Nov 30, 2011

Hint of Hope

Got a call from the Endocrinologist.  Most of the tests he ran came back "Normal".  That's good news, just not very helpful in pointing us in the right direction.  One DNA test is still pending. 

But one test did show something abnormal.  Not sure how helpful it is yet ... I'll be having an appointment next Tuesday with the Pediatric Metabolic Geneticist and she should help shed some light on it. 

So here's what we know.  He for sure has Merosin Deficient Congenital Muscular Dystrophy.  That explains the vast majority of his symptoms.  There are just a few (like the progressive nature of the smooth muscles that are being affected) that cannot be as a result of the Merosin.  So we're hunting for the cause of THAT.  And we think it metabolic and maybe related to mitochondria.   

With that in mind, the test showed a very low amount of carnitine in his blood.  There are two things they measure, Free and Total.  His was about 30% low on both. 

From what I can find so far ... Carnitine is what helps digest fats.  (Here's a link ...  Ironically, that link is produced by the NIH ... which is the group that has been collecting all of Manny's reports, etc. for an evaluation in January. 

If this is at the root of his second problem ... we may be on to something. From what I'm reading, it DOES make a lot of sense.  It can cause GI dysmotility.  It can be triggered by periods of fasting (like when his PICC line cracked and he collapsed quicker than he should have). 

On the other hand, his carnitine level could be just a "red herring".  Like it might be low because he's on TPN. 

The Endocrinologist suggested that they add carnitine to his TPN solution.  They passed the suggestion along to the GI as well as to the Geneticist. 

What does this mean?  It MIGHT mean we are on to something.  It's certainly not a quick fix but even a hint is very hopeful these days.  It's a HOPE that he might be able to eat again!  And THAT is worth fighting for. 

Nov 29, 2011

Released from the Ortho

Monday was Zoe's first day back at school. About 10am, get a phone call from the school nurse.  Zoe was up at the clinic due to the horrible swelling on her hand from the bite.  I was clear across town getting blood and urine tests done for Jacob and Kaley.  She'd been sent to school with Benadryl and cream on her hand.  I had an appointment for her later that day. Not much else we could do for her.  Zoe wasn't wanting to come home anyway.  So they gave her some ice and sent her back to class.

Monday afternoon, took her to the doc and we all feel it's a fire ant bite, just a really, really bad reaction to it.  At age 2, she fell into a fire ant bed and got probably 150-200 ant bites by the time we got them all off.  So this reaction is likely caused by that experience.  Next time?  It could be really, really bad.  Doc gave her prednisone and an antibiotic to go along with the benadryl and hydrocortisome cream.

Tuesday was Zoe's second day back at school.  About an hour into the school day, I get a phone call from the school nurse.  (Hearing a theme here?)  Seems like Zoe was in the classroom and just happened to slip.  Of course, she landed on the hip that had the bone taken from it.  Teacher freaked.  Called the nurse.  Nurse called me.  I assured her that Zoe should be fine, that landing on that hip should be no different than falling on the other hip at this point (she's 4 weeks post surgery).  I wasn't able to talk to Zoe but I assured the nurse that Zoe is fine, I'm sure.  (And besides, I was also across town again at a doctor appointment.)

Once I got her from school, I told her the nurse called me.  She didn't know why.  I asked about if she fell.  And she told me she didn't cry.  It barely hurt.  It was no big deal.  (Phew).  Seems like the teacher was just extra cautious/concerned.  (Which I appreciate.) 

Hoping not to get a call tomorrow from the nurse. 

Thrilled to be the Mama of this strong, spirited little girl. 

Meanwhile, across town, I was with Manny at the doctor appointment.  His Orthopedic doctor is inside the hospital where he and Zoe have been going.  Last week I was cleaning out the toy closet and had tons of big items that he never has been able to use that people hav given him.  (It's truly amazing how many toys he cannot use due to it being to hard or requires standing, etc.)  So I wanted to donate them to the play room at the hospital.  Today was the day I delivered them.  Yay!  The toys are finally going to be played with. 

Then we went to the appointment.  7 months ago, we went to this Orthopedist.  At the time we didn't have an official diagnosis.  He wanted to see us again in 6 months.  So here we were. 

I was very concerned about the contractures on his feet.  Remember all the serial casting he's had to have done?  Remember the Houdini act he does to get out of the casts?  Remember how he's got AFO's (braces) for his feet?  Remember how he couldn't wear them most of September - November due to being in the hospital with IVs in his feet? 

I was worried that he would want to do some more casting for the feet.  But NO!  Yay.  He said as long as I can still getthem in the braces, he was fine with it!  Now if this baby were running around or even standing on those feet, this wouldn't be enough.  But he can't do that at this point so no need to cause him more pain. 

