A Little Bit of Christmas

Just a quick update that is mostly pictures.  It's December and no time like the present to get in the Christmas spirit.  It's been several years since I've taken Christmas pictures of the kids.  This year, I decided it was time for another Christmas photo shoot.  Despite not being high on their list of favorite things to do on a Sunday afternoon, the kids were mostly cooperative.  After a lot of "sit up straight", "look at the camera", and "smile", here is a sample of what I got.

It's difficult for Santa to say "no" to this face. 

(oh, how looks can be deceiving!)

I tried to get them jumping in the air.  I couldn't get a shot that wasn't blurry with my camera (a professional photographer I am not)  

iPhone picture

(a little better)

We also decorated Jack's room this weekend.  I decided to add extra lights to his room this year so I strung lights through a few of the jungle branches hanging from his ceiling.  Pictures don't begin to do justice to the "coolness" of his room.  Nothing but the best for Jack! 

I still love a real Christmas tree, so we'll get that sometime this week.  We have our annual cookie decorating party planned the weekend before Christmas and we'll be looking to all our Facebook friends to cast their votes for the best cookie. Our kids have become quite competitive with this event and it's always so much fun!


On a completely different note, my cell phone rang Saturday afternoon and it was Jack's St. Louis neurologist calling just to see how Jack and I were doing. We talked for over an hour and it was a very much needed conversation on many levels.  I had actually wanted to call her last Friday because I had questions about some things that are going on with Jack, but I never got around to calling her from work.  So, her timing was perfect.

Have a great week friends!

Heartfelt Thanks

Many thanks to everyone for your kind and supportive words here on Jack's blog and on Facebook.  Jack and I feel the love from all around the world - literally.  The discussions and decisions of late are weighing me down and I really need to come up for air and focus on the lighter, brighter things in life. So, that's my plan!

Thanksgiving was a lot of fun.  I hosted it at my house and Jack tolerated sitting in his chair for several hours and was able to join in the festivities.  For those not on Facebook, here are a few pictures from the day.  These were taken with my phone and didn't turn out very well. (Hey, Christy - these qualify as crappy iPhone pictures!)

Our Traditional Tullamore Dew (Irish Whiskey) toast to my mom

FaceTime with my niece in NJ

Eric spent most of his Thanksgiving at the computer playing Minecraft

(yes, a better parent would have made him get off the computer, but this parent was busy enjoying her family and the festivities)

My dad and nephews

FaceTiming my brother and his family in Alabama

Cousins

and their moms (in same order)

Next stop, Christmas.  This year I'm adopting my friend Jenny's "Tis a gift to be simple" approach and am limiting my kids to three gifts each.  It's not that difficult for the girls, but Eric is having a tougher time putting his list together.  Now, I just have to try and keep it simple in ALL aspects of the season.  I think I'll start by not putting up Christmas lights or getting a Christmas tree.  

Okay, not really.  I love my Christmas tree!

Have a great week my friends. 

xoxo

My new Life is Good® shirt sporting my motto -

SIMPLIFY

To Be Honest

I have been called forthright, honest, very honest and, no doubt, a lot of other things that I’m not aware of.  For better or worse, I say what I mean and mean what I say and don’t hold much back.  I know people appreciate my honesty when sharing Jack’s journey because I keep it real.  But, [to be honest], I find myself struggling with how much I want to share and how honest I want to be as we navigate this journey after an exceptionally difficult year.  Things have changed and the course is no longer steady, but rather uncertain, unpredictable and confusing.  The decisions to be made are no longer straight forward and clear-cut and are often fraught with doubt.  I worry about being judged and yet, know that there isn’t a single person out there who can judge me more harshly than I judge myself.   

All that being said, it doesn’t seem right to stop sharing the journey just because the going has gotten tough. So, I will do my best to continue to share our journey and to “keep it real” despite how difficult and raw that may be at times.  And, I’ll try to be mindful of what Jack’s new pediatrician pointed out to me:  “this is your journey and no one else’s.”

So how are things going?  Last week we saw the nephrologist and got the results of Jack’s repeat 24-hour urine test that looks at the composition of Jack’s urine and identifies those things that are indicative of an increased risk for kidney stones.  It’s not really encouraging when the nephrologist pulls out the report and says, “Jack, you are giving me a headache.”  In other words, his numbers don’t make any sense.  In particular, his oxalate level more than doubled since the last test.  If you Google “high oxalate level in urine”, you’ll see that there could be any number of reasons for a high oxalate level.  Before the nephrologist got too far into the discussion regarding the test results, I told her about what we learned in St. Louis regarding Jack’s decreased heart function and let her know that, for a number of reasons, we are now taking a palliative care approach with Jack.  After getting that on the table, I asked her what her next step would be as a result of the abnormal numbers.  She said she’d start by ordering several different blood tests.  She also wanted to re-check Jack's potassium level after the high level they got two weeks ago, which incidentally came back normal when it was re-checked the next day.  I told her, at this point, I want to hold off on doing any blood work.  

