Overwhelmed

I arrived home from work on Friday night, the eve of Christmas Eve, feeling far from in the Christmas spirit.  For obvious reasons it has been difficult for me to muster up a festive mood this year.  My commute to and from work on some days is my only chance to relax, listen to music (adult, not Ellie's), and think.  Most of the time a portion of the drive is spent crying.

When Owen was diagnosed and we returned home from the hospital, Ellie caught me crying one day.  She immediately started to cry and from then on I have done everything in my power to not cry or act sad in front of her.  So, that leaves me my commute to and from work to cry in the privacy of my car without bothering anyone.  Sound depressing and honestly, it is. On Friday, my gloomy mood quickly disappeared when I walked through my front door.

The elves were busy at my house on that afternoon while I was at work.  I arrived home to hear that Santa had just stopped by to meet Owen and have a picture taken together.  The back story to Santa's special visit is that Owen slept through their first meeting, which had happened the previous Saturday.  Bernie, who runs the daycare which Ellie attends, holds an annual party for all of the kids and their families.  Every year, Santa marks it on his calendar and shows up to have pictures taken and give out a few gifts.  Ellie was willing to give Santa a hearty high-five, but Owen was sound asleep. If you have learned anything from this blog then you know we were not going to wake him up, even for Santa. I commented in passing that I was disappointed that we did not get a picture of Owen sitting in Santa's lap, and like magic, a week later Santa appeared on our doorstep.  Bernie must have his direct number to orchestrate such a wonderful surprise so close to Christmas.  Here is Owen's very special picture:

Hearing this story started to lift me from the down mood I was in, but there was more joy to come.  One entire section of my kitchen counter was covered in gifts.  There was a gift basket from friends in one of Ellie's playgroups, cookies galore from neighbors and members of our church, an enormous dish of homemade lasagna, and gifts for Owen, Ellie, and me from many special people in our lives.  It really made me pause and think about how fortunate our family is.

Owen's illness is sad.  There just is not a positive spin to put on what has happened to him during his very short time with our family.  But, through this journey, we have been overwhelmed with offers of support, meals, guidance, and love.  We are truly grateful.  Our Christmas was a happy one where lots of wonderful memories were made. I hope everyone else was touched this weekend by a little bit of joy as well.

Home Again

Owen has completed his second and hopefully final stay in the hospital.  I drove him down on Monday, December 19, 2011 and had him admitted to the PICU at noon.   The goal was to capture one of his seizure-like episodes on an EEG to confirm they are actually seizures.  About an hour after our arrival, the EEG technician arrived to place the sensors on his head.  This time they were adhered using glue and the process was a bit more time consuming.  Owen was pretty unhappy with the process at first, but once he had his pacifier, he was able to settle down.

Here is the thing about Owen, he is a people-pleaser.  And by people, I mean doctors. My little boy, the same one who screamed all morning long and had one of his seizure-like episodes twenty minutes before we left for the hospital, was an angel on Monday afternoon.  He sat contentedly in his crib sucking on his pacifier not making a peep.  I actually sat in a chair and leafed through the latest US Weekly.   A little later in the afternoon an a cappella group came and sang Christmas carols for the patients on the floor.  In general, it was a fairly enjoyable afternoon. Then the doctors went home for the night.

Well-behaved Owen returned to regular Owen and the screaming and irritability ebbed and flowed for the rest of the evening and overnight.  In the 48 hours Owen was hooked up to the EEG machine, he did not have any of his seizure-like episodes.  During rounds and visits with his specialists, he sat in my arms like a happy little baby without a care in the world.  Owen behaved in a similar fashion when he was admitted to the hospital back in November.  Yes, the overall goal is for Owen to be comfortable and peaceful, but he could demonstrate a little bit of his drama for the doctors.  There is no need to save it all for the privacy of our home.

Did we accomplish our goal of recording one of these episodes?  No.  Owen was taken off most of his medications in hopes of eliciting an episode, but it never happened.  When I arrived back at the hospital Tuesday night after working all day, I saw the Owen I knew in September.  He was inconsolable, fit-full, and a very unhappy baby boy.  This was the Owen I never wanted to see again because I knew his behavior was troubling for him.  Comfort and quality of life;  those are the goals.  My heart ached all of Tuesday night into Wednesday because Pete and I had knowingly made the decision to return him to this state.  And for what? We had captured an episode on video and all of his doctors agreed that it looked like a seizure.  If the bottom line is that Owen is never going to gain skills- physically or developmentally- then why were we putting him through yet another test?

