Caring Causes: Bailey Believes

Jessica DeDio’s son Bailey has Cystinosis. Bailey’s kidneys were failing and the family was using social media as a way to alert friends about Bailey’s urgent need for a transplant, specifically through the Bailey Believes – Donate Life page on Facebook. Within minutes of a new Facebook page update, Jessica received a call from Mary Jordan, whose son, Joey, also has Cystinosis. Jessica and Mary had met several years ago at Stanford, where their sons were both participants in a study for a drug commonly used to treat Cystinosis. Bailey and Joey had also hit it off immediately, and are now best friends; brought together by this ultra rare disease.

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.

What is Cystinosis?

Cystinosis is a rare metabolic genetic disease that causes the amino acid Cystine to accumulate in all organs of the body and blood stream. Cystine builds up and attacks the kidneys, liver, eyes, pancreas, brain, heart, muscles, bones, etc. The kidney is the first organ that needs transplanting as it filters out waste from our bodies. There are less than 2,000 people worldwide that have this disease. Until recently they were primarily children, as people with Cystinosis had a very short life span. Here is what the National Institutes of Health (NIH) has to say.

How did you become connected with Jessica and Bailey?

My son Joseph and I met Jessica and Bailey when all of us were starting a study for RP103 (Procysbi). This new medication released into the intestines and not the stomach so the children would have less stomach issues. It was also time released so they only needed to take it twice a day instead of four times a day. Joseph and Bailey hit it off right away. They were the same age, both red heads, same interests, and it was the first time that they had really spent a large amount of time with another person with Cystinosis. The boys looked and acted like twins. Through them the families became friendly. The study went on for a few years, and our two families would always be there together.  oe and Bailey became close friends. The rest is history!

What compelled you to donate a kidney for another family?

There are many people waiting for kidneys and being on dialysis is a horrible way live. Many of those people die while waiting. We only need one kidney to live a normal life. When my son Joe needed a kidney transplant, I was tested and was a blood match. The problem was that as Joe was adopted, we had zero genetic markers in common. I would still be able to donate to him, but he would have a greater risk of rejecting the kidney. Jessica and Bailey emotionally supported us throughout the journey. We put Joe on various waiting lists for a kidney and went on with the chance he would have my kidney. A miracle happened and a cadaver kidney became available that had various genetic markers similar to Joe. We rushed him down and he had a successful kidney transplant. As all of this was going on Bailey went into kidney failure and was put on dialysis. Family and friends were tested to see if they could donate but for various reasons they could not. Joe was doing great and Bailey’s health was failing. I had already been tested and accepted as a donor at Stanford so I said I’d donate if I was a match. I was a match and Bailey received my kidney. I donated because I love Bailey and his family. He is a great friend to my son. Also as I stated earlier, the wait for a kidney is long and you only need one kidney to live a healthy normal life. Kidneys from live donors make the healthiest kidneys for transplants. I would do it again if I could.

How can people support families that are struggling with serious, rare diseases?

Families that have children with rare diseases are just like everyone else. What they need is understanding and friends to listen to them when they are feeling down or overwhelmed. They need friends to stick around and treat them like any other family. They may need help with babysitting the children that are sick, or even the children who are well, at all times of the day and night as you never know when you need to see doctors or stay in the hospital. Unlike illnesses which are short term, or even illnesses which treatments are a couple of years, families that have children with diseases like Cystinosis deal with the disease for their entire lives. Day in and day out. There is never a break and in most cases no cure. There is also a financial burden on most of these families, so just asking if one can help in any way is great. Remember unlike cancer, diabetes, etc., orphan diseases do not get federal money or a lot of support, so another way to help is to donate money to groups such as the Cystinosis Research Network, or the Cystinosis Research Foundation. Families like ours need to raise our own money for research and hope for a cure.

What words of advice do you have for families with children struggling with serious diseases?

I would say let your children be as normal as possible. Do not bubble wrap them. Let them do all the activities any normal child does. My son played baseball, soccer, flag football, snow boards, and other sports. Yes we have had to deal with more fractures than most and use extra precautions for overheating and other medical issues, but it was worth it to see him smile. Remember our children have a disease they are not the disease! I would also advise them to join support groups of the various diseases. Talking to someone who is going through the same thing you are is priceless. 

How can people find out more about being a live donor to help other families?

To find out more about becoming a live kidney donor please look at the National Kidney foundation for information. There are also organizations all states and counties. When you give the gift of your kidney, you are giving the gift of life. Nothing feels better.

I also had a chance to interview Jessica De Dio.

What is Cystinosis?

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.

How can people find out more about being a live donor to help other families?

We are big Donate Life Supporters. This is our 2^nd 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.net

and register to become an organ donor.

Please come out to our 4^th 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.