Oct 28, 2011

Before/After (Some Graphic) Pics



 About 6 hour post surgery with her new bear Mr. Brown (a gift from her speech teacher)
24 hours post surgery ... lots of swelling. Bruising on inside of lip. Feels minor to moderate pain controlled by motrin/tylenol alternating. 

28 hours post ... still bleeding some from the mouth.  Sounds VERY "stuffy" and both nostrils are filled with dried blood.  Her voice is still very strange sounding (likely from the breathing tube irritating her throat).  She has a great attitude and just spent an hour at the playroom doing arts and crafts but was certainly ready to get back to the room and her bed. I don't have any pictures of her actual graft yet as it hurts a bit too much to touch it. 

In the playroom playing with the pots, pans and food.  (She said she was hungry.)



Oct 27, 2011

Zoe's Bone Graft

Yellow spiderman scrubs. Check.
Hair pulled back in pony tails.  Check.
Overnight bag packed.  Check.
Art projects and movies for Zoe.  Check.
Snacks and drinks for Mama while Zoe is in surgery.  Check.
Excited little girl.  Check

That's our morning so far.  For a few weeks now, we have been talking about the bone graft surgery.  Zoe understands what it involves, what it will feel like and what it means to her.

And why is she excited?  She will finally get teeth!! (More about that in a sec).

So as I wait for Zoe to get out of surgery, I will take this moment to tell all of you what is involved in a bone graft in "regular" words. 

You know how you have top teeth that go into gums and right behind is the roof of the mouth.  Well, if you have a complete cleft lip and palate, you will have a hole or "cleft" where there should be lip, teeth, gums and a roof of the mouth.  At about 1 year old, Zoe's lip was sewed up.  Then at 18 months her palate was closed (with skin mostly). 

But she has no teeth in the area of the cleft.  (Hers is considered quite large and goes almost down the middle, just slightly shifted to the right side and through the right nostril.)  She is missing quite a bit of bone in the roof of her mouth.  Right now, her roof of the mouth is almost entirely skin and that skin gets holes in it called a "fistula".  If there was no surgery done, she would likely never have teeth in the front (she's missing 6 on top). 

Bone continues to grow up until late teens.  Her bottom jaw will continue to grow.  But without a bone on top, that will not grow and she will have an increasingly larger underbite.  Her top teeth already don't match up with her bottom teeth (not sure how she chews, but she does) and this would just get worse. 

Without that bone, she will never have teeth up there either.  Nothing to anchor to.  Oh, and the roof of the mouth is also the base of the nose.  So without that, the nose will continue to look like it is "collapsed". 

So they need to get a bone.  Where do they get that?  From the hip.  And then they sew it into the roof of the mouth. 

The mouth will be sore for a while (as you can imagine).  They will have her on a liquid only diet for a week.  Then another 3 weeks or so of "soft diet".  (Things you don't have to chew ... mashed potatoes, overcooked pasta, etc.)  Last time she was on this type of diet it was for the pharyngeal flap and she was on it for 3 weeks.  And she lost 3 pounds (she was 48 pounds).  She still hasn't gained it back (she's 46 pounds as of today).  Everyone is always telling her how lucky she is to get to eat ice cream and pudding.  Poor baby, she is not a fan of those.  She likes salad and chips.  Like Mama like daughter.  In June we got really creative and will do it again. 

They all say the worst part of the pain is the hip.  Surgeon said the pain will be pretty intense for about 3 days, then really sore for a good week.  Then eventually, the pain will go away and she'll feel like playing but can't.  Nothing rough for at least a month.  (THAT will be hard for her!)  No ball, bike riding, swimming, wrestling, etc.  So she'll be up and around for Thanksgiving. 

They require her to stay home from school for 2 weeks and then it's optional after that. I will send her only when I feel she can stay safe (Like no balls to the face or hip, will eat the right foods, etc).  If not, it's only 3 weeks until Thanksgiving break.  The teacher already sent home work, books, activities. 

The surgery is about 2 1/2 hours.  The surgeon says they usually call after the hip bone is harvested but before they put it in her mouth, just to keep us in the loop of how things are going. 

One cool thing I haven't mentioned is that we are not doing this alone.  A while back, Tara (a friend and fellow adoptive mother) said that her son Clayton is having his bone graft surgery too and wishes we could do this together.  So we thought, Why not?  Asked the Surgeon. She said yes.  Clayton was adopted from China too, is 6 just like Zoe.  He has a bilateral cleft and is in Zoe's book!  So how cool that both of them are doing this together. 

As I was typing all this, Tara and Clayton stopped by the waiting room on their way to surgery prep.  I always buy Zoe a little something for her hospital stays like art supplies.  This time I got her some cool stencils and such.  And we bought the same little kit for Clayton.  Well, apparently, Tara and I think alike and she bought Clayton some origami kits and she brought some for Zoe.  (How sweet!) 

10:05am, got a call from the surgery nurse, "The bone has been harvested".  That news means that the first half of the surgery is done.  She was doing well.  Anesthesia is always a higher risk for her due to her retroverted airway ... and this is the longest she's ever been "under".  But so far so good. 

As I hung up the phone, it hit me just how "not normal" of a sentence that was.  I go through the motions of life, trying to be "present" in every moment.  Trying to suck the marrow of life and learn all I can.  I don't really stop and reflect on how my life looks to others.  But then ... there's a sentence like that ... and it hits me.  My life is not normal. In so many ways. 

11:10  Zoe is out of surgery ... took just over 2 1/2 hours. They were able to get the whole graft from one hip. Lot more bleeding from the mouth than I thought it would be.  Morphine is my friend. Within 30 minutes of surgery, she was already taking sips.  (Did I mention how tough she is?)

For those curious folks, here are other details: she is on an antibiotic for 7 days, a special rinse (2 times a day), has to do saline rinses when she eats/drinks. Soft diet for at least 4 weeks and we'll go from there. (It will depend on her healing.)

Her graft was considered straight forward ... her cleft is very wide but it is unilatereal and I understand that complicates the graft a bit because it has to be pieced. Hers is unilateral so it can all go in as one piece.

She has disolvable stitches. We have a follow up in 1 week, 2 weeks, then we'll go from there.  The hip has tegaderm which is waterproof ... so she can shower but not soak (bathe).

More details to follow as they come.

3pm, the child life team came by to see if she wanted to go to the playroom.  I was SURE she would say no (afterall, she is still spitting blood).  But trooper said YES.  And she walked down the hallway!  No offense meant to those who do not have this kind of child, but ... Man, my kid is tough! The nurses were in shock.  She was going slowly and carefully.  She wasn't rushing or racing.  She was careful and deliberate.  But she did it.  She did a quick art project and then was ready to head back to the room. 

The rest of the afternoon she took turns alternating between being fine and throwing up.  Zofran helped finally.  She has round the clock pain meds going and more morphine as needed.  (So far ... NONE.) 

She's walked down the hall way a few more times.  The nurses have been in shock at her.  Oh, and tonight, the nurse asked for Zoe to open her mouth so she could see inside.  And she opened it WIDE open, just like she hadn't just had mouth surgery! 

We are thankful for the prayers.  We know God hears them! 

Oct 21, 2011

Home again

Thursday morning: "I'm going home I've done my time" ...

that line from that tune from my childhood keeps repeating in my head. And all I can say is "It's about time."

3 weeks ago when we got here, I was worried they'd send him home too early, without seeing the problem. Then Dr. Jolley "got it" and I knew we were good.

Then they worked tirelessly to get us an "adequate source of nutrition" and tried to avoid TPN. He even told me that they would plod along scientifically and he wasn't in a hurry to send us home until *I* felt comfortable with the decision.

And I can honestly say ... today is the perfect day to go home. Not one day too early or too late.

So in about 2 hours, he will be disconnected from the TPN for the drive home. 3 hours home. The nurse and supplies will be there. Training. Reconnected.

And so life with our new normal begins.

