Taking our Show on the Road

We are getting comfortable at home and have taken the little steps towards being a mobile family- going to the grocery store, going to lunch(buffets are best) and so on but always returned home around 4 PM to get ready for the veening routine. Tonight we started to move toward the final frontier-sleeping away from home.

For a low-risk dress rehearsal we packed up and came the full 6 miles from the safe zone at home to spend the night at Abby’s sister’s house Wayland for the night.

We are a long way from traveling light.

It took six bags to get all of Ellie’s essentials loaded in to the car. Between what we knew she would need (food, TPN, pumps, an IV pole, etc) and what she could possibly need (a new ostomy bag, a new central line dressing, etc) it is a lot of stuff. Add in the normal baby stuff like toys clothes and books and a bed and it is quite a load. Add Gus on top of that and we almost forgot our own clothes and toothbrushes.

I am beginning to see the light about minivans even for us with only a baby and a dog.

Going to Wayland was nice for a test run because it is not so far that we are in a world of trouble if we need something important from home.

Which I did not once but twice this evening.

The first was to pick up cotton balls to fill her ostomy. A simple brain fart on my part to forget a staple of our day. Nothing really urgent and we could have solved it with a trip to CVS.

The second was a bit more important since I screwed up and punctured the only IV bag that we had for the night when I was driving the spike into the bottom on the bag. I simply rammed it through the side of the bag.

Not a big deal when the replacement was 6 miles away, but it was an eye opener for what could have been if we had been three hours away.

Only two return visits to get more supplies and a third scheduled for the morning since Abby was bit spastic in the new environs and blew through our supply of saline syringes while hooking Ellie up tonight.

On the bright side, Ellie is a tough bird who played hard all day:

http://www.youtube.com/watch?v=mruXUHpd-IA

Then she fell asleep on the bed while we set up her PN tonight.

We’ll see what she thinks about sleeping in the Pack n’ Play tonight.

If all goes well we will probably do this again to fine tune the routine. Then it is off to the races......

Nap Time

File this under normal baby stuff that still freaks us out.

Ellie melted last night at 5:00.

Abby was cooking dinner, I was finishing up work for the day and Ellie was in her Exersaucer, playing with toys and watching Abby cook.

I went into the other room and came back to find Ellie in this position:


She had simply run out of gas and fallen asleep in a comfortable position. This wasn't a doze, she was out. So far out that I could pick her up and lay her down in the Pack n' Play in the living room for a 30 minute power nap (but not before taking some pictures, of course):


It is still the 'normal baby' stuff that causes us the most trouble.

Was this normal?
Was her blood sugar low or high?
Had we fed her too much after increasing her rate to 55 ml/hour?

Nope.

Just a sleepy baby.

She rallied at 5:30 and was out for the night at 7:30.

We are well over 500 ml of formula a day these days. Gotta love that pump.

In the groove

We have been home for just over two months now and have gotten our feet under us to the point that both of us are scared that we are getting too comfortable and will slip up because the procedures have become second nature.

We attach and detach her pumps so often that we sometimes forget the gravity of what we are doing and what a tiny slip up could mean for Ellie's health. If you do something twice a day, every day, you are bound to have good days and bad days. We individually were afraid that the bad day may mean a serious slip and when I brought it up this morning, Abby told me that she had the same fear.

This leads us to have moments of anxiety at strange times. For example last might at 2 AM I was absolutley sure that we had forgotten to hook Ellie up to one of her pumps. I had the monitor right next to my head and could hear the pumps going but for some reason knew that I had forgotten something and that the pump was sitting next to us on the floor. I woke Abby up, she knew that I was dreaming and ignored me. I woke her up again and she ignored me. Finally at 3AM she woke up enough to tell me to stop worrying and everything was the way it should be.

It was, I was just being a bit whacko.

It is good to have a co-pilot.

Ellie was up at 5:30 (after sleeping 10 hours straight) this morning and quietly standing in her crib and exploring when Abby went in to check on her.

