Where Are We?

I've started the first sentence of this post about ten times already and can't seem to go anywhere.  I'm so tired I can't think, but I want to give you all an update.  As I sit here trying to compose something, three feet away the nurse practitioner is poking all over Jack with a needle trying to get a line in him for the third time today.  He lost two lines today.  They've exhausted the feet and hands, they are now going for the chest.  The day Jack was admitted, the IV team suggested that they order a PICC line. Apparently, no one listened and Jack is suffering the consequences.  He's now on IR's schedule tomorrow for a PICC line.

Anyway, here's where we are.  Yesterday evening they decided to start Jack back on his regular feeds, with the hopes of discharge today.  We usually just pour the entire can of Pediasure in his feeding tube, so they did the same.  Mark was here for the evening feeding and he said that Jack seemed uncomfortable when they fed him, but he was tolerating it okay. After I got back to the hospital later that night, the nurse gave him another can.  Within minutes of giving him the can, he started showing signs of being in distress again.  He was crying (Jack almost never cries), grimacing, turning red, heart rate going up and sats going down. As I've described it before, it looks like the pain comes in waves and it is relentless. I asked the nurse if she could pull the food back out of his stomach to see if it would give him some relief.  While she was doing that, Jack's heart rate shot up to over 200.  The highest I saw was 220.  I haven't seen numbers that high since Jack was a baby.  The nurse was really studying the monitor while this was going on (I now know she was paying attention to the heart wave).  His heart rate then dropped down to 100 pretty quickly and then went back up.  At this point, she listened to him with her stethoscope and called the resident. I'm looking at Jack and he is still crying and he's clamping down and not breathing well and the monitor is alarming for all kinds of reasons. The room filled with people and a bunch of tests were ordered "stat". From what I could decipher, Jack was in ventricular tachycardia.  They gave him several meds, including ativan and he eventually settled down and his heart rhythm returned to normal. Also, earlier in the day they had switched him over to his home vent as another step towards discharge. During the event with his heart, they put him back on the hospital vent.  I asked the resident this evening exactly what happened to cause the v-tach and he said that Jack was hypo-ventilating (due to the pain) and he became acidotic and that's what caused the heart arrhythmia. 

Needless to say, I couldn't sleep after watching all that transpired. Thankfully, Jack feel asleep with the ativan on board. Too bad it wasn't a buy one, get one free deal, because I could have used some ativan myself. 

This morning during rounds the plan was to start Jack on his feeds more slowly using the feeding pump.  I can tell you, I knew it wasn't going to make any difference slowing the feeds down, he wasn't going to tolerate them. But, it's not as if they were going to take my word for it.  They started his feeds with the intent of giving him one can over two hours.  I think he got about a twenty minutes into the feeding and it started up again - the pain and everything that goes with it.  I asked Jack's nurse to get the resident and Intensivist in the room so they could observe what was happening.  They stopped the feedings and ordered a KUB.  The Intensivist called me over to look at the x-ray and it showed that Jack was still impacted in the middle part of his colon. (Yet, he'd just had a KUB in the ER two days earlier and nothing was noticed and, he was supposedly all clear at the end of his last admission less than 10 days ago.)  The plan - do another clean-out.  Been there, done that and it hasn't resolved the problem yet.  I'm becoming very irritated with this plan and I'm sure Jack is pretty darn sick of it too.  Regardless, I don't get to write the orders.  So, we are doing clean-out number three.  After they think Jack is cleaned out they will get (yet another) KUB to make sure.  Then what?  I'm going to make sure that he stays in this place for at least 24 hours, maybe more, to make absolutely sure that Jack tolerates his feedings once they are resumed.  Every other time we've gone home after a clean-out, Jack is good for about a day or two and then the pain rears its ugly head little by little until it reaches the point of no return - which is the point where Jack's breathing is affected and we are back to bagging him again.  Do I think the third time will be the charm? No. My gut tells me there is more going on, we just haven't figured it out yet.  

