YOUR HEALTH: When the body turns to crystals –

Posted: September 1, 2020 at 2:54 am

SAN DIEGO Cystinosis is a disease that slowly and aggressively attacks your organs, tissues, muscles, bones, eyes, even your brain.

It's a genetic disorder with no cure.

And right now, the only option for treatment is an army of pills to slow it down but missing even one dose can be devastating.

Now one man is "patient one", the first to try a new treatment that may save thousands of lives.

21-year old Jordan Janz is living with the rare, unrelenting disorder.

"I was diagnosed at eight months old and basically have been living with it my whole life."

In Jordan, cystine, an amino acid, gets trapped in his cells.

When cystine levels rise, crystals build up all over the body leaving a trail of damage... even causing him to vomit up to 13 times a day.

"It's not how strong you are physically," he said.

"I think it's how strong you are mentally when you come into this."

Traditional cystinosis treatments aim to slow the build up of cystine inside cells.

In order to do that Jordan takes 56 pills each day, but now he hopes to change that, Jordan is the first patient to test a unique gene therapy.

UC San Diego professor Stephanie Cherquie's took stem cells from Jordan's bone marrow, re-engineered the cells, introduced genes that will produce cystinosin, then reinfused Jordan with his own cystinosin-producing cells.

"So, then these cells become a source of healthy stem cells for the rest of the life of the patient," said Stephanie.

Jordan had to take chemo twice a day, but he hasn't let that scare him away.

"I'm doing this obviously for other cystinosis families, right?," said Jordan.

Hoping that many others after him will now get the chance at a better, longer life.

For those born with cystinosis who make it into adulthood, the average lifespan is around 28 years old.

We're told Jordan Janz is making a good recovery. though it is still too soon to tell his long-term prognosis.

If this story has impacted your life or prompted you or someone you know to seek or change treatments, please let us know by contacting Jim Mertens atjim.mertens@wqad.comor Marjorie Bekaert Thomas

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YOUR HEALTH: When the body turns to crystals -

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