I told the doc the official diagnosis.  Informed him of what it was, what it involved, etc.  Merosin kids often have contractures of the ankles, hips, wrists, elbows, etc.  So he double checked all of those (he has none besides the ankles - which he was born with).  And these kids also are prone to sciolosis.  (He has no signs of it.)  I already knew all of this ... but it's good to have it confirmed by a doc. 

So given his diagnosis, the fact that he's clinically stable with the bones at this point, and has a mother who knows what to watch out for, he doc felt that we didn't need to be seen on a regular basis.  Make an appointment only if we have something new.  Gotta love it!

Menawhile, this doc couldn't stop saying how cute Manny is!  When he walked in, Manny said, "Hi Doc" and it cracked him up.  He has a kid the same age as Manny and couldn't believe how verbal he is.  At the end of the appoitment and as he walked out, Manny yelled, "Bye Doc, Thanks" and I heard the Doc crack up the whole way down the hallway. 

Kaley finds it fascinating how EVERYWHERE we go, Manny brings people happiness.  He says "Hi" to everyone in the elevator (and usually invites them to Chuck E. Cheese or the beach).  He greets people in the grocery store.  People will be walking around in their tunnel-type gaze and he "snaps" them out of it.  Young, old, male, female ... doesn't matter.  He brings joy to people.  There is just something special about this kid and I hear that dozens of times every single day. 

Can't believe I get to be his Mama. 

Nov 27, 2011

In summary

Yes, I've been silent for the past 12 days.  Thanks to all of you who checked in on us to see if we're doing OK.  Yes, we're just crazy busy (even for us). 

In summary ... Zoe is now 1 month post surgery and I would love to post pictures but she is getting very private about her owies and I am going to respect that.  I have pictures that maybe she'll let me post some day in the future but for now, I'm not going to break that.  How is she doing?  Physically fairly well.  The hip scar look amazing and she has no pain.  She is off all restrictions for activity. 

Her mouth?  The stitches are still in.  They shold be out by now but are not.  We are supposed to help work them out by brushing and rubbing but they are not budging.  She is also not allowed to eat in the front part of her mouth ... but that's fine as she has no teeth there anywhere and never does.  She is not able to eat sharp foods (chips,crackers, etc) but that's about it. 

Emotionally?  This has been the hardest part for her by far.  She is struggling with the whole thing and I'm not sure what made this time different.  We're spending a lot of time talking. 

Manny ... Since his broviac - all is going well.  He's getting weekly dressing changes and weekly labs.  So far, so good.  He did get a pretty bad chest cold last week and he still has a lingering cough but we have all the equipment at home that they have at the hospital so we are just nursing him back to health at home. 

Meanwhile, I've talked to some fellow moms of kids with Merosin Deficient Congenital Muscular Dystrophy and even some docs ... they are in agreement, there is more going on with Manny than just the Merosin. So it was recommended we send his stuff to NIH in Bethesda, MD.  For the past 2 weeks, I've been collecting MRIs, reports, genetic testing results, the muscle biopsy slides, etc etc from numerous docs and getting them shipped to Maryland. 

Long story short, they all arrived and the doc looked at them.  His initial reaction is that he does indeed have Merosin (we weren't doubting that).  But he also has some very atypical things going on in conjunction and he would like to see us.  Monday I'll making the schedule of when we can fly out there.  (Probably January.)  Looking forward to that meeting... it will be the first time we've ever seen a doctor who has ever even SEEN another Merosin kid!

Thanksgiving.  We had a wonderful time here.  I always cook for an army because if I have to work that hard on a meal, it better last for several other meals!  We have my parents, sister, nephew and several others who didn't have a place to go.  It was very nice. 

Thursday night, we gathered all our stuff for our yearly camping trip with our church.  Yes, that's right. 

Friday morning we packed up the van and headed out to the campsite.  And before you all freak out, Manny didn't sleep out at camping.  Dan came home with him at night and hung out with us during the day.  But the rest of us were there having hotdogs and making smores, getting filthy, finding strange animals, play games, etc..  My kids LOVE being outdoors and could be out 24/7.  

We always go to the same group campsite and for the first time, we found a snake.  One person thought it was poisonous.  (I never saw it, thought I'd have nightmares if I did.)  But they gathered it up and drove it about a mile away and released him there.  We heard owls arguing.  We laughed.  We got filthy.   The weather was 80 in the day and 55-60 at night.  Perfect if you ask me. 

