Hope is the thing that is willing to take a chance on the future. Hope is the capacity to see something on the horizon that we are willing to move toward. If our hope gets us from today to tomorrow, and in that new day we are ready or able to deal with something we thought we couldn’t face, then hope has done its job. There is a worse thing than false hope. It is no hope.
When you spend 17 days in the hospital and you have an iPhone, well, you take lots of pictures. When you have lots of pictures, you might as well share them. So, here you go . . .
Our trip to St. Louis is around the corner and I'm somewhat prepared. I packed Jack's bag of supplies over the weekend. I basically went through his closet and pulled extras from every drawer and box I could find. After I finished and zipped the bag up, it occurred to me that I might want to write down what I had packed so that I could go through the list before we leave and make sure I have everything. I've definitely come a long way from our first trip to St. Louis when I had lists that I double and triple checked. I've learned to throw his supplies together fairly quickly because of all his stays at Ryan House.
We've now added a new piece of equipment to our list.
They tell me my kid is complicated. "They" being the more than one doctor we've encountered over the last few weeks.
Today we had our appointment with the new nephrologist.
Let me back up a bit.
Jack was discharged on Wednesday. He had a relatively good Thursday, Friday and Saturday. He had such a good Saturday that we thought it would be fun to all go out to dinner. Jack lasted long enough for us to get to a restaurant and get seated at a table and then it became very clear, he was on the downhill decline of needing bagging. So, we left before we ordered.
Sunday he stayed in bed all day and had good day. Monday, he had two appointments. One with the orthotist to pick up his new DAFOs and hand splint and then to PCH for a blood draw. He was stressed a good part of the time and needed bagging at least once that I know of. I didn't go to the Monday appointments - Mark took him, but I was kept in the loop with texts from both Mark and Jack's nurse.
Today, we had appointments with the pediatrician and the nephrologist and Jack was stressed the entire day and required lots of bagging and even then, he was still in pain.
What.the.hell.is.going.on?
It seems now that Jack is fine when he's in bed, but when he's in his wheelchair and out of the house, he has issues. It's hard for me to believe that it's just being in his wheelchair that is the issue when two and a half weeks ago, he was in extreme pain when he was in his bed. We are still at a complete loss as to what is the source of the pain/desatting. Every doctor we talk with has an opinion based on the information I provide at that moment in time. We (me, Mark and Jack's nurses), who see him every minute of every day, can't find a trend or common denominator to pin the pain/desatting episodes to when we look at his entire course.
Yesterday, I received an email from Jack's St. Louis neurologist asking when she could call me about a possible new path for discovering what Jack might have. I emailed her right back and told her what has been going on with Jack and that I was available anytime to talk. She called within minutes of me sending the email and we talked for almost an hour about Jack, her experiences with other kids with neuromuscular diseases and her ideas of what might help Jack. It was so good to talk to someone who not only knows Jack, but knows kids with neuromuscular diseases. Her timing couldn't have been better.
One of the things Jack's neurologist mentioned was that there could be an anxiety component to Jack's episodes and that we should give him something to deal with anxiety to take that out of the equation. When we saw Jack's pediatrician today, I asked about getting a script for Ativan and while he agreed it made sense to try it, he wouldn't write the script. He suggested we contact the Pain Clinic at PCH. Thanks.
After seeing the pediatrician, we headed down to PCH to meet with the nephrologist. She spent well over an hour with us. The first thing she says: "Jack, you are a complicated kid." The nephrologist we saw when Jack was in the hospital focused on his low potassium and low citrate. Today's nephrologist focused on his low creatinine, which she said was due to his a muscular dystrophy. She also changed things up a bit and is not requiring the every two week blood draw/UA (she said that's not nice to Jack). It's a little concerning to me that one doctor felt the need to monitor Jack's potassium very closely and the other one does not. Today's nephrologist also added another prescription to the mix and, likewise, suggested we see the Pain Clinic.
Guess I'll be calling the Pain Clinic tomorrow.
