Family Spotlight

Steven González, Jr. ...
COTA Hispanic Teen's Community Fundraising Efforts Result in More Than 400 Individuals Added to the Bone Marrow Registry

The morning of October 14, 2006, will be forever etched in the memories of the González family of The Woodlands, Texas. It was on that morning when 12-year-old Steven González, Jr. was camping with friends near the Gulf of Mexico and woke up with red dots on his face. Later in the day, Steven took a tumble and received a large gash on his hand, which was then exposed to the water of the Gulf. When his family rushed him to the doctor, they were told that Steven's platelet and white blood counts were dangerously low, as was his ability to fight infection.

"We will never forget those words that day," said Mom Rosie González. "Preliminary diagnosis ... leukemia. Then the sky opened up over Houston and the streets flooded. It was as if all of creation was sharing our pain. Ten days later, we were told Steven had Acute Myelogenous Leukemia (AML M6 Leukemia), and he had only a two percent chance of survival. But we never gave up hope."

Steven's only hope was a bone marrow transplant. In preparation for the transplant, Steven began his first chemotherapy treatment on Halloween of 2006 at the Children's Cancer Hospital at M.D. Anderson Cancer Center in Houston, Texas. Just when the González family was starting to think Steven would beat the odds, there came more devastating news. The family's insurance company would not cover the search for a bone marrow donor for Steven. Without a donor search Steven's possibility of finding a match, and receiving a second chance at life, was severely reduced.

According to Dad Steven González, Sr., "We were beside ourselves wondering what we would do, and then we heard about the Children's Organ Transplant Association, COTA, and our hope soared again."

Within weeks of learning about COTA, the González's friends and families quickly mobilized and started working to raise the necessary funds to help pay for transplant-related expenses. Steven's bone marrow registration drives caused excitement, and not just in his hometown. Over a period of several months, bone marrow drives were held in the Texas cities of Houston, El Paso and San Antonio, as well as in New Jersey, New Mexico and California. According to Steven González, Sr., "We quickly learned the bone marrow registry does not have the needed representation of the Hispanic community. In order to help our son, we had no choice but to work to increase the number of Hispanics in the registry."

As of February 2008, the National Marrow Donor Program (NMDP) reported that of the 7,000,000 adults registered with NMDP, only 650,000, or less than 10%, are of Hispanic or Latino descent. While more than 3,000 Hispanics received a tissue or organ donation during 2007, the Hispanic community is in desperate need of organ and tissue donors.

According to Steven González, Sr., "The response to Steven's bone marrow drives was tremendous and resulted in a significant increase in the Hispanic representation on the nation's bone marrow registry. With COTA's assistance, and with the dedication of our friends and family, we found Steven a match and provided hope to numerous other Hispanic families who might not have found a match."

A total of 14 bone marrow registration drives were held in honor of Steven González, and they resulted in more than 400 individuals being added to the bone marrow registry. According to Steven's COTA Campaign Coordinator Linda Bankerd, "I held a bone marrow drive at work. I don't know how many people signed up, but I can tell you that many people were touched. I was extremely honored to raise awareness of the critical need of minority bone marrow donors, and I am very thankful to have opened the eyes, minds and hearts of even just one person who signed up as a donor. I will forever be changed after working with COTA and after witnessing Steven fight this battle to find a donor."

Steven's Aunt Melanie González coordinated bone marrow drives in New Jersey. She recalls, "It was difficult being so far away from Steven when he needed the help the most. Coordinating bone marrow drives allowed me to feel like I was helping from afar. The truly wonderful thing was I wasn't just helping my nephew, but other people too."

Steven found a donor, but from an unexpected source. The parents of two Hispanic baby girls donated their umbilical cord blood to Steven. On February 13, 2007, Steven received his transplant, and his second chance at life. He set many records post-transplant. Steven's new cells engrafted in just 11 days; Steven was released from isolation in only 12 days; and he was released from the hospital a mere 18 days after receiving the donor cord blood.

According to Steven González, Sr., "Just like any other father, I have a deep desire and need to provide for my family, no matter what the circumstance. When the expenses started to add up for the bone marrow donor search, I truly didn't know how I was going to be able to provide for my family. COTA enabled the generosity, the prayers and the loving gifts of family and friends to lift us up and to offer financial relief during this time of great need."

According to Rosie González, "Watching my child face a serious illness is one of the most difficult things I have ever had to go through, but COTA helped in so many ways ... by offering financial assistance and by allowing us to keep in communication with our family through Steven's COTA website. COTA helped make an impossible situation seem a little bit easier."

Today, Steven is 18 months post-transplant and is living life just like other 14-year-old boys. His family truly believes his diagnosis; his journey to transplant; the community support to help him and his family through COTA, and the effort to add new Hispanics to the National Marrow Donor Registry are all reasons he is thriving today -- to impact lives by telling his story and increasing the country's awareness of the need.