A couple of things went wrong ... Saturday night, Zoe said her finger hurt.  It was a tiny bit red like she had a bite of some kind but she didn't remember being bit.  Now here it is 24 hours later and her finger is quite swollen and painful.  I'll have to put a call into the Doc tomorrow if it doesn't start to look better. 

Oh, just thought of one more thing ... you know those glow in the dark necklaces?  One broke and sprayed right into Lukey's eye!  It's toxic I'm sure so all the ladies jumped up and immediately started flushing it out.  Didn't take too long before he was good as new.  (Who knew!)  Funny, he said, "At least it was a blue one because I have blue eyes so it matches." 

And my tooth completely broke.  The one right behind my eye tooth so it's completely visible.  It's not a partial break or a small thing ... the whole thing is GONE.  Yes, it was a bad tooth and I should have taken care of it a long time ago but didn't have that kind of money.  They were talking THOUSANDS of dollas (5 or so) just to fix this one tooth so I chanced it.  Now I have this huge gaping hole and no dentist.  Guess I'll be working on that tomorrow.  And in the meantime, not talking or smiling much.  (Yeah, right.)  And still don't have a clue how I'll pay for it but don't really think I should go without a tooth.  One in the back? One on the bottom?  Who cares! But this is right in the front.  (sigh)

The next couple of weeks?  Tons and tons of doctor appointments.  I'll try to do better about keeping everyone up to date on the happenings.  But I can't promise.  I get to living life ... and I feel like all I'm doing is spinning plates, just trying not to let one fall. 

Hope everyone had a blessed Thanksgiving!  And may you have something to be thankful for, every day of the year. 

Nov 15, 2011

From PICC to Broviac Photo Version

Here's a photographic view of our recent hospital stay.  He was fine, crashed, got a central line and is now home. 

By the time we got to the ER, Manny was looking like this.  I couldn't wake him up.  He was completely passed out.  VERY scary. 
This is him after he is stable and in the playroom.  His first time out of the room in all of our hospital stays as he is ALWAYS on precautions.  He was thrilled to be out of the room. 
 Taken a few minutes before his broviac surgery.  I wasn't exactly sure where they were going to place it but I wanted the picture of him "before" any incisions. 
 That last moment before he was wheeled away to surgery.  He is a charmer and had ALL the staff ooooing and aaaaahing.  Do you notice the lady in green?  If you look closely, she is holding his hand!  I told her this was a secret to keeping him calm and she got the lucky job of being the hand holder she said!  He was smiling and calm. (No drugs, they dont like to give him anything as it can lower his respiratory rate.)
 A few minutes after surgery.  Here's what your looking at.  His left arm has he PICC line in it. The low tummy of course is his GJ feeding tube.  The center chest is the broviac. The two patches at the top are the two attempts the surgeon did prior to getting a viable vein.  (Personally, I am happy that the final broviac is in the center and not at the neck.  Just on looks alone!)
 Closer view of the broviac.  The circle section is called a biopatch.  The tube has the clamp on it and then there are extentions at the bottom.  This version's opening is one for needles.  We now have a extender that has a screw top that affixes to the TPN and syringes.  It's all held on by tegaderm since he is allergic to all tapes.  (Hoping he doesn't develop a tegaderm allergy.)
 About 4 hours post surgery, they invited us down to a "Bead party".  He didn't want to make a necklace.  He just wanted to play with the beads.  Yes, we supervise him with not allowing him to put them in his mouth.  But the truth is ... as a child who has been "NPO" (Nothing per oral ... or nothing by mouth) since 8 months old, he is seemingly unaware of his mouth and has yet to attempt to put things in his mouth. 
Manny on his diaper changing station at home.  I'd just changed out the bandages for his PICC line.  For those wondering, they took out his PICC line and bandaged it up. We remove the bandage 24 hours later.  This is important to be extra careful with bleeding issues since he's on Heparin (a blood thinner) so that the lines in his veins don't develop clots.  But of course this makes him more prone to bleeding. 

The dressings on his neck were supposed to be changed at the 24 hour mark too.  BUT, when I went to change it, they were still oozing blood.  I called the surgeon's office and talked to one of the Physician Assistants who assisted in the surgery.  I needed to talk to her and not just anyone.  Why?  She was IN on the surgery and knew all the extra incisions they made and how it wasn't a typical surgery.  She told me to give it a few more days since he was still bleeding.  Normally it would be fine to remove it now (and recommended) but since he was still oozing blood, it obviously needed more time.  There was likely a little bit of trauma to those veins in his attempt to use them for the broviac. 

