Today was an emotional roller coaster.   During rounds today, everyone felt that Hannah was ready to be extubated.  Even though I was nervous about the procedure, I thought she was ready too after being given a few extra days of rest to help “get the junk out.”

Because they were very well educated on what could happen to Gaucher kids upon extubation, they were ready with every possible thing they thought they would need from a crash cart to lidocaine recommended by her ENT with, it must have been about 8 or 10 people there.  I was a nervous, nervous wreck.   As soon as I heard “the tube is out,” I sprinted to the end of her crib.

She extubated very easily!   As soon as I saw them sit her up without the tube, I had a brief glimpse into seeing “my baby girl” coming back.  Within about 20 minutes or so, the sedations had really started wearing off, and she was looking right at me, trying to get on her tummy many times, and was really trying to move around.  She was incredibly weak and shaky, however.   She was unable to put her paci in her mouth, and even when we did, she was so hoarse trying to cough and get used to not having the tube, that she had a hard time keeping the paci in.

About an hour after extubation, I was able to hold her.  I sat in the chair, and the nurse placed her (and all her various IVs and leads) in my lap, cuddled in her blanket Daddy brought from home.   She immediately tried to maneuver herself into her normal position that she does when we are at home and she wants to sleep.  She snuggled right into my chest, and she closed her eyes.  I melted.  I had my baby in my arms, finally.

Unfortunately that did not last long, within about 20 minutes or so, she was starting to have difficulty catching her breath.   Her heart rate started to jump up into the 180s, and her blood pressure was incredibly high at 140/60-something.   She was getting progressively worse in what seemed a very quick time.  They tried giving her a breathing treatment, but that did not help.

At that point, they were considering doing a high-flow nasal cannula (she was on 3 liters of oxygen, and this would bump it up to 6 or so liters) or put her on CPAP.    However, even during that short time they were discussing it, one of her doctors, Dr. Sam (who has seen her almost every day), decided that she was just having too much difficulty and said she needed to be reintubated.

My heart sank.   Daddy and I left the room, and we walked into the hall.  I just lost it.  I don’t know what it is about me and intubation, but these have been the only two times that I really have just fallen apart.    With everything going on in our lives right now (the move, Daddy’s unemployment, Hannah’s health, Ethan and Abigail missing mommy, etc), it just was too overwhelming.

I keep feeling like she needs to be given a break, you know?  We have been dealing with her illness since she was two days old.  TWO days old!  She just turned 22 months old today.  Poor thing just needs a break.

She reintubated fine, and now the plan is to wait a few more days.  She still has a lot of junk in her lungs, so they have started to “cup” her (vibrate her chest with a cup to loosen up the junk in her lungs) every 4 hours plus continue to suction the secretions out.   It is obvious now, that she does not have the cough reflex and/or strength needed to do this on  her own.  Also, she became very acidotic, and even though she was getting the oxygen in her body via the cannula (her O2 sats were great), her body was not able to get the oxygen really into her body.  Her CO2 was 127 (very high), and her pH dropped to 7.02 or something like that (7.35 is lower normal I believe).

I am so sad for her.  She is back to being sedated on the vent.  I am actually more worried now on whether she will be able to get off the vent.  I mean, she could have this pneumonia junk for weeks in there.  Would she have to be sedated and vented that long?   Of course, I keep fearing in the back of my mind that we are getting closer to the trach situation, as this would probably be a prime situation where it would come into necessity.

She just needs a break.   I got to hold her briefly today, and she looked into my eyes.  I want that back again.

No extubation today.  Hannah’s chest xray looks unchanged since last Tuesday, and they are still suctioning a lot of yellowy junk from her lungs, so they wanted to give her another day on the vent.  They ended up doing another CPAP trial again today, for 60 minutes, and she did great.

They seem to believe she won’t have any problem breathing off the vent — the big question mark is what will she do if she feels the congestion again — will she have another breath-holding spell which may turn into seizure?  Will she just gag and cough like she normally does?

