Monday, October 29, 2012

Weekly Update

I vaguely remember those days when this blog was less about "Jack's Journey" and more about the "and Then Some" because things were status quo with Jack.  Anymore, life seems to be all about Jack.  Well, not really because I have three other kids who are giving me a run for my money, but so far, it's not life and death run for my money, so Jack trumps them.

As for Jack's journey, last week was a mixed week.  Jack had a generally good week, which is to say, he didn't require any bagging, his heart rate was good, he tolerated his wheelchair, he did well for his teacher and he even shared a few smiles.  He had such a great Saturday that I decided it was time to venture out to get Jack new glasses because his prescription changed after he saw the St. Louis ophthalmologist.  No sooner did we get Jack loaded in the van and his disposition changed.  He was stressed and not happy.  Really?  Why?  I do not get it.  I considered that maybe it was an anxiety issue more than anything else because he had been FINE all day long.  I decided to give him a dose of  ativan to see if it would help.  I guess you could say it helped because this was Jack at the eyeglass place:

Yeah, kind of difficult to measure one's eyes for glasses when they are closed.  I told them to just use the measurements they had from last year.  *Sigh*

Our Saturday evening out led to a perfectly crappy Sunday.  High heart rate and generally miserable kid all day long.  As I said before, I don't get it.  I cannot for the life of me figure out what the "trigger" is.  Every time I think we are on the upswing, Jack changes directions.  Nevertheless, things are exponentially better than they were just a few weeks ago because we haven't had to bag Jack since we've returned from St. Louis.  I guess if the best we can get is no bagging, then it will have to be good enough.

Today, Jack and his nurse carved a pumpkin.  Doesn't Jack look thrilled? (she says sarcastically).  Where's the smile Jack?

Jack, Eric and I will be hanging out at Ryan House this coming weekend while Mark and Hilary travel to California to attend the Equestria LA conference in Anaheim where Hilary has registered for a table and hopes to sell some of her artwork.  I hope she does well, but, if nothing else, it will be good exposure and a good learning experience for her.

My sweet Jack has a birthday this coming Sunday.  I'm a mix of emotions as I always am when his birthday rolls around.  I haven't yet put my thoughts to words, but I'm sure I'll come up with something by Sunday. :)

I close with thoughts of my friends and family along the East Coast who are riding out "Sandy".  Those I've heard from say the worst appears to be past them but, according to one of my friends who lives in New Jersey, "the East Coast is destroyed".  My niece, who also lives in NJ, was supposed to be on a flight home tomorrow morning with her fiance for an engagement party my sister has planned for them on Sunday. Hopefully, they'll still make it.  I'm very bummed for my St. Louis friends Jenny and Jason who have been waiting nine months for their appointment at the NIH for their son, Ben.  Their flight has been canceled and if they can't get a flight out and be there by Wednesday, it will be months again before they can get another appointment. Prayers that they can get there and that the NIH is open for business this week.

It's been a crazy year on so many levels.  It's hard to believe we are heading into the last two months of the year.  I'd say I'm ready for this year to be done with, but there's something about the year 2013 that doesn't give me a warm fuzzy feeling either.

Have a great week my friends.  If I am able to capture a smile with my kid this week, I promise to share it!

Monday, October 22, 2012

What A Difference A Week Can Make

Last week I was tired and feeling down and Jack was feeling blah and unsmiling.  This weekend, Jack seemed to turn a corner. He was happy, smiling, not requiring non-stop suctioning and clearly feeling so much better.  I had enough energy to get things done around the house on Saturday and then head up North for a few hours of hiking on Sunday, capped off by a birthday party at my house last night for all the October birthdays in my family.  I fell into bed last night feeling tired for good reasons and, for the first time in a long time, felt like I had enjoyed my weekend rather than just survived it.

I'll take it.  Jack and I are both due.

We have upcoming appointments with the new (potential) pediatrician and with the nephrologist. Other than that, I don't care to see the inside of a hospital or doctor's office for many months to come.  I am so done.  Typically, Jack sees his Phoenix doctors about once a year, at best.  I think it's time to get back to that schedule. I still need to decide whether we are going to have cardiology locally follow Jack or if we make the dreaded trip back to St. Louis again next year.  This is how I explained it to Jack's neurologist when she asked how our trip home went:

We made it home safe and sound vowing that we cannot make this trip ever again - which we say every year and, yet, cannot stay away from the superb care and amazing friendship SLCH provides.  

