Family spreads awareness about bone marrow disorder that threatens son’s life

Chris Ishida had planned to be in Southern California right now. The 19-year-old freshman should be enrolled in his second semester at Pitzer College, working at his part-time job, and hanging out with his new friends in the Asian Pacific American Coalition on campus. Instead, he’s back home in Chicago’s northern suburbs, fighting for his life.

While spending the winter vacation in his native Illinois, Chris went on Dec. 28 to see a dermatologist about some dots that had appeared on his legs a week earlier and had since spread to his arms, shoulders and face. The dots turned out to be burst capillaries called petechiae, so as part of the examination, the dermatologist took a blood test, promising to provide results the next day. That night, however, Chris started bleeding unabatedly out of his nose, and his mother urged him to visit a hospital. When the staff there noticed his petechiae, he received another blood test, and this time learned the results right away.

They were not good. His supply of platelets — the cell fragments required for blood clotting — equaled 2,000 per microliter of blood. The normal range falls between 150,000 to 450,000; serious bleeding can begin to occur when the number falls below 20,000.

“It was pretty scary,” Chris recalled about learning of his precariously low platelet count.

He ended up staying in the hospital over the New Year’s holiday weekend so that his platelets could be monitored. The staff plugged up his leaking nasal cavity with a gauze-wrapped balloon called a trumpet, which he described as “a real nasty piece of work.”

Initially, Chris’ medical team had trouble figuring out precisely why his platelets were so depleted. The hospital discharged him, but he would eventually spend the rest of January in and out of care facilities after a bone marrow biopsy revealed his condition: severe aplastic anemia.

A potentially fatal disorder, aplastic anemia results when a person’s bone marrow fails in its normal function of producing not only platelets, but also red blood cells and white blood cells. To address this shortage, Chris started receiving transfusions of those first two components only, as transfusing white blood cells tends to be problematic. Although somewhat buoyed by this temporary solution provided about once every five days “just to keep me going,” he remained vulnerable to infection and kept getting sick. Having suffered through fevers, aches and chills, he said, “It’s like the flu that never really stops.” The minor prick of intravenous needles left massive bruises across his arms, and his ability to heal diminished radically. “I’ve lost 12 pounds due to lack of appetite and just pain,” he reported, explaining that “I have sores in my mouth, so it’s hard to eat.”

As he summarized, “It hasn’t been too fun.”

In the meanwhile, his medical team pursued a more permanent treatment. Chris’ best bet would have been a bone marrow transplant from his younger sister Sachi, but hers proved an unsuitable match for his tissue type, information the family learned on Jan. 20. On Jan. 29, Chris embarked upon his next safest option, autoimmune suppression therapy. Experts say this approach presumes that the immune system is mistakenly attacking the bone marrow, and must therefore be subdued to allow the body to rebuild its capacity to generate blood components.

The specific procedure typically entails in-patient administration of an elaborate cocktail of intravenous drugs for four consecutive days, eight to 12 hours per day. The cocktail’s primary ingredient — antithymocyte globulin, or ATG for short — derives from the antibodies of either a horse or a rabbit. Chris received the horse variety.

Having completed his marathon therapy session on Feb. 1, Chris can now expect to wait between three to six months before he knows if it was a success or not. His odds stand in the neighborhood of 70 percent.

Taking nothing for granted, the Ishida family has been preparing for contingency by seeking out potential bone marrow donors, as Chris will require a transplant if the therapy turns out to have been ineffective. Finding a non-familial match, however, looms as a daunting challenge because Chris is multiracial. The people most likely to share his tissue type are those who also share his Japanese and Caucasian genetic background, a criteria that drastically narrows the donor field.

Despite this obstacle, the Ishidas have pressed forward. Unfortunately, they have lots of practice managing health crises — Chris’ mother Susan has been waging her own battle against melanoma, the most dangerous form of skin cancer, for more than five years. She’s had tumors in her brain, liver and spleen, and at the outset of 2008, she was told she had between six to 12 months left to live.

Not only has she soldiered on, but in between aggressive treatments and clinical trials, she found time to form a nonprofit to promote melanoma awareness and research. Launched nearly a year ago under the name Skin of Steel (Susan’s maiden name is Steel), the organization is a family affair; teenage Sachi sits alongside her mother and many other seasoned professionals on its board of directors, while husband Masuo serves on its board of advisors.

The Ishidas hope to take the strategies they developed during Susan’s ordeal and apply them not just to Chris’ plight, but to the plight of others as well. His mother said, “We know from our experience with melanoma that we can use our expertise in reaching out to specific niches in the population to affect change. And we knew that there’s a possibility that we could find a match for Chris, but there’s an even stronger possibility that we can change minority registration for the bone marrow registry.” Such change would be welcome, given that compared to Caucasians, ethnic minorities have half the likelihood of finding a match through the service Susan mentioned — the Be The Match Registry, a national listing of millions of prospective donors.

