When is it Enough?

Pete and I made a pact a few years ago, long before Ellie and Owen had joined our family, we would try hard to not over indulge our children.  This rang particularly true around the Christmas season.  We refer to it as the gluttony of Christmas- where present after present is opened without a second glance.  Where the importance of the gift is lost under the sheer volume of presents.  Our goal is to never have that scene played out in our own living room.

This year, as we shopped for Ellie, we kept our gift goals in mind: something you want, something you need, something to wear, something to read.  We did pretty good and only fell about four gifts over this mantra.  It took restraint for most of December and then a little bit of UPS luck in the days leading up to Christmas to regain the perspective we held strong all month.

On the Friday prior to Christmas, Pete and I had a moment of weakness as we watched our daughter respond to the siren call of the Disney princesses on TV.  The Disney Princess Dream Castle, the gift Ellie had repeatedly and joyously proclaimed, "I want that for Christmas!"  As we watched our daughter stare enraptured at the TV, Pete and I quietly agreed.  Ellie deserved the Disney Princess Dream Castle.  We deserved to give it to her.  Our resolve was broken and the hunt for a $150 piece of plastic became the mission.  We involved family members in the search and stalked UPS up until Christmas Eve awaiting the arrival of the revered castle.  But it didn't arrive on time.  And Ellie has never mentioned it. Her playroom is so fully stocked with great new toys she has forgotten all about the castle.  When she opened her gifts on Christmas morning it took over two hours.  Not because of the quantity, but because each one was so thrilling for her we had to open the gift and play with it briefly.  This is what we want our Christmas mornings to be like.  The Disney Princess Dream Castle arrived today, and we will return it without Ellie ever being the wiser.

Christmas is a tremendously difficult holiday to celebrate when one of your children is missing.  Pete and I put intentional thought into how we wanted to include Owen in our holiday traditions.  On Christmas Eve, Pete, Ellie, and I visited Owen's hospital to drop off a donation and give thanks.  On Christmas day, Pete and I walked the beach holding Owen close to our hearts.  That night, we released a lantern into the sky, all the way to Heaven, each taking a turn to say what we missed most about him.  I am not able to express the grief Pete and I feel without Owen here.  To say I miss him is like saying I enjoy eating brownies.  Words will never be able to fully express the extent of my emotions.

A few weeks after Owen passed away, my brain started to shift into overdrive.  Pete and I wanted to have our kids close together in age, hence Ellie and Owen were only 16 months apart.  If Pete and I were going to add to our family, where would the age gap fall?  Mental calculations became snippets of conversation which led to my days being consumed with trying to decide which path our family is supposed to take.  Do we pursue adoption? Foster adoption or private?  Do we pursue having another child, but eliminate one of us genetically?  Do we eliminate me or Pete? If we eliminate Pete, do we gender select to ensure that Owen's disease wasn't x-linked inherited?  How long does the adoption process take? How long does IVF take? Enough.

Why is it about gaining and adding?  Our family of three is doing pretty well.  Maybe I should be focusing more on the child I have instead of dreaming of the child I think I need.  Ellie wanted the Disney Princess Dream Castle, but she didn't need it.  She has enjoyed every gift she received.  The Disney Princess Dream Castle is a reminder to me, a lesson I needed to revisit, that in life you don't get everything you want.  Within that story, Ellie is the reminder that you can find joy in what you are given.  There is no need to always be looking for more; be happy with what you have.

I am not one for resolutions, but I will be thrilled to say good-bye to 2013 and all of the bad news it has brought this year.  My goal for 2014 is to focus on my amazing family of three.  Will there be another child in our future? Maybe, but for now we need to enjoy we what have and not what we want.  If Owen has taught me anything, it is life is fleeting.  We spent two years making memories with our sweet guy and now it is time to make more memories with our amazing daughter.

A Castle on a Cloud

About a year and a half ago I imposed the 'happiness' rule for myself.  The basis of this rule is as follows: idle time will be filled only with happy topics.  Gone were TV shows like Dexter and Breaking Bad and replaced were shows like Big Bang Theory and Parks and Recreation.  The same applies to the books I read at bedtime.  As I searched the library for 'happy' stories I realized I had a whole series in my house I could re-read.  The Harry Potter series is one of my favorites and evokes many happy memories of my college years as I would devour each book as it became available.

Having young children only affords me reading time before bed.  This translates to averaging two pages each night, nodding off to sleep about one and a half pages into the story, and then needed to re-read at least one page from the previous night to remember what was happening in the story.  At this pace, it will be a long time before I finish the series and need to look for another 'happy' book.  Owen passed away as I was delving into book five, Harry Potter and the Order of the Phoenix.

At the end of book four, Harry witnesses the death of one of his classmates, Cedric Diggory.  As expected, watching a peer die is a traumatic event.  Harry feels isolated from his friends; it is hard to resume a carefree lifestyle after baring witness to such a tragic event.  Owen has been gone for almost two months.  Around me the world continues to move forward, but I remain consumed in the grief of missing my sweet baby boy.  

I think about Owen frequently, I cry about Owen daily.  I field questions from Ellie ranging from sweet stories about how she plans to go to Heaven and marry him, to asking when she is going to get a disease and go to Heaven.  In our house, Owen is still very present.  His cape hangs on the wall in Ellie's playroom (the room we had picked out to be his bedroom).  Last night we put up our Christmas tree and it is adorned with many of his cherished ornaments, pictures, and hand and foot print from last year.  His stocking is on our ottoman as I type this: do we hang it next to ours over the fireplace? Ellie averages two family drawings a day from preschool and Owen is always featured in them.  He may not physically be present in our house, but he is still my little boy.  He will always be a part of my life.

Watching Owen die, sitting next to him during his last few days on Hospice, having doctors explain what happens as the body slowly shuts down, changes your view on life.  I find myself silently impatient when I hear people complaining about trivial things.  My problems are no more important than the next person, but it is frustrating to see people focused on things that really do not matter.  It is as though I am now viewing the world through a tinted lens, they are definitely not rose colored, but the view is different.  When Harry returns to Hogwarts School of Witchcraft and Wizardry the next term, he is shocked by what he sees.  After attending four previous years and riding in seemingly horseless carriages, he now can see the carriages are pulled by Threstals, animals invisible except to those who have witnessed death.  I am experiencing my world differently now as well.