More than one person (including some of Jack's doctors) has asked whether it's really necessary to bring Jack to St. Louis. My answer remains "yes". The fact is, my kid is complicated. We left the hospital a week ago believing we had solved Jack's issues. We haven't. I've received varying and inconsistent opinions about what his problem(s) could be and how to manage them. I want another set of eyes to look at Jack, evaluate his information (which I'm gathering from his PCH records) and give me his or her opinion. I need to know that I've done everything I can to find out if Jack's pain has a source that can be identified before I take the position that this is Jack's new normal and take it from there. More than that, part of me wonders if this will be our last trip to St. Louis with Jack, in which case, I feel even more compelled to go.
So, to St. Louis we will go.
I could write an entire blog post on how I'm doing - but, it's late and, really, it's not about me. But, I will tell you that this is difficult. Really difficult.
Onward.
For those who aren't on Facebook - we made it home! Thanks for all your love and support over the last few weeks. I'll update tomorrow - too tired tonight.
Notwithstanding the fact that the urologist didn't find or remove any stones in the OR yesterday, Jack is a new kid today. I can honestly say, he looks the best he has looked in months. The intensivist commented this morning that the sparkle is back in Jack's eyes. I asked the urologist again, what exactly he did in the OR since there were no stones to remove. He said he did some lasering of the tissue in the kidney. He also got out a large blood clot that resulted from the placement of the neph tubes. When I asked what could be the reason for the drastic change in Jack's pain level, he did say that trying to pass the blood clot could be as painful as passing a stone. That would explain Jack's weekend from hell. But, what about the pain that got him admitted? We do know that he had a UTI and a lingering impaction issue (although that is debatable), but you wouldn't think that would be enough to cause the pain that put him in here and kept him here for two weeks. How do you go from having extreme pain to having no pain with no absolute source or fix? If I didn't know for sure that I have actually been sleeping on a plastic chair and/or bench in an intensive care unit the last 16 nights, I'd think I made this whole thing up.
They transitioned Jack to his home vent today which, surprising to me, wasn't an easy transition. Jack apparently likes the support of the hospital vent better. But, after a couple of hours, he settled in on his home vent. They pulled the neph tubes this afternoon and unless Jack throws us a curve ball, we are OUT.OF.HERE tomorrow! Thank you God.
The nephrologist on service just stopped by and she shared a lot of good information about what the potential causes could be (there appear to be more than one) for the kidney stones, what we need to monitor and what we can give Jack, medication-wise, to help reduce the recurrence. I always connect better with doctors who give me logical explanations for things and what this doctor had to say was very logical. So, basically, I'm not walking away a skeptic, which I tend to be when it comes to doctors.
I'm cautiously optimistic that we have our "happy Jack" back. Time will tell. Thank you for all your support and well wishes - Jack and I cherish all of you.
You gotta love when life throws you a curve ball. After three hours in the OR, the urologist came out to tell me that he didn't find any stones in Jack's kidney, ureter, bladder, etc. What he did find was that Jack's entire right kidney was lined with a coat of calcium that, apparently, looks like stone on the CT scan. The urologist said that he can't remove the calcium lining. The urologist is stumped. My concern is, if there are no kidney stones, what is the source of Jack's pain?
What has also been nagging at me for awhile now is the reliance on what they see on Jack's CT scans. I order copies of the reports for all the CT scans and what every report has in common is the following language: "evaluation is significantly limited by spray artifact" (this is due to Jack's spinal fusion hardware). The radiologist acknowledges that he can't see certain things well or at all due to the artifact. So, I get frustrated when I hear, "the CT scan shows ...", when it's a given that the CT scan is limited in what it can actually show. I don't know what the alternative is, if anything. I just know that we still don't have answers. Post-op, Jack has been fine from a pain perspective. We'll see how his night and tomorrow goes and then figure out where we go from here. I'm hoping it's out the doors of this place!
I'm glad I decided to make an appointment with the urologist in St. Louis, I think a second opinion is definitely needed in this case.
Onward.
(shortest update on record! :))
This is our third Sunday in this place and I wish I could say Jack is much improved. He's had a crappy weekend. It seemed like we had the pain mostly under control during the week, but it's come back with a vengeance this weekend. The pain has been relentless. Jack's urologist said that placement of the neph tubes likely stirred everything up in the kidney. He is now on Lortab and morphine as needed. He's needed both pretty much round the clock, topped off with some Ativan on occasion. Jack was requiring quit a bit of bagging last night and the same intensivist who was on when Jack was admitted was on again. Two weeks in, he was surprised to hear that Jack is dealing with the same issue (respiratory distress due to pain) that he presented with.