I also added:

 I really need to get someone to document our travels though - it would make for a compelling reality show.  I don't think anyone can truly fathom all that is involved in transporting Jack across county. But it is so worth it.

In any event, I'm not even going to contemplate what we are doing next year until at least next year.  And next time we make the trip, I've got to find a way to document it.  You'll either find it compelling or determine we are crazy and need to be committed.

On a side note, I requested and received in the mail this weekend copies of Jack's reports from cardiology. I only requested his echo report and the report that is sent to Jack's pediatrician from this last visit. They sent me all his records/reports going back to 2005.  Very interesting reading.  But, I was a bit shocked when I read the report that was sent to Jack's pediatrician in 2005 - the year the cardiothoracic surgeon placed a pericardial patch between Jack's trachea and aorta to create a barrier in the event Jack's trach tube should ever erode through his trachea (Jack has a right aortic arch and his aorta is located right next to his trachea and if his trach tube were to erode through his trachea it would hit his aorta -- not a good thing).  The report included this statement:

"Of note, during the median sternotomy, the innominate vein was lacerated resulting in a brief period of hypovolemic hypoperfusion which required volume resuscitation."

I'm thinking if this was "of note" to Jack's pediatrician, it should have been "of note" to his parents as well and should have been shared with us by the surgeon when he came out to talk with us after surgery.  It was not.  Okay, so it happened 7 years ago and Jack survived, but it really makes you wonder how much information is not shared with parents and, but for me getting copies of all Jack's reports, summaries, records, etc., I'd be in the dark about a lot of things when it comes to Jack.  You don't know what questions to ask when you don't have all the information.  It's annoying to say the least.

Anyway ....

I'll work on getting some updated pictures of my "Happy Jack".  In the meantime - for those not on Facebook - here are a few of my favorite pictures from my hike (with my sister, Maureen) up North in Oak Creek Canyon.  The Fall colors were abundant.

Have a great week friends!

Monday, October 15, 2012

Calm Waters

An update is in order.  Thankfully, things have been relatively calm since we returned from St. Louis.  I desperately needed a break from being consumed with all things "Jack".  It's not to say that Jack still doesn't consume most my time, but I'm at least able to focus on something other than Jack when I'm not at home -- like work, for example!  

Jack is doing okay.  He's requiring tons of suctioning, which is really making it hard to leave his side for any length of time.  His secretions are thin and clear - it's like we are suctioning water out of him.  The thought that comes to my mind is that it could be related to his decreased heart function.  I know when adults are in heart failure, their lungs are "wet".  I'm wondering if the same thing is going on with Jack?  I was talking with my doctor friend (the founder of Cure CMD and an expert in all things muscular dystrophy related) today and she agreed that there might be a connection between the low heart function and the watery secretions.  The other thing we talked about was whether Jack's decline in cardiac function is not a progression of his disease but rather the result of some event from the last several months. The cardiac event that happened his second night in the PICU comes to mind.  At the end of the day, it doesn't really matter, but you can't help but wonder. I also emailed Jack's former pulmonologist and asked her about the connection between the heart and secretions and I'm waiting to get her thoughts.  I'm going to be really bummed if the copious secretions and non-stop suctioning are another part of Jack's new normal.  

I received a phone call from the St. Louis cardiologist's office today asking what Jack's blood pressure has been running.  I'm very impressed that they are following up and staying on top of things.  Jack's blood pressure has been running 80/56-60.  Low, but not unexpected because we know the heart medicine he is on (Enalapril) causes low blood pressure.  The cardiologist told me that if it gets below 80/40, I'm not to give the nighttime dose.  As you can imagine, having a blood pressure that low makes for a very "blah" Jack.  You have a kid who went from being on zero meds to being on four meds 2x a day.  I understand medications are necessary especially when they keep the UTIs and kidney stones at bay and keep your heart pumping, but the fact is, there isn't a single drug that doesn't have side effects and Jack is being bombarded with side effects from four different drugs.  No wonder he feels like crap.  