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Transplants Today

First Heart Transplants in Children Following Donor Cardiac Death

The practice of donating organs after cardiac death, rather than brain death, has gained acceptance in recent years, but only for organs other than the heart. There has been one report of a successful heart transplantation in an adult that involved a donor who died from cardiocirculatory causes, but concerns about the vulnerability of the heart to ischemic injury has limited further cardiac transplantation in adults from donors who have died from cardiac causes, according to Dr. Mark M. Boucek, Joe DiMaggio Children's Hospital in Hollywood, Florida.

But in children, heart transplant has been performed after prolonged ischemic injury in a donor, and the results were similar to those for donors without ischemic cardiac injury, Dr. Boucek and his team noted. And the need for more pediatric donors is pressing, he stressed. Dr. Boueck reported his team’s investigational findings in the August 14, 2008, New England Journal of Medicine, and the transplants were conducted at Denver Children's Hospital, in Denver, Colorado.

"There are a significant number of kids who have congenital heart disease or develop disease of the heart muscle that prevent them from surviving, and their only chance is a heart transplant," Dr. Boucek reported. And, "the worst age for donation is in those under one, not because there are not a large number of children who die at this age," but because doctors feel a little less comfortable with declaring even brain death in such infants, "so many of these children are not offered the option."

According to Dr. Boucek, the parents of dying children are amenable to organ donation, stressing the positive feelings that arise for the donor's family from giving the potential for life to another child.

When kidneys, lungs, livers, and other organs are transplanted after cardiac death, there are a number of differing opinions as to the length of asystole required before pronouncement of death, with times ranging from two to five minutes routinely used. But autoresuscitation of the heart has never occurred any later than 60 seconds after asystole, so this two- to five-minute period is a somewhat arbitrary concept, Dr. Boucek explained.

And when the discussion moves from transplanting other organs to transplanting the heart, this time period is vital, he said, because, "the heart is the most vulnerable organ, and it needs to work at its optimum right after transplantation." The longer the period of time following asystole that is required before pronouncement of death, the more likely the heart to be transplanted will become damaged, jeopardizing the recipient.

Hence, in their protocol, Dr. Boucek and his team started with a period of three minutes after asystole for the first transplant they performed, but thereafter, "the feeling from the entire team was that we should shorten this, as we may have jeopardized the recipient." So, following extensive discussions including the hospital ethics committee and the data and safety monitoring board, they adopted a period of 75 seconds for the next two transplants.

"The outcomes after transplantation of hearts from donors who died from cardiac causes were similar to those associated with traditional organ donation," Boucek said. "All three recipients survived despite being at high risk for death," and the six-month survival was 100%, compared with 84% for 17 control infants who received transplants procured through standard organ donation.

The one great limitation of this procedure," said Dr. Boucek, "is that you have to have a donor and a recipient at about the same time." If, however, the concept of heart transplantation after cardiac death could become mainstream and a donor heart procured following cardiac death could be flown anywhere within reason (as is the case currently for hearts donated after brain death) "this would have a huge impact," he stressed.

"For pediatric heart donation and transplantation involving patients who die from cardiocirculatory causes to become a more frequent option for end-of-life care and to affect significantly the nationwide risk of dying while waiting, the concept of distant sharing of donated organs from these donors should be considered," Dr. Boucek and his
colleagues concluded.

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Bone Marrow Transplants:
Cancer Drug Shows Promise Against Graft-Versus-Host Disease

The University of Michigan scientists tested the effects of the drug SAHA, as well as another member of a group of drugs known as HDAC inhibitors, on key immune system cells called dendritic cells. In mice, both drugs were able to significantly diminish the destructive inflammatory effects that these cells cause in graft-versus-host disease. Currently, plans are under way at the University of Michigan for an initial trial of the drug in people as a new way to prevent graft-versus-host disease. Researchers expect to begin a trial within a year.

Graft-versus-host disease occurs when immune cells in the transplanted bone marrow mount a misguided attack on body tissues. If HDAC inhibitors turn out to be safe and effective in people, they might offer a treatment option preferable to the immunosuppressant drugs used now to treat the disease. These immunosuppressant drugs leave patients vulnerable to infection and have other significant side effects.

"To make bone marrow transplants more effective, we need better control of the very powerful reactions between the immune systems of the donor and recipient. This study shows how drugs like SAHA regulate those reactions, and takes us a major step closer to bringing this new approach to patients who need transplants," said James L.M. Ferrara, MD, Director of the University of Michigan Combined Bone Marrow Transplant Program and a senior author on the study.

"These HDAC inhibitors were thought to just kill cancer cells, but at lower doses, it's possible they can modulate a number of immune diseases," said Pavan Reddy, MD, the study's lead and corresponding author, and an Assistant Professor of Internal Medicine at the University of Michigan Medical School. "The mechanism we have identified in
graft-versus-host disease may be involved in autoimmune diseases as well."