To follow-up on the neph tube situation, Jack's urologist came by the next day (Thurs) to check the tube and to try and draw urine out of it and he couldn't get any. He said it could be clogged with blood. They put Jack back on the IR schedule to have a nephrostogram, where they inject dye into the tube to see where it goes. When we got down to IR, the IR Fellow asked me if I knew what they were going to be doing (because I had to sign a consent for both the procedure and anesthesia). I told him we were there to try and find out why the tube is not allowing urine to drain. He then told me that, no, that was not the reason, but rather because when they placed the neph tubes the day before, they didn't get the placement exactly where they wanted, so they were going to go back in and try and get better placement.
Needless to say, a few questions popped into my head. Prior to discovering that the neph tube didn't drain, there was no intention (that I was aware of) to return to IR to try and get better placement. So, but for the lack of draining neph tube, Jack was, apparently, going to go into surgery with less than ideal placement of the neph tubes and I wasn't even made aware of this? My issue is not that they were not able to get "perfect" placement of the neph tubes - I get that you do what you can do with the patient you are presented with and that, when it comes to Jack, things are more challenging at times. My issue is that I was not told that the neph tube placement was less than ideal, so much so that they wanted a second try. I think that is information I should know. I was told - again, that the neph tubes were not intended to be drainage tubes, that they were solely access tubes and were supposed to remain capped. Again, that is inconsistent with what my urologist thought/told me.
I talked with the IR Fellow at length after the second procedure was over. I asked him to please give it all to me, that I could keep up with him. He finally got it out that the reason there is no drainage tube is because they tried, but were unable to get one in. You know, a lot of confusion and questioning on my part could have been avoided if I had been given this piece of information from the very beginning. I was told that the reason placement is so difficult in Jack's kidney is because the majority of kidney stones cause blockage and, therefore, the kidney is full of urine - which gives them a space to work in (like a blown up balloon). In Jack's case, there is no blockage, and, therefore, they are trying to get placement of the neph tubes near the stone in a "shriveled up" (his words) space. There is very little room to work and, again his words "you can only puncture the kidney so many times" when trying to get the best possible placement of the tube.
Okay - waaaayyyy more than you wanted to know about placement of Jack's neph tubes.
Jack has spent most of this weekend "snowed" because of all the pain meds he's been given to keep him comfortable. So far, he's been doing well from a respiratory stand point - he's on the hospital vent, but still on his home vent settings. However, I do worry about his lungs eventually taking a hit from all the sedation and inactivity (relatively speaking, since Jack isn't otherwise out running marathons in his spare time). Thankfully, one of the RTs suggested an IPV machine for Jack, which he has been using twice a day for over a week now. I think it's been the key to keeping Jack's lungs healthy so far.
Surgery is scheduled for tomorrow (Monday) at noon and it's expected to last about 3 hours. The urologist stopped by this morning and I pretty much begged him to please get all the stone out this time because Jack can't take much more of this. He, of course, said he hoped to. I asked if he was going to have to go back for a second look a couple days later, and, at this point, he doesn't know. It will depend on how much he gets out tomorrow.
Jack has had to endure so much pain the last few months, the kid desperately needs a break. I can't help but think of the words to the song from his birthday video last year. It sums up exactly how I'm feeling right now.
Today is the 10-day mark for this hospital admission and we are assured another 7 days, at least. What I wouldn't give to sleep in a bed again. After a week of sleeping on a fold-out chair for 7 nights, when the nurse manager came into Jack's room last Sunday and asked how things were going and if I needed anything, I jumped at the opportunity to request a room that has a bench bed. They moved Jack that night into a new room. At least the accommodations have improved this last week.
It is the little things after all.
Morning rounds were much improved on day 2 and 3. That said, I don't feel as welcome to participate with this team as I did with last week's team. But that's okay. This week should be a low key week as long as Jack stays the status quo. Speaking of staying the status quo, Jack wasn't on his home vent but two hours and he needed to go back on the hospital vent because his CO2s were too high after he had another episode of pain.