Another thing that I observed, as has the Intensivist, is why can Jack tolerate the GoLytely (the clean out solution), but not his food?  If it is a matter of his intestines being inflamed and irritated because of the UTI and/or impaction, he should be just as uncomfortable with the GoLytely as he is with his food.  Is it possible that Jack has developed a sudden intolerance to his formula?  I think we need to get GI involved and I think they will if, after this clean out, Jack continues to decompensate when he is fed. I really have no idea where we are going with this. But, if it is as simple as a clean out, then, in my opinion, it would have worked before.  I get that we were dealing with an undiagnosed UTI with the prior admissions. However, the Intensivist also wonders why, with the UTI resolved, is Jack still having all the GI problems.  

Jack is just complicated because his body is a complicated mess due to the progression of his disease. I always request copies of the written reports from all of Jack's tests that he has done here at PCH.  I received the reports from his last stay in the mail yesterday.  Reading through the various reports and the description of Jack's body from a clinical perspective, it really hit me that we will never get Jack back to 100%.  At this point, it's more a matter of keeping Jack pain-free and comfortable - basically, palliative care.  It puts making the trips to St. Louis in a new light - not that I'm going to stop taking Jack to his St. Louis doctors anytime in the near future, but it's food for thought.  In any event, right now we have our work cut out for us with the kidney stone, UTI and GI issues and I just hope and pray that we can get these resolved here and now with this admission because Jack is tired ... and so am I.

It's 1:30am again as I finish this post and I wonder why I'm so tired I can't keep my eyes open during the day?

Thanks for all the love you share with your comments here and on Facebook, and for your texts, emails and phone calls.  It's the most awesome feeling to know that my Jack is loved by so many.  I'll close with part of an email I received from a friend of mine who also takes care of Jack as his nurse on a PRN basis:

"I love that kid and it breaks my heart that he's going through this.  There is something so deep about Jack that I love and he is so much fun to hang out with."

There truly is something very "deep" about Jack that is hard to explain, but you can't help but feel it when you take the time to be present in his presence.

Love ya'-

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.

As Expected

As I expected, the GI doctor wasn't going to fit Jack in his schedule, so off to the ER we went after I finished up a couple of appointments Thursday morning at work.  I told the ER docs to look at Tuesday's CT scan that was in the system and that we were here to be admitted to get the issue resolved.  I think the ER Attending spent all of 10 seconds in Jack's room (and will no doubt bill us $1,000 for his services) and we were a pretty quick admit to the floor.  They started the same routine they did last time and Jack pretty much had the same results as last time.  I went head-to-head with the Attending (Hospitalist) on the floor because I wanted a CT to confirm that this time Jack was cleaned out. The Attending took the position that because it's been a month since Jack's last admission, this was a new episode and that as long as the KUB showed he was cleaned out, that was good enough. However, because I persisted, he called in the GI doc to back him up and I asked the urologist to back me up.  (As a side note, the Attending told me that Jack's GI  doc was a friend of his, so he called him and asked him to come talk to me.  I had to bite my lip not to say how considerate it is of the GI doc come to talk to me about HIS own patient just because the Attending asked him to.  At PCH, the GI docs don't follow their patients when they are admitted, they turn the care over to the Hospitalists.  On the other hand, the urologist was there because he heard Jack got admitted, so he stopped by to check on him and talk with me. This, even though Jack wasn't admitted for a urologic issue.)  In the end, we both compromised. I didn't get my CT, but they did do something called a Gastrografin enema in radiology which, they assured me, showed that Jack was all cleaned out.  I can tell you as I sit here today, I'm skeptical.  Trust me, you don't want all the details of bowel clean-outs, but based on the results I saw, it's simply not logical that all the impacted stool cleared from Jack's bowel.  One of us is wrong and I'm just hoping it's not them because Jack will be the one suffering the consequences.  Time will tell ... once again.  Jack was discharged 24 hours after he was admitted.

Jack's urologist got us in to see a new nephrologist in the group.  When I looked her up, I saw she did her residency at St. Louis Children's Hospital.  I already like her! :)  We are scheduled to see her mid-September.