Standing in her crib. Not crying or looking for us, just having a look around at the world.

We got her up before she could hatch an escape plan and spent the morning in bed:
We hung out until her pumps beeped at 7:30 and we started the rest of our day. She loves watching the Today show with us, and even likes an early morning go-round of SportsCenter...

Did I ever tell you how lucky we are?

Every time we meet or hear about another kid that has short gut or another condition/disease/disorder that requires them to be on TPN, all that Abby and I can say is that we are unbelievably lucky to have stumbled into the great things that are going on at Children’s.

Plain old shit luck.

To think that we were torn for a while at Yale about whether or not to come to Boston.

How different would Ellie’s condition be right now?

Would she have liver disease? Would she be very sick? Would we be wearing the transplant beeper waiting for multiple organs?

The answer to all of those is disturbingly probably, yes.

Instead of the very pale, very happy little girl who eats Cheerios as fast as she can find them: we would be looking at serious liver disease.

Sometimes we forget that there are kids all over the place that are on the ‘old’ Lipid and that there are families that are living through the really bad, scary parts that we were afraid of when we heard about the reality of Ellie’s condition in April.

Liver disease has all sorts of nasty symptoms. All things considered, we got off pretty easy with a bit of jaundice and some yellow eyes.

Shit luck.

We didn’t have inside information or a relative that knew gastroenterology. We simply came to Boston and had this fall into our lap.

But the encouraging thing is that the word about Omegaven is spreading.

I learned last week when I went to the hospital to get meds it is spreading so well that the folks at Children’s can barely keep up with the parade of sick kids that are making the pilgrimage to Longwood Avenue to get on Omegaven.

We know of another family that is at Children’s today from Indiana and hope that their visit goes well.

In the meantime we will keep spreading the gospel of Children’s and Omegaven along with our understanding that this may not be the answer but it is a lot better than any other option.

There aren’t any secrets and we all want our babies to be healthy.

So I will keep ranting about our great luck and great care in Boston until someone tells us to stop.

Knowing the work habits of the folks at Children’s, it probably won’t be them that yell uncle.

More mobile everyday. IT WORKS NOW.

Big fun on a Sunday morning....

I've had bad luck with You Tube so here it is on Google Video.

Good old Google...

Make sure to turn up the volume. My film making skills are blossoming...

http://video.google.com/videoplay?docid=-6227528559150447512

Winter

We finally got a real snow storm this week and this weekend it warmed up enough to take Ellie for a ride in her sled. We bundled her up very well and packed her pump under the covers and off we went:



















Ellie thought it was great fun to ride along in the sled and even thought about falling asleep.

All told we were outside for about half an hour of fresh air which was good for all of us and gave us another glimpse of what things are like for parents who don't know TPN from TP or the difference between a G-tube and a GJ tube.

Ellie's Boyfriends

Ellie got a chance to go visit her boyfriend Christian, and pick up a new one, when we had our first ever play date yesterday. We went into Jamaica Plain and visited Christian and another little boy named Max. Christian is doing great post transplant and Max is here from Denver to get on Omegaven.

It was great to see both Christian and Colleen and Christian looks like a new little boy. First thing he did was push all the toys away from Ellie and then started hitting him self in the head with a couple of other toys. Such a boy!



Ellie got even though she later tried to use Christian as a stepping stool. He didn't really like that. He didn't like that, but they did have a good time exploring the great range of toys that Christian has.

Ellie does have one major problem though. She scares all the boys away.







When she gets really excited she lets out an incredibly loud squeal. It was this squeal that made all the boys cry. I don't think Christian or Max minded having Ellie come to visit, they just wished she would have been a bit more quiet.

Max is 8 months old and has been on Omegaven for 3 weeks now. It usually takes around 30 days for Omegaven to do it's thing, so they have a bit longer to wait. He is very cute hopefully we will get to see more of them before they go home.

