Absence Makes the Heart Grow Fonder

I am sitting here in MCV yet again, waiting for William.  His port has developed issues over the last couple of weeks.  He was scheduled for a port study this morning, but after the issues of the weekend, it is being replaced...and it is taking forever because the previous port was deeply installed and is taking a while to get out.

So while I am actually sitting still, I figured I would finally log onto the blog and see about updating it.  And then I realized how long it has been since that was actually done.  So, how about the short version?  Is there such a thing?

Will was back in the hospital in April in what was one of the hardest admissions we have ever experienced.  It was really the first time that an attending just would not even try to grasp the situation which resulted in Will's care being less than what was needed.  The nutrition department decided to eliminate almost everything from his menu due to his food allergies, which resulted in their providing significantly fewer calories than he needed, which meant that I was buying his food and bringing it to him.  Needless to say, by the time we got home, my temper was in rare form and I was ready to shake some trees to ensure that this never happened again.  Dr. T graciously heard my complaints and is managing the situation for which I am grateful.

The brain injury is creating fewer issues in my life, but the night vision issues and fatigue and occasional "what is that word?" seem to still exist.  I am grateful for improvement but will be very happy to have this fully resolved.  I am once again cleared to run, but trying to figure out how to fit that back into our lives seems challenging.

Caroline currently is not a candidate for surgery.  While the mito question still looms, the surgeon in Ohio believes that her brain stem is fine and will not require intervention.  Good news is rare in our world, so it was really hard to believe, but we are indeed grateful for it!

The best news...of course is saved for last.  William "Cole" Winyard arrived Saturday early morning.  Caty had just sent the message that she thought labor was a myth, but alas, she learned otherwise.  :)  While pregnancy can feel like it lasts forever, it really doesn't...and at the end, you are blessed with a beautiful son. Cole weighed in at a lovely 8 lb 2 oz and appears healthy and happy.  Nana (I) certainly enjoy(s) holding him! 

So, Will and his new port are about to return to this frigid room we call recovery. I will try to be more faithful in the updates.  Life has just seemed so challenging in the last few months...

UMDF Central VA

So, for the last few years, I have heard about, chatted online with, and read about other people with mito...but we almost never meet anyone face to face who lives a life similar to ours...someone whose life revolves around IV fluids, doctor appointments, medications, insurance companies, and medical bills.  Rising gas prices affect us not because we may have to cut soccer from our schedule, but because we have to figure out how to cut something from our budget because the doctor appointments can't be cut and the medical bills still need to be paid.

Tonight, we got to meet them...a mom with whom I have been facebook friends but have never met, someone who reads the blog and was was so encouraging about how it helped her, and the mom who organized the new UMDF chapter who also helped pave the way with some of the local physicians.  There was a room full of parents who truly understand...and it was so encouraging to see that we are not alone.

I often contemplate a post to the blog, but I fear it is just another repeat of, "This is hard, but God is faithful." and that people won't want to read it anymore, so I don't write.  It is hard.  It's painful to watch your child sitting in a chair looking awful, and other than adding in fluids, there's just not much you can do about it.  You fear the worst, pray for the best, and fight with all your might against an invisible enemy.  But to know that putting it in words helped even one mom was such an encouragement.

So thank you, Judi, for having a vision for a Central Virginia UMDF chapter.  I know the time needed to get the job done was time that you didn't sleep.  Thanks to the parents who showed up.  And thank you, Dr. Cohen, for willingly dedicating your life to caring for and working toward a cure...or at least help...for those with mito.  You make a huge difference in our lives.

Living with this disease is, to say the least, unimaginably difficult.  Knowing that there were others out there is helpful...you can post questions and get answers and know that you aren't alone.  But actually sitting in the same room with others who understand?  There are not words...

Stubborn, maybe. Hard-headed...NOT

When we do something, we do it right.  We Colemans are not halfway kind of people.  We didn't set out to be, "If you are gonna go...go BIG," but we apparently do it without realizing...it is as easy as breathing.