I had a mini-breakdown during the Wednesday morning rounds.  Probably not appropriate, but after four months of sleep deprivation I felt we had reached a crossroad with his doctors and my mind was not thinking in terms of appropriateness.  Besides, I was on the PICU floor- there is no way I am the first mom to cry during rounds.

Owen's care needs to be switched to comfort care.  This is not a new conversation, in fact it is one we have had with his doctors many times.  The sensors were removed, a DNR has been issued, and we are home again as a family.  Owen's life is now strictly going to be about comfort and Pete and I finally feel like we are taking a step in the right direction for him.  It is painful to make a decision like this about your child and I can't help but cry even as I type the words.  Owen may be with us for only a short time, or we might still have many years. Either scenario is fine as long as I know he is at peace and his troubled brain is no longer distressing him.

On a bright note, Owen and Pete got to meet a few Bruins players who stopped by the PICU for a visit on Tuesday afternoon.  Here is the picture:

Back to the hospital

Owen will be returning to Children's Floating Hospital on Monday.  After his long day of tests on December 9th, we thought we were done with life in the hospital.  If I have learned anything from Owen it is to expect the unexpected.

As I have mentioned before, Owen is having seizures.  Unfortunately, they are getting worse and more frequent. This past week he had a seizure on Monday, Tuesday, and Wednesday.  When an episode happens, he is starting to lose consciousness and that is why we are re-evaluating our decision of no further testing.

We have been going back and forth with Owen's doctors for a few weeks now about whether these episodes are actually seizures.  When he was first admitted to the hospital, they did an EEG on him (for only about 40 minutes).  Owen actually enjoyed it.  The EEG consists of someone placing about 32 sensors, each about the size of a Skittle, all over the top of his head with a Vaseline-like glue.  Owen loves having his head rubbed and I believe he thought he was getting an extra special massage. At that time, the EEG showed no sign of seizure activity - not surprising since Owen had never had one.

About two weeks after he was released from the hospital, Owen had his first seizure.  It was frightening and lasted from start to finish about 30 seconds.  He has since had many more. I have stopped counting, which means he has had far too many.  After each one, we call his neurologist and palliative care doctors and describe the behavior.  They agreed that these sound like seizures, but were not convinced and suggested we try to video a seizure for them to watch.  This is much easier said then done.  When your child is in the middle of a seizure the last thing you are thinking of is, "Ohh, run and get the camera!"  Last Tuesday night, we were finally able to capture one.  It was a four minute seizure where Owen turned blue and did not breathe for the first two minutes.  Beyond frightening.

We diligently tried to send this off to his doctors, but the quality the video, when emailing it, isn't great because it is so large.  Or maybe it is because we really don't know how to email four minute videos- we are not part of the YouTube crowd.  Regardless, it has been decided that these episodes are one of three things: seizures, reflux posturing, or an exaggerated startle reflex.  Pete and I feel very confident we know which one it is, but of course we are not doctors so there is a bit of doubt in our minds.  The only way to know for sure is to do another EEG on him and have it record one of the episodes.  Back to the hospital we go.

The main reason we are doing this is because if these are seizures then Owen is clearly telling us what his MRI didn't.  He is letting us know his disease is progressing and he needs more help.  If this is reflux or or an exaggerated startle reflex then we would obviously treat these very differently.  We hope that our stay on the PICU will be short in length and are eager to be home with our family in time for Christmas.

Questions

Friday was an incredibly important day for Owen.  He was scheduled to undergo a lot of testing which would hopefully shed more light on what is troubling him. The cynical side of me was confident we would leave the hospital knowing no more about Owen and his diagnosis than when we entered.  I was partially correct, but we did learn a few things.

