Trach surgery most likely tomorrow

We are still in the PICU with Samantha (2 weeks and 3 days for those who are counting).  A couple of days ago they tried weaning Sam's vent rate down, and each time they tried, her CO2 levels went up.  At this time the doctors do not feel comfortable trying to remove the breathing tube.  Val and I have therefore made the decision to go ahead with the trach surgery.  The trach will give Samantha a ton more freedom that she has now, and will hopefully free up some energy that she had been using to fight to breath for the last month or so.

Currently Sam is an "add on" tomorrow and the schedule is light.  We assume the surgery will happen at some point tomorrow, we just are not sure what time.

Once the surgery is complete we will need to spend another 7 days in the PICU followed by another 5-7 weeks in a step-down unit, learning all of the things we will need to learn in order to take home a child with a trach.  Val and I are hopeful that our overachieving selves will become comfortable with trach care in no time, so that we can all be home again.

We never know what the future holds. Although Sam needs the trach now, there is always a possibility she may not in the future, depending on how things go with her trial drug.

In the meantime, we will continue to laugh and smile and play with our adorable little girl.  Her uncle Richie had her smiling and "Raising the Roof" today with her arms, despite still being on some generous narcotics.

Thank you again to everyone who has been so kind to us during this difficult time.

Until next time,

Monica

Brief update

Samantha's g-tube surgery went well yesterday despite being delayed until about 5 PM. She was just started on 1/2 feeds to see how she tolerates them.

Last night she had her central line in her neck removed. And while I am glad it is gone because there is always risk for infection, blood draws now mean extra sticks again for Sam. She is still semi-sedated due to the breathing tube, so perhaps she won't mind the sticks as much.

As for the breathing tube, the plan is still the same. They will try to wean her. If it seems she is unable to we will schedule a trach surgery for sometime soon.

As always, we plan on taking it one day at a time.

Until next time,

Monica

G-Tube Surgery Tomorrow

We are still in the PICU and will most likely be here for a little while longer. Samantha still has her breathing tube in place.  Sam's been extra sedated today as we have noticed that she has trouble sleeping with the breathing tube.  Her heart rate is a bit higher than we like, although we aren't sure why that is. When she is awake, she has a look of either disgust or fear on her face.  My feeling is that she is sick and tired of being in the bed, and in the hospital.  I can't say I blame her.

Tomorrow afternoon, barring any complications between now and then, she will be getting her g-tube surgery.  For friends and family who do not know what that is,  I like to think of it as a life line for Sam.  It will be a small little button in her stomach that we will hook up liquid foods to for Sam.  She isn't always able to eat or drink enough and therefore she has fallen off of the weight charts for her age/height.  In addition, we have noticed her choking more often (especially when sick).  Finally, kids with metabolic disorders need more fluids/calories than a typical child would.  All of these things combined equal a recipe for disaster without extra supplementation now and then.  Our hope is that Sam will still be able to eat by mouth (perhaps only certain textures) and we will use the tube for supplementation and her medicines.  I admit that getting her meds/supplements into her has not alway been easy. So, at the very least I hope she is happy about not having over a dozen meds forced into her mouth each day.

The current plan is for the breathing tube to stay in for at least several days after her g-tube surgery.  At some point we will try to remove the breathing tube and see how she does without it.   If Samantha is unable to do well without the breathing tube (or with something like bipap) we would then move toward something more permanent--a trach.  However, that is a few days, and many decisions away.  For now I'll just focus on her surgery tomorrow and hope it all goes well.

Until next time,

Monica

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

Struggling

Samantha has been struggling these past two weeks and we are not sure why.  It started with a change in breathing patterns while sleeping about two weeks ago.  At first all we noticed was the breathing changes. Although that is worrisome in and of itself (and we immediately alerted as many of her doctors as we could/set up a pulmonary appointment etc), the first few days it was going on she still seemed to be her typical self.

However, slowly but surely she has become more and more lethargic.  She has just about refused to be mobile these last few days (actually, I think she has refused outright the last two days but she did walk about 8 steps on Sunday night to get to her cousin). Not only has she not been walking, she isn't crawling either. She just sits.  She talks in a whisper constantly, and also seems to zone out a lot, especially after eating. 

Samantha hasn't been sick as far as we can tell. She has no fever or anything like that.  But something is obviously very wrong with our little girl.  Sam's still the same as far as cognition/language (with the exception of whispering rather than talking), so that is a positive.

We have a pulmonary consult tomorrow at CHOP to discuss the breathing issues. And, she has several more appointments coming up as well this month. Sam will also be getting a sleep study done at some point soon. Val and I just aren't sure what is going on or what we should be doing NOW, if anything.  It has been very scary to watch her go downhill so quickly.

The truth is that this may just be disease progression and there is nothing to do for now but wait and see if things improve again.  Or, perhaps she may need some type of assistance in terms of her breathing issues.  (And perhaps that will help with the obvious energy issues she is struggling with right now.)  Or, maybe we are missing something entirely and one of her many doctors will assist in a way we aren't even thinking of yet.

We just don't know.

Hopefully we will get some good insight during our appointment tomorrow at CHOP. 

Until next time,

Monica