Merry Christmas

A Bit of the Details

The last week has been a rough one for Jack.  While honesty has always been my motto, I have to admit, it's difficult to share the details of this part of the journey.  I can easily talk about what is going on with Jack, but writing it down isn't so easy.  You would think that living it every day would make it real enough, but documenting it means I can't take it back, I can't ignore it, I can't see only what I want to believe.  Transitioning Jack to hospice came with the realization that Jack is going to die someday.  But, someday was just a day out there, somewhere off in the distance - a day I didn't want to focus on.

Someday is getting a little closer to home.

While pain is always an issue, we've been able to stay on top of it for the most part. He still has break through pain on occasion and it takes quite the arsenal of morphine and ativan to get him comfortable.  The more concerning issue is Jack's heart rate.  It's all over the board and has been much higher than normal, even when he's sleeping and presumably not in pain.  The other change is that Jack is sleeping a whole lot more.  I still struggle with getting out of the mindset of wanting to know what is wrong and how do we fix it.  There simply is no fixing what is going on with Jack.  

After reaching my mental breaking point because I couldn't let go of needing some information, some answers as to what is going on with Jack, I sent a text to Jack's St. Louis neurologist this evening.  She immediately called me and we talked for over an hour.  She is such an incredible source of comfort for me.  She totally calms me down because I trust her.  She knows more about Jack's disease and how it affects him than anyone else.  She reminded me that our focus for Jack is comfort care.  There's a lot we do every day as part of Jack's routine - that has been part of his routine for years, that needs to stop because it's just too hard on him.  As to why the changes we are seeing in Jack, most likely, he's in heart failure.  We knew his heart function had decreased significantly after he saw the St. Louis cardiologist last year.  Given the higher baseline heart rate and the increased sleeping, it appears to be getting worse.  That's part of his disease process.  

According to Jack's neurologist, someday could be tonight, it could be tomorrow or it could be months down the road.  But, it's no longer out there, out of sight or out of mind.  It's beyond difficult to live with such uncertainty.  Do I stay home, do I go to work, do I sit in Jack's room with him 24/7 just in case?  What if he dies when I'm not with him?

What I love about Jack's neurologist more than anything is that she doesn't limit her conversation to just medical information.  We know each other well enough to talk about God and heaven and how Jack will be okay no matter what.  And he will be. 

So, my friends, that's where we are right now.  This is hard. Really hard. But, as my beautiful friends - Jenny, Christy and Sarah (who are likewise facing very difficult medical challenges with their own children) know all too well, 

WE CAN DO HARD THINGS

(even if we don't want to!)

Onward. 

The Days of Our Life

Onward.

More Good Days than Bad

Once again, I'm remiss in updating the blog.  Most of my updating takes place on Facebook - it's quick and easy to update.

Jack has had a run of mostly good days.  He's been feeling and looking better than he has since we transitioned him to hospice. Which means ... we have his pain under control.  He's getting a decent amount of morphine round the clock and it seems to be working.  It's not to say there haven't been some rough days.  After one particularly difficult day, Jack ended up sleeping for almost 24 hours straight.  It scared me. If I had to describe what this "ride" is like, it is very much like those first months of Jack's life when he lived in the pediatric intensive care unit.  The intensity and stress of the unknown on any given day is much the same. What's different is that I'm 15 years older and much more worn down.  I don't have the stamina for this.  I seriously worry about my own health these days.

I want to comment on my post before the birthday post.  I realized after the fact how very raw my words were.  At the time I wrote it, I just sat down and let the words flow.  Interestingly, I heard from several moms who told me that they could relate to much of what I wrote because of what they are dealing with in their own lives with their children, but they hadn't yet been able to put their feeling into words. I spoke for them as much as for myself. And that is why I write, and why I keep it brutally honest most of the time.  I speak from a place that very few can understand, but for those who do, there is a connection that reaches across the miles and forms an inexplicable bond between people who've never even met in person.

Despite Jack's run of good days, I'm still beyond tired because of the round-the-clock meds we have to give him. So, this update is going to be short and sweet.

The sweet part is that we had family pictures taken last weekend and, thankfully, Jack had a good day.  I think the pictures turned out great.  For those not on FB, here are a few of the pictures and at the end of this post is a link to the album of pictures (which will only be available online for a short time).

(I got the idea for this picture from another blog I read. Cool, huh?)

Stay tuned ... I have another very raw blog thought just waiting to be written.

xo

Birthday Notes

On Jack’s 5^th birthday, I sent out my first email to family and friends sharing my thoughts about Jack, myself, and this journey we are on.  I’ve continued this tradition every year since, having moved to sharing my birthday messages on Facebook the last few years.  Jack’s birthdays are always a time of great retrospection, soul searching and emotion and each year’s birthday message has its own unique tone, largely a reflection of the events of the prior year. 