The first thing I learned happened when we stepped off the elevator on the 6th floor of Children's Floating Hospital at 7:30am.  Contrary to what I thought, PICU is not a type of sedation. In my mind, PICU fell into the sedation list below general and above local anesthesia.   I could not have been more wrong.  What it actually stands for is Pediatric Intensive Care Unit.  My mind never connected what PICU actually stood for until I saw it printed out on the wall in front of me.  It must be one of the saddest places on Earth.

I also learned that men who pass out or get dizzy (PETE!) during epidurals are quite possibly weenies.  I watched my son have a lumbar puncture and it was fascinating.  The neurologist who was doing the procedure assured me it is almost exactly the same as an epidural.  They took vial after vial of Owen's spinal fluid and it reminded me of a tree being tapped for syrup.  Owen never felt a thing.

The third thing I learned is that Owen does not like to part with his blood.  He was stuck in every vein and artery they could find and it took almost an hour to draw his blood.  In fact, they were not able to get all of the blood they needed, but DNA from his skin biopsy can be used to substitute for some of the missing blood.  During all of these procedures, including the multiple needle sticks by 5 different doctors and nurses, Owen slept blissfully on his IV of Propofol.  I watched his respirations and heart rate intently and never once did they fluctuate during a poke.  It was wonderful to be in the room with him and know he was okay.

Owen's MRI shows progression of his disease.  It is now located in the frontal portion of his brain as well as the posterior portion.  His MRI is being shared with other neuro-radiologists at various hospitals in the Boston area because what it shows is very rare and open to interpretation.  A final read has not been done, but that should happen on Monday. We were able to speak with his neurologist about the results and none of it is really new information.  We know that what he has is rare, untreatable, and progressing.

Final results from the MRI and all of the labs will come in the next few weeks.  My anxiety about 'knowing' is gone.  I truly believe we will never know exactly what is wrong with Owen.  Dr F, Owen's palliative care doctor, said to me at the end of the day, "No matter what the results of the MRI or labs show us, the most important thing is to listen to Owen.  He will tell us what he needs."

Owen, I am watching and listening.  I love you.

December 9th

When Owen was discharged from the hospital at the beginning of November, we left feeling pretty confident we knew what was wrong with him.  Leukodystrophy is a broad term, like cancer, to describe a terrible illness that presents in many different disease forms.  Owen's doctors felt fairly confident he had Krabbe Disease, but we had to wait on a blood test to confirm the diagnosis.  This disease is rare and to get the results the blood could only be sent to one lab in the country.  So we had to wait- and wait- and wait.  It took almost 3 weeks for the results to come back; Owen does not have Krabbe Disease.

Sadly, this in no way changes his prognosis.  Pete and I know that the doctors based his prognosis on the severity of the brain disease shown on the MRI.  Having a name for it isn't going to change what Owen's future holds.  When I look at my baby boy my mind struggles to believe what they are telling me.  He is beautiful. I do realize that Owen does not behave like a healthy baby, he does things that I can only describe as "neuro".  There are ticks and spastic movements, the out-of-your-mind crying,  stiffness in his body, and his eyes will shake in a unusual fashion.  These behaviors are not always present and it is at those times that my mind wanders and I hope, and pray, and wish.  What if they got it wrong?  Maybe he really isn't as sick as they originally thought? If only it was an infection in his brain and a antibiotic would make it all better.  Or let him still have his 'neuro' behaviors, let him be a special needs child, let him live.

Tomorrow is a day that will hopefully provide us with some answers.  It might even allow Pete and me to take a full breath again after weeks of holding it in.  Owen will be returning to Children's Floating Hospital to go under PICU sedation.  He is going to have another brain MRI, a lumbar puncture, blood drawn, and a skin biopsy.  The tests will be run again and this time they are looking at the rarest of the rare diseases.  Pete and I would love to know what exactly is wrong with Owen, but we can live without ever having the answer.  This is the end of testing for him unless his doctors can convince us more is necessary.  I do not want my baby to become a pincushion- especially since in the end it isn't going to change anything.

Knowing what is wrong with Owen would allow us to test Ellie.  We know this is genetic and is a problem between Pete and me.   It would also give us a chance to have more children in the future- they would just take out the faulty gene and that would be the end of it.  Imagine.  Neither of these things have to happen.  They have assured us that Ellie would already be showing symptoms if she was sick like Owen.  If we are not blessed with any more biological children then we have plenty in our lives to be thankful for.  We just want Owen's time with us to be peaceful.

