Feb 24, 2012

Life as usual from room 211

Bad blogger.  Been feeling blazay.  (Note to self: that is a much under utilized word in my current vocabulary, increase the frequency of use.) 

Monday was surgery.  One night in intensive care.  Uneventful except for constant need for repositioning and pain meds.  The next 2 nights, ditto except in a regular room.  (Right next to where we were last week ... not sure why they feel the need to constantly challenge my short term memory of where I live.) 

By Thursday, he didn't even need any more pain meds.  They thought he had an ileus (psuedo bowel obstruction) but things resolved and all was working again by Friday. 

He's been in his wheelchair for 2 hours on Thursday.  Friday he sat on the couch for a bit. 

Day by day, he's getting stronger and back to himself. 

Surgery has cleared him for home.  Infectious Diseases will clear him Sunday for home.  Then we should head home either late Sunday or Monday. 

That is ... if he doesn't get any more fevers.  Today, he decided to spike a small one for about 8 hours.  Stinky butt.  As a result, they had to draw labs tonight. 

And me?  Getting ancy.  And tonight, must have eaten something that didn't agree with me as I feel like I have food poisoning.  It's the right amount of time after dinner until symptoms.  HOping this passes sooooon.  Ugh ugh ugh. 

Kaley is still an amazing helper and friend. 

In the meantime, I've been doing a bit of writing.  Mostly things about the hospital.  I wrote an article on how I feel that with "family centered care" that the pendulum has swung too far and patient care is actually suffering as a result.  (It's a whole philosophical shift.)  I gave it to the Nurse Manager on the floor as well as the head of the IV team.  I'm also beginning to collaborate on 2 articles with the IV team leader.  She came up with several ideas and we're jumping on them.  (More to come on that.)

But one is a case study of Manny and how the literature suggests 2 central line infections before starting an ethanol lock therapy.  But we're doing a preemptive jump and asserting that 1) because his first central line infection was so devastating to him he might not survive a second one, and 2) because this is his ONLY life line for food, and 3) he is running out of viable veins for central lines ... then why do we have to wait for 2?  It will be the first of it's kind. 

The second article will be a point/counterpoint of nurse/mom on all the times the IV team and Manny have interacted in the almost 2 years we have been coming here.  She said there are numerous case study type articles written by the nurse, but she's never seen one where it included the thoughts of both sides simultaneously.  She feels it will be a good read. 

I'm also trying to finalize the wording for Manny's book about Muscular Dystrophy.  I've been toying back and forth with the idea of making it just about MD vs. the idea of showing that MD is a type of Neuromuscular Disorder (the bigger picture).  I struggle because it's such a HARD, difficult topic that most adults don't understand, can I possibly make it simple enough for kids to get.  0r am I biting off more than I can chew?  I'm also waiting for a few people to submit some pictures to include in the book.  I need to take and get the cover picture to Shelli who is going to design the cover for the book.  I need to nail down the title.  Once all that is done, I'll typeset it on my Mac the way I want it and take it to the publisher for final editing and then publication/printing.  I was hoping to have it together by next weekend ... the local MDA walk... but it just wasn't possible while living in the hospital.

So that's about it.  Just life as usual from room 211. 

Feb 20, 2012

2 less body parts

Sunday night, Manny stayed up about an hour longer than normal.  When I went to put him into bed, he objected.  But I went to wash my hands from the diaper change and when I came back, he was already asleep!  Peacefully just drifted.  I was sooo happy it was easy.  He slept peacefully.  And I did a lot better than I thought I would.  (Being sleep deprived has it's perks ... like easy to fall asleep, easy to stay asleep.) 

Morning came and was quite uneventful.  Visitor after visitor came by to see him, mostly staff from all parts of the hospital.  He's quite the charmer and has friends throughout the whole hospital. 

My parents came by to be with me too.  (In fact, they just left after I got settled in ICU.)  Here's a quick couple of snapshots I took of Manny with my Dad.  One with a nurse (she gave him the bear and then Manny suggested the photo! LOL) One with Kaley just before surgery. 

He was fun, funny, happy and amazing with everyone!
Many others were emotional, but he encouraged them all. 

These two pictures are JUST before he went back.  We were in the surgical holding room.  The surgeon came in and talked to us and Manny was being his usual charming self.  With him acting the way he was, you almost wonder if the surgery is necessary.  They figured "Might as well" go ahead with it. 

Mom, Dad, Kaley and I waited the two hours.  They seemed to blink by in a second!  (I know that was a gift because they normally D R A G!) 

About half way through I got a page from the doctor.  My heart about lept out of my chest.  He wanted to speak with me.  I was "thankful" that it was "just" a call about the appendix.  It looked a bit off too so he asked if I wanted it taken.  I agreed.  (Thankfully Cartaya had told me this sometimes happens or I would have freaked out.) 

About an hour later, they were done.  The surgeon took me to a private room.  He said the gallbladder was "Horrible!"  I asked if it was leaking and he said not exactly, it was seeping.  (Clearly a bad source of infection for him.)  Then the doctor was very serious and almost in the verge of tears.  He was saying that he couldn't believe the baby that had been playing, sweet, happy and laughing just before surgery could have just that sick of a gallbladder.  He called Manny "Amazing".

People are always so impressed with this little guy.  You couldn't possibly have know he was THAT sick based on the way he was acting.  Just goes to show how much of a fighter he is. 

Unfortunately, the surgeon didn't feel that he could get the tissues safely and decided not to proceed with the tissue samples to be sent off to pathology.  I very disappointed but know that it's more important that Manny is SAFE and due to a few complications during surgery and general "issues", they didn't do the retrieval. 

I mostly feel we let down a few other people (like the clinical trials where the tissue was to go) but I know they will understand and wanted Manny's safety above all things. 

About 30 minutes later, we went back into recovery.  Here are a few "after" shots.  This is back in the recovery area. 

