Mixed bag of emotions

 Monday night … Manny’s heart rate was erratic.  Normally he sleeps at about 90 but it was like 160’s.  This usually only happens when he has a fever (he didn’t), pain (he said he didn’t), stressed (he was sleeping) or electrolytes off (they weren’t).  So it’s a confusing picture.  But when does Manny do anything like he is “supposed” to?

The morning started very early and abruptly with a breathing treatment.  The Respiratory Therapist put on his albuterol/pulmicort mask and left the room.  I was still laying in bed trying to wake up and he was in his bed basically asleep when the heart rate monitor went off the chart.  He was choking.  I jumped up, pulled off his mask and saw his throat was completely full of liquid.  I grabbed the suction machine and started suctioning him.  It was scary and it all happened so fast!  So glad I knew what to do and was paying attention. 

Soon after that he went back to sleep and his heart rate went to 215! He was basically asleep.  The reading was real as I felt his pulse.  It all lasted about 10 minutes.  (And no, it wasn’t from the albuterol as he gets it 2 times a day every day of his life and it never affects his heart rate.) 

Through the rest of the day … his heart rate stayed high.  He’s usually around 110 but it was like 160, then an hour later 150, etc.  130s for the rest of the day. 

And more strange symptoms … he’s lethargic.  All day he’s wanted to stay in bed.  NOT like him as he always wants to get right up and in his wheelchair. 

Next, he’s weak.  Now I know you might think that a crazy thing to say but I can tell the difference.  He can’t sit today.  He had a hard time holding his head up. 

Next, he took a 2 hour nap! That never happens.  And no, he wasn’t awakened all night long so it’s not that. 

Next, he started scratching his wrists and his tummy.  Rash.  Raised, red itchy rash.  You gotta be kidding me. 

Finally, his labs from the afternoon came back … mostly pretty normal.  But his segs are high which usually points to something viral.  So that’s probably the cause of all the things I just mentioned.  I just hope it’s a “nothing” kind of virus. 

Unless anything changes, we should be going home on Wednesday. 

Now regarding the GI.  I talked to our Hospitalist and she says she really likes this particular doctor.  She will be giving her a call to see if she will take us and if so, how fast.  The good news about that is that she practices at our current hospital so we don’t have to lose all of our specialists!  That’s huge news!  It’s not a done deal but the hospitalist says she’d be shocked if this GI won’t take us.  She says she is the best GI she knows. 

…

Tuesday night, pretty uneventful other than a wide awake baby that did NOT want to go to sleep!  Heart rate still a bit high but nothing like the night before. 

7:15am, The head of the new GI group came in.  That makes it official… we have a new GI group! We will go home today with a script from the current GI to write a week’s worth of TPN. Then within the next week, I will see the new GI and they will take over the script writing and lab ordering, etc. 

I have a feeling they have no clue just how complex of a kid they just inherited is … but nevertheless, they got us.  :) And the other good part is that the Nephrologist will now have to follow him because of the new diagnosis.  So his labs will be reviewed! :)

Manny has hit all his discharge criteria and I now feel he’s stable enough to go home.  He’s not well.  He has some goofy symptoms but we can manage these from home. 

Also, Thursday morning, I have the first appointment with the ENT.  Hopefully she can provide some insight about his relentless post nasal drip.  The pedi had wanted to put him on Zyrtec but one of the side effects can be urinary retention.  And that would take us right back where we started!

I already packed, even though this hospitalist hadn’t come by yet.  But I know these things.  I’m such a professional hospital goer that it’s absurd. 

One unresolved issue is … well, me.  People have to realize, that even though I’ve done this hospital thing 100 times (not really, it just feels like it), and that in the past 2 ½ years I’ve been in the hospital almost as much as out … it still never gets easy.  In fact, he’s gotten so much more complicated that my learning curve is huge.  And if I miss something, it could cost his life.  That’s a ridiculous responsibility to have on a mom’s shoulders.  But I truly don’t see how to relinquish this responsibility.  Who would take it up?  Noone.  So I carry on. 

But it’s never easy in the hospital.  I’m physically tired from having no sleep.  No privacy.  No downtime.  I’m emotionally strung out because if he’s sick enough to be here, he’s SICK. So I worry.  And then to be on guard from medical mistakes constantly, it’s too much.  So under these conditions, I’m also supposed to have the emotional fortitude to stand up for him and confront the things that are going wrong.  I’d have a had time with that when I’m feeling strong.  I feel I’m “numb” almost the whole time I’m here.  It’s how I’ve survived all these admissions. 

And when some error happens, I’m supposed to remain calm. Use my big girl words. Confront with kindness. Stick to my guns. And be his voice and his advocate.  All of which sounds completely plausible when I’m under normal amounts of my lifestress.  But hospital stress is different and it circumvents all these skills that I possess when I’m home. 

And now that numb is creeping into my home life.   I’m exhausted.  Physically.  Mentally.  Emotionally.  I’m about to hit bottom.  2 weeks ago, I had a mini-breakdown in fact.  It only lasted a couple of hours but I felt like it was a panic attack.  I couldn’t breathe right.  Nothing had triggered it, just EVERYTHING.  I decided everything could wait.  I got the kids out some leftovers to eat. And I sat in my lazyboy chair and just STOPPED.  They knew something was wrong with me because I never do that.  I told them I was “sick” and they left me alone.  Dan was gone that night but I was able to muster enough emotional strength to get them to bed, nothing fancy, just got the job done.  And I went to my room, turned on mindless TV, drank a Dr. Pepper, ate popcorn and did NOTHING.  Went to bed early.  By morning I could breathe again. 

But it was a warning sign.  THIS CAN’T continue at this pace.  I just don’t have a clear solution yet!

And yet … I know that I don’t talk about this much because … I am his mother and it’s my job to do this so I suck it up.  I’m wound so tight right now that there’s nothing left to suck up!  

And to be quite frank, I feel like if I complain or say something about this, then it means I’m not able to fight for him anymore.  And I will NEVER stop fighting for him.  I have told him in no uncertain terms … that as long as he is fighting to stay here with us, I WILL FIGHT FOR HIM!  Bottom line. 

