What I am trying to achieve!
WHAT I AM DOING: At the end of March I will attempt to ski 450 runs in 48 hours and raise $30,000 for Cystinosis Research! Starting on March 23rd I will ski 250 runs at the first ever 24 Hours of Sun Valley. I will then finish my quest the following week by skiing 200 runs at the 4th Annual 24 Hours of Schweitzer.
WHY: It is simple really, if a lot of people do a little bit, then big things can happen.
WHO AM I: My name is Matt and I am just a normal guy, with a normal job, leading a normal life. It was about 4 years ago that I was fortunate enough to meet a young boy who has been dealt a tough hand. Diagnosed with an “orphan” disease which makes him constantly feel as you or I would at our weakest and most tired moment due to copious amounts of medication. There has been a foundation created which organizes events geared towards raising awareness and money on his behalf to fund the research needed to find a cure. Primarily the events have been 24 hour endurance events held once a year. As great as those events have been, it was not until following the most recent that I felt compelled to find a way to continually “pay it forward”. By creating a place where people can support my physical efforts in various things I hope to give many people the opportunity to do a little thing that will hopefully make a big impact on the lives of others.
WHO IS HANK: Hank was diagnosed with Cystinosis in November 2007 after seeing a team of specialists at Children's Hospital in Seattle. He was 16 months old. He then began the demanding medication schedule of five different medications every 6 hours, 8am, 2pm, 8pm and 2am.
Before Hank was diagnosed the disease had already caused him to develop other problems including Fanconi Syndrome (kidney failure) and Hypophosphatemic Rickets (bone disease that causes soft, bowed bones). The nutrient imbalances due to these conditions cause increased urination, thirst, dehydration and abnormally acidic blood levels.
In May of 2008 Hank had a successful operation for the placement of a g-tube in his stomach so his parents can administer the medications through the tube instead of forcing him to drink them. This has been a tremendous help in being compliant with his med schedule.
Since being diagnosed Hank takes 22 doses of medicine per day to maintain his condition. He also takes a Growth Hormone shot 6 days a week. He has occupational therapy, physical therapy, and speech therapy weekly, blood draws every two months and is seen by a kidney specialist quarterly to monitor his kidney function.
Despite having a terminal illness, Hank is a happy and courageous little boy who makes those around him appreciate life and the true gift he is.
WHAT IS CYSTINOSIS: Cystinosis is a rare metabolic disease that affects approximately 500 people in the United States (mostly children), and about 2,000 people worldwide. This recessive genetic disease causes the amino acid "cystine" to accumulate in the cells of the body. Over time, the cystine damages various organs including the kidneys, liver, muscles, white blood cells, eyes, and central nervous system. Other complications include muscle wasting, growth loss, difficulty swallowing, and developmental delays. Termed an "orphan disease," Cystinosis is a disorder which has not been "adopted" by the pharmaceutical industry because it provides little financial incentive for the private sector to make and market new medications to treat it or prevent it. Although there is a medicine that prolongs the lives of cystinosis sufferers, there is no cure. Cystagon, the powerful medication used to treat cystinosis, must be consumed every six hours. But the medicine’s severe side effects and demanding dosing schedule, for which parents must awaken children nightly from their sleep, often results in poor compliance. Eye drops are available to stop the painful accumulation of cystine crystals on the corneas but they must be refrigerated and taken every waken hour. Researchers at the University of California at San San Diego developed a slow-release medication to enable cystinosis sufferers to take the medicine every twelve hours instead of every six hours. Raptor Pharmaceutical's adopted the new drug and has just completed the Phase 3 clinical trial. We hope that the new medication will be approved by the FDA in 2012.