie's taste in movies has evolved from the Wiggles and Barney to more watchable movies like Toy Story and Monsters, Inc. We will still bring Kipper along for the ride. Somehow his accent is an anesthetic for all of us.
It seems afte the pre-op appointment on Friday that the only thing to resolve how we are going to tear Ellie away from the Christmas tree and her collection of animatronic snowmen that came from her great grandmother:

Monday, December 08, 2008

So far, so good


Well today marks a week of the great Ellie Brogan TPN experiment. Since we have been told to look at the weekly trends in results rather than the day-to-day ups and downs that may swing depending on small things, here are the notes for the first week.

She is feeling great and doesn't seem to notice the difference between TPN and hydration fluid.
She still has lots of energy, is happy and, if anything is a bit hungrier in the morning than she was in the past but that may be our imagination. It is always great to hear her tell us that she is hungry.

She is keeping her weight up. This morning she weighed in at almost exactly where she was a week ago. That counts as a win in my book.

Fish oil is agreeing with her. We have started to give her liquid fish oil through her g-tube to replace those great omega-3's that she isn't getting from omegaven anaymore. The antiinflammatory properties along with the other great things that omega-3's have done for her are too good to pass up so we have done our homework and have a bottle of lemon-scented fish oil that replaces some of the oils that she was getting. I say some because I did the math (yes, I can still do stoichiometry from chemistry) and she would need a huge amount of liquid oil, about 30 ml or one ounce per day, to replace the dense good oils in her omegaven. We're not that brave and are pretty sure that she will dump that right out of her ostomy so we are giving her about half of that and will add it as we go.

We won't know if she is missing the other parts of TPN like vitamins and proteins for a while and will have those checked next week when we go in for her stay at Childrens for her tests.

It will be like clinic. With a bed.

So we keep on moving, eating and watching Ellie do her tricks.

Ellie is really enjoying Christmas and thinks that our tree and some well-timed snow is the greatest thing in the world. Even though we only got 1/2 an inch yesterday, we made a snowman (all 8 inches of it) and threw snowballs. The tree is up, the animatronic snowmen are singing and it is off to the races.

Sunday, November 30, 2008

Last dance with Omegaven...

Abby drew the short straw last night and got the honors to put together the last go round of full-fledged TPN tonight. I serandaded her with my version of Donna Summer's 'Last Dance'.

Yup. No more splitters, no more caps each night....

we have gone from this with every other night's set up:

To this:Instead of an ingredient list that reads like a twinkie wrapper (I still don't know what all of the components are) we now get a very simple list of things that are going into her central line: water, salt, sugar. And we are down to just two backpacks instead of the Omegaven set-up that required special handling to keep the glass bottle upright.

Bye bye Omegaven. Thanks for all of the good that you do. Maybe we'll see you again sometime. Hopefully not. Go on and save some other kid's life.

Day one of the no TPN experiment starts tonight. Results to follow.

Tuesday, November 25, 2008

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.

Sunday, November 23, 2008

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!

Friday, November 21, 2008

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!!!!!!

Thursday, November 06, 2008

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!!


Monday, November 03, 2008

Don't Forget to VOTE!!

VOTE VOTE VOTE VOTE VOTE VOTE VOTE VOTE VOTE VOTE



PLEASE REMEMBER TO VOTE TODAY


VOTE VOTE VOTE VOTE VOTE VOTE VOTE VOTE VOTE VOTE


Saturday, November 01, 2008

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.

Sunday, October 19, 2008

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.

Friday, October 03, 2008

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!

Sunday, September 21, 2008

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.

Friday, September 19, 2008

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:

Tuesday, September 16, 2008

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.

Sunday, August 31, 2008

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.....