The urologist called me yesterday with the plan which was neph tubes placed in IR on Wednesday and surgery next Monday. I'm not real happy with the five day lag between tube placement and surgery, but the thinking is that if there are pieces of stone breaking off from the large stone (how that would happen I don't know, it's not as if Jack is engaging in contact sports), then putting in the neph tube will give the stone an exit from the kidney without causing Jack any pain.
Jack had the nephrostomy tubes placed today. They placed two tubes because there are stones in different parts of Jack's kidney and the urologist wants two routes to go in and get them out. I wish I could just say that Jack went to IR, got the neph tubes and I'm 100% comfortable tonight with where we are. It took two and a half hours to get the tubes in. That's really much longer than it typically takes for these things. Jack's anatomy makes the placement much more difficult. When Jack returned from IR, I saw two really thin tubes extending out of Jack's back and they are both capped off. One is a long tube that extends past Jack's back so he doesn't lay on the cap and the other one is really short causing Jack to lay on top of it. Several things immediately jump out at me: (1) there is no bag attached to either of the tubes; (2) the neph tubes look nothing like the neph tube he had last time - and came home from the hospital with; and (3) the cap on the tubes has two hard plastic "wings" and Jack is laying on the cap and the wings are digging into his skin.
I raise my concerns with Jack's nurse and the NP. Jack's nurse wrapped a big wad of gauze around the cap, so I think we are okay with preventing any skin breakdown from that. The NP told me that the IR doctor said that both tubes are supposed to be capped. That's not what the urologist told me when he stopped by Jack's room this morning. I was told that one of the tubes would drain urine. When the resident stopped by Jack's room later, I raised the question with him and he agreed that it was his understanding that the neph tube was supposed to allow urine to drain. But, he reiterated what the IR doctor said.
Am I missing something here? Why would you even ask the IR doctor - she just placed the tubes at the direction of the urologist. The urologist is the guy in charge of the whole kidney stone thing. So I called the urologist's office and left a message for him. He called me back right away (love him!) We talked and he confirmed that, yes, one of the tubes was supposed to be hooked to a bag to drain urine. He said he'd call the unit. Shortly thereafter, everyone was on the same page. However, the challenge became finding the supplies to hook up to the tube. After a little creative thinking and a little cutting and taping, they came up with tubing that would hook to the neph tube to drain the urine. Here's the problem, not one drop of urine has drained from the neph tube and it's been 6 hours since they hooked everything up. Clearly, something is wrong.
*Sigh* Can't anything ever go according to "plan"?
Don't answer that.
I realize with each passing day my perspective becomes skewed. What starts out as a team effort (Rah! Rah!) becomes an "us" against "them" mentality. I know in my heart that these are good people who all want to help Jack as best they can. I remember when my mom came out to visit me, oh so many years ago, when Jack was taking up residence in the St. Louis Children's Hospital PICU. She spent one evening with me in the PICU (I think Jack had been inpatient about 2 months by this point) and she told me I had "ICU-itis" and I needed a break. Turns out, I just needed a little Xanax. :-)
Well, my friends, that's the update for today. I'm hoping Jack has a good night and that the urologist can shed some light on the situation tomorrow morning.
Onward.
I now know why you don't want to spend more than a week in the hospital. Today started a new week and a new team consisting of a new intensivist and a new resident. This morning, we were the last stop on rounds and by the time they got to Jack's room at 10:30am, I was more than a little stir crazy.
I'm starting this post at 3 o'clock in the afternoon, that way I'm assured of finishing it the same day I start it.
The issues when Jack was admitted one week ago were three-fold: (1) kidney stone; (2) UTI; and (3) impaction/slow gut. In a week's time, they have resolved the UTI, determined that Jack has no significant GI issues and adjusted his feeds to account for the slow gut. We knew Jack had the kidney stone coming in, but didn't believe it to be the most pressing issue because we've (knowingly) been dealing with kidney stones since February and haven't seen Jack decompensate due to pain like he has this past week. I really thought we were on our way out of here by tomorrow at the latest, but Jack had another episode of decompensation this morning and the intensivist says that it has to be due to the kidney stone at this point. So, where do we go from here?
We could go home, try to manage Jack's pain with round-the-clock pain meds and, if necessary, continue to bag Jack through periods of distress until he can get on the surgical schedule for removal of the stone. I think the docs here would rather not send us home knowing that Jack would likely continue to decompensate when his pain level is high.