The broviac's bandages get changed once a week by a home health nurse at first.  Eventually, they will train me how to do it.  (For now I'm happy for HER to do it!)  They draw labs at the same time. 
 and put him in his favorite pjs ... his monkey shirt.  Every time he sees it, he immediately says, "oooo-ooooo, aaaaa-aaaaaa".  Makes me smile. 

So thrilled to be home.  And thrilled to have a way to feed him again!

Nov 13, 2011

Broviac 101

TPN is a great thing ... Manny basically slept through the night.  It was amazingly wonderful. 

The day was uneventful (like I like it). We discovered that surgery is scheduled for 9:45 Monday morning.  If all goes well (like we expect it to), we MIGHT be able to go home Monday evening.  Thanks for the prayers.   

Answering Questions (AKA Broviac 101):
I had a couple of people ask why we got the PICC when we need a Central line and also why do we still need the central line if we now have a PICC.  Great questions.  Here's my attempt to answer.

All of his doctors (here in Tampa and Gainesville) decided he needs a central line.  And we are going to get that on Monday - surgery time set and everything. BUT before then, he needs to be stable. They tried with IV but it wasn't happening and they realized he'd need to be on TPN likely to get stable. PLUS, he is
needing a LOT of blood tests over time and he has not a lot of veins. So the PICC makes sense in the short run just for all of this.

In addition, our experience has been that the PICC lines are easier to put in him than the regular IVs and less traumatic.  We are able to do it with light meds (not full sedation) and they have the ultrasound so they can truly see the veins.  They are also going into a bigger section of the vein (up high on the arm) and not on the wrist or hand like is typical of IV.  The IV nurse explained that due to his very very low tone, he has not really developed many veins that are usable.  And he has many "collateral" ones ... meaning his body is trying to make some but they are like dead ends with no blood. 

SO the real question is ... do we still need the broviac. Yes. We just don't have to have it by this Monday. The new PICC buys us some time. So there is no real reason that we HAVE to do the broviac now ... but since that was the plan, we are going to do that. In addition, if something were to happen to the broviac, there are apparently a lot better options of repair, etc. AND they told me that if something happened to the broviac, they can go back to a PICC temporarily IF the vein has been PICC free for a certain period of time. So they want to spare the precious few veins he does have.

Right now, he has one good vein and 1 sorta OK vein. They used the good vein the first time and they are having to use the sorta good vein right now. They doubt this vein will hold up more than a couple of weeks. And the repair of THAT problem is HUGE (if it "blows" the vein) and we're in another emergent situation. And to define good vein ... they want at least 2 millimeters, bigger is better.  The OK vein is less than a millimeter.  There are no other veins in either arm that are even that big. 

Finally, this OK vein is sitting "on top" (PICC nurses word for it) of the artery.  She had a hard time making sure this went into the vein and not the artery.  So any kind of injury or infection to this vein would be very, very bad.  She insisted, "Do NOT go home with this PICC."  She knows just how precarious it is because it's clear on ultrasound.

So with all that ... he needs the broviac. Like I said, not necessarily this Monday but soon and why not do it while we're already here, he's finally stable and it's scheduled. 

I've also had questions about PICC versus Central line/Broviac again.  I'll do my best to explain (while I muse on while I went the PhD route and not the MD route):
PICC line goes into the arm.  It's like an IV but with IV, the fluids/meds are put right there in the tiny veins on say the hands, wrist, foot, etc. and it circulates through the body. With the PICC, it's put in the upper (bigger) section of vein and then this long catheter is threaded down and near the heart where it's circulated. Unlike an IV, you can also get blood out of this vein.  PICCs are fairly easy to put in.  The problem with it: it's very hard on that vein.  So for short term use, this is perfect.  
But for long term use (like Manny's), it's really not a good solution.  So they go to what's called a Central Line.  There are two main types of that.  A port ... that is completely under the skin.  To access it, there has to be a needle inserted into the skin and into the port.  (This is not a great option for him since he has daily access/usage of this.  A port is more often used for things like chemo where it's only periodically accessed.) 
The other main option is a Broviac.  It looks a lot like the PICC but it's in the chest.  The lumen is hanging out and it's "tunneled"  (I'll give you a picture of all this when we get it inserted and all will become clear.)  We have opted for the Broviac.  Even though it's hanging out of the body, we've gotten used to that with the g tube, then two lines with the GJ and then the one with the PICC ... since we've had him, he's had SOMETHING hanging out of his body.  (Yes, it's freaky looking!) 
The reason to do the Central line is this is a more stable, longer option.  It still has really high infection rates and clot issues, etc.  But seriously, if you've gotten to the point where this sounds like a good idea, that tells you a lot. 