Pete and I had the opportunity recently to watch a production of Les Miserables.  Our niece, Holly, was playing the role of Cosette and we were gifted tickets to watch her performance.  She was outstanding.  I have seen Les Mis at least five times and love the music and story.  This was my first time watching it since Owen has died.  Much like Harry, I was surprised by what I saw.  Watching Fantine die, witnessing her anguish about her inability to help her child was heart wrenching.  I could relate to her desperation.  She sold her body to make money to help her child.  There were no limits to her love for Cosette.  I cried openly as she begged Jean Valjean to care for Cosette with her last breaths. I was shocked by how much this scene moved me.  I have watched it passively numerous times, but for the first time I felt connected to Fantine and knew her feelings of helplessness.

As I collected myself, the play progressed and then Cosette was singing her solo, "A Castle on a Cloud".  As I listened to the words, ones I have heard numerous times in the past, the tears returned:

There is a castle on a cloud,
I like to go there in my sleep,
Aren't any floors for me to sweep,
Not in my castle on a cloud.

There is a room that's full of toys,
There are a hundred boys and girls,
Nobody shouts or talks too loud,
Not in my castle on a cloud.

There is a lady all in white,
Holds me and sings a lullaby,
She's nice to see and she's soft to touch,
She says "Cosette, I love you very much."

I know a place where no one's lost,
I know a place where no one cries,
Crying at all is not allowed,
Not in my castle on a cloud.

 I can clearly see Owen in his castle on a cloud.  My sweet boy who struggled so much for two years, is now at peace in his castle.  I can envision him clearly playing with the boys and girls, enjoying an endless room of his favorite toys.  His pain is gone, crying is not allowed, he is happy and whole.  It is everything a mother could wish for, except how I yearn for the castle to be here and not in Heaven.

The pain of missing Owen is consuming.  I am shocked by it.  After a two year journey with a chronically ill child, I naively assumed my anticipatory grief would ease the pain once he was actually gone.  Pete and I both feel it continues to be more painful the further we move away from October 16th.  The weight of his body is no longer fresh on my arms, the warmth of his delicious cheek is no longer present on my lips.  It is becoming harder and harder to conjure the feelings of his physical presence which means the reality is here.  Owen is gone.

Owen is where he should be, I do not wish for him to be back with our family.  We miss him so much, but it is comforting to know he is not feeling any pain.  Ellie continues to struggle with the concept of Heaven.  She often looks to the sky and asks us questions of where it is located and why we cannot go and visit Owen.  Above those clouds, there is a mysterious place she is desperate to explore; she misses her brother.  Ellie often looks to see if Owen is poking his toes down at her  and I pretend to look with her.  It has become a bit of a game for us, but now my eyes search with a new purpose.  Maybe someday I will catch of glimpse of Owen in his castle on a cloud.

Wearing the Badge

I strongly believe that women who give birth naturally (i.e. without an epidural), deserve to wear a badge proclaiming this feat.  I have delivered two children and can attest to the excruciating pain involved in the entire process.  I have not earned a badge and that is perfectly fine by me- epidurals are amazing and I highly recommend them.  I have tremendous respect for women who chose no epidural- it takes a lot of strength, determination, and effort.

I envision these badges to be unassuming, but something all women would be able to immediately recognize.  I could pass by and dip my head with a slight smile in recognition of their hard work.  Perhaps it would fuel conversations for those who have not yet experienced child birth. Fellow badge wears would be able to recognize each other immediately and know they share a common bond; a unique experience that not everyone is able to participate in.

Since Owen has passed away, I have come to realize I want to wear a badge.  I am not sure what it should look like, but I want it to say things like, "I get it!", "Is there any way I can help you?", "You are amazing and  doing the best you can", and "We may look like a typical family of three, but we are far from it".  Without Owen, Pete and I have worked very hard to stay busy.  We fill our free time with projects and activities because idle time allows for the grief to sneak in.  It is with us all of the time, but if we remain busy we feel like we are tricking ourselves into moving forward.

A few weeks ago, we took Ellie to the Franklin Park Zoo for a Boo at the Zoo event.  We all wore costumes, visited the animals, and even got to Trick-or-Treat in a section of the zoo.  It was a fun day- our biggest challenge was finding parking.  What a different day it would have been if Owen had joined us.  The packing of supplies and equipment, making of medicine and back-up medicine, extra clothes and diapers, formula and bottles, concerns about the weather, the long drive to the zoo, pressure sores from sitting in a car seat and then wheelchair all day... and the list goes on.  This trip just entailed having money to get in and not forgetting the Rapunzel hair for Ellie's costume.

My first realization of needing a badge happened soon after we arrived at the zoo.  As we entered the first exhibit we encountered a mom with two little girls, both under the age of five.  One girl ran ahead pointing out the animals as she spotted them.  As the mom reminded her not to get too far ahead, she remained close by her other daughter, who was blind.  There was a look on this mother's face of calm intensity- she was focused on helping her daughter navigate a crowded hallway and making sure the children running by in all directions did not knock her off balance. I immediately felt a sense of camaraderie with this woman; Owen was also blind.  I wondered if she was bothered by the fact that her daughter could not see the animals? Then I immediately thought of all the other senses coming into play in the enclosed bird exhibit.  The echos, the dim hallways with brightly lit animal enclosures (perhaps she had some vision like Owen and could see contrasts in light and dark?), surely she could smell  the smells you only find at a zoo.  I wanted to touch this woman's shoulder and tell her I understand.  I get it- I can imagine all of the things going through her mind and she is not alone.

A few exhibits later, we came across a little boy in a wheelchair.  We were once again in an indoor exhibit.  I don't remember the animal, but I do remember, to get to the glass, you had to walk down long stadium seating.  No problem for Ellie and the numerous other children, they bounded down the elongated steps and got up very close (perhaps too close to that glass...all those germs...).  The mom and the little boy hung back at the top step.  She would not be able to navigate the wheelchair down those five steps on her own.  This time I did say something, I offered to help carry him down. I mumbled something incoherent about having a son with a wheelchair and how I understood.  The mom declined my offer and I retreated.  She didn't know I was her a few short weeks ago.  I know what it is like to have a child in a wheelchair who is missing out on the freedom a typical child enjoys.