The MRI is the real test we are waiting for.  This will be compared to the one he had done at the beginning of November and will tell us how rapidly the disease is progressing. It will hopefully give us some answers on how long we have with Owen and that is all we really need to know.

Seizures and Cereal

Exactly one week ago Owen had his second seizure.  His first one happened two weeks prior to his second one and his third seizure happened about four hours ago.  Seizure 1 left me shaken to the core and I could hardly find words to use when speaking with his doctor.  My hands shook for many hours after it happened and my heart continued to race.  Watching your child- a mere 3 month old infant- stop breathing and tense his whole body in your arms for 15, 20, 30 seconds is beyond frightening.  Seizure 1 came and went.  We upped his medication and moved on.  Seizure 2 was just about the same as seizure 1, except I handled it a bit better.  We also added a new medication after that one.  Seizure 3 happened in my mom's arms.  She called me in from the other room and I watched intently as he lay stiff in her arms.  Eventually he relaxed and started breathing again.  Another call was made to his doctor...more medication was dosed...and on we moved. My hands no longer shake for hours afterward and my heart only races for a few minutes following the seizure.  It is amazing how quickly one can become accustomed to such a terrible thing.

Seizure 2 will forever be etched in my memory.  The seizure itself was terrible, but what happened afterward will always bother me.  Owen  was prescribed a new anti-seizure medication (he is already taking 3 others).  I do not think this pill is designed for a 3 month old baby because it comes in capsule form.  How are we ever going to get him to swallow it?  I was told to open the capsule and shake the 'sprinkles' into a spoonful of rice cereal and spoon it into his mouth.  So I did. Hours later I realized what had just happened.

This story really starts when Ellie was about 6 months old.  She was an eager little eater and Pete and I decided she was ready for her first taste of solid food.  Mmmmm rice cereal.  Pete spent an evening assembling her new highchair as Ellie and I went out and shopped for all of her new meal-eating supplies.  Spoons, bowls, bibs, and the important box of rice cereal.  Then we read and reread the instructions on how to mix the cereal, pouring more and more milk into it until the cereal was just the right consistency for her to gobble up off the spoon. When it was ready, we placed Ellie in her shiny new highchair, and with Pete manning both the video camera and digital camera, we documented each and every bite of her first meal. It was so much fun!

Owens first foray into rice cereal went like this: Pete was in New York on a business trip and I could not figure out how to get the 'sprinkles' into Owen.  When his doctor suggested the rice cereal I quickly dusted off Ellie's long abandoned box, mixed up a spoonful, and stuffed it way back in his mouth to ensure his little tongue didn't push it out.  No highchair, no video or photos, no Daddy to watch him reach this new milestone.  It wasn't really even a milestone since he is far too young to be eating cereal.

I think about this every day, and like a true mom, feel hugely guilty over it.  My poor little boy struggles in every aspect of his life and it is just so unfair. Even the accomplishments really aren't accomplishments, they are just means to another end.  When Owen rolled over at 3 weeks, Pete and I were BEYOND proud.  Ellie didn't roll over until she was about 4 months old and would only roll left.  I thought for sure there was something wrong with her.  We learned while Owen was hospitalized that a baby should never be able to roll over that young.  It just meant he had increased tone and was an indication something was wrong.  Another milestone taken from my baby because of this terrible disease.

Two Month Check-up

Every mom knows the drill when it comes to the monthly check-ups with your infant. Most are met with anticipation: How much have they grown?  Where are they on the percentiles? Is my baby a genius because he can already roll over?  Owen's two month check-up was the turning point for me.  I left the office knowing that my child might have something seriously wrong with him; never in my wildest dreams could I predict what the following days would tell us.

As I undressed Owen for his big two month weigh-in, the nurse ran through the checklist of questions they ask about the milestones he should be achieving.  Yes, yes, yes, I answered distractedly until she asked, "Is he cooing? Smiling?" My hands paused on his diaper and my mind raced.  Should he be?  All he does is cry and sleep.  How had I not realized that he should be smiling at me?  With Ellie, I read the What To Expect the First Year book like an instruction manual for my baby.  I knew everything she should be accomplishing and obsessed over it day and night.  "No, I haven't seen him coo or smile." And on we moved.