 

...
Wednesday 1pm:
I know he's met discharge criteria to leave today.  And then ... about 20 minutes ago, he got a fever.  100.5.   We haven't seen the doctor yet and I don't know if his discharge criteria is under 100.4 or under 101.  So we're in that gray area. 

1:30pm ... confirmed:  Staying.  (sigh)

Head is spinning

8am Monday … Joe (The PA for the surgeon) came by.  He is blunt and direct and I like that.  I don’t need things sugar coated!

So for a history, Joe is the PA for Dr. Martinez.  Dr. M has done all of Manny’s surgeries up to this point since we adopted him.  He was the one who took out the sick/dead/ruptured gallbladder and was almost to the point of tears when he told me of how sick it was and how stark that was in comparison to Manny’s affect/behavior.  He did his two central lines.  The one that lasted only 3 weeks and 2 days.  The one that’s lasted a year.  He’s the one who told me Manny is out of veins in his chest. 

Joe has been there through all this with us.  So when he comes in and says we will have to have this line replaced, he knows this is a BIG deal.  I asked him to be creative and think out of the box and see if there were ANY ideas of trying to save this line.  He said there were a few things we still needed to try, things they wouldn’t normally try, but they go to heroic measures to save his lines! And I appreciate it. 

Among the ideas: Possible one more TPA attempt.  Maybe a dye study.  May try Interventional Radiology to see if they can do something to unclog the line.  There’s a very slim chance one of these things would help … but I’ll take any little bit of hope.  I told Joe, “I believe in miracles.” 

IV team will be by sometime after 9am.  They will troubleshoot with me as well. 

…

The day has FLOWN so it’s 7:30pm as I finally get around to writing the update of the day.  Trying to recreate it all from memory. 

9:20 IV team came by.  The other day, .5 of TPA went in but they were only able to get .3 back.  So that left .2 in the line since late Saturday night.  Wendy went to pull it out and with it came an air bubble.  That’s HUGE news.  She pulled some more and we felt like something was budging.  She took off all the connectors and looked directly into the Hub.  There was a block of “gunk” in it.  We could see it just sitting there.  That was NOT there Saturday! So it worked its way there.  That’s huge. 

Over the next 30 minutes or so, she milked it and even dug some of the gunk out.  Long story short, it worked!  The line opened up!  We were able to get blood out and fluids in.  THAT’S HUGE HUGE HUGE.  He gets to keep his line! It was still a bit sluggish so they did one more line of TPA.  And now it’s basically back to new.  You all need to start screaming shouts of praise! 

Then the job of cancelling all the consults began!  Happily. 

Manny got his IV iron. 

Nephrologist came in … I like her!  We discussed acute and chronic managing of his labs.  We are on the same page.  (Gotta love that!)  She felt like if his labs are stable, we can go home tomorrow (Tuesday). 

One of the things that I started researching was the CAUSE of the new diagnosis of RTA.  And I couldn’t really see how any of them fit Manny.  So I thought about it in a new way … wonder if RTA is related to mitochondrial disorders?  So I googled it … yup!  I found the most interesting article that shows most of his deficiencies might be mito related, even things I thought were unrelated. 

Paula from the MDA came in and they’re going to run a mitochondrial test.  FINALLY might have answers eventually here.   Why does it matter?  Because some are treatable.  And if he could even gain some ability to use his gut, we could lessen the risks of TPN.  So it’s worth trying. 

While Paula was here, our current GI came in and said she felt like he needed to be stable for a few days with his labs before she felt comfortable discharging him.  So we’ll have to see which specialist Doc sides with.  I’m good either way as long as he’s safe. 

She also said that as of Friday (March 1) she is no longer able to take any patients with his type of insurance.  So literally, as of Friday, I have no GI.  Now all the rest of my kids have no GI , so clearly you can live without one. That is, unless you’re TPN dependent.  We get bags of TPN made up a week at a time. So we have a max of one week to get a new one.  But that’s not really accurate since the new one will have to meet us, assess him, establish him as a patient and start writing TPN orders. 

Trying not to panic but the truth is … we are running out of options!

The rest of the day was me researching, emailing, calling doctors. 

My head hurts. 

I know that God has this.  I know He will provide like He always does.  Of that I am completely confident.  In the meantime, I continue to walk it out. 

A new diagnosis ... RTA

Noon Sunday …

Had some company today (always a nice surprise) and they brought chips and salsa!  Gotta love good friends.  :)

Hematology came by … we are staying the course of giving iron IV and getting his iron stores up.  I LOVE that!  Why? Because doctors have been happy thus far with getting his Hemoglobin to around 10.  Then they discontinue the iron and hope it doesn’t fall.  But we’ve established a pattern that he NEEDS the iron.  So I was concerned that they would change the plan when his Hemoglobin is currently 10.8, the highest it’s been in almost a YEAR!  But his iron levels when directly measured are always very low.  So yay! She ordered the last two doses of iron anyway.  I’m a happy camper. 

Nephrology came by.  Most of you know my oldest son, Jacob, has FSGS (a kidney disorder) and he is followed by a Nephrologist.  That one wasn’t available so we went with her partner.  But this weekend, Jacob’s neph is on call at the hospital.  I like this lady.  We’ve known each other for 2 ½ years now so we didn’t have to start from scratch with that, we just had to start at the beginning with Manny and his diagnoses and current situation.  That helps a lot. 

She is very smart, thorough and caring.  She knows kidneys well! She appreciated all my details, the historical data I had and the spreadsheets of the labs.  She was drooling over them basically and kiddingly said, “Want a job?”  LOL

From the labs and the clinical picture, she agrees he has a kidney disease.  She believes he has Distal Renal Tubular Acidosis.  RTA has all the symptoms he’s been having that I’ve been asking about for several weeks now!  We will not be able to “prove” it via labs because his creatinine levels are false low due to his muscle disorder.  His levels will never show high. 