We also have Jack's next surgery for the kidney stone removal on the books.  Originally, we talked about having it done the first week in September to give Jack time to recover before our trip to St. Louis. But, I really want to get a second opinion from the St. Louis urologist, so I asked our urologist here if he thought it would be a problem waiting until we got back from St. Louis for the surgery. He  didn't think it would be a problem because the stone isn't blocking anything.  We are on the schedule for the third week in October for the surgery.  After my experience this time on the floor and dealing with a condescending Hospitalist, I reminded the urologist that I again wanted Jack to go to the PICU following surgery, not the floor.  He agreed.  I absolutely will not deal, post-op, with physicians who are on power trips and, for some reason, there seem to be an abundance of them on the floor.

It's been an absolutely crazy year with all these hospital admissions.  I feel like I did Jack's first year of life when he bounced in and out of the hospital many times and no one could seem to just fix the problems that kept landing us there.  You'd think by now I'd accept that doctors don't have all the answers and they can't fix all the problems - especially when it comes to Jack.  It's still beyond frustrating at times.

I'm just hoping the next few weeks are calm.  In two weeks, I'll be joining Team Cure CMD for another half marathon. This time it's the Disneyland half.  We are all going over and we'll spend a few days at Disney after the race.  If nothing else, Jack will ride Small World (at least 10 times with me, hehe) because I know that ride is wheelchair accessible and I know he'll love it!

That's the update from here.  I'll leave you with a few pictures from our stay at Phoenix Children's.

Looking out the window of Jack's room at sunset.  

(iPhone camera with an HDR filter)

The next evening - looking to the Northeast out a window on the 6th floor

Jack happy to be bailing from the hospital

CT Scan Update

The urologist called me today regarding the CT scan.  Where do I begin?

First we talked about the appointment with the nephrologist yesterday and I shared with him that I didn't think the guy was a good match for me or Jack and told him why.  I asked whether it was possible to switch doctors within the group and told him who our pulmonologist recommended.  He said that it is not a problem at all, that it happens and that it's okay to say that it's not a good fit.  The guy our pulmonologist recommended is also the same guy the urologist has spoken with in the past regarding Jack.

We then talked about the kidney stone, which has gotten larger since the CT in June. It is now 1.7 x 1.6 cm in size.  He discussed all the options for treating the stone, but based on its size, he suggests the same procedure he had last time, the percutaneous nephrolithotomy (another 5-day minimum hospital stay).   I told him we were going to St. Louis in October, so if we are going to have this done, it needs to be done soon or we'd have to wait until we got back from St. Louis because we don't want to travel with Jack right after surgery. (I didn't share that I might just wait until after we see the St. Louis urologist to make a decision.)

Finally, he said that the CT showed that Jack is still impacted with stool!  He said he measured the amount of stool and there is 6-7cm of stool in the colon.  It's near the rectum and it's pushing on his bladder  - which explains why Jack doesn't tolerate his wheelchair. When he sits, he hurts and it's reflected in his sats (low) and his heart rate (high). We knew back in July after the inpatient "go-lightly" treatment that the stool was not out - regardless of what the KUB supposedly showed.  It was obvious to us that the issue was not resolved, but x-rays don't lie, right? (wrong!)

After we talked through all the above issues, the urologist said (1) he would call the other nephrologist and see if he will see Jack asap; (2) he would call one of the general surgeons regarding the bowel issue because at this point, he said, it's more than likely a surgical issue, not a GI issue; and (3) he would check his schedule and see if he can get Jack's surgery scheduled within the next three weeks.

I am now sick to my stomach.  My heart just aches for Jack and all that he has to put up with because he can't tell us how much he hurts.