I hope the boys had as much fun as Ellie did, she fell asleep almost as soon as she got in the car and took a nice long nap when we got home. It was great to see Ellie interact with other kids, we may need to work on her manners for next time. She just has to remember that you play with the boys not climb on them. Maybe next time Christian can teach Ellie how to say "ball" or how to do his great monkey impression.

All Reglan is not created equal

Since Ellie left the hospital she has been on a whole string of medications to help her gut do more of what it is supposed to do.

These are not your average drugs and can’t come from the CVS or Walgreens down the street. Instead, the have to be ‘compounded’ by a special kind of compounding pharmacy. This is usually the older independent pharmacies that you may not even notice in your town.

A couple around here still call themselves apothecaries, a term that I thought went away with Romeo and Juliet, phrenology, and leeches.

Apparently I was wrong.

When we left Children’s in December we got Ellie prescriptions filled at the apothecary that the hospital knew that is close by the hospital. It is a tiny place in the basement of a building with beakers and vats of stuff cooking. It is a bit of a pain to get to, but it had the drugs that Ellie needed and made them quickly for us so we got her meds there to start. It also has all sorts of people getting their custom meds which is a good sign.

With two return visits to Children’s, that original 30 day supply managed to last us almost 2 months.

This past weekend the Reglan ran low and to save a trip to Brookline for a refill we called the small independent pharmacy here in Wellesley to ask about getting it refilled.

Sure , they had it and could fill it for us. Great. We don’t have to go to Brookline to get her meds, just to the pharmacy next to the grocery store.

Some meds, some dinner and I’ll be home.

A great plan which worked just fine until Abby tried to give Ellie her evening Reglan-Ellie wanted no parts of it and spit half of it out before barfing the rest onto her bib.

Something wasn’t right with the smell of the liquid and the taste was off as well. It tasted like Chocolate/Vanilla instead of baby fruit. We stopped right there and waited for morning.

Reglan is a nice drug but we stop and start it when things seem odd.

We called the pharmacist at Children’s the next morning and found out that all Reglan isn’t the same. Most likely what she got was the adult version that had the same dosage but also had a lot of alcohol and sugar in it- two things that are no-nos for short gut kids and can cause all sorts of other issues.

Strike One.

On to pharmacy number two, in Needham which mixed up a batch of ‘alcohol free’ Reglan with ‘just a tiny bit of sugar in it for the watermelon flavor’.

Ellie took it.

But then the fireworks started.

Instead of pooping 2-3 times a day, Ellie has pooped six times for the past two days and was just grumpy in the night.

Strike Two. Pooping six times in a day brings a certain Johnny Cash song to mind…..

Poor little girl.

We’re not sure if the new version of Reglan is the culprit but we stopped it yesterday and her poops are back in order.

Her poor sore bum seems to better, too. We have really good hospital diaper rash cream, powder and ointment which takes care of the rash very quickly. How we will ever go back to plain ol' desitin is a mystery.

Abby is off to Boston today to get the good stuff directly from the pharmacist Children’s to tide us over until the next prescription is due.

We’ll be driving to Brookline for meds from now on.

Love is a burning thing
and it makes a fiery ring….

One small step for Ellie, One great big pain in the ass for us

http://www.youtube.com/watch?v=XlWx3QeCW_Q

After a Valentine's Day wardrobe change because of a leaky G-tube (who else would have more than one outfit for today?), Ellie decided to stop messing around and start standing up for us and took a few steps today.

These are steps two and three.

It took us a second to find the camera.

Note the multitasking as she blows raspberries as she stands and steps.

We are in for a big change and a lot of Ellie chasing....

Happy Valentine's Day

Just a quick note to wish all our friends and family a happy Valentine's Day.






Ellie is having a great day watching the snow/rain fall. We have had such a mild winter that she has never really had a chance to see that much snow. We are hoping that the snow will stick around long enough for her to try out her new sled.
She is spending the day seeing how far she can get on the first floor and trying this new thing called standing up and letting go. Her current record is about 30 seconds. She gets really excited and we get a little nervous. But she is definitely having a good time.
Thank you to all our friends, family and complete stangers out there who have been following Ellie's story over the past year. We have felt very loved and supported. I know Ellie would like to give you all a big wet open mouthed kiss. Thank you and happy Valentine's Day! XOXO

One night down, Six to go

We made it through our first night without PN without any noticeable side effects.

