How We Got Here and What We Know

Disclaimer: This is written on about 2 hours of sleep and 40 cups of coffee since Sunday morning (it's now 11pm Monday night), so cut me some slack on the typos, okay?

I knew the weekend was going to be a long one after getting a report from Jack's nurse, Kristi on how his Friday went.  Jack's episodes of distress where his heart rate skyrockets and his sats tank have been happening more and more frequently over the last few months.  We know he has a new stone in his kidney and we know he has issues with being impacted.  We keep finding ourselves inpatient to deal with these issues and, yet, Jack's episodes of distress haven't really let up.

All hell broke loose starting Saturday morning during Jack's vest treatment.  About half way through Jack's treatment, he had an "episode", so I stopped the treatment and proceeded to suction Jack.  The suctioning didn't help and his sats continued to drop.  I grab the ambu bag, turn on the oxygen tank and bag him until his sats go back up and the color returns to his face.  I skip the rest of the vest treatment and give him about a half a can of Pediasure.  I turn on his TV and step out of his room to go do something else. It had to be no less than 15 minutes when I hear his pulse-ox alarming low sats again.  I go back into his room and the color is fading from his face again, he looks distressed and his sats are dropping.  I bag him again until I get his numbers to an acceptable level.

The dropping sats, distressed Jack and need for bagging at least once every hour continued throughout the day. Mark and I were convinced that Jack still had bowel issues because we didn't believe they got him completely cleaned out either time he was inpatient to deal with the issue.  Believing it to be an impaction issue, we didn't think there was really anything we could do to help Jack except continue with the Miralax routine prescribed by the GI doc and just continue to support him through the periods of distress.  However, by late Saturday afternoon, I was a little worried because we had never had to bag Jack so continuously to make him comfortable. Maybe once or twice a day, but certainly not all day long.  I decided to increase the rate on his vent significantly and to give him continuous oxygen bled into his ventilator.  I was able to keep his sats up by doing this, but it was clear that Jack's distress was not respiratory, it was pain related.  I also decided to give him a dose of Lortab, which I had been avoiding because it would only contribute to the constipation issue. As much as I hated to admit it, I needed to take this kid to the emergency room because he was really hurting and I couldn't help him.

After having made the decision to take him in, it hit me that we didn't have our van because Mark took it in for service prior to our upcoming trip to California.  It was after 5pm and the shop was closed until Monday.  We don't own another vehicle that will transport, or even hold, Jack's wheelchair and Jack.  I was stuck.  The only choice I had was to call 9-1-1.  I really didn't want to make the call and was trying to come up with something ... anything that I could try to make Jack comfortable and avoid taking him in.  All the time hearing the pulse-ox alarming and knowing that Mark was bagging Jack.  So, I bit the bullet and dialed 9-1-1.

"9-1-1, what's your emergency?"

"I have a 13 year old son who has muscular dystrophy, is trached and ventilator dependent and he is in respiratory distress.  I need to get him to the emergency room, but I don't have a vehicle to transport him. I don't need the flashing lights and sirens, I've got things under control, I just need a ride."

"Ma'am, if you have a child in respiratory distress, you are going to get the lights and sirens."

Well, crap.

I didn't have too much time to worry about what the neighbors would think of all the commotion because within 2 minutes there were five fire department personnel at the door.  Mark escorted them to Jack's room where I had taken over bagging him. I explained to them what was going on and said we just needed a ride to Phoenix Children's.  Wisely, they didn't feel the need to do a whole lot to Jack other than get an EKG strip.  They asked a lot of questions, but pretty much took their lead from us. They loaded Jack in the back of the ambulance and told (requested) me to ride in the back with Jack.  They said they don't usually let parents ride in the back with their kid, but they'd really appreciate it if I did.

By this time, the Lortab had kicked in and Jack was a little less distressed.  He was hooked up to  oxygen, so his sats were good.  I'm looking at Jack thinking "Really? REALLY?"  You make me call 9-1-1, take a 30 minute ambulance trip down to PCH and your numbers are all good.  Kid, you are killing me!

PCH wasn't terribly busy, we were seen by a first year resident who was 12 years old.  (Really, she looked 12 years old, I'm not kidding!)  I hand her Jack's 3-page history that I keep current and take with me every time we see a new doctor or visit an emergency room.  I tell her what has been going on.  After conferring with the attending (I'm assuming), she orders a KUB.  After the KUB comes back "clear", we talk about our concerns and she agrees to order the CT that I wanted before our "clean-out" discharge earlier in the week.  The report gives us the same findings we always get - kidney stone.  Nothing new.  The resident looks at us like, what do you want to do? I told her that admitting us won't bump up the surgery to have the stone removed.  Been there, done that.  It's 1am and I just want to get home.  Of course, we need an ambulance ride home.  I can only imagine what this evening just cost our insurance company.  We get home around 2am and I pump another dose of Lortab into Jack and just hope he will get some sleep.  Then I think about what just transpired and I'm not happy.  Why didn't they do any blood work, get a chest x-ray or run any other tests?  I'm more mad at myself for not asking "why" when we were at the hospital.

