Mary excitedly reported that her blood type matched what was required and that she could be Bailey’s donor. Just like that; no hesitation, no second-guessing. After more testing at UCLA confirmed Mary as a qualified donor, surgeons removed one of her kidneys and transplanted it into Bailey about a year ago. Today Bailey and Joey are doing terrific! A family friendship born out of a shared health crisis led to this life-saving transplant.
I had a chance to interview Mary Jordan to learn more.
Cystinosis is a rare “orphan” metabolic disease that afflicts approximately 500 children and young adults in the US and approximately 2,000 worldwide. Cystinosis causes the amino acid cystine to accumulate in the body’s cells. As proteins are degraded within the lysosomes of cells, individual amino acids that make up the proteins, are transported from the lysosomes to the cell’s cytoplasm via specific transporters. In children with Cystinosis, the transporter for cystine is defective causing cysteine to accumulate. The cystine content of the cells is 50-100 times greater than normal.
As the cystine accumulates in cells, it slowly damages all cells and organs including the kidneys, liver, thyroid gland, eyes, muscles and brain. Because the disease affects such a small population, research money is almost non-existent. Cystinosis is a common cause of the Fanconi Syndrome, a rare renal tubular disease. By about one year of age the urine volume increases in cystinosis patients and they excrete large amounts of salts and other minerals in their urine. Without treatment these children progress to end-stage renal failure by an average age of nine years old. In the past this meant death. Today, these patients can receive renal dialysis or transplantation but even with successful transplantations, they develop abnormalities in other organs.
Bailey takes 85 pills per day to stay alive and eye drops every hour to keep from going blind.
What words of advice do you have for families with children struggling with serious diseases?
Conferences, Facebook, talking to each-other, listening to one another and being there for each other and loving one another.
We are big Donate Life Supporters. This is our 2nd year having a Donate Life Team for the Donate Life Walk @ Cal State Fullerton. Bailey walks it with us <3 124="" a="" amp="" are="" awaiting="" beautiful="" can="" children="" don="" donate="" gift="" give.="" go="" heaven="" here.="" href="http://donatelife.net" in="" is="" knows="" lease="" life="" lord="" men="" most="" nbsp="" need="" organ="" organs="" person="" recious="" states.="" t="" take="" the="" them="" there="" to="" transplantation="" united="" we="" women="" your="">donatelife.net3>
Please come out to our 4th annual Bailey Believes Dirt For A Cure on November 7, 2015. Please visit www.cystinosisresearch.org/donate-for-bailey for updates. 100 % of ALL donations go research to find better treatments and our wish a dream a cure for Cystinosis. All costs are underwritten by friends of Cystinosis Research Foundation.