Today, on Jack’s 15^th birthday, there is so much I could share.  However, this year, my words will be shared solely with Jack. 

For everyone else, here is a compilation of my birthday notes from the last ten years.

Jack’s 5^th Birthday

Hi Everyone:

I just want to share with you that Jack celebrates his 5th birthday today. If you had told me five years ago that Jack wouldn't walk, talk, eat or breathe on his own at the age of five, I think I would have crawled into the nearest hole and died. In the beginning of this journey, we all had so much hope. I, along with Jack's doctors, nurses and therapists worked so hard to achieve independence for Jack. Independence from the wheelchair, the feeding tube and most of all, independence from that damn ventilator. But, despite all our hard work . . . it's not to be.

Today, I have new hopes for Jack. I hope that every morning when he wakes up and every night before he closes his eyes, he feels safe and knows that he is loved. I hope that his days on this earth are lived without pain. I hope he achieves a way to communicate his feelings and desires to others. I hope he stays healthy. I hope he accomplishes all that he is capable of.

As for me, my idea of happiness has changed considerably. I'm happy to only have to suction Jack 10 times a day, rather than 100. I'm happy if he only pukes once a week, instead of every day. I'm happy when the DME company gets my supply order right the first time. I'm happy to never see the inside of a PICU again. I'm happy to see his contagious, beautiful smile. I'm happy to hear him laugh. I'm happy if he's happy.

It has taken many years, a lot of tears (and an occasional Xanax), but I have finally come to accept that Jack will always be who he is today - wheelchair, feeding tube, vent and all. With this acceptance, comes freedom. Freedom from always pushing him to do what he isn't physically capable of doing (he's so thankful for that). Freedom from always having to be in control (well, most of the time). Freedom from wanting to have an answer to everything and, most importantly, freedom from having to know Why? With freedom, comes peace.

On a lighter note, it has been said: 

"Life may not be the party we hoped for,

but while we are here we might as well dance"

So, Jack and I are going to dance the day away ~

The last five years have not been easy for sure, yet, I consider myself lucky because I've met so many remarkable people along the way. I feel truly honored to be the mom of such an amazing and inspiring little boy. Thanks to each of you for being part of our journey these last five years.

Jack’s 6^th Birthday

(after Jack disconnected himself from his ventilator and suffered an anoxic event)

Hi Everyone;

Jack celebrates his 6^th birthday today.  As you may remember, last year on Jack’s birthday I shared with you that I had finally achieved acceptance with regard to Jack’s limitations and challenges.  What a difference a year can make.  A year ago, Jack was chasing his little brother around the park in his wheelchair. Today, it takes all he can give just to get his hand to the controls of his wheelchair.  I am renewed in my struggle to understand and accept why Jack must now work harder to achieve even less than he could accomplish a year ago.  Yet, despite his new challenges, Jack still manages to face each day with his trademark smile.  I suppose it’s not fair to ask for anything more.  I want to thank each of you for helping Jack, and for helping me, get through this most difficult year.  Jack and I are forever indebted to you for your continued and unwavering support and encouragement.  Together, Jack and I will tackle another year of challenges and, hopefully, manage a few successes along the way.  Thank you for allowing me to share with you my little hero’s very special day.

Jack’s 7^th Birthday

Dear Friends,

Today is Jack’s 7^th birthday . . . simply, amazing to me.  Jack’s birthday is always a time of reflection for me.  I think to myself . . . another year of “THIS” . . . we’ve actually made it through another year.  There was a time when I hoped that each passing year meant we were that much closer to the end of “THIS” and we could then get on with living the life we had planned for Jack.  Obviously, “THIS” is here to stay and, over the years, I’ve learned to appreciate and enjoy this life with Jack.  Jack is such a gift.  He defines what is important in life – patience, persistence, contentment, simplicity and unconditional love.  He challenges me on so many levels, he keeps me grounded and he gives my life such purpose and direction.  A little boy who can speak no words, speaks volumes by the way he lives each day.  So, on his special day, I share with you the one thing that defines Jack, the one thing that has remained consistent through all of “THIS”   . . . . . . . . “THAT SMILE”!

Jack’s 8^th Birthday

Dear Friends,

Jack is celebrating his 8^th birthday.  What a ride this last year has been.  It has been a rough year for Jack from a health standpoint – with many respiratory illnesses and a difficult recovery from his spinal fusion surgery.  Yet, Jack managed to get through it all with his amazing spirit and incredible resilience.  This year on Jack’s birthday, rather than share with you my words, I share the words written by a dear friend regarding her reflections on Jack – as someone from the outside looking in:

Jack's existence has meaning and opportunity for every person who meets him. Jack is no genetic mistake, his life on earth gives multitudes the chance to be awed by their own good fortune, to be humbled by Jack's patience and strength, and the opportunity to really matter, by being there for him and his family. 