I suppose most people who see Jack wouldn't really notice any change.  After all, it wasn't as if Jack was out running marathons before the events of this year.  The difference is Jack's sparkle - it isn't quite as bright these days.  Nevertheless, he's not in pain and he's not requiring bagging every day and that is a good thing.  All we can do is take it one day at a time.  We just keep swimming and right about now, we are enjoying the calm waters. 

Jack and mom 

Yes, I'm as tired as I look.  It's time for me to put on my running shoes, get out of the house and start pounding the pavement again.  Exercise is the best way for me to re-energize.  


And, on a completely different note, what about them STL CARDS!  (I don't even like baseball, but it's hard not to get caught up in the hype, especially when you have so many St. Louis friends.)  Go Cardinals! :)

That's the update from here.  Thanks for checking in.  ♥

Saturday, October 06, 2012

St. Louis Recap

We made it through a very long and very tough week.  The impact of everything Jack has been through this year was clearly evident in how sleepy, weak and “out of it” Jack was the entire week.  I did have to remind myself that Jack did just spend two and a half weeks in the PICU just a few weeks ago and he did have significant trauma to his kidney that he is still recovering from.   That said, I fear the changes we are seeing in Jack are, in fact, his new normal.  His body is tired and he doesn’t have any reserve so it doesn’t take much to knock him down.  Simply sitting in his wheelchair wipes him out.   It’s even hard for him to muster a smile these days, but he tries.  He really, really tries.

In any event, the detail oriented me, will now give you the detailed update.

The Trip

Jack tolerated the trip well.  It was the right decision to get the motorhome because he was able to travel laying down.  We used the sling from Jack’s lift to get him in and out of the motorhome and into his chair – with Mark and I being the “lift”.  It made the transitions in and out of the motorhome much smoother and easier on Jack … and us.   

Eric was in heaven and claimed that this was “the best road trip EVER!”   However, by day three, he was asking if he could fly home. 

Our wonderful friends, David and Amy, who so graciously allow us to invade their home for the week, were happy to see Jack.  

 The weather has been beautiful for most of our stay, turning cold just yesterday.  I love St. Louis this time of the year.

Jack enjoying the beginnings of Fall foliage.

The Appointments



Our first appointment of the week was with the cardiologist.  The appointment started off with a trip to the “Heart Station” where Jack got a routine EKG and echocardiogram.  In the past, after these tests, we see the cardiologist and he says everything is fine and we go on our merry way.  I don’t really know Jack’s cardiologist that well because I’ve never had to interact with him much. 

This time things were different.  I suspected something was up when they took twice as long as usual to do the echo.  If you recall, I suspected that Jack’s heart had taken a hit after everything he has been through this year.  I based this on the extreme changes in Jack's heart rate that would regularly occur. Sometimes it would hang out in the 40s and other times it would stay in the 140s+.  

After finishing up at the Heart Station, we met with the cardiologist to go over the tests.  The EKG was fine, but the echo showed a significant changed in the ejection fraction of Jack’s heart.  Two years ago, Jack’s ejection fraction was 45%.  It is now 27%.  Two years ago, it was in the high normal range.  Now it is just below the low normal range.  The concerning part is not so much the number (although the number is far from great), but the extreme drop in only two years.  The cardiologist presented the information to me and said that we need to start Jack on a medication that is used to treat people in heart failure.  The medication can cause drops in blood pressure, so I had get the prescription filled and then come back to the clinic to have them give the medicine and then have his blood pressure monitored for several hours.  We also had to call in and give them his blood pressure readings the remainder of the week (from when it was taken at other appointments and during his time in Same Day Surgery).  So far, there have been no issues with blood pressure.

Needless to say, that while the news of Jack’s decline in heart function didn’t necessarily surprise me, it certainly has affected me – as well as a lot of other people who know and love Jack.  We’ve all been in a funk this week.  The significance of the decline has been made very evident in the subtle reactions I’ve observed from Jack’s other doctors with whom I’ve shared the information.   According to Jack’s neurologist, Jack’s ejection fraction is equivalent to a 25 year old with Duchenne Muscular Dystrophy (a form of MD that they know for sure affects the heart muscle). 