Friday night: We've been home just over 24 hours now as I write this. Hospital life and home life collide hard. It feels like one is running clock wise and the other is running counter-clockwise and when I try to merge the two worlds, a tornado ensues.

Like since being home ... Manny took a 2 hour nap on the way home so he wasn't exactly ready to sleep last night. I was ready to collapse and he wanted to play. Then he screamed (pitched a fit) for almost 2 hours as I was about ready to cry I was so tired. Dan was out jogging (he didn't get to do that while we were gone since he had Zoe and couldn't leave her here alone). Manny woke up many times during the night to be repositioned ... he did better in the hospital bed so I might see what I can do to find something more like that for home.

Then this morning ... just trying not to drown in all that has to be done. Steep learning curve on some things. Run around with insurance things. Denials of meds that he needs. Working out appointments. Rescheduling ones we missed in the 3 weeks gone. Getting Zoe squared away. Cleaning up the house enough for nurses to come to our house and not condemn it. Trying to get Zoe over this cough she has. Dealing with children who have been motherless for almost a month. Trying to wade through the laundry that got tossed in the rooms as my sister was trying to help pack up the boys. Scrambling for dinner without going to the store. Bank run. Pharmacy run. Post Office run. pick up Jacob at my parents. 2 nurses to come by today to check on him and check on him. Not help, just ask questions. (That's a few things I did today.)

But on the positive side ... it's 10pm and everyone is asleep. I was able to get the TPN connected and disconnected and reconnected all by myself. (Even got the nurse's stamp of approval.) My kitchen is clean and staying that way. We made a small dent in the laundry. We cleared a path in the girl's room so we could walk in there. Had a healthy dinner of baked fish, rice pilaf, fresh steamed spinach with sauteed garlic, sauteed onions and fresh bread along with raw veggies. (I missed good food at the hospital!)

In other words, I survived the day! One step at a time. Plod through. Before I know it, I'll be back in the full swing of things and doing this stuff in my sleep.

And for now, I already can't believe it was almost 3 weeks gone. The memories are already fading on just how sick he got and how the heck we survived those 7 weeks of torture until we got to Gainesville.

We are all (including his doctors) hoping for a miracle. Hoping that his brain and gut all start working together in unison. We are also all at peace with our decision to go to TPN.

Except for being a little skinny minnie (and a shorter temper) ... Manny seems no worse for the wear. His heart rate is down, he's not retching or coughing or congested. His oxygen levels are perfect. In other words, ALL his issues were related to the food and the inability to process that food! So without food in his belly, life is "good".

Very grateful to all of you! I know most of you read and dont' comment. (That's fine.) I know you pray and you talk about Manny to our heavenly Father. And I KNOW that He hears you. I am asking God to bestow many, many blessings on each of you. You're lifting up this precious little guy before the Throne of God. And I do the same thing ... I might not know you or know your name. But I know God hears your every prayer and I'm asking Him to return the favor.

Oct 20, 2011

day 20 and counting

Look what we woke up to:

Wednesday nothing much happening.

I'm sure there are tons of things going on behind the scenes but nothing that I know of.  As of right this moment, I HOPE we are still going home tomorrow, since I haven't heard otherwise. 

Dr. Novak did come in and tell me all the tests from yesterday were normal.  (Good to know, what we were expecting.)

The rep from the home health nurse team came by to do some basic training (she was a bit flighty and had 6 phone calls during her 20 minutes here).  Thankfully I read the manual that I found online.  So I am good. 

5pm, they disconnected us for an hour from the IV and we got to go outside.  We expected to hang out outside but it was COLD. (OK, cold by Florida standards if you're wearing sandals, tank top and shorts.)  It's been in the 90's ... but the cold front came in yesterday.  Guess I should have looked at the weather before going out!  (But 20 days inside and you don't really think of weather!) 

Before going to bed, he saw the Buccaneer's shirt and said, "Pirate".  Kaley got the bright idea of making him a hat.  He wore it for a few minutes but was generally not a fan.

At one point I left the room to get some ice and when I came back, I saw the most angelic thing.  I stood for a minute, just watching.  Trying to absorb it all in.  I tried to capture the moment in my mind, heart, soul and being.  Pictures could just never do it justice. 

Oct 18, 2011

The past 48 hours

Someone asked how long that Manny has had this problem.  Truth is:  Parts of this he has had (in varying degrees) since we got him at 9 months of age.  (I have no clue about 0-8 months.) 

He's has troubles aspirating, retching, (and a whole long list of symptoms) since then.  There have been days and even weeks when all was "fine".  But those days and weeks have been few and far between. 

It's gotten progressively worse over time and it got REALLY bad in mid August.  He was in a lot of pain, screaming, retching, etc.  It's been unrelenting since then.  Life has been sooo difficult.  Hard to watch him choke and suffer. 

Sunday, the TPN "kicked in" because he woke up "himself"!  He truly was back to his old self.  He was laughing, playing, engaged.  It wasn't until then that I realized just how far down he'd slipped.  These 2 1/2 weeks in the hospital have been VERY hard on him and I didn't even realize just how difficult.  But to get him back fully now, the contrast was stark. 

When the docs walked in Sunday, he was playing with his letters.  He knows the name and sound of most every letter.  He also knows all his numbers 0-9 and can count up to 15 objects consistently.  He was showing off for them! 

I was THRILLED that the grumpy baby was replaced by my happy baby!

And now Monday starts the work of trying to get things arranged so we can get home.  (That will take a lot of work, by the way.)  We'll need so many new supplies, machines, nurses, meds, etc.  EVERYTHING has changed. 

Monday - 1:30 pm: Dan, Zoe and Kaley get here.  We hung out and had a fun time.  Manny even got the okey-dokey to go with us downstairs for lunch.  They disconnected the TPN for 30 minutes and we headed down.  It was so nice to feel "normal" again, if only for 30 minutes.  We were sitting at a table, all eating lunch, chatting, being a family (well, part of one).  Forgot to take a picture. 

4 pm:  Dr. Jolley came in with the results of the biopsy:  Basically normal.  (Nothing that would account for his strange problems he's had lately.)  So we will start changing him to what they call "cycling" his TPN (which means some times he will be on and sometimes off of the feeds vs the 24 hours a day.).  If that goes well for a day or two, we can go home.  Meanwhile, they're working to get our supplies, meds, nurses, etc. set up for when we get home. 

5 pm: Dan and Zoe head home.  Manny was not happy.  He's fine now but was not ready for them to leave.

It's nice to have Kaley back too.  She keeps me entertained and by having her here, I eat.  I think of her needs and then realize I should probably eat too so I do.  She is also a lot of fun and can help play with Manny so he's not so bored with me.  (I only have so many tricks ... and after almost 3 weeks, I'm about out of tricks.)

Monday night ... all is going well.  He basically sleeps through the night with a few "Help me Mamas" along the way (His way of asking for me to reposition him).  I cannot begin to tell you how nice it is to go to bed NOT terrified.  I am not afraid of him choking and retching and aspirating and desatting.  It's still hard to believe now just how bad it got and for how long and how we survived it!  And now to think that is over. 

We now begin new things to watch for and be concerned about and be vigilent about.  But those days are over and I couldn't be more thankful!  Like I've said before, we didn't come to this decision to stop feeding him (via tube) lightly. 

Tuesday, 9:30 am, the nutritionist Mackenzie came in.  She's been consulted on the case since the beginning.  She has been the one researching and tracking down these strange and unusual formulas.  But we hadn't met until today.  So it's nice to put a face with the name.  And she was happy to meet Manny too. 

10:30, Dr. Novak just left.  He had a million questions quick fire style.  He thinks we're on the right track too.  He wants to order another gall bladder ultrasound (I told him they did one in September but he wanted another one anyway).  And tomorrow, when they draw blood, he wants to add a pancreas test.  These have already been done so we're not expecting to have new results.  He basically said he felt that due to the circumstances, there is probably nothing else that can be done to keep him feeding via the digestive tract.  I told him that I was at peace with the decision.  That I knew the team did EVERYTHING and I trust the decision.  (Not that I like it, just that I'm at peace.)

