Sep 27, 2011

Manny says

Manny: "Cloud"
Mama: "Where do you see a cloud?"
Manny"  "Right there. Up in da sky."
Not bad for a 26 month old, huh? 

I have a million conversations like this with him.  Always amazed at his vocabulary and he's even working on the syntax. 

Or how about this ... the other day the Neurologist was in trying to assess his mental state.  Manny was playing with a vehicle.  Doc asked, "What is that?"  and Manny answered "Tractor".  (His articulation is poor due to muscle weakness so the doc didn't understand but I did.)  The doc asked again, "What is that?" and Manny said more slowly and deliberately, "Trac-tor".  Doc was like, "Did he just say TRACTOR?"  I nodded with a proud Mama smile.  He says, "Not just 'car', but 'tractor'. Wow!"  I then handed Manny more vehicles, "Bus", "Truck", "Car", "Train", by the time we got to airplanes and helicopters, the doc was in shock. 

The Doc reminded me that he's never seen a child with such extensive white matter brain involvement that was cognitively intact.  He said, "If you see his brain scans, you'd just know that this child was in a true vegetative state, not talking."  Manny is defying all the odds apparently!

A little later, the nurse was talking about the electronic fish aquarium that Child life let us borrow.  There is a clam, 2 fish and a star fish.  We had NEVER talked about it.  She said, "How many fish are there?"  He looked at all of them, pointed to them "One, two ... two bish".  I said that I doubted he knew the starfish was a fish.  So she said, "How many stars?"  "One tar". 

The other day we were at a resort and he was relaxing on the lounge chair.  He looked up and saw coconuts in the palm tree.  But what he said was, "Applesauce".  What??  "Applesauce up in the tree."  And then I had to make the association.  Oh... he calls all things in trees "apples" usually but this time he got confused and called it "apple sauce".  I find that especially funny because, well, he can't eat! So food is a little "Mysterious" to him. 

Tonight, Dan took him in the backyard to explore.  He came in with flowers in his hand and he said (as clear as day) "I have flowers for you."  In the other hand is a little berry from a tree (he is back to calling them "apples") and says, "I have apple for you."  I think those are pretty darned good sentences! 

Daily, this little guy shocks me.  If you see Manny on paper, it's "Tube fed, oxygen dependent, non-ambulatory, complex special need child".  That conjures up a certain image.  And I guarantee that image would not be any where close to reality. 

Manny is continuing to teach people about life.  He is defying logic.  He is a Miracle. 

And me?  I'm the blessed one who gets to snuggle with him every day. 

Sep 20, 2011

Tube out? In? What is going on?

How do I say this? 

We're in the hospital again. 

You know that GJ tube we waited 4 1/2 days for?  By Sunday, it was clearly out of place.  I pretended it was not the case but I knew it was.  By Monday morning, I put a call into the GI.  By 1pm, they called me back.  2:30 headed to the Hospital.  3:30 appointment.  4:45 finally see the doc.  5:30 admit papers in hand.  7:00 headed to our room.  (Change of shift, naturally.)  11pm, Manny finally asleep.  Kaley is with me again.  (My trooper!) 

Manny?  He handled it beautifully!  He's in a great mood.  The IV team did his IV.  (Only took 3 sticks.)  And fluids were finally in at 9:30pm ... so only about 8 hours without fluids. 

Dora balloon!

The plan?  Wait for another tube.  NO, I am not joking.  I would NOT joke about something like this.  SO hopefully they got 2 when they ordered the last one.  This time, the GI wants to put it in himself.  He wants to see if there is something they've been doing wrong or something anatomically going on that would cause it to malfunction so quickly.  (These are supposed to last about 6-9 months and this will be the 5th in 6 months.) 

We also discussed doing some tests... not sure what all they will be but we'll see tomorrow. 

Also discussed that it could be the retching that causes the tube to go out of place.  So what is causing the retching?  He felt it's neurological (based on symptoms) and not GI that causes the retching.  And if that is the case, the only thing that can be done is to do a "major surgery". 

I have fellow tube moms who feel that he needs a separate J and G tube.  (Means 2 holes on his body.) But no way for this to happen again.  And these tubes can be reinserted at home, not at the hospital, etc.  All sounds great to me. 

For some reason, they don't want to do a "straight j" placement, they would only want to do the Roux-en-Y procedure and that is MAJOR surgery he said.  So tomorrow, I have a surgery consult to discuss.  I'm not clear on why the "staight j" would not be an appropriate surgery. 

But either way ... I hate these choices.  They all stink if you ask me.  I'd like my baby to wake up tomorrow asking for a cheeseburger and we walk out of here. 

The night was uneventful.  (Love having a nurse and a nursing assistant ... no machines beeped all night!  They were on top of things.)

7am they came in to take us to Radiology for a small bowel series.  Barium in the tube and watch it (via xray) go out.  The test came back Normal.  The tube was in place. 

NOT the news any of us expected!  Why?  Because how does milk end up in his stomach (in large amounts) if the tube is in place?  Very, very strange mystery.  AND why is he continuing to retch enough to cause respiratory distress?
waiting for a clean gown... saying "Cheese" and playing with Diego (his fav)
So I sit here at 12:30pm and I am questioning my sanity.  I KNOW there was tons of milk in the tube.  How do I know?  I collected it and have it with me!  And I KNOW he has these desats due to retching.  How do I know?  Because I have video of several of the episodes. 

But as I sit here, I think my docs know me well enough to know I didn't make either of these up ... but as a sane person, I also know they've GOT to be considering that possibility or that I messed up or made a mistake. 

Meanwhile, he hasn't retched today.  His tube is in place.  Maybe I just take that news as great news and go home.  Part of me wants to do that, the easy thing.  Just bury my head and pretend that there's nothing wrong.  And yet, I KNOW that later today or tonight or tomorrow or soon, we'll be right back where we started... with milk in the wrong place and retching. 

But I also question how much "fight" I have left in me!  And if this is the time to PUSH, or just go home and regather myself. 

Up until the MRSA came back negative, he had to wear the mask when he left the room to go get his IV. 
He is not a fan of the mask.

So while I'm sitting on the ground playing in the water bucket with Manny, I hear a familiar voice in the hallway.  Dr. Cartaya.  I'd asked to be on his service but the GI felt he didn't need it.  I opened the door and he saw us and was in shock that we were back... again!  I ran a few things by him.  He had the same questions/concerns I did.  Seems the Roux-en-Y j tube placement is very aggressive and he's wondering why that would be the next step versus the simple straight jtube placement.  (Me too.)  He also wondered why the retching and what could be done (his theory is nothing).  And why the heck the milk would be in the wrong place.  (He was as confused as everyone else.)  Seems Manny keeps everyone guessing!

4pm, Mom and Dad came to help.  I had previously scheduled to be on a live TV show to promote cleft awareness ... and the set is at home (45 minutes away).  So Kaley stayed here, they stayed and watched Manny.  I booked it home, did my hair and makeup, ironed a shirt, ironed Zoe's shirt and did her hair, and with 1 minute to spare, got sitting for the interview.  They say it went well (Zoe wouldn't talk, little stinker).  But the live streaming will be available as early as Wednesday ... will post the link if it's not too horrible. 

8:30, back to hospital.  Manny is asleep in my Mom's arms.  Apparently, a surgical assistant came by very late this evening.  Not sure what all was discussed as Mom/Dad don't have all the questions to ask him, etc.  So apparently, consult will come by again tomorrow, trying to schedule it when the Neuro is here. 

