Cystinosis is a rare, inherited, metabolic disease that is characterized by the abnormal accumulation of the amino acid cystine in each cell. The build-up of cystine in the cells slowly and eventually destroys all major organs of the body including the kidneys, liver, eyes, muscles, bone marrow, thyroid and brain. Although medication is available to control some of the symptoms of this insidious disease, Cystinosis remains incurable.

Friday, May 18, 2007

My Wife

I wish that I could report positive news. I wish more than anything that I could communicate that things are progressing well and that the adjustments that we are making are getting easier to deal with; the fact is things have taken a negative turn. Holt's levels continue to decline and his meds dosages continue to increase. I realize it sounds like a broken record but the reality is that Holt has an incredibly rare genetic disease that will signifcantly impact every major organ of his body. He is going to need a kidney transplant, he is going to need eye drops every waking hour to prevent him from going blind and he is going to have to take a cocktail of meds every 6 hours for the rest of his life. He will never get a full night's rest and he will continually be a prisoner to words like research, funding, experimental and trial. I have to be able to keep this in better perspective. At the end of the day though there isn't a thing in the world that we can do about it but stay positive, ensure that he gets his meds on time, provide a stable, loving and caring environment in which we all can thrive and do what we can to enable research. This post isn't just about Holt- the cuts get deeper. As a disclaimer, I realize that there are a lot of folks who read this and I appreciate that. I do also need to set an expectation that as much as this is about providing friends and loved ones with information; it's equally as much therapeutic and cathartic for me to be able to write it out. If it gets or seems harsh, banal, draconian, relevant, irrelevant (you'll form your own opinion)... it is what it is and hopefully you now at least can potentially understand why.

I often struggle that if I'm going to write about Holt and his condition then isn't it also fair to give a holistic view into how we are trying to deal with this new life? At an idealistic level, I guess that my hope is that, in some way over time these posts will provoke you to slow down a little and think a little bit differently about the world in which we live and the people in it. Maybe it will enable you to make your own changes in a meaningful and positive way- or at least to truly appreciate the "what we have" instead of the "what we want".


The words "failure to thrive" are taking a toll on Chrissy. It's hard to relay the internal despair that I experience when I look into her soul. She has this incredible will, laser focus, tireless dedication and enthusiastic spirit and I know that as hard as she works and as much as she puts into it; that she ultimately feels helpless... it's maddening to me. She sleeps with a cell phone next to her ear every night- wakes up at 4am to deliver the worst tasting, worst smelling meds to an 18 month old child that she has to wake from a deep sleep; calm, force feed, and comfort him back to sleep...and then she starts her day. I don't know what other people do in situations like this; I cannot imagine all of the different coping mechanisms that people use to try to get back a semblance of normalcy when facing true infinity. I guess we will keep learning. It's important to me for you to know what an amazing person, wife and mother that Chrissy is. Never in my wildest dreams could I have imagined being able to live my life with someone who is as patient, kind, devoted, caring and talented as she is. She is a one of a kind; the real deal and I'm the luckiest man in the world to be able to walk lock-step with her.

I do know that Holt's condition has changed the physical nature of our lives but I haven't concluded yet how it will shape our life purpose, goals and objectives. The easy answer is to go straight to research funding-but I want more than that for Holt; he deserves more than "just a cure"-- he deserves a better conclusion than what he is signed up for. At least, I want him to be able to live his life knowing that because of him, other people are able to be...better people. Is there a better legacy to leave than to enable a positive change in others and for them to reflect that change in others?
Until next time, thanks.

1 comment:

Katherine said...

Jason,

You have brought tears to my eyes. What you said about Chrissy was SO beautiful and SO true! I have always admired her as a person and friend and even moreso as a mother. She is someone that I look up to. She has SUCH an amazingly positive attitude, she is SO strong, together, smart, beautiful, and just a loving, caring, giving person. I LOVE HER!

As for you Jason, YOU are amazing! You are an incredible husband, friend and partner to Chrissy and give her SUCH strength and encouragement when she needs it most! You both walk hand in hand in all that you do and it is SO evident the level of respect, love and admiration that you have for one another these past 15 years. You also are an incredible father to your 3 children, and I KNOW that they look up to you greatly! I love you too!

I pray for TOTAL and complete healing for Holt. I remember when my Dad was diagnosed with cancer almost 3 years ago (and going strong), someone told me to THANK GOD IN ADVANCE for healing my Dad. Be SO confident and faithful about it and just praise him and thank him for total healing. I am doing that for Holt. I also pray for Holt to be pain free, for Holt's medicine miraculously one day to start tasting like the most FAVORITE tasting food that he knows, that his eyes are healed, that his vomiting gets less and less and just STOPS, and that he just feels good, pain free, happy and strong.

I am SO glad that he is enjoying Spicy! I am sure that the bond is mutual with Spicy enjoying him.

God does not make mistakes. He chose you two to care for Holt because of the AMAZING parents that you are. HOlt has been SO blessed to be born into the "Grier" family and I know you all are receiving more blessings from this than you would ever expect. Your appreciation for the "simple" things in life: family, friends, good times, quality time, having fun together, caring for your precious baby, and just an overall "different" outlook on life. That is where the biggest blessing occurs!

I would love to help take care of Mary Logan and Jack while you all are in Austin for the conference in July. Ask and I am there!

I love you guys!
God bless and much love,

Katherine
AKA: KD