In attempting to locate a donor for Chris, the Ishidas have emphasized the broader importance of people joining the Be The Match Registry. Masuo pushed this idea when he recently spoke about his son to the congregation of Chicago’s Midwest Buddhist Temple. Congregation member Mary Doi described the presentation as “very poignant,” and expressed frustration that the larger message hasn’t gained enough traction in the Japanese American community.

“I think it’s a hard sell,” she said, speculating that misconceptions or apathy have held people back from signing up for the registry. “So much education needs to happen.”

Perhaps more Nikkei would feel a greater sense of urgency to register if they knew that Asians have a higher incidence of bone marrow failure — four times as much as most other ethnicities, according to Neil Horikoshi, chairman of the board of directors for the Aplastic Anemia & MDS International Foundation (AA&MDSIF). A survivor of aplastic anemia himself, Horikoshi pointed to the prevalence of Asian ancestry among his fellow board members — all of whom have been touched in some way by bone marrow disease.

The organization that they pour their passion into has been around for a quarter century, existing to support patients and families living with bone marrow failure, be it aplastic anemia, paroxysmal nocturnal hemoglobinuria (PNH), or one of the myelodysplastic syndromes (MDS). In recent years, AA&MDSIF has helped allocate roughly $10 million dollars from Congress for research on this category of disease. Horikoshi hopes such effort will result in significant medical progress.

“The goal is to find out what is the source — what triggers the bone marrow failure in all of us, and why do Asians have a higher propensity?” he said. “We believe there’s an answer.”

In a parallel initiative to find that answer, AA&MDSIF has also been advocating for the passage of the Acquired Bone Marrow Failure Diseases Research and Treatment Act (H.R. 1230). This resolution was introduced nearly two years ago by Rep. Doris Matsui (Sacramento), whose late husband Robert spent 26 years in the Congressional seat she now occupies before succumbing to MDS-triggered pneumonia on Jan. 1, 2005 at age 63. The Congresswoman’s Website states that in addition to backing increased study of bone marrow failure, “H.R. 1230 would also authorize coordinated outreach and informational programs targeted to minority populations affected by these diseases.”

The House of Representatives approved the resolution last September, but the Senate never got to it before the end of the year. In a statement issued to the Nichi Bei Weekly by her office, Matsui said, “Due to the bipartisan way in which my legislation passed the House last fall, I am optimistic that when I reintroduce the measure this Congress, it will pass the House and be taken up by the Senate in a timely manner.” She furthered, “My bill will enhance treatment options for those that suffer from bone marrow diseases, and provide a focused approach to research that could improve detection and save lives.”

While waiting for scientific inquiry and political leverage to deliver this brighter future, those coping with aplastic anemia and its cousin disorders must keep their heads up.

“Part of this is the will and desire to be cured and to get well,” Horikoshi attested, directing encouragement to Chris: “Tell him to stay positive, because I survived, and for me it’s been 11 years already.”

For his part, Chris has remained optimistic. “I got to believe that I’m going to get better,” he said. “I got to believe that I’m going to make it through, because there’s just too much for me yet to do and experience.”

There will be a bone marrow registration drive in Claremont, Calif. on Friday, Feb. 18, from 9 a.m. to 3 p.m. in the Green Room at Harvey Mudd College, a member of the Claremont University Consortium along with Pitzer College. Although it is being held near his school, the drive is not for Chris specifically; his family does hope that awareness of his situation on campus will give a boost to the event, which is in its 12th year. Appointments can be made by e-mailing morrison@THUBAN.AC.HMC.edu or calling (909) 621-8024.

To track Chris’ progress, visit www.caringbridge.org/visit/chrisishida.

For more information about the Be The Match Registry, visit www.marrow.org or call (800) 627-7692. Prospective Asian American donors are encouraged to contact the Asian American Donor Program, one of the registry’s official recruitment groups for Asian, Pacific Islander and multiracial communities, at (510) 568-3700 or through www.aadp.org.

Joining the registry entails filling out a registration form, signing an agreement and providing a swab of cheek cells or a blood sample. Donors must meet certain health guidelines and be between the ages of 18 and 60.

For more information about aplastic anemia, visit the National Heart, Lung and Blood Institute’s Website at www.nhlbi.nih.gov/health/dci/index.html. For more information on the Aplastic Anemia & MDS International Foundation, visit www.aamds.org/aplastic or call (800) 747-2820.

To track H.R. 1230, visit Rep. Doris Matsui’s Website at www.matsui.house.gov or call her office at (202) 225-7163.

For more information about Skin of Steel, visit http://skinofsteel.org.

Much of the medical information contained in this article was sourced from the above Websites.