So two weeks ago, while I was talking to the pharmacist with Will's IV company and rescuing my granddaughter from the wrong side of the bed where she was stuck, I managed to slam my head into the corner of the dormer in Caroline's room.  Next thing I know, I am face down on the trundle bed, still talking to Lonnie, Chloe is still screaming, and I am trying to sound as if everything is perfectly fine while discussing how many bags of fluid Will will need this week.  Here, I am in intense pain and can't hear for the screams, but it is all fine.  Really.

Well, I ended the phone call, rescued the child, and wrote some ridiculous facebook status about my life being a country song.  Went through the day with a sore spot on my head and a developing headache, but what do you expect when you slam your head into a dormer corner?

So I drove the kids into town and waited for them to finish their lessons while the headache worsened and I was getting really tired.  Grandkids were napping in the van, so I sat quietly and waited.  Having a boatload of children will make one tired, so again, no alarm bells.

But on the drive home...alarm bells started clanging really loudly over the ringing in my ears and the dizziness and nausea that were quickly setting in.  I pulled over, texted that info to Billy, then drove home.  The rest of the night is a bit of a blur.  I could type (uses a different pathway of the brain) but speech was getting challenging.  When Will asked me a question and garnered a blank stare, he texted Billy to find out when Billy was taking me to the hospital...where I could not remember the year (Um, I know it is in the 2010's...I think.), my phone number (which I have had for at least 15 years), or my social security number (which I have had for a lot MORE than 15 years).  Hmmm...oh, and just getting to the ER involved turns and curves which required me to laboriously inform Billy that if..he...did... not...take...them...more...slowly, ...I...was...going...to...throw...up!

The CT was clear, there was no brain bleed.  They gave me pain medication, I requested the wonder drug Zofran for the nausea, and they sent me home to rest quietly.  Which I then proceeded to do for the next...week...and still was not right.  So back to the doc we went...who then sent me for an MRI...which then set off this weird shaking, staccato speech, rapid blinking event that landed me back in...the ER...on my birthday.  Really.  I know how to have fun...on my birthday...and this was...NOT ..it.

So here we are...two weeks later, and I am better but still not...back to normal.  I can almost consistently talk in complete sentences that don't take five minutes to deliver. I still cannot multi-task well, and lots of noise and bright light are still painful.  That buzzing in the ears is annoying, but it stays to a low roar until I get tired, which is when most of the symptoms increase in intensity.  Driving is still an issue, especially at night with the lights. You don't realize how MUCH you are multi-tasking while driving until you can't multi-task. 

So I sleep...a lot...and rest...a lot...and have friends who handle the driving...a lot.  Fortunately, we have a respite home health nurse who has been helpful with William who has been atypically cooperative by being moderately stable during this whole event.  We don't know how long my recovery will be...we were essentially told a week to a year....we are just hoping that it is closer to a week (which I realize has already passsed...a couple of times!).  I have a race in 6 weeks...it would be kinda nice to run it...actually...it would just be nice to  this run year...and not IN a year.

Not What We Expected

So here we are...still in the hospital for what I thought was going to be a fairly quick, routine admission.  I thought Will needed continual fluids with carnitine every 8 hours for a few days, then he would be back at baseline, and all will be well.  That isn't quite the way it worked out.

This admission has been anything but routine.  Not only is Dr. T unavailable, but the team of physicians that were treating Will were essentially unaware of the challenge that he can provide.  (Just ask his nurses... "May I take your temperature?"  "No, it is mine and you can't have it!")  For some crazy reason they really do like him, and they have treated him for long enough that they have a healthy respect for his medical condition and how fast his status can change...especially around 4 every morning.  The residents were skeptical, but their learning curve was fast and steep.

So despite all of the early challenges, things were finally settling in.  We had consults consulting and running tests, and fluids galore entering his veins.  His numbers were looking better...until 4 am...when he tanked...every night. 

Well, interestingly, he didn't tank last night...but that was because of a test that was being run that basically gave a jolt to his system to see how his adrenal gland would respond.  The short answer is...it didn't.  His initial labwork showed that his cortisol level was extremely low, and after the jolt they administered, it should have skyrocketed, but instead, it barely rose to the low side of normal. 

Dr. K, standing in for Dr T this week, was standing beside the endocrinologist when he stated that news, and she looked at me and said, "Not at all what I expected."  Well, apparently that makes three of us.  But upon reading about it, it makes absolute sense.  Will has been receiving more and more infusions but he is responding for shorter periods of time.  Something had changed, but I didn't know what.  I mistakenly thought that he had just fallen too far off baseline and needed time on continual fluids to give his body a break and return to "normal".  Not happening.