I knew what we looked like to these moms: a perfect family; Rapunzel the typical three year old, a Red Sox player dad, a cat mom, and even an extra body to help out- Meme the witch.  What they couldn't see was the gaping hole following us through the zoo where Owen should have been dressed as a pumpkin.  I didn't have my badge on to let them know I was actually one of them.

When we went places with Owen, I would make eye contact with numerous people, we would acknowledge each other silently and know that we each understood the mutual challenges we face each day.  I am no longer part of that group and I still want to be.  If I had a badge, one easily recognizable to parents who have children with special needs, maybe it would provide comfort to those families.  It would provide comfort to me.  I have lived life with a healthy baby girl and I have lived life having a special needs child. My family may appear to be healthy and complete, but it is not.  Owen is missing and it hurts to not be able to share that part of me at all times.

 I want everyone to know our son is missing from our group.  I want people to know that my offers to help come from a deep need to make their day easier.  I understand how difficult even the simplest outing can be.  If I was wearing my badge, maybe the mom with the child in the wheelchair would have let me help bring her son closer to the glass.  She needed a helping hand, and unfortunately both of mine have been empty for three and a half weeks.

Two Weeks

Today marks two weeks since Owen passed away.  I have composed many blog posts in my mind since then, but have not been motivated to type one out until now.  Two days after losing Owen, we moved.  This was actually really helpful for Pete and I- we were consumed with the stresses of packing, moving, and unpacking (and ripping up questionable wall-to-wall pink carpet).  We only slept in our Clinton Street house for one night after Owen died and it was very obvious to me I had emotionally moved on from that house.  My dining room-turned playroom-turned-Owen's room felt empty without my boy sleeping within sight of the living room.  I turned my head to check on him many times on the last Thursday in the house.  I was happy to leave- the neighbors are dearly missed, but that house is not.

I am less confident the timing of the move was helpful for Ellie.  She has struggled with Owen being gone.  We have a wonderful family therapist and my best friend is a psychologist.  They have both been very helpful in explaining the level of understanding Ellie has of everything that has happened at a 3.5 year old level.  She misses Owen, she initially asked frequently when he was going to come back from Heaven, but seems to have now accepted he will not return.  She then decided that she was going to Heaven in two weeks to be with him.  She also expressed concern about who was next to go to Heaven.  She has now moved on to making 'wishes' such as: before I eat this piece of candy I am going to make a wish: "I wish that Owen would come back from Heaven." It is heartbreaking.  We work hard to speak concretely to her about what has happened.  When Owen was living, we often pointed out the ways Owen's body was different from hers and how it did not work properly for him.  We have continued this conversation with her now that he is gone.  We speak plainly about how his body stopped working for him, that he died, that no one did anything to make it happen, that we miss him, that Mommy and Daddy are not going anywhere, and that he is in Heaven.

The concept of Heaven is a bit abstract for her.  You can see how her mind is working to understand where Heaven is located, what exactly constitutes an angel, and will gobble  up any nugget of telling regarding these subjects.  A few days ago, she asked me, "Mommy, do you think sometime if we look up at the clouds, we will see Owen poke his toes down at us?"  I can only hope- I really miss those long, pale stinky feet.  They were some of the cutest toes I have ever encountered.

For me, it has been far more difficult than I anticipated.  Loving Owen was an exhausting endeavor.  His need of round-the-clock care, round-the-clock medication, and unpredictable moods left Pete and I with very little reserve after two years.  I do not miss those parts of Owen's life.  I sure do miss holding my boy.  I miss dressing him, bathing him, I miss calling to check on his day when I am at work, kissing his face until he would grimace, I miss listening to him breathe and hearing him clang his toys on his toy bar, I miss feeding him bottles and having him pee on me almost daily, I miss putting lotion all over his body as he would wiggle and protest, I miss rubbing Aquafor on his face and telling him he wasn't going to be a crusty boy, I miss putting gel in his hair to make those red curls look adorable.  I could continue this list for a long time before I ran out of all the things that made Owen such a blessing in my life.

I do not miss the making or dispensing of medication, the constant trips to CVS to pick up medication, the cleaning of syringes piled up endlessly next to my sink, the long phone calls to insurance and pharmacy companies for supplies, the stress and planning required to take him out of the house for a simple trip, or watching him have seizures, cry out in distress, or struggle to breathe.

I am thankful that his body is now at peace and his soul is in Heaven, but I know it will be a long time before I will be able to get through my day without feeling like a large piece of me is missing. The grief I feel comes in waves and unfortunately it seems to be harder two weeks later than it did in the days after Owen passed away.  I remember the dark days after Owen was diagnosed as a two month old.  I was deeply depressed and did not feel  I would ever be capable of feeling joy again.  At some point, I came out of that place and was able to live life and love my two kiddos as any mom does.  I know the overwhelming sadness I feel right now will eventually dissipate.  A dear friend, who lost her son a number of years ago at the age of twenty-three, shared with me she still has days where she cries over the pain of him being gone.  It is daunting to know I will forever have these dark days of sadness and ache for Owen.  If this is the return for the two years that I got to love my boy then I can accept that exchange.

Flying High

My beautiful baby boy earned his angel wings this morning.  Here is the obituary written by my wonderful friend, Lisa.  He was so very loved.

Owen O’Donnell Marshall, age 2, died peacefully while in hospice care on Wednesday, October 16, surrounded by his family.

Born in Newburyport, Mass., on August 27, 2011, he is survived by his parents Peter and Sommer Marshall of Georgetown, formerly of Amesbury; his big sister Ellie Marshall of Georgetown; his maternal grandparents, Marylou and Tony Hammond of Minneapolis, Minnesota; his paternal grandparents, Jason and Linda Marshall of Tewksbury; and his many aunts, uncles, cousins and friends.

Owen, a blue-eyed, red-haired, loving little boy, was cherished by his parents, big sister, family, friends and caregivers. He earned the love and affection of many supporters from across the state and country for his courageous battle with a brain disease—documented by a blog his mother updated faithfully, chronicling his triumphs and challenges.

“This blog is my way of celebrating the blessing he is in my life and sharing the painful journey he has in this world,” his mother wrote.