I have no clue the plan.  I just know that when he retches ... it's bad.  It's scary.  It's life threatening.  Some moments he's fine ... even a whole day here and there.  But even one could prove to be "too much".  I feel like we're missing a critical piece of the puzzle. 

But for now, little bit is sleeping.  Milk is going the right direction.  No retching.  So all in all, it's a great night. 

Sep 15, 2011

The rest of the hospital stay story

(Written Tuesday night after the tube was placed but we weren't discharged.)

Since the last post, I've been doing some heavy duty thinking.  I was very, very patient with how the whole tube debocle went.  And clearly, I used up all my patience on waiting on that.  Because now, I am out of patience. 

As you know, he got his tube in at 3 but didn't start eating until 9:30pm.  Why?  Because they forgot to write the orders for food, it just said "Fluids".  The next problem?  He started retching and desatting. 

Finally got the OK to start feeds. 

Next problem?  About 10:30, I went to change Manny's diaper and it was wet on the outside.  His bed was soaked.  Why?  The nurse had not put his milk into his tube correctly and it was laying in his lap.  Seriously? 

Next problem?  When he is on feeds, he is suppose to have erythromycin (it helps the motility in the stomach).  He started feeds but he didn't get that OR his Prilosec (for GERD).  2 more "over sights".

Next problem? He had a rash/infection starting on his gtube area (I told them) and they were to write the order for some bactriban so it doesn't turn into cellulitis.  I reminded them 3 times.  FINALLY at 11pm it showed up.  (Only took 8 hours. sheesh)

So now the issue is next steps.  They really should not release a baby to go home with the kind of symptoms he's having ... his retching causes respiratory distress.  But I have a feeling that they will just overlook that and discharge him tomorrow.  Which is fine ... because I'm leaving tomorrow one way or another.  I have lost almost all confidence in this team today.  I don't know if the doctors got the messages of what was going on or not.  I have a feeling the nurses didn't relay the messages.  (Just a guess as his doctors are usually right on top of things.)  I think everyone was soooo relieved to get that stupid tube in that they lost sight of the patient.

I love the nurses up on the 8th floor.  Somehow down here, we're not a priority to the nurses.  We're in the oncology wing and there are very sick little kids around here.  So I guess a kid with desats and respiratory distress who hasn't eaten in 5 days is way down on the totem poll.  (Or maybe I'm just in a bad mood.) 

I have no clue why this has been soooo botched but it's quite bad. 

(Written after I got home and had some time to reflect and synthesize.)

Tuesday night I got so upset that if I'd had a car, I would have left in the middle of the night.  I was THAT upset.  One time during that night, the nurse came in with water (flush) bottles. (For those who don't know, you have to Flush tubes with water every so often ... need syringes and water.)  I reminded her that she never brought the syringes and said she's get them.  2 hours later she came back (without syringes) and started writing on the bottles.  She said that JCO was coming by in the morning and they wanted to make sure everything was perfect.  What about the syringes.  Oh yeah.  (By the way, I'm STILL Waiting for those syringes.)  So basically, patient care was non existent. 

She said we'd likely be discharged the next day since he was tolerating feeds.  I was like, "I am for sure going home tomorrow since the doctors don't seem concerned about the retching."  She was like, "Yeah".  (She thought I meant that THEY weren't concerned so *I* shouldn't be concerned.  But really, I was like ... I'm OUTTA HERE BECAUSE they are not concerned.")  She was a few fries short of a happy meal though so it was all lost on her. 

I was ticked.  But decided to channel it all in writing a letter to my pediatrician.  I left out ALL the emotions and only put in fact after fact.  The end was a plea of what to do next.  That gave me enough focus to finally let it go and go to bed. 

The next morning, I packed my stuff and waited.  By the morning, I'd had time to process and realize: What is it EXACTLY that I'm fighting for. 

1)  What to do when he is retching? 
  • Is it causing permanent heart/lung damage?
  • When should I bring him to the ER? 
  • how do I know when it's him not tolerating the feeds versus the regular retching?
  • anything that can be done about it? 
2)  What happened to the second tube so we don't do this again?

Eventually, Dr. Cartaya got there.  It was the PERFECT doctor for me.  He asked if I was ready to go home.  I pointed to my already packed bags but that I had a concern.  I asked him those questions above and got answers.  (And by the way, he WASN'T notified of the retching!!  Next time, I call him myself!)  We discussed all sorts of things (maybe I can include those in another post). But basically, there's likely not a lot we can do during these episodes, probably need BIPAP to support him.  And no one knows about the second tube.   (ugh)

He left and I felt a peace.  In the night, I'd already written the pediatrician.  I'd already written the specialist doctor with Manny's condition.  I'd already written the muscuar dystrophy clinic for recommendations of doctors. etc.  So I had a plan in works.

He came in a few minutes later and said, "Change of Plans ... you have to stay another week."  To which I replied, "I will be needing one of those AMA forms to fill out."  (Meaning I will be leaving Against Medical Advice.)  He laughed!  He was just teasing of course and was only in to ask if I needed refills on meds. 

He hugged me and patted Manny on the head.  And we were discharged.  The nurse came in and took out the IV. 

There were more debacles ... like his feeds are supposed to be continuous. I told the nurse that his food was almost gone. I had no formula in the room. He never came back. An HOUR later, he came in and I reminded him of the missing formula and he went to hunt again. He never did come back with formula until I was walking out the door.

Kaley and I had bags in hand and were walking out the door when the GI's Physician Assistant came in.  She apologized she didn't get here before we were discharged but said Cartaya told her we still had concerns.  I told her about the retching and desatting ... her eyes got HUGE and she was clearly upset that we'd be discharged in that condition.  I was in shock too ... but also ready to get out of there.  I told her the story and to put her brain around it.  I'll go back next week for a follow up and maybe they'll have a plan together by then. 

Soooo glad this hospital visit is over.  And remind me, don't ever do a hospital stay when JCO is in town!  Run for the hills. 

Sep 13, 2011

Rollercoaster Day

Woke up this morning to a baby getting a finger poke.  Amazingly, he barely cried.  And the CMP (what they ordered) was "basically" normal.  A few items better, a few worse but overall about the same.  So no worse for the wear ... amazingly!

But ... no tube! 

Dr. Cuenca (One of the GIs) came in today and calmly (with smiles) said that she has taken this issue all the way to the administrators of the hospital.  Yes, she knew that JCO was here this week ... but this is not right.  She asked Manny what color pen he was holding.  "Red"  .  She then walked out to the nurses station and began to give the third degree to several people on the phone! 

Kaley ... the world's best helper ... still has an amazing attitude and a helpful heart.  Today, she was combing his hair.  What do you think?  What a handsome dude!

12:00 Dr. Cartaya comes in and discusses how he's doing overall ... which is amazingly well.  And leaves.  About 5 minutes later, he pops his head in the door and yells, "We have a tube."  And closes the door!  What?!? Yippee!! 

12:20 they are ready for us to go down. 

Arrive in IR ... not quite ready for us afterall.  No room for him.  But that's OK, we had his wheelchair and some toys. 
Eventually, Alisha came to get us.  There are two options for placing this tube.  1) Anesthesia where they knock him out, the whole sch-bang.  (comes with many risks).  2)  Alisha gives him some mild sedative just to take the edge off and does it with him awake.  I much prefer option 2.  Sometimes I'm not given the choice ... today I was. 