She is still incredibly hard to keep sedated.  Today they have used a combination of Versed, Fentanyl, Chloral hydrate, Pentabarb, and Precedex (the last two which are really heavy duty).   She still has a lot of difficulty staying sedated, even at incredibly high levels of medicine.  They are going to start weaning her down off the IV drip sedation (Versed, Fentanyl, and Precedex) and started giving her methadone and Ativan via IV.  So she has been on SEVEN different sedation meds today, used in combination.

The nurses in the PICU have nicknamed her the “Texas Tornado” because she really does thrash around when the sedation starts wearing off.  She tries to break out of her wrist restraints (succeeded a few times), pull out her Central line in her leg, and she is giving herself her own physical therapy in her legs because she kicks a lot.  She also keeps trying to turn herself on her stomach, probably because she never sleeps on her back!

We will see if they will be ready to extubate tomorrow.  If they do extubate, she will be in the PICU for at least an addition 24 hours once that is done just to monitor her and to wean her sedation meds.

We will see what tomorrow brings for my little “Texas Tornado.”

It is hard to believe that she has been here at TCH for one full week now.  Tomorrow will be the beginning of the second week.  Geez…

For the most part, today was a status quo day.   She did a trial CPAP test for 60 minutes to see how she would tolerate a CPAP off the vent.   She did well on that.  Their hope, as of this morning, was to do another CPAP trial test tonight for 60 minutes and then if she does well, then extubate her tomorrow.

Sounded like a great plan up until about 3 hours ago when I went to visit her, and she had spiked a fever of 103.5.  She had been at 99.8 and 101.8 since we had been in the PICU (Wednesday), so this was an unwelcome surprise.  They took urine, blood, and sputum cultures and sent them off to be grown out to see if her Adenovirus is kicking up a notch or if something else is brewing.  Honestly, I hope she caught something bacterially so it can be treated with antibiotics, but who knows.

They gave her tylenol, and about an hour later she was back down to 100.4.

The nurse thinks they are still planning another CPAP trial tonight and possible extubation tomorrow.  However, I am a bit nervous about extubation tomorrow.  Screw that, I am HELLA nervous about it.  I have heard other Gaucher family stories about extubation leading to reintubation because of laryngospasms and even trachs as a result of it.   I’m a nervous wreck right now just thinking about it.

We will see how she does overnight.  At least if they extubate, they know what they are prepared for, as they are going to be bringing ENT in just as a precaution.

Another problem that they are having is trying to keep her sedated.  They are using continuous fentanyl and versed with ‘as needed’ fentanyl and versed plus they even tried choral hydrate and pentabarb today.  Nothing seems to last and give her good sedation anymore.  Most likely it is because she is hypermetabolic, but it sucks see her so agitated and unhappy.

She is definitely getting more restless and agitated.  She has gotten out of her wrist restraints a few times now, and she just wants that tube out.   I feel horrible because she looks at me, and I know that she is wondering why she has this tube in her mouth, why is she restrained, and most of all, why the heck am I not picking her up!

I will admit that it is starting to take a toll on me.  I have not hugged my baby in almost a week, and all I can do right now is hold her hand, rub her forehead, or find another 2″ space on her body that is not covered with a tube, wire, or lead.  I want to hug her so badly.

I’m in one of the nap rooms of the Ronald McDonald house right now.   This place has been a godsend, I tell ya.  The main RMH is about a mile away, which is used for families of longer-term patients.  This one in the hospital is just for PICU and NICU families.

Today I had lunch with a group of moms, provided by the RMH, and they were all new parents.  The mom that I saw last night came down and sat next to me and thanked me for coming to her last night.  I told her how happy I was to see her.  Her new baby girl, a week old, is having significant respiratory issues and apparently had turned blue repeatedly while they were in there, requiring being put on a vent.   They were warned last night that the baby will most likely require a trach.  Small world, huh?   But I really was so glad to see her and to know that she was doing okay.