Owen brought joy to all those who knew him and melted the hearts of those lucky enough to catch a glimpse of his bright, blue eyes. He loved being held and snuggling and became quite the traveler, taking special family trips, including one to New Hampshire and Santa’s Village this summer.

Like his parents, he was an avid sports fan. He wore his jerseys proudly on game days.

Owen recently celebrated his second birthday with a party and gifts with his family and friends, including a special dinosaur cake baked just for him.

To celebrate Owen’s life, a memorial service will be held Sunday, October 20, at 1:00 p.m. at Main Street Congregational Church, Amesbury, MA. A small reception will follow.

In lieu of flowers, donations can be made in Owen’s name to: Pentucket Early Intervention, P.O. Box 356, West Newbury, MA, 01985.

Genetics Follow-up

Pete and I had the opportunity to meet Owen's new geneticist on Thursday afternoon.  Owen has been very ill and did not make the trip with us into Boston, but he was still the star of the show.  Owen's previous geneticist moved out of state at the beginning of the summer and we were ready to start anew with a different set of eyes on Owen.  We had very low expectations about what would be discussed at the meeting, but left two hours later with renewed hope.

We are running two new tests for Owen.  One deals with a more detailed analysis of Owen's mitochondria.  When we did the muscle biopsy in June, the results did show an abnormality.  It is possible that this was explored and ruled out by Owen's previous doctor, and it did not show up on the Whole Exome Sequencing (WES), but we all felt it was worth investigating.  They are also going to run a few tests related to Owen's creatine.  There have been abnormal levels that our new geneticist, Dr. J, feels are worth exploring.  The third new path is related to the abnormal sodium channel gene that both Pete and I passed on to Owen. When we received the  WES results, this was the only useful bit of information it included.  Pete and I both have an abnormal (although benign) gene.  We each gave it to Owen.  It is possible this is related to his disease. Seizure activity is related to the sodium channel genes, and we all know Owen has a very severe uncontrolled seizure disorder.  Dr. J contacted some researchers in Michigan who study this gene exclusively and they are VERY interested in Owen.  I have always felt there was someone who would find Owen fascinating; we just needed to find them.  I am hopeful that Owen will provide some interesting information to these researchers; maybe they will be able to help solve the mystery that is my boy.

The second part of our meeting was genetic counseling.  Pete and I are hopeful we will be able to expand our family in the future.  We were seeking the statistics related to the genetic risks we face with future children.  We have Ellie, so we know it is possible for us to create a healthy child.  Our discussions were very scientific, there will be no wine or candles involved in creating a possible baby #3, but we have renewed hope for another child in the future.

All in all, it was an encouraging meeting.  It is nice to have a glimmer of hope related to genetics after feeling like the door had been closed on us.

Owen Being Owen

My guy has yet again pulled off an amazing feat of trickery.  Owen became very sick on Tuesday.  To backtrack a bit, Owen has basically been sick since the end of August.  He has battled cold after cold, relentless seizures, respiratory distress, fevers, and irritability.  September was a long month with Owen.     On Tuesday, his overall discomfort reached a point where we felt it was necessary to give additional medication to sedate him.  By Wednesday, his 'illness' had reached such a point of respiratory distress that we were asked to contact Hospice because his doctors were concerned he had reached an end of life stage.  I was called home from my job as his work to breathe became frightening and he was having frequent apnea episodes with his nurse.

As hard as I try to not 'drink Owen's drama-filled Kool-aid', he suckered me in once again.  I have never seen him look so ill or so in distress.  I was prepared to say good-bye to my sweet little boy.  Over the course of the next three days, Owen improved slowly.  The fevers have slowly come down and the breathing has very slowly regulated back to his previous poor baseline.  Unfortunately, Owen continues to struggle with uncontrolled seizures and intense irritability.  He has almost zero positive awake-time right now.  He cries, he arches, and he is not consoled by being held.  Putting him down is impossible when he is like this.  We have drastically increased his medication to provide him with comfort (ie. sedation), because he is achieving a failing grade on our 'Quality of Life' scale.

I don't know what this means for the future with Owen.  I assume the irritability is related to the unrelenting seizures, but we do not have an effective way to stop those.  It breaks my heart to think we are now moving into an moment in time where sedation is the only way to keep him calm and peaceful.    Living a life with a mystery child- one with no diagnosis to help guide us though what to expect for him is a form of torture.  Every decision made is second-guessed because we never know what is approaching next.  I did not foresee this in Owen's near future at the end of August as we celebrated his second birthday.

We meet with a new geneticist on Thursday, Owen's previous doctor moved out of the area around the time of his surgery this past June.  Maybe there will be hope for answers or new ideas on Thursday?  I really don't think so, but Pete and I have no other choice right now.  We need to keep searching for why this is happening to Owen.  We just need to keep moving forward- even if the future is filled with terrible things for our boy and his life with us.

As Owen battled for his life on Wednesday, Pete was with our amazing doctor, Karen.  He had the opportunity to speak on a panel at a palliative care conference for about 200 area physicians.  Pete provided a parent's perspective about coordinating care with medically complex children who have a poor overall prognosis.  I am extremely proud of him for doing this.  I read over the notes he had written prior to the panel, and the message he had to share was so important: We can accept the realities of our child and diagnosis, but we need help and guidance about making decisions for care related to the big picture.  With palliative care, it is less about 'fixing', and sometimes those who have spent their adult life training to heal and fix have a hard time recognizing this reality.

Even in the dark times we are experiencing with Owen right now, he continues to inspire and motivate us.  It is occasionally hard to feel blessed to be Owen's parent as he screams from 2-4am, but in the quite moments, Pete and I know he has shown us how important it is to live life in the present. We try to be grateful for every moment we have with Owen...even the 2am moments.

Lennox Gastaut Syndrome

Owen had another EEG on Wednesday afternoon.  By my count, in his two short years, he has now completed six EEGs.  The good news is he really enjoys them!  Owen loves having his head touched and rubbed, so having sensors glued on to it is like a giant massage for him.  The past month has brought increased seizure activity and irritability.  After a few months of Owen being in a fantastic mood, he is reverting back to the archy, screamy, crabby, awake little boy I know so well.  

