Losing Max...he is forever in our hearts

The beginning of this post was written a few weeks before Max passed away. I felt it necessary to share my thoughts and then update on our current status.

You don't realize how many kids and families go through extra challenges in life until you experience it first hand or have a friend or family member who does.  I'm a part of many special and private Facebook groups that basically are there to support you through the process.  Those who have feeding challenges...those with Krabbe...those with other medically complex issues... There are posts daily about kids making through open heart surgery, struggling for their life who need extra prayers, posts of the success of getting enough calories for the day and of course the posts that I dread each day that I open up social media....the loss of a child.

We haven't been down that road yet but have known a lot of families who we have met through social media who have.  I know it is our future...but I can't fathom how we will get through it.  Max has decided to give us continuous scares the past week and we have come to see that it is disease progression...he is having trouble catching his breath when he gets upset and dips into the 30s...20s or 10 and under with oxygen.  Every time it happens I can't help but think...is this it? Am I ready for this? How will I get through this? What will I tell Emma?  How will we get through our day without our sweet Max? Then in an instant he bounces back and we go on with the day.  He smiles with his eyes...settles down and is fine!

I know I shouldn't even go there in my mind. It is a dark place. But with a terminally ill child...how can you avoid it? Everything is put into perspective again.

UPDATE:

...June 24, 2016 we were woken up about 4 am that Max had stopped breathing and we lost him. He had been struggling for the past month and we were terrified he'd struggle in his last moments. We had a night nurse on her shift and suddenly the pulse ox couldn't find a pulse and she thought the equipment was faulty (which it had been before) but he wasn't breathing. All I remember is  Josiah yelling "what!" over and over...we both ran downstairs and I picked him up, held him and started trying to resuscitate him...but he had left. I continued for 35 minutes until hospice got there and confirmed he had passed. We had decided that if Max had taken a breath we would have rushed him to the hospital. The crazy part of it all was he looked so Calm and so normal...like he was sleeping..he had been struggling the last few weeks and turning purple often. He just looked peaceful. Emma was still sleeping so the mortuary came and got Max and Josiah and I both woke up Emma and told her Max went to heaven. She smiled and said "I'm so happy because now Max can do everything I can do."

We tried to get to the mortuary quickly but I was physically sick. It us amazing how your body responds to deep sorrow and uncontrollable stress and grief. We were able to arrange for Max to donate some nerves and brain tissue to help find a cure. While controversial for some...our whole story we've tried to advocate and help others and I truly hope it helps in a cure.

We had a great celebration service for our little boy...something you should never have to plan...but the amount of support we received for all who came out was so appreciated. We ended the service with a balloon release. Emma picked out a Buzz Light year balloon, I picked lightning McQueen and Josiah superheroes. We each wrote messages and released them to the perfect song...I Lived by One Republic...because Max lived and he helped us live in the moment and do our best to look past this horrible disease.

Each day I miss him. I miss his touch and his noises and his smell. I miss everything but Krabbe... I don't miss his suffering or questioning if what we were doing each day was right. I've continued to do my research to try and get this in front of the right people in Arizona for newborn screening but I'm not getting far. I'm not giving up either.

The best daily reminder from Max are the butterflies. They are all around us and fly in front of us in completely obvious ways...when we are at events or doing things as a family...he lets us know he is around...I truly believe that. We continue to do our best to move forward but some days are better than others. We will always hold Max's memory close. He taught us so much and will be on our hearts forever.  As we enter 2017, I am so sad that I'm entering a year without him. It is scary. We will continue our "firsts" without him here and continue navigating through life the best we know how.