Hannah did great through the night.  They gave her a dose of Lasix overnight because her urine output was getting low, most likely a side effect of the medication she has been given.

I was there when they did rounds on her this morning.  Very interesting being in a teaching hospital.   The resident wanted to try and extubate her today, but the attending (whom I have talked to every day since she has been in the PICU) said that he wants to wait and see how she is on Monday.  Had she been a ‘normal’ child, they would have attempted a “extubation trial,” but as the attending said, he does not want a ‘trial’ with Hannah due to her Gaucher’s and it’s problem with laryngospasms.  So they really will only extubate her when they feel she is ready.

Again, the attending had said to me with a huge smile on his face, “She really is doing great.  Just as we had hoped.”

She does look good though.  Definitely getting more fiesty, and I am starting to see more personality shine through.  She is having to be sedated more now because she is waking up more and less lethargic.  But she has been trying to turn over onto her stomach and side to sleep (they have her on her back), and she is grabbing at all the lines and tubes on her left side with her hand.  They had to move her down towards the end of the crib to keep her away from them — even WITH wrist restraints.   Also, she got lose from the wrist restraint once and tried to pull out her tube.

I had to laugh when they told me that — THAT is my  Hannah.

If all goes as hoped (I have learned not to say “planned” here in the PICU/NICU since nothing seems to go as planned with many of the parents I have met), she will be extubated on Monday and put on a CPAP machine.  Hopefully from there, we can go to the PCU (intermediate care unit) and eventually up to a regular room on the 10th floor where we started.

A sense of relief came over me today when they were saying that she is ready to be extubated.  That means she has definitely improved since Monday when this all started.  I am also relieved that the attending also agreed to wait until Monday to give her a few more days to get the junk out of her lungs from the pneumonia and virus.  I don’t mind the extra couple days being on the vent if it means that the light at the end of this tunnel is very close by and not a distant dream.

I just left Hannah, and I was in a great mood, ready to write a very positive update.  I had just left my kids and husband who came down for dinner and was walking back to the Ronald McDonald house.

In the hallway by the NICU, I saw a mom who I had met in the RM house and have seen around the hospital during the past week.   She was crouching down by the wall in the hallway, crying hysterically.   I went up to her and crouched down next to her, just rubbing her shoulder, and tried to talk to her.  She didn’t say anything, she was just too upset.  Her husband came back about 2 minutes later with a wheelchair (she had given birth a few days earlier), and he had tears streaming down his face.  I left them alone and walked back towards the RM house.  I turned back to look at them, and he was consoling her, still crouched on the ground.

Just yesterday, she was saying to someone on the phone how her new baby was doing better.  They were pleased with his progress.

I don’t know what happened to her baby tonight.  But I can only imagine.  Those weren’t tears of a little setback.  Something disasterous had to have happened.  I could not get them out of my head the entire walk back to my room.

But Hannah had a wonderfully stable day.  Her color looks better.  Her fever is still hanging between 99+ and 101+.  The night nurse gave her a bath, washed her hair, and put her hair in pigtails.  She looks adorable.

They turned down another setting on the vent, something called a SIMV (how many breaths a minute it makes her breath) because she was breathing on her own over the setting.  However, they weren’t ready to bring down the amount of oxygen yet because they wanted to give her more rest.

They stopped one of her antiobitiocs, vancomycin, because that culture came back negative.  She is still on the antiobitioc for the pneumonia, the seizure meds, Protonix for her  reflux, and Versed/Fentanyl as sedation.

The doctor came up to me this afternoon and said “She is doing great.  I am very happy with her progress.”   It put me in a great mood all day long.

That is until I walked by that family tonight and saw how fast that could change.

I know Hannah is not out of the woods yet.  But she gave us no surprises in the past 48 hours, and she is doing what she needs to be doing — resting.  For now, that is good enough for me.