Thursday, March 31, 2011

A Short Stack for a Tall Cause

We want to invite you to a fundraiser to support the United Mitochondrial Disease Foundation in honor of Lucy Marlett on Sunday, April 10th from 8 to 10 am.

Applebee's Flapjack Fundraiser 

Long time preschool teacher and friend of our family, Lori Lang, came to us and asked if we would be willing to participate with the preschool in this fundraiser for Lucy.  Each year the preschool celebrates the Week of the Young Child, and one of their focuses during this week is to reach out to the community.  We were so honored when Lori told us that they wanted to support our family in a cause that is so very near and dear to our hearts!  We would be just as honored to see and possibly meet some of you.  If you are interested in purchasing tickets for this event, leave a comment with your e-mail address and I will get in contact with you.

We want to thank you for all of the love and support, thoughts and prayers said for our family!

Wednesday, March 23, 2011

About Yesterday…

Sorry to leave you all hanging…for some reason I had difficulties posting on our blog from the hospital network.  When all was said and done it was  nearly 7:00pm when we rolled out of there.  It was a loooong day(we woke up at 4 am) and by the time we came home, picked up our other three children, infused meds, got everyone ready for bed, and crawled under the covers it was after midnight…not joke.  I was too tired to think, much less type.

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Overall the day went better than we anticipated it going, I think it was due largely to the fact that we had researched this particular test more than any test Lucy has had… to say that we were prepared would be an understatement.  Any test that you have to put your child to sleep to check their anatomy and have the testing mechanism placed, automatically qualifies it as being complicated.  I know I have said this before, but anesthesia and mito are not a good combination, add an arrogant anesthesiologist to the situation and now it becomes difficult in addition to being complicated.

Lucy woke up on the wrong side of the bed as usual from anesthesia.  She was grumpy and made sure everyone knew it.  She was not impressed with the way they taped the manometry catheter and foley,which we used to attach a drainage bag,  to her stomach.  She is a creature of habit and does not like it when you go and change things on her… at least not before getting her approval first.  We try and prepare her as best as possible for what is going to happen when she has anything done to her, experience has taught us this.  I guess we assumed that since she has so many tubies attached to her body at all times that these would be no big deal to her.  You know that saying, never assume anything…

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After about an hour we left the PACU and were transferred to the GI suit where Lucy was connected to the maometry machine.  I could try and describe it, but I took pictures instead. 

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The test itself took six hours.  Dr. B and I discussed the details of how the study was going to be done a few weeks ahead of time. The first two hours were spent measuring her stomach and small bowel contractions while “fasting”.   Lucy was connected to her TPN the entire time due to her severe hypoglycemia. The third hour we clamped her drainage bag so that her stomach would fill with bile and secretions thus simulating “food”.  The fourth hour we administered IV erythromycin, a medication used to stimulate contractions in the stomach to help move food.  This made Lucy very uncomfortable.  She complained of belly pain, was nauseous and retched from the medicine.  The fifth hour we unclamped Lucy’s drainage bag and administered octritide, a motility medication used to stimulate the small bowel.  Octritide is administered via a shot that you have to place in the leg or arm, not very fun for anyone involved.  During the sixth hour an x-ray was taken to check for placement of the manometry catheter, and then we were done with the study.

We arranged for Lucy to have just a g-tube placed after the study.  We did this because we knew the IR department at CHOP does not allow parents into the procedure rooms when they place GJ tubes.  The IR department at DuPont does, and so we much prefer having our gj-tubes placed at DuPont.  Plus after having 12 tubies placed in the IR at DuPont we have developed quite a good relationship with the team there.  Lucy’s stoma, the hole in which you insert her tubies, was tight when we went to put in the g-tube, it took quite a few people and attempts to get it in which made for a dramatic end to a very long day.  Stoma sites can close fairly quickly, even stomas that are as old as Lucy’s.  Because the tubes we used for the study were smaller than Lucy’s typical 16fr diameter tube, her stoma closed in around the other tubes.

Several times throughout the test Dr. B stopped by to see how things were going.  At first glance it appears that the study confirms what Drew and I already know, Lucy has slow to no motility.  The medications we trialed do not appear to have done anything.  Dr. B looked surprised and commented to us that once he interprets all of the data we will need to sit down with Lucy’s current care team and come up with a plan.  Drew and I are well of the fact that Lucy’s GI system has been declining, we did not do this study to tell us that.  We did this study to see if there was anything we can do to improve her very poor motility.  Good or bad we knew that we needed to do this study to rule in or out any possibilities we may have at giving Lucy the best quality of life.  We know there is not cure for Lucy at the current time… but we are always going to hold on to hope.  

