A Foundation Research Update

It feels like my gaps between writing are getting longer and longer.  Truth is, I really miss writing … I just find the days slipping away from me between work, Koa and pregnancy (can you believe i am 32 weeks already, eek!!).  I am just not sitting down to gather my thoughts at the end of the day like i wish I could.

First an foremost I want to thank you all SO much for reaching out, the cards, the balloon releases and so much more that you all did to celebrate Wylder on what would have been his 6th birthday (May 15th).  We miss him so much, and this year seemed more bitter sweet then ever as we were reminded that we had 3 beautiful birthdays with him and this was our 3 year without.  We celebrated him, like I feel like we do most days, but this day with a bit of cake and balloons too.  Koa is starting to learn and understand more about his big brother and points to his picture when we ask “where is Wylder” and can now say “Wy” to let us know he is in his heart too.  I wish they could have known each other in this life.



As most of you know, our second annual Living Like a Warrior Gala is just write around the corner.  With that fast approaching Steven and I thought we would share some pictures and information about a project that Wylder Nation Foundation is funding and very excited about.

This is a Phase 1 dose response study of the prevention of sphingomyelin-induced brain pathology by intrathecal injection of Adeno-Associated Virus Serotype 9 carrying a competent copy of the human acid sphingomyelinase gene (AAV9-hASM) in pre-symptomatic ASM knock out mice…So what does all that mean?

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Essentially this is to find an optimal dose in mice for a potential gene therapy treatment to prevent the neurological manifestations of Niemann-Pick Type A. The hope when all is said and done is to replace the defective gene (SMPD1 gene) responsible for causing the neurological components of the disease with a working copy of that gene through what is known as an Adeno-Associated Viral Vector with a specific serotype that has been developed by a lab that we are working with at UCSF.


We are confident if we can find an optimal dose of this therapy that can spread throughout the brain of these children without causing any detrimental side effects, the neurological manifestations of Niemann-Pick Type A can be permanently fixed with a single, fairly non-invasive infusion. At the end of the day this treatment could potentially be used in combination with an enzyme replacement therapy developed by Genzyme that is currently in clinical trials to assist with the systemic manifestations of Niemann Pick Type B (the non-neurological form of the disease) to treat NPA Children.


While there is still a lot that needs to be tested and worked out for this potential treatment combination, HOPE IS FINALLY ON THE HORIZON for the children and their families suffering from this devastating disease.

It feels like all things are possible because of Wylder and we thank you all so much of your continued support.  We will share more updates, projects and happenings about the foundation at the gala.  So if you haven’t purchased your tickets already, be sure and snag yours AT A DISCOUNTED RATE today!  click here to purchase

We love you and we miss you Wylder.  See you in our dreams little darling. Keep your littlest brother safe and thanks for the zestyness of Koa, we love him so much.  xoxo

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  1. That research sounds incredibly promising! It is a long road, but the journey has started! Shelby us also starting to point to Dan and say his name. And it hurts every time. I believe that they know each other in their hearts.

  2. Yeah!!! I keep rereading this. I’m sooooo happy there is hope when we had none. I remember that horrible day so well and I’m so thankful that very soon no one else will have to hear what we did. Thank you for all you do. Jarrett and I were actually just laying in bed looking at his work schedule and trying for him to come:) happy bday wylder,!! We love and miss you!

  3. Cathy Johnson says:

    Such wonderful, wonderful news! Hope IS on the horizon! Thank you Steven and Shannon for listening to your hearts and letting Wylder lead you in these efforts! I am so excited about the possibilities of this research that my heart is bursting. I simply cannot imagine what this means to the mamas and daddies of all of the sweet NP angels. I so hope to see you all at the Gala this year, but it’s still a maybe. Becky is getting married in September, so wedding plans abound and we have some necessary travel this summer. But – you never know! Again, so happy to hear this news and to catch up with you. Koa is simply adorable and Shannon, your bump is beautiful! Hope you are feeling well. You sure look beautiful! Sending love – xoxo

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