I thank each one of you for embracing the opportunity to share in Jack’s life and for being there for Jack and his family - you matter more than you’ll ever know.

I also share with you a short video I put together which includes pictures of Jack’s journey over the last 8 years.  Click on the link and make sure you have your speakers on.  Enjoy the ride.

(Video “8 Years of Jack’s Jack’s Journey” under Montages – left sidebar)

Jack’s 9^th Birthday

Several weeks ago, I told a friend of mine that this year on Jack’s birthday, I won’t be sending out a birthday letter – there will be no profound words or entertaining video because I’m just not up for it.  While it’s true that I haven’t prepared the traditional birthday letter and I won’t be sending out a email on Jack’s birthday . . . you’ve got to know me well enough by now to know that I couldn’t let Jack’s birthday pass without saying something. 

This year, I don’t feel particularly happy or optimistic, nor do I feel sad or disappointed.  I do feel like there should be something more in life for Jack – I just don’t know what it is.  I don’t know if that something more requires me do take some affirmative action or whether it is as simple as  recognizing and accepting that this is as good as it gets for Jack.  Here is a passage I recently came across in my favorite book that really hit home with me:

It is true that we must experience some successes if we are going to keep on trying things for our children.  Sooner or later we have got to get a hit or, yes, we will quit batting.  But we might not get one right away.  We might not get one for quite a while.  We are going to miss. We are going to strike out.  We are going to have slumps, but we will never get a hit if we don’t get up to bat. 

The first few years of Jack’s life, I got up each day ready to bat.  I took a swing at anything and everything I could reach to help Jack achieve success. The problem was that I defined success to mean no vent, no trach, no feeding tube and no wheelchair.  I struck out on all accounts.  With each passing year, it is exceedingly more difficult to get up to bat because the hits are few and far between.  I no longer approach each new year with a set of pre-determined goals to help Jack work towards.  I can’t define Jack’s successes in terms of measurable progress because then there would be none.  I must now look for Jack’s successes in retrospect; I survey the events of the last year and try to identify those things in Jack’s life that can be considered a success.  It has come to the point where I have to measure Jack’s success on a very primitive level.  Is he happy? Does he feel safe? Loved? Without pain?  Yet, isn’t feeling happy, safe, loved and without pain success enough  . . . for any of us? 

 So, as I reflect on this last year, I find successes in Jack’s smile and his laughter, in a year without surgery or pain and a year of good health.  I find success in the knowledge that Jack is loved and feels the love of many.  Yet, I still wonder … is there more out there? Is there something else I should be going to bat for? Nine years into this journey, I recognize that it’s unlikely I will ever accept that this is as good as it gets for Jack.  I suppose it’s my struggle with the reality that is and the reality I want that forces me to step up to the plate and take another swing.  I don’t always know what I’m swinging at, but I have to keep on trying. 

Jack’s 10^th Birthday

Today we celebrate 10 years of Jack.  That’s a lot of years in “Jack” years!  As I reflect on this somewhat of a milestone 10^th birthday for Jack and think about where we started and where we are today, it’s tough to acknowledge that not much has changed in ten years for Jack.  Yet, if I allow myself to stand back and look at the whole picture and not simply focus on Jack’s progress, I recognize that despite all the difficult times and lack of progress over the last ten years, I really am very fortunate.  Jack has spent minimal time in the hospital since his discharge from the PICU in 1999; we continue to have good health insurance; we have nurses who are dependable and who truly care about Jack; Jack is happy; I have three other seemingly well adjusted and happy children; I am still employed and I am still married. Yes, I have much to be grateful for.  Ten years into this journey and I can finally declare . . .  it.is.okay.  Not easy, not a life I would have chosen, but definitely okay. 

Some of you are probably wondering, who is this person?  I’ve been told by more than one person this last year that I have “mellowed”. To quote one person, I have “mellowed beyond description”.  Initially, I wasn’t sure this was a good thing.  Does my lack of intensity mean that I have given up on Jack?  Should I be pushing him harder or doing more?  Yet, I can say with confidence that “no” I have not given up on Jack, and “no” there isn’t more that I need to be doing.  I finally get that all Jack ever needed from me was to be loved.  I recognize that my relentless pursuit of progress was never really about Jack, it was about me.  It was about what I wanted, not what Jack needed.  I’m done pushing Jack to be anything other than who he is.  It’s no longer about where I can lead Jack; it’s about where Jack is leading me.  And, oh the places we are going!

Jack and I approach the next decade with the resolve to use what we’ve learned these last ten years to reach out to more parents of medically fragile children through The Willow Tree Foundation, and to reach out to our future physicians through a new project - Touchstones of Compassionate Care.  What a privilege it is to make a small difference in this world together with one special little boy by my side.  A little boy who leads me well without ever uttering a word. 

