The Battle of a Lifetime
Date: December 1, 2004
In 1996, a Johns Hopkins investigator uncovered and re-opened a 30-year-old clinical protocol that offered the promise of a cure for patients stricken with the rare and deadly immune disorder known as severe aplastic anemia. This is the story of 17-year-old Jae Jin, one of the first to be treated with the experimental therapy.
By his own admission, Jaehoon David Jin’s (Jae) life was heading in the wrong direction. Throughout middle school, good grades had always come fairly easy to him, but now things were different. His parents got divorced and his application to a magnet high school was rejected. If this was what working hard had gotten him, he would show them all. In anger and disappointment, he slacked off on his studies and fell in with a “bad crowd.” The one-time honor student was now getting average grades. Getting in trouble was about all Jae was excelling in. “I had always worked really hard at school, but now I just didn’t care,” says Jae. What he didn’t know was that the real battle of his life lay ahead. And, if he thought he’d already seen how difficult and unfair life could be, he was in for a big surprise.
Jae almost never got sick, and when he did, he usually just pushed through. So, when he started feeling tired and short of breath, he tried to ignore it. Soon, the boy who could play basketball for hours could barely make it up a flight of stairs. Then he developed a fever that wouldn’t go away and noticed bruises developing all over his body, and he didn’t remember injuring himself. He knew he needed to go to the doctor.
Blood tests revealed that Jae was one of the two in a million children stricken with a disease known as severe aplastic anemia (SAA). SAA causes the immune system to attack and destroy blood cells. Production of oxygen-carrying red cells, infection-fighting white cells, and clot-forming platelet cells is cut off explaining all of Jae’s symptoms. Without immediate transfusions, Jai would slip into a coma and die.
Giving Jae blood to replace those cells lost to SAA was only a temporary fix. A bone marrow transplant, in which Jae’s malfunctioning bone marrow (the factory for all blood cells) would be destroyed with drugs and replaced with an infusion of healthy donor marrow, is the standard treatment for SAA, but unfortunately was not an option for Jae. To undergo a bone marrow transplant, a patient must have a bone marrow donor whose immune system matches his. Brothers and sisters have the best odds of being matches. If a donor is not found in the family, patients must look for unrelated donors among a pool of volunteers who have agreed to donate a portion of their bone marrow if, by chance, they happen to have the same key cellular characteristics as the patient. Usually, matches are found among similar ethnicities, and as most of the donors on the national registry of unrelated donors are Caucasian, minority patients have a more difficult time locating matches. Jae already had two strikes against him. He did not have any brothers or sisters, and he was Asian. Jae was sure he was going to die.
Jae’s disease would have many little lessons to teach him. The first would be about his stepfather. A corporate CEO, he took time off from his high-powered job to travel the country and talk to leading SAA experts about treatment options for Jae.
It was an experimental treatment being run by Johns Hopkins Kimmel Cancer Center SAA expert Robert Brodsky that most impressed Jae’s stepfather. The new treatment was actually based on an old one developed 30 years ago by former Hopkins investigator Lyle Sensenbrenner.
In the early 1970s, 10 patients with no other treatment options, were given very high doses of the immune-suppressing drug cyclophosphamide by Sensenbrenner. When following up on these early studies, Brodsky learned that seven of the patients were still alive, and the treatment had cured them of their aplastic anemia. Brodsky returned to his laboratory to figure out how the drug worked against the disease. He learned that high doses were strong enough to wipe out diseased blood and immune cells, but did not affect bone marrow stem cells, immature cells that differentiate or change to form all new blood cells—red cells, white cells, and platelets. The high doses of cyclophosphamide were enough to kill off abnormal cells, giving the stem cells a second chance to repopulate the bone marrow with healthy, normal blood cells. Now, it was time for Brodsky to test his theory on a new set of patients. Jae Jen would be one of them.
Jae entered the hospital on December 6, 2001, his 17th birthday. “It was a present I could of done without,” quips Jae. He was the seventh pediatric patient to receive Brodsky’s experimental treatment. Because he was only 17, Jae was admitted to the Hospital’s Children’s Center oncology unit under the care of Dr. Allen Chen, director of the Pediatric Oncology Bone Marrow and Stem Cell Transplant Program. (Though aplastic anemia is not really a cancer, it is usually treated by oncologists and hematologists.) It was the first time in his life that Jae had been around sick people. He met children of all ages with various forms of cancer. Some got better, others did not, and Jae was uncertain which of those groups he would be in.
Weeks passed following his treatment with high- dose cyclophosphamide, and Jae’s bone marrow was still not making blood cells. Two months passed. Sick from the cyclophosphamide and the aplastic anemia, Jae wondered if he could endure. “Crazy thoughts go through your head. Two months of lying in bed, sitting up only to throw up. You begin to wonder if it’s all worth it. I didn’t know how much more I could stand,” says Jae. While his disease had remain unchanged, his priorities had. “I wanted the simplest of things—a breath of fresh air, to be free of pain, to feel rain on my skin, to see the leaves change color. The time and energy I had spent being angry at my parents over their divorce and myself for not making it into the magnet school now seemed like a tragic waste of time,” Jae reflects. He vowed that if he recovered, he would make the most of every second of his life.
Finally, Jae got his wish. His counts began to recover, climbing slowly but surely until he could finally return to his Northern Virginia home. He had been ill or in the hospital most of his junior year of high school but worked with teachers throughout the summer to catch up on his studies and return to school and graduate with his class. This time he did not waste time on hallway skirmishes. “I came to realize that all people had value. I didn’t need to be in a group or fight with people I didn’t agree with. I wanted to listen to what everyone had to say. I was alive. Just being around people was a privilege,” says Jae.
Jae worked so hard at his studies that he was accepted to Johns Hopkins University. Living up to his vow to live every moment to its fullest, Jae is president of his class, a member of the University’s nationally ranked a-cappella group, and a Hopkins volunteer. When he graduates, Jae hopes to get accepted to Johns Hopkins School of Medicine and become a pediatric oncologist. He says, “I hope one day to help children like my doctors helped me.”