It was a little bit nerve-wracking for us since it required new plumbing of her IV lines to run just one fluid in but we figured it out and made it through just fine.

We also managed to deal with our pessimism that is attached to new things with her care. We were both sure that Ellie's night off of PN would be accompanied by a trip to the ER for some unknown reason. It is just the way things seem to work with Gut Girl sometimes

Fortunately, we had a smooth night without a hiccup.

A night off of PN wasn't all that much different than a night on PN. Instead of PN with its amino acids and vitamins, Ellie was hooked up to a steady stream of 'D5', a solution of 5% dextrose and saline at her normal PN time. This kept her hydrated and kept her blood sugar normal.

Regardless of the small difference, it is a big step and represents a step towards hopefully losing the PN all together at some point.

It sure would be nice to lose the central line and not have a huge infection liability sitting in the line of fire just under her nose:

If you look closely you can see bananas, sweet potatoes, cheerios and rice cereal...

That will come in time as we get her to eat more and more.

She hits 400 ml most days. 500 shouldn't be that hard by next Thursday.

Ads

You may have noticed the square ad to the right and the blinking ad at the bottom.

These aren't accidental or part of a big plot to show lots of ads all over the internet. As a blog, you can make $$ selling ad space but that's not what this blog is for.

Instead, that are links to an organization that supports organ donation, something that has become near to us and some of the families that we know and we wanted to spread the word about how important this is.

Clinic Visit

Ellie had her periodic clinic visit today at Children’s. Abby took her while I went to New Hampshire for the third day of interminable fisheries management meetings.

In addition to charming everyone in the place with her smile, laugh and walking tricks as they did laps around the clinic, Ellie also had a bang up day with her tests which is great.

It was a meeting of the Omegaven families club today with old and new families there to see the docs. Abby met lots of families that were only names on the internet or email and even met a family that learned about Omegaven through this blog that we didn’t even know about. The community of families that has assembled around Boston is really something and growing by the day.

Please come to Boston for the Fish Oil....

Her weight is up. Up 320 grams from the last visit which is more than the 280 that the nutritionists and GI docs had hoped for. She still is in the 50^th and 75^th percentiles for weight and height respectively but ahead of where they want her to be. She now weighs 8.75 kilograms or 19.25 pounds. Quite a load that is giving Abby quite a set of arms.

As promised, we have reduced her TPN again. This time, in addition to reducing the overall volume, we are getting one whole night a week without TPN. She will still get fluids by IV on those nights since her colon isn’t fully attached to absorb fluids but no TPN.

The weaning continues and will continue as long as we can keep increasing her feeds. The new goal is to be at 500 ml per day when we go back in a month. Since we have already gone past 500, this shouldn’t be a problem. Shouldn’t but you never know.

We are still hesitant to increase too fast and Ellie will have an upper GI dye study done to check on the progress of her bowel and it hopefully shrinks.

We got word from the docs that we are doing well with foods and got the okay to add new foods to her diet. As long as it doesn’t have lactose in it, we are good to experiment which should be fun and let Ellie exercise the tooth that is trying to come in her top front.

She is happy and growing and absorbing lots of the food that passes through her ostomy.

Lots of folks are very happy with the way things are going.

Mobile. With an anchor

Ellie is getting more mobile by the day. She migrates across the room from furniture to furniture and will leave the room every now and then as she walks along the wall. ehr coolest trick is that she will now stand for 5 or so seconds without holding onto anything, long enough to look at us as if to say, 'isn't this the coolest thing?'.

We even lost her this evening when she migrated to the far side of the table with both of us in the kitchen. Her hiccup gave her away.