It comes as no surprise that his seizure diagnosis has progressed from Infantile Spasms to Lennox Gastaut Syndrome.  Here is the information about this syndrome as described by the National Institute of Neurological Disorders and Stroke (NINDS):

What is Lennox-Gastaut Syndrome?

Lennox-Gastaut syndrome is a severe form of epilepsy. Seizures usually begin before 4 years of age. Seizure types, which vary among patients, include tonic (stiffening of the body, upward deviation of the eyes, dilation of the pupils, and altered respiratory patterns), atonic (brief loss of muscle tone and consciousness, causing abrupt falls), atypical absence (staring spells), and myoclonic (sudden muscle jerks). There may be periods of frequent seizures mixed with brief, relatively seizure-free periods. Most children with Lennox-Gastaut syndrome experience some degree of impaired intellectual functioning or information processing, along with developmental delays, and behavioral disturbances. Lennox-Gastaut syndrome can be caused by brain malformations, perinatal asphyxia, severe head injury, central nervous system infection and inherited degenerative or metabolic conditions. In 30-35 percent of cases, no cause can be found.

Is there any treatment?

Treatment for Lennox-Gastaut syndrome includes anti-epileptic medications such as valproate, lamotrigine, felbamate, or topiramate. There is usually no single antiepileptic medication that will control seizures. Children who improve initially may later show tolerance to a drug or have uncontrollable seizures.

What is the prognosis?

The prognosis for individuals with Lennox-Gastaut syndrome varies. There is no cure for the disorder. Complete recovery, including freedom from seizures and normal development, is very unusual.

Owen demonstrates all four seizure types many times a day.  We are sad for our guy that he continues to be on such a steep downward trajectory.  The good news is the prognosis of his cuteness remains excellent as demonstrated here: 

Bucket List Accomplishment

Owen has checked 'Day at Santa's Village' off of his bucket list.  On the Saturday prior to Labor Day, our family journeyed in to the mountains of New Hampshire to find our own version of the North Pole.  We were lucky enough to bring along Ellie's Godparents (and my cousin and cousin-in-law), Kevin and Carrie.  It was a great day for our family and I am so thankful we were able to have Owen join us.  After a long day of mini-donut eating, water slides, twirling rides, and reindeer feedings, we drove west to Littleton, NH to stay at a hotel with an indoor pool.  This was Ellie's first hotel/pool experience and she really enjoyed swimming the night away.

Owen did not take a dip, but he did sit in a lounge chair pool-side with me for part of the evening.  At one point, a family came into the pool area and almost immediately surrounded Owen and me.  At first I was a bit confused about the attention, but after chatting with the mom and her lovely son it was apparent that a great moment with Owen was unfolding.  This family was also at Santa's Village and had noticed Owen and our family.  They wanted to come over to us to say hello, but decided to give our family some privacy.  Seeing us at the same hotel that evening provided them a moment to introduce themselves and share their story.  Before me stood a lovely young man who manages some serious medical challenges.  He proudly showed Ellie and Owen his scar on his belly where he once also had a g-tube placed.  He spent time touching and talking to Owen and sharing his story with us.  It was amazing to see what kindness, compassion, and interest this preteen boy demonstrated to my children.  I can only hope that someday Ellie will behave with the same grace and maturity.  It was a great experience to wrap up a wonderful day as a family enjoying a traditional New England vacation.

In other 'Owen News', he has developed a granuloma around his g-tube site.  Apparently, this is very normal with new g-tubes and we have been visiting Owen's wonderful peditrician to slowly burn it off with silver nitrate. Owen has also needed his anti-seizure medications adjusted.  The past two weeks have brought near constant seizures.  Owen has gained a bit of weight, he is now up to 18.5lbs.  My hope is we are not seeing increased seizure activity, rather a need for more medication due to weight gain.  We will be visiting the neurologist on Monday so I will know a bit more then.  Owen seems to be developing what I have labeled 'phantom fevers' over the past month or so.  He will spike a 101-102 fever out of the blue, but it will then go away with no other symptoms.  Motility continues to be a concern- I noticed twice yesterday that he had green liquid in his belly when opening up his tube.  This is concerning because he does not have regular or frequent bowel movements.  We are also now venturing in to the area of pressure sores.  This has always been a back burner concern, but he seems to be developing redness on his buns, hips, and tailbone area after just 20 minutes of being in one position.  We are now needing to adjust his position every 20-30 minutes to help prevent breakdown of the skin.  He is also sharing 2-3 projectile vomits with us each week.  I am considering handing out plastic ponchos for people who come to visit us at our house.

These are the medical concerns I really hoped and prayed we would avoid with our sweet boy.  I long ago gave up any control on this journey.  We are just continuing to provide love and care for Owen.  When awake and alert, he seems very attuned to his surroundings and is so lovable.  On Sunday, I will be running a half marathon in honor of Owen's birthday and for another sweet little girl, Lizzie.  Last year, my friend Jenny and I ran a 5K on the anniversary of Owen's diagnosis.  I decided to set a goal of a half marathon to celebrate Owen turning two.  It amazes me that my guy is already a toddler- let the tantrums begin...

Ellie at the wheel

!

Enjoying the Merry-Go-Round 

Ellie riding the reindeer and 'pulling' us in the sleigh

Owen with Mommy's favorite elf

Ellie on the roller coaster- she is a thrill seeker

Owen among 'his' flower- Black Eyed Susans

A family ride around the park 

Not our best picture- we are all saying "Cheese Balls" 

Partying: Owen-style

Owen turned two yesterday and had a blow-out pool party bash to celebrate.  Here are a few pictures of the fun.  Owen slept through the night on Monday night and was up at 8am on his birthday for a kiss and bottle.  Then he powered back down and slept until 1:30pm when I picked him up to get in the car for the party.  He was clearly just planning ahead and conserving his energy because he was awake the remainder of the day and night taking in all of the festivities.  I am confident he had a blast!  Thank you to everyone who helped my little guy have such a special day!  There was also an amazing lantern launch at the end of the night. All in all a great day!

First present of the day was an Adrian Peterson jersey from Daddy

Dinosaur cake made with love by Mommy

Like any party animal, Owen had his hair done at the party so he was looking his best.  He brought his stylist Kelly as usual.  He is becoming so high maintenance....

