I wish it need not have happened...

"I wish it need not have happened in my time," said Frodo.

"So do I," said Gandalf, "and so do all who live to see such times. But that is not for them to decide. All we have to decide is what to do with the time that is given to us."

-- The Lord of the Rings, Book 1, Chapter 2


Processing the situation that we have found ourselves in, it is so natural to be like little Frodo.  Shaking our fists at the heavens and yelling why.  Like Job we gladly accept the blessings and reject what seem like curses.  

Why does Hannah have this genetic disorder that undermines that primitive and basic drive to breath in life.  Why does my little girl have to face life with this hurdle.  Why do I, as her parent and co-carer, have to take this difficult road.  Rushing like mad from the hospital to make it home in time to see Matthew wake up and to get him off to school I was thinking dark and angry thoughts about the yoke my family has and about the challenge that it has been to Bonnie - to Matthew - to Hannah - to me.  

I am not an emotive sort of fellow, but I realized I am shaking my fist at the heavens and screaming why... in my own way.  I am angry at God and resent the panic and fear that surfaces when I see Hannah struggle for breath.

As I was driving Matthew to school, he was weepy about us not having been there for him and he said that he was jealous of Hannah because of the extra attention that she gets when she is sick and all the time.  Now, from multiple sources, I know he had a blast the night before with my mom keeping him for us, but his sentiment is valid and resonated with where I was too.

It a moment of grace and clarity, God gave me something for him.  Matthew and I have been in the world of J.R.R Tolkien since he was three years old.  First through vague storytelling, then through more detailed oral versions, and finally starting this year through the original novels.  We recently started the Return of the King, the last book.  He loves Gandalf.

We don’t get to choose what challenges we will face in life.  Some will be avoidable and some will not.  Some will be results of bad decisions and some will be no one’s fault.  The ring passed on to Frodo, not choice or any fault of his own.  He had to decide what to do with his challenge. He rose to the challenge and surprised the wisest and bravest in middle earth by taking the difficult path because it was the right thing-- the only thing-- for him to do.

We didn’t choose for Hannah to need to wear a mask (a representation of her disease).  We didn’t have a choice and it isn’t anyone's fault.  Now, like Frodo, we have a journey to make together.  It’s the right thing and the only thing to do.  And we have to do it together... all of us.  It is OK to not like it-- Frodo didn’t-- and it normal to be scared, sad, or angry.  But it isn’t anyone’s fault -- certainly not Hannah’s or yours.

Thanks Gandalf

Count Your Many Blessings

I have been doing a lot of remembering the last week or so.  The last year has been a blur and learning to keep things in perspective has become an important personal goal for me. 

A year ago today I said goodbye to Matthew and put him on a plane to fly home to Nashville with Grandma to start kindergarten.  As he has gotten into the groove of being in a first grader this year I am wildly cognizant of how glad I am to be here for him this year and how sad I am that I wasn't there for him last year when he needed me.  I am not the best father, but if nothing else, I want to always be there for my kids when they need me. 

I was talking with friends this weekend at the park where we were enjoying watching out kiddos play during the first weekend in ages where the temperature were not so hot that we couldn't really spend much time outside.  They were talking about how kindergarten was going for Matthew's good friend and I was struck sharply by the realization that I have no idea how Matthew did with his two weeks of school.  It burned my heart.  I haven't missed much, but that was a big deal for him that I wasn't a part of.

A year ago today Hannah was in Tulsa being kept alive by a ventilator.  She was beginning to be weaned off of the sedatives and painkillers and was having the rate of her breathing support cut so that her carbon dioxide levels would climb and perhaps the feeling of suffocating might cause her to breathe on her own.  Watching her was so very hard.  It was rough to watch her be mostly out while intubated, but watching her become aware and having to tie her arms to her bed so that she didn't pull out her breathing tube was so much worse. 

We were celebrating every change or possible improvement and were so wishing to know what was going on.  Over the next couple of days she would show improvement in her respiratory drive, but would begin to have what we now recognize were withdrawals for the medication.  At the time we mistook the shakes as seizures.

We would remain in Tulsa for another week and would then have Hannah transported via lifeflight to Vanderbilt.  We would then spend another week at Vandy celebrating lots of small victories and finally being sent home on August 27th with the false hope that this was all a transitory illness that had passed.

We were sent home with a monitor and a child that still didn't breathe consistently when sleeping.  We watched in horror for two weeks as her oxygen saturations would dip dangerously low.  In short, we were a wreck.  Then on the 15th we got a call from her doctor to come in and she told us that she had tested positive for CCHS.  In short, that is when I lost my shit and hope faltered in my heart.  This wasn't going away and this was seriously real.

It would be another two weeks before we changed what we were doing and actually started ventilating our daughter.  Thinking back on all this, the craziest and most dangerous part of the whole ordeal is that month between being discharged and the weekend in the hospital when we titrated her ventilator.   The hardest and most hopeless period was the next few weeks.  Hannah didn't want to wear a mask over her mouth and nose when sleeping.  No big surprise there.  Night after night we would take turns restraining her while she went to sleep.

Life has gotten better.   

It is easy to lose that sense of perspective.  Last night at bedtime I put on Hannah's sensor and then read her the books that she had picked out.  When we were done reading she begged for more, but it was getting late.  I told her it was time to put her mask on and she hid her face behind her hand and onto her pillow... as usual.  Then she acquiesced, and slip her mask into place.  She connected her tube to her mask.  Then turned off her lamp.  It took her a while to fall asleep and I laid with her until she was asleep.  She slept great without waking during the night and woke chipper and happy a little after 6.   Most nights are like this. 