He was stable pretty much since he's been out of surgery.  He's on room air and still doing well.  As I write this, it's 7:15 pm.  He woke up a few times but only to cough or tell me his tummy hurt.  Then some morphine and right back out. 

The plan is to stay in ICU only one night and go from there. 

When I got to ICU (we just left here a few days ago), the doc said Manny is like a bad penny that keeps showing up everywhere.  (He didn't mean it rude.)  And I said: At least he's cute.  (They all agreed.)  And because he's so complicated, they say they are always worried about him.  To which I replied, "But it will be easier now ... there are two less body parts to worry about." (I thought it was funny but the doc didn't laugh.)  A few minutes later (as if it took a while to get the joke), he said, "2 less body parts! ha ha ha".  I think I just took him off guard with my completely off beat, irreverent sense of humor.  It's the only way I'm surviving any of this. 

The biggest complication we are trying to avoid from here is respiratory issues.  Even with "regular" kids/adults, this is a common problem.  The nerves to the respiratory system apparently run close to the area that was cut.  There is pain.  Therefore they don't want to breathe deeply.  Then there becomes pooled secretions and voila ... pneumonia. 

I've talked with the docs and respiratory therapists and we plan to be proactive with Manny and not let him GET to that point.  I can hear him right now and he sounds like he is "rattling" a bit.  I'm suctioning him ... often.  Praying he sleeps tonight. 

Thanks for all the prayers for our little warrior. 

Feb 19, 2012

Fair Pictures

Yes, they are eating off his tray!

Some earlier promised pictures of the fair.   He loved it!

Catch 22

Back in October, Manny and I were in Shands for almost a month.  The goal was to find what was "wrong" with his digestive system that caused it to back up instead of go the right direction.  After many tests and many weeks, it was decided to pull him off all food that went into his digestive system.  Instead we put him on TPN which is "food elements" through the veins. 
Cute little feet. Precious!

The veins were never designed to feed us.  It's a distant second to regular feeds (and that includes by mouth, g tube or j tube).  It's hard on the body, the liver especially.  But some people have lived on it for a lifetime ... more than 3 decades. 

A few days before the decision was made to switch him to TPN, I was getting that "knowing" sensation.  God seems to always prepare my heart and mind and soul for something hard coming.  I wasn't sure exactly was down that road, but I had the hardest time with this decision. 

I remember the day well.  It was grey and rainy outside.  Manny was especially somber that day.  Kaley was not at the hospital with me.  And I just "sat" in the sadness.  I mourned the loss.  I grieved.  It's as if I FELT all the weight of the obstacles ahead of me. 

At that time, I had NO clue that in the next 4 months from that decision that he would have:  8 PICC holes, 2 broviac surgeries (including one that took 4 attempts), one central line infection that almost killed him, one false positive line infection, 2 bouts with cellulitis, a gallbladder surgery, and nearly 100 days in the hospital.  ALL directly related to the decision to start TPN. 
If I prop his hiney, he can move his legs now!

And somehow, I felt it and knew it.  Not the specifics, but the heaviness.

At the same time, TPN has been a wonderful thing for us too.  For those (short) moments when we are out of the hospital and doing well, THOSE moments have been our best.  He's growing, learning, healthy.  He's the best he's ever been. 

That's part of the issue to me ... he's not chronically sick.  He's GREAT and then HORRIBLE.  Nothing in between.  Hospital Manny is so different than Regular Life Manny.  And that's part of what makes this hard ... and at the same time, part of what makes it all worth it.  If he were in pain all the time, well ... let's just say I think it would make life so much harder.  I'd second guess myself on everything.  I'd hunker down and just try to make his life as comfortable as possible. 

But instead, his life is GRAND and fun and adventurous.  We have a lot of fun.  His life is FULL.  And it makes me fight very, very hard to get back to that state of being.  I'm willing to go to extraordinary efforts to do this. 

And there is the Catch 22. 

So how do I do it?  One day, one problem at a time.  Today we prepare for the Gallbladder removal surgery.  Tomorrow we do the surgery and start on recovery.  After that, well, I have plans to try to get off this rollercoaster ride of GREAT and then HORRIBLE. 

And me?  I'm willing to fight, willing to be "all in" until the last moment he is still wanting to fight.  Right now, I don't see that anywhere on the horizon.  So we're "all in".  Just like in Poker ... but in EVERY hand, I hold nothing back.  Every hand I play my best.  Every hand. 


The details of the surgery ... as of right now, today should be very boring. Just a pop in by each of the doctors verifying everything is still on track (and it is). 

Monday, 12:30 is the scheduled time for surgery.  They'll have us down there early of course.  The prodecure itself should take about 2 hours give or take. 

While at it, they're going to try to harvest some abdominal wall tissue to send for research.  It MIGHT give us some hints of what is going on or it might not.  It might have implications for other families.  It might shed some light on future research and clinical trials. 

Apparently, the tissue bank has many muscles from legs, ankles, spines.  (Merosin kids often have those types of surgeries.)  But they have none from the internal organs.  So this is a big deal.  They won't take it if they can't do it safely. 

The surgery will be done by the 2 Senior Pediatric Surgeons here.  One is about 65 and the other about 55 and they've both been cutting their whole careers.  The one surgeon is the one who did both of Manny's broviacs (and knows he had a difficult time with the one).  He also did his muscle biopsy.  They're adding the second one "just in case" since they expect it to be a difficult surgery.  The anesthesiologist is the same one who has been there for almost all of his procedures (about 7 or so). 

Therefore, I think we have the best team assembled that we could have.  People who are good at what they do, PLUS they have worked on Manny before. 

After the 2 hour or so procedure, he will go to recovery.  After that, we're not sure if he will come back to the regular floor or go to the ICU for a few days.  It will depend on how well the surgery goes and if there are any complications.  He will be monitored very closely. 