And this is an example of how the decision-making process has changed.  In the past, I would have moved forward full steam ahead to get the blood work done, gather the information and insist that we do something to try and resolve the problem.  But, now I stop and think about (1) whether gathering the information will be a painful procedure for Jack; and (2) once I get the information, what will I do with it. Will it lead to more invasive testing and/or treatment? 

Palliative care focuses on relieving and preventing suffering and not on curing the underlying cause or disease.  Because Jack is doing well right now, I don’t feel like I should subject him to a nearly impossible blood draw to gather information that I don’t know that I’d do anything with once I obtained it.  Yet, after having made this decision, I still struggle with whether it was the right decision.  I feel like I’m sitting on my hands and, after fourteen years of aggressively “doing” for Jack, it is very hard to just be still and think before leaping.  There is nothing that says I can’t change my mind later on, as no decision is final.  But for now, we wait, watch and take our cues from Jack.

One of the many benefits of switching to our new pediatrician is that we are now enrolled in the palliative care home program.  On Monday, I met with someone from the program.  I actually knew of her and she of me because she refers parents to The Willow Tree Foundation for respite opportunities.  She told me about the program, offered her support and let me know that one of her jobs is to check in with me on a regular basis to see how I’m doing.   It’s very new for me to allow myself to not only admit that I could use the support, but to accept it.  It’s one thing to look to your friends who “live the life” for support and quite another to accept it from an “outsider”.  But, to be honest, I’m tired, I’m spent, I’m weak and I’m willing to acknowledge that I could use the help.  I was told that I’m suffering from “caregiver burnout”.  Imagine that.

I’m incredibly thankful to have been given the opportunity to have Jack followed by this new pediatrician.  It’s clearly where we need to be – for Jack and for me.

I'm also incredibly thankful for all those who follow our journey and who care about my Jack.  Thank you for checking in. Thank you for caring.

Stuff

We celebrated Jack's birthday last Sunday at Ryan House.  Jack and I stayed at Ryan House last weekend while Mark was out of town with Hilary. Jack hadn't stayed at Ryan House since March and I wanted to get him back there so they didn't forget him (not really!)  Actually, I had lots to do last weekend and without Mark or nursing, I'd have been stuck without Ryan House.  What I love about Ryan House is that they have separate rooms for the family - which are just like hotel rooms. I can hang out in my room and relax, read and/or sleep and be there to check up on Jack or answer any questions they might have, but still have time to myself. Or, I can be gone all day (which was the case most of last weekend) and come back and stay there the night with Jack.  It's just an awesome place all the way around ... with really awesome people who work there!

The family rooms have names that encourage one to rest, relax and sleep! 

I woke up the morning of Jack's birthday to find this on the door of his room:

When I peeked in on Jack he was asleep and his numbers were all good, which made for less guilt as I left to go to my sister's house for my niece's engagement brunch.  My niece Bridget and her fiance, Joe, live in New Jersey and almost didn't make it out for their engagement party because of Hurricane Sandy.  They didn't get out as early in the week as they had hoped, but they made it out by the end of the week and the engagement party went on as planned:

Congratulations Bridget and Joe!

After the engagement brunch, everyone headed over to Ryan House to celebrate Jack's birthday:

Ryan House graciously allowed us to use their kitchen area/dining room for Jack's birthday celebration.  

The big appointment of the week was with Jack's new pediatrician.  Her speciality is children with special needs/palliative care/hospice.  I love her!  She knew what questions to ask, she understood where I was coming from and she helped me (and Mark) make some difficult decisions now and gave us some difficult decisions to consider that will have to be made at some point in the future.  The best way I can explain how I felt after meeting with her is that for so many years, I've been leading the "charge" for Jack. I decided what I wanted for Jack and did everything in my power to make it happen (to the best of my abilities).  I was the captain of the ship and I was making all the calls - at least I'd like to think I was. :)  This last year, however, has not only taken a toll on Jack, it's taken the wind out of my sails.  I'm tired and, if I'm honest, I'm a bit lost.  I'm willing to release my grip on the reins and let someone else lead as we continue to navigate this journey with Jack.  I found this person in the physician I met on Thursday.  She came highly recommended and I was not disappointed. She was also not taking any new patients, but, thankfully, I had a "connection" who got us in.  I feel such a sense of relief knowing that I now have a pediatrician on board who will manage Jack in all aspects of his care and will not refer me out when the going gets tough.  