Allison

Better Place

Music is something that helps me every day.  While in the hospital with Max, I heard a song from Rachel Platten, "Better Place"...the lyrics are probably about a crush or romance but for me, it expresses how I feel since my children have come into my life. I'm not the best singer but started singing this song to both kids to express how much they mean to me.  It is a better place since they've been in my life, regardless of the struggles and mountains we've faced. I seem to get an expression and reaction from Max each time I sing this to him (audio here https://www.youtube.com/watch?v=50gPTzjhmhA):

I'll tell the world, I'll sing a song
It's a better place since you came along
Since you came along
Your touch is sunlight through the trees
Your kisses are the ocean breeze
Everything's alright when you're with me

And I hold my favorite thing
I hold the love that you bring
But it feels like I've opened my eyes again
And the colors are golden and bright again
There's a song in my heart, I feel like I belong
It's a better place since you came along
It's a better place since you came along

I see the whole world in your eyes
It's like I've known you all my life
We just feel so right
So I pour my heart into your hands
It's like you really understand
You love the way I am

And I hold my favorite thing
I hold the happiness you bring
But it feels like I've opened my eyes again
And the colors are golden and bright again
And the sun paints the skies and the wind sings our song
It's a better place since you came along
It's a better place since you came along

Now I'm alright, now I'm alright
Everything's alright

Cause it feels like I've opened my eyes again
And the colors are golden and bright again
There's a song in my heart, I feel like I belong
It's a better place since you came along
It's a better place since you came along

The month of February was a scary one for us. Just when you think you are doing everything you can to stay out of the hospital and you have all of the equipment needed at home to keep Max comfortable...the unthinkable happens.  Max was rushed to the emergency room on Feb 3rd because he couldn't breathe. His home health nurse saved his life and kept him stable until the paramedics could get there.  We are so grateful she was there that afternoon. Although x-rays never showed that the pneumonia was completely gone, clinically he was doing better and had slowly been able to do more and didn't seem to be in as much pain. We thought we were on the right track.

When I got the emergency call, I was at work and ran out of the office and sped to the hospital. Once at the hospital, Max was put on a ventilator. That was the scariest decision to have ever made - although there wasn't much choice for him.  We weren't sure if he'd ever come off when we made the choice to put him on it. The ER staff was amazing - told us what was going on and what was happening. I had held it together until we made the choice to put him on the vent...in my mind I wasn't ready to lose him and to have to make a choice to keep him on or take him off in my mind was too much. Seeing his lifeless little body on the table was terrifying and I lost it and had to sit down because I thought I was going to pass out - it caught up with me. How did this happen so quickly? It turned out that Max had both bacterial pneumonia AND RSV (which can be a cold to most people but in small children, it can take their life - even kids without Krabbe).

The days to follow were very up and down. Some days he was doing great - other days they couldn't turn the ventilator up much more without harming his lungs. He went into the hospital as a one year old and came out a 2 year old. On his birthday, he had a good day. His stats were good. He got to meet Ironman and we had a small family party celebrating Max. We decorated his room in a superhero theme and everyone who came into his room smiled.  The Monday after his birthday, we didn't know if he'd make it through the night the way the doctor was talking. Somehow this little superhero decided that it wasn't his time and he fought to a point where they thought they could take him of the ventilator. They told us the vent wasn't doing much so they thought they'd try to take him off.  That first 24 hours were very up and down.  Everyone would come racing into his room because his stats would fall so low and then slowly his numbers would return to normal slowly on his own. Since we had been in the hospital, we hadn't had the opportunity to hold Max because there were so many wires and tubes and lines connected to him. It had been a rough night after taking him off of the ventilator and the doctor that morning during rounds asked if we had held him yet - I told him I wanted to. I held Max for 3-4 hours and then Josiah got to do the same.  You could see him relax...his numbers stabilized...the doctor came in and looked at both of us..."I can do a lot of things medically to help him...but I can't do that. Good job, Mom and Dad." Tears filled my eyes.