The other option is to try and get Jack on the schedule now to have the stone removed. I know our urologist will do everything he can to get Jack on his surgery schedule as soon as possible. The hold up is interventional radiology. The first step in having the stone removed is to have a nephrostomy tube placed and that is done in IR and getting on IR's schedule is the challenge. The reason it is so difficult to get on IR's schedule is because they only work 8-5, M-F. It's beyond my comprehension why this is. But, I'll refrain from a commentary on all the things that bother me about PCH.
Jack's urologist has been called and I was told he'll be in to see Jack either today or tomorrow. It's really going to be his call whether we have the surgery done sooner than later. I think he'll do everything he can to open up his schedule. But, he's also going to have to be the one to push IR to fit Jack in their schedule.
What we are running up against is our trip to St. Louis. The further we push this out, the closer we are to the first week in October the less time Jack would have to recover from surgery to make the trip. The original plan was to have the surgery to remove the stone when we got back from St. Louis, but there is no way that Jack can wait that long. If it looks like Jack will have the surgery too close the the St. Louis trip, I'll be on the phone trying to rearrange our St. Louis appointments. All of Jack's St. Louis doctors know we travel far to see them and I'm fairly certain that if I am unable to get everything rescheduled in the same week by going through their schedulers, I can go directly to the doctors and they will get it done for us.
Could I just skip the trip to St. Louis? My answer would be no, for several reasons. During this hospital admission, we've noticed that one of Jack's eyes has a cloudy half-moon area just below the pupil. Looks like a new cataract has formed on the eye he had surgery on last year. While not an urgent issue, he needs to be seen by his ophthalmologist and when it comes to Jack's eyes (which are very complicated -- surprising, I know), I'm sticking with the guy who has been operating on them since Jack was one year old. The other issue I really feel needs to get checked out this year is Jack's heart. He's been through a lot this year and I want to make sure his heart hasn't taken a hit as a result, or if it has, I guess I want to know that too. The Heart Center at SLCH is one stop shopping, so to speak and, it's where I'm most comfortable with Jack's heart care. Seeing the neurologist, ENT and ortho doc are not necessary, but if we are out there, we might as well see them.
That's what I know as of now. Jack's days are good as long as his pain is under control. As of today, they are giving him a scheduled dose of a non-narcotic pain med with orders for ativan/morphine for breakthrough pain. So far, he's not needed anything but the scheduled pain med.
I realize that these updates are ridiculously detailed. But, hey, I'm a detail kind of person, so it's what you are going to get from me!
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UPDATE: As I was typing this entry, the urologist called me on my cell phone and said that he will contact IR on Tuesday and try and get something set up. He also said that he has to get the equipment lined up that he has to use for the surgery and he has to clear his schedule. He is hoping to get Jack on IR's schedule this coming week and have the surgery the end of the week or next Monday at the latest. Looks like we are here for at least 2 more weeks. Serenity now.
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Just have to share that while I'm hanging out in hotel PCH, my sister Joan and her family are enjoying parents' weekend at the United States Air Force Academy in Colorado Springs where my nephew is a first year cadet (or whatever they are called):
I'm going to try and make this a short report because, big surprise .... I'm tired.
I believe I left off at the decision to commence clean-out number 3 and the PICC line.
Yesterday afternoon when I came back up to Jack's room after he had the PICC line put in, I was stopped by the GI nurse practitioner. The PICU team had requested a GI consult and apparently, the GI docs are too important to conduct the initial consultation, they only talk to you after their NP has.
The NP made a comment about us being from St. Louis, telling me that she was from St. Louis too and had worked at Barnes (the adult hospital next to SLCH) for years. The St. Louis connection gave me a comfort level with her she probably wish I didn't have. I pretty much unleashed all my frustration on her and told her that I thought Jack needed a scope, that I was tired of all the admissions and getting no resolution to Jack's pain and if something wasn't done soon to help Jack, I was putting him in the car and driving to St. Louis (as if that was even a possibility or that she cared?!) She asked a few questions, but mostly just listened.