In other news, I received an email a few weeks ago from my friend and the co-founder of Cure CMD, Dr. Anne Rutkowski, letting me know that the world renowned physician/researcher at the NIH and an expert in the field of congenital muscular dystrophy (the same physician who met Jack a few years ago in San Diego) agreed to enroll Jack in whole genome sequencing to identify the causative gene for Jack's form of muscular dystrophy.  I was put in touch with the person who would arrange for us to get Jack's blood delivered to the NIH for testing.  I was told that they like to get the parents' blood as well when doing the testing. I mentioned that I had a daughter who we though might be mildly affected because she has low tone (Hilary), so she said to include Hilary's blood as well.  Knowing we'd be down at PCH for scheduled blood work that the nephrologist wanted, I asked if they could get us the supplies for the blood draw as soon as possible because the only place we'd be able to get Jack's blood drawn was PCH because he is such a difficult stick.  Getting PCH on board to draw Jack's blood for the NIH was a challenge. Getting someone on the other end of the phone to understand what I needed (someone to collect the blood from Jack and put it in a specimen tube I would provide) and what I didn't need (someone to spin, freeze, test or ship the blood) was the first hurdle.  After that, figuring out if they could even do it because how would they bill it? became the next issue.  I'll spare you all the details of our multiple phone conversations, but it was ridiculous all that was involved in just getting them to draw Jack's blood for me. Actually, they didn't agree to draw Jack's blood for the NIH, what they did agree to was that after they finished drawing Jack's blood for the nephrologist's order (which they could bill for), they'd fill the extra tube for the NIH.  As for the rest of us, my PCP's office agreed to draw our blood without hesitation and for no charge. (Thank God for the people who go out of their way to help us because they are the ones who replenish the energy depleted by those who go out of their way to put up hurdles.)  In any event, we managed to get all the blood drawn and shipped off the same day we were down to PCH for the appointment with the new pediatrician.

drawing the liquid "gold"

As Jack's neurologist pointed out, "giving Jack's muscular dystrophy a name does not benefit Jack, but it definitely helps us understand a very severe form of muscular dystrophy which may benefit others."  

Unlocking the mystery of Jack?

After spending a good part of the day down at PCH on Thursday, I thought I was done for awhile.  However, I received a phone call Friday morning when I was at work from the nephrologist's office telling me that Jack's blood results came back with an extremely high potassium level and I needed to get Jack back down to PCH for a repeat blood draw stat.  I was also told that the blood that was drawn the day before had hemolyzed and, therefore, that could have skewed the results. Needless to say, I was not happy about having to leave work, pick up Jack and make the half hour drive back down to PCH!  My mood didn't get any better when I arrived at PCH and was greeted by a security guard before I even got to the entrance of the parking garage and was asked "what brings you here today?"  Really? What brings me here today?  With an exasperated tone (because, I really was not happy at this point), I proceeded to ask him, "what do you THINK brings me here today?  This is a Children's Hospital, is it not?" I mean, really?  I wasn't there to do my grocery shopping!  I have an issue with PCH's security anyway because you'd think it was the Pentagon with all you have to do just to get in the parking garage.  I understand having to sign in and get a badge once you get inside the hospital, but the third degree at the parking garage is unnecessary!  After we entered the hospital, I discovered why the question prior to entering the hospital grounds -- Garth Brooks and other country celebrities were on campus for a fundraiser/publicity event.  

We made our way through the crowd of cameras, lights and people to get to the lab on the second floor. The lab tech tried twice to get blood out of Jack without success. They have a "2-stick" rule and after that they have to call the ordering physician's office, have them send a request for the IV team and then the IV team is paged and we wait for hours until the IV team can make its way to the lab.  Two plus hours and three sticks later, they finally got their vial of blood from Jack.  

I was told to hold Jack's dose of Potassium Citrate until they called us with the lab results.  No one called me on Friday with the "stat" lab results, so I have no idea if Jack's potassium really was high or if it was, in fact, due to the bad blood from first blood draw.  I'm inclined to think that his levels were fine with the repeat test.  I'll call tomorrow and find out.  

This week we have more appointments - with the nephrologist and the pulmonologist.  I swear, I am so over appointments with doctors!  I'm vowing to take the rest of the year off.  Think I'll be successful?

That's all the "stuff" from here. Have a great week friends!

Happy Birthday Jack

Happy 14^th Birthday Jack!  Every time I think I have this gig called life figured out, you remind me that life is a journey, not a destination.  For all you’ve given to enrich my life, I wouldn’t change a thing.  For all you’ve had to endure, I’d change everything.  You face the hardships of this life with unprecedented joy and grace and you challenge me to do the same.  To say you are my inspiration does not begin to express how profoundly you guide me in all that I do.  Thank you for the last 14 years, my son.  May this next year bring an abundance of good days and be filled with more smiles and less tears.