I get annoyed that she has such a hard time falling asleep.  Sometimes I make her go to sleep by herself.  Sometime I get angry about the resistance to putting on her mask at bedtime.  I wish I didn't lose my temper with her, but I do. 

She is doing great.  She puts up with more than most kids I know with a giant smile.  She is loving and loved.  We are blessed.  We get overwhelmed by the special restrictions of living with CCHS and the constant vigilance that seems to be required to keep her healthy and well.  The allergies, sensitivity to heat, special napping considerations, fear of illness, and other considerations that are becoming normal are still annoying at times. 

I need to learn to appreciate the blessings and to take the challenges in stride.  Hannah is healthy today.  Our family is all together and all in all, we are doing great. 

  

Hannah's Big Adventure

So, many people have been curious what happened with Hannah and her exciting three week hospital stay so here is the story in short (by dad):

Hannah has been a really healthy little girl aside from the occasional toddler cold. On Friday August 5th Hannah was in Tulsa, Oklahoma with her brother, mom, and Nonna. They were there visiting her Uncle Hef and Aunt Katie. Friday and Saturday were normal days where the kids were having fun and dealing with both crazy prairie storms and excessive heat. But that wasn't slowing anyone down.

Saturday night Hannah had a hard time sleeping and woke up a lot. By morning she was weak and was having labored asymmetrical breathing. Matthew stayed with Nonna while Bonnie took Hannah to urgent care. By some fluke urgent care wouldn't take Hannah and she was forced to take her to the Children's Hospital at St. Francis. This proved to be a blessing!

Hannah spent the day Saturday being treated for asthma-like symptoms and by evening Daddy was on his way driving the 600 miles to Tulsa. By morning, Hannah was worse and it was discovered that her CO2 levels were climbing dangerously high and her O2 levels were dropping dangerously low "Acute Respiratory Failure". Sunday she was moved to the Pediatric Intensive Care Unit and about 4 hours later she was intubated and put on a ventilator.

Her blood gasses returned to normal with help from the ventilator. Everyone could then take a deep breath and begin to try to figure out what was going on. She was heavily sedated and underwent a battery of tests over the next week or so. They did two spinal taps, two MRI scans, two CT scans, an EMG, an EEG, and they took 50 ml of blood over a period of a week for diagnostic testing. Everything was normal.

She had a nava catheter in her nose that measured her diaphragmatic activity and they started to notice more and more activity. Once they stepped down the sedation they discovered that her breathing was improving. After two weeks her daytime breathing had improved to a promising point, but her breathing would stop when she fell asleep.

After 14 days at St. Francis we were finally able to get insurance to approve a transport and admission to the Vanderbilt Children's Hospital. It took a lot of fighting and some help from some pretty high up friends at Vanderbilt to get her moved. We still have a lot of thank yous to make for that one.

Bonnie and Hannah was flown by Vanderbilt LifeFlight on a King Air 200 fixed wing plane to Nashville on Saturday August 20th. The flight went smoothly and Hannah was in the PICU at Vandy by dinnertime. St. Francis was great to us, but the standards of care and the resources available at Vanderbilt were refreshing.

Once they had her stable and had familiarized themselves with her history, they began working on Hannah with renewed vigor. Dr Mace, our wonderful pediatrician, had been involved from day one by keeping in touch with us and our doctors and getting Hannah's records to anyone that it would make since to show them to. She was now on hand to help the new attending physicians and neurologists to get the full picture of what was going on.

The doctors decided to repeat the MRI scans and EMG tests. News tests were run and scores of doctors visited Hannah. Still no one was any closer to knowing why she went into respiratory failure. Everyone agreed that she seemed to be doing better.

On Tuesday August 23rd they decided to pull her tube out and see how she does. She did great with her breathing, but she didn't really sleep for the next couple of days. The PICU doctors really wanted to see her sleep well before moving her to another floor, but in the end we decided that the PICU unit was the reason she wasn't sleeping. We got her transferred to another floor with her own quiet room on Thursday August 25th and she started sleeping with healthy breathing almost immediately.

By Saturday she was ready to go home. She is doing great and apart for getting the strength back that she lost from being in a bed for three weeks she is getting back to her old self.

The best that they doctors have is that she had some sort of "Post-viral neuropathy" (such as "atypical Guillain-Barre syndrome" that caused "Bilateral phrenic nerve paralysis" through demyelinating that was specific to the diaphragm.

Any questions?

Father's Day...and Mother's Day, Too!

Daddy enjoyed a great Father's Day with his super kids and lots of World Cup soccer:


Matthew and Daddy sporting matching shirts ("Tweets are for Birds") received on Fr's Day, watching Sesame Street on Mommy's iPod.

Hannah glowing after her first homemade curry dinner, made with love for Daddy. She devoured it, of course!

Mother's Day was SO MUCH cooler outdoors, and called for a trip to the zoo...Hannah's first that she noticed the animals. Mommy played hard with her big boy, and everyone had a rockin' good time!


(yes, Matthew picks out those pants himself--whenever possible.)

Tennessee River Camping Trip

We went camping on Memorial Day Weekend with a bunch of great friends.

The kids had almost as much of a blast as I did!

Experienced Camper

Novice Camper

Refuge from thunderstorms with 8 other not shown