"My brother went to college in America, and it was very hard for my parents to send him there. My father worked two jobs. I’d always hear him talking to my mother about money troubles. So when I graduated from high school, I went straight to work, to help pay for my brother’s school. I never resented it, because I knew he was more intelligent than me, and he deserved it. But now he has a great job in Australia, and I wish that I’d gone to college. But you know what? That same brother married into a family with two sisters. He married the older sister. And at the wedding, I met the younger sister, we danced, and now we are married. Her name means ‘angel,’ and she is my angel. And I tell her every day that she’s better than being a millionaire. So my brother got his job. And I got my wife." (Dhana, Jordan)
My name is Josh Tejkl.
I’m 26 years old.
I’m also a Fabry Disease patient.
You’ve probably never heard of this unless you or someone in your family has it.
Fabry Disease is what is considered a ‘rare’ condition, about 1 in 40,000 to 60,000 males have it, in total it is estimated that 1 in 117,000 people have it.
The life expectancy of males with Fabry disease was 58.2 years, compared with 74.7 of the general population.
Fabry is the result of a defective gene, one that is inherited, it a lysosomal storage disorder.
What this means is that my body doesn’t break down complex fatty acids like a normal person’s would.
Overtime, the build-up of GL-3 in the body causes cell damage and eventually damaging major organs like the kidneys, heart, and nervous system, and usually one, if not more, end up failing.
There is a treatment for Fabry Disease, but no cure.
Every two weeks, I receive enzyme replacement therapy.
A year’s worth of treatment costs upward of $160,000.
I wake up 6, leave by 7, and arrive at Toledo Hospital by 8 for my infusion.
I’m lucky if I get out by 2.
I sit in the oncology department.
If I ever find myself feeling down, I remember the people from my infusions, an oncology center is full of people fighting cancer. One that I cannot imagine.
Every two weeks.
For the rest of my life.
Unless a cure is found.
The science is progressing, especially in gene therapy, but I don’t get my hopes up.
There is a lot of money to be made in treatments and public relations campaigns, not so much in cures; that would require revenue streams to dry up.
I don’t normally talk about this.
Not my parents.
Not my close friends.
I don’t want to be viewed as a victim, or someone that people take pity on.
I know that I’ll probably die before most of my friends, but I want to be viewed as normally as possible during my time on Earth.
Some people think I’m lazy. They don’t know the pain I’m in, or how tired my body is.
That’s okay, I do the best I can, and make do with the rest.
Life has been a struggle, and it will continue to be, but the struggle makes life that much more beautiful.
I don’t do 5Ks, half-marathons, or marathons.
I can hardly run.
Sometimes my feet hurt too much to even walk.
My feet burn.
My hands hurt.
My body aches.
The reason why I’m writing this is because of the cold water challenge.
I first want to say that I have a tremendous amount of respect for Pete Frates, and anyone else suffering from a life altering condition.
However, raising awareness of ALS or any other condition doesn’t do much for those with it.
Helping people find a cure would be tremendous, but this doesn’t come from simply throwing money at problems.
In the United States, it is estimated to cost over $4 billion for a new pharmaceutical drug.
One of the main reasons for this is FDA regulation.
The barriers that scientists and researchers have to jump through are ridiculous.
Without the outbreak of Ebola, the FDA is finally letting some patients have access to treatments that may cure them, but why do they wait so long? Many people with life altering conditions should not have to wait until they are on their death bed for drugs to be available to them.
The conversation I would like to see take place is one that asks how we can make it easier for scientists to find cures.
Not one that simply promotes awareness of a condition.
Raising money is great, but it’s merely a drop in the bucket compared to the costs.
Once we see these costs come down, once the barriers to entry are lowered, we will see real progress in the human condition.We should also ask ourselves, our community, and political leaders, why do we have money for war, but no money to help those who cannot help themselves?
If anything ever happens to me, I would like to think my friends would step-in and help my mom. However, right now, while I can still function and manage my day-to-day life, I want to be treated as normal, at least as normal as can be. A condition doesn’t define me. Yes it’s a part of who I am, but it is only that, a part, not the whole.
I don’t want people to dump cold water over themselves, I want them to ask how they can actually make a difference. This isn’t about ‘likes’ on Facebook, making it to SportsCenter, or having Mark Zuckerberg acknowledge Fabry Disease. This is about getting cures for people who need them. Many people talk about raising money, but what they should be talking about is why it cost so much in the first place.
Once again, thank you for reading,
Among other things, I’m known to be a “reductionist.” In my line of work you must be good at pinpointing what to focus on – that is, the major underlying truths and problems in a situation. I then become obsessive about solving or fixing whatever they may be. This combination is what perhaps…
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