12:30, Ultrasound of the gallbladder, pancreas, spleen, kidneys and bladder... not sure what they are looking for in all of those body parts.  And now they've ordered a urine sample.  Since he can't go on command, they will have to affix this sticky bag around his pee to collect it.  (He is not a fan.) 

12:45, Dr. Lawrence asked if I am comfortable with my pediatrician monitoring the TPN for Manny.  Yes, she is awesome.  So that means they're starting to get a few ducks in a row for life outside of here.  That's great news.  There's still a lot to coordinate - machines, medicines, feedings, nursing, supplies, etc.  And once I get home, I have to learn how to use the machines, set up the TPN, change the dressings, do the sterile technique for all of this, monitor his symptoms, etc.  EVERYTHING has changed!  Lots to learn. 

1:30, Discharge nurse was working things out with me and letting me know what is going on.  She feels that (unless something changes between now and then), we will get everything wrapped up tomorrow and then be discharged Thursday.  We have almost 3 hours home (we usually have to stop on the way to help Manny move around) and then several hours of training from the home health nurse Thursday afternoon. 

3pm, The rep for the home health nurse and the pharmacy that will deliver his feeds and supplies came by to chat and arrange a few things.  THIS is what they do so they specialize in it.  And there happens to be a pharmacy right in Tampa so if there was ever a problem with supplies and such, they are local (which is a huge plus).  And they are all over the country so if we ever travel, they can arrange to have supplies sent to where we travel. 

The rest of the afternoon was quiet.  Love having drama free days and nights!  And I'm soaking up the last few days of "calm" before the proverbial storm.  When we're discharged, we hit the ground running.  Load up the van with 3 weeks of stuff we've accumulated.  Drive 3 hours home.  Immediately get trained by the nurse on what to do and how to hook up and disconnect his feeds.  Try to get readjusted.  The kids all have Friday off so the boys should arrive some time Friday morning.  It's trying to get used to the new schedule.  Try to reconnect with the other 4 kids (the boys I haven't seen in almost 3 weeks!)  And try to keep Manny away from Zoe who is currently ill.  (Hacking up a lung apparently and too sick to go to school.) 

Whew!  Better get to bed and rest while I have the chance. :)

Oct 15, 2011

Some explaining

Thanks for the prayers.  The calls.  The emails.  The comments.  They mean a lot to us. 

I should address a few things and clarify a few things. (Tired Mamas don't make themselves as clear as they think they do.) 

Manny is on TPN via a PICC line.  This is like iv solution but has more to it than just the water and electrolytes.  People can live on it for years.  The PICC line is in his arm but threads to near his heart.  They put it in via an ultrasound.  Then they check the placement of it with an xray machine.  His was 2 centimeters too deep so they had to back it out and rebandage it. 

(And for the record, they said he was a very difficult stick even with the ultrasound ... no wonder those nurses have a hard time with IVs.)  They gave him a little ativan for the anxiety and that helped a lot.  They let me in and I just had to wear sterile outfit like the medical team. I wasn't prepared for all the blood and how long and wirey the picc line would be. 

For now, there is nothing going in his GJ tube.  No formula, no pedialyte, nothing.  Even meds are going to be given via the PICC line for the time being. 

The docs want to keep looking for solutions for why his bowel isn't functioning.  I am ready to say enough is enough for now. 

A person can use a PICC line for about a month before it has to be changed out. So for Manny, they might keep the temporary one and then eventually change to a more permanent one but not sure when that would happen.

At some point, he will end up with a port (under the skin) near the collarbone.  I had said before that it goes into an artery and I misspoke that.  It goes in a vein still. 

Next, many people are being very helpful in suggesting types of formula and alternate milk sources to try.  So here is a list of things we've already tried.  (This might not be all of them as I'm doing it off the top of my head.) 

Pediasure, Peptide, Peptamen Jr, Peptamen Jr 1.5, Elecare, Compleat Pediatric, Pedia Smart, E028 Splash, Soy, Almond, Rice and Breast milks.  They all have the same effect ... just to varying speeds and intensities. 

The only thing that was going down was Pedialyte and even that started to have some troubles.  That's why they chose TPN for him.  The only conclusion one can draw is that the brain has stopped telling the bowel what to do.  It's in essence unusable.  This is NOT a part of what typically happens to kids with his disorder so it's something unusual. 

His condition is very serious and complicated.  He will live though and no way to tell how long before this starts to affect his liver or get an infection.  We're also concerned about the tapes ... they have to bandage this thing onto his arm and it's a lot of tape to hold it in place.  He's so sensitive to them ... that can be a source of infection in itself. 

So like I said ... this is very serious. 

And to state the obvious (some people weren't understanding this).  He eats and drinks NOTHING by mouth.  He hasn't since 8 months old and they feel he never will gain that ability.   He was being fed into his stomach until that quit working.  Then he was being fed into his bowel and now that has quit working.  So the only other choice for nutrition is via the veins. 

Oct 14, 2011

So very hard

Thursday we did an Upper GI Endoscopy, expecting normal results.  All went well with the procedure and the doc said all looked normal.  He took a biopsy of the stomach and duodenum just to be sure but it was as we expected.

Today, Friday, we're going to switch to TPN ... they're calling it temporary.  For now.  As I type this, I am waiting for the doc and his thoughts today.  I know yesterday he seemed a bit discouraged, like we were running out of options. (I already knew this.)

You see ... God prepares my heart and soul for the things to come.  I'm not sure if everyone has this experience but I do. I get "feelings" of what's coming and it's a way to get adjusted to it before it happens.  I've learned to listen to this little "voice" (no, it's not audible).  For many, many days now, I have had the feeling that we're headed to TPN.  I know they are saying it's only temporary, but I have that feeling.

So yes, I know that this is what needs to happen.  Yes, I know it will make certain parts of life easier (no constant IV sticks, easy blood draws, etc.). I am aware that this is going to make the retching stop and probably help him not aspirate.  He will not need the salivary surgery.  It will be easier on his heart and respiratory system.  Life improves! 

So what's the problem?  It's another loss.  I can hardly breathe when I think of the things Manny has already lost.  Here's a short list:  A birthmother, a birthfather, his native culture, his native language. He lost the ability to drink from a bottle or eat with his mouth, most of his stomach, then all of the use of his stomach and his bowel. He cannot move. I don't know when it will hit him but some day I'm sure he will be not very happy about not being able to run, jump, climb, play ball, etc.

And yes I know that he's gained so much too! I get that. I'll have to write that blog some day but for now, another loss seems HUGE and I am not able to do anything to stop it.

And today's loss is ... another loss.  It's a huge step in the WRONG direction.  When he went to G feeds, all he had to do was gain the ability to swallow again and he could again eat.  The he lost his stomach's functions and he had to gain swallowing and get the tummy to work again.  Now we've lost bowel and he'd have to get ALL of that back just to eat. 
I know kids who have lost all this and are perfectly FINE with it.  They don't care.  But Manny DOES.  He is always asking to have something to eat and drink.  He WANTS food.  I've started hiding in the corner of the hospital room so he doesn't see me ... it's soooo pitiful.  He has never been able to eat this food but he often asks for a hamburger and chicken nuggets! 
There's a part of me who is greatly disappointed in the fact that our ways are not God's ways.  HE could heal Manny in a nanosecond.  He could touch Manny and we walk out of here and grab that hamburger on the way home.  So I get disappointed when our strides are backwards. 
His disease is not a progressive one.  We're not supposed to lose functions.  This isn't typical with Merosin Deficient Congenital Muscular Dystrophy.  So something else is going on with Manny too.  Because this has certainly been progressive. 
And with each new loss, I wonder ... what's next to go? 
11am ... Doc just left ... said we'll try the TPN, slowly crank it up and see how he tolerates it. He still hasn't given up on using the J for feeds, he is just not sure what else to try yet. He said, "I'll be on call all weekend" ... which is code for we're not leaving any time soon. I'm considering how much longer I should let this continue. My intuition says it's almost time to go home and get on with our new normal.

