Thursday, October 30, 2008

The Diagnosis - Leigh's Disease


This might be the hardest post we've ever made. We knew this day might come where we may finally get an answer, but not all answers are the ones you want to hear. Braden has been diagnosed with either Leigh's Disease/Leigh's Syndrome or Leigh-Like Disease/Syndrome. It really doesn't matter, as the outlook and prognosis are the same. Braden has shown signs/symptoms since his birth, so his outlook means we are already on "borrowed time". (If anybody has any songs or poems you could send me about being on borrowed time, I'd love to have them). But ultimately, we are ALL on borrowed time. God is the only one in control, and we are not giving up hope, that God will bless our family with more time with Braden. I guess no matter how much time we get, it probably won't ever feel like enough.

Braden is such a fighter, so his fight may buy him more time with us. We have put him on the supplement regimen, what they call a "mito cocktail"... his will consist of CoQ10, thiamine, and carnatine for now. The supplements cannot cure his disease, but they can help make him more comfortable, and may help slow the progression of the disease. Braden also doesn't perfectly fit into all the things associated with the disease that are symptoms/markers/indicators... like "failure to thrive" is not in his vocabulary, as the double chin, puffy feet, dimples for knuckles, and the size of his wardrobe will all tell you.

We still believe everything happens for a reason, and this visit to OLOL is no exception. If our doctor had not wanted Braden to have this special ventilator, we wouldn't have been waiting around for an extra few weeks, and allowing us to come to the decision that since we had the time, and it had been a year since Braden's last MRI, that we should use the time to get a good look at his brain to see if there were any notable changes. And if the ventilator wouldn't have malfunctioned, we would never have gone back into the hospital, and Braden would not have been in such a worrisome condition which prompted a new MRI to compare it to the one before we left. Since both MRI's showed signs of progressive brain damage, we did an MRS (like an MRI, but looks at the cell function in the areas where there is damage) which confirmed the suspicions of our neurologist that what we have is Leigh's disease, and it IS progressing. We do know the area of the brain that is affected is related to his respiratory issues... and they say that these kids usually die due to respiratory or cardiac complications. The closest thing I can tell you about what we can expect is that he won't die of the disease, he will most likely suffer complications from respiratory failure or cardiac failure. In our case, it will probably be respiratory.

So where do we go from here? Well, we have a new ventilator (totally different brand), the LTV 950. Braden will be "resting" on the vent for several weeks as we transition him home into our regular routine before we can start trying to wean him off again. Want to hear some good news? Our little man has been on ROOM AIR with the vent for a whole day!! He is not requiring any oxygen support to maintain his sats in the 97-100 range! This means he is able to oxygenate on his own! :-) Our medical team will be doing what they can to get us home as soon as possible, so we don't spend any more time than necessary there.

THE BIG MEETING:
We did meet with Braden's entire care team: intensivist, pulmonary specialist, nursing manager, respiratory manager, social worker, vent/supply company, and the overseeing physician (who also happens to be our cardiologist). They were all extremely receptive to our concerns about what happened to us, how to make sure this won't happen again to us, and our suggestions on policies/procedures to implement to ensure better quality care for ALL patients who follow in our footsteps. They had already recognized one major breakdown in communication, and Tuesday had started what daddy and I are affectionately calling "Braden's Rule"... where the specialists, intensivists, social worker, child life, pharmacy, and respiratory all do rounds together at the same time (like a mini-family meeting) once a week for ALL vent-trach patients!! We were so pleased to see that they were already taking steps to ensure future family communications were more inclusive for all parties responsible for Braden's care (and the other kids too)!!!! We made some suggestions on transitioning from the hospital to home too that I think they will take to heart and try to implement for families going home on vents. They thanked us for taking the time and having the courage to confront all the issues with the entire team responsible for his care, and they thanked us for our candid comments and suggestions on how to make it better for EVERYONE. It made us feel like what happened to Braden was not all for nothing, they really do want to implement change where it's needed. They realized this need for change, because it could have ended differently if the parents were not as involved and astute to their child's needs like Brad and I are. It was a wake-up call, and they responded in a manner that makes us more comfortable about the future.

