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