She was a bit shocked that none of the doctors seemed to understand that.  In fact, they’ve been telling me, “Can’t be a renal issue since his creatinine levels are so low.”  But it’s going to make the interpretation of the labs virtually impossible.  But that’s OK because they will treat him like he has RTA.  What’s the treatment? Close observation of the labs and close administration of the electrolytes!  JUST WHAT I HAVE BEEN ASKING AND PRAYING FOR!!!   If he weren’t TPN dependent, it would be virtually impossible to monitor close enough.  But this is relatively “simple”. 

So yay!  Go God. 

Meanwhile … line …

On Saturday night, The TPA at 7pm didn’t work.  TPA at 11pm didn’t work.  Doc wants to do one more round of TPA.  That’s not standard to do a third but Doc knows just how precious his lines are! So one last ditch effort is warranted.  If this works, great.  All done.  If it doesn’t, we will call IV team to trouble shoot on Monday morning.  And then we will call Surgery consult to place a new line. 

NOT thrilled at this. 

What happened to the line? We have no clue.  It worked when I used it from home.  The first time they went to use it here, it wouldn’t work.  So no one did anything wrong.  They didn’t screw up.  It’s just one of those things. 

…

Now it’s 9pm Sunday and nothing new happened the rest of the day.  There will be a lot of consults tomorrow … Vascular Access, Surgery, Hematology, GI, Palliative Care, Nephrology and they’ll all have ideas that need to be tried.  But for the night, I’m just happy to have a little boy who is stable, is peeing and has fluids going in. 

 ...

1am … Manny’s IV went bad.  His arm got extremely swollen.  We had hoped we could wait until 9am to get everything done with the IV team … new iv, labs, dressing change, trouble shooting, etc.  But it didn’t work that way. 

I put out a prayer request.  Our nurse called around and the people who felt most comfortable doing a difficult stick were in the ICU.  I told them where they had found the extra veins the other day and they were able to get it in on the first stick!  That’s huge.  It was the least problematic IV stick not done by the Vascular Access team.  It’s on his ankle but given that he doesn’t walk, it’s not a huge problem to be there. 

He has started his wet cough again.  I personally think this is what is going on … he has something in his head that is chronic.  He gets on an antibiotic and it clears.  When he’s off, it comes back as a post nasal drip type thing that drives him nuts.  It gets caught in the airway and blocks off his airway.  He can’t get it up or down and he loses his breath.  Suction doesn’t quite go deep enough to help.  Deep suctioning causes him to bleed (his platelets are low) and then he ends up choking on blood.  We have an outpatient appointment with the ENT on Thursday.  And why is this happening now?  His last round of antibiotics ended on Friday night.  Got here Saturday and it’s gone downhill.  For now it’s not in his lungs.  It’s all upper airway and we’re trying to keep it that way. 

 

I'm going to stop this blog at this point because I know today is going to be a wowzer of a day with lots of details. 

 

So thankful to have you all with me on this journey!

Nothing ever goes as planned

After many days of very decreased urine output, and trying to get fluids at home, and consulting with the Nephrologist … we were finally admitted to the hospital on Saturday. 

So Manny is all about the drama as many of you know. He is off TPN for 4 hours and then has to get back on soon or blood sugar will crash. At the 4 hour mark, his line wouldn't flush. So we hit emergency mode. Now it's about someone who can get a peripheral IV line in him ASAP.  There is no IV team today so called the ER and thank God they got it on the first stick!

Then he passed out so we knew his sugar... was low. Got the sugar dosage in and he eventually woke back up. Now we are getting his fluids in. He cries any time someone walks in the room (and they do that often).  They are babying the IV because if it stops working, they have to get another one.  No one wants to do that!  He’s pretty pitiful.  He covers his IV and points to his broviac and tells them to “Use this one!”  Smart dude. 

Now regarding the decreased urination … He's had one diaper in 36 hours and it was 79 ml! That's a squirt. So something is off.  But what?

His labs are coming back in and they are relatively normal. So even more confusing!   Nothing shocking. 

In the meantime, regarding his clogged central line … the IV team isn’t on for the weekend so we’re on our own.  They have an on call person for a few hours on Saturday morning but that’s it.  They put a call in anyway just in case.  I texted my IV team contact.  She asked me to call her and she walked me through what to tell them to do.  THANKFULLY! 

They have instilled something called TPA in his broviac.  It has to dwell in there for 2 hours to get it’s full effect.  We’re really hoping it does the trick and we can once again get blood back and put fluids in.  If so, we can pull his IV and use his central line again.  Putting it in was a trick. The nurses on this floor had not done it so they had to consult someone who had.  They thought they had it all together but … there was a problem and it didn’t work.  So I again called my IV friend and I put her on speakerphone to walk them through it.  It worked!  The TPA went in. 

I’m so very thankful for friends that are resources.  If she had not been available, I was going to call my home health nurse who draws our weekly labs.  She also does TPA and could have walked us through it.  I had my plan a, b and c. 

Now we wait to see if the line cleared.  We will know at 9pm. 

9pm … the nurses and I actually prayed before they did it.  But alas, nothing.  The TPA didn’t work. 

At the same time, I noticed he was getting quite ademic (swollen with fluids).  Like his socks which are normally loose were tight on his legs, the ID band was cutting into his leg, etc.  And at this point, he’d had 450 ccs of fluids in (over 5 hours) but nothing out!  This is called third spacing where fluid goes into the tissues themselves instead of staying where they belong.  It was even going in his lungs and he developed a pretty good wet cough.  (This can easily lead to pneumonia for him.) 

Nurses called the doc who ordered a bladder scan, urinalysis and another round of TPA along with a chest xray to show placement of the central line.  The bladder scan showed barely any pee in there, which means it was all over except where it was supposed to be!  He just needed to pee. 

Over the next few hours, he did eventually pee just a bit and it was enough to get the urinalysis which showed nothing abnormal. 

The 2^nd dosage of TPA went in at 10:45pm.  And out at 1am.  Again, nothing.  :(

Chest xray showed the central line is in place and not kinked. 

Meanwhile, I contacted the other central line moms to see what they have ever heard, seen, experienced regarding this.  And they all said the same thing … two failed TPAs and then it’s time to replace the line.  Just typing that sentence makes me want to throw up!