Nov 12, 2011

My Kaley

12 years ago on 11/11/99, a tiny little girl was born.  Her birthmother knew (due to her circumstances) that she was not the best choice of a parent for her.  This little girl was born in a home in Tampa and then the paramedics were called.  The birthmother said she wanted her placed for adoption.  That was the last time they saw each other.  She didn't even give her a name.  So for the next two years, her legal name was "Baby Girl King." 

This tiny girl was taken to St. Joseph's hospital.  (If that name sounds familiar, it's where I always go with Manny and Zoe has her cleft surgeries.)  She was tiny but likely not premature.  She was drug addicted but otherwise healthy.  She spent 9 days in the hospital before going to a foster home.  After 2 days in that foster home, the system discovered she had a half sibling in the system.  They moved her so they could be together. 

For the next 3 months, she was there ... it was not a good foster home.  (I'll leave it at that.) 

Meanwhile, during all that time, her adoptive family was working towards their certification, classes, home study, background screenings, etc.  This family found a wonderful adoption agency ON THE DAY she was born (but not knowing it at the time) and decided that was their agency. 

Her brother was legally freed for adoption and matched with this family.  They met him, fell in love and that was that.  At the end of the meeting, the social worker said, "You know it's both or none, right?"  And I was like, "Both what?"  And that's the first time we heard about the baby sister. 

Crazy part is how we met. 

I was with hubby and we were about to ride the elevator up to the meeting.  We knew the foster mother was coming and it was not customary (or even approved of) for the foster kids to come to this initial meeting but she had no baby sitter.  So she brought the son.  As we waited for the elevator, a white mother walked up with a double stroller ... an 18 month old black boy and a baby in the back.  We knew instantly this was the little boy we were there to discuss.  (Meanwhile, remember?  We had no clue there was a sister, much less that she was in the back of the stroller.) 

So the whole meeting, I was playing with this child who turned out to be our Jacob.  I completely ignored this other baby in the back of the stroller.  Afterall, why play with some random baby versus bonding with my soon to be son?

So at the moment they said, "Both or none" and I said, "Both what?"  They picked her up and said, "This is his 2 month old baby sister."  ::pause::  WHAT? 

By ths time, the meeting was over and I'd blow the chance to see her, hold her, touch her.  And now we had some thinking to do.  We went in knowing we were going to get an 18 month old black son with some special needs and now they throw in a bonus newborn with special needs too???  Were we ready for this??? 

I instantly knew YES.  This were our children. 

The little girl was not legally freed for adoption like Jacob was so we had to do 3 extra weeks of paperwork to be licensed.  So we did.  During those 3 weeks,  I went to visit the kids almost daily.  Doing baths, taking walks, feeds, etc. 

March 22, our daughter slept in her foster home for the last time.  From that point forward, she was our little girl (not legally at first, but that came).  We changed her name ... legal name was "Baby Girl" but they called her Vanessa.  (Which meas "butterfly" ... and she is tiny, delicate and beautiful like a butterfly).  But we decided to call her Kaley ... "Black Princess with powerful energy" was the meaning I'd read. 

Over the past 12 years, this little girl has turned out to be an amazing gift.  I am so thankful for the "bonus baby" God decided to give to us. 

You all know a bit about Kaley and her amazing heart... like how she stayed with us the whole 3 weeks in Gainesville.   You have read about her giftedness with Manny.  I have bragged before about her amazing creative and artistic abilities. 

This is one special, sweet, amazing gift to me and to the world.  I don't know exactly who she is going to be when she grows up.  She has the ability to be "the boss" and get the job done (don't let her small stature fool you into thinking she is weak).  But what ever she decides to do in life, she will do great.  And I know one other thing ... she will have a proud Mama standing beside her, cheering her on. 

One special thing she said to me a long time ago when we had a miscarriage (Kaley was old enough to understand) ... she was very sad for "the baby that died".  she said that she will have a baby in her tummy some day and she wants me there when that baby comes out since I didn't get to have that.  I was in shock as that was something we'd never talked about but she "knew" and she understood. 

One funny thing she said to me when she was about 4:  we were talking about what she wanted to be when she grew up.  Doctor? Vet?  Teacher?  and she said, "I want to do nothing.  Just like you."  LOL  (She has since recanted that statement ... but it certainly was funny at the time.)

My message to Kaley today and every day:  My dearest Kaley.  I don't know how I got to be the luckiest mama in the world to have you be my daughter, but I'm very thankful that I was chosen.  You are a bright spot in my day.  You lighten my load daily.  You make me proud to call you daughter.  I will be there for you.  I will hold your hand and kiss away the hurts of life the best way a Mama can.  I will cheer for you and help you be the best "you" possible.  You are one of the biggest gifts of my life.  Daily, I'm amazed at you.  May God continue to shine His favor on your life.  You're a blessing my sweet daughter. 