If you'd like to read more about the disease, here are some links:
http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene&part=narp
This one is the most comprehensive and the exact same 32 page print-out the neuro doc gave us, and a lot of it is doctor-speak that's over our head, but there is some really good info if you sort through it to the layman's portions:-)

http://www.ninds.nih.gov/disorders/leighsdisease/leighsdisease.htm
http://www.healthline.com/galecontent/leigh-syndrome
http://www.rareglobaldiseases.com/leighs_disease.php

Continue to pray for us. Braden needs strength and healing so he can come home. Mommy and Daddy will need strength, courage, patience, peace, and wisdom as we start making decisions about his care, and put together a plan for future parameters on how much support we will give our son to sustain his life, because technically the body can live forever on a ventilator. These are not easy decisions, and ones we probably won't share with everyone, as these are very personal decisions we have to make, and no matter what we decide, I am sure there will be opinions on both sides. I remember feeling gut-wrenched over the woman in Florida who's husband and mother did not agree, I would have never imagined that one day we could be in that situation too. We are praying for God's hand to guide us and let us carry out His will for our son. After all, my friend Stacey always reminds me that Braden does not belong to us, we were only asked to care for him here on earth for the amount of time decided by the Father. We will have to give him back someday, I pray that we have the strength for when the day will come.

We still have HOPE. Braden has already beaten the odds so many times it's almost unfathomable how much he is willing to fight to stay with us. We will keep HOPE that Braden being the anomaly that he is (because he still doesn't perfectly fit into Leigh's with all the symptoms), that he will once again prove the doctors wrong, and we will talk about our diagnosis for many years to come. Maybe we can be that 25% who lives longer than the current expectation.

Thanks for all your continued support, and for the prayers we know you will be sending up in the days ahead. We need them.
God's love and blessing to everyone!
The Wilson's (Braden, Kodi and Brad)

23 comments:

Doris Glass Heckert - Jackson's Mommy said...

Kodi, Brad, and Braden,

Ya'll are in our prayers, as well as those of our family---if there is ANYTHING we can do, please let us know...

Ashley said...

Kodi and Brad, We are praying for Braden. We are placing every moment in His hands. We are only here on earth for a moment and I am glad that we have all got to spend these moments with Braden. He is just so precious and of course nobody knows this more than Mommy and Daddy. Love you all!

Anonymous said...

Kodi,

As always, my prayers are with you all. Please let me know what you need and call me when you can.

Christine Loftin

B said...

Please know that we LOVE you and little Braden and our prayers are with you during this time of recovery, decision and preparation. Also remember that you are a part of a HUGE supportive community that will be here for you no matter what tomorrow holds. However sad the diagnosis, there is some comfort in knowing "the Beast" your fighting so that you know how to align your tactics. As you and many others have said, Braden is God's special child and it is clear that His purposes are being served.
Brandy

shalamarie said...

We will all wrap our arms around you and band together and make it through this. We love you. Shala & Paxton

Peggy Ransom said...

I have followed little Braden's journey, since his birth and I am humbled by your faith and strength. I pray for peace and comfort for Braden, as well as his mommy and daddy. You all are precious children of God and He loves you so much. May the days ahead be ones of much joy and little sorrow, as you continue to care for your sweet little boy!
Peggy Ransom, friend of Nana Wilsons'

Peggy Ransom said...

I have followed little Braden's journey, since his birth and I am humbled by your faith and strength. I pray for peace and comfort for Braden, as well as his mommy and daddy. You all are precious children of God and He loves you so much. May the days ahead be ones of much joy and little sorrow, as you continue to care for your sweet little boy!
Peggy Ransom, friend of Nana Wilsons'

Anonymous said...

Kodi,
You don't know me, but, I, too, have been following Braden's story for quite some time. Though a different story, my husband and I also faced a decision regarding our son's life support almost 10 years ago.
If you ever need an ear to listen or other eyes to read an email, please allow me to be of assistance.
You may email me at: mmcwhirt@hotmail.com
Melaney
May your faith sustain you and continue to give you all strength and healing.

Trucks & Tutus said...

I just cried as I read this - I am so sorry but I am also so glad that God has given Braden YOU and BRAD as his parents. I will continue to pray for all of you and will trust the fact that only He knows what is truly best for little Braden. God Bless you guys.

Anonymous said...

We will continue to pray for all of you.

God knows what he is doing, he sent Braden to you because He knew you would do the best ever for Braden.

Let us know if there is anything we can do for you other than pray.

Ray and Dorothy

kathy laurent said...

Dear Kodi and Brad, I have read the websites, I hear what the profesionals say, but like you I also KNOW WHO HAS THE ANSWERS! You will continue in my prayers as you seek God's voice on these tough decision that you will have to make in the coming days, weeks and even years. You know where I am and I have hugs waiting. I will try to see you soon. Love, Kathy P.S. give my little frog some "neck-sugar" for me!

The Strong Woman said...
This comment has been removed by the author.
The Strong Woman said...