Now none of that is confirmed as I type this yet.  The doctor is unaware that the second TPA failed.  We figured it didn’t change anything to call him in the middle of the night so why bother him for it?

So we wait. 

In the meantime, he’s got a peripheral IV.  It’s on the inside of his wrist (those teeny tiny veins).  The IV team calls them “the mean veins”.  They hurt.  And that’s the only way for him to be getting fluids or medicines.  In fact, he’s not going to be able to get some medicines (like Iron) without the central line.  And we have to protect this IV like crazy or he’ll have to get a new one and that’s not an easy feat.  But these peripheral, small veins tend to “go bad” pretty quickly. 

Stay tuned … (Finished writing this Sunday midday)

 

                                                                                                                                                          

Nephrology take one

Thursday ... We went to the new nephrologist today.  We could have seen Jacob's but she was out of town and this one could see us less than 24 hours after our inital call. 

I always think it's hard meeting a new doctor ... they have no perspective of us.  There's no context.  I don't know their abilities.  They don't know about my ridiculously accurate gut.  They dont' know how hard we've been fighting or what we've tried or how many things I've caught.  We also usually have 15 minutes at most and that isn't enough to cover the emergent things, much less offer any perspective. 

THIS IS HARD.  And I think it's hard for them too. It's like dancing with a new partner.  It just takes a few times of practice to flow together. 

So today ... we saw a new doctor.  And given the above situation, we did alright. 

Details addressed: 

Basically no urination in 3 days.  This was not addressed today directly.  I said that I felt he was dehydrated.  I think he needs more fluids in his TPN.  I dont' know why he is needing extra fluids, but my guess is he is.  She agreed to add more fluids.  Now, that's great.  But what if I'm wrong about the reason he's not urinating? And how long will it be before he gets those fluids?  We just got our week worth of TPN tonight.  So theoretically they won't give new formula for a week.  Wondering if there's a second way of adding fluids.  BUT ... no one is working on that since the doctor wrote "add more fluids" and knows it will happen but has no clue it can't happen until next Friday. 

Kidneys and kidney function not specifically addressed. 

Anion gap, not addressed. 

What she was very concerned with was the horrible butt rash he has that he's had since mid December.  She is wanting to be aggressive which is great, but what she wants to try is not going to work in my opinion.  I'm willing to try it in case I'm wrong and it wont' hurt him if she's wrong.  So no biggie.  And once the rash is cleared, she wants to do some follow up tests to see if he is metabolizing the TPN.  (Something I'm greatly interested in knowing!!)

And she addressed the issues with the TPN and the weekly labs.  A funny thing is ... GI doctor had written up some lab orders to do the next time we draw labs.  I took those to the Nephrologist so she could see what was already ordered and order more if needed.  The Neph said, "The GI is on the right track with these labs.  I can see what she was thinking.  Good job."  and I sadly had to inform her that all of those labs were MY idea!  lol  From the research I have been doing, these labs should have been done already.  Some periodically and some regularly and they have never been done.  Nevertheless, they will all get drawn on Monday so all is fine. 

...

GI History and Current

Going to see if I can (semi) quickly sum up the latest events.  LOTS of medical info but I’ll try to make it concise yet understandable.

Manny is not doing well on the TPN.  It’s not something horrible, but lots of subtle signs that are relatively easy to overlook unless you’re his Mama.  I’ve been noticing them and asking questions and commenting on these signs for months now but I also don’t KNOW that I’m right so I keep researching and learning.  My personal take?  He's not metabolizing it well. 

History lesson … Manny was with birthfamily from birth until 8 months.  Arnold Palmer for a month where he had extensive GI stuff done.  Went home with us 9 months.  Has had troubles the whole time.  We were with GI group A and they didn’t get it at all.  I was admitted in the hospital with him in severe pain and problem when they were going to discharge us without even doing any testing, etc.  It was the final straw with this group and I “fired” them.  My first and only time of firing a doctor. 

An hour later, GI group B came in.  They did tests and by that afternoon, had determined his stomach had completely stopped working.  They have been our GIs ever since. 

During the time with them, however, we were advised to go to GI group C for a consultation and we did.  They ended up admitting us and we were there for almost a month.  He got so weak and sick that they had to put him on TPN.  At that time, they thought it would be only a few weeks to “Get him out of nutritional deficiency.”  But they SAW the horrible reactions he had to feeds. 

So Group C wrote the TPN and Group B did all the rest. 

Well, months went by and they still didn’t have any new plans.  So I devised a new plan to go to GI Boston for motility study and see if they could see what was going on.  I finally got there last September.  Now it had been almost a year by this time that we were with Group C and this group is 3 hours from us. 

They had originally started out doing relatively well with the TPN but towards the end, someone who had no clue what they were doing was writing the TPN and it got BAD.  Bad enough he ended up in the hospital!!  So I figured, might as well have GI group B write his TPN as well.  I figured he’d be starting feeds per the Boston study and we might as well have all his GI care in one place (the closer place). 

Uh … mistake

So Group B started taking over the TPN and initially it was better.  That was in September.  But it was never quite right.  Something is “off” in the TPN.  Missing an enzyme? Vitamins?  Something.  But darnit, I don’t know enough about this to even speculate! 

Within this group are two doctors.  We saw Dr. A.  This doctor told me point blank, “I will not babysit TPN”.  And he was being truthful with me.  He did not.  He was pushing for a surgery that wasn’t going to work and Manny would not likely survive.  ALL of my doctors thought it was a bad idea. 

 

Eventually I got our Palliative care doctor to call the GIs and work out a solution for me.  I would be switched from Dr. A to Dr. C. 

Thankfully, Dr. C agreed to take on our care even though it’s complex and messy.  I haven’t seen Dr. A since November.  That’s right, a child who should be seen every 2-4 weeks hasn’t been seen by a GI in 3 months. 

CURRENT: 

All that so I could tell you the current situation. 

Manny is less stable now than ever.  I’m very concerned about several things going on.  Weekly he gets labs and they are trending in a bad way.  But I am POSITIVE I am the only looking at the labs from a GI perspective.  Why? Because Dr A thinks Dr C is handling and vice versa. 