So Alisha takes him away and he cries (much separation anxiety) but I know it won't be long before he gets meds and chills out. 

1 hour and 15 minutes later, she comes out with him.  He's groggy and chill but fine.  As we head up to our room, she tells me it was very difficult to get it in.  Maybe scar tissue.  He has weird turns and twists in his bowel.  And she also thinks that this tube might not be quite long enough either.  This is the first time we're going from a 22 to a 30.  The only other length is a 45 (which she said would "Be out his butt").  But his tube was clearly out of place for a long time she said because the hole from his stomach to his intestine was reclosed. 

So now it's 3:15 pm and we're back in our room.  We're waiting for orders to actually USE the tube.  Seems the orders weren't written to start feeds.  (Not sure why ... if it's oversight or if they purposely wanted to do something else first.) 

I know she wanted to talk to me about using Reglan.  And I'm just not ready to go there yet.  The potential side effects are just too big and possibly permanent.  So I need more time on that. 

I also need to find out what happened with the second tube they were trying to order.  Did it come?  Did they find a new way to get it to us?  What happened?   Must find out ... can NOT do this again. 


OK now on to a new subject.  I called the Pulmonologist just now to get the results of the sleep study.    "Basically Normal".  What the heck?  Guess it's great news ... it's just CONFUSING news.  Apparently, the respiratory symptoms were COMPLETELY caused by the GI issues and have basically no bearing or effect on his respiratory system.  Seriously??   He desatted a few times but not too bad or for too long.  Low 80's but never stayed down for more than 5 minutes. 

So don't get me wrong.  This is WONDERFUL news.  It just caught me off guard. It shows his lungs have not been compromised.  And that is PRAISEWORTHY news!

And just for fun, while I was typing this ... he went into respiratory distress - retching, etc.  (And no, they haven't started feeds yet).  Nurses came running from everywhere.  I got it on video as "proof" of what he was doing.  It all only lasted about 3 minutes.  But it sure gets your blood pressure going!

We thought it was all about food in his tummy ... but clearly, it's not.  There's nothing in his tummy.

Over the next hour, he's done this 3 times.  His pulse goes way high, his sats go way low.  He stops breathing.  Now what?

So it's 5:15 and he's napping.  Those retching episodes always leave him exhausted.  (Back last year, they thought these were seizures because he looked so "spent" after.)

His retching has slowed down but it's not over and he still hasn't been fed.  He did NONE the days without the tube in. 

7:30 Dan arrives with the kids and dinner.  What a welcome sight!  The baby is soooo grumpy again.  Yelling "OWIE" and not telling me where.  He's not been like this since his tube came out on Friday.  We sat and discussed our options. They had to leave at 9 to get home to bed as the kids have school tomorrow.  (They usually go to bed at 7pm and it's a 40 minute ride home.) 

9pm Just told the nurse to call again.  Now.  We're stuck in limbo.  He's doing less retching (only one this hour).  They still haven't started the feeds.  They were SUPPOSED to start feeds at 3pm when he came back from the procedure but the formula hadn't arrived from dietary.  So while we were waiting for it, he was doing the horrible retching with desats.  Now the food and pump and bag are all sitting here ready to go but without the Okey Dokey to do it.  He's still on the TPN like he's been on since Saturday.  But I'm am about to lose it here.

9:20 Nurse came in and said the doc said to start the feeds on half rate and we'll go from there.  At least it's progress.  I'm just supposed to notify the nurse if/when he retches. 

Tired Mama.  Grumpy Baby.  Thankful for happy big sister. 

Sep 12, 2011

No Tube Yet! aaaaauuuugggghhhh

We were in hopes that today would be the day we could have our tube and go home.  That was Plan A.  But alas, it was not to be.  So we wait again.  The tube SHOULD be here by tonight.  If it is, then we'll be the first one to go to surgery tomorrow.  And then a few hours to make sure it's working.  And then home.  That's the plan anyway.  Plan B. 

None of the doctors want to come in here today.  Normally, we see the Hospitalist and the GI.  But today, they both sent in their assistants.  (Even though they were just outside.)  I think they didn't want to be the ones to tell us the bad news.  (Our nurse told us first thing this morning.)  And they are all fit to be tied! 

So it's been clarified what happened.  The hospital is working their butts off Friday afternoon, all weekend and even today to locate a tube.  The DME that carries them is closed all weekend.  Seriously.  That's it.  So the hospital assured me they made the DME send two.  They are bound and determined that this will NOT happen again!  5 day hospital stay for a 45 minute outpatient procedure is just insane. 

The nurse told me that everyone is asking "How's the Mom taking this?"  And of course, they're all in shock that I'm making less of a stink about it than the Doctors are.  They're TICKED.  I'd rather them do the yelling and let me be the nice one.  (Yelling doesn't help anyway!)  The staff that know me know that I'm as calm and cool as they come. 

So while we wait, we're just trying to keep ourselves entertained. 

Here are a few things we're doing: 

This is what we call a "hand bath".  We played on the floor for about 2 hours.  Warm water in the basin.  Add floatable toys.  Add animals.  Add bubbles.  Splash.   Soak Mama.  Repeat.  He pitched a huge fit when it was over because he wanted to keep playing.  Guess you could say it was a "hit".  But we eventually had to get up, Kaley and I could only sit on the floor curled up for so long before our legs cramped up.  We have to sit behind him because he can ALMOST sit independently, but not quite. 

This is a view of me holding him and Kaley helping hold the bubble wand.  He takes the BIGGEST breaths in order to do this.  But he's a pro now at bubble blowing.  We do it to help him inhale and exhale.  He can do this forever too!

Kaley also put him in the bed surrounded by pillows and kept making the bed go up and down.  It was a great arm workout for Kaley and a fun ride for Manny. 

The rest of the time?  We've been writing, doing letters, watching DVDs (especially Baby Einstein), playing on his laptop, playing with cars and animals.  I came quite prepared.  I knew we'd have to be her until today so I was all set. 

11am  We get to leave the room!  OK it's only for a procedure, but hey ... it's an adventure out of these 4 walls.  We go to get an ultrasound of his liver, gall bladder and pancreas.  (I expect them to all be normal.)  But they're checking anyway.  Some of his liver enzymes are always "off".  Not sure why. 

And while we're talking blood tests, his amylase and lipase came back normal.  His WBC is still quite high and several of his labs are "off".  Much of it is about muscle wasting though if I'm reading and interpreting them correctly.  (sigh) 

2pm  So I smell this "odd" smell and decide to investigate.  Sure enough, his tube area is getting infected.  We can't tell if it's from the foley or from the tegaderm.  There's redness and I'm keeping an eye on it for now.  But I know from his past that he can go from fine to septic in no time.  (Long time followers will likely remember THAT!)  So trust me, I'm "on it" quickly when I see that. 

Rest of the day ... pretty unadventurous. 

6pm Manny falls asleep and no way to awaken him.  It's tooooo early.  But then again, if we have to get up early for the procedure tomorrow maybe it will work out.  But what if he wakes up at say 10pm when I'm ready to crash?

7:30, Mom shows up with a Whopper! Yum.  Thanks Mom.  I know it was a very long day and it just added to your long day to stop here.  But it certainly was nice to chat. 