Bone marrow stem cell transplants are most commonly used to treat leukemia and lymphoma. By replenishing depleted blood cells, the transplants allow higher doses of chemotherapy to more effectively get rid of cancer cells. But as many as half of bone marrow transplant recipients develop acute or chronic symptoms of graft-versus-host disease, which can affect the skin, liver and gastrointestinal tract. Dr. Reddy calls the disease, "the single biggest barrier to bone marrow transplant."

The study suggests a novel way to treat graft-versus-host disease with an already available drug that is stirring considerable interest as an anti-cancer agent. The FDA approved SAHA, marketed under the name Vorinostat, as a treatment for one kind of lymphoma in 2006 and for leukemia in 2007. SAHA is being used off label for other cancers, including lung, brain and colon cancer.

The University of Michigan study adds to a growing body of research suggesting HDAC inhibitors also may be useful tools to reign in misguided immune responses. Researchers elsewhere have recently shown that HDAC inhibitors have been beneficial in animal studies of lupus and inflammatory bowel disease. Other studies suggest the drugs could be useful in regulating the immune response in heart and islet cell transplants.

The University of Michigan researchers studied the responses of immune system dendritic cells in mice given SAHA and ITF 2357, another new HDAC inhibitor. Dendritic cells are important immune system cells whose varied roles are beginning to be fully understood. The scientists looked at the two HDAC inhibitors' effects on mouse and human dendritic cells in culture. They found that as they suspected, the drugs acted to diminish the dendritic cells' key role in promoting pro-inflammatory proteins called cytokines. Specifically, the researchers found that the HDAC inhibitors increase the expression of IDO, an enzyme which represses dendritic cell activity.

The researchers tested the HDAC inhibitors in mice bred to display the effects of
graft-versus-host disease. When they injected the mice with dendritic cells treated with the drugs, they found the drugs were able to reduce the disease's effects.

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COTA News

COTA Receives $52,000 Donation from the Carroll Shelby Children’s Foundation

"It’s an honor to help a fine organization like COTA," said Carroll Shelby, Founder of the Carroll Shelby Children’s Foundation. "COTA’s fight to promote organ transplants for kids across the country is very close to my heart. I established my foundation 15 years ago to achieve the same goal of providing better lives for youngsters in need."

The funds were raised during the 6th Kids’ Automotive Art Contest, which concluded in April and was hosted by the Carroll Shelby Children’s Foundation, Road & Track and the AFAS. The Foundation matched the $26,000 total raised during the contest, resulting in the $52,000 donation to COTA. Sponsored by Wells Fargo and numerous automotive companies and Road & Track, AFAS Kids’ Automotive Art Contest challenged children to draw or paint the car they would like to drive when they grow up. Money was raised for COTA through contest entry fees
and donations.

COTA President Rick Lofgren accepted the donation on behalf of COTA with two COTA post-transplant patients at his side. Two-year-old Christopher Padua of San Francisco, California, received his liver transplant almost one year ago. Thirteen-year-old Myles Nesbitt of Fremont, California, received his kidney transplant nearly five years ago. Both boys are doing great post-transplant; and both boys received a standing ovation, and a lengthy round of applause accompanied by loud cheers at the Carroll Shelby check presentation on Saturday, August 16th.

"The entire COTA family is extremely grateful to the Carroll Shelby Children’s Foundation for helping us to continue giving hope and making miracles for transplant-needy children nationwide," said Rick Lofgren, President of COTA. "This gift will be used to help our most needy transplant families ensure their children have a second chance at life. Carroll Shelby understands better than most how a life-saving transplant can change a life forever, and we are thankful for his compassion and his commitment to COTA’s miracle-making mission."

About the Carroll Shelby Children’s Foundation
The Carroll Shelby Children’s Foundation was created by legendary racer and automotive manufacturer Carroll Shelby. After undergoing a successful heart transplant in 1990, he established the Carroll Shelby Children’s Foundation. Thanks to the support of the Shelby Companies, Ford Motor Company and loyal fans worldwide, the Foundation is helping children battle life-threatening illnesses and promoting the importance of organ and tissue donation. More information is available at www.cscf.org.

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COTA Fast Fact

The Children's Organ Transplant Association (COTA) provides:
Funds Available for Patients' Transplant-Related Expenses
Funds raised through the Children's Organ Transplant Association (COTA) on behalf of patients are available for ongoing transplant-related expenses that include transportation, lodging, and food for follow-up transplant center visits; medications; co-pays and deductibles; and medical care. Changing insurance plans, inflated premiums and deductibles, and benefit caps are long-term issues for most transplant patients. Having COTA funds available for post-transplant care helps alleviate these concerns. There is no deadline for the use of funds.

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