What does this decrease in heart function mean?  We know that it can’t be reversed, even with medication.  It’s likely part of the reason Jack doesn’t have as much energy and why things like just sitting in his wheelchair exhausts him.  I don’t really know how much the medication will help with this.  I think more than anything, the thing we really need to focus on now when making decisions regarding Jack's care is his quality of life, not the longevity.  It doesn’t mean that Jack is going to die tomorrow.  It could be tomorrow or it could be five years from now.  Obviously, no one knows.  But certain decisions have to be made based on this new information.  It’s not my intention to “dramatize” this new finding, but the fact that Jack’s heart is now being affected by his disease is a big deal.  It’s why when I get home I need to find a new pediatrician -  someone who can help us navigate this new phase of Jack’s disease progression. 

Anyway, on to the rest of the week.



Not surprisingly, the “talk” at our appointment with Jack’s neurologist centered around the news of Jack’s declining heart function.  Jack’s neurologist already knew my concerns regarding all that has been going on with Jack because of our recent telephone conversation.   We hung out in clinic and talked for awhile and then we went to lunch together and continued our discussion.   After lunch, she walked with me to the hospital pharmacy to pick up Jack’s heart medicine and while there, she wrote a script for some Ativan for Jack (something my pediatrician wouldn’t do).  She then went with us back to cardiology and helped me get Jack out of his wheelchair and changed.  She spent close to three hours with us just being there to listen and offer her support and guidance.  I am so blessed and grateful to have such a special relationship with Jack’s neurologist.  She is SO good to us.

Anesthesia Pre-op

I met with an anesthesiologist prior to the procedures on Friday to go over Jack’s history.  The anesthesiologist was somewhat concerned about the news regarding Jack’s heart. But, I reminded him that Jack had just undergone several two and three hour procedures/surgeries in Phoenix and he tolerated them fine, so he should do fine with the broch and EUA – which didn’t required the same level of sedation.  He agreed that the fact he tolerated the recent surgery without incident was a good sign. 



Nothing surprising from this appointment.  There is no new cataract forming. What we thought was a cataract is the capsule of the lens implant. 


Jack’s orthopedic surgeon compared Jack’s x-ray immediately after his spinal fusion surgery six years ago to a current x-ray and the degree of curvature was exactly the same, despite the fact that Jack looks like his curve is worse.  The ortho said it’s just a factor of Jack getting bigger and getting “more muscle”.  He clearly had Jack confused with someone who doesn’t have a neuromuscular disease.   

I have to say I was a bit of an emotional mess on Thursday.  Both the ophthalmologist and ortho were running an hour and half behind – so there was a lot of down time.  Jack was unusually tired – he had his eyes closed most of the day, which is so not Jack.  I’ve never seen him sleep during the day except when he’s under the influence of drugs – which he was not on this day.  All the waiting, seeing Jack so out of it and reflecting on the news about Jack’s heart just had me fighting tears all day long.  I try and reserve my tears for when I’m alone, but Thursday it was tough to keep the tears at bay.


Bronchoscopy and EUA of Eyes

Jack’s ENT stopped by before the procedure to chat.  He shared with me that he had read something I had written about our experience with SLCH on “your blog or maybe it was on Facebook.”  Yikes!  In thinking about it, I’m pretty sure what he read was this article that was on SLCH’s website and Facebook page.  I really doubt he reads my blog. 

The news from the broch is that Jack’s airway looks good despite thirteen years of suctioning.  He did say that the area where Jack’s trach tube had caused some erosion and where the pericardial patch was placed back in 2005 definitely looked abnormal and if he hadn’t known the history behind it, he would have been concerned. 

The EUA didn’t show anything of concern.  The pressures in Jack’s eyes are good, so there was no need to do any lasering. 

Everyone in Same Day Surgery knew we had appointments to make that afternoon, so they were good about moving things along and getting Jack discharged as quickly as possible.