One common complication (even for "regular" adults/kids) is pneumonia.  I know how they help prevent that with adults but not sure how they will mitigate it for him.  They will also monitor for infection and bleeding.

I will update on Facebook as quickly as I can during and after the procedure.  I will blog after. 

So thanks, in advance, for the prayers for Manny Monday 12:30 EST especially. Specifically that it goes with no complications and that the recovery is as painless and quick as it can be. (I HATE seeing him suffer!)

And some peace for the family.  We've been through many procedures/surgeries with him but this is the most major surgery he's been through with us.  (Similar to the nissen fundoplication he had at 8 months before we adopted him.) 

Feb 16, 2012

More medical lessons

More "Medical Lessons" ...
Most kids, by they are the time they are 2 1/2 like Manny, have heart rates approximately similar to adults.  100 would be a tad high, 70's would be about normal.  (Give or take).  But Manny?  His is faster.  Not sure why but it just always is.  Even at home, his day time rate is the 120's-130's and his night time rate is 110's.  I've NEVER seen it go below 100.  (He sleeps on a pulse ox at home that shows the rate.)
Wednesday night, Manny's heart rate kept dropping to the mid 70's.  Now this wouldn't normally even alert a nurse because that is "normal" ... but not normal for Manny.  So I mentioned it to his nurse who kept an eye on it for the rest of the night.  I was up quite late investigating the possible causes and if I needed to worry about anything. 

Eventually it stabalized at near normal so I could sleep. 

Picture from last week post broviac surgery

By morning his heart rate was still a little low but his blood pressure was VERY low. They redid it several times just to see ... it was accurate.  But thankfully, as the day progressed, his blood pressure returned to a normal range and has stayed right all day. 

Therefore, we got to move from ICU and to our "regular" room.  It was crazy how much better it feels over here!  I have spent so much time here that it feels like home, surrounded by (mostly) friends who are in our camp.  People here fight over who gets to take care of Manny. 

The strangest part is that Manny perked right up in this room!  He was doing better in ICU but now he's acting just like himself again!  (Just with periodic bouts of pain and a very sore, right abdomen.)

I put him in his bed and he did this huge sigh.  He said, "I home."   A very happy and sad statement all at the same time.  Happy because I am glad to know he likes it here and is not traumatized.  But sad that the hospital is considered a pleasant place to be.  It's a strange resolve.

smiley baby with his new bears, Thanks, Arlene!

The plan??  We are scheduled to have his gallbladder taken out on Monday.  It's called a cholecystectomy.  These days, they usually do it laparoscopically... which means small incisions and very little recovery time.  But Manny is not a candidate for that type of surgery. 

He has had other abdominal surgery so it would be better to do this as an "open cholecystectomy".  The surgeon will use the same previous cut to reopen Manny's belly, take out the gallbladder and visualize the liver.  We talked about "looking around" while down there but he doesn't want to because it could cause more adhesions and therefore more troubles down the road. 

He'll also have a cholangiogram... an imaging study of the biliary tree to see exactly where the blockage/problem is just prior to the surgery. 

Recovery for this is about 1 week in the hospital give or take.  Then several weeks recovery at home.

Meanwhile, we're about to start a new protocol to help reduce central line infections.  The team here is very excited because Manny will be their first patient that they establish within the hospital.  They have a few kids who come in with protocols and they follow them.  But they wrote and established the protocol for Manny.  So everyone is very excited.  They weill be doing some training for the staff and will include me on the training since I will need to be trained on the procedure so I can do it at home too. 

But the quick version is ... Manny goes on TPN for 18 hours.  He is off for 6 hours.  During those hours, we will put in an Ethanol Lock.  Push the liquid in his tube.  It remains there.  When the 6 hours are over, pull the solution out.  Feed.  The idea is that the ethanol doesn't actually enter the bloodstream (and if it accidentally did, it's not a huge deal) instead, it stays in the line/catheter to prevent things from growing in it.  We will do this Monday, Wednesday and Friday.  

They are most excited about this whole thing because most studies show that central line infections went down to basically ZERO while on this protocol.  And in the rare cases where it does get infected, basically zero need to be removed.  That is HUGE for him. 

Another cool part is ... there are no kids with this exact protocol (I'll specify more later) and so the IV team and I will be writing a case study involving him in the near future. 

8pm:  Notice Manny is scratching at his chest.  I open his gown and low and behold ... there's a bad rash in the exact shape of the tegaderm around his broviac.  And the dressing has completely come away and is exposing the site.  We've called the IV team. 

He's also started on a new low grade fever.  And a little cough/rattle. 

Like herding cats to stay on top of all this for him! Truly a full time job. 

Feb 15, 2012

Blood is a good thing

So the good news first ... the blood transfusion went well with no complications (just like last time). Not sure why his body is so weak and overrun with infection that he NEEDED it, but he did. 

He liked that transfusion so much that today he is sitting up and playing with his ipad.  He's mostly back to himself ... except ...

for periodic bouts of very bad pain.  I have some friends who are nurses here and they were here for one of the more mild episodes.  He starts to sweat profusely, he increases saliva, he starts screaming "Owie help me" and is generally distressed.  The one nurse had gallstones when she was pregnant and she said it looked like that kind of pain to her.  (He doesn't have the stones, but the incredibly inflammed gallbladder.)  Then the episode is over and he goes back to playing. 

I got the blood results this morning and they look like you'd expect from someone who was very sick and then got a transfusion.  Hope it's enough to keep him stable to fight this infection. 

The night last night was long and hard.  It started with him not able to go to sleep (it was just after the transfusion and I think it made him aggitated).  Eventually he did sleep, about 11:30 pm and I decided it was my chance to slip away and get a shower.  (Let's just say you should be thankful you weren't here to smell me yesterday.) 