Feb 5 I write a TPN guru at Chiidren’s Boston.  She is the one I talked to back in September and shared my frustrations and she ran some labs and determined, yes, he was having troubles.  I remembered she said there was an outpatient TPN guy who might be able to help.  I couldn’t figure out how to get hold of him so I emailed her directly.  Never heard back from her and figured it was a dead end. 

Feb 18 Labs are drawn and I get them Feb 19.  There are some things on there that are “low” that are NEVER low for Manny.  And I start to digging.  It all points to kidneys having a hard time.  Meanwhile, he stops urinating.  Completely stops.  He went 36 hours without any pee.  Now we’re 72 hours in and only one diaper.  His potassium is low and dropping (more about that in a second).  And I have not seen GI!  Finally, he has a ridiculous diaper area issue … the pee is scalding his skin.  Looks like a chemical burn.  This has been happening since Mid December when I noticed certain things start to go awry.  EVERY treatment under the sun has been tried and we were referred to a wound care specialist.  Yes, it’s that bad.  His anion gap is 21 (normally it’s single digits.)   

Feb 19 I go to bed asking for prayers on FB.  He’s a sick little dude. 

Feb 20, I wake up in the middle of the night to discover the TPN doc from Boston has emailed me back.  Turns out she went on Maternity leave on Feb 5 and just got my email.  She suggested I go to see them ASAP! 

Feb 20 I have our first appointment with Dr. C (Our new GI whom we know but has never been in charge of our case until now.) 

Now here’s the cool part … I brought her the Quest magazine where we are the cover, a Mighty Manny book and a nice bag of chocolate.  I want this meeting to go well! Ha.  Before we even get started I thank her profusely for taking over our case.  And she stops and admits that he is way over her head.  I tell her that he’s over EVERYONE’s head but I thanked her for not giving up on us!!  That was HUGE. 

I also told her of my middle of the night email from Boston offering to take over his TPN management if/when we can get back up there.  She said YES.  And basically asked how quickly we could get there!  She will work with them on coordinating local care. 

As we proceeded with the visit, she had a lot of concerns about his current health and heard all of my concerns.  She felt there are a lot of adjustments that need to be made as well as a lot of tests that need to be run.  There are also tons of things she wants Boston to sort out.  Specialized tests.  So we might be in Boston for a while if/when we go. 

All of this was a direct answer to prayer.  THIS is EXACTLY the solution I wanted.  A local doc who gives a care and a TPN team who knows their stuff.  J

Meanwhile … remember those symptoms I told you about earlier?  They are very concerning and contacted my pediatrician on 2/20 and she agreed.  She got me in to a nephrologist on 2/21.  As I write this current part, we haven’t been to that appointment yet … it’s in a few hours.  But his constellation of symptoms is concerning.   

For example, his potassium:  Should be 3.9-5.  He used to live around 4.5 which is basically perfect.  For the whole time he was on TPN he was stable with potassium.  He was getting 20 meqs per day.  Then in about September, his potassium level started to drop below normal or hover around 3.9.  So they added more potassium in the TPN.  Repeat. Repeat.  And now he’s below normal with 80meqs per day.  Why the increased demand for potassium?!?  And if you’re not really paying attention to the behind the scenes it’s easy to miss that.  Like Monday’s labs?  His potassium was 3.9, the lowest it can be and still be normal.  If you were to just glance at those labs, you’d see it was fine.  But what you miss is … what all did it take to get that barely in range?  And did that cause his system to become too acidotic? Now he’s breathing weird and his Co2 level is low.  He’s dehydrated.  His anion gap,  Etc. 

Finally, he’s needing oxygen last night.  He NEVER needs oxygen. 

Oh Manny … you love to be complicated don’t you?!?

 

If you can't say something nice ...

My mom (Like most moms) taught me that familiar adage: if you can't say something nice, better to say nothing at all. 

So I've been needing to say something but couldn't figure out the nice way to say it.  Here goes my attempt. 

In the past 3 years with Manny, I have built a team of doctors.  We are all pretty much on the same page most of the time.  A true partnership.  It's working.  I trust their judgment and they trust mine.  I even have 2 that put in their orders at the hospital "PRM" which stands for "Per Mom"!  :) 

There is one exception to this happy little team.  And I've tried several different alternatives but still haven't found a working solution.  And it's not a little area, it's, in fact, the thing we need most help on. 

It's become obvious to me (and pretty much all the doctors on the team) that I need another specialist in this area.  So the search started.  I did lots of research and found this place.  For anonimity, I will call it "AP".  My doctor called over to AP, sent the records, I sent records, CMS sent records.  They agreed to see us and to get us in quickly.  I spent hours and hours organizing my files and writing the historical perspective of how we arrrived to this point and what our current needs/concerns are.  I felt ready for this appointment. 

As we left, Dan and I prayed for God to make it clear if this was the right place to help us or not. 

Manny, Kaley and I drove just under 2 hours (traffic) and happily got to the room.  They checked us in, did the triage part (blood pressure, weight, temp) and put us in a room to wait for the doctor who should see us "any minute now".  As time drug on ... I got a sinking feeling.  I even wrote a Facebook status update for people to pray as I got a horribly uneasy feeling come over me. 

An office manager walked in abruptly and demanded I say what I was there for, what I expected from them, etc.  I calmly explained that I felt he was getting "substandard care" and that I wanted him to be seen there.  I said that I would love for his care to be transferred to them if they felt they could help us. 

Eventually, that office manager and another business lady came in and asked for my records which I happily handed over.  They started saying things that made me know they were hesitant to see us at all.  They said that the doctor wouldn't be able to give a second opinion of such a complex child in such a short time.  I explained that I felt this day would be a "meet and greet" and it would take time to establish the long term relationship.  I had time. 

They took my records to the doctor they said.  Less than 3 minutes later, they were back and said the doctor had reviewed my files.  (Bull, I had a 5 inch stack!)  They said, "The doctor doesn't feel it would be in the child's best interest to be seen so far from home.  He doesn't want to take on such a medically complex child."  And with that they ushered me out of the room. 