In a little bit, Dan is coming with Zoe for a quick visit.  Then he is going to take both girls (Zoe and Kaley) 2 hours away to visit his sister and her family.  Kaley has been quite conflicted.  She wants to stay here and help me but also wants to go have fun and see her cousins. 
I have left the decision completely up to her.  Why?  Because I NEED her here.  Our lives are so much better by having her here.  I can take a shower, go get lunch, get some hot tea, etc. without Manny completely wigging out.  She is also wonderful at playing with him. 
At the same time, she's a kid.  She deserves to have fun and to get outside and play and have a life.  And I want that for her.  
With much distress, she chose to go with Dan.  So we packed her up.  
This is going to be a long, quiet weekend. 
2pm Dan and Zoe arrive after a longer than expected trip due to traffic.  Just as they got here, they were doing the ultrasound on Manny's arms to see if they could find a decent vein to use.  He cried so hard that they decided to give him a little something (ativan ... lowest dose possible) to help his anxiety.  
I was allowed back in the room with him and he cried the whole time.  It was less crying than with the IV sticks (due to the meds on board) but you could tell when it hurt.  This team does basically only PICC lines and the main person said this was a HARD stick.  Even with an ultrasound to SEE the vein, she was having a hard time.  He has so very few veins already and then they're weak and "spindly".  She believes it's because of lack of use of his arms and legs.  The veins are atrophied.  
Much blood and many tears later, he has his line in.  We got back to the room and he just hung out with Daddy for a while.  Daddy is not the type of man who cries a lot or easily and sorry to out you Dan, but I could see tears just inside those eyes.  
Well, soon after (about 4:30), the sedative drug knocked him out and he's still out at 9:30pm as I type this. (wasn't supposed to do that).
6:30 Dan, Zoe and Kaley left. Kaley was so conflicted that I finally had to just basically tell her that Manny and I would be fine.  She deserved to go have some fun.  I could tell she was about to cry as she left.  She wanted to be here for us.  I assured her we would be just fine and I'll tell her all about what she missed. 
So now I am sitting in a quiet hospital room.  Since Kaley has been here this whole time, I have had some one to talk to and play games with and chat with.  We just hung out after Manny would fall asleep at night.  Tonight, the silence is very stark.  
I guess I need some reflective time.  
I have been holding my very weak baby wondering where we go from here and how much more I can/should put him through before we say "Thanks for trying so hard, but it's enough."   
So very hard.

Oct 12, 2011

Meloncholy Wednesday

Here is my Facebook status from Tuesday:

I haven't written a blog for the day ... I just can't make myself put my thoughts down. Why? Because we're starting to have some difficult talks with the docs. We're not out of options .. but getting close. And with each test, it seems to indicate that another body part has stopped working. I'm thankful he's alive. I'm also deeply saddened by his suffering. I'm reminded that God keeps EVERY tear in a bottle (Psalm 56:8) so that means all of mine and all of Manny's and all that you have shed on our behalf. It counts for something.

What's new?  Nothing.  That's the problem.  One thing after another they have tried in hopes that something will work.  When the truth is ... nothing is working. 

The truth is ... they have no clue what is wrong with him so they have no way to fix it.  I point blank asked the doc what he thought was going on ... liver? gall bladder? other?  And he said, "I think it's the bowel".  I asked, "Are we talking serious dysmotility or is the bowel dying?"  And he just shook his head yes. 

They still want to try a few more things before we go to TPN.  Many people are curious/confused about what this is. 

Total Parentral Nutrition = TPN

OK so entral feeding is the traditional way ... the digestive system (like mouth, esophagus, stomach, bowel) or where the food goes into any of those places.  (Like G tube feeding is straight into the stomach, where J tube feeding goes into a part of the bowel.) 

So TPN is where it bypasses that entirely.  It's similar to IV fluids (most people are aware of that).  But instead of going into your veins, the go into an artery.  There is something called a PICC line which is where they put TPN into your arm.  This is best for temporary access. 

TPN typically goes into a Central Line (in your chest, near your collarbone).  And there are several types of access.  Some are above the skin and some are surgically implanted into your skin.  You'll hear about "ports" and such.  The type of use will determine which type of access they use. 

Now the entral feeding takes the whole digestive tract to help deliver nutrients and eliminate waste.  Versus with TPN, it all is filtered through the liver ... which makes it very hard on the liver. 

It also goes right into a major artery so if there were to be an infection at the site, it's extremely dangerous as it would immediately be into the blood and the patient becomes "septic". 

They do a lot of monitoring of body function while on TPN and you're under close watch (which is good).  We might even need to consider some level of home health nurse just to be sure. 

Oh, and you can't get it wet so no showers or baths or swimming.  (We had just signed up for some water therapy.  We live in Florida for crying out loud.  We go to the beach and we have a pool.  So this was news that bothered me too.) 

Finally, the big deal about TPN is ... for him, they feel it would be quite life shortening.  He'd stop retching so quality of life goes back up, but eventually, it would become a problem and too hard for his liver to take.  So they are taking this very very seriously before they go this route.  And I appreciate that.  If we go to TPN, I need to know we tried everything else first. 

Here is my Facebook status for Wednesday morning:

Thanks for all the prayers and kind words to my last status. Last night was rough ... he normally sleeps but he didn't, he screamed a lot. I think I'm most struggling with the feeling of "impending reality" versus wanting him to be restored and healed. And I feel trapped between those two worlds. Then throw in the fact that I don't want him to suffer (and he is) and my Mama's heart can't bear to watch him struggle. He still has a lot of "happy" moments too, mostly those. But the bad is bad and I stand by watching, helplessly, uselessly with uncertainty of how to proceed. And just wanting to wake up from this nightmare.

So why is this so "heavy"? because it means another body part has shut down. We're going backwards.  I am completely good in knowing God is kind and loving and merciful and still in the healing business today.  We are still standing in faith for that miracle (or series of miracles). 

I also know that God sees the big picture and we only see in part. 
I also know His ways are not always our ways.
I also know that Manny is in the palm of the Lord's hands. 
I know that live or die, Manny has a glorious testimony.

So what's the struggle?  I am his Mama.  If I don't fight for him and for his life and his quality of life ... who will?  It's my God-given job to feel every birth pain, to cry when he cries, to hurt when he hurts.  It's my responsibility to protect him (so imagine how I feel every time I subject him to another experiment or treatment or IV stick ... I feel like I'm letting him down, though I know it's not true ... but it FEELS like it). 

I am the one here in the middle of the night when he yells out, "Mama, help me".  I'm the one here when he says, "I mad" when they're doing something painful to him.  I'm the one who tries to help him breathe again when he's choking.  And I'm the one to console him when he can breathe again and says, "I choking."  And I can't explain to him why, just try to comfort him. 

And I wouldn't trade this job for anything else in the world.  It's just that some days it feels like I didn't get all the manuals for this.  I didn't get all the super powers I clearly need.  I missed the memo about how to proceed.  I wasn't equipped with a heart guarding vest that I feel I need some days.  I didnt receive the extra brain power I will need.  And no one told me to buy stock in Kleenex. 

But what I was given was a love for this little man beyond words. 
And THAT proves I'm the right person for this job ... I'm his Mama.  So I suck it up and get on with another day. 

Oct 11, 2011

Another Theory Bites the Dust

Failed experiment after failed experiment. 

That's been the past few days.  They are testing out theories.. and one by one, Manny proves the theory wrong. 

Painting Lightning McQueen ... he is a very neat painter!

Today's failure was the most intense of all.  We tried him on a food based formula (compleat pediatric) and I had to shut off the machine and call for the nurse.  117 ccs had gone in and 100 had come out.  Then he started retching ... badly.  Once it even came up in this throat.  How do I know?  He said, "Yucky!"  He had a hard time catching his breath.  He was screaming OWIE and pulling up his legs (best he could). 