Enjoying everyone singing Happy Birthday with Mommy, Mammie, and Brielle

A smooch from Daddy

Celebrating Two!

Celebrating Owen's second birthday is a pretty amazing feat.  I really did not think I would have the opportunity to whisper, "Happy Birthday, my sweet boy," to him another year.  Owen is leading me down a rocky path, but he has proven time and again he is here to fight.  His life is on his own terms and I am just chasing behind trying to keep up with him.  A mother's eyes are always biased, but I know my guy is one of the cutest kids on the block.  His red, curly hair and gorgeous blue eyes draw people to him.  The irritability of his first year and a half of life has mainly subsided.  He melts his body into mine, wraps his long fingers tightly around my hand, and I could spend an eternity 'a-gooing' with my delicious little turkey.  I have never been more in love with my sweet Owen, which makes the realities of his life so difficult.

This past year has brought us the diagnosis of Infantile Spasms, the disappearance of smiles and giggles, a confirmation of legal blindness, respiratory complications, loss of movement in his legs and torso, a confirmation of genetic rarity and mystery, dramatic progression and disappearance of white matter in his brain, a feeding tube, regular bowel issues, a full-time team of home nursing care, and the list goes on.  Instead of a thriving, skill-gaining two-year-old, we have a wheelchair bound, heavily medicated, seizure-laden little boy who continues to lose the little bits of life he has.  These are the realities of life with Owen.  They are harsh, but they are facts.

Is he still amazing, sweet, handsome, delicious, and a blessing to our family? YES! Can he feel the love that surrounds him by our family, his therapists, and doctors? YES! We will celebrate every year with Owen that he chooses to share with us.  Our goals for him remain the same; comfort and quality of life over quantity of life.  Owen has taught us a lot about parenting a medically complex child.  We can make statements like: "No feeding tubes!", but the reality is we are unwilling to watch our child slowly starve to death.  That does not fall into the category of quality of life.  I cannot watch my child writhe in pain due to bowel obstructions; I am going to medically intervene even though the cause of these obstructions lies within the realities of his brain disease.  At what point do you say enough is enough when the medical world is at your finger-tips?

Two-year-old Owen

I have no idea what the next year will bring for Owen.  My hopes include a diagnosis and comfort.  I just recovered from grieving the possible loss of him in early August.  Yesterday, Owen spiked a fever and vomited for the afternoon because he spent time outside.  My sweet boy has such a hard time maintaining his body temperature- very common with neurologically involved children.  It is so sad to see him struggle through days like yesterday.  His disease puts limits on every single aspect of his world.  Everyday is a roller coaster with my guy.  Will there be a 'Celebrating Three!' post in the future? I am not sure- and it doesn't matter to me.  If Owen wants to celebrate another year then I will be along side him.  Happy Birthday to the sweetest little two-year-old around.  I love you!

This is my alert happy guy a little over a year ago. He is so adorable! 

This is Owen demonstrating that just a year ago he could pick his feet up and move them around.  He could also bring his hands together and bring them to his mouth.  He cannot do any of these skills one year later. 

This is the last picture captured of Owen smiling.  It was taken in July 2012.   It was a great moment in time with his cousin Andrew, but who knew how special this photo would become? 

...And The Search Continues

The results are back and Owen does not have a mitochondrial disease.  Our guy is one of the rarest among the many rare kids out there.  In some ways it is like hitting the lottery, but not in the oodles of money kind of way.  I had been eagerly anticipating these results for the past month, only to be 100% distracted when they finally arrived.  It was just a blip on the radar of an intensely emotional week for Owen and our family.

Owen has been dealing with a cold for the past few weeks.  His baseline respiratory status is labored and not normal.  When he is sick he must work a bit harder- his baseline breathing is a struggled inspiration, but when sick he struggles with inspiration and expiration.  He receives a nebulizer treatment three times a day- often it doesn't do much to help with the labor of breathing, but he seems to like the sound and air...so he gets it.  This past Monday, there was a noticeable change in his breathing status - he was working much harder and sounded pretty bad.  His oxygen was at 86% (Owen usually fluctuates from 90-100% throughout a typical day).  His nurse and I agreed if he didn't sound better by Wednesday, I would bring him into the pediatrician.  Wednesday's doctor visit resulted in a tentative diagnosis of pneumonia in his left lung- he had a fever and his oxygen was at 88%.  A follow up on Thursday confirmed this assumption and his oxygen had dipped into the range of 85-87%.  This was easily the most sick I have ever seen Owen.  We were prepared for an end of life situation and attempted to emotionally prep ourselves.  In typical Owen fashion, Friday brought improvement of the fever and slight respiratory improvement.  Saturday continued to be fever-free and his breathing was close to back to his usual baseline.  Did he have pneumonia? Possibly or possibly not, but he was very sick, and he is a fighter.  Pete and I marvel at how strong our little boy is.  I am so thankful he is recovering and have spent the past few days smothering him with kisses.  Owen's cough still sounds pretty terrible, so I know he is still fighting something, but he doesn't appear to be sick so perhaps this is his new baseline.

Last night we took Ellie and Owen to their first viewing of fireworks.  We had a lovely family evening and the show was great- Pete and I really enjoyed it!  Ellie was far more interested in the $2 glow bracelets we bought for her and Owen demonstrated zero signs that he could see- or hear- the fireworks.  I have spent my morning sneaking up on him and banging things loudly to see if he would react.  Finally, a few moments ago, I banged a glass on the wood floor near his ear and he startled.  A mean Mommy, but I was seriously concerned that he might have lost his hearing!  This kid likes to keep me on edge!

Asleep while out to dinner at Flatbread's

Looking handsome in his wheelchair at a birthday party

Two sleeping kiddos after the fireworks

Asleep holding on to his favorite bead toy

Owen's 'I'm Two!' photo (it is a picture of a picture so the quality isn't the best)

Owen working out in his stander- we will slowly increase the angle to help his legs bare weight.  Very cool and he loves it!

Summer Fun

Picking up where the last post left off- Owen's ileus has resolved and he is doing well.  We are still working out his feeding schedule through the g-tube and have hit a few hiccups (well, projectile vomiting actually...), but it is working great for medication.  Owen was at the hospital yesterday for his follow-up after surgery and had some stitches removed.  After two stinky weeks, my guy finally got a bath last night.  He looked so great sitting in his tub chair and smelled delicious afterward.  Those bath wipes you use post-surgery leave something to be desired...

