I went down this morning during shift change (7 am) to check on Hannah.  They ran liver function tests (LFTs) at 4 am, which was scheduled yesterday from rounds.  They were concerned with chloral hydrate’s affect on the liver since that is where the chloral hydrate metabolizes and since her liver is still mildly enlarged from her Gaucher’s.  Three of Hannah’s numbers came back abnormal.  I don’t know to what extent (I will learn that in a couple of hours when they do rounds), but I know that GGT was one of them.

However, Hannah’s GGT has always been slightly abnormally high as well as another value was slightly higher than normal when she had her liver function tests a few months ago.   So I am hoping when they compare the two, the values will be less skewed than what they were originally concerned with.

On a separate note, a ChildLife services came to visit with Ethan and Abigail when they got here after school yesterday.  She brought two “trach dolls” with her, and she, in a kid-appropriate conversation, explained to the kids what Hannah will likely be getting.  These dolls had real trachs and straps on them, and the kids got a chance to see what they looked like, play with the suction catheter just like we would be suctioning, etc.  They were able to keep them as well.

Abigail asked a lot of questions, very mature questions.  You can tell she has a medical mind.  I mean, what 6-year-old asks “So this trach will help her lungs do the work that her throat is not?”  Ethan didn’t ask anything.  He soaked it all up, but you could tell it was a bit uncomfortable for him.  They each got to keep their dolls to bring home.

ChildLife really helped us bring up the conversation to the kids.  Even if we end up not traching Hannah (which in my mind, I feel we are going to), they will have the exposure with it.

She is still on the vent.  She is still heavily sedated and very difficult to keep sedated.

Not much really happened today except that she failed her two-hour CPAP trial.  She made it an 1 hour 40 minutes before they stopped it because she was struggling to breathe.   She has been passing her 1-hour CPAP trials without any problem, but now we know where our area of weakness starts – whether it is due to the sedation or due to her throat weakness or combination is the question.  So the doctors decided to help her exercise her lungs more by bringing down the pressure settings on her vent requiring her to put more effort into breathing, which they did.  She has done fine so far in the 6 or so hours since that change has been made.

Aetna turned us down for the medi-flight to Vegas.  No surprised really.  The hospital is going to appeal this decision, but after what I have researched, it will be very shocking if we go through with it.

The more I think about it, the more I think we would like to have Dr. Roy do the surgery here in Texas, and that would require Hannah remaining in the TCH PICU for 7 or so days to recover from surgery.  The PICU team here in Texas already knows her.  Then, we think that the additional recovery time needed in the intermediate care plus regular room recovery could be done in Las Vegas.

Regardless, we have to fly Hannah to Las Vegas via a medi-ambulance of some sort.  Even if we spent the full month here, she will not be allowed to fly with a fresh trach, and there is no way in hell we can drive cross country with her (the doctors won’t even give that one a consideration).   The doctors say they think the flight is around $10,000, but my dad called me tonight to tell me he looked it up and it is around $25,000 or so because flights require a nurse and a paramedic.

We need to find a way to fly her (and me) to Las Vegas regardless of what we do.   How the hell this is going to work out, I have no idea.

My head hurts… I just want this over and bring her home, with or without trach, and start our lives over in Las Vegas.

She has been hospitalized 11 days now — ELEVEN!  She has been on the vent for 9 days now.   I want my baby back!!!!!

Today was actually a very quiet day compared to yesterday.   The Texas Children’s Hospital discharge planning staff was supposed to have a conference call with our insurance company today late afternoon to see if they would cover the medi-flight to Vegas (which requires a doctor and nurse, I believe).

Also, we are working on getting more information on this really tough decision we are faced with making.

I know that Dr. Bhakta, our pediatrician, talked to the fellow in charge of Hannah’s care this morning.  I was not able to find out from Dr. B what he got from it, but since Dr. B seems to always email late at night, hopefully I will hear from him (this man really goes all out for his kids).

I also hear from Dr. Roy, Hannah’s ENT, who has been in South America this week.  He was very adamant about not allowing any invasive procedures to be done until he got some more information.  So he was able to get his partner to come and evaluate Hannah today.  I am so pissed I missed him (I fell asleep in the RMH room), but hopefully he and Dr. Roy will touch base tomorrow.  Also, Dr. Roy will be back in the states this weekend, and he has offered to come to see her when he gets back into town, hopefully Monday (which I know is a holiday, so we will see!).

It works out timing wise, since we would not be able to leave for Las Vegas until sometime next week anyway, and since it is the weekend, they would not likely do a surgery until next week either.   Dr. Roy’s opinion is probably the most important one to us because he knows her better than any of the doctors here, and I think that if he said that she needs the trache, then I will feel much better about the decision.   Or maybe he will want to try another extubation attempt and give her another shot.