Apparently, the nurse called he Resident and he called the Head Doc.  Both showed up in the middle of this so they could SEE what was happening. 

A few minutes later, Manny collapsed and just basically passed out.  Even his blood pressure bottomed out.  (75 over 50).  As I type this, he's still sleeping it off.  He's back to pedialyte. 

The doc said he has no clue what else to try at this point ... but he'll come up with something.  He was very intense as he said this.  You can tell he is taking this as a personal challenge.  (I think that it helps that the morning and every other day, Manny has been a real flirt.  He's quite charming and engaging.  When he is on pedialyte, Manny is "hiself".  But ... to see him like this?  Your heart just breaks for him!  You MUST figure it out and get him back to his cute, charming self. 

The nurse came in and said that they are going to restart his IV fluids ... just for extra calories since he's losing weight.  His foot vein hasn't been used in a couple of days but they flush it periodically.  She flushed it and all looked great. 

About 30 minutes later, he was crying about pain again and this time ... it was his leg.  Sure enough... his leg was infiltrated.  That means instead of the fluids going IN the vein, they go around the vein and into the tissues.  That's not good.  It's quite painful.  His leg is swollen up to his knee and hard as a rock. 

That also means we needed a new IV.  One hour and 3 pokes later, they got one.  In his thumb.  You're getting desparate when you resort to thumb veins, trust me.  And I think they're now officially out of veins to try.  We might end up with a port or a PICC line by tomorrow anyway just for the veins. 

Today has been particularly hard, depressing and agonizing.  He's suffered a lot today.  And I wonder how much more I can/should put him through.  When do I call it and say enough is enough?  I'm getting there.  But with every new day is new hope that THIS will be the solution.  So we plod on.

Oct 9, 2011

Cute Manny Things

OK ... so tired of talking about the stupid medical things (nothing's changed ... they have no solution and they're still trying). 

So how about telling you a few new things Manny's doing while we've been here? 

When a person comes in our room that we haven't met before, he immediately says "Hi.  I Manny" (and puts his hand on his chest).  Then he points to me and says "That Mama" and then to Kaley, "That Kaley" (or sometimes he says "That Sissy").  He just started this in the past couple of days.  It always gets a great reaction from the new person!

Next, he apparently likes our door closed.  If someone walks in and doesn't close the door behind them, he will tell them to close the door repeatedly until they close it.  He says it kindly but insistantly!

This morning we were playing with some Uno cards.  I do different things with them like have him match colors.  This morning I was having him identify the numbers and them match them with another one like it.  I'd hold up a 7 and he'd say 7 and then I'd have him hand me all the 7's he could find on his tray.  We got to the "reverse" card (has 2 arrows on it).  And I asked him, "What is that?"  (I expected him to say he didn't know.)  He said "That way".  Isn't that cute?  I think that's a brilliant way to describe an arrow.

During one of his recent choking episodes, the nurse was in here with us and she was trying to get him to talk and see how he was doing.  She asked, "You OK?"  Once he could talk again, he said, "I just choking".  (Isn't that just soo stinkin' pitiful?)

He can count to at least 13 without missing any numbers (counting objects). 

He can recognize many of his letters and most of the numbers.  He knows the basic colors.  He knows the basic shapes (square, circle, heart, smiley face, triangle, etc.) Oh, and when he sees a triangle, he calls it a "Chip, a triangle"  ... Does that tell you I must eat a lot of tortilla chips!?!   :)


Now about the medical stuff.  In summary, they gave him a day off on Saturday.  Sunday at noon we started with 10% formula and 90% pedialyte.  He didn't handle it very well either, but not as bad as the full strength.  So we decided to try it again for his 6pm feeds ... we're still in the middle of that as I type.  Clearly, his body is bothered by "something" ... fats? proteins? carbs? combo?  and we'll have to sort that out.  We have a looong way to go from where we are to where we need to be.  And I appreciate the team is sticking with this and going to proceed until we figure it out!

Dan and Zoe drove the very long way up here to see us.  It was good to see them but also very hard ... makes us all realize how much we're all missing out on.  And when it was time to go?  Let's just say there were tears.  I plod along here, putting one foot in front of the other.  But the truth is ... it's hard. 

Oct 7, 2011

Formula doesn't add up

So in summary, Manny did great on IV fluids (his baseline) and he did the same on pedialyte (with only a slight increase of saliva but not a biggie). Thursday night they were going to switch him to formula at the midnight feeds.  I asked if we could switch it earlier at the 6 pm feeds so it would have more time to observe him.  They really thought he was going to do fine and we'd be discharged today ... I had doubts since they hadn't addressed the milk in the wrong place yet. 

6pm feeds ... within a few minutes, he was already showing signs of distress.  By 7 he was coughing. For the previous day on pedialyte, he had nothing in his stomach that needed to be vented.
7:30, he had 55 ccs of bile in his stomach returns.  8pm 50 ccs stomach acids.  11pm 50 cc milk. (Stop feeds at 11)  11:30 another 40 ccs milk. 

The on call peds doc came in and evaluated him.  She said not to resume the feeds and that she'd call the on call GI doc.  They all agreed just to go back to the pedialyte and we'd reevaluate a new plan in the morning. 

He never got horribly bad ... but it was bad enough.  They didn't want him to totally fall apart when they didn't have to.

By midnight, all his symptoms were gone and he slept back at baseline.  Oh, and from midnight until now (11:30 am) he's again had nothing that needs to be vented in the gtube.  I'm telling you ... it's night and day with him on and off food. 

So there are two questions to be addressed.  1) How is milk ending up in the g tube when that is not "possible"?  And 2) What can we do to give him a reliable source of nutrition? 

Waiting for the plan. 

The plan came today in the form of more questions.  I'm smart enough to have done the research to know what this eventually comes to if we can't find feeds that work ... TPN... which has it's own set of new, very serious problems.   So basically that means we'll try ANYTHING to make sure we exhaust ALL the options prior to giving up.

I'd done some research that seemed to indicate it's possible that his reaction is allergy.  He does get congested instantly, etc.  So I asked the Doc... is that possible?  He didn't think so based on the symptoms but certainly worth a try until we can figure out a solution. 

Meanwhile he's on pedialyte only.  So he's hydrated but getting basically 0 nutrition and 0 calories.  Can't sustain on only water for too long.

6pm... started the new formula. One hour in, we already knew it wasn't going to work.  Same reaction as last night (just minus the congestion). 

Here's the biggest symptom of concern.  We put formula in the J tube.  It is supposedly not connected to his G tube in any way (as verified by several xrays).   So things put in the J should not end up in the G.  Why is it bad to have things in his G?  Because his G (stomach) doesn't empty.  So I even have to drain any stomach acids, saliva, etc.  And that is MINIMAL. 

For perspective, in the 18 hours today that he was on pedialyte, I drained 20 ccs of liquid and 750 ccs went in during that time. 

But once on formula:  in the 3 1/2 hours he was on formula, 149 went in and 168 came out. 

So he's LOSING fluids by being on formula?!?  How is that even possible? 

By 9:30, they called it a failed experiment and turned it off and back to Pedialyte.  We'll regroup tomorrow. 

Oh, and just for fun, about 4pm, he started scratching like mad.  I took off his hospital gown and sure enough, he was covered in a rash and some hives.  The only change that has happened that could do that is a change in his motility drug.  (It's a low dose antibiotic.)  The one he switched us to is "Virtually" the same.  But clearly, it's different to Manny!  So we got benadryl ... and he got GRUMPY PANTS.  Yikes. 

So poor little guy ... he's had a 24 hours. 

Many of you have asked what has to happen for us to leave the hospital ... he has to have a way to eat that doesn't make him sick.  Sounds easier than it is. 

Oct 6, 2011

Then and Now

How's THAT for a blast from the past?  This was Manny at 11 months old, only 6 weeks after we had him.  It's our first trip to the hospital, the one where we were told to make funeral plans and went home with Hospice and a DNR. 