All my love,

~ Mom

Nov 11, 2011

Stable... not the horses kind

Let's start with some stats:

Friday night 11pm- blood sugar 154! (very good)
Saturday morning 5am - blood sugar 136!  (Very, very good)

For those who are wondering, here's his height and weight: 

27.7 pounds, 12.595k
88 cm 34.8 inches

As he went to bed last night, I couldn't tell how he was because ofthe effects of the versed.  (That drug makes him look just like he blood sugar issue did.)  As the night continued, I could tell the versed was wearing off and he was grumpy!  He cried ... from 1:40-2:45 he pitched a huge fit ... I'm sure it was a blood sugar or versed issue.  He did it again at 4am for 30 minutes. 

When it was time to wake up this morning, he was (basically) himself!  I'll have to get the official results of the labs to call him officially Stable, but *I* (the non-medical doctor that I am) says he is.

9:45 took us down for a bowel, stomach xray (he was very tender in that area yesterday).

10:00 Surgery consult from one of the Docs (yesterday it was a PA) and he suggested a pyleloroplasty.  It took great restraint to listen to what he had to say as this is NOT NOT the right answer.  This is the opposite of a good idea, in fact.  But I have to realize that he is not as familiar with Manny's case as I am so he might need a little time to catch up.  (Not meaning that in a snotty way... well, maybe just a tad.)  But there is noooo way I'd sign for that surgical procedure. 

For those not familiar.  They would open up his abdomen, "clean out" (as he put it) the lesions in the stomach and first part of the bowel and make a larger hole in the bottom of the stomach.  The purpose of this procedure is when you have gastroparesis (delayed emptyping of the stomach).  And yes, he DOES have that.  So what's the problem?

If you're a regular follower of Manny, you probably already know the answer.  Food backs up from the bowel and back into the stomach.  So if you feed through the stomach, (no matter how large the hole), won't it eventually end up back in the bowel??!!??  How the heck is this a good idea? 

Thankfully, this is not his main surgeon, just the on call doc.  And thankfully I only need a broviac at this point in time.  IF the whole team ever decided this was a good idea and they could convince me (doubt it) I MIGHT consider it.  (doubtful again). 

BUT ... there was something good that came out of this conversation ... Manny is on the schedule for a Monday Broviac. 

10:30 The Endocrinologist came in and we talked for a long time.  He didn't know of anything off the top of his head that would cause his symptoms.  So it's going to be something obscure. 

I have no clue the ODDS of having TWO strange, rare disorders but it seems that's what's going on.  This doc was also not shocked at all about him crashing so quickly with TPN withdrawls.  We need to expect he will in fact from now on.  Also, he discussed the dextrose issue.  Most people are on about 45-50% and he is on 70%.  Not sure why it's so high. 

This doc has ordered quite a few very specialized tests and we won't get results for about 2 weeks probably.  He'll also send the results to the Geneticist since she specializes in Metabolic disorders.  (Why wasn't she already tracking this down?  Because when he got the diagnosis, everyone stopped looking for anything else.) 

11:15 Cartaya reports that his lab are all perfect again!  I already knew blood sugar but sodium and bicarbs are also right on.  Oh, and the WBC was 31,00 is now 9,000 (perfect).  So I was right ... he does NOT have an infection brewing.  He is NOT sick.  His body went wacko from the stress.  Which makes me realize, I MUST have a back up plan for if/when his TPN line is unusable for whatever reason.  He cannot wait even one night. 

The other lesson is that people need to believe me.  Seriously. Yesterday, the ER doc was scaring me suggesting all sorts of bad things (due to the extreme lethargy and the WBC of 31,000 and not responding to sugar).  He suggested it was sepsis.  (Always a high possibility with the PICC line and with TPN.)  But I knew he wasn't acting sick, no signs of infection, NO symptoms until the TPN died.)  He was also getting ready to do a spinal tap.  WTH??  I was not on board with that either as I knew it was sugar related. But I had no proof at that time. 

I have that proof now.  His WBC is back in range.  PROOF it's not sepsis, and proof he didn't need a spinal tap.  (phew)

I mentioned the surgeon's idea about the pyeloroplasty and he was just as baffled by that suggestion as I was.  (again, phew)

12:30 his blood sugar is 115 (PERFECTION and his norm).  So he is officially stable.  (Not just by Mama terms, but by the doc's tests.) 

Rest of the day ... uneventful. 