I didn't get to see one medical person! 

I left in shock.  I barely made it to the car before I burst into tears.  I was angry and frustrated and sad all at once.

I started making phone calls. 

Clearly, this was an answer to prayer!  It was a CLEAR, "NOOOO!"  So for that I'm thankful.  But now I'm still without the most important specialist I have.  Manny should be seen by this type of doctor at least once a month.  We haven't seen one since November.  And he's not medically stable in this area.  I don't know how long we have until he crashes.  So I got scared. 

My pediatrician was also quite upset.  She had arranged this meeting.  She demanded to talk to the Doctor directly.  And sure enough, she couldn't get him to budge.  What a ridiculous situation!  I felt like this ... we were in a car wreck and my arm was falling off and spurting with blood,  I went to the doctor and he basically said, "We don't see patients here who need help! Go somewhere else." 

Now, as the result of this meeting gone wrong, a few things came out of this.  1)Don't go to AP.
2) the Pediatrician and several other specialists are now on the ball and trying to get us help.  (Before we needed the help but noone knew it and now lots of people are on board with helping us locate the right specialist.  So that's HUGE. 

As I write this, it's Sunday night, Feb 17 and we still don't have a new specialist.  Well, technically I have an appointment with a new doctor.  But the appointment is more than 2 months away and that won't work.  And we aren't positive they'll take on a complex case like Manny either.  So ... monday morning, the records will all be faxed over from the various doctors.  Our pediatrician will call and talk to the doctor directly asking if he will take this case and if they can get him in sooner. 

Can't wait to report we got a new specialist and that he/she listens to us.  I will shout it from the roof tops when he starts to get the right care he needs in this area.  Like I said, the REST of the things are under control and doing relatively well.  The rest of his systems are relatively stable.  

For example:

Pulmonology ... now that we've stopped feeding him via his tube and he is only getting the TPN feeds, his lungs sound clear.  He's not needing oxygen.  His pulse ox is 98-100 every night.  No complaints in this area. 

Cardiology ... he has an innocent murmur but it's stable.  His latest Holter monitor showed nothing of concern.  His heart rate is pretty decent, especially for him.  While awake it's about 100-120.  While sleeping it's about 85-100.  He has periodic times where it dips too low or jumps too high but relatively no concerns. 

Neurology ... nothing new was happening on this front for quite a while.  Recently, our current Neurologist referred us to another neuro in Orlando.  Our appointment is for Mid April.  And then just last week, I got a message out of the clear blue from our usual Neuro that he was researching and was wondering if Manny has MNGIE?  Now this is a diagnosis I have stumbled on over and over in researching.  He has most of the symtoms for it so it's always possible.  It stands for Mitochondrial NeuroGastroIntestinal Encephalopathy.  I never pursued this because as you research MNGIE, one of the differential diagnoses to consider/rule out is Merosin Deficient Congenital Muscular Dystrophy (which Manny has via the muscle biopsy).  But it's still been in the back of my head all this time. 

Besides, the muscle biopsy shows a secondary nonspecific deficiency.  Says it right on the biopsy report.  And no one has ever pulled at that thread.  Not sure why.  His Merosin diagnosis shouldn't come with the GI complications that he has!  And this MNGIE diagnosis would account for all of it.  The problem is... it's a very expensive test and our insurance won't pay for it.  There are secondary tests that point to it without actually proving it.  Those might be helpful and I want to pursue those.  They are simple blood tests and not super costly to run. 

Why does it matter?  Currently, we have a mystery ... a primary condition that should not cause any GI complications.  But his GI system is out of control.  But the one test we had done (motility in Boston) showed a "basically normal" GI tract.  So we know it's a brain/GI thing.  Several GI doctors think that feeding shouldn't be a problem so they push it.  But we all know it IS a problem so I have to put my foot down.  I would LOVE LOVE to have documentation to prove I am right and that they should not push feeds on him that he cannot tolerate.  Without it, it's just my word for it basically.  I want more than that.  I also would love to know the diagnosis because it might change a bit of his treatment plan. 

Vascular Access ... That's his central line (broviac) and we have just hit the one year mark for no central line infections.  That's HUGE, especially considering his last line was in only 3 weeks before he was in the hospital with the line infection that went so horribly wrong and he barely survived.  We have done a lot of changes to get this system working but all those things are coming together to help reduce those line infections. 

And those are the basic systems that are working well. 

Stay tuned! 

Good news, lots of it!

Can you handle Good News??

So I posted the blog about "Frustration" on Wednesday and then within the next hour, got 3 pieces of GOOD news.

I posted a blog about the night last night and the frustrations I'm having with immovable stupid doctors. I'm OVER IT.

But the cool part ... I might have some movement on all the fronts! It was late this afternoon after I already wrote the blog when I found out all the cool new things that we've been working on for a looong time.

1) One doctor was being difficult about not putting Iron in the TPN. It's the easiest and safest thing to do. Instead of the iron going over 30 minutes, it goes over 20 hours! Also, it would take me 3 seconds to put it in the TPN at home versus 5 hours to drive to the hospital, get the infusion and drive home. Next, it's better because I only connect him one time a day versus several times by getting the infusion thus decreasing his infection risks. Next, it's better because we don't have to go to the hospital ... where all the sick people are!

So why wouldn't the doctor do it? We didn't know.  NOOOOO! End of discussion. So one of my hospital friends bailed me out today. He is the in hospital TPN pharmacist and one of the most kind, decent, humble men you'll ever meet. He loves Manny and is always so sweet to us. He was the one who helped get the Selenium in after MONTHS of me working on it and he got it done. Well today, he called the GI. The GI was on vacation in Miami but he called anyway on the personal cell phone.   They came to an understanding that worked for us.  It was agreed it could be put in if the Hematologist would write the script, be in charge of it and follow those labs.  But bottom line ... Manny is getting what he needs the easy way! I just have to work out the nitty gritty details. (That means about 10 phonecalls Thursday but well worth it.)