8pm, The night nurse shows up and says she isn't positive if the tube has arrived or not.  Darnit.  It was supposed to be here before now and should have been in the chart.  Trust me, many, many people in the hospital are aware of our situation.  (And not due to me, I've been stuck in here trying to keep a hungry baby happy.)  It's not a good sign.  But there's still hope.  I'll feel better when I hear the words, "It is here."

Ironically the accrediting board for the hospital is here this week (starting today) and they're all a bit on edge, crossing every t and dotting every i.  Ironically, WE are something that has proven to be a MAJOR systems failure.  I think they all want to keep me happy and keep JCO away from me. 

9pm finally lay the baby down in his crib.  He'd been in my arms this whole time.  I knew laying him down could wake him up but I also knew I couldn't hold him (or my bladder) all night.  So it had to be done.  I was successful!  It worked!! 

And JUST as I was walking away, the Respiratory therapist came in, knocking LOUDLY, talking LOUDLY.  She's one I have had before and do not enjoy her.  We get into some pretty interesting conversations but tonight it's not going to work and there's NO way she could just come in, do the breathing treatment quietly and leave.  So I say (kindly), we would like to refuse all breathing treatments for the night.  Thank you. 

Guess it hurt her feelings because later, the nurse had to tell me "The RT felt HORRIBLE..." blah blah blah.  So I said, "It's no big deal, everything's fine. I am just trying to achieve some normalcy for this baby." 

But between all the interruptions, the beeps, the loud people ... it's now almost 10pm and guess who is WIDE AWAKE?  (One hint:  NOT ME!) 

Oh, and the nurse informed me they need new labs in the morning. 5am! You have gotta be kidding me.  We talked about him being a difficult stick.  Hoping a bullet will be enough and if so, they can do a finger poke.  If she does it right, she can get it to drip and not hemolyze.  And then HOPEFULLY off to the procedure. 

This is Plan B. 

Hoping there will not be a Plan C. 

Sep 11, 2011

Sorta Slow Sunday

Uneventful night ... except the nurses keep forgetting to reset the fluids when they come in.  They're only supposed to do 2 hours at a time and they're supposed to come in once every 2 hours so it's supposed to be a reminder to check the patient. But it just turns into 20 minutes of beeping followed by me having to get up, hunt down the nurse and get her to fix it.  Seriously thinking of learning to reset it myself.  Looks easy.  I know I'm not supposed to but seriously.  This is getting old. 

Then at 7:15 am, the respiratory therapist came in.  Seriously??  She only had to see him once today and she has from 7am-7pm to get that in.  There wasn't possibly anyone else who needed say 4 treatments?  I was very upset ... it wakes him up and he was screaming.  But thankfully, he went back to sleep so I guess it was fine afterall. 

Dr. Cartaya made his usual rounds.  Nothing new. 

Baby's heart rate is going back up again.  I have noticed it's either a fever or pain which cause it to rise.  And since he has no fever, I have to assume he has pain somewhere... just don't know where.  (But I keep checking and looking.) 

Dr. Cuenca (the GI) arrived.  I got her to stay for a few seconds longer than normal.  I know they want to take care of the acute nature of this stay.  That's a no-brainer.  But I also wanted them to start addressing the chronic nature of this.  We're on his 4th jtube in 6 1/2 months.  (And that's with us waiting 3 weeks one time while it wasn't working and this whole month where something was wrong.)  So we need to get to the bottom of it. 

It's possible, it's just a function of the nissen fundolication.  Retching can be a side effect.  (It's like taking a headache medicine and the side effect says "may cause headache".)  I asked about a formula change ... she thought that wasn't it but we can address it again later.  I asked about switching to separate j and g tubes and she immediately was like, "DON'T DO THAT."  She explained why it would be bad for his situation and it does sound like a problem. 

She said there are some surgeries to open up the bottom part of the stomach better for the gastroparesis ... but it's a bad surgery too.  (And we're not there yet.)  She suggested to vent him more.  (We already do once an hour and all night ... but he might need 24 hour venting.)  And the only other thing is to use a more powerful prokinetic.  Reglan is the only one left.  It has side effects so I'm not so sure that's a great idea either.  But clearly, we have to do something. 

She then came back a bit later and said she'd ordered him an ultrasound of the gallbladder, liver, etc.  And ordered some more blood tests (at which point I cringed as I know how hard that is to get from him!)  But clearly, she was thinking about the behind the scenes of this and not just doing the quick fix. 

They aren't sure if the tube will arrive by tomorrow or not.  We won't know until tomorrow.  But if it arrives,we will definitely do the replacement then, start the feeds and if all goes well ... go home tomorrow (Monday).  If not, we have to wait another day.  So good thoughts! 

Then we were sitting around talking to the nurse.  Kaley and I were sitting on the couch, Manny sitting in a chair.  No one moved ... nothing ... and his IV just popped out of his arm!  Seriously.  Not even kidding.  I've never heard of such a thing.  The nurse said, "If I wasn't here to see it myself, I would have NOT believed you!"  (She told several nurses and they didn't believe her!)  But it's the truth. He'd been complaining about wanting it off earlier in the day.  Sure 'nuff ... once it came off, his heart rate went back to normal.  He was in pain.  Oh, and of course, he has an allergy rash all over his arm where the tape was.  (sigh). 

We were just about to go to the room where they put in the ivs when Dan and the kids arrived.  They were only going to stay about 30-45 minutes (it often takes longer than that to get the IV in).  So we did the visit first.  They were funny ... at home they never want to watch Tinkerbell... but it was on the screen and they were GLUED! 

Time flew and they left.  It was soooo sad.  When they left, I was holding Manny and he said, "Let's go Mama".  He was confused why we couldn't go with them!  I was at the doorway of our room and he was leaning saying, "Go Mama".  If he could walk, he would have torn off after them! 

Then time to go to the IV room.  It was "only" 2 more sticks.  (7 this week if you're keeping track, not to count the ones from last weekend.)  But they got it.  We're on the oncology/hematology ward ... so these people are used to blood, needles, ivs, etc ... and the charge nurse was the one doing it.  She said it was a definite challenge and she wasn't sure she'd be able to get it.  Oh, and I am once again glad I was here ... the GI doc told me they were going to do an amylase and lipase test.  But she didn't tell the nurse.  I insisted the GI told me there was a blood test to do ... glad I did.  She looked deep in the chart and found the orders.  Without me paying attention, they would have had to go back later for yet another blood draw. 

Now it's 5:30 pm and we're back in the room.  He's watching Diego on a video we brought and all is right in the world again. 

tray, with dan, gang

Sep 10, 2011

It's Saturday so I must be in the Hospital

Uneventful night (well, as uneventful as a hospital visit can be).  And the best news of all is that there was NO retching.  NO heart issues.  NO breathing issues.  Apparently, this was all GI related. 

We don't know if his tube was out of place or what but ... this round is over so we're good.  Now we just wait until Monday when they can replace the tube. 

In the meantime, hydration and calories are the biggest concern.  He can get plenty of fluids to keep him hydrated via the IV line.  But it's not enough calories or nutrition.  So he'll end up getting very weak by Monday.  Dr. Cartaya came in today and said that was his concern too ... so he's ordering some TPN.  Now normally, that goes in a central line (he doesn't have a central line).  They don't want to do a central line (involves surgery) just to give him nutrition.  So Cartaya is going to do some "Mickey Mouse TPN" as he called it, meaning TRYING to get him at least SOMETHING with some calories and SOME nutrition. 