Love, Mom

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!

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!

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.  ♥

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

TUESDAY:

Cardiology

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.

WEDNESDAY

Neurology

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. 

THURSDAY

Ophthalmology

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

Orthopedics

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.

FRIDAY

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.

Urologist

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.

Nephrologist

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.

Cardiologist

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

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

Hope
(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!)

Hospital Days - In Pictures

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 . . .

Hospital Days .... In Pictures 

Make a video - it's fun, easy and free!
www.onetruemedia.com

(Music by Jason Mraz - "I Won't Give Up"  - Instrumental)

Update of All Sorts

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.

I know many of my friends have had feeding pumps since the time their kids first came home from the hospital.  This was just another one of those things that I fought hard not to acquire from the very beginning.  We lasted almost 14 years.  I feel like we are going backwards.  The only reason we need the pump is because the nephrologist wants us to get more water into Jack and the only way to get the volume she wants is to give him water through the night, and the only way to give him water through the night is with the feeding pump. So, he now gets his night time can of formula and a bunch of water over 10 hours at night.  Add a bunch of water, along with the maintenance antibiotics and well, you can just imagine.  Needless to say, there is very little down time now when it comes to Jack's care.

But,  . . . he is so totally worth it.

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After getting two different opinions from two nephrologists on how to manage/prevent Jack's kidney stones, I decided to add an appointment with a nephrologist in St. Louis to our list. I was surprised that I could get a new patient appointment with only a week's notice.  I think the fact that we come from out of state and they could see in their system that we have appointments with other doctors throughout the week probably helped our cause.  We will now be seeing seven doctors in four days.  I really don't think Jack can be managed long distance by the St. Louis nephrologist and/or urologist, but I'd still like to get their opinions on what's been found, what's been done and how it's all being managed.  Jack's toughest day will be next Friday - he has to be at the hospital by 6am for a bronch and EUA and then has an appointment with the nephrologist at 1:30pm and the urologist at 2:15pm. It's going to be a very long day for both of us.  I haven't yet figured out when I'm going to have time to visit my St. Louis friends because our days are so jammed packed with appointments.  But, we will make it happen, we just have to!

In the past, we've always made the drive over two days (except for last year), but it's now a three day drive (each way) because 8 hours a day in the car is about all Jack -- and Mark and I - can manage.  This year we are doing things differently because we are renting a motorhome for the trip. There is just no way Jack can handle the trip sitting in a van seat the entire time, nor can we manage all of Jack's care while he is sitting in the seat.  We are getting a medium size motorhome which has a big bed for Jack to lay on during the drive.  Needless to say, Eric is very excited for the trip!  Once we get to St. Louis, we're renting a wheelchair accessible van because we can't exactly park a motorhome in the parking garage at Children's Hospital.  It's definitely not a budget trip, but . . .

 . . . he is so totally worth it. :)

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I exchanged emails with Jack's former pulmonologist, who is now in Florida, to give her an update on Jack and ask her some questions regarding his vent settings.  After hearing about Jack's pain issues, she said that one of the things that she sees a lot in her neuromuscular patients as a source of pain is compression fractures.  If you recall, compression fractures were mentioned by another doctor friend of mine earlier this summer and our local orthopedic doctor said that Jack couldn't have compression fractures because he is fused.  Just to confirm, I emailed our St. Louis ortho and asked him the same question and his response was "Dr. White is correct".  Good to know.   That being said, I'm definitely going to ask our St. Louis ortho to take a good look at the x-ray he will be getting of Jack's spine to make sure there is absolutely no possibility that Jack's pain is spine/orthopedic related.

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Remember how I was told to call the Pain Clinic by my pediatrician?  I called.  I was told that we have to be referred by our pediatrician, he has to send in the referral and Jack's records and then they will decide if they can help Jack.  I'm thinking the Pain Clinic is not going to be our answer.  I'm also not sure our pediatrician is the best fit for us at this stage of the game.  I need someone who will step up to the plate and try and help Jack, not refer us out.  I've got the name of a few pediatricians who care specifically for kids with special health care needs and who are also palliative care doctors.  It's something I'll work on after we get back from St. Louis.

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Jack has had a decent week.  He's not particularly happy, but he's not super stressed either. He's just kind of "blah".  While I'm not really expecting to get a definitive answer to what is going on with Jack when we are in St. Louis, there's always hope that someone can shed some light on what is going on.  I'll just be happy if Jack can get through the week without the need for bagging.

And, on that note .... you have my rather disjointed update.

Thanks for reading.

Thanks for caring.

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The "Just Keep Swimming" bracelet I ordered

(it's my new mantra)