Later in the appointment, Liz, Owen's nurse practitioner, mentioned that his head was small.  Really?  His head looked completely normal to me. When I looked at the growth chart on her computer screen he wasn't even on the chart! He was sitting below the one percentile! Odd, but not that concerning because I knew Ellie also had a small head when she was his age.  Liz also mentioned that Owen wasn't tracking with his eyes.  How is it possible I hadn't noticed that either?  I reminded Liz that all Owen does is sleep and cry. The acid reflux was terribly painful for him.  I wouldn't feel like cooing or smiling either if I was suffering through it.  Once he outgrew the reflux I was confident Owen would have plenty to coo and smile about.

At the end of the appointment Liz conferred with one of the pediatricians in the practice and they suggested I take Owen into Boston to see a neurologist.  Nothing to worry about they assured me, but since he had a handful of odd symptoms better to have him checked so it could be ruled out.  The appointment was set up for five days later. I spent every ounce of my spare time before his neurology appointment trying to get Owen to smile, coo, and track.  They were right- he did not do any of them.

At the neurology appointment, Pete held Owen and tried to settle him down since he was out of his mind screaming while I tried to answer the questions Dr. T, the neurologist, was asking about him.  After about five minutes, Dr T asked me to hold Owen while he examined him.  There was some poking and prodding and then his mini flashlight came out.  If our life was followed by a soundtrack this is where the music would shift and the dramatic drum beats would start.  Dr. T's demeanor completely changed as he looked into Owen's eyes.  Both Pete and I noticed this and we asked if there was something wrong.  His answer was distracted and he just mentioned wanting to do an MRI on Owen's brain.  The next few minutes were a bit of a blur, but his total appointment lasted less than ten minutes and the end result was admitting Owen to Children's Floating Hospital so a series of tests could be run.  Those included numerous blood draws, a MRI of his brain, a lumbar puncture, and a EEG.  Two days later was November 3rd.

Owen's Journey

When I think about Owen's short life it is segregated into two categories: before we knew and after. The first portion started on August 27, 2011, when my beautiful baby boy was born.  He weighed a healthy 7lbs, 12oz,  had all ten fingers and all ten toes, a fabulous mohawk of red hair, and two perfect dimples.  He arrived on his daddy's birthday and is an amazing gift. Our first two months with Owen were bumpy because he had the trifecta: colic, reflux, and a dairy intolerance.  Tough stuff for such a little guy to handle and subsequently he spent his days either screaming or sleeping (although mainly screaming).

The after portion arrived on November 3, 2011, my dad's birthday.  After spending two days in the hospital with endless testing we learned that our beautiful baby didn't just have reflux and a dairy allergy.  Something much more terrible then that was the cause of all of Owen's crying. Owen has Leukodystrophy, a very large word for abnormalities of the white matter of his brain.  Severe abnormalities.  Life limiting abnormalities.  In one swift moment we went from thinking our child had three benign (although exhausting) problems, ones he would likely outgrow by three months, to realizing that our son is not going to live.

I have decided to write a blog about our life with Owen for two reasons.  The first reason is I need to chronicle his life and tell his story.  I want to talk endlessly about Owen because I fear he will be forgotten once he is gone.  Every day I have with him is an equal mix of exhaustion and amazement.  I love my little boy and I never want to forget how amazingly soft his cheeks are when you kiss them.  When he sneezes he always makes a little coo and kicks his legs up in the air (often four or five times in a row).  When he is upset I know it helps to calm him by rubbing his head.  He is still so small, but incredibly strong.  I love my little boy.

The second reason for this blog is for Owen's big sister, Ellie.  I want her to know everything about her baby brother.  She has loved him from the moment he arrived and is just waiting for him to sit up and play with her.  Tonight she brought over one of her Little People toys and placed it by his head.  She is ready and willing to share her toys, but is never going to get the opportunity.  Ellie is amazing with him and I am afraid that she will be too young to remember how special he is.

The length of Owen's time with us is still a question mark, but every day brings a new story.  Some are heartwarming and some are heartbreaking, but I want to share them all.  I love you Owen O'Donnell Marshall.