Monday, March 21, 2011

A Big Day…

We are heading to CHOP bright (well, it’s still dark outside) and early this morning for a big day of procedures and testing. Lucy is scheduled first thing this morning to be put under general to have an endoscopy and manometry catheter placed for a six hour long manometry study which will begin after she recovers from anesthesia. Please say a pray that she has the strength and ability to endure all that she will today. Also please say a prayer for our little Sophie that she begins to feel better today. She has been sick all weekend with a terrible stomach bug that has wiped our little bug out. I wish I knew how to be in two places at once as I am torn today to stay home with Sophie and stand bedside with Lucy. Thank God for loving and wonderful friends who love our children like they are their own. The big kids are off to school with the Puff’s this morning, and Sophie will be loved and taken care of by Alicia.

I will try and post updates throughout the day…

Wednesday, March 16, 2011

Not again…

Thought I would share a little e-mail conversation between the good doc and I.

Me: Tube’s out…AGAIN! 

Dr. R: Ugggghh…

Me:  What’s a Mama to do?

Dr. R:  Let me get back to you on that.

Frustrating would be the word that sums up my day.  We had a two hour pre-anesthesia appointment for Lucy’s manomentry study at CHOP in which I had to recount Lucy’s ENTIRE medical history to a nurse practitioner, one organ system at a time.  She keep saying to us, “lets just start at the beginning,”….seriously.

Again, just thought I would share.

Another day, Another GJ…

That’s right, Lucy is now the proud owner of gj-tube lucky number eleven.  Lucky that is,  if it stays in place for longer than a day.  Last week after a very frustrating motility week, we made an executive decision to not place another gj-tube, and instead place just a g-tube.  It was obvious that Lucy’s body was not going to allow us to keep a j in place and since we have so many decisions on the table right now as to what direction we are going to go with her motility issues, we thought we would stop all feeds, drain her belly, and try and give her meds via her g-tube.  We knew that things would be uncomfortable for her, but aside from placing a new gj-tube every day, it was our best option.  After a few days of this, our best option was not working either.  Meds were not getting into Lucy, after administering her meds and clamping her drainage bag for over an hour, when you went to unclamp it meds came pouring out into her bile bag.  In addition,  Lucy has so much pressure coming from her belly that we could not keep the extension tubing connected to the g-tube.   It continuously kept popping off leaking bright orange and yellow bile on everything.   The color of her bile was enhanced by her meds just sitting around in her belly making Lucy miserable.

After stain treating everything Lucy wore last week, every shirt I wore last week, every sheet she slept on and blanket she slept with, and spot cleaning what seemed like every square inch of carpet in our house, I contacted Lucy’s doctor and asked if would could try and place another gj in Lucy.  The mess was one thing, but the obvious discomfort our little girl experienced was enough to send this mama over the edge.  If I haven’t already told you how wonderful our doc is, let me just tell you he is!  After stressing to him all that we had experienced, he said to me “I am fine with placing another gj…I think that we should keep going, I am always a fan of keeping going!”  He is almost always optimistic, even when our options are getting fewer and fewer.  The only time I saw him look defeated, was the day he told me that we had no longer had any options with regards to feeds, Lucy needed to have a more permanent central line placed and go home on TPN.    

While placing Lucy’s newest gj-tube the radiologist was able to take enough pictures of her anatomy that we did not have to do an actual upper GI study.  Those of you who have a kiddo like ours, where stuff just sits, and sits, and sits around in the GI tract know that barium, which is used for an upper GI, is not a good thing for a GI tract like our little ones.  I was so happy for Lucy, and well…frankly for all of us that have to be around Lucy, that she did not have to have barium on Monday.  If you let barium sit in the GI tract for any length of time, it can turn to concrete pretty darn quick.  Drastic measures need to be taken to rid the body of the barium, and I for one and Lucy for two hate the drastic measures we have to use to get rid of barium.

We are off to CHOP today for our  pre-anesthesia appointment for the antrodoudenal manometry study that Lucy is scheduled to have preformed on Monday.  I’ll be honest, we are still not sure we are going to do this, but we are leaning towards it.

Sunday, March 13, 2011

“Mommy my birthday…now?”

“Yes, Lucy your birthday is NOW!” (She has been asking me this question over and over again for about the last week.)

How old were you yesterday Lucy?

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Umm, no silly girl…how old are you today?

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No, not quite…

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That a girl,now you’ve got it…now you’re three!

Happy Birthday Lucy Grace!

You’re simply…amazing!