Finally, to those of you who have been in the trenches with us over these last ten years, keeping Jack healthy and keeping me sane …. THANK YOU! We couldn’t have made it this far without each and every one of you.

In closing, I share with you a short montage of “Jack and Mom” over the last ten years. Enjoy!

(Video “10 Years of Jack and Mom” under Montages – left sidebar)

Jack’s 11^th Birthday

Eleven years ago today, Jack and I began our journey down a road on which I spent many years desperately searching for a U-Turn.  I never found that U-Turn, but I discovered so much more along the way: courage, hope, patience, perseverance, acceptance and abundant friendships.  What a privilege it is to be Jack’s mom.

Jack’s 12^th Birthday

As I sit at my computer poised to share my thoughts on this, Jack’s 12th birthday, the word that most predominately comes to my mind is “AWE”.  I’m in awe that it’s been twelve years since this journey began.  I’m in awe of all that has transpired over the last twelve years.  I’m in awe that Jack and I are both still here to talk about it.  Mostly, I’m in awe of the love of life that Jack exudes every day despite all that has been stolen from him by the insidious disease that has riddled his body.  This child who cannot purposefully move any part of his body, save a few fingers; who cannot sustain his breathing without the assistance of a machine; who has never enjoyed the experience of eating food; and who has never spoken the words his mind so clearly holds - this child wakes up every morning with a smile on his face and eyes that sparkle with the anticipation of a new day.  I am in awe of this child. 

My greatest wish for Jack this birthday is that more of the people who cross his path would acknowledge his presence and take a moment to appreciate what he has to say.  Jack may not speak with words, but his face and eyes speak volumes.  If you’d just stop and speak to him and then look (really look) into those eyes, I promise that you will get a response that will assuredly warm your heart and put a smile on your face.  There is a person inside that silent body who has a profound awareness of all that surrounds him.  Take the risk to get to know Jack.  He is so worth it.

Jack’s 13^th Birthday

Jack is 13 years old today. A teenager!  Thirteen years ago I could never have imagined the journey that Jack and I would travel.  Even today, I stand back and still can’t quite wrap my head around where we started and where we are today. Who would have thought?  In the beginning, not many of us, that’s for sure.

Jack is loved, Jack is happy, and Jack doesn’t know any different.  Today, we celebrate all of those things.

Rather than share a bunch of superfluous words, I share a song that perfectly expresses how I feel thirteen years into this journey.

(Video “Jack's 13th Birthday Video” under Montages – left sidebar)

Jack’s 14^th Birthday

Happy 14th 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. 

A Less than Uplifting Post

Clearly, I struggle to update the blog.  Part of me feels like it's the same thing, different day, so what is there really to share. But, the reality is, our days are anything but consistent. This leg of the journey is not linear.  There are many ups and downs.  There is baseline, a rough patch, a new baseline, and repeat.  And so it will continue until the end.

The end.  Isn't that really what we all want to know? When will this end?  I certainly do.  Not because I want this to end.  No parent wants to lose their child.  But, as Jack's neurologist reminded me when I asked her to please help me get past having to know the why of what is going on with Jack ..... we know the why.  Jack has a progressive neuromuscular disease.  Not a new revelation by any means, but also not something that I've focused much on over the years.  Other than the fact that Jack's disease has never allowed him to walk, talk, eat or breathe on his own, he has never been a chronically sick kid.  For the most part, we just accepted (okay - more like, adapted to) his limitations and moved on with living life the best we could.  In other words, his disease was mostly a non-issue.

Obviously, that is no longer the case.

So where are we at this point in time?  After a very rough evening last week and an after hours call to the hospice team, we've now increased Jack's morphine dose yet again and he's now getting it every four hours around the clock.  No more stretch between the midnight and 8 am dose.  Getting up to give the 4 am dose is brutal.  Mark and I flip a coin to decide who has to get up. Not really. We're actually pretty good about divvying up the job so that we are equally tired. That's only fair, don't you think?

It's important for you to know that when Jack is not in pain, he looks good.  He smiles and he's engaged.  It's not all doom and gloom. However, when breakthrough pain takes over  - it's a challenge to get a handle on it.  It's heartbreaking to see Jack suffer. But, the fact is, Jack isn't going to die from pain, so, in theory, this insanity can go on for a very long time.  Which brings us back to the question of,  when will this end?

I haven't a clue.  No one does.  I think it's safe to say that it's not anytime soon.  It could be months, it could be a year. We will continue to deal with ups and downs and pain management issues.  But,  there will ultimately come  a time in this journey when exceptionally difficult decisions will have to be made. Quality of life and end of life decisions are very personal and very private decisions. But, I'm confident that when Jack has had enough, he will let us know.  As Jack's mom, there will be no happy ending to this journey. But, I unequivocally believe in God and in heaven, and I take great comfort in knowing that when Jack is ready to go - he will be going to a most amazing place.  I believe he knows that too.