The only real issue could be that some people have allergic reactions to Iron.  Manny has had two different types of iron and done well with it so odds are he will continue to have no issues.  And in the remote chance that he does, I know what to do and how to handle it.  So I feel great about this decision.  He will need to get his iron levels back up to normal (he's EXTREMELY low right now) and then find the right level maintenance dosage. 

2) My pediatrician contacted the Orlando Arnold Palmer GI group I asked her to. They will see us ASAP. That's HUGE HUGE HUGE. I have no clue if they can help us or not but worth a shot. They see us next Wednesday.  I am hoping they will be willing to give advice regarding his TPN and even manage it possibly.  They might have ideas of what to do for feeding him too but we'll see.  I'm not willing to put him through the torture again unless it's a good, NEW plan. 

3) Doc came by today.  I told him about last night and how weird it was. How he immediately got better with the antibiotics, etc. And how many times he's had these same symptoms of high fever and post nasal drip but the viral panel is clean. So he got to thinking. We brainstormed. He wants me to see an ENT ASAP. We think possible chronic sinus infection. It would make sense! The ENT he wants me to see is the one that Zoe sees. She became a friend, we even went to lunch and started working on a mission trip idea. I called her office and they are no longer taking his insurance (state). I asked to leave her a message and see if she'll make an exception for us. I'm sure she will say yes. She is the only ENT I trust around here! But there is possible hope and help!! Really?? So excited.  Thursday I got a call that yes, she will take us. (Shock.  Ha).  But the problem is ... Doc wanted us to see her in 3-5 days and the first available "new patient" appointment is for Mid March.  That is just not going to work.  Wondering if I should email her directly and get in sooner.  Because if he were an established patient, he could be seen next week.  But policy keeps us from it.  (But as I just pointed out, we have already run into one policy issue and overcame that.)  Still deciding.

So right after I wrote the woe is me frustrated blog post, I got the first good news in a while!

Thought you might want to know!

Now the last piece of good news for the night ... Today as I write this is Thursday, February 7.  It's a good day.  Why? 

Because one year ago today, he got the broviac (central line) he still has.  He has not had one central line infection in the year. 

Why is that a big deal?  In the first 3 months of a central line, he had 8 lines.  He almost died by going into septic shock in December 11 due to a central line infection.  That line had been in 3 weeks and 2 days. 

It's virtually unheard of to a go a whole year with no line infection!  So what worked?  I got training.  I now handle basically all his line care.  And we got a special medicine (ethanol lock therapy) that was controversial.  All this is due to a Vascular Access nurse named Stephanie. 

So today we are celebrating a happy birthday for a broviac! 

Frustration

Life in the hospital was going all peacefully, according to schedule.  THAT never happens.  I put Manny to bed on Tuesday (Feb 5) about 10pm (they'd finally done all the "messing" with him for the night).  Before 10:30 he was already having troubles breathing. 

For the next almost 2 hours, I was suctioning, repositioning, praying and trying not to let my brain get ahead of itself.  This could be a "fluke" where he is able to cough it up.  Or it could be the start of another trip to ICU.  There was no way to tell at the time. 

He was desatting, his respiratory rate went through the roof and his heart rate was almost 200.  (No fever and he was asleep.)  He's choking on post nasal drip from some unidentified viral infection.  It was blocking his airway and started sinking into his lungs.  You could hear his rattle from across the room, no need for a stethescope! I asked the nurse to call for Respiratory Therapy to come by for an extra treatment.

 So thankful to report it worked.  It cleared the airway blockage and he slept at 99-100% saturation (without supplemental oxygen) the rest of the night!  He slept so well (and so did I) that he didn't even wake up to ask to be repositioned in the middle of the night.  We slept straight from 1am-6am! He then went back to sleep from 6-7:30 when the morning tech decided it was time for us to wake up I guess. 

It's 9:30am as I type this on Wednesday and he's mostly fine.  He has a bit of a cough ... seems like it's from post nasal drip.  He now has a runny nose (clear) and gunk in his nose.  He's sneezing and coughing.  I don't feel it's enough to keep us here any longer, but I reserve the right to be right back if he starts to have respiratory complications that are beyond my home skill level. 

With that said, the blood cultures are still negative.  He's fever free.  So we have met our discharge criteria.  That means we just wait for the doctor to come discharge us. 

>>>>>>>>>>>

Now on to a new subject.  How to deal with Chronic Frustration?  There is an ongoing GI issue (details not relevant) that we have dealt with off and on since we adopted Manny.  He has had days and even weeks at a time where it wasn't an issue, but it's pretty much chronic.  And as of right now, no one has a clue the cause or how to deal with it.  Everything is just a bandaid at best.  So very, very frustrating. 

I believe this is something quite rare as I can't even find it in the literature. Or perhaps it's several things all overlapping causing multiple systems to be involved.  The mystery of it is so hard. 

On one hand, I feel like pushing and pushing for a definitive diagnosis to KNOW what we are dealing with.  With the diagnosis, there might be changes in treatment and prognosis.  But on the other hand, we could chase the diagnosis and never find it.  Or we could find it and it NOT change the treatment/prognosis.  Would that be for naught?  And I find myself vascilating between those two competing thoughts. 

I've actually been TOLD things like, "How hard are we going to work for a child with a terminal condition?" and "Why does it matter?" 

Well ... what if it DOES? (sigh)

OK so lets say we do decide to pursue the diagnosis. Where to start?  The whole thing is just so complex that it's kinda overwhelming. 

Now, with all this said, we're told that NONE of it has anything to do with his underlying Muscular Dystrophy.  So not only does he have this extremely rare and not understood" terminal" condition ... he has a condition that NOone seems to know what it is or how to handle it.  And ironically, if we don't figure it out, it's MUCH more likely that THIS is what will take his life and not the Muscular Dystrophy. 

So I'm frustrated. 

My pediatrician has been working with me to find a solution for part of this issue.  (She's been our kids' doctor for 12 years!)  She made arrangements for me to see a specialist in Orlando. It will be almost 2 hours one way to get there but if they can help, it will be well worth it.  I'm supposed to call as soon as we're discharged and they can get us in within 24/48 hours.  Hmmm.  By comparison, I called for the next available appointment for the same type of doctor locally and they gave us 6 weeks quote.  And we're an established patient with them. 

