Zachary's Story

Severe Aplastic Anemia Patient Helped by Unrelated Donor

Young Zachary has packed a lot of experience into his twelve short years. Born in Bangkok, Thailand, Zak moved to Colorado at age 4 1/2 when he was adopted into a new family with Mom, Kim, and Dad, Gary, of Monument, a small city near Colorado Springs. He didn't speak any English when he came to the US but his dad remembers Zak was very trusting. After changing climates, continents, and cultures, Zak settled right in to begin the life of a normal American kid.

When he started school, Zak struggled a little. He was diagnosed with learning disabilities and attention deficit-hyperactivity disorder (AD-HD), but he loved the outdoors and active sports, especially soccer, a game his two older sisters Katie and Alyssa also played. It was after a weekend of soccer and Cub Scout camping, the summer Zak turned nine, that the first sign of health trouble showed up.

The family was at dinner in a local restaurant when Zak's nose began to bleed. The family left the restaurant and took Zak home. His nose continued to bleed for over an hour. Their worry mounting, Gary and Kim drove their son to Penrose St. Francis Hospital, where blood tests revealed Zak had a very low level of platelets, the cells that help blood clot. Zak spent the night in the hospital; his doctors ordered a bone marrow biopsy for the following morning. Zak's older sister Katie recalls, "After many tense hours of waiting, a doctor came out and said he had some good news and some bad news. The good news was it was not . . . leukemia. The bad news was they did not know what my brother had and he had also lost 95 percent of his bone marrow to the disease, which for now was labeled 'unknown'."

The biopsy provided a frightening diagnosis: severe aplastic anemia, or SAA. Aplastic anemia is a very rare disease in which the bone marrow stops producing blood cells. Except for a relatively small proportion of cases linked to genetic syndromes, the causes of aplastic anemia are unknown. An individual with SAA has such low levels of red blood cells, white blood cells and platelets that he is at life-threatening risk from anemia, infection, and uncontrolled bleeding.

Because of the danger of uncontrollable bleeding if he received even a slight injury, Zak's parents were told they would have to put tight restrictions on their son's participation in sports and other activities. Zak began a series of transfusions of red cells and platelets, while his doctors and his family worked on a strategy to cure his disease.

Dr. Smith, Zak's hematologist, told the family about their two main treatment options: immunosuppressive therapy using a drug called anti-thymocyte globulin (ATG), which kills the cells in the body that are attacking the bone marrow, and bone marrow transplantation. Dr. Smith wanted to try ATG, the less invasive treatment, but he advised the family to begin searching for a bone marrow donor. He also referred them to the University of Minnesota, a major transplant center, and to Dr. Norma Ramsay, a leading AA researcher and bone marrow transplant pioneer. When two courses of ATG therapy failed to raise his blood counts, it became obvious that Zak would need a transplant.

In a bone marrow transplant, the patient is first treated with intense chemotherapy and radiation to destroy his own malfunctioning bone marrow. At the completion of this grueling preparative regimen, the donor's marrow is infused into the patient's bloodstream in a process no more complicated or traumatic than a blood transfusion. The transplanted marrow travels through the blood into the cavities of the large bones. During the next few weeks, if the transplant is successful, the new marrow will "take root" in the patient's bones and begin producing normal blood cells.

Bone marrow transplants can fail if the patient's body rejects the transplanted marrow. This is called graft rejection. They can also fail if the donor's marrow perceives the patient's body as foreign material and begins attacking it in an immune response. This is called Graft Versus Host disease. For a successful transplant, the genetic makeups of the patient and the donor must be closely matched. A patient with a perfectly matched brother or sister has the best chance but, because Zak is adopted, the family would need to search for an unrelated donor. In an effort to find the best possible match, a nationwide search was conducted in Thailand, Zak's native country.

That search was unsuccessful, but the family was also working with the National Marrow Donor Program (NMDP), a volunteer organization headquartered in Minneapolis with a registry of more than four million potential donors. Through the NMDP, Zak was matched to a young man from New York. The match was not perfect, but good enough for the transplant to go ahead.

Zak received his transplant in September 1998 at University of Minnesota Amplatz Children’s Hospital. Dr. Ramsay was his physician. Gary and Kim were thankful their son was in the care of a medical team so experienced with unrelated donor transplants. Still, the process was hard on Zak and on his family. Gary and Kim had to leave their teenaged daughters in Colorado during the five months Zak was being treated in Minneapolis. Gary, a pilot for American Airlines, was able to arrange his schedule to alternate three days on and off work so he could relieve Kim at the hospital. Zak suffered painful mouth sores from the chemotherapy and radiation and a serious case of Graft Versus Host disease, a result of the incompletely matched donor marrow. In spite of the hardships, however, the family never regretted their decision to go ahead with the transplant. They are grateful to Dr. Ramsay, and to Zak's donor Joseph, for giving them back their son.

Two years have passed since the transplant. Zak is in good health, attending fifth grade, playing soccer, and enjoying the outdoors with his family again. Zak's family has become active supporters of the NMDP and of other families of SAA and bone marrow transplant patients. Zak has been chosen by the NMDP as their Young Marrow Recipient of the Year. And they recently met the family of Zak's marrow donor on the Montel Williams television show. Zak is plunging back into life with the enthusiasm and trust he showed as a small boy newly arrived from Thailand.

Currently, he is conducting a fundraising drive at his school to help the family of a classmate with multiple sclerosis get a motorized lift for their van. In the words of his proud mom, Kim, "Zak noticed that his friend's mom often had a sore back from lifting her son in and out of their van, so he and his friends are working on eliminating the problem."