In the meantime, we cherish him, we comfort him and we love him.  And, I will do my very best to continue to share him with all of you.

xo

Short Update

An update is in order, but this will be a short one.  I've spent the last two weekends traveling and I'm exhausted. I know -  you don't feel one bit sorry for me.  That's okay.  But, as much as I enjoyed the amazing weekend with my sorority sisters at Coronado Island and the time we spent in New Jersey/NYC celebrating my niece's wedding, my sweet Jack was having a very rough time back home. The stress of being away from him when he is hurting cancels out the fun.  Not that I regret taking the time away.  It's just not as fun and exciting as you might think.

It wasn't that long ago when I could say that Jack was having good days and bad days.  Now, it's more like Jack is having good times and bad times in a given day.  Jack isn't having any "good" days anymore.  We've now added continuous oxygen and Ativan to the mix.  I still struggle with wanting to know why?  The one thing Jack's doctor assured me of when we decided to transition him to hospice was that I could still pursue testing and treatment if I wanted to.  At this point, I need to know why his pain is increasing instead of staying the status quo.  Even if we choose to do nothing once we have the information, I still need information. I'm a detailed oriented, need to know kind of person.  Based on Jack's symptoms, I would like to have some blood work done, at a minimum.  It's possible that we won't know any more than we know right now after doing the tests.  But, I'd at least feel better knowing that we aren't dealing with something obvious and treatable that is causing Jack to be so uncomfortable.  I'm hoping to hear back from Jack's doctor tomorrow.  As much as there are upsides to being on hospice, I'm also discovering that access to certain things is subject to hurdles that I didn't have to deal with in the past. It's very frustrating and makes life more difficult at times.  And the last thing I need is for life to be more difficult right now.

I could go on, but being negative just creates negative energy which saps what little bit of positive (and productive) energy I have left.  At the end of the day, all of the people in Jack's life care about him and want to help.  I'm overly tired, sensitive and emotional.  All we can do is press on.  One day at a time.

I want to share pictures of my very special weekend with my very special friends for those who aren't on Facebook, but I'm too tired tonight.  I'll try and post pictures tomorrow, but in the meantime, here is my favorite picture of the weekend - this pretty much sums it up.

A Week of Calm and Connections

Jack is doing okay.  Despite my conviction to cease my "need to wean", I still tried to space out the time between Jack's doses of morphine just a little bit. But, Jack wasn't having it.  So, it's now written in stone - well, actually, it's written on a piece of paper in Jack's bathroom, that Jack is to get his morphine every 4 hours.  No matter what.  Except, between the hours of midnight and 8am, because it's helpful if we get some sleep.  For that 8 hour span, we give Jack a higher dose and, so far, our schedule seems to be working.  And, I'm done messing with it.  Really, I am.

This week has been the first calm week in what seems like forever.  We've have consistency with morphine and consistency with nursing and Jack is having mostly good days.  He's not on such a high dose of morphine that he's out of it.  Although, you can't be on morphine and not be somewhat affected.

This week was also a week of connecting with people who've cared for Jack at St. Louis Children's.  For the first time ever, Jack is having skin breakdown around his trach stoma (for my non-trach friends, the stoma is the hole in the neck where the trach tube is inserted). The trach tube has created a sore in his neck that is above the stoma.  The sore is a ridge in the skin and the trach tube now pulls up and sits in the ridge. I have a picture I could share, but it might be too much information for most of you.  I emailed the trach nurse at St. Louis Children's on Sunday night and I received a call from her by noon on Monday.  Have I told you how much I love SLCH?  She gave me some suggestions and said that what we are seeing is not unusual for a kid who has had a trach as long as Jack has.  It was great to talk with her and to catch her up on what is going on with Jack.

I also sent an email to Jack's former St. Louis pulmonologist, who is now practicing in Florida, to give her an update on Jack. When she emailed me back, she shared this with me:

"Give him a hug for me and know that I am thinking of all of you. I see him every day as I keep a picture of him on my office door." 

Totally made me tear up.  And, I know that she isn't the only former doctor of Jack's who has his picture on their office wall.  It so comforting to me that the people who cared for Jack so many years ago still think about him and hold him dear to their hearts.  That's what is missing here. The doctors here have never connected with Jack - or me.  I get why, for the most part (although, that doesn't excuse this).

Last year, when Jack was inpatient at PCH, I was discussing this lack of connection with another one of Jack's former SLCH doctors and, as I explained to him:

"You have to remember that the connection I have with you and several other of Jack’s doctors is based, first and foremost, on your connection with Jack. The doctors we encounter here at PCH don’t feel any connection to Jack.  Jack is not that cute little baby that everyone fell in love with 13 years ago.  It’s not that the people here at PCH are not good people, it’s just human nature to be attracted to babies, not 13 year olds with neuromuscular diseases."