I was only gone a few minutes (maybe 10) and when I got back to the room, he was desatting.  Now you have to understand, he was at 100% this whole time.  He's never been below 98, even when he was non-responsive.  He tends to go from "Fine" to "crash" because he's compensated for so long.  So I did my usual thing of rearranging him, suctioning him, making sure the lead was on right, etc.  (All the trouble shooting things.) And he was still dropping.  Called for the nurse and she got the Respiratory therapist to come and give him breathing treatments.  They seemed to help.  The last thing we need is a respiratory illness on top of all this!  And he tested positive for Rhinovirus (the virus responsible for the common cold, but can turn into much more). 

I was up often suctioning and repositioning him.  At one point, the nurse got scared and ran in here because he was coughing/choking and couldn't catch his breath.  But this morning, after he woke up, he's better able to manage his secretions and has been at 100% all morning.  Keeping a close eye on this. 

Got the ultrasound report back.  Basically no change from Saturday's.  There is still a very inflammed gallbladder. There is still fluid (that shouldn't be there) outside the gallbladder.  This is after 5 days of draining it.  Waiting for the doctor to see what their plan is with it.  So far, it's just to keep him on antibiotics and wait.  I'm not so sure I'm comfortable with that plan since I know this thing could rupture easily as he has all the stages of advanced acute acalculous cholecystitis.  I'm not convinced that it's not a slow leak into his gut in fact. 

As the day drags on, I'm hearing more and more congestion.  He's starting to "rattle" and that is NEVER a good sign.  He's doing fine still and we must keep it that way. 

Talked to the Doctor in the ICU and she was blown away at how alert and responsive he is today.  (Blood makes all the difference!)  She felt he was stable enough to go to the floor tomorrow as long as he is monitored pretty closely. I'm good with that.  I will have a toilet, shower, bed and refrigerator there.  (So HEAVEN compared to here.)  And I know the signs to watch for. 

The GI doc is trying to stabilize him and then get us to a motility expert.  She said there are only like 5 pediatric ones in the whole country.  I'm liking that she is taking this seriously this time.  (Last time they didn't... how we ended up at Shands.)  We have a lot of thoughts circulating.

"Doc" stopped by to see me and I told him my concerns in a round about way.  He said, "I hear what you're thinking and I dont' have any answers."  But at least we were still on the same page again.  If the night goes well, we'll be transferred back to his service where he makes the final call on all these things.  I like that because he has the whole perspective of Manny over time, how sick he gets, how fast he crashes, etc etc. 

Bottom line is right now, there is an infection in his body (not his blood thankfully) that is raging out of control.  It's causing pain, blood labs similar to sepsis, etc.  We don't want it to get ahead of us.

Tonight, the surgeon stopped by.  He wanted to come earlier but got swamped.  He said that he and the Infectious Diseases doc were discussing that the gallbladder IS the only source of the infection raging in his body.  He suggested it come out.  The surgeon said that he is ready to discuss that possibility with the team tomorrow. 

He will talk to Cartaya, the GI, Infectious Diseases, etc. and see if that is the best idea. 

In the research I've done, they often find cholecystitis as an incidental finding while looking for something else.  In that case, there are no symptoms, so no need to remove.  Typically a round of antibiotics and it's all back to new.  But in cases where there are symptoms, I can find very little evidence for the wait and see method. 

Saturday the gallbladder was enlarged.  Tuesday, it was bigger. They've drained tons of fluids out of him.  In fact, he left last week weighing 13.2 kilos.  Admitted (4 days later) at 13.8.  Tonight he's 12.9.  (And for those who don't "speak" Kilos, that says he has lost almost  2 1/2 pounds in 5 days.) I am telling you, they drained a LOT of fluid off of him.  And yet, the gallbladder is still just as inflammed or "hot" as they call it.  It's akin to an appendix about to burst.  And the results are similar if it does. 

As I write this tonight it's 9:40 pm and he's finally asleep for the day.  He didn't nap, he was awake all day!  It was a good day.  The monitors are showing his pulse rate is slowing down.  Not worried yet because maybe it's just a sign of his pain reducing and he's not as distressed.  On the other hand, I can't help but consider it could be something else.  Time will tell.  And I love that he's on a monitor so we know instantly if something were to go "wrong".  But normally he sleeps around 120 when he's well, not stressed.  And it's around 90 right now (more typical for his age, but not typical for him). 

His blood pressure is low and dropping.  I'm hoping this is a GOOD thing.  A sign of good things to come and stability.  But my Mama gut says nope ... it's going to be a long night.  Praying I am wrong.

Why Adopt?

"Why did you adopt all these kids?" It's a question that has come up in whisperings and hints around me but no one comes outright and asks it. I know I don't "owe" anyone an explanation/justification ... and there's no way to do it adequately in a few paragraphs but here goes a quick overview attempt. (And I don't think there's a way to do this without offending a few, but I will try.)

Dan and I grew up in a church that had a general philosophy of "When the Kingdom comes". There's a thinking that "In the world tomorrow" x, y and z will be fixed. When Jesus returns, things will be made right. Many people understood the concept "correctly" but it was also used as an excuse (to many) to do nothing. No need to evangelize, no need to be kind to fellow humans, etc. I mean NOTHING.

Dan and I got married ... Christmas Day (yes, you read that right) in 1994. A week later, the church announced some major changes and the church we grew up in became splintered in several directions. So we have friends/family with hugely varying religious ideas. (Understatement).

Before we got married, we wanted to adopt. Somewhere along the way, it became clear to us that we should adopt those who wouldn't have had a family otherwise. I feel God showed us that path and we took it. At first, the choices were fairly easy. Jacob and Kaley, for example. People don't adopt kids who are African American out of the Foster system. Period. The odds are (at least in our state) that if you hit age 1, you have less than a 5% chance of ever being adopted. So it seemed a no-brainer. Along the way we discovered their medical conditions. AFTER we adopted them. But by then, they were "ours" and you just buck up and do what any parent would do.