6:30pm, Dan and the kids arrive with Tacos.  Hung out.  Kaley decided to stay with me (even though she doesn't have a change of clothes).  Manny is still very, very grumpy periodically.  I know he's just hungry waiting for his TPN to arrive.  He cried hard when they all left. 

They weighed him again tonight and he is now 12.4 Kg which is about 27.3 pounds so he's down a tiny bit from yesterday. And about a pound down from last week.  His sugar was 109.  (I was told anything from about 80 to 130 for him is target, but close to 100 is best.) 

About 9pm he snapped out of it.  A few minutes later, the TPN arrived and we're very hopeful that this will bring back "Happy Manny". 

10pm, he asked to go to bed.  He says, "Blanket and kiss" ... his way of asking to be tucked in! (Sweet, huh?) 

And because Kaley is here tonight, I was able to take a shower!  And get some hot tea. If you haven't read the blog about her, please do ... it will melt your heart. 

Good night from a tired but blessed Mama. 

Nov 10, 2011

You gotta be kidding me

Darnit! I *KNEW* our lives were getting too quiet and I hadn't been in the hospital enough this month yet. Tonight, Manny's PICC line cracked! What does that mean? The past 3 hours I spent on the phone with the home health nurse, then the GIs and we came up with a plan ...
 What happened?  How did it crack??  There is NOTHING that we do that could crack it. We don't even TOUCH it. (Mostly because the more you touch it, the more likely the infection.) There is this tube like thing that come out of the skin. At the end of it is this "hub". Those parts are all permanent and part of the PICC line. After that, there's an extender. This part is changed about 1 time a week by the home health nurse. We put the "milk" into the extender. So literally, we NEVER touch the hub. THAT is the part that has a huge crack right in it.

I discovered it because tonight, I hooked up the milk like normal and then an hour later I saw a puddle under his high chair. I then ran the line to find out where the hole was and discovered it was the Hub!

Called the home health nurse and they confirmed what I knew ... nothing can be done except a new line. and of course friday is a holiday and then the weekend.

Thursday night he went to bed normally and all was fine.  The night was uneventful until early morning when he got aggitated and was alternating between grumpy and dead asleep.  When it was time to wake up to take Zoe to her post op appointment, he was very difficult to wake up.  He would talk for a few minutes then right back out cold.  (Not like him.) 

Went to Zoe's appointment and on the way I called the pediatrician. She said to take him to the ER and she called ahead.  I went ahead with the appointment for Zoe since we were there.  (More about that later but quick version is all is well.)  The office staff noticed Manny was not acting right and I told them what happened.  They told the doc and she took us right in.  While in with Zoe, the surgeon got an idea to call the IV team (that's who the ER would have called) and got Mani on the phone.  (I've mentioned her before several times.)  And she said to bring him to this certain spot in the hospital. 

This hospital is across the street from where we were.  Mani looked at it and knew immediately it couldn't be repaired.  The site looked great but she knew that due to the large crack, infection can set in from the outside AND a clot can start on the inside since we can't flush it... so she took it out.  (He didn't even wince!)  We decided I should call Cartaya (also mentioned him before) and get the ball rolling to get the central line/broviac placed. 

Cartaya felt we were probably good enough to go home on pedialyte and be admitted on sunday night to get the thing placed on Monday.  (He told me the warning signs to come back sooner.) 

And I headed to the van, relieved. BUT ... this gut feeling said not to leave. So we ate a snack and chilled for a few minutes.  While they did that, Manny was acting VERY weird.  He said, "I not OK".  I was praying for wisdom.  My "gut" said to stay.  But I also wanted to go home.  So I called Dan and he said to get it checked out, to go with my gut. 

Went into the ER.  The doc immediately noticed he was "passed out" and his skin was FREEZING so he decided to do an accucheck ... and his blood sugar was 36!!!  Seriously, dangerously low.  They gave him 350ccs of dextrose solution and in one hour his blood sugar was 251.  She didn't believe it so she rechecked it and it was 261.  (Dangerously high.)

Thankfully I thought to bring in the TPN bag to show the solution he is currently on.  They were in shock that he was at 70% dextrose ... apparently, that's excessively high.  But while on it, his sugars are stable and normal. 

So the question is ... why does he have to have that much dextrose to keep his sugars stable?  AND what can cause him to crash that quickly (when they thought he should be fine for 4 days off of it and on pedialyte alone.   Cartaya is thinking some kind of metabolic issue.  (Interestingly enough, Shands thought the same thing about why he couldn't handle food but never followed with that train of thought.) 

So tomorrow they are going to take some more labs and I will be seeing an endocrinologist for the first time.  The current thought is that Manny must have not just one but TWO rare conditions.  (My thought for a while now since all his symptoms can't be explained by the Merosin alone.) 