Jack's Sunday pretty much matched his Saturday, except by Sunday afternoon, I could tell his pain was unbearable and I couldn't stand to watch him suffer any more. I decided to call PCH and find out who was on call for urology, hoping it would be Jack's urologist because I needed to hear from him whether or not he thought it was the stone causing Jack all this pain.  I felt like the stone was a red herring and everyone jumped to that as the source of the pain because it was an easy find.  Thankfully, Jack's urologist was on call.   He called right back, said he'd take a look at the CT and KUB that were just done and compare to the previous ones and call me back.  About an hour later, he called and said that the stone looked stable and he didn't think it was the source of the pain.  We needed to take him back in.  Two trips to the ER in a 24 hour period - you know I had to be worried to agree to that.  The urologist called the ER and gave them a heads-up and he told me not to let them discharge us to home this time.

I was NOT going to take another ambulance ride down to PCH. So, I asked our friends who have one of those big, four door horse trailer pulling trucks if they would drive us down to PCH. We put Jack's wheelchair in the back of the truck and laid Jack down in the back seat with his head on my lap.  And, no, he wasn't seat belted in (nor was I) because there was no way to do it.  It was Sunday evening and the ER was packed. I knew exactly how to get us right back.  We put Jack in his wheelchair and unhooked the oxygen from his vent.  I knew his sats would drop pretty quickly (but also knew we were steps away from the ER doors and an abundance of oxygen).  I rolled Jack through the doors bagging him and his color wasn't looking good. I looked right at them and said "this kid needs oxygen".  They took one look at Jack and back we went and, in a flash, we were surrounded by people.

This time around, they did an array of tests and the plan was to admit him.  I told them I wanted to be admitted to the PICU.  The ER doctor said he didn't know if that was possible.  I made it clear that the PICU was the only place I was going to go (and knew that if I needed to, I'd get "my people" to talk to "his people" and make it happen).  They got Jack a bed in the PICU.  No need to get the "peoples" involved. Yay!

Are you still with me?

The entire time we are in the ER, Jack is clearly in extreme pain.  He is grimacing, his heart rate and blood pressure are high and, despite being on a vent, he is taking really shallow, short breaths.  I have never in my life seen Jack in this much pain and it was relentless.  They got him settled in the PICU, but hadn't yet done anything to address his pain.  I forced myself to call the Intensivist into Jack's room when he walked by and asked him if we could talk about code status.  In all of Jack's hospital admissions, we have never thought about or brought up the issue of whether Jack was a full code (which, made him a full code).  But, it's something Mark and I have been discussing and we've made certain decisions about how far we want them to go if Jack ever codes.  Articulating our wishes for the very first time was extremely difficult.

We got to the PICU around 9pm and it had been since at least 3pm that Jack had been suffering immensely with pain.  Something had to be done now.  They started off giving him Tylenol, which did not make me happy because I could do that at home.  I asked them if they expected him to continue on like this through the night?  It was unbearable for me to watch and, quite frankly, had me in tears.  They transferred Jack over to the hospital vent and gave him some morphine.  They also checked his blood gases and his CO2 came back at over 100! (normal is 30-40s.)  At this point, they significantly increased the vent support and around 2am, Jack finally fell asleep.  Last night was by far, one of the most brutal nights for both me and Jack since he was born.  I couldn't sleep and have yet to sleep because the stress was so intense.

So that's how we got here and here is what we know based on my conversations with a very knowledgeable PICU Intensivist:  Jack has a significant urinary tract infection and has probably had it for months. The urinary tract infection causes pain and slows down the GI tract - which is why Jack is having issues with constipation/impaction.  The constipation/impaction causes pain.  The pain from the UTI and inflamed GI tract results in suppressed breathing.  In other words, the pain is so bad, Jack is essentially holding his breath to get through it.  The suppressed breathing caused elevated CO2 and is also why Jack needed so much bagging.  This all makes sense to me and explains why even though Jack would get "cleaned out", a week later he would be full of stool again - because his gut has slowed down and is not moving things through.  The Intensivist was the first person who was able to put all the pieces of the puzzle together and explain how everything going on with Jack was interrelated.  As he told me, the specialists only focus on their area of expertise, while he focuses on the entire child and all that is going on when making his assessment.  His approach was very logical.

We now have a PLAN that makes sense to me.  Treat UTI + stay on top of pain before it gets out of hand (with non-narcotic pain meds) + help move things through GI tract (with Miralax and laxatives) = normal breathing while we wait for surgery to remove the kidney stone and find out why the stones are forming so we can prevent them from coming back.

Jack is still having periods of pain and is not tolerating any food in his stomach yet.  We need to get the UTI under control and, as of now he is on a broad spectrum antibiotic until the culture comes back and gives them the sensitivities.  They are weaning down the vent settings because now he is over ventilated (his CO2 was in the 20s), but he is still on the hospital vent.  They are giving him IV Toradol  on a regular basis to deal with pain. He'll need to stay on antibiotics prophylactically to prevent a recurring UTI until he has surgery to remove the stone (or, maybe even longer).  I'm guessing we are here at least through Wednesday.  I'm still hoping we can make our trip to California over Labor Day weekend for the Disney 1/2 and mini-family vacation.  If we can get Jack comfortable, there really is no reason he wouldn't tolerate the trip. In theory, he should feel better than he has in a long time.

It's now 1:30 am on Tuesday and I've been awake for too long - I am fading fast.  Hopefully, this amount of detail was worthwhile and digestible.  Summing up Jack in a few paragraphs is impossible.  Thanks for checking in, thanks for reading and thanks for caring about my sweet Jack!  We love you all.