Eventually we got to China and you can't "unsee" after you have seen something. These kids are just babies needing a home, needing warm clothes, needing a stranger to be kind to them.

Matthew25 comes readily to mind. Do this unto the "least" of these... clothe, feed, etc. If you haven't read this powerful chapter lately, you might want to reread the whole thing. The context of it is quite chilling.  It talks about an ACTIVE participation.  DOING, not just saying. 

But bottom line ... by the end of the chapter, Jesus says to one group (the doers) "Well done, welcome to Eternal life" and the other group (the non-doers/waiters) "Depart from me, I never knew you." And I don't know about you, but that makes me WEEP. And all I know is, I have NOOO excuse. I know what I am to be doing. It is in my power to help. I must not withhold my help.  And beyond that, God has equipped me to do just that.  He's given me talents (also talked about in this same context) and expects me to use them to propel His Kingdom. 

James 2:14-17 is another powerful section. In paraphrase ... faith without works is dead. If a person says, "Hey, I'm cold and I need a coat" and you say, "Hope you find one" ... uh, it's a problem. But I know soooo many people who do this in regards to kids who need a home. They see the need. They close their eyes and say, "I hope you find a family".

Am I saying everyone should adopt? NOOOOO ... Trust me, this road is not for everyone. But there's stuff people can do for the orphan. Mostly: Don't turn a blind eye and shut off your heart.  God has probably prepared YOU to do something for His Kingdom.  Do THAT. 

So ... back to Manny specifically. Our family had been being prepared for a while. God even prepared our hearts for taking in a child that would not live long. (A string of converstations, trainings, experiences, etc.) I'll write that blog soon... "Why Manny". 

But when Manny was presented to us, we initially said No. He was too severe. His needs were very unknown. Even his condition was unknown. And I can write out the details of the 2 weeks where we kept saying No and God kept saying Yes. (It's quite remarkable.) But ultimately, we said yes.

And by saying YES to Manny.... it was the right decision. (I know some of you might question that, and it's OK.) We would do it again in a heart beat.

With that said, to say YES to Manny meant we had to say NO to a lot of things too. I had China projects that have been put on complete hold. Our house is needing maintenance. My relationship with Dan and each of my kids is sometimes strained (like a month at a time hospital stays). Dan's business has suffered. We have said no to countless things. Life is VERY hard sometimes.

So why did we do this? For so many reasons. (Some mentioned above.) But bottom line is ... Jacob, Kaley, Sam, Luke, Zoe and Manny deserved a family. They deserve to walk out this life with people who love them. People who will fight for them. People who will teach them of God.  And we wanted to be parents.  A match made in heaven. 

And I believe that MY role as a Christian is to do the best to walk the talk (to the best of my ability), to show God's love wherever I go.  ("You shall know them because they show love to one another") And I have chosen to walk the road of adoption. 

I hear all the excuses of people as to why they can't adopt.  (Not sure why they feel the need to let me know their reasonings ... guilt perhaps?) But among them are cost, too old, not enough room for more, not sure they could love someone that is not their "own", time consuming, too much paperwork, fear of the unknown, etc. 

I am so very thankful that God didn't use any of those excuses when He chose to adopt me!  I mean I'm SURE I was costly (Jesus Christ's sacrifice comes to mind as just one of the high payments He made for me).  I'm sure God is old, but never too old.  He always has room for another child.  I AM His "own".  etc. 

So why adopt?  I think the better question would be "Why NOT adopt?"

Feb 14, 2012

More blood transfusions, short on answers

The night was long.   Blood pressure is more stable, heart rate very erratic and high. Fever spiking all night even with tylenol. refusing to swallow his secretions so he is trying not to aspirate/choke on those all night so we're suctioning every 5-10 minutes. There were several things that needed to be done last night and he was not happy about any of them. Just the least distressed and he was hitting heart rate 200. But we caught a few winks in between all that. Like a newborn's mom, I sleep when the baby sleeps.

Blood tests were taken at 8am and I always get a copy of the results so I can study them.  Suffice to say that they were bad, worse than yesterday.  Back in December when he had a central line infection, his numbers looked like this the day he crashed and landed in the ICU.  Lucky for us, we're already there. And he's being monitored (unlike last time) very carefully. 

The good news is that he is responsive today. He even sat up for a few minutes at a time and asked to play with his ipad.  He couldn't do it long as he's very weak but "Manny" is back. 
Starting to wake up, putting moisturizer on his lips.

He was supposed to go down to a procedure/test this morning but they ended up cancelling it again.  They didn't feel he is stable enough to do it.  It's a simple procedure (similar to an MRI) but it would require some mild sedation and given his labs, they felt he wouldn't be able to do it.  So it was postponed a second day.  (The procedure was to help determine further bowel function and a possible source of the problem.)

Yesterdays labs showed he needed a blood transfusion but those are hard on the body too.  So they wanted to wait.  This morning, the signs were MORE clear of the need for the transfusion.  They started it about 5:30pm.  They also did the Ultrasound of the gallbladder, etc at about 6pm tonight.  Awaiting the report of that anxiously.  (I was in the room with the tech who did it, but darn it, I didn't go to Radiology school!  So I just saw gray blobs.) 

 Today is Valentine's Day. And I remembered that I was in the hospital with Manny last year on Valentine's day too. I remember it because the volunteers came around with a balloon and he was sooo excited. He kept playing with it!

The only other thing to report today is the heart's desire answered.  Our room doesn't have a restroom in it so I usually try to sneak out when he's sleeping.  But this afternoon, he wasn't sleeping and I really had to go.  He cried the whole time I was gone.  His heart rate spiked, etc.  So when it came time to figure out what to do for dinner, I wasn't going to leave him.  The bathroom is like a few steps away and the cafeteria is clear across the hospital.  I couldn't do that to him.  I was dreaming of something like spaghetti, green beans, and maybe some salad or fruit. 