GI hasn't been by today.  I think they're going to only want to deal with the acute issue, the dislodged Jtube and not address the chronic nature of why he was retching and why this keeps happening.  BUT I'd like to at least discuss it and get it on their radar. I have friends with tube fed kids and they're all offering ideas for us such as switching formulas, switching to a separate j and g tube, etc.  Cartaya suggested his nissen funoplication might be loosening. 

1pm ... his foley just Popped out of his body again even though it was taped.  I called the nurse for another foley and began to take off the gauze and tape so it would be ready for the new one. 

Guess what?  Horrible tape allergy again.  Even to the ICU tape that is supposed to be the lowest allergy around.  Well, it's clearly obvious when the red parts are the EXACT size of the tape and there is a normal skin part where the gauze was.  The picture doesn't do it justice.  The rash is spreading to his whole tummy.

So they put on a new type of tape but VERY little of it. 

2:00, GI comes in and sees his foley.  She immediately took off the tape and gauze to see the site and noticed the foley was way too far in.  She taped it where she wanted it to be. 

But wouldn't you know?  The tape that had only been there for an hour had already caused a rash ... and it peeled a bit of skin off.  And this is the same tape the GI just used on him. 

We discussed what could be going on and I described what is happening.  She said he might need a nissen revision because clearly it's not completely effective. 

So the GI leaves and the nurse and I work on getting the tape off but keeping the foley where she wanted it.  This time we're trying tegaderm.  We'll see. 

7:30 and the old tape redness is still there.  As are the patches where the skin rubbed off.  But the tegaderm might just be the winner!  (Good to know).  And it's holding the foley in place. 

They wanted a stool culture ... but of course, he hasn't gone all day.  Then again, they didn't order the CPT vest (the shakey machine which actually helps him do this too).  And he hasn't been eating, only IV fluids so I doubt he has a lot to poop anyway. 

I've noticed as the day has gone on, he's definitely getting weaker.  He's not able to hold his head up right now, much less trunk support.  It's all gone.  He isn't super grumpy yet, but in the past I know he usually gets that way eventually. 

As of a few minutes ago, the day nurse just left and said they have still been unsuccessful in ordering the kit that he will need that has his new tube in it.  What a crazy system!?!  If they can't order it first thing tomorrow, there's no way to get it here by Monday.  Seriously?  So they're ordering us two.  I know we're not allowed to have one of these kits because it is surgical (guess they don't want people trying to put them in themselves!) but they might be able to get around it by say giving it to our CMS nurse or something similar.  Where it is "his" but not in our possession.  (Works for me.)  Then we won't have this trouble again.  Then the only issue could be ... who can put it in soonest. 

We got this switched to a j tube February 16.  We had a new one put in End of February (the thing migrated).  A third one placed May 25 due to blockage.  And now this one in September.  So 4 in 7 months.  I think it a GREAT idea to have one on hand considering this is averaging one in less than 2 months. 

All in all, a fairly uneventful day.  (Besides the foley coming out and the multiple tape allergies.)  And once again, I say ... can't imagine what could/would have happened if we had not been here with him.  (shudder). 

So I leave you with this one last view.  He was sitting in my lap and this is the view *I* see when I look down at him.  Pretty precious, huh? 

Sep 9, 2011

Annnnd They're Back

Apparently, we like to spend the weekends in the hospital.  Because here we are again.

Last weekend, it was respiratory distress and atelectasis, c-diff and conjunctivitis.  He went home and t. 
hat same night, he was having all the respiratory distress symptoms again plus retching. 

Late yesterday, after brainstorming with the CMD doctor, we realized this whole thing could be GI related with an incidental finding of atelectasis and c-diff.  Sooooo ... I started to put together a plan

Last night he did horribly again, waking often and mild respiratory distress.  This morning I was weighing what to do and how to handle it.  I put a call into the GI.  While we waited for the call back, I went to Walmart for our weekly groceries. 

As I was getting Manny out of the van to go in the house, he was saying, "Owie" and when I picked him up, I heard this POP and saw that his tube was out of place.  Normally I can only see the button, but now I could see about 6-8 inches of tube.  The balloon for the G tube (which holds it in place) was burst.  Because the tube didn't come completely out, I knew I had a couple of hours to get to the ER/Hospital. 

I could feel my blood pressure shoot up.  I kept reassuring myself that this was fine but I was in SHOCK, not believing that this had truly happened.  But at the same time, I said aloud, "Thanks for a clear answer."  You see, earlier in the day, I wasn't sure if we should be in the hospital or what ... but now it was PERFECTLY CLEAR. 

I put in another call to the GI and a call to the Pediatrician.  The Pedi called back first and I told her I knew we'd need to be admitted. 

Why?  He has a gj tube which must be surgically implanted under general anesthesia and they do it in Interventional Radiology ... they insert dye and then watch it go in. A G tube insertion isn't helpful for him as his stomach is completely shut down (even his stomach acids and saliva have to be vented out normally).  So he has to have IV fluids immediately or he will dehydrate.  He's on continuous feeds and can have nothing in his mouth or stomach ... so it either has to go in his jtube (which is obviously not there) or his veins.  Thus the need to be admitted.

We arrive at the hospital and remember how I said the jtube came almost completely out?  It was hanging in there while we got to the hospital. As we were sitting and waiting for admitting, the rest of it just popped out with a sound. And now it's a true emergency as they only have so long before the stoma (hole) closes up completely. So they rushed us through admitting (we were admitted last weekend so not much has changed since then!) and got us to our room.

Guess who wants to be like Mama?

I did a quick, "Hi ... his stoma needs to be kept opened" and that's how we met our nurse today. Meanwhile his shirt and pants were SOAKED from all the stomach contents leaking. (So much fun)  They put in a temporary "Place holder" to keep it opened and a bunch of gauze to keep the stomach contents from leaking everywhere.

Kaley is going to spend the night with us tonight and help keep him entertained. YAY me!! This means I can actually go to the restroom, take a shower and leave to get food. And with her here, I will remember to EAT too (I usually forget!)

And I should add, he WAS in pain but once the thing popped (10am), his pain went away.
They were able to get the IV in on the first stick (YAY) and one of the blood vials but they needed two.  They tried a finger stick for the second one as they only needed a small amount (Bullet).  But it failed to give them enough so they gave him a break.  Later they came back and did a bigger stick on the finger and it was enough blood.  Unfortunately, it clotted and couldn't be used.  So they came back with another nurse and she found a vein, went for it but it blew.  So 4 sticks and still not all the blood they need.  They will send in the IV team later.
Now it's almost 7:30 and uneventful.  Getting all set up for the evening routine. 
9pm and he starts to cry a LOT.  Owie, owie.  I check all the usual spots and he doesn't want me to touch his stomach.  So I pull up his shirt and this is what I find: 
What you're looking at ... the tube thing is called a foley and it's keeping the hole (stoma) opened until Monday when the tube can be reinserted.  (They close up fast ... like in hours.)  To hold it in place, they have to tape it.  And the gauze is to absorb all the things that leak out of his tummy.  Well, they used paper tape ... even though it's on his chart as an allergy.  But they chanced it.  Sure enough, ALLERGIC reaction. (Do you see the slight red just above his diaper, to the right of the tape?) The bottom corner has actually pulled away some of his skin.  And the skin is ooozing (I know you can't see that in the picture).  Seriously ... after only about 6 hours.  There's some on his foot and he's fine but not on his tummy.  So they replaced it. 
9:15 the IV team came in ... Mani is her name (Pronounced the same).  She hugged me when she saw me ... we're becoming friends LOL.  She said she didn't recognize the name but when she opened the door, she knew us immediately.  She said the staff all were like, "You know her, she's the Barbie looking lady."  LOL LOL LOL ... years ago I heard people say that about me but not recently.  I feel 100 years old these days. 
She got it on the first stick (she always does).  I even asked for her at 2 when we arrived but Nooooo, they all had to try it first.  (ugh)  I will insist harder next time!   But she had to use a wrist vein ... she calls them the "Mean veins" because they hurt the most.  But she was out of other options of viable veins.  But it was quick and it's over. 
Oh, and I forgot to mention, Dr. Shamma, the doc from last weekend is our doc again.  She had just come from the 8th floor (our usual floor) and she said that she told them she was coming to see Manny.  They were all jealous!  They were upset they didn't get him.  Me too, actually ... but their floor was full except for double rooms and since he tested for c-diff, he has to be in isolation (which is fine with me).  But I thought I might be able to slip in and out without them knowing, but word has spread.  :)  
Now it's 10 and he's sleeping in his bed.  He told me he was ready for bed.  Asked for a kiss and drifted off watching Baby Einsteins (Thanks Donna Gower!  They have been life savers.) 
So until tomorrow.  Bye from Beth, Kaley and Manny.  Hoping for an uneventful night.