The urologist went over all of Jack’s CT scans with me.  He agreed that the CT scan in June showed that there were indeed some stones still remaining in Jack’s kidney after the procedures done in April/May and that the most recent CT scan taken after the most recent procedure (where the urologist didn’t find any stones) does in fact show no stones.  He doesn’t have any explanation as to what happened to the stones if we didn’t see any pass, except that maybe they broke up into such small pieces somewhere along the line and that Jack did pass them, but they were so small they weren’t visible.  Regardless, he said he would have proceeded the same way our Phoenix urologist proceeded and that he wouldn’t do anything more at this point given the most recent CT scan.  I wanted a second opinion, I got a second opinion and I’m comfortable that our Phoenix urologist is doing a good job at managing Jack’s kidney stones.


The nephrologist went over Jack’s history with me and she was in agreement with the medications our Phoenix urologist has Jack on and she is also in agreement that Jack doesn’t need every two week blood draws or UAs.  She asked that I fax her the results of the next 24 hour urine and blood work that Jack will have done in a few weeks.  After meeting with the St. Louis urologist, I am comfortable with the care and plan of treatment being provided by our Phoenix nephrologist.

I know some people probably wonder why I even feel the need to question our Phoenix specialists.  Right or wrong, I just have a level of confidence in the doctors at St. Louis Children’s that I don’t have with the doctors in Phoenix and I will always prefer to receive care at SLCH over Phoenix even if I can’t always secure it.


After finishing up with our afternoon appointments, I called the cardiologist office and asked if he was available to answer some questions I had about the implications of what we learned earlier in the week.  He graciously took time out of his schedule to talk with me.  I know, in general, cardiologists are not known to have hearts of their own (and I’ve met one or two who would fit that profile), but Jack’s cardiologist has a very kind and gentle demeanor and he was great to deal with this last week.  He told me he would like to see Jack back in June.  I didn’t say anything to him, but it’s really not likely we will make a trip back there in June just to see the cardiologist.  I need to find a cardiologist in Phoenix to manage Jack.  Finding one I trust will be the challenge. 

Friday ended a very long and draining week of doctors’ appointments.   To say I am exhausted is an understatement. 

Good Times

The week was not all doctors’ appointments and serious stuff.  This trip to St. Louis was exceptional in good ways too.

One of Jack's former Phoenix physical therapists moved back home to Illinois six years ago.  She happened to be at Children's with her son on Tuesday, so were able to see her while we were there.

After having reconnected on Facebook with Jack’s very first pediatrician, we met up with her one evening after she finished at the office.  It was so nice to see her again.  I only wish she could have seen the happy Jack, not the tired, stressed out Jack.  Thus, the reason for no picture.

I had dinner one night with three of Jack’s former nurses.  One who took care of Jack in the Special Care Nursery when he was born, one who took care of Jack when he was in the PICU and one who took care of Jack at home (and who is now the nursing supervisor of Same Day Surgery/PACU at SLCH).  What a treat to spend time with these special women – all of whom have been part of Jack’s journey.  I’m so lucky that after all these years, we’ve kept in touch and remain friends.

We ended the week visiting with our friends Jason and Jenny and their kiddos.  I first "met" Jenny years ago on the Tracheostomy message board.  We reconnected (amazingly) on the Cure CMD message board after her son was diagnosed with a congenital muscular dystrophy.  I didn't know Jenny when we lived in St. Louis, but we've become good friends since I moved to Phoenix.   

 We ended this fun evening with Mark backing out of their driveway and into a parked car across the street smashing the side of the car with our rental van.  Argh!  But, in the scheme of the events of this last week, that's a "little thing" and not worth getting stressed out about.  Right?

That's the St. Louis recap.  We are on the road again tomorrow for the trip back home.  Lots of information to digest and lots to do once we get home.

Thanks for checking in and thanks for caring! xoxo


(Inscribed on the Reflection Pool located on the Washington University Medical campus)

(Emily Dickenson)

Hope is the thing with feathers 
That perches in the soul, 
And sings the tune--without the words, 
And never stops at all,
And sweetest in the gale is heard;
And sore must be the storm 
That could abash the little bird 
That kept so many warm.
I've heard it in the chillest land,
And on the strangest sea; 
Yet, never, in extremity, 
It asked a crumb of me.

(Reflection Pool)

(I had a really moving encounter at the Reflection Pool one day when Jack and I were hanging out there waiting in-between appointments.  I'll share it with you another time because I need to finish this post!)