There is no cure here.  Man, but that sounds famililar.  And the question that everyone is asking is...what happened.  Mito happened.  The strain of the mito not working sufficiently has affected yet another system.  Progression happened.  We know that mito is progressive.  We know that things can change on a dime with it.  But when a doctor walks in and tells you that you have essentially lost yet more ground to this heinous diesease, then it hits like a punch in the stomach. 

It took a while for the news to really set in today.  I heard it, but I didn't really process it.  Then when things got calmer, I started reading and talking to the nurses.  Then it hit me.  Wow.  This does really change things.  This takes his fluid management to new levels.  When he gets sick, we won't be able to keep him home.  He will need to be in the hospital so his levels can be supported appropriately.  That means more hospital time...more family strain...more grief...and honestly, more work.  Tossing this lovely little variable into a fragile system really is incomprehensible to me at the moment.

What does this mean for today?  It means I am going to go to sleep soon so when his alarms start beeping at 430 I won't be cross eyed.  It means we will be here until the steroid we are now giving him reaches a level that helps stabilize his blood pressure.  That means we may go home tomorrow (Wednesday), and we may not.  It means we get to add a specialist and a medication to his current (what I thought was full!) arsenal of specialists and medications.  It means that caring for Will just got a little more...challenging...and not in a good way.

God is still on His throne, and the sun is still shining way above the clouds.  But, here, at the moment, it is gloomy and dark and stormy.

Middle of the Night Musings

It is 430 am.  I am sitting on my "bed" in the PCU  watching Will sleep while keeping an eye on his monitor.  I have had only 3 hours of sleep a night since our arrival here on Thursday, so why I cannot return to sleep is a bit of an enigma.

I sit here, watching his blood pressure and heart rate drop yet again and my heart is heavy.  While we are in the hospital, Will is fully loaded up on fluids and carnitine.  He basically is returned to his best possible physical condition.  Most people leave the hospital and need a period of recovery time.  But Will is at his best.  So, if at his very best, his vitals drop so significantly, then what are they doing at home while he sleeps when his vitals are typically lower?

We have had endocrinology and cardiology consults during this admission.  More testing will be completed today.  But I doubt they will find much of anything.  Between the mito and the brain stem issues, his autonomic nervous system is not going to function properly.  This is all likely just more of the same...a progressive disease complicated by a brain stem that is apparently not recovering from being compressed. 

The doctor is tossing around options, like sending Will home with continual IV's.  Lots of people live their lives always attached to their line. But Will has already stated that if living a more active life involved an IV every day, then he would sit on the sofa. As his mom, that was a difficult statement to hear.  I can only imagine what he will say if/when he is told that continual IV therapy is the next step for every day life...not just an active life. 

There are not words to express the emotions that flit through me during the middle of the night musings.  I listen to the beeps and alarms and realize that they are alarming for Will.  His bp is low.  His heart rate is low.  I fear what may happen if they drop too much lower.  I know that Will is in God's hands and that He has appointed a time for Will to be with us.  But I want to scream and cry and I want to pretend that he is just sick today and after this admission things will be better.  I want the doctors to find a reason for the precipitous drops in vital signs.  But I really do know that this is just more of the same.  I just pray that I have the strength to hear and Will has the strength to handle whatever news they give us...and that our family can somehow find the strength and desire to love and support one another in the midst of it all.

The Inevitable

Not sure why I delude myself into believing that I can postpone or avoid the inevitable, but as I sit in Will's room at MCV, I have lots of time to contemplate it.  Lots.  Of.  Time.

I have this incurable need to make everyone happy, and for me to say that Will needs to be admitted means that pretty much everyone in my household is less than thrilled.  Yes, they love Will and want what is best for him, but it makes life hard all the way around.  Billy is at work fielding issues from home.  Erlene has an extremely busy week yet is sacrificing her time to help with my littles who would like their mom home.  Will, of course, would like to be anywhere but the hospital, and the older boys get to fend for themselves or care for younger siblings which they have had to do way too often.  But, there are times when the inevitable has to happen and so here we are.