A half hour after the urologist first called me, he called again to let me know he talked with the other nephrologist and, unfortunately, the guy is having surgery of his own tomorrow and will be out for two months. But, he did recommend a couple of other doctors in the group who would be aggressive in dealing with Jack's issues. The urologist said he will call tomorrow and see if he could get us in to see one of them.  He also said that he got ahold of one of the general surgeons who was able to pull up Jack's CT. The surgeon told him that it's bad, but not the worst he's seen.  He suggested a couple of things from doing mineral oil enemas to having GI do a colonoscopy.  I told the urologist that at this point I am not wasting time dealing with enemas. This needs to get resolved now and that I want the colonoscopy done asap.  The challenge is getting GI to see Jack.  Based on my previous experience with them, they don't really give a rip if you have an emergent issue, they will not fit you in their schedule.  The urologist did say he would give them a call, but he doesn't know if it will help.

My plan is to call the GI first thing in the morning and ask them to let the GI doc who saw Jack in July know what is going on and ask him to please review the CT.  Let him know that this needs to be dealt with now and that, based on the recommendation from the surgeon, I would like a colonoscopy to be scheduled asap.  I will let them know that I need to know what the GI says by noon because if he refuses to help us then I'll have to go with Plan B.  Plan B is to take Jack to the ER and get him admitted for a colonoscopy via the ER.  I'm fully prepared to leave work tomorrow afternoon and head to the ER because I absolutely refuse to allow Jack to suffer with this any longer.  I fully expect for GI to not help us on an outpatient basis, but one can always hope for the unexpected.

Kuddos to our urologist who has gone above and beyond by calling the nephrologist and the general surgeon to try and get Jack some help.  He's a great guy who really does care - which makes me feel a little guilty about considering having all this dealt with in St. Louis.  But, I suspect if we end up going that route he would understand.

I just want to get the GI issue dealt with first and then make a decision regarding the kidney stone.  You know, most people hear Jack has a kidney stone and they think no big deal. But, this is a big deal.  It's not the kind of stone that will pass. It requires a fairly major surgery to get it out and for a kid as compromised as Jack - surgery is a big deal on so many levels (not the least of which is the avoidance of breaking his bones in the process).

In any event, I think I've rambled on enough.  The news from the urologist has me overwhelmed, mostly because I'm already overwhelmed with work deadlines and with every day life.  I think I may need more than a few drinks to get me through the next few weeks (months) - a lot of chocolate is also in order.

Wish me luck tomorrow - it should prove to be a challenging day to get people to think outside the box to get Jack the help he needs.  I'll have my "Kindness" Touchstone held firmly in my grip to remind me to be firm, but kind.

Stay tuned...

And You Wonder Why I Drink?

I don't like to whine.  But, I left the house at 7:45am this morning and finished up with all of Jack's appointments at 5:15pm and it's freaking 115 degrees outside.  So, if this sounds whiney, blame it on the heat.

We saw the nephrologist today.  I didn't have high expectations and I was not disappointed.  The doctor was nice enough, but I left the appointment without having any confidence in him and the likelihood that he will help us with the "why" and "how do we resolve the problem" of the kidney stones.

Let me just take you through my day - because if you are reading this, then you must be interested in the minute details of my day, right?

Our appointment with the nephrologist was at 9:30am. I managed to schedule the CT scan that the urologist ordered for 10:30am.  I was instructed by the radiology scheduler to go to radiology and get registered before our appointment with the nephrologist so that we would be good to go once we arrived. We got on the road early and thank God for the commuter lane, arrived at Phoenix Children's by 8:45am.  When I signed in at the front desk and told them we needed to register for a CT scan with radiology, she looked at her "list" and told me we weren't on the "fast track" registration and that I needed to start with admitting.  Not what I wanted to hear.  Not what I was willing to do.  I informed her that I was told to go to radiology and register and I was not going to go to admitting.  She didn't argue with me. It is never my intention to be obnoxious, but darnit! I don't have the time or the energy to mess around doing things that I shouldn't have to do and I was not going to waste 20 minutes sitting in admitting to then go to radiology, especially because they have all our insurance information from the  multiple times we've been to this hospital in the last 5 months!  We were able to get registered at radiology and then head over to a different building to see the nephrologist.