Thought it was about time to remind everyone just how far he's come since then. And we can never stray from the knowledge that God the Creator of Heaven and Earth needs to be praised daily for what He has done in Manny's life.  I truly believe to the core of my being that Manny is still drawing daily breath because of the prayers of the Saints. (Most of whom have never met him and only know him through this blog.)  I am overwhelmed! 
Now about today.  The operating theory is ... what if he can handle some food and some tummy rest?  So we had to verify if he could handle pedialyte.  So for the past 24 hours he's been on pedialyte and YES.  He can handle it.  He's the same on pedialyte as he is on IV fluids (which they consider "baseline"). 

So now we have to see how much formula he can handle before he has troubles. 

6pm we started formula.

7pm, he's already showing signs of "distress".  (oy!)  For those who want to know those signs, for him: it's excessive sweating (head), 60 ccs of stomach contents (in one hour vs. 0 in the past 24), increased saliva, coughing (likely due to increased saliva).   Also his heart rate is already up 20 beats per minute.  (He was about 110 and now is in the 130s.) 

Meanwhile, many of you have asked about Kaley and how's she's doing.  First, she is amazing.  I couldn't possibly express the gift that she is to me.  I can leave the room to get ice and water without him screaming.  I actually left the hospital for about an hour to go to Target to get a few supplies (like toys, food and a bed for her).  And I felt TOTALLY safe about it.  I would have NEVER been able to do that without her.  (I'd just do without.)

Kaley is homeschooled and I found the most marvelous book in the gift shop that has all the subjects in it and she thinks it's a game book (so shhhh ... don't tell her).  We're having a blast with it and she's staying on schedule basically with curriculum this way. 

She also plays with Manny.  They have so much fun together.  Bubbles, play doh, stickers, trains, etc.  She's amazing.  I think she thinks it cool to have a little "Mommy time".  She'll be 12 in a month and I think we'll always remember these times together.  They're not completely torture to her ... she actually WANTED to come.  (And I try to make it pleasant so she feels she made a good decision.)  So when you think of us, pray for Manny's healing and my wisdom/decisions.  But also pray for extra blessings on this special little angel we call Kaley. 

Every now and then (after a week in the hospital) you get a little punchy ... here's her tonight with her "Olive eyes". 

Oct 5, 2011

Already been here 5 days ... can you believe it?

Not much happening today. 

Dr. Lawrence (GI) came in to check on Manny's night.  He's stable but there are definite noticeable differences of Manny ON food and Manny OFF food. 

These are symptoms that were here when we arrived, disappeared when the food was stopped and returned when the food was resumed. 

Excessive sweating (especially the head), increased saliva (enough that he is pooling and trying not to aspirate again), choking, crackling/wheezing in the chest, increased heart rate, increased respiratory rate, mouth breathing.  Now none of this is dramatic yet... but it's certainly noticeable. 

We had a visit from some of the Gator athletes.  Swimmer, volleyball player, soccer player, soft ball player.  They couldn't come in the room so they stood in the hallway and we brought Manny to the door in the wheelchair.  Manny was NOT happy.  I think he thought they were going to do poking or prodding or something who knows.  Then I told him that they play ball and he said, "PLAY BALL ... OHhhhhhh".  And he started "dancing" in his chair.  Very excited.  They gave us a poster (a volleyball one since I played volleyball in college too). 

Later in the day this lady, Margaret, comes in and asks if I know Elsa.  (I've mentioned her many times before ... she and her hubby Rich always bring us Balloons and Fruit when we're in the hospital!)  Anyway a mutual friend of theirs called and asked if they could bring their therapy dogs to visit us. 

So "Bud" and "Weiser" (HUGE golden retrievers) were at our door.  Now Manny couldn't touch them and vice versa.  But they got to see each other.  They were panting quite heavily and it was hilarious when Manny would imitate them! hee hee heee

About 4pm, Dr. Lawrence came in and was sooooo excited ... they have a plan!   They'll do some higher calorie formula, some IV and some pedialyte.  They'll also do some basic blood tests while they're at it (since he has to get a new IV anyway).  I'll be interested to see if the pedialyte has the same effect as the formula... so we'll know tonight. 

Once we find a combo that doesn't stress him out, we can go home.  I'm sure we'll have to tweak things along the way.  I love that this doc is concerned that he's stressed at all by the food.  Manny should be the same ON and OFF food.  And right now, that's not the case.  If the volume issue can fix things, that's easy.  If not, we move on to tougher things like TPN or Gastric Stimulator.  We also still might need to have that salivary surgery since airway is the most important.  We'll see. 

Listening to music on Kaley's PSP
So at 8pm the night nurse came in to take some basic blood tests (they hadn't done that yet this trip) and reinsert the IV.  It only took 5 nurses and 3 attempts before they got it!  He was amazing. 

About 10pm, I noticed the slightest amount of formula in his stomach.  How ironic because we're taking him off formula and switching him to pedialyte over night anyway.  So ... since it's clear, not sure how I'll tell it's saliva vs. pedialyte. 

Oct 4, 2011

Help has arrived

The night Surgical resident just left. he's the same one for the past 3 nights so the only consistent doctor we've seen. His dr jacket actually says ENT on it and he's on a surgical rotation. So I tried to impress him with my ENT knowled...ge (like last week and I went to the ENT and they suggested tying off the Parotid glands and removal of the submandibular glands) and it piqued his interest. Also, tonight Manny's already increased in his salivary output just in the few hours he's been back on feeds ... enough so that this ENT noticed it too. I then threw in my story about Zoe having a parotid gland duct fistula (apparently that is soooooo rare, less than 100 in the world we're told). And every ENT so far has been very impressed with that... this one was no exception.

So after I buttered him up with my ENT knowledge, I told him that I was usually happy with the conservative approach but in this case, we've BTDT and this isn't going to get to the bottom line issue. I also asked him questions about his thoughts on the articles about foregut dysmotility in neurologically impaired children. (I knew he didn't know about those studies, but I also knew this would send him to the internet to look it up.) If he does look it up, he can be the "hero" tomorrow at rounds when he has this bright idea. I won't even out him if he does!

I tell you ... leave me in a room for too long and don't let me leave and I'll come up with all sorts of devious plans apparently!

5:30am, I hear this doc outside of our room talking to a group of people.  Then they leave.    I knew what that meant... we were no longer a surgical consult.  I had a feeling (and later it was confirmed) that they recommended our discharge. 

7:30am, 3rd year resident comes in (same one as yesterday).  I knew that he would be in charge of this case during rounds so I gave him all the good info to help him out. 

10:00am, that resident came and got me and asked me to join them for rounds in the hallway.  I walked out and there were like 20 doctors and nurses.  Some I've seen and some were new.  The resident presented the case and did a nice job.  I was silent.  The main attending was right beside me and she starts talking "out patient" following of Manny... ie. Discharge and trying a few simple things that we've already tried.  I bit my tongue.  (I already had Plan B: To stay nearby and bring him back when his stomach freaks out again.) 

Then this new man pipes up and says, "I'm GI".  And I said, "Hi GI".  He started addressing the attending that it's premature to send him home without a reliable feeding source.  YIPpEEE!!  Someone "gets it".  He says to the attending, "Let's assume Tampa tried all those things, right?" (looking at me) to which I replied "Ad nauseum". 

He asks me multiple questions and we start a dialogue between the two of us.  He can tell I know a thing or two, that I have tried many things that they were suggesting and that this is not straight forward.  Everyone else is just standing listening to the conversation. 

He decides to try him on a new med.  I ask about the effects of it on his c-diff.  He asks about his heart. I tell him we have a cardio consult for next week.  He orders an EKG and echo.  The attending suggests that he seems to be stable (to which I replied that holding feeds is a MIRACLE CURE).  He caught my tongue in cheek subtle passive aggressive reference.  He said that there was no need to recreate the horrible symptoms.  More questions.  

Eventually, he attending again asks how soon until we can be discharged ... and he says that he wants to observe him for at least 2 days with the new meds (inpatient).  Then if there's no effect, he will consult surgery Dr. Islam for a consult for a possible gastric stimulator (often called a gastric pacemaker).  And it wasn't until THIS POINT that the rest of the team started to catch on ... OH ... there *IS* something going on with this baby!