PS She loved the cardboard box that her present came inSmile

Thursday, March 10, 2011

Up and Down and…

I am describing our emotions, they are up and down and all around.  So much has been going on medically in the last three years, but more in the last six months.  Lucy’s motility, the way her GI system moves food, meds, and stool through her body has been declining rapidly in the last six months, but even more so in the last two months.  Really I don’t have the energy tonight to go into too much detail, so I will skim over what we have been thinking about and dealing with recently.  As many of you know in addition to being 100% TPN dependent Lucy has a gj-tube, we use the j port of her tube to give her meds and very small amounts of feeds, and we use the g-port to drain her stomach 24/7.  We drain her stomach because she can not tolerate having anything in her stomach.  It was our hope and desire when we started Lucy on TPN back in October that her belly would  wake-up so to speak, and she would be able to start tolerating feeds again.  This hope is no longer a reality, at this point none of her doctors believe that Lucy will ever get off of TPN.  It is believed that Lucy will not be able to take in enough nutrients via her GI tract and therefore TPN is necessary to keep her properly nourished and hydrated.  Some of you may have already come to this conclusion, but as parents it is not an easy reality to accept. Perhaps by me writing this it will sink into my heart, as I do comprehend the science behind all of this, it’s the emotional stuff that takes more time to process these days.

We are having problems with Lucy’s gj-tube.  Specifically we are having a terrible time placing them and having them stay in the right spot in her intestines.  A typical tube placement should last three months, then you call IR and schedule a tube change.  We are going on tube number ELEVEN in ten weeks, but even worse we are going on tube number three this week alone!  Lucy’s motility is so poor it is moving backwards, pushing things back into her stomach.  In medical terms this is called reverse peristalsis, peristalsis is the movement of the GI tract, it should move down and out if you know what I mean.  In addition, Lucy’s belly does not empty well as she suffers with delayed gastric emptying or gastroparesis, which has progressed as well.  When we attempt to place a gj-tube in Lucy part of our struggle with placement is getting her stomach to empty so that they can thread the tube into her jejunum.  Once placed, her motility is working against us and pushes back the tube that was just placed.  On Monday we witnessed this on x-ray after spending quite a bit of time trying to place a new tube. 

What’s a mama to do?  Good question, after her tube placement in IR and the radiologist giving me a look like how many more of these are you going to put her through, I marched on over to our main doc’s office and shared with him what we had just witnessed and then picked his brain some more about a motility test that Drew and I have seriously been struggling with making a decision about. Why do we keep trying to force feeds?  The simple answer according to Dr. R, “if you don’t use it, you’ll loose it!”  Coating the bowel wall with even the tiny amount of food that we are feeding Lucy is the best way to keep the bad bacteria from taking over.  We are at war with the bacteria in Lucy’s GI tract, and at the present time we are fighting three pretty potent bowel bacteria and yeast.  How are we going to med and feed her if we can’t keep the hardware in her that we need to do this? Another excellent question, and this is where we stand at the present time.  Dr, R is talking with a GI specialist out of the area that works with one  of his small bowel transplant kids, this GI has done a procedure in which they place the j-tube endoscopically and secure it to the bowel wall with a clip of sorts.  We are not even sure that this could be an option for Lucy, but at this point we need to think way outside of the box.  A surgical j, where surgically a separate hole for a j-port is made some where along the jejunum,  is just not an option for our girl because there are too many risks involved with the procedure.  Lucy has intestinal myopathy, her muscles in her intestines are floppy and are deteriorating.  Preforming a surgery in which you rearrange the intestinal wall is extremely risky in a kiddo like ours.

I mentioned above that Drew and I have been thinking hard about a motility study that may or may not yield some information about how the nerves and muscles function in Lucy’s stomach and small bowel.  Our new GI at CHOP who specializes in motility disorders said to us in February that he can not make any recommendations for Lucy until after she undergoes an antrodoudenal manometry study.   It is an invasive study, that may or may not yield valuable information… how’s that for clarity.  Our little one is complicated, and so is this study, two complicated issues make for one extremely complex situation.  We have many things we need to consider and do to prepare Lucy for the study.  A few of which include having an upper GI done next week to even determine if we can do the study, and… oh yeah… our main doc being out of the country while Lucy is scheduled to undergo this study, stress on top of stress…that seems to be our family motto.

I know I stated in the beginning of this post that I was only going to skim over the issues…well, I did.  The issues together are so complex that it is impossible to write about them in one post, so look forward to future posts in which I will try and dissect the matters into easier to digest pieces(pun fully intended).

Tuesday, March 1, 2011

Meds…

Enough for a weekend!   These are just her J-tube meds, we have four more meds that are IV, and four meds that we add to her TPN.  Our wonderful nurse, Helen, helped to create this time saving dosing system.  Just a glimpse of our daily life…

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