Here are some pictures of what Owen has been up to the past few weeks.

Daddy and me at a birthday party

Hanging in the cottage at York Beach, ME

Snuggling with cousin Connor at the cottage

A morning snooze amid toys

The bead toy on my left is my FAVORITE toy- and has been for over a year.  Also, I am now wearing t-shirts as opposed to onesies- my mom had lots of fun shopping for me post g-tube surgery

At the neighbor's house- was planning on a swim in their pool, but was so comfy on the pool float that I never even dipped a toe in the water!

Grilling on the deck with Dad

A snooze with Ellie's favorite mouse- she gave it to me to snuggle 

Fun at the park- my first time on the merry-go-round and I LOVE it!

Mommy and me at a birthday party

My first chance joining in the annual kids photo- Mommy was a bit nervous leaving me in Ellie's arms, but she and I did just fine!

Ellie demonstrating just how yummy beach ice cream really tastes!

Long Sands at York Beach, ME

Trying out the slide 

Results of Owen's Surgery*

*I am starting this post with an asterisk because I am sure some of my medical information will not be 100% accurate.  I will do my best to share what Owen's doctors told us last Thursday, but on a day that was very stressful...some of it might have become a bit muddled.

The good news is...Owen is home.  We came home on Sunday afternoon and were very happy to be out of the hospital.  The doctors, nurses, and staff at Children's Floating Hospital are amazing and accommodating, but I still hate being at the hospital.  Unfortunately, Owen has not been fairing well over the past few days.  Last night I brought him to our pediatrician with a very distended belly.  He has been miserable since Monday - belly bloat, arching, opening up his stitches, the whole shebang.  (of course- my guy was a cool cat on Sunday in the hospital and then Monday was screaming for the whole day.  Nice Owen.) The consensus last night between our pediatrician and Karen was Owen's bowels never restarted- Ileus.  We have reduced a medication in hopes to get things moving.  It is possible he will end up back in the hospital, but as of 10:30 this morning, the belly looks much better...although he still hasn't stooled. Poor guy!!

Here are the procedure reports from head to toe (actually thigh):

MRI:  Owen's brain MRI was read and compared to his last MRI which was in May 2012.  In a year, the white matter of Owen's brain has significantly thinned.  The brain stem appears to be functioning normally.  Owen's neurologist has been slowly steering us away from the category of Leukodystrophy for a few months now and this MRI further supports his theory.  Owen will always fit into the definition of Leukodystrophy because the white matter of his brain is abnormal.  He does not fit into the disease categories of Leukodystrophy because his disease is not progressing in the same manner.  A few examples are that his seizure disorder is much more significant than most Leukodystrophies and the progressive thinning of the white matter is much more rapid compared to most Leukodystrophies.  Owen's two brain MRI's -a year apart- show progression typical in an eight year time span of other Leukodystrophies.  The findings are significant and confirm that Owen's brain disease is progressive.  The only positive to take from this news is it helps Pete and I feel confident in our decisions to provide love and comfort to Owen for as long as he chooses to be with us, but we will be very cautious with medical interventions beyond comfort care.

Bronchoscopy: Owen has Laryngomalacia.  His vocal cords are collapsing which is the cause of his noisy, struggled breathing.  In most cases, this is something that can be fixed with a surgical procedure. Owen's ENT spoke with us about how this procedure will not work for Owen because the cause of his collapse is neurological and based on low muscle tone.  His recommendation is we put in a tracheotomy.  At this point, a tracheotomy is not in the care plan for Owen.  We will continue to treat his large adenoids with Flonase to help create breathing room.  His oxygen saturations fluctuate from 100% down to the low 90s depending on how hard he is working through out his day.  Our goals will be focused on keeping Owen calm and comfortable.  In the future, we are willing to provide supportive oxygen if needed, but we will not surgically intervene to open up his airway.

G-tube: Owen had a Mic-Key gastronomy tube placed.  So far it is working well for Owen.  We are now able to provide him with his medications via the tube rather than by mouth.  We will continue to bottle feed Owen during the day and then he will be on a 10-hour drip feed overnight for additional nourishment.  We are slowly working up to our goal feeding schedule - and Owen's belly bloat issues are making this difficult.  In the grand picture- this tube will be amazing.  Owen often sleeps for many hours and will miss scheduled medications and food.  If he is sick his sleep pattern extends significantly and proper hydration is always a concern.  These problems are now solved.  If Owen has an airway emergency in the future, we now have another way to provide him with supportive medication quickly. For all of these reasons, we are glad Owen was able to have the tube placed.

Muscle Biopsy: A biopsy of Owen's thigh muscle was taken for testing of mitochondrial diseases.  This is the only definitive way to test for this type of disease.  We would love to hear results of a diagnosis for Owen, but we are more likely to finally rule out mitochondrial diseases.  It will take about three weeks for the results.

Thank you for all of the support and prayers for Owen and our family.  We really feel blessed to have so many people cheering for the cutest red-head I know.

Playing with shaving cream the day before surgery

In his surgical garb before surgery

A very early morning waiting for anesthesia

Resting after surgery

Ellie and Mommy visiting the bear outside the hospital

Ellie playing in the amazing playroom at the hospital 

For those who are interested in why Owen's hospital is called the 'floating' hospital, I borrowed this from the hospital's website:  Floating Hospital’s history as a pioneer in pediatric care began with the Reverend Rufus B. Tobey, a kind-hearted Congregational minister who was struck by the sight of indigent women and their sick children enjoying cool ocean breezes on Boston's waterfront on sweltering summer evenings. Health care at the time had few means of therapy and fewer cures, but many believed in the cleansing and therapeutic qualities of sea air to ward off poor health and specific diseases. To help families, Tobey proposed taking sick babies and their mothers for a day's outing on Boston harbor. The Boston Herald reported Tobey's suggestion and the story brought in a flood of donations to help make it a reality. On July 25th, 1894, the Boston Floating Hospital was born on a rented boat named the "Clifford." Each day, babies received needed therapy, mothers learned how to care for and feed their children safely, and patients, parents, doctors, nurses, volunteers and crew enjoyed the "beneficial harbor breezes." For 33 years the hospital offered medical care for sick children while cruising Boston harbor. The Floating Hospital ship was destroyed by fire in the spring of 1927; fortunately, no patients were aboard.  Rather than rebuild the ship, the trustees decided to expand the on-shore program.  in 1931, the Jackson Memorial Building opened at the hospital's current site.  .