Sep 8, 2011

Sleep Study

We drove over to the sleep study (almost 45 minutes away) and it was already bedtime when we left.  I knew it was going to be darned hard to keep him awake with  no help in the backseat.  But I did it,  sorta. 

When we got there, it was the same room as the last time.  And it was the same tech as last time.  (She remembered us.)  I have a dear friend named Stephanie who ironically has been helping me with all this and guess what the tech's name was ... Stephanie!  Thought that a very good sign.

Got him all hooked up.  At first he was cool with all the "stickers" all over.  And he was even cool with the things in his hair. (Kaley has been putting barrettes in his hair, much to my dislike ... but tonight it paid off ... must thank her.)  But he didn't like all the stuff on his face and at this point, he had a meltdown. 

Got him to the room and the tech finished with the electrodes and off she went.  We watched a DVD I brought.  By the end of one, he said, "Off" and went to sleep.  Pretty uneventful. 

I settled in the very, very uncomfortable, LOUD chair next to him. I was shocked at how it didn't exactly lay down, how I got no pillows, how it creaked loudly every time I moved ... then realized, it wasn't MY sleep study so just get over it. 

It wasn't long before I noticed he stopped breathing a few times.  In our room, the only machine was one that read his respiratory rate and his ETCO2.  RR was VERY high all night (except when he stopped breathing).  I saw it as high as 51 (while sleeping) and low of 9.  Most of the night it hovered around 44.

The ETCO2 was very low.  And every time I looked at the ETCO2, it was very low. I have no idea if it ever spiked, but never while I was looking at it. The highest I caught was 37. The lowest I saw was 6. Most of the night it hovered around 20.  I'd been focused so much on high co2 that I didn't research low. I'll do that today.

The middle of the night was uneventful from my perspective. He woke up a lot (like he does at home recently). He also is doing increased coughing spurts. He has NOOOO cough during the day.

hTe tech came in at 5:13 and said "the test is over." She started to take a few leads off then said, "I'll be right back." While she was gone, he started the strange retching thing ... but the test was already turned off. (Seriously?) When she came in, she saw what he was doing but the test was off as were some of the leads. I told her THIS IS WHAT HE DOES. And she didn't seem worried, saying she already had it. So maybe he did it in the middle of the night and I didn't wake up for it? Who knows. But I was very upset that it was SOOOOO close to being "caught" and then missed by like 3 minutes? (Hopefully that's not the case.) And the episode lasted long enough that by the time we got to the car (about 15 minutes later) it was still going.

The tech she seems genuinely interested in him. I told her that last time, the sleep study was "shockingly normal". When asking her what she COULD tell me about the night she said, "They won't be able to say that it was normal this time."  She said they had some good data that they will be able to analyze and then determine what is the best course of action for him.

So it made me feel better that they saw "something" ... I just don't know what it was.

It was very clear to me that he works very, very hard all night long.  In the day time, he has a normal respiratory rate and sounds "normal".  He doesn't sweat.  But at night, he is DRINCHED like he is doing a marathon all night long.  He sounds like he's heavy breathing (like you'd do if you just ran really fast or very long).  It is not right. 

Have no clue how long it will be before we hear about the results but it could be as late as Monday or Tuesday! Can you believe that??!!??

Going to be a loooooong weekend.

Sep 5, 2011

Cute Manny Stories

Enough depressing medical stuff.  How about some cute Manny stories?

We were bored.  And in our boredom, I come up with some crazy stuff for us to do.  I can't help myself and everything will eventually turn into an opportunity to learn something or to do speech therapy, occupational therapy or physical therapy.  (Can't help myself.) 

For example, we had a trash can in our room that had a foot petal.  I would hold him in my arms and he would say, "Up" and I would make it go up.  He would say, "Down" and I would make it go down.  I'm sure I did it over 1000 times.  Well, my parents came to visit and Dad had to throw something away.  Manny noticed Papa push the foot petal so Manny started saying, "Up, Down" and I explained our game them.  Mom said, "I'm sorry you're so bored!"  Never even occured to me that would be a sign of boredom.

Or here's another game.  I will blow bubbles and I would have Manny reach his hand up and pop the bubble.  Eventually, he started getting some of the gum on his thumb and forefingers.  He'd play with that for a while and then ask me "Off pease".  Each time I would take the gum off his fingers he would say, "Thank you".  Then one time he said, "Xie Xie" (which is the Chinese word for Thank you!).  I cracked up.  I know he watches Ni Hao Kai Lan but I had no clue he understood what that meant, much less how to use it properly.  And again tonight, he was still saying thanks in Chinese when people were doing things for him! LOL

And here's another story.  While Dad was at the hospital he asked if I'd seen any football.  Manny overheard the conversation and said, "Pirate".  It took me a second but then I realized it was him free associating.  Remember how we went to a Buccaneers's football game?  Their uniforms have pirates on them!  I was very, very impressed how he made that leap. 

I said, "You're right Manny, the pirate is from the Buccaneers".  And then he pointed to his ears and said, "Ears? Buc ears?"  His way of asking me, "I know what ears are but what are BUC ears??"  LOL LOL

Finally, tonight I wanted to give Manny a bath and de-hospitalize him.  We have a thing that we can use in the bath when we want to shower him but he HATES showers and loves baths.  So Kaley usually has to get in with her bathing suit on and hold him.  Tonight we decided to try something new.  I bought a swing for him that we want to hang on our patio but haven't hung it yet.  And it worked!  It was just enough support that he could sit in the bathtub.  Kaley was still in with him but she didn't have to hold him and he could play.  They were in there for over an hour!  What fun. 

I just don't want everyone to forget.  I know you all are fervent and heartfelt in your prayers for this little guy.  And he needs every one of them.  He is a sick little boy and there are a lot of struggles in his life.  But I also don't want you to only know the medical conditions.  I want you to know and love MANNY.  And he, knows how to live life.

Guess what

Manny did not desat or go into respiratory distress last night so he must be getting better. That's the good part, that his acute status is seemingly "under control". Praise God.  Yippee!  Yee Haw. 