One of the sad and fortunate things of Gut Girl learning to walk is that her milk pump sits on the floor. She gets up and moves but unless we move her pump backpack, the tube to her G-tube gets taught and she will stop like a boat on an anchor. The tube is attached to her clothes by a safety pin so it doesn’t pull on her belly.

As she gets better on her feet we have been trying the backpack on for size and to see how well she does. We are concerned that it might not be a good thing to put a backpack on an 18+ pound infant and have only done it for limited times until the PT folks tells whether it is okay or not.

She loves the freedom of movement and loves to explore with her new wall walking trick.

Who would have thunk in April that she would do this in February? Every day's fun is extra cool when you think about how far we have come.

Gut Drugs.

Ellie is on a string of drugs every day to help her deal with her limited plumbing. Off and on, she has been on these and some others forever.

Reglan (Metoclopramide if you are our generic friendly insurance company) is a drug that helps Ellie move things down the pipe and through. Her motility has been pretty bad in the past and Reglan may help to boost this. This is one of the standard list of short gut drugs that seemingly every short gut kid is on. Some parents question whether this is necessary but it seems to do some good for Ellie. She gets this four times a day, either by mouth during the day (‘p.o.’ as the docs say) or through her G-tube when she is sleeping.

Actigal (Ursodiol) This is bile salt that is found in bears. No kidding. But, instead of finding Yogi and milking his gall bladder, they synthesize it.Bile salts help you digest fats in your intestine. Since Ellie doesn’t have full function in her intestines we give her this to give her digestion a boost. It also has good effects for her liver. Another of the cocktail of short gut drugs that every kid gets. She gets this twice a day either by mouth or by G-tube.

Iron- For some reason Ellie is anemic. To boost her iron levels she gets an iron supplement twice a day. We have just started this and will keep her on it until the measure of her iron ‘hematocrit’ comes back up to normal levels. She gets this twice a day p.o or in the g tube.

All of that brings the grand total to 8 times a day that we have to draw up the drugs in the tiny little syringe and pump in into her. 8 times that we have to remember to do this. Checklists and alarm clocks help, but we miss them every now and then. We are getting better. Hopefully they will help help get better at digesting and absorbing.

Playtime

Playing is a lot more fun at home than in the hospital.

Ellie gets to play with Mom:

Whoa! Everything is upside down!!!

And play with Gus:

This place smells like dog farts!

Up up and away

We are settling into a routine again after our time in the hospital and have adjusted to adding Ellie's wound care to our list of tasks each day. It is remarkably calm around here while being very busy at the same time.


The wound where her suture line opened a few weeks ago is healing nicely and getting narrower and shallower each day. Hopefully it will heal completely by next week's clinic visit which will take that off of her list of care items.

Using a combination of bottles and the pump we have exceeded 300 ml every day this week and on some days even gone over 400 ml. During the day we can feed her at 50ml/hr which adds up quickly and we have to stop her feeds early on most days when she reaches the 400 ml mark. We don't want to overfill the small bowel that we are trying to shrink and until we get an upper GI study done on Thursday to get a status of her bowel, we are going to stop at 400 each day.

Ellie is loving food by mouth and will chew through a dozen cheerios in a short time. Bananas and apple are also good. Rice cereal needs some banana in it to get her to eat it. It is encouraging to have her eat.

She is very close to walking for us and has started to let go of the table for longer periods. She will also migrate around the room. Floor to table to couch to table to wall to dog bed. Last night she took herself for a walk and went into the next room while Abby and I watched.

Last night Abby walked in for a middle of the night check and found Ellie sitting up in bed looking at her. apparently they were each surpised by this one-Abby because Ellie only rarely sits up by herself, and Ellie because she wasn't quite sure of what she had done. We are very glad that we have the covers on her IV lines as she gets more mobile I ahve nightmars of the lines getting tangled around her neck.

We got good news yesterday that a little boy that we know from Children's who had a multivisceral transplant a little while ago had his central line and G-J tube removed this week, leaving him with just a g-tube. Great news.