We got up to  room and they did all the regular checks ... and now (after being off the dextrose for 4 hours), his blood sugar should have stabalized but it was 296!  Going the wrong way.  Call into the doctor.  They put him back on dextrose and pedialyte.  They'll check him again at 11 pm. 

Meanwhile, Surgery came in for a consult and he remembered us.  He did our muscle biopsy in April which gave us the diagnosis of Merosin.  He said that clearly manny needs the central line ASAP but they can't put that in until he is stable.  To get stable, he will need to be back on TPN.  To get that, we needed another PICC line.  (sigh) 

The same Mani ... from earlier in the day who removed his PICC is the same one who is called back to do it.  We all laughed.  She said they NEVER do PICC lines on kids this age without sedation nor with a parent in the room.  But based on my word that I could keep him still and calm and that he would be fine, she tried it.  She was not confident at all that this would work and we all decided that we would abort at any time that we needed. 

We proceeded and long story short, it was a piece of cake!  This was easier and faster than a regular IV line.  He was given some light versed to take the edge off and he was FINE!  She was in shock at how easy and fast (about 1 hour faster than expected).  I was so grateful, she was so relieved.  Now Manny can get his labs done in the morning PAIN FREE.  And we can start PTN back up tomorrow once they get that solution made (it takes time).  (insert breath sound here) 

There are soooo many details I'm leaving out because this is already so long.  If the details are still fresh and relevant tomorrow, I'll write it then.  If not, it wasn't meant to be. 

Also, don't be shocked to see me writing more blogs soon as I still have to send the one I wrote about Zoe's post op to surgery and I will write one about Kaley (who turned 11 today!) and a few other random thoughts. 

Summary:  Manny's not a typical kid (in either his highs or his lows).  He's still not out of the woods right now and they are watching him very closely.  He's not considered stable yet ... but we think we have a solid plan in place to get him there. 

start him on pedalyte via jtube tonight. First thing tomorrow, we see if (on a holiday) there is a team that can put in a central line. If not, we have to wait until Monday likely. And that means we'd be admitted likely tomorrow. The doc... said they only let us stay home tonight because of the kind of Mom I am (awww). Ironically, we were supposed to see the Doc on Monday to discuss the need for a central line and how soon to do it. Guess Manny just had a different timeline.

Nov 3, 2011

Dear Stranger

Dear Stranger,

You made a comment. 
What you said makes no sense. 

"You have your hands full." 
 I'd like to make a suggestion... This is a phrase that needs to be eliminated from your vocabulary, never to be used again.

Only exception to this rule ... if a person's hands are ACTUALLY full.  Then you are allowed to say it.  Then again, I am not sure why you'd want to say it then either.  Why would you want to point out such an obvious thing?  It's like saying, "You're a girl" or "You have on a dress." 

So maybe the real only exception to the rule should be that it needs to be a phrase that is completed such as,  "You have your hands full ... let me help you with that."   

But I find the phrase is never really used in that context. 

So what ARE you trying to say when you say that? 

I was at an appointment today.  The announcement came on, "Joan Smith to window 2".  Joan Smith immediately got up and started walking to window 2.  I heard the worker say (in incredulous tones), "You have your hands full."  Why?  Because the woman had a 3 year old, a 2 year old and was very pregnant. 

So exactly, what was the point of saying that?  *I* certainly don't know. 

I hear it all the time directed at me.  And I never know what they mean or why they say it or how I'm supposed to respond. 

To me it means:  You are in over your head.  You have too many children.  You look harried and overworked.  You look horrible.  You don't know what you're doing. Or perhaps it means You are doing something that I could never do.  I would never have that many kids. 

I can see people saying it if my kids were running around like wild children.  Or if they were loud and out of control.  Or if I was looking harried and overworked.  But no, even sitting nicely, everyone smiling, playing nicely together... they feel the need to say this inane phrase. 

I cringe when I hear it.  It's quite insulting. 

I'm sure you just meant it as an off-handed comment that means nothing to you and you just tossed out this phrase without thinking (which is my point).  What you don't understand is that I hear that phrase about 20 times a day.  And I don't like it. 

Since I don't know what you're trying to say, I find I have no rebuttal that works.  I am left to just sit there and give a half smile/half grimmace.  ::awkward pause:: 

To me, it's no different than me pointing out, "You sure have your mouth full" when you take a bite of food. 

I hereby request that you and everyone (all 7 billion people in the world) refrain from using that phrase. 


P.S.  Next phrase I will tackle?  "Are they all yours?"