Anyway, it doesn't really matter at this point. The fact is, those doctors and nurses who connected with Jack so many years ago in St. Louis still feel connected and they still care.  At this point in the journey, I probably need them more than Jack does and, thankfully, they are still showing up for both of us.

Speaking of connections, bright and early tomorrow morning, I'm hopping on a plane bound for San Diego.  I'm spending the weekend with three of my sorority sisters who I connected with online many years ago.  All of our kids are trach kids and three of us are bloggers.  This will be the first time we meet each other in person (except for me and my friend Sarah - we worked together on the TouchStones program at CHOC).  I'm looking forward to a weekend of respite and relaxation on the beach and a weekend of making memories with some wonderful ladies. I know the minute we see each other and start talking, it will be like we've known each other for years.  Because, well, I guess we have.

If you are on Facebook, you'll no doubt see pictures.  For everyone else, stay tuned.

xoxo

~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~

Seriously, who couldn't fall in love with those eyes?!

It's A Process

When you are the parent of a technology dependent child, your focus very early in the journey (when you don't have a diagnosis and you still have hope) is to do everything in your power to get rid of the technology.  You get in "wean mode".  I spent the better part of the first three years of Jack's life trying to get him off the ventilator.  The longest he could ever go off the vent was about an eight hour stretch. In retrospect, he should never have been off the vent that long - or at all.  But, being the goal oriented, task master that I was, I wasn't going to cut the poor kid any slack and he gave it his all during those times he was breathing on his own off the vent.  If he could have talked, he no doubt would have told me to give him back the damn vent. If he could have moved, he no doubt would have grabbed the vent circuits and hooked himself back up.

Even after I accepted that Jack wouldn't ever get off the vent,  I still couldn't get out of wean mode. If I couldn't get him off the vent completely, then I was at least going to work on weaning down the amount of support he needed.  He didn't get very far.

When Jack would get sick and require increased vent settings and supplemental oxygen, all I could focus on was getting the vent settings back down to his baseline and getting rid of the oxygen.  Until recently, Jack has never been on routine meds.  My emphasis has always been "less is more".  My need to wean was never meant to torture Jack, but to have something to work towards.  All parents want to see their kids make progress, it's what gives us a sense of purpose and accomplishment.  It's what gives us hope.  Despite Jack's ultimate diagnosis and lack of progress, I continued to wean and to push because I still had hope.  Hope - it's a funny thing. 

Getting out of wean mode is a process.  It takes time.  And that brings me to where I am today and Jack's need for morphine.  Initially, Jack needed a high dose of morphine around the clock to control his pain and allow his body to rest.  Eventually, wean mode kicked in and I tried to get Jack down to morphine on a PRN basis.  The problem with that was, when the pain comes, it hits hard and Jack ends up in respiratory distress before the morphine can kick in.  We went to scheduled morphine every four hours.  Jack did well. So, of course, what am I thinking?  Wean down to every six hours or maybe he can make it to every eight hours.  I know this sounds crazy to most of you, but all I can say is, it goes back to feeling like you are making progress and with progress, comes hope.

The bottom line is, Jack needs morphine on a scheduled basis and he needs it more often than every eight hours. This is what he is telling us. I need to be a better listener and I need to change my way of thinking.  Letting go of the "need to wean" mentality is hard to do.  But, I need to do it for Jack.  Forget progress, forget hope, forget a sense of purpose or accomplishment. It comes down to what will make Jack comfortable.

As my friend, who is one of the docs on my support team, recently shared with me about weaning Jack's morphine:

" ... could not agree more with getting away from weaning as victory.  Victory over what? The disease? We will all have to let our kids with CMD go gently into the night though we have spent the better part of our lives making every effort to do just the opposite - attempt to defy progression, make small concessions but not without a fight and go down paths with no right answer  . . . "

And she is absolutely right and I love her for her honesty.  I can't say enough how blessed I am to have the most amazing people guiding me and supporting me in this leg of the journey.

I'll close by saying to all of you, despite the tone of this post - don't be sad.  Jack's pain is controlled and he is doing well.  When he isn't hurting, he is happy.  He really is so easy to please  - "Give me my vent and give me my morphine and life is good, Mom." 

Okay Jack, you can have your vent and you can have your morphine.

xoxo

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A couple pictures of Jack off  the vent 

(for those who are wondering, the tube going to Jack's trach is not a vent circuit, it's hooked to a compressor that is delivering humidified air. Jack is breathing on his own in these pictures)

How We Are Doing

So we are two weeks on hospice and I think it's fair to say that Jack is doing much better than his mother.  Then again, he has unfettered access to morphine and, well ... I do not.