From the get go, it was clear that the nephrologist was not comfortable with Jack. I had received a copy of the lab report from Jack's urologist, so I knew what all his numbers were and thanks to PubMed, I had a list of questions and ideas of what I was hoping the nephrologist might want to investigate as far as what might be causing the high calcium oxalate and low citrate.  I went in with an open mind willing to hear whatever this physician had to say.  Problem is, he didn't have anything particularly enlightening to say.  He discussed the results of the 24-hour urine collection - which I already knew because I had a copy of the report.  He suggested it might be due to his diet - contrary to what Jack's urologist and pulmonologist both said - they said because he is on Pediasure, it is not a dietary issue.  It really isn't worth repeating the play-by-play of the appointment, but I will say that he mentioned that he could put him on a citrate supplement to address with low citrate in his urine (citrate is what helps to keep stones from forming), yet we left his office with no prescription for citrate.  I asked if he might want to do some blood work - which he agreed to (but why did I have to suggest it?) and then he checks a bunch of boxes on the lab form and asks me if there is anything else we might want to check.  I suggested checking his Vitamin D level based on my discussions with Jack's St. Louis neurologist last week and his response was "that's a good one, we were just talking about that last week." Who is the doctor here?!  Really, I have no doubt this doctor is well educated and knows his stuff, but for Jack - he is not the right guy.  We left with no information and no plan.  Maybe when he calls me after he gets the results of his lab work, he'll have a plan.  But, again, I'm not optimistic.

Next stop was radiology and the CT scan. We got right in, right out and it was uneventful.

After CT, it was off to the lab.  Maybe I'm expecting too much, but PCH just built a brand spanking new Children's Hospital and you'd think along with the new clinics, facilities and offices, they would have nice big signs that direct you to where you need to go.  We were told the lab was on the second floor next to the clinic suites.  We arrived on the second floor and there was no sign that said outpatient lab anywhere.  I asked at one of the clinics where the lab was and was told "next to the bathrooms".  Nice.  We went around the corner to the bathrooms and saw a door with a small sign next to it that said "blood draws".  Right! Of course, the outpatient lab should be a closed door next to the bathrooms with a tiny sign that makes reference to blood.

The lab tech asks me where are they usually able to get a good blood draw on Jack.  I tell her that it usually takes the IV team to get a blood draw, but the best place to look are his feet.  To which she informs me that she can't draw blood from his feet that only the IV team can and if they can't get blood and need the IV team, then we have to get an order from the doctor who ordered the blood draw to request the IV team.  ARE YOU KIDDING ME? This is a Children's Hospital, right?

I won't even expand on my thoughts about the whole blood draw thing because it wouldn't be nice.  Fortunately, they were able to get blood from Jack's hand.  It took forever for him to bleed enough to fill all the vials, but they got what they needed ... I hope.

It's now noon and we are finally done at PCH. After I pull into the driveway at home, I hear Kristi say, "we have a problem".  I look at Jack and he is pale/gray and looking quite out of it.  #$%@!  I tell Kristi we need to bag him.  I grab the ambu bag and Kristi grabs the suction machine.  After a few minutes, we "revive" Jack.  The way our van is set up, Kristi sits in a seat behind Jack, so she can't see him when we are driving.  Not a good set up, obviously.  We don't travel with the pulse-ox and it's never been a problem, but apparently, we need to keep a better eye on Jack when we are driving around town with him.

We were home a few hours and then back on the road for an appointment with the pulmonolgist - who we were supposed to see in May to follow-up on the change over to the new vent but never did see because we've been dealing with kidney stone issues and hospital admissions and I've already missed a ton of work so there really was no time for a follow-up appointment in my schedule.  But, I like our pulmonologist - he totally gets me and he is so good to Jack. Knowing that we had multiple appointments today, I just bit the bullet and decided to take the day off and fit the pulmonologist appointment in.  I brought him up to date on the kidney stone saga and my thoughts on the appointment with the nephrologist today. He was understanding and supportive and gave me some ideas and also gave me the name of another nephrologist in the group we might want to see. He also suggested that we might just want to wait and have this dealt with in St. Louis.  I told him the problem is that I know too much. His response was that I can't ever know too much, that I have to stay on top of things to be the best advocate for Jack.  Like I said, he totally gets it.

