Day 11 of PICU stay....

I apologize for waiting so long to update, but I was hoping that we would be out of the hospital much sooner and wanted to update when we were home. Unfortunately it seems it may be a while before we are home again. So, I will try to summarize as best I can.  (Please assume I am forgetting various things as it has been a long and stressful 11 days.)

In my last post I mentioned that Samantha was not doing well and we had a pulm  appointment for the next day due to a change in breathing patterns.  The pulm appointment came and went and a sleep study was recommended and scheduled for Sunday night. The pulm noted that Sam's lungs were clear and her o2 levels were fine. That was Wednesday.  By Friday I was growing increasingly concerned about Sam.  She hadn't walked since Sunday. She was lethargic. Her breathing continued to be labored.  And, she was having episodes of "zoning out".  I called both her metabolic doctor and neuro on Friday morning.  They both called and discussed my growing concerns and felt that an admission was necessary to get things sorted out.  In addition, her metabolic doctor felt that the PICU would be a better place to observe Samantha due to her rapid changes.   On Friday night we finally got a PICU bed. The next few days were spent bothering Samantha with various tests such as an EEG, a sleep study, blood draws and a bipap trial.  Sam also earned herself an NG tube for feeding when she choked in front of the speech therapist.  

The 24 hour EEG did not show seizure activity. The sleep study on Sunday night showed central apnea but they did not feel it was severe enough to warrant bipap at that time. Sam's lungs continued to be clear. Val and I were happy that there were no seizures and breathing support was not necessary. However, we were growing increasingly concerned about how Samantha was acting.  She was not herself, and was getting worse.  We weren't sure if it was the environment she was in, or if there were something else going on.  On Tuesday morning Sam woke up sweating profusely and her heart rate had been climbing up into the 150s.  Her blood pressure was 168 over something (sorry I forget the exact number, but it was HIGH).  Labs showed that her sodium level had dropped to 117, and the decision was made to place a line so that they could continue to monitor sodium while infusing large amounts of sodium into Sam.  The team attempted to place a line into her groin area but was unsuccessful.   They came out of the room and explained they would have to intubate Samantha in order to place a line in her neck.  

Despite her sodium levels going up, Samantha continued to struggle after Tuesday. Her blood pressure was controlled with medication, but her heart rate was slowly climbing.  She was refusing sleep.  She was not herself and was not very interactive. Sam seemed miserable and out of it and we were not sure what was going on. By Thursday night her o2 level was dropping despite her lungs still being clear.  She was still fighting sleep but her sats were going up with the o2.  First they used one liter, then two.  By Friday morning she needed bipap and the team had a discussion with us about what we wanted to do if she were to go into respiratory failure.  We let them know we wanted them to insert a breathing tube if necessary to save her.  For a few hours she seemed to do ok on bipap.  But all of the sudden, her o2 sats dropped dramatically (the lowest I saw was 12) and the team went into action to intubate her as she went into respiratory failure.  The intubation was successful and finally Sam was able to get some rest on Friday after not sleeping for days.

On Saturday Sam finally woke up from the sedation while still intubated and it was the first time I saw "Sam" in days.  She seemed to be herself despite being slightly sedated and unable to talk. We had as good a weekend as you can have while intubated.  She was communicating by waving/raising her hand when she wanted something.  It was very nice to have Sam back with us, even though she was breathing through a tube.  I was very happy to know that her previous change in cognitive status was a result of her exhaustion earlier in the week.

Val and I have noticed as the vent settings are lower, Sam seems to be losing strength/interest again. But for now the goal is to try to remove the tube and see how she does.  Her numbers on the vent are good enough to remove the Breathing tube, but the team is waiting to see when her g-tube surgery (we feel a g-tube will be great for Sam going forward as it will allow her to get nutrition/supplements when needed and help with her energy/weight issues) will happen before they make a decision to remove it.  If the surgery will be tomorrow, the tube will stay in today.  

Needless to say that when we entered the PICU on Friday we did not suspect Sam would be going into respiratory failure the following Friday.  We are very grateful to the PICU team at for saving our little girl.

Throughout this stay, our friends and family have been amazing and wonderful.  Both Val and I have large families, and I suspect that Sam has had more visitors than anyone else in the PICU. Our laundry is getting done. Food is being delivered.  We know that if there is anything we need, a simple phone call will have it to the hospital within hours. In addition to family, many, many friends have offered support in a variety of ways.  It has been nothing short of incredible and Val and I are so very grateful.

So, thank you to everyone. 

At this time things with Samantha continue to be uncertain. We suspect her breathing issues are a result of disease progression, but we are not certain if it is something she will bounce back from or if she will need ongoing respiratory support.   Val and I will utilize any and all medical interventions to get Samantha stable and home as we feel cognitively she is still very much with us.  We suspect we will be in the PICU and the hospital for a while longer. But, obviously we hope our stay is as short as it can be.

I will update more when I can.  Thank you again to everyone who has offered assistance, calls, notes, texts, emails, thoughts, kind words, and prayers.

Until next time,

Monica