Dr. T is out of town, so we are pretty much being managed from a distance through a doctor that doesn't understand (and who apparently did not read his chart before entering the room) which makes life...challenging.  We were put in a real room (Dr. T is gone, so no one realizes that he is supposed to be in a monitored bed...but since we have walls, a door, and a window, we are not complaining...too much) but unfortunately, the nursing staff is not familiar with us, with Will's unique challenges, nor are they working very hard at understanding.  Combined with their inability to meet his dietary needs, this has become a really frustrating experience. 

It is so funny how we grow so displeased with what we have...and want something different.  We wanted a room, a real room.  Well, we got that, but it is at the sacrifice of better care for William.  So what we wanted wasn't necessarily what was best for us.  Hmmmm....

That being said...while it has been an experience fraught with frustration with residents (really?  We have previously had issues, but they have been resolveable.  Not this time!) and less than stellar monitoring of his condition, we are where we need to be.  He is on fluids around the clock, carnitine every 8 hours by IV, and he is back on his meds.    He was in really rough shape this morning when his bp, heart rate, and body temp all plummeted, but he is slowly coming back up to levels that aren't sending the nurses to the phone in a panic. 

How long will we be here?  No clue.  I wish we had better information, but we don't.  I miss Dr. T and her infintely vast knowledge of and care for Will.  :(  I am thankful for Abigail and Chris willingly staying with the kids and grandkids yesterday while Will and I headed to the hospital.  I am thankful for insurance that allows us to be here (despite now officially meeting that  insane deductible in one fell swoop!).  And I am thankful that Dr T is a phone call away.  I just wish all of this weren't necessary.

Remember Me?

Yes, it has been a while.  I like to think it's because I've been incredibly busy, which I have, but a good part of it is that I have to actually sit and contemplate what to say, and there are times where dwelling on the reality is just...too hard.  You can make it through the day by taking it minute by minute, but if you sit down and dwell on the full reality it's all just overwhelming.

Will attended a friend's birthday party in December.  It was cold, and the party centered around a full blown airsoft battle, and Will really wanted to go.  So, his brothers helped ensure he had sufficient gear, he spent the afternoon running through the woods shooting his buddies, and he had a blast.  One of the moms asked if Will was going to handle this ok, and I shrugged and said, "He'll be sick in two days, but this is the most fun he's had in ages."  It was also the most activity he had had in ages.  He has been in physical therapy for months, but Will was challenging his body in ways it hadn't been challenged since his Make-A-Wish trip.

Two days later...he was sick.  When he gets sick, he gets really sick.  And he crashes.  A lot. The next two weeks became a daily battle to keep him out of MCV while knowing it's really what he needed...but he kept looking at me and stating that he wants to be home for Christmas.  Can you blame him?  I just can't.  So we gave lots of IV fluids and prayed and gave even more IV fluids and prayed some more.

And he has been really difficult to stabilize since that time.  We keep infusing more fluids, and he just keeps crashing.  I talked with his physical therapist today and he recommended that I call Dr. T tomorrow.  The question is...do we put him in the hospital for a few days to give him a boost that will hopefully get him back to baseline, or do we just keep doing what we are doing?  What is the definition of stupidity?  To keep doing the same thing expecting a different result?  That's not working for me so well...When he gets 5 IV's a week, which we've been doing for a few weeks, then maybe we need an alternative solution?

So tomorrow I'll be calling the amazing Dr. T and seeing if she has any pearls of wisdom.

As for the lovely Miss Caroline...well, I'd like to give you answers.  But I don't have any to give.  She clinically looks like a candidate for surgery, but the neurosurgeon here states that her images don't support that conclusion.  We do have an appointment in Cinci in May with Will's neurosurgeon, so we'll see what he has to say.  She is currently at a sleep study to determine if sleep apnea is an issue.  It would provide another piece to the puzzle of how well her brain stem is working...Stay tuned for those results...I may actually blog them before June.  :)

If you have time and want to see what Chris is up to...check out http://candidcameraphotography.blogspot.com/2011/12/smoke-photography-next-up-how-to-take.html or http://www.wix.com/cmc451/candidcamera/main-page#!__main-page/about.  He's done some amazing things with his new camera...