I was wondering what to do about that when I hear a knock, "We have an extra tray. Do you want it?" Sure!  I figured no matter what it was, it was better than nothing. 

It was lasagna, green beans, salad and fruit. 


Ever wonder if God is really listening to your heart's desire? 

Feb 13, 2012

Which horse?

The night was better.  Not great, but better.  Very little screaming in pain.  They kept him on pain pills throughout the night and helped me reposition him every 2 hours. 

His blood pressure is slowly creeping lower and lower.  His heart rate is creeping up.  His fever is creeping up too. 

But he's starting to wake up.  He's used a few words this morning.  He's about 10% of his normal self, but it's improvement. 

As I studied over the blood tests last night (most of them I knew what they were, their purpose and their reference range) but some were new tests.  If I'm interpreting corectly, seems his liver and gallbladder are VERY sick.  Most of the tests point to very high levels of "liver disease". 

My biggest concern at this point??  That it's the TPN causing it and they will have to take him off of it.  No one has even mentioned it at this point but I am reading handwriting on the wall so to speak.  It seems this is toxic to him. 

So if you're following along with the blog ... you probably had your stomach fall by the thought.  I know I did.  IF we get there ... then what?? 

We must have a miracle.

2:45 pm all day we've been very popular with miscellaneous doctors.

Bottom line with all of them ... he's likely septic.  The blood cultures haven't shown that but everything points to it!  EVERY one of his labs are indicating he is.  He is acting like he is.  His vitals are showing he is.  This is just like last time in December.  He is DEFINITELY not doing well and they are monitoring him very, very closely. 

I got an email from the main doc at the NIH that we were going to see today offering to help. 
I've had several phone calls, emails and text with the director of the CureCMD, the "experts" on CMD offering help. 
I had the GI doctor from Shands asking what is going on and offering help. 

We have a lot of minds on this case.  And right now, ALL of them seem a bit stumped.

His latest blood pressures are 86/42, Mean arterial pressure of 53. 
81/43, map 52
heart rate is hovering in the 150's
Many of the labs are looking like they did last time just before he crashed into septic shock. 

8:45 ... having a hard time writing on this blog today and trying to update everyone.  There's nothing shockingly new... it's just all about the same.  So why so hard?  Because of the handwriting on the wall.  I can do math so to speak. 

At one point today, "Doc" came by just to visit. He is not our doctor while we are in the ICU but will be again when we're back on the regular floor.  I asked him a couple of pointed questions. And he said, "Let's go talk".  We left the room (where the nurses were) and just let me ask whatever I wanted and he shared with me his concerns.  We were on the same page. 

He used the analogy of which horse is going to get to the finish line first.  For most kids with Manny's muscular dystrophy, it would be respiratory failure.  But for him, that horse is so far behind.  There are many other horses that are way further ahead.  At the front of the pack are the horses named, "Running out of viable veins", "Sepsis" and "TPN". 

With each sepsis, it's hitting his body harder and harder.  This time we can't even be certain it's sepsis ... the blood cultures are still negative. (A great sign.)  But he is certainly acting like he is ... so it might as well be.  (Like right now, his hemoglobin is barely above 7 ... they usually transfuse at 8 but they're not doing it yet, holding out to see if his body can react on it's own.) 

And TPN is a problem because it is apparently caustic to his body.  2 weeks ago, when the TPN accidentally got in his arm, do you remember the horrible reaction it had??  Well, it's doing similar things to the rest of his body.  So at what point do they feel it's not worth feeding him with TPN any more?  Then what?  Or they keep feeding him the TPN and it destroys his liver and gall bladder and we deal with organ failure. 

See why I didn't want to write the blog?  It's just so darned depressing!  And in the middle of this, his story is being told EVERYWHERE.  He is FAMOUS in this hospital alone.  Like today the nurse called the IV team to get a dressing change on his broviac.  They asked, "Can it be done in the very late afternoon?"  I told them to say, "It's Manny".  She did.  They were here in less than 5 minutes.  I walk down the hallway and nurses that I haven't met know Manny.  His story is circulating. 

Tonight, Dan and the kids came to visit. He was mostly lucid and was able to say a few words to them.  He cried when they left.  He got so distressed that his heart rate went to 200.  In the ICU, the little kids aren't allowed to come in but they make special arrangements/considerations depending on the circumstances.  They let my kids in.  Could only be about 5 minutes ... but that was enough as it is hard for the kids to see him like this too. 

And I sit in this room ... basically numb.  I am going through the motions.  I am doing what needs to be done.  I am doing my job.  If I let myself go there, it's just too overwhelming of how much suffering he is experiencing.  It's too much to handle emotionally. 

I can't tell you how many nurses have broken down in thinking about him, talking about him.  They try really hard not to ... but it's only natural that people get attached to him. 

Praying the pain pills are enough tonight. 

One day at a time. 

Very Sick

Somehow I didn't have time to update the blog all day today.  So tonight will have to be a summary version. 

Manny had a very rough night.  Woke up often, crying he was in pain.  I'd reposition him (which requires getting out of the chair and walking over there) and it would help for a little while.  Then repeat ... all night long. 

By morning he was still miserable and then "unconscious" alternating.

11am, fever. Tylenol via suppository. (He didn't like that so he tried to push it out.)  Well, then he had a major poop and voila! He felt much better. He even became a little more alert.  That lasted about 2 hours.  He wasn't himself. Wasn't talking (couple of words) but would respond.  Like I said something about the word "Blink" (in reference to lights) and he did. 

The rest of the day he was in and out of pain and consciousness.  Then about 4pm, he spiked a higher fever.  Pain increased.

By tonight, he's still a little more "awake".  But his blood pressure is trending downward.  His fever is trending upward.  His bowel sounds are diminished.  The pulses on his right lower legs are diminished.  He is showing signs of mild respiratory distress. 