Eventually, he and his assistant and I go into our room where Manny is and he closes the door - leaving all the rest of the docs outside.  It was at this point that he started asking me about Merosin (and at this point, I'm the best expert he's got as he's never heard of it of course).  I offer what I have such as the contact info for some of the Muscular Dystrophy docs (including the one who wanted me to see THIS doctor), the standards of care article, the doctor from LA I've been emailing, etc.  And he takes it all.  (That's a first by the way!) 

I could see his brain spinning trying to come up with new ideas, versus trying the things we've already done that didn't work.  On the table (all new ideas) are to see what he CAN tolerate in terms of volume and then supplement the rest with TPN.  So like maybe feed him one day and then TPN the next or half and half one day.  Maybe he can handle 2 days before needing supplemental. etc.  I DO think it's a volume issue (as indicated by the fact that it takes a few days of build up before he starts the horrible retching). 

Before one hour was up, we had an EKG and an Echo done!  Can you imagine?

EKG ... he thought the "stickers" were way cool

Echo... not a fan of the goop on his tummy
About half an hour later, Dr. Lawrence, the GI's assistant came in to get a more detailed, thorough history.  She was awesome.  Friendly, caring, intelligent.  She actually READ the standards of care.  She told me they had already batted around a lot of ideas and that they were researching. 

So now I sit in my room, breathing a hugh sigh of relief.  I have HOPE again.  I have someone who thinks of this as a puzzle to solve and I have the feeling he is not used to failing.

His new Spongebob Pillow case as he plays with cars

Oct 3, 2011

Tired, frustrated Mama

The night was (relatively) quiet.  Manny had napped during the day so he was fine.  And then at bedtime, he fell right to sleep ... but then the interruptions started.  Respiratory came in.  Back to sleep.  Nurse came in.  Back to sleep.  Tech came in ... wasn't ready to go back to sleep. (But *I* was!)  So by the time he WAS ready again ... I was about to pass out.  I'd had basically no sleep the night before.  Kaley was still awake next to me on her air mattress, watching a movie. 

The nurse and tech were pretty stealth.  Baby didn't wake up much for pain or repositioning. 

At Radiology, waiting the test, wearing the new Tiger shirt

6:30, the WHOLE Surgery team came in.  I could hear them in the hallway discussing the case before they came in.  But darnit, only some peoples' voices carry enough to be understood.  I think they were just helping the new people catch up.  I saw several faces we've been talking with for days now and some new ones as well. 

We will be going to do an Upper GI fluoroscopy this morning.  He's had one done before back in February.  It will be interesting to see how/if anything has changed.  Which makes me think, I wonder if they would like the original study for comparison.  I'll ask. 

And in the meantime, we wait.  He's starting to get a tiny bit grumpy late yesterday.  Next will come weak unless they can figure this out quickly or switch him to TPN or something more than "water". 

Every person who comes in is asking how he's doing.  Truthfully?  FINE! I tell everyone that they have found the miracle cure for his situation ... to just stop feeding him.  (I know, I know ... I have a twisted sense of humor for sure!)  And the scary part is ... that IS an option for real.  I don't think we're there yet.
Manny with a child life volunteer and his new balloon. 

10am we head to the GI study.  NORMAL. 

What?  (sigh)

Don't get me wrong, normal is great.  But all it means is that we still haven't found the problem.  Apparently there were 2 different interpretations of the test results from the other day.  One group of people said it was the J in the G and loose Nissen.  Another group said all was normal.  (I wasn't told that.) 

This test shows all is going like it should.  (aaauuuugggghhhh)

Now what? 

They are going to start feeding him as per home.  Last time we resumed feeds, it took 24-48 hours before it backed up into his stomach.  Hope this time it doesn't take as long!  They need to see this.   So I'm actually HOPING for him to have troubles, to retch to have feeds in the wrong place.  Ugh.  

4pm ... half feeds started for 2 hours.  Then increase to typical feeds of 50 per hour. 
4:15... the surgery team came to tell me the results of the earlier tests (I already knew of course).  Their plan of action is to start feeds and observe him.  Really?  The Pediatrician already decided that and we've already started them.  I asked questions about dysmotility considering his neurological involvement and non-ambulatory state. 

The lead doc suggested instead that we would do things like higher calorie formula and less volume.  (By the way, that won't be a solution ... know why?  Because he's at the minimum number of CC's per day for his minimum level of hydration... but I didn't point that out yet.  I will if that's what they actually do. I happen to know that off the top of my head that he needs 45.3 ccs per hour to have full maintenance fluids. So reducing the volume of calories will make me just have to substitute water for hydration.  So how does that help?)  And he said we would just play with the amount and timing of the formula.  (Trust me, that is NOT what is going on!)  He used the cooking analogy that it might take time to get the recipe just right. 

When I again brought up the idea of  "what happens if that doesn't work?" He also said that he will discuss with his colleague (who is like the inventor of the gastric stimulator) and see his thoughts. You have to understand ... I LOVE conservative.  But you also need to know ... we've BTDT.  We've TRIED all the combos of timing and volume and amounts ... that is not what is going on.  I have been doing the conservative approach for far too long to do this route again.  It's pointless. 

And so we wait... we wait for his colon to fill up beyond the point of where he can't handle it.  OR they discharge me before it shows up. 

Now it's bedtime and he's starting to cough just a bit (that's been gone since he's been off feeds).  His mouth is again filled with extra saliva (that was gone too).  Still no sign of formula in the tube but ... it's just a matter of time; something I don't have much of before they send me on my way with that knowing pat on the head. 

I've been frustrated all day.  Researching.  Finding issues that I think really "fit".  There's the one with the dysmotility issue that is overlooked often but quite common in kids with neurological impairment.  How about we bark up THAT tree? 

Tired, frustrated Mama signing off.  Hoping to dream of a solution. 

Oct 2, 2011

Getting Settled In

So why has it been 4 hospital admissions in one month?  That speaks VOLUMES to me.  Not sure what it says, but it certainly is saying something.  For the record, I think we're all "over it".

Yesterday was fast-paced, full of phone calls, driving, tests, admission to the hospital, talking to 300 doctors.

Today? Crickets. Yes, I know it's Sunday and nothing happens on a weekend in a hospital.

So I have worked on making life here more bearable. First, I figured out how to get the internet up and running. (They have a pamphlet that shows how to do it, with user id and password (case sensitive). Problem? It's not right. I go to the nurses station and ask if there's a secret or if it's been changed or anything. She tells me, "You have to type it EXACTLY like it's there." To which I tried not to be snotty back. Anyway ... I played around with it long enough and actually landed on the right combo! (Yes, I told the nurse later.)

Next? We got one sleeper bed that will work for tonight.

Then? Discovered there's a Wendy's on campus and it was not too far away. So I dashed down and got us all sorts of food. Kaley and I ate only one meal yesterday (lunch) and didn't get breakfast today. So we were hungry. Thankfully there's a microwave and refrigerator.

So with access to the internet, a place to sleep and lunch in my belly, what more could we need? Snacks, toys and a bed for Kaley. There's a Target around the corner! Never left the baby and Kaley before with no supervision. (And I've only left them one time WITH supervision.) But we were in need so off I went.

45 minutes later, I was back with all we need (and a bunch of stuff we DON'T need).

Manny with a new PlayDoh Dog, Kaley took the picture
Got to testify to and encourage a mom who was here to do respiratory therapy for us.  Her daughter is newly diagnosed with a complex syndrome.  She's in the early stages of grief and loss and was searching for wisdom, guidance, advice, comfort, courage.  God provided it all.