Jack is doing better. Being able to control his pain and allowing his body to rest has really helped bring him back from that far off place he escaped to when he was trying to deal with the pain.  He is much more alert and attentive these days and is even sharing smiles on occasion. I've been able to wean his morphine down from the maximum dose every four hours around the clock to PRN with a lower dose.  However, every time I think I might have him weaned down to needing no morphine, he lets me know that is not the case.  He's still needing it a couple of times a day.  The million dollar question is why? I've slept very little the last two weeks because I'm relentlessly haunted by the "what ifs".  What if we are missing something obvious? What if whatever is causing Jack's pain is a simple thing that can easily be treated? What if he was being seen by his St. Louis doctors instead of the doctors here?

This, my friends, is where faith comes into play.  Despite all the "what ifs",  my gut tells me that the decision to transition Jack to hospice is the right one.  And, I believe with all my heart that my "gut" is God driven. If I can't believe that, then I've got nothing to go on and I desperately need something to go on.

Being on hospice does come with a few perks.  For one, the coveted morphine is delivered right to your door - no trips to the pharmacy in the middle of the night required.  Your hospice doctor also makes house calls.  I had scheduled an appointment with Jack's pediatrician/palliative care doctor awhile ago, but because Jack is on hospice, she came to the house yesterday - along with the hospice nurse and the social worker.  A bit overwhelming, but also much appreciated and welcomed.  It's a bit of an adjustment to get used to so many people being there for you. I mean really being there - in person, for you.  It's uncomfortable and good all at the same time.

As for me, I used to think I had a pretty good handle on juggling the whole medically complex kid + regular kids + working full-time thing.  Not so much anymore. I completely forgot Eric's parent-teacher conference last week.  But, all I had to do was throw out the "Eric's brother was transitioned to hospice" line and it was all good. Another perk of being on hospice - people cut you a lot of slack.  And lately, I've needed to be cut a lot of slack because I'm missing a tremendous amount of work due to lack of nursing coverage.  The upside to the lack of nursing is that I'm getting to spend a lot of time with Jack - just mom and Jack time.  It's been nice ... and a lot of work.  It's been years since I've done a trach change, changed out the vent circuits and other equipment that needs to be changed out every few weeks or even given Jack a bath, because his nurses usually do those things.  But, I so appreciate this time I'm getting to spend with Jack that I otherwise wouldn't have. Life has a way of working out how it's supposed to, even if it doesn't seem like it at the time.

Mark has been out of town all week and won't be home until late Sunday, and, as much as I appreciate all the quality time I'm getting with Jack, I'm in desperate need of some sleep.  So, Jack and I will be heading to Ryan House this weekend where I can sleep and he can get the love and attention he deserves.

Well, that's the update from here. Sorry for the long break in blog posts. It's difficult to put all that I'm feeling and thinking into words, especially when I'm so darn tired.  Most days, I seriously have to remind myself to BREATHE.  I do feel like Jack is in a stable place at the moment. We are able to control his pain without snowing him - I'll take that.  One day at a time.

Onward.

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Just some pictures of Jack .... Just because.

Circa 1999  (~ 9 months old)

Circa 2000  - Jack is in his stander and Elmo is strapped to the oxygen tank stand :) 

The "H" Word

Picking up where I left off a couple of posts ago ...

After two nights and two days of almost no sleep because of pain we could not get a handle on, we took Jack down to the emergency department at 4:30am Wednesday morning. After spending most of the day in the emergency department and having a thorough work-up of tests, there was nothing they found that pointed to any specific thing that would be the source of Jack's extreme pain.  Mark and I decided that we weren’t going to admit Jack and go on a fishing expedition to try and find what could be causing the pain.  If the source wasn't obvious, we weren't going to put him through a bunch of invasive tests that may or may not give us any useful information.  This decision was based on all that Jack had been through the last year and a half and what had already been done as far as surgeries and procedures.  

The decision was made to transition Jack to hospice.  Hospice is a scary word, I know that.  I've been avoiding the word ever since we transitioned Jack to palliative care. But, it’s important to understand (as I’m trying to understand) that hospice does not only deal with end of life care, it also provides comfort care. It is possible to be discharged from hospice. Jack may or may not come through whatever issue is causing him the pain.  But, by being on hospice, we can make sure Jack gets the pain control he needs without visits to the emergency department and without hospital admissions. To be clear, we are not precluded from pursuing further testing if we change our minds down the road. 

At the moment, Jack is getting morphine round the clock and, as of today, he is significantly better than he was just two days ago now that we have the pain controlled. He is awake and alert, but certainly not his usual self. I really hate not knowing what is going on. It’s beyond my understanding why the doctors can’t readily find the source of the pain.  But, I’m fortunate to have the support of a trio of doctors who are also my friends – Jack’s St. Louis neurologist, a PICU intensivist and the founder of Cure CMD.  All exceptionally bright people who know more than anyone else about Jack’s disease and who, most importantly – know and truly care about Jack and me.  They assure me that the tests that have been run would find the source of Jack’s pain if it was something that could be easily found and treated. I trust these people implicitly and I will continue to consult with them as things progress. 