Some new labs are coming back in and they're not good.  The early labs were all fine but as they know what is going on, they are able to get better tests.  Like a huge concern is for sepsis ... not through the central line this time, but from the gut.  The blood cultures aren't back yet so they don't know but they are treating it like he is and he's showing some initial signs that could be that. 

They also did a procalcitonin level.  This has been correlated with sepsis.  (And that test comes back within hours, not days.)  His level on Saturday morning (upon admission) was 0.2 ... they consider 0.25 to be the cut off to consider it "likely positive".  So in the gray area. 

Then tonight, his PT and PTT times came back elevated.  This is often associated with sepsis too... last time he had this on his decline ... and they gave Vitamin K (both times) to try to stop the slippery slope. 

No matter what is going on, he's very sick.

Feb 11, 2012

What the heck?

We were discharged on Tues, Feb 7 with a shiny new broviac.

Tuesday evening, low grade fever.  Thought little of it.
Wednesday, higher fever and rash (looked like 5th's disease).
Thursday, moderate, then low  fever and rash (looked like Scarlet Fever)
Friday, fever gone and very little rash.  (Only on chest.)

Friday we also had a grand time at the Florida State Fair.  FUN!

Friday night went to bed just fine.

3am ... woke up screaming in pain every half hour or so. 
5am ... woke up screaming in pain every 10 minutes.
6:30 ... screaming in pain continually.  Saying it was his "tummy" but reaching for his Broviac. 
Got dressed and headed to the ER. 

8am ... he was still in pain but also falling asleep.  I figured that due to his intermittent sleep in the night, this was no problem.  But then started to notice, he would scream in pain and then almost "pass out".  His eyes would roll in the back of his head and he would be out again. 

Since about 9am (I write this now at 7pm), he's been "asleep".  He is not waking up for more than about 30 seconds - 2 minutes at a time.

The doctors have gone through many theories today.  They've tested them all very quickly and efficiently.  His blood tests are all (relatively) normal. His chest xray shows broviac in place. ALL good news!  But that doesn't explain the pain and the unconscious state. 

Ultrasound seemed to indicate he had Intussusception, a bowel issue that could explain MOST of his symptoms.  And the child can actually be in so much pain that they basically "Pass out".  But we did a further CT and it didn't show that.  Instead, it showed acute acalculous cholecystitis ... an inflammed gallbladder without stones. This is something that can happen with long term (defined as over 3 months) use of TPN. 

This doesn't explain his altered mental status but does explain his pain.  UNLESS it's already in his blood stream and in that case, it can cause sudden sepsis, which could cause the mental status.  The blood tests don't show that but it's also early so it just might not show up yet. 

For tonight, they are going to put in an NG tube.  That's a tube that goes from his nose to his stomach.  Normally they are used to put food into the stomach.  In his case, they're going to use it to drain the bile, etc. OUT of the stomach.  It's producing so quickly that he's gained over a pound today.  Normally, venting via Gtube is enough but today it is not. 

They have him on massive amounts of various broad spectrum antibiotics.  They are watching for a fever to spike.  He's on a heart monitor, CO2, respiratory rate, blood pressure, etc. to continually monitor him. The plan for the night is just to watch him... closely.    And then reevaluate in the morning.  Surgical team has already been contacted and is observing him.  Infectious Diseases will be here tomorrow too.  Neuro consult likely if the mental status doesn't change. 

In the meantime, all the vital signs remain eeerily "normal".  From the blood work and vitals, you'd think everything was perfect.  By looking at him you'd think something is VERY VERY wrong. 

11:30 pm, he's still altered.  But is waking up more and more often with retching (like he's trying to throw up but can't) and pain again.  Poor guy.

What the heck is going on??

Feb 5, 2012

Overview Summary

Thought it was about time to give an "Overview Summary".  I know some are confused about what is going on so hopefully this will help clarify. (And I'll throw in  a few pictures just for cuteness.)

Manny was born with extreme hypotonia (floppiness) and muscle weakness.  Genetic tests done, no results.  (And the reason is because his condition is EXTREMELY rare and they wouldn't have tested for it.) 

8 months old, his birthmother took him to the hospital (he was very sick) and placed him for adoption.

Last week after Manny's PICC reinsertion procedure.  Surgical cap and Versed on board!

During that month, they took him off oral feeds and moved him to a gtube with nissen fundoplication.  Up until that point, he had been able to drink via bottle, but now he was aspirating every swallow and had GERD (acid reflux).

9 months, we picked him up from the hospital to adopt him.

He retched, a LOT.

11 months, he got pneumonia and landed in the hospital (Same one we're at now). Dr. Cartaya ran miscellaneous tests including a brain MRI.  That was interpreted as "End Stage Leukodystrophy" and the prediction was he wouldn't live even another month.  We went home with hospice and a DNR.

On the way to a procedure in his chariot, er, I mean wagon

He retched, even more. And would affect his breathing/heart rate. 

By 14 months or so, I pushed to have the "palliative care only" removed so he could begin some treatment.  He was not behaving like his condition would indicate.  I began to press for a different diagnosis. 

By February 2011, he has worsening GI symptoms so a study is done.  Stomach has stopped working.  Put him on a GJ tube. 

Multiple issues with the GJ tube over the next few months. 

April 2011, muscle biopsy results show the actual diagnosis is Merosin Deficient Congenital Muscular Dystrophy.  We begin to learn what this entails. It describes him to a T ... except the extreme feeding issues.   

Continued GJ issues. Increasingly worse to the point where I was afraid for his life.

September, 3 hospitalizations regarding extreme retching (to the point of respiratory distress, tachycardia of close to 300), etc.  Realize we need more help.  His Muscular Dystrophy doc suggests Shands.