The pulmonologist was just here and from his point of view, we're cleared to go home today.  And most of me is thrilled with that of course.  We can do most of the things at home that they were doing here.  And we can monitor him.  I know the signs.  And I think we'll all be more comfortable there. 

We haven't been cleared by the hospitalist or the GI (for his c-diff) but I have a feeling they'll let me monitor this at home since I have before.  But that's just a guess. 

My concern, though is that the chronic nature of this is taking a toll on his heart. We are scheduled for a sleep study on Friday (already had the appointment before this illness).  And that will tell us a lot hopefully. 

But like last night, his heart rate when he first falls asleep is about 95-110.  When he's awake, it's about 120-145.  When he's completely deeply asleep in the middle of the night ... it's 170 plus.  SOMETHING is going on and I think it's going to be resolved by BIPAP.  I mentioned this to the pulmonologist and he said that we need a cardiac consult.  He also said that the sleep study will have a EKG on the whole time so they'll be able to monitor that then too.  I don't think this is dangerous enough to push for something NOW but it could be.  I think I have the time to wait until the sleep study.  They'll have the exact numbers and be able to go from there.  If it's as bad as I think it is, it will show up.  If it's not as bad as I think, it will give me peace of mind. 

So now I sit and wait for the Hospitalist and the GI to come by and tell me their plans.  Hoping we're all on the same page and we get to go home today.  And if that's the case, we owe his miraculous recovery to nothing other than the many fervent prayers of many people around the globe who love this little boy most have never met.  And for that, we will always be eternally grateful.  I cannot believe how many people rally their support for us when Manny is sick.  And we're also grateful to God for having mercy and hearing those prayers and giving Manny much needed relief. 

12:15 ... GI here and gave the all clear to go home! 

Sep 4, 2011

Feeling a bit of relief

Feeling a bit of relief.  I have no clue that I have made the right decision or not but I am feeling peace (for now) about how to proceed. 

The quick version is ... there's a difference of approach on how to treat Manny's current condition.  One group says to treat aggressively, the other says a more conservative approach is best.  I needed a tie breaker.

So I emailed my brother in law, Pat.  He's a doctor of radiology and I figured he was uniquely qualified in that he knows me, what kind of Mom I am, Manny, and would be able to interpret the xray report for us.  Sure enough, he called and was a HUGE help.  First, it helped to know that this was indeed a difficult position to be in.  He concurred that there was some gray area here.  Eventually, after I gave him all the facts I knew, answered all the questions he had, he gave his vote.  The conservative approach. 

Why?  One is the actual radiological report.  Apparently, it says, "Sub segmental atelectasis" which is extremely "minor" and he felt we had time and that it wasn't a huge emergency that needed to have these tests and treatments done immediately or else. 

So while neither of us are 100% positive this is the right approach, we have decided to go with it.  I breathed a small sigh of relief for the tie-breaker.  And now I'm vigilent.

Meanwhile, I had about a 45 minute discussion with the Pulmonologist.  He was very steadfast in his position and I asked why he and the CMD docs were looking at it quite differently.  He basically said, "I know Manny."  (Which is true.) 

But ... he also wrote orders up tonight to help us a bit.  There was some gray area last night and tonight there shouldn't be hopefully.  We also discussed a few of my questions.  Like how can he be in respiratory distress while his pulse ox says 98?  Basically, the 02 number is about the inhalation.  The respiratory distress can be about the lack of exhale.  (Thus the CMD's recommendation about doing blood gases to determine.) 

I know ... it's all very complicated and confusing and it's even harder to describe from such a tired Mama. 

Meanwhile, this morning, Manny started with c-diff looking diapers.  They ran cultures and sure enough ... it's positive.  So we've started on more meds and hopefully this doesn't complicate his healing process any further.  He has started retching a bit more today (which might mean he has to go off feeds ... which would be BAD). 

Oh and for fun, they put a catheter in his pee to collect urine since his WBC was 25000 they knew there was a lot of infection somewhere. He did not approve. (This was before the c-diff was positive ... it's likely what contributes to the very high WBC.)

He's starting increased retching and more fluid in his stomach when I vent ... a LOT of fluid so I asked my brother in law and he said that gastroenteritis can cause that. So he might have a stomach bug on top of the collapsed lungs, the c-diff, bronchiolitis and sinusitis.

I will just have to see what he does tonight and we will go from there.  To me, he seemed a tiny bit better today so it might actually mean he's on the mend.  I hope so.

Baby just fell asleep so I'd better too!  Thanks for bathing us in prayers.  We can truly feel them and know we're not alone.  I might be in this tiny room in isolation but we don't feel by ourselves.  ((Hugs)) to each of you.

And he has conjuctivitis.

What else can go wrong with a kid? No, don't answer that.

Beth Gore

All So Confusing

I know too much to be oblivious or not concerned, but not enough to understand what is going on or the best course of action.  Last night, Manny had a semi-decent night sleep (that's new).  And he woke up with a mild (not high) fever.  He did desat but not as long as before (but they also did immediate therapies like deep suctioning and the metaneb which helped).  So everything is "borderline".

And if we go by the way he looks and by his pulse ox numbers, he's doing pretty well.  But if we go by his xray, he's very, very sick.  I found out last night that he actually has some atelectasis is BOTH lungs.  The radiology report doesn't report how much so that I don't know.  And in both lungs it's in the middle section.  But on the left lung, it's also in the lower lobe. 

From my research, with kids with neuromuscular disorders (like Manny has), the lower left lobe is a very dangerous place for it to be and most difficult to treat. So I think I should be appropriately concerned based on the xray, the duration of his symptoms.  Even if he didn't have a super bad night last night.

Meanwhile, I love my team of doctors.  They have helped Manny so many times before and always made the right call.  So do I go with their thoughts on this one?  OR ...

I recently registered with this group called Congenital Muscular Dystrophy International Registry (CMDIR). Their goal is to find as many kids with CMD as possible, gather data, devise a standard plan of care, etc.  I was in touch with them recently giving them more updated medical info on Manny and while I did, I thought it would be good to send them a paragraph about his story and a picture so they could put a face and personality to the data.  They loved it and asked if they could put it on their website.  Yes.

Then they forwarded it on to another group called CureCMD.  I've been talking via email for 2 days about scheduling a time to chat with "Anne" (didn't know who she was) about using his story on their site too.  So on a lark last night in the middle of the night, I asked her opinion of how to know if I go with the literature or the local docs on how to treat Manny.  (The treatment plans are RADICALLY different.)  She gave amazing advice.

Then I began to wonder how to get my docs to "hear" this point of view.  Meanwhile, I was emailing 2 moms with Merosin Kids to get their BTDT advice since both of their kids had atelectasis.  One suggested I call Dr. Anne R.  And I immediately recognized the name as the one I'd been emailing back and forth all night. 

Turns out ... it's the Same person.  Dr. Anne R is the chair and founder of CureCMD, she's a Doctor who is a specialist in CDM and is actually a mother who has a child with CMD.  And she says we are on the wrong treatment path for Manny. 

So now I sit here waiting for our doc to arrive.  I'm praying for wisdom on what to say and how to say it so I'll be heard.  I have a gut that the CMD people know what they're talking about and I should go with that ... but it would require a much more aggressive approach. 

Praying for wisdom and clarity.  And just wanting my kid to be well. 

Sep 3, 2011

Mystery Solved

Yesterday I posted about the mystery and by today ... it's solved.  How?