I thought long and hard before sharing this information.  However, I’ve always been honest in sharing this journey, so I can’t stop now just because the topic is an uncomfortable one.  I can say that today things don’t seem as dire as they did a few days ago. But, I really haven’t a clue where this is heading. 

That's the update my friends.

Onward.

__________________________

P.S. For the second year in a row, I was unable to participate in the Disney half because of Jack issues.  I think Jack has a problem with me going to Disneyland without him!

Dear Attending

Dear Emergency Department Attending,

My son recently came under your care.  He was the "14 y/o male, dx muscular dystrophy".  He was the child in the wheelchair, trached and vented, with the contracted limbs and the drool cloth under his chin because he can no longer swallow.  He was the child staring off into space, lost in a sea of pain.

Next time, walk into the room and talk to me.  I understand doctor speak and, yes, I'm going to ask pointed and tough questions.  I don't expect you to have all the answers or fix all that is broken with my son.  But, when it's time for me to make difficult decisions regarding my son's course of treatment, I need to know I'm talking to the physician with the most experience.  I need to know I've asked all the questions, received expert answers and have left no stone unturned.  I need to know I am making an informed decision. After fourteen years of living in the medical trenches, I've earned the right to your presence.  I've earned the right to ask you questions and question your answers.  Respect that right.

Next time, walk into the room and touch my son.  Put a stethoscope to his chest. Examine him.  After fourteen years of living with a disease that has stolen everything from him but his spirit, he has earned the right to your presence.  He's earned the right to your touch, your eyes, your ears, your experience and, I dare say, your heart.  Respect that right.

Onward.

Deja Vu

(picture courtesy of my friend Eric Fisher)

At the time we transitioned Jack to a palliative care doctor last November, we were given a script for morphine.  Since November, we've had to give Jack morphine here and there because of pain, but he's not needed it on a routine basis.  For the past few days, Jack has been in significant pain. His heart rate is high and his sats are low enough to require supplemental oxygen.  As we always do when his sats are low, we approach it first as if it might be a respiratory issue.  We give repeated rounds of the Vest , breathing treatments and extra suctioning.  When that doesn't resolve the problem, we know the low sats are pain related.  I gave Jack the morphine in the amount and frequency as written. He got minimal relief.  I decided to sleep with Jack last night so that I could keep an eye on him. As I lay next to him watching him struggle to close his eyes and get comfortable, it was killing me because I just didn't know why he was hurting.  After watching him for awhile, I couldn't stand it anymore.  I needed to rule out anything obvious before I jumped to the conclusion that it has to be the damn kidney stones.  Knowing how fragile Jack's bones are, I always start there.  I moved Jack's arms and legs to see if anything hurt when moved.  I noticed when I got to his left leg that his knee was slightly swollen and it didn't look right. I heard a popping sound when I bent the leg at the knee.  Jack didn't wince like he has in the past with a broken bone, but he definitely didn't like it.  My gut told me that his leg was broken.  Jack eventually fell asleep, although his heart rate hovered around 100 all night, telling me that he was still in pain.

First thing this morning, I called and was able to get Jack in to see his orthopedic doc. The x-ray showed that he has an impact fracture of his left femur - a very obvious fracture on x-ray. This is the second time he has suffered this kind of fracture.  They splinted Jack's leg with a knee immobilizer and sent us on our way.  A couple of people have asked how or when the fracture happened.  I have no idea.  I don't know when it happened, other than it likely happened within the last week according to the orthopedic doc.

Jack continues to be in a lot of pain despite having his leg immobilized and getting morphine as often as he can.  I called the palliative care doctor this afternoon and asked if we could increase the amount of morphine because the dose as written wasn't touching Jack's pain. She doubled the dose and as I sit here right now next to Jack - he is still in pain.  His heart rate is high and he's requiring oxygen to keep his sats up. We cannot seem to get him the pain relief he needs.  I find it difficult to believe that the broken leg is causing the amount of pain he is still in. So the question is, how much time do I give it before I haul him to the ED to find out if it's something else? And where do we start looking? Do we consider kidney stones as the source even though he had a CT scan just a month ago that showed that the stones were stable?  I can't begin to adequately articulate how incredibly stressful and disheartening it is when you have a child in pain who cannot tell you where it hurts.  I just want this child to get some pain relief so he can get a restful night's sleep.  He is so tired and he can't get comfortable.

I'm supposed to leave for LA on Friday for the Disney half marathon.  I think Jack must not want me to do this race because, if you recall, this time last year we were inpatient at PCH and I had to miss the race.  I'm still optimistic I'll be able to go to LA, but I guess time will tell.

Que sera, sera.