October, spent the whole month in Shands in Gainesville as they are the best Peds GI around.  After MANY MANY tests, they determine he would have to go on TPN and give him a PICC.

November 10, his PICC cracks and they put in a temporary one until they can do a Broviac.
What his rash DID look like when they changed out the PICC (See yesterdays post for the current picture)

December 2 -28 hospitalized for a central line infection (Sepsis).  Remove the broviac and go home on a PICC.

January 25, 2012 - PICC acting up, rewire it.  Go home with a rash around the site. 

February 3 - 6 days later, readmitted for cellulitis around the PICC site. PICC removed
February 4 - awaiting negative blood cultures so we can put in another broviac.
February 5 - surgery scheduled for Monday or Tuesday

Meanwhile, on Feb 13, we are scheduled to go to the NIH.  Their Neuromuscular Disorders Clinic has actually SEEN some kids with his underlying condition. We sent all of Manny's files there, including MRI, muscle biopsy, etc.  They were struck by his file since he definitely has Merosin Deficient CMD but these GI issues are atypical for the condition.  We're hoping they can figure out what is going on. 

Combine THEIR thoughts with the Metabolic Geneticists thoughts, we have an excellent team.

Why are we fighting so hard?  Simply put:  I don't think it should be life-threatening to feed my child. But currently it is. 

Feb 3, 2012

Which floor?

Thursday:  Manny went to the Pediatric Metabolic Geneticist. This meeting was to help discuss next course of treatment/testing. 

I presented the info to her this way:  Bottom line is ... either the literature is wrong and Merosin DOES affect Smooth Muscles or Manny has a SECOND very rare disease.  After a long discussion, we decided the best thing would be to contact the NIH (where we are supposed to go next week) and collaborate with them. 

There are some tests she wants done on the actual muscle that was biopsied.  It's the best hope of finding the issue.  The hope is to get to the bottom of it so Manny can eat again.  Plain and simple. 

I was very excited about this visit and the outcome. 

Thursday night:  The kids had a PTA fundraiser at Chuck E. Cheese.  Because it's Manny's favorite place, I took them, even though Manny wasn't 100%.  His arm is the only problem and we have it thoroughly covered so I figured why not? 

Friday:  Manny had an appointment with the cardiologist.  The doctor was able to hear a new murmur.  He thinks it's an "innocent" murmur but wanted to be sure so he strapped a 24 hour holter monitor on him. 

While we were on the hospital campus anyway, I decided to look up the IV person, Stephanie.  She is the one who helped put this thing in last Friday night.  We talked for a while and then called Dr. Cartaya "Doc" to come help decide what to do (since this was HIS idea). I talked with him privately for a bit ... told him my concerns about not being able to keep the site sterile because the skin was still weeping and causing the bandages to peel up.  We bounced some ideas. 

Stephanie also called the Wound Nurse who had great ideas.  After a long discussion, we came up with a plan of action on how to care for the wound.  (The one I'd been seeing every day for the past week.)  Doc left. 

Got into the room.  Pulled off the dressing.  And I was in SHOCK at what we saw.  This was no longer skin being irritated or red or raised bumps.  The whole thing was PURPLE and smelled.  There was infection.  There was DEEP tissue cellulitis.  It is BAD.  This picture doesn't even begin to do this justice!
The skin is weeping. The purple is deep, deep cellulitis. There's an on smell on the skin. There's a red rash beyond the borders of the bandages. There is lymph node involvement under the armpit. The arm is more swollen today than day 1

The nurses were shocked too.  Stephanie immediately called Doc to come look at it.  He walked in and eyes got big.  He basically asked which floor I'd like to be admitted to.  This thing HAD to come out NOW! 

Now you realize, I'd come for a 2 hour doctor appointment.  I had nothing with me.  NO cell charger, no laptop, no clothes, nothing.  (Not to worry, I had a bag packed still at home ... sadly, I had a feeling that it was wise to keep it packed.) 

I text Dan, "Being Admitted".  He replied, "Long or short".  (I think that conversation says it all... we got to the heart of the matter... no emotional outburst, just business.  Sad.) 

Stephanie gets an IV in.  Draws blood.  Takes out the PICC line.  Attends to the arm.  All this is done and we are not even admitted.  We are not in the system at all.  This was sooo not the way it was supposed to go.  We are very "off script".   They can't even send the blood yet because there are no labels, etc for it. 

Cartaya goes and writes up the orders.  I carry the drawn blood with me.  I get registered.  We are admitted.  Back to the same floor we just left, less than a week ago. 

Dan gathers up my stuff and brings us dinner.  Kids are all too comfortable with this environment too. 

Tonight, he's all comfy in his big bed.  No crib was available so they put him in a regular bed.  He looks so tiny in it!  But there's room for us to snuggle, which is nice too.


Me?  I know you're all wondering.  I am on pause again.  Truthfully, I wouldn't be shocked to hear that he has a blood infection/sepsis.  I am not hoping for that, just a gut feeling.  And I am praying that my gut is wrong this time.  (It rarely is!)  So I am praying that God just heal him and heal his blood. 

The plan?  Wait for the blood cultures NOT to grow again.  IF they don't, we will try to put in a Broviac on Monday.  If that goes well, we could even go home as early as Monday evening.  That is a good plan.  I like that plan. 

If it does grow something, we're here for the long haul again.  We hope and pray that this isn't ahead of us this time.  I do not like this plan. 

So ... if we get Plan A, I'm good.  I'm fine.  I'm just a bit numb. 
If we get Plan B, I think I will need some kind of psychiatric intervention.  (Mostly kidding, I think.) 

So for now, I hunker down.  We go back to living one day at a time.

If anybody is still reading and along for this VERY bumpy ride, Thanks!  We're still in need of all the support we can get.  This story needs to be told high and low.  Apparently, God wants to get a lot of Bang out of Manny's story and He wants many people to know about His goodness.