So that was my day in not-so-much-a-nutshell.  I'm frustrated.  I want answers and I want a resolution to the kidney stone saga.  Is that asking too much?  I'll wait to hear from the urologist regarding what the CT shows and then I'll forward all the information I have to the St. Louis urologist and depending on what he says, we'll have to make the decision as to whether this issue will be resolved in St. Louis or Phoenix.  It's a difficult decision because this appears to be a chronic, ongoing issue.  Not something that is really conducive to appointments with doctors 1500 miles away.

And, my friends, you wonder why I drink?

The Saga Continues

I talked to the urologist today regarding the report from the lab in Chicago that analyzed Jack's urine.  According to the report, Jack has "extremely" high calcium in his urine, low citrate and lower than normal volume for a 24 hour period.  The question is "why?" That's where the nephrologist comes in.  For some reason, I'm not all that confident that we will get any answers to the "why" other than, "we just see this sometimes" and then they'll set about trying to treat it.  Call me a pessimist.  You'd be right.

We see the nephrologist next week.

Removal of the new stone that has formed still remains an issue.  The end of June, the stone was already 1.5-2cm.  Keep in mind that the majority of stones that send people screaming to their local emergency room are measured in terms of mm.  We have to get yet another CT scan to see just how big the stone has grown and then the urologist suggest going in again via the percutaneous nephrolithotomy to remove the stone.  Another big surgery, another 5-day hospital stay.  The urologist mentioned that we could try lithotripsy, but because of its size, it would require multiple treatments and he's not sure it would break up the entire thing.  I agree that the more extensive procedure is the way to go because I just want the thing out all at once.

More than one doctor has told me that the stone shouldn't be hurting Jack because it's not trying to pass through anything, it's just sitting there in the kidney.  I'm not convinced.  Our bodies do not like foreign objects in them and while Jack may not be in excruciating pain at the moment, I can tell he doesn't feel well.  He hasn't felt well for the majority of this year, and it's breaking my heart.

In the scheme of things, I realize things could be much worse than a kidney stone.  But, the fact is - there is very little I can do as Jack's mom to make a difference in his life given his disease.  I can't stop the disease from progressing, I can't do anything to help him walk, talk, eat or breath on his own.  The only thing I can do - and the one promise I made to him six years ago after his spinal fusion surgery, was that I would do everything in my power to make sure he doesn't hurt. Seeing what Jack went through with the spinal fusion surgery brought me to my knees and I vowed to protect him from having to endure that kind of pain ever again.  It's why I'm wicked protective when I turn over his care to anyone else.  I've not been successful in keeping my promise this last year and it makes me sad.

I hate that Jack has to endure another procedure and, to be honest, I'm losing confidence in having this dealt with locally.  As his urologist reminded me today, things didn't go as planned last time because they perforated his kidney.  Shit happens, I get that. But, what if it happens again? I'm once again struggling with whether to have this dealt with here or in St. Louis.  I hope to talk with the St. Louis urologist before our appointment in October.  Depending on what he says, we might just wait until we are in St. Louis to do anything about the stone.  I'll just have to take my cues from Jack as to when, where and how we move forward.

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Speaking of Jack's spinal fusion surgery, it's hard to believe that it's been six years since the surgery (on July 28th).  More unbelievable is that I have been blogging for six years.  That's crazy!  And some of you have been reading for all six of those years!  I never intended to become a blogger, but once I started, I found that sharing my experiences and my thoughts was both cathartic and a great way to meet some amazing people. It's been a ride and I'm thankful for all of you who have ridden alongside us, offering your unending love and support over the years.

A reminder of what Jack went through six years ago and why I made my promise to him -

How's that for some hardware in your back?

As straight as they could get him given the quality of his bones

This face pretty much says it all

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And, speaking of reminders, here's another one .... For The Record