Not singing, he's crying... but 2 minutes later, he was sleeping through the CPT vest

One of the Surgery residents came in and asked how he was doing (grumpy from lack of food).  And the only news is that they have scheduled the next test for tomorrow morning ... and upper GI fluoroscopy.  It's a test to see how the esophagus, stomach, nissen, etc. are all doing and to see if anything is abnormal.  Not sure why this test exactly (neither was he ... he's new to the surgical rotation and I had to help him pronounce Nissen Fundoplication).  But that's OK since he's just the messenger  He's not doing the test or interpreting it.  Everyone's got to learn somehow.  (Now don't get me wrong ... when it comes to dangerous procedures and such, I insist on the best.) 

I guess I should mention ... this is a teaching hospital (associated with a college) so lots of people in various stages of learning are coming through.  There are pros and cons for sure.  With a regular hospital, you get one specialist, usually well trained and having specialized for a long time.  They have BTDT.  They know the way things go.  But the other side is that they have limits to their knowledge/experience.  There's only one point of view and if the person isn't willing to learn, they won't.  With a teaching hospital, you get a multitude of ideas, thoughts.  But you also feel like you're educating people (Hand holding a bit).  And you have to tell the same story 100 times.  But ... personally, I like this type better as I find it easier to work with hungry "young beavers" who are looking for a way to impress their boss versus working with an "old dog" who won't learn new tricks. 

The interesting thing about Manny is .. his diagnosis and current condition are pretty rare.  So lots of people want to come and take a gander.  I was told there are only about 100 kids with Merosin Deficient Congenital Muscular Dystrophy in the US.  So most of these people have never seen and likely will never see another case of it in their lifetime.  And at a teaching hospital, that's a big deal.  Then throw in the unusual anatomy and current GI complications, he's a virtual jackpot! (I've been thinking of Grey's Anatomy a lot during this visit as it's about a teaching hospital and their up and coming surgeons ... the people I am dealing with.)

Anyway ... it's bedtime and we got Kaley's airmattress blown up and she's happily laying on it tonight. 
And finally, you have to realize I live in Florida and don't need a lot of long sleeved shirts.  I only have a few.  And for some reason, it's always cold in hospitals so I pack a long sleeved pj shirt.  (Did I mention I always keep a hospital bag packed? And for some reason, I always pack the same one.) 

As I was getting ready for bed tonight, it HIT ME!  The answer has been right in front of me all along.  As I sit here typing this tonight, the irony is very strong. 

Maybe I should just let the picture speak for itself. 

Most of you will recognize this.  For those who are not college football fans, this says "Football" and is a picture of a Gator.  As in the University of Florida Gators.  In Gainesville.  (Are you getting it?)  I am currently at their children's hospital! 

So every night that we've been in the hospital for the past year as he's been suffering, I've been wearing this shirt.  Prophetic?  Maybe.  I've been praying for answers ... and I had one with me all along! Now I'm wondering how many other things are RIGHT IN FRONT OF ME that I'm missing. 

The continuing saga ... but with hope

There is ABSOLUTELY no way to be able to catch up thoroughly so summary will have to suffice. 

In August, Manny started having troubles with major retching and respiratory issues and we landed in the hospital with atelectasis.  Released. Eventually, the tube popped out and we waited 4 days for a new one.  Still retching. Released. Tube seemed out of place so back to the hospital.  Tests showed it was in place. Released. 

Frustrated Mama.

Each doctor seemed to think it was a different body part than the one they were in charge of. 

For the next 1 1/2 weeks, I did a dance.  A very, very dangerous dance.  Food was ending up in his stomach (which to him is lifethreatening).  So I would have to vent that out so he wouldn't aspirate too much.  But if I pull too much, he'll end up dehydrated. 

GI was convinced this wasn't a GI issue so I rethought the whole thing and decided ... if the retching isn't coming from below, then maybe it's actually choking and it's from his head/throat. 

I go the the ENT.  He sees a small episode and looked in his throat ... tons of fluid and he is aspirating.  He said there are only 3 ways to deal with secretions that can't be managed.  1) Medicine (such as atropine) but we're way beyond that with him.  2) A Trach.  3) A surgery to tie off the 2 parotid glands and remove the 2 submandibular glands.  And he suggested we go with option 3.

I was in shock.  I even posted on Facebook that I had news but couldn't share it yet. 

So with that news in mind, I started to rethink the problem.  The retching can certainly be explained by aspiration but why is there still formula in his stomach??  I went back to the basics:  The simplest answer is almost always the right one.  In this case, the simple answer is human error ... that I'm putting formula in the wrong port.  (Let me assure you ... that's not it!)  So assuming that I'm not an idiot, what's the explanation?  His Jtube is out of place and has looped back into his stomach (again). 

I set out to prove it.  I contacted a friend of a friend and they suggested the dye test.  Pull stomach contents in a syringe and save.  Put one drop of blue dye in 5ccs of water.  Put in the Jtube.  Wait 30 seconds.  Pull stomach contents.  Compare.  If they're the same, then repeat after 5 minutes, then 10, etc. 

His results?  Before syringe was formula.  After 30 seconds it was blue formula.  VERY VERY clear results. 

I was MAD.  Why am I having to figure all this out?!  why is no one believing me?  why is no one taking this seriously?  For almost TWO weeks, I've been putting the milk in there on faith that the tube was in deed in place.  I was having a hard time not screaming. 

Call the GI ... let's just say he doesn't believe in the blue dye test, is certain the tube is in place and will see me Wednesday in his office.  Thankfully he hung up pretty quickly before I lost it on him.  I don't want to do that... I believe in not burning bridges. 

Meanwhile, every day Monday-Friday, our Muscular Dystrophy Clinic doctor has been calling some GI docs he knows in Gainesville, Shands Children's.  Couldn't get hold of them.  But that's where he would like me to go.  They have won several awards and were top 5 in Pediatric GI in the nation. 

Dan, Manny and I go to dinner at friend's house (Rich and Elsa) and they ask what is going on.  I tell them about the whole thing.  They used to live in Gainesville and Rich's brother Bill still lives there.  So he called him.  It was Friday night about 9:45.  Turns out, Bill's wife plays tennis with a Pediatric Doc at Shands. 

Friday night, I am very conflicted about what to do.  I feed him, in faith, basically.  His heart rate is erratic ... a sign of dehydration.  He's not peeing.  We have very little time to figure this out. 

Saturday morning, Dr. Thompson (the friend) calls and asks what is going on.  I explain.  She calls the ER attending, the surgery team, the GI ... all believe we need to get to an ER ... soon.  They work out that we can come there. 

So Kaley, Manny and I head up (took almost 3 hours due to the Gator football team being at the swamp). 

We arrive before 3pm.  They are all very nice and had already been informed about what is going on.  They start running tests. 

Chest xray shows diffuse fluid, but not pneumonia.  (GREAT news). 
They do a contrast study to show where the G and J are.  And to my shock and horror ... the test showed everything was NORMAL.  What/?????? 

I was in tears.  I just knew these people wouldn't believe me either.  I just knew that they would chalk me up to being psychotic or something.  For about 5 hours, I sat there just praying for answers.  Every time a doc would walk in, I would tell them that I just KNEW something was wrong, even if the tests weren't showing it. 

Doesn't he look tiny in there?  They have a nautical theme and this is the door to his Emergency Room

FINALLY, a test came back.  It was conclusive.  The J tube is completely in the G.  And his nissen fundoplication is not functioning right.  Apparently, his anatomy isn't typical.  Part of the bowel sits on top of his stomach. So from a certain angle, you can't see that.  So they waited 3 hours (when the contrast should be basically completely out) and then put him under xray in several positions.  This proved the bottom part of the J is IN the stomach. They only kept doing tests because I was soooo sure I was right.  The whole time I kept feeling crazy but kept pushing anyway, going with "gut", "intuition", "the Holy Spirit" ... whatever you want to call it.  Thrilled that I was listened to; trusted enough to keep doing tests even though ALL tests showed nothing is wrong.

I just about lost it.  I felt relief for the first time!  I was right.  Now we can formulate a plan. 

What is that plan?  Stay tuned.  I'll let you know when I know.  Just wanted to get this all out since many people are wondering what is going on.