For those who are interested in why Owen's hospital is called the 'floating' hospital, I borrowed this from the hospital's website: 

Floating Hospital’s history as a pioneer in pediatric care began with the Reverend Rufus B. Tobey, a kind-hearted Congregational minister who was struck by the sight of indigent women and their sick children enjoying cool ocean breezes on Boston's waterfront on sweltering summer evenings. Health care at the time had few means of therapy and fewer cures, but many believed in the cleansing and therapeutic qualities of sea air to ward off poor health and specific diseases.
To help families, Tobey proposed taking sick babies and their mothers for a day's outing on Boston harbor. The Boston Herald reported Tobey's suggestion and the story brought in a flood of donations to help make it a reality. On July 25th, 1894, the Boston Floating Hospital was born on a rented boat named the "Clifford." Each day, babies received needed therapy, mothers learned how to care for and feed their children safely, and patients, parents, doctors, nurses, volunteers and crew enjoyed the "beneficial harbor breezes." For 33 years the hospital offered medical care for sick children while cruising Boston harbor. The Floating Hospital ship was destroyed by fire in the spring of 1927; fortunately, no patients were aboard.  Rather than rebuild the ship, the trustees decided to expand the on-shore program.  in 1931, the Jackson Memorial Building opened at the hospital's current site. 
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Surgery update

Owen had surgery today for a g tube placement, muscle biopsy, bronchoscopy, and brain MRI.  He tolerated general anesthesia well and the breathing tube was safely removed in the PICU this afternoon.  He will remain in the PICU for the next few days and I will post a more detailed entry soon.  Thank you for all of the prayers and well wishes!

Super Owen

We are less than a week from Owen's surgery date and my emotions surrounding it could not be more mixed.  Yesterday at work, I was sick to my stomach all day thinking about what he was going to undertake in less than a week...and that I was voluntarily choosing these procedures for him.  Owen needs to have a feeding tube placed.  I know this.  To place the tube he has to go under general anesthesia...and if he is going to do that, then it only makes sense to perform a muscle biopsy to rule out mitochondrial diseases.  I know this.  I just hate to make him go through all of it.

Pete and I brought Owen to the hospital last Monday to meet with the surgeon who will perform the muscle biopsy, skin biopsy, and work in conjunction with GI to place the g-tube.  I instantly liked him and feel very comfortable knowing he will be in charge of my sweet guy for a few hours.  While I can't remember his exact words, he had the ability to share with me that a large portion of the muscle in one of Owen's thighs would be removed (because Owen is so small and skinny), yet he said it in a kind and slightly joking manner; he made it less frightening in that moment and okay.  Of course, now I worry endlessly about how painful the recovery will be for my little guy, but it needs to be done.  I know this.

Owen needs to return to the hospital this Monday to meet with anesthesia and ENT.  Owen's surgeon feels it is important for the anesthesia team to meet him prior to the surgery so they are prepared for all the fun Owen will be providing them on Thursday.  Owen's airway continues to be a concern.  It has been progressively getting worse over the past year and is starting to be at a place that truly frightens Pete and me.  ENT has been added to help address the collapse and provide us with options.  They may chose to perform a bronch on surgery day (I believe this is a scope-type procedure where they will go down his throat to ascertain exactly where the collapse is happening).  I will know a lot more after we meet with ENT on Monday.  Karen plainly laid out the fork in the road that we are approaching with Owen.  Our first option is surgical intervention: a Uvulopalatopharyngoplasty (UPPP) and then eventually when that fails, a tracheotomy.  The other option is sedation.  We use medications to sedate Owen so that he does not become agitated and that will allow him to manage his airway more effectively until it collapses entirely.  Can't say we really like either choice...so we will meet with ENT and hope they have an option that falls somewhere in the middle.

Owen's temperament the past few weeks has been great.  He is calm when awake and seems content.  This is partly why I am struggling so much with the impending surgery.  We are achieving the quality of life for our little turkey right now that we are always striving to maintain.  It would be a lot easier to send him into surgery if we in the midst of weeks of poor eating and intense irritability.  This surgery should have happened in April when Owen was having relentless seizures and really struggling.  Given his happy mood, I have been taking him out and about a lot more.  Owen has been enjoying his wheelchair which makes him a lot more manageable outside of our house.  We even tackled the grocery store the other day and I think he loved it!

There is a super special family living in Chicago that I have come to care deeply about via blogging.  This mom reached out to me over a year ago to tell me how much she enjoyed reading about Owen.  I immediately started reading her blog about her family and specifically about her son who has a very rare genetic disease.  She is really an inspiration to me- her writing has always focused on the triumphs of her son and has really helped me on the days when I want to wallow in the "why me", "why Owen", "why our family" doldrums of living life with a special needs child.  After a post about Owen's impending surgery, she emailed me and said she had something she wanted to send Owen.  In the mail we received a personalized cape from a group called Tiny Superheros.  What an amazing gift- if ever there was a super hero in our family, it is Owen! Owen happily wears his cape wherever he goes on the back of his wheelchair.  I think it totally ups the cool factor for my sweet boy.  We are so thankful that Owen is part of this amazing group and we immediately knew we wanted to pay the generosity forward.  There is a Tiny Superhero we know who totally deserves a cape.... and it will be arriving soon!

I will try to post one more blog with the updates from the appointments this coming Monday.  Surgery is scheduled for this coming Thursday, June 27th.  I hope everyone who reads this blog with hold Owen close in their hearts that day and say a prayer for him.  Here are a few pictures of Owen until next time:

On the swing at the Early Intervention Resource Room

Ball pit at the Early Intervention Resource Room (he didn't love it which was really surprising....)

Bath time- I love that he demurely adjusted his leg for privacy during the photo shoot! Who knew when we bought our house how much we would love having a giant claw foot tub in the main floor bath! 

As cute as can be

Early morning snuggles- these two crack me up!