It took another 4-5 days until Max was stable enough to go home. The doctor told us that for most kids, they wouldn't be released because he was still on oxygen and other things...but because of his condition and because we basically have a small hospital of equipment at home, we could take him home if we were comfortable to do so. I have to be honest - I was nervous. I didn't want this to happen again but I was so grateful to all be under the same roof again.  No more trips back and forth from the hospital...no more trying to find out who to keep Emma if both of us needed to be at the hospital.  No more worrying from home when it was my turn to be home and Josiah was at the hospital. He did great and slowly we got back into a routine and we were happy to all be home.

In the weeks to follow, Max was assigned a local pulmonologist who has given us a game plan of what to do when he is well, getting sick and sick...so his lungs stay open and clear. It has worked well...I found myself getting the flu shortly after being home - my body shut down with little sleep and finally just being able to be home. Max was starting to get junky again, probably because of my illness - but the treatments helped tremendously and he seems to be back on track.

We are back to our "normal" which to most people is anything but normal. We are grateful to have more time on Earth with our son. It was an emotional rollercoaster but we wanted to thank all of the most amazing nurses at Banner Thunderbird and his doctors in the ICU.  Everyone took care of Max and us.  They helped us all through tough and weak moments but we were able to joke and laugh during the good moments with them.  Thanks to all of our family and friends who helped with Emma, visited the hospital, sent us care packages, sent Max birthday gifts and sent messages, prayers and love.  With such an amazing amount of love surrounding our family - we are able to get through the hard times and come out stronger.  Thank you for your support.  There are never enough words to express our gratitude.

We are looking forward to taking Max out again soon and celebrating each day as a complete family. We are in a "Better Place" and hope to stay here for awhile.

~Allison

Diagnosis Day

Shortly after diagnosis

Last night a few people sent me messages and the Krabbe community was buzzing on social media about a child being diagnosed with Krabbe on the show Chicago Med.  I have never personally watched the show, but I was curious how it would be portrayed so I checked the timing and recorded the show. After the kids were in bed, I asked Josiah if he would watch it with me. I had mixed feelings myself about watching it and I could tell he did as well.  But we both sat down and watched it together just to see how it was portrayed.

Overall the portrayal of the disease and treatment options was correct.  We are glad that the awareness of the disease was featured on a popular national show. It was an episode that pulled at the heartstrings...it is our reality in a lot of ways. There were some unrealistic things...timing to get the test results back for Krabbe in the show and being diagnosed so quickly is rare...it takes weeks. The child was a little older than the more common versions of the disease but it isn't unheard of and can happen with late onset. Even having a doctor who knows to test for Krabbe is so rare... That would have been amazing in the real world...so many kids go misdiagnosed for so long (but again it is only a 60 minute show).  The hardest part for the two of us was the scene where the doctor had to tell the parents that their child was terminally ill and there was no treatment unless diagnosed when there were no symptoms present. It brought back diagnosis day....we both remember it vividly.

Max with Josiah - starting to struggle holding his
 head and couldn't sit on his own anymore.

We all went through a month of hospital visits, tests and doctor's appointments to try and figure out what was wrong. December 1st, 2014, we were in the lobby of the neurologist's office for an appointment to talk about additional procedures we could do to try to figure out his diagnosis.  We saw so many children and families with visible disabilities...oxygen machines...machines and monitors I'd never seen before.  Here we had Max in a baby carrier...he was fussy but relatively normal looking on the outside.  He was starting to lose the ability to hold his head up strongly and needed to lean on us.  He could sit up on his own but leaned very far forward and recently was falling over all together. We both looked at each other without words but both asking each other with a simple look...what is wrong with our child...is this our future....will this be Max?  Little did we know he was struggling on the inside. We finally got called back into the room and both were waiting anxiously for the neurologist to enter the room. We were both telling Max how proud we were of him and how all of this was a speed bump and he'd prove the doctor's wrong and get healthy soon so we could get back to our normal routines.  Then it happened.....in walked the main neurologist and her assistant.  In walked Max's genetics doctor...we had been waiting for results from his bloodwork taken 2-3 weeks prior....but why was he here with the other doctors? They work at the same hospital...but they aren't in the same office...and it wasn't planned that he be included in this appointment...