Hopefully that means this is an indicator that they understand the gravity of the situation. 

By making this appointment, however, they need our medical records from the last doctor.  This will alert them that I am not quite happy and am looking for another opinion.  I have a feeling this will be a problem for us.  I imagine they will ask us to no longer be patients with them.  I hope not, but it's entirely possible.  And if they do, it's likely to cause a domino effect.  It's a bell you can't unring.  And the new group might not even be able to help us. 

So I'm anxious. 

But at the same time, it's possible they CAN help.  It's possible I can start to be the Mom again in this specific area and not be managing medical pieces of his care that I have no business doing.  I would LOVE to turn over this part to a qualified doctor. 

I know some of this is purposely vague.  I have friends at the hospital who now read this blog! I'm at this hospital way too often to burn my proverbial bridges.  (For perspective, we've been admitted 18 times in the past 15 months.)  So I'm sure people can read between the lines, but I'm trying to be diplomatic at the same time the way I share this with anyone who might be reading this. 

Which leads to the next topic: When diplomacy is over rated.  I wholeheartedly believe in the more flies with honey than vinegar principle.  I can tell you 1000 stories of how it's worked.  I can only count a handful of times I wish I'd been more forceful. 

Advice is often given to moms of chronic kids for us to get loud and forceful and don't worry about being a B*tch and they tell stories about how they did and the results they got.  But that sentence is almost always followed by something like, "Because I know I'll never see these people again." 

See the problem with that sentence??  Remember how I just said that he's been here 18 times in the last 15 months?  Often for weeks at a time?  Uh, ... I WILL see these people again.  I WILL need their help again.  I NEED them to work with me.  And so I believe diplomacy is the best way to handle it. 

And yet ... I think there are times when enough is enough.  And there are several people who this admission who have noted there is some "frustration behind my smile".  More and more doctors are asking the obvious question of "Why isn't Dr. X doing something about this?"  They see there is one glaring hole in our care, even without me pointing it out. They are frustrated FOR me and then they are frustrated by running into the same immovable object I keep hitting. 

So diplomacy might just have to take a back seat until I can get this straightened out.  And that solution can come in several forms.  Will be interesting to see how this unfolds. 

The one thing I DO know ... things can't stay status quo.  Manny's health is suffering with status quo.

UPDATE:  4pm ... Discharged!  Now we just have to keep him breathing well at night. 

Fever... again?

So our journey has taken us back to the hospital.  Like you're shocked, right? 

My scene as I drove away.  They're both making heart signs.  Sniff.

Manny has been fine.  In fact, we've been celebrating how fine he is!  We have been out of the hospital since Dec 23 so this is HUGE that he didn't go in until Feb 4.  That's the longest stretch out of the hospital in almost a year. 

What landed us here this time? Fever.  We were at a doctor appointment this morning when they got a 101.1 temp and that's an automatic admission for us.  I took off his jacket and asked them to do it again and they got a bit lower one.  So we held off.  He was borderline the rest of the day but acting fine.  No symptoms. 

Then about 3pm he started acting very lethargic.  He was just not acting right at all.  He was quite flushed.  Sure enough, 102.8  So no way around the admit now.  Dan was on a business meeting several hours away so I called Mom to come watch the kids and I did my last minute packing.  (The main packing is always done but last minute things like the laptop and phone charger always have to be packed.) 

About 4pm, he started trying to throw up.  Now due to a surgery he had, it's virtually impossible for him to throw up.  But it doesn't stop him from trying to.  I suctioned out his stomach contents (the benefit of a gtube) and it was full of nasty bile.  So after that was gone, he started acting much better.

When we got here, he started in with a runny nose (clear) and nasty smelling diapers.  So something is brewing.  In fact, I knew this was on it's way.  How?  Earlier today, we had blood labs drawn and they were pretty "wonky".  I even told Dan that I knew he'd be admitted within 24 hours.  I just didn't know it would be within 2 hours! 

But for tonight, he's sleeping.  He's still himself.  And tests are being run to see what bug we're fighting.  That determines the type and duration of treatment.  If he can keep his fever down for 24 hours, and his blood cultures remain negative, we can go home after 48 hours. (Wednesday night).  But if symptoms progress, we could be here for a while.  So obviously we're hoping for a mild viral bug, something easy and boring and normal so we can go home and get back to living. 

Tuesday ... the night was relatively uneventful.  I finally got to bed at 2am (meds issue kept me up until then).  Then the machine malfunctioned for an hour from 5-6am.  Change of shift at 7am so I was up for the morning.  By 8am he was awake and ready to go.  Me? Not so much.

All day it's been one doctor after another.  We're trying to sort out a few of his TPN issues.  All but one doctor is completely on board with the plan.  But that one has caused a complete  halt in the process.  I'm working on Plan B and C and D.

I'm trying to say this calmly but I am very, VERY Frustrated. Stay tuned. 

Now I'll share a couple of "lighter" things. The children's hospital where we go has colorfully colored doors.  On the way to the hospital he picks out what color door he hopes to get.  (He always picks a different color so no way to predict).  This time he said "Red, no I mean Blue."  When we got here, we got a red door.  It was up at the front of the hallway.  The kid next door to us was having some GI troubles and there was the most ridiculous stinch that filled the whole area.  I was wondering how we'd sleep with that smell and started praying for whoever was that sick in that room. 

So I guess a benefit of being a "frequent flyer" there ... the charge nurse came up and offered us a different room further down the hallway!  We accepted.  You'll never guess the color, right?  BLUE!  But of course. 

Then today?  There was a "code gray" which means security is needed.  Yup, it was in that first area where we would have been if they hadn't moved us!  Perks! :)

7pm Tuesday ... Rapid strep negative.  Flu negative.  Rhinovirus negative.  Etc.  The Respiratory Viral panel caught nothing.  Good news.  It just means we don't know what he has. 

But the super good news is that he hasn't had a fever for a while.  If he continues fever free tomorrow with no additional symptoms, we can go home Wednesday!