A couple of hours later, Jack's nurse came in and turned off the GoLYTELY. When I asked her why, she said because GI said the KUB did NOT show stool in the bowel. I was a little annoyed that GI was all the sudden calling the shots when I hadn't even been graced by a physician's presence yet. I asked Jack's nurse to let the resident know that I wanted to talk with her. A few minutes later, both the resident and the Intensivist showed up. I asked why the change in plans and why GI was now calling the shots. (I'm not even sure why I was upset that GI was calling the shots, I wanted them involved, didn't I? I think it was their approach. I prefer the team approach, not being told what we are going to do). In any event, the Intensivist said that he asked GI to consult and that we just need to remember that whatever GI comes up with, it's only their opinion. He said we were free to accept or reject their opinion, but that we at least needed to hear it.
I have to digress here and say that all the planets and stars must have been aligned just right (and by the grace of God), the Intensivist covering the PICU this week couldn't have been a better fit with my personality. He's a high energy guy and he handles my high maintenance energy personality very well. He "gets" me and he and I think alike. I also get the sense that he is very protective of his patients, he likes to provide all the care as much as possible and he doesn't like specialists coming in and tromping on his territory.
Anyway, later that afternoon, the GI doc came by Jack's room to talk with me. He, obviously, had been prepped by the NP because he didn't really have much to say, but suggested that maybe we should scope Jack's stomach. I added that I thought it would be a good idea to get a colonoscopy too. He was okay with that, but told me that he wasn't covering the hospital on Friday and that it would be another GI doc performing the scopes. Shortly after the GI doc left, the Intensivist stopped by and asked if I was okay with the plan, which I totally was. He also told me that the GI doc who would be performing the scopes was very good - that she was a "thinker" and a good person to have trying to figure out Jack.
The scopes were done this afternoon and I was so hopeful that they would give us the information we needed to pinpoint the source of Jack's pain. After the scopes were finished, the GI doc went over the procedure and provided me with copies of her reports. To give you an idea of how things went, the Endoscopy Report provides "the procedure was incomplete". The reason it was incomplete was because she was unable to get the scope down Jack's throat due to his enlarged tongue and his jaw contractures, which made it impossible for her to manipulate his jaw and neck to get the scope in. Rather ingeniously, however, she scoped his stomach through his g-tube site. She didn't find any ulcers or anything obvious in the stomach that would be causing him pain. She did take biopsies.
The next report was the Colonoscopy Report, which likewise provides "the procedure was incomplete". In this case, she could only go so far with the scope because it would have required manipulation of Jack's body that she was not comfortable with given his fragile bones. She didn't feel it was necessary to see further into the colon because there wasn't a concern that Jack had a bowel disease that would require looking further up (such as Crohn's).
The doctor felt like she saw enough of what she needed to see to come to the conclusion that Jack doesn't have an ulcer, impacted stool or anything obvious that would explain his pain. She thinks it's a motility issue. The recommendation is to change his formula to a pre-digested formula and give it to him over time instead of giving bolus feeding. I asked her how someone like Jack - who has had severe muscle weakness all his life, can all the sudden have motility issues. She mentioned something about maybe having a virus or some infection that triggered it. Doesn't make sense to me. (According to my friend who is a doctor "motility is what they end up with when they don't know what else to call it").
The Intensivist and I are both a bit skeptical because Jack had another pain episode this morning and he hadn't had any food in his stomach for several days (only the clean-out). However, we have nothing else to go on right now. They started Jack on feeds tonight at a very slow drip (starting at 5cc/hour) and we'll see how it goes until, as the Intensivist said - "he throws us another curve ball".
The one thing they have not checked is Jack's gall bladder. I mentioned it to the resident this evening and she seemed to be on board with ordering an ultrasound of the gall bladder. If they don't bring it up in rounds tomorrow morning, I will. The GI's biopsy results won't be back until Tuesday. At this point, I don't have any idea how long we will be here. I just know that I'm not leaving until I'm comfortable that I won't be back within the week.
Obviously, we won't be going to California and I won't be participating in the Disney Half Marathon with Team Cure CMD. I'm sad and I'm mad. Yea, I know .... life isn't fair. The only thing worse than spending the weekend in the hospital is spending a three-day weekend in the hospital. But, here we are and all we can do is try and make the best of it!
So much for a short report. For the third time, I've started a post on one day and finished it the next! Have a great weekend friends.
Thank you for riding along with us on our journey.