Dear Kodi, Brad and Braden,

I am a friend of Shala's and I have been closely watching your blog with my heart now broken hearing this news. Your family is the epitome of strength. You have all overcome obstacles and continue to do so. I read your words with such awe and am impressed with all you do.

I, too, have a daughter with special needs. When she was 11 months old, she suffered an abusive head injury- one of the worst head injuries they have seen anybody survive at Phoenix Children's hospital. As she came out of surgery (brain), with much brain matter removed and damaged, I watched her on life support, laying in a coma... and I wondered.. what should I do? Do I allow this to continue? Should I take her off life support? She is alive and well even with the odds stacked against her.


So, I guess what I am saying is, kids are strong. Kids are survivors, (Braden is a perfect example of a fighter) especially when they have an amazing family such as you are. Keep up the good fight, may Braden be in that 25%. He is in this world for a reason.

Keep up the great work you all are doing.

Just know that you have a huge community of supporters rooting you on, crying tears for you, and sending tons of love for each and every one of you. Please give Braden a huge, sloppy wet kiss from my daughter, Emma and I.

JOHN said...

I sorry that I havn't keep in touch during this whole experience. Being a father at a later stage in life has really changed my outlook at things.
This whole Braden experience, I couldn't even imagine. I give you both a graet deal of respect for doing what it takes as a parent to do what ever it takes for your child.
Don't think that because you havn't heard from us, that we are not thinking of you.
On a lighter note, Hey Brad,looking a Braden reminds me of you at that age, and me riding around Grandmas yard on my Yamaha 80..I wish you could remember. You would laugh and giggle the whole ride. Love Uncle John and Angie

Caralyn said...

Songs for Braden, to lift and encourage those who are loving him through his fight:

Held by Natalie Grant, Praise You in this Storm, and Love Them Like Jesus by Casting Crowns

"The gifts lie in wait, in a room painted blue Little blessing from Heaven would be there soon
Hope fades in the night, blue skies turn to gray As the little one slips away
You're holding her hand, you're straining for words
You're trying to make sense of it all
They're desperate for hope, darkness clouding their view
They're looking to you

Just love them like Jesus, carry them to Him
His yoke is easy, His burden is light
You don't need the answers to all of life's questions
Just know that He loves them and stay by their side
Love them like Jesus"

"Chorus:
This is what it means to be held.
How it feels when the sacred is torn from your life
And you survive.
This is what it is to be loved.
And to know that the promise was
When everything fell we'd be held. "

Anonymous said...

your blog brings tears to my eyes...strangely these are a mixture of heartache and happiness. your family is an inspiration to us all.

Hillary said...

Kodi,

I can't believe this news. I am in shock. Braden is such a special child to me, and watching his fighting spirit is just so amazing. I've told you before that Braden's my hero, and I truly mean that. He is an angel, you very own cherub. But so are you. Your strength and smile and always positive nature has pulled me through time and time again. You can never know how much you guys mean to me. I can't imagine what you're going through right now, but know that I am always here for you if you and Brad ever need anything. Day or night. Check out 'Angels in Waiting' by Tammy Cochran. It was written about her two brothers who both passed away in childhood from a genetic disorder. I love you and am always praying. Give him a kiss for me.

Kristi said...

Kodi & Brad,
I have been keeping up with all of you through your blog, Gayla, and the Heckerts! All of you will remain in my thoughts and prayers. Let me know if you need anything!
Kristi Penouilh

mandycon2 said...

From LA to WV to CA to KS and beyond...I think that Braden and your family has a super large network of hope and prayers. Be strong and faithful.

DL, Mandy

Anonymous said...

i hope this disease will someday be curable..my niece was also diagnosed with leigh's..we are still hoping she can make it..she's still in the hospital..we are not losing hope.. as the song says, there can be miracles when we believe..

Chris said...

I do know how you feel because I am a parent of a Leigh's child. There are many causes for the disease and I have done extensive research in this field. There is little known about this relentless disease so it has given me the strength to return to medical school to find a way to help with coping, or maybe even curing it. Please feel free to email me so we can discuss and exchange stories. I have learned a lot as you did I'm sure. I know it is hard sometimes to read or even think about, but we all need to stick together and help these precious, innocent children. Thank you. Chris.

Hossnat01@gmail.com

Anonymous said...

Hello,
I pray for you all, I hope that someday this disease will be curable. Your blog is an amazing inspiration to all. I Do not know your family, but your story brings hope and strength to those who read it.

Kelli Oldfather said...

Kodi- Am praying for you and your family, may you blessed with the guidance and strength you need to continue this journey with Braden. Take care, peace be with you <3 God Bless