"We got the results of the bloodwork," the genetics doctor told us.  "It is confirmed to be a leukodystrophy which is what we were testing for."  He had told me at the initial appointment not to google leukodystrophies...just wait until we determined if we were heading down that road...but I needed to know what we were looking at...and what we were facing.  "Max has a leukodystrophy called Krabbe disease."....my heart sank....I had read about so many different leukodystrophies but this one stood out to me as the one I was hoping he didn't have.

"Is that the one the football player's son had...Jim Kelly's son?" I asked.  I had researched a bit about it online.

Almost instantly I received a head nod..."Yes, " he said. I knew that it meant no cure. I knew that it meant short life.  Everyone told me the odds were in our favor and the blood work was precautionary. "There unfortunately is no treatment or cure for the disease."  Tears were welling up in Josiah's eyes - I rarely see him cry.  I on the other hand was in such shock to the point that there weren't any tears that I could release..fight or flight mode...just trying to get information and stay focused enough that I could get the info to help Max.

"Was there anything I did wrong in my pregnancy with him?" I asked.

"No it is completely genetic and nothing you could have done.  You both have the gene that creates Krabbe.  You both have to give that gene to your son.  There is a 1 in 4 chance for each of your children...a chance that neither of you pass the gene, a chance that one of you passes the gene and the other doesn't and vice versa and a chance where you both pass the gene like in Max's case, " he said.

"But it has never run in our families.  We've had no prior knowledge of this," I said.

"You both have to carry the gene for it to even be possible which is very rare," he told us.

We asked if there were treatments, things we can do to make him comfortable.  He told us he'd research it because he didn't get many Krabbe patients and that we should schedule for a follow-up neurology appointment in 2 months...that was it...no other direction.

Max sitting with his friend Abby 1 1/2 months
before diagnosis (end of Oct)

"Do you have any questions?" he asked.  "We are really sorry to have to break this news to you."

We shook our heads no and thanked the doctors for their time.

We both left numb.  We walked through the waiting room and everyone was going about their business...making appointments, answering questions, filling out paperwork, waiting for their turn...yet to us...the world stopped . Our son had just been labelled as terminally ill. We couldn't wrap our heads around it. There is nothing we can do. Where do we go from here? How can the world continue moving?  How can we go on with our daily lives?

We got into the elevator with another person and I started getting tears in my eyes.  We had the long walk back through the hospital to our car.  Somehow we made it without falling over.

We didn't say much - but we both were devastated, shocked and scared.  On the car ride on the way home we vowed once again to stick together and support each other (it was the only way we could get through this) and do everything we can for Max. We called our parents and told them that we needed to meet with them immediately in person. And we gave them the news.  There was a lot of emotion over the course of the next few weeks.

I started doing searches on the internet for any glimmer of hope.  We reached out to Hunter's Hope (The Kelly's Foundation) and were given some direction and support.  They introduced us to a group of families on Facebook who ask questions, vent and support one another. I value this group so much.  We are family by genes.  One of the first people I connected with was Hannah's mom (PA). She suggested us getting to PA immediately to see the Krabbe specialist and find some guidance and help.  Finally...support and direction.

Opening gifts for his first Christmas (shortly after diagnosis)

Shortly after - we consciously made the decision that we needed to stop grieving and rather enjoy Max while he is here on Earth with us. There will be plenty of time to grieve in the future but we wanted to provide him with experiences and moments that he may not get to experience. We've accomplished so much since his diagnosis - more than I've done in my lifetime.  Some of the opportunities presented and the people that we have connected with have truly been amazing and such blessings to us.

We continue to enjoy our son (and our daughter) every day.  Don't get me wrong - some days I don't even want to get out of bed because I go back to diagnosis day. But he's here...he's still physically here AND he is amazing.  I'm going to cherish him for as long as I can.

Every day with him is a gift and I don't want to waste it.