I keep searching for a sign that the trach is the right decision for her right now.  I think Dr. Roy’s opinion is going to be that sign.   And honestly, as selfish as it sounds, it sort of takes a lot of the pressure off us as the sole decision makers as to whether we are rushing this trach now or whether it is just time.   Even though we would have the ultimate decision, I know that he has Hannah and our family’s best interest at heart in this decision.

It is so hard to find doctors like Dr. Roy and Dr. Bhakta.  They are truly a gift to the medical world.

I just hate having her still remain on the vent.  I talked to the fellow today (well, many times a day), and he said that there is no harm on leaving her on the vent for the next few days.  The main issue is if she gets an infection due to the vent.  However, she is already on antibiotics for MAY be a bacterial infection from the vent already.

We just have to bide our time for a few more days and keep the Texas Tornado girl calm and sedated.    On a positive note, her pneumonia is improving.  The chest xrays are starting to look a bit better, and they are suctioning out less thick yellow junk each time (although she still has a lot of junk still in there each time).

What I would give just to be able to hold her…

It has come to the point where I wander these hospital halls at night going between the 3rd floor (PICU) and the 4th floor (RMH) in a total daze.   Almost like I am on autopilot.  It would be one thing to go down, and Hannah was awake, alert, etc., so that she could interact with me.   But no, I walk down the halls pretty slowly, knowing that I am going to be seeing my heavily sedated daughter in her crib, wrist restraints on, tubes and wires and leads everywhere, totally unresponsive to me.

It makes me very sad.  I wonder if she hears me.  Sometimes she gets very agitated because the sedation wears off, and then she does sometimes notice me, but then there is a look of “Help, get me out of here in her eyes.”  Not sure which is worse, heavily sedated or the look of wanting out.

I don’t feel like going into details about today because it will such a difficult and emotional day.  But long story short, the doctors had a meeting with us today and feel that Hannah will likely fail re-extubation because they feel her failure was more due to her neuromuscular airway weakness then the pneumonia.   They are willing to give re-extubation another try, but the more we have to extubate/intubate over again, the more dangerous it becomes.

Also, she has already been intubated 9 days now, and that is a long time.   She has also developed a bacterial infection in her vent line, so they started her on antibiotics.

They aren’t trying to rush us to make a decision.  But Daddy and I both saw how fast she failed reextubation.  She cannot cough.  She does not have the airway strength, even before she got sick, to clear her throat and cough.  She gags, and she hyperventilates.   Also, if she gets hit with pneumonia again, especially in the next 6 months when she is most susceptible for a relapse, it will be an even harder experience on her.

She is so incredibly weak now.  This virus/pneumonia (and we haven’t even dealt with the seizure issue yet) has really knocked her body out.  Her muscle recovery is going to take much longer than the average child because of her hypotonia.   I felt so bad that she lost the ability to pull to stand a few months back because of a cold at home — we will be lucky if she can even grab for a toy or keep her head up during tummy time for prolonged periods of time.

We met with the trach team this afternoon and went over what would happen if we went ahead with it.  Surgery, then a 7 – 10 day stay in the PICU, 7 to 10 day stay up in the PCU (midcare unit), and then at least a week or more in the regular floor recovering.  When she is on the recovery floor towards the end of her visit when she is all healed up, they would start Daddy and I on trach care education.

In the middle of our conversation, we asked how we would get Hannah to Las Vegas after this recovery period since airlines apparently don’t allow “fresh traches” on flights.    It was at this time, the trache education met with Hannah’s attending and fellow, and they all agreed it may be a good idea to medi-flight Hannah to Vegas before surgery and have the surgery and recovery in Las Vegas.  This way, the surgery team will already know Hannah instead of getting a child ‘post surgery’ to observe.

So the discharge planning nurse is looking into the feasibility of this option.  It would make things so much easier on our entire family.   If we do it here, it would just be me and Hannah here for a month without anyone because Daddy and the kids leave for Vegas on June 4th.  They can’t stay.  We have to be out of the house.  Daddy would fly back and forth, but that would be incredibly stressful.    If we are able to do it there, we would have family support — which would be huge.

I talked to Dr. Bhakta tonight, Hannah’s pediatrician.  He is going to call the attending doctor tomorrow and get full details.  He did not seem convinced that she needs a trach yet, but as he said, he has to know what is really going on.  I so wish Dr. Roy, Hannah’s ENT, was not out of the country this week — of all weeks to be in another country and unreachable by cell or email…

I hope tomorrow brings better news.  I am just really, really sad now.   I’ve been dreading this trach idea for almost 2 years, since she was diagnosed.  It is not just the physical trach itself that makes me sad… it is mostly that this is slap-in-the-face “I told you so” progression of her Gaucher’s disease.

It is that invisible hump that once we climb over, we are in a whole new world — the world of a truly medically fragile child, not a child with just some special needs.

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.”