Manny had 104.1 fever, 200 heart rate, respiratory distress, etc. so I took him to the hospital.  One xray later and it was confirmed ... atelectasis (along with bronchiolitis and sinusitis).  What is that?  Basically when part of the lungs collapse.  For normal, healthy adults it's not a huge deal.  For kids with neuromuscular disease it can be life threatening.  And treatment is tricky apparently.

I am thrilled to have my favorite team of docs on the case though.  As I write this, I have no clue what the course of treatment will be or the duration of our visit or the prognosis or the criteria for discharge.  I'll update as I have it. 

2pm Hospitalist came by and gathered a little bit of history, did a quick exam and told me they think it's viral so no need for antibiotics at this time.  I'm conflicted about this decision.  1) The standard of care for atelectasis for kids with neuromuscular disorders is to do an aggessive treatment of broad spectrum antibiotics as a secondary bacterial infection inevitably sets up in the lungs.  2) However, when he has antibiotics, his c-diff flares up and he has to go off feeds, has chemical imbalances and multiple other problems.  Sooo ... there's always this debate.  Pulmonary wants the antibiotic.  GI/Infectious diseases doc says no.  I don't know what to think.

This doc also said that if he has any respiratory issues tonight, he'll be sent to the PICU for bipap or cpap.  Apparently, at this hospital, the first time you're put on bipap/cpap, you have to be in the ICU.  That would be a great inconvenience to say the least.  (Less visiting hours, no bed, no TV, no space for "stuff", even his wheelchair can't be in there with him and that's his only seating, etc.) 

The Pulmonologist also seemed to think this was "no big deal" and I hope he's right.  Because what I read about atelectasis with kids like Manny ... well, it's very serious.  So I hope they know Manny. 

3pm and we had visitors.  Dan (daddy) and our friend Bob came by.  I'd driven Manny to the hospital by myself at 4am so the van is here with me.  We only have one vehicle so Dan is stranded without it.  So Bob swung by the house, left his wife Ann with the kids and brought Dan here.  Since he was on his way anyway, I had him bring us a few more things that I thought of we'd like to have while we're here (like a chair for Manny and some food for me). 

Nothing like hanging out with Daddy ... even if y don't feel well.  And those of you who know Manny can tell by the picture that he isn't himself.  Even his voice sounds weak right now.  Poor baby. 

They left at 4pm and just as Daddy was leaving, Manny started to drift to sleep.  That's yet another proof he's not 100% as this kid doesn't nap ... even when sick!  He must be wiped out. 

Other symptoms he has that I haven't mentioned.  He has the most disgusting yellow goop draining from his eyes.  When he woke up this morning at 4am, his eyes were sealed SHUT.  Then it continues to drip all day long.  Plus, his eyes are now "bloodshot".  Neither was yesterday and this morning only one was.  But now it's both.  The Doc says it's likely viral.  (From what I read it is more likely a sign of bacterial but either way, I'm being extra careful so I don't get it. 

It's now 8 and I've laid him in bed to watch a Baby Einstein video and hopefully doze off to sleep. (Doubtful on that part since he had a nap today and the never ending stream of people in here but I'm trying anyway.) 

Part of me hopes he's perfectly fine tonight and this is all done. Part of me knows that's not the case and that he'll have the same issues again. 

But I also have this weird fear that he'll be fine tonight, they discharge us tomorrow and then he does it all over again tomorrow night at home and we start again.  So instead of that, I'm hoping that he actually does what he's been doing at home.  Probably won't know until at least 2-4am if the pattern holds from this previous weeks' episodes. 

And now we wait. 

Sep 2, 2011

Mystery to Solve

As you know, Manny was sick with respiratory stuff last week. By last Saturday, it was completely cleared and not even a cough.

Sunday and Monday were normal.

Tuesday he woke up with 103.2 fever and no other symptoms. (Nothing). That resolved by midday.

Wednesday same 103.1 fever, only symptom was some dried blood around g tube site (more than usual but not significant). Fever was gone by 10 am.

Thursday fine … no fever. Only had a strange bowel movement (more loose than normal).

Thursday night … went to be very grumpy. Did a pulse ox and it was great. 96 or so and a 95 heart rate! Turned it off. Woke up a lot to be repositioned (more than normal and saying owie). 2:30 woke up SCREAMING and would not be consoled. Finally fell asleep around 3pm. (Not his normal.) 4:20 woke up screaming again and this time had 104.4 fever. Gave him Tylenol. (He has no covers, sleeps in light clothing, not a hot room, etc.) Turned the pulse ox on as I could tell his heart rate was sky high. 250’s. Even when he was laying still and quiet and fever was coming down, his heart rate was in the 230’s. He was pointing to mid area (heart? Stomach?) and saying “Owie right there”. Then he started retching so I drained his gtube and out came 80-100 ccs of fluids. (Usually there is nothing … if there’s anything it will be stomach acids or saliva. This was not those and was not milk. Have no clue what it was.) He finally was able to settle down about 7:30am (after 3 hours of dealing with him and trying to make him comfortable). But now, while completely asleep his heart rate was still in the 190’s. (No fever, my guess is pain.)

I decided I’d call his doctor when he woke up for an appointment … BUT … When he woke up at 9am, he was fine. Acting totally normal. Symptoms all gone. So instead I sent her a fax including all the details on here.  She agreed with me, this is strange with no clear cut answer/solution. 

I am telling you all this for a few reasons. I want to keep you informed. And also to let you know that if he does that again tonight, we will take him to St. Joe’s.

Such a strange mystery.  Anyone know why he'd have troubles at night but not the day?  The only things I can think of that's different. 1) The room he's in at night is a different room we're in during the day.  Perhaps an allergy in there?  2) He is at a different angle.  During the day we keep him completely upright (like in a wheelchair, high chair, etc). But at night, he can't sleep at that angle so he's at more like 45 degrees (the doc recommends 30 minimum).  And do you guys think it's heart related or stomach?  Do you have any clues about what's going on?  We're all ears.  You can respond on here, on facebook, or privately (  Thanks

Happy labor day weekend!

Beth and Manny

Sep 1, 2011

Jacob's Nephrology visit 9.1.11

I am so many blogs behind that I might have to start writing 3 a day to catch up. 

But today's blog is about Jacob's nephrology exam today.  He goes about every 2-3 months and gets blood and urine tests along with a physical exam.

The results are mixed.  Overall, it's good - sorta.

I won't bore you with medical terminology or details but suffice to say that 0 is the right number for most of the tests and the higher you get the worse it is.  On one element, his numbers have gone down (good news).  On one element, the numbers are steadily creeping up (but they are not at his highest levels that he's had, but we thought we had this under control).  And the rest are holding basically steady.  (Which is not good news.)

How is stable not good news?  Well, they should be declining.  The numbers need to go down.  He needs to go into "remission".  Until then, his kidneys are still at a very high pressure.  Eventually it's going to "blow".

Think of the kidneys like a dam.  There should be no holes.  But this one has many holes.  The pressure of the water (No matter how hard it's trying to hold) will eventually push through and collapse the dam, it will just "burst".  So you'd have to find a way to plug the hole or reduce the pressure.  With every day that passes, it's increased risk of rupturing the dam. 

The analogy isn't perfect but I think you get the idea.  FSGS kids can go from "holding steady" to EMERGENCY in no time.  (Like over night we're told.)  The doc is actually a little surprised that he is still doing so well considering how long these numbers have been so high. 

In summary ... we're thankful he's not declining.  We're still in major need of a complete healing.