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The Honolulu Advertiser

Posted on: Sunday, September 5, 2004

Family's very survival depends on donation

 •  Marrow donor happy to help

By Robbie Dingeman
Advertiser Health Writer

A way to collect life-saving stem cells from donors without surgery may offer hope to three Honolulu brothers — ages 11 to 14 — struggling with a rare and fatal disease.

Lani Nguyen and her children. They are Natalie (left), 8, Tuan (right), 14 and Kevin (bottom), 13. Anthony, 11, is not pictured.

Eugene Tanner • The Honolulu Advertiser

Anthony, Kevin and Tuan Nguyen all have a rare inherited blood disorder called Hyper IgM Syndrome, which only affects boys and men and leaves them unusually vulnerable to infections, said Dr. Randal Wada, medical director for the Hawai'i Bone Marrow Donor Registry.

Kevin, 13, and Tuan, 14, are eligible for a transplant now and Anthony, 11, could benefit later, Wada said.

Wada explains that a transplant of stem cells — donated by adult volunteers through one of two different procedures — can save their lives by removing their defective immune systems and replacing them with marrow or stem cells that work.

While bone marrow transplants are more widely known, another technique called PBSC, for peripheral blood stem cell donation, has been available in Hawai'i for the past six years, Wada said. It allows the donation of life-saving stem cells through an office visit similar to a long blood donation, he said.

BECOME A DONOR AT THESE DRIVES

People who want to help the Nguyen brothers and others who need stem cell or bone marrow transplants can donate at drives sponsored by the Hawai'i Bone Marrow Donor Registry

Donors must be between the ages of 18 and 60 and in generally good health. If you've registered before, you need not register again but you can check to see if all your contact information is up to date.

• Tuesday, 10 a.m. to 2 p.m. The Shops at Dole Cannery, first floor facing Costco parking lot.

• Thursday, 10 a.m. to 2 p.m. at the University of Hawai'i-Manoa's Campus Center second floor.

• Next Sunday, 10 a.m. to 3 p.m. Kekaulike Mall in Chinatown

• Sept. 18-19 as part of Hawai'i Woman Expo ($5 admission) 10 a.m. to 9 p.m. Saturday 10 a.m. to 6 p.m. Sunday

• Sept. 23 5 to 9 p.m. Vietnamese Festival at Kapi'olani Park.

For more information, call the registry at 547-6154.



TWO WAYS TO COLLECT STEM CELLS

Bone marrow transplant

• Overnight stay in hospital.

• Anesthesia required for surgery.

• Procedure requires doctors to remove cells from the pelvic bone through an incision in the back.

• Some soreness or stiffness from where the marrow was removed.

Peripheral blood stem cell

• Donation done in a four-to-five hour visit without hospitalization.

• Donors get shots for four or five consecutive days of a drug that increases stem cells.

• No anesthesia.

• Some donors experience bone and muscle pain, headache or tiredness or a flu-like feeling that begins with the shots.

For more information on the Web, check www.marrow.org.

Although doctors, not donors, determine which donation technique is used, the less-intimidating technique may appeal to donors who shy away from an overnight stay in the hospital. The Nguyen family needs help.

"They're in a race," Wada said. "This whole thing has really swept up this family like a big wave." Because there was no donor match found in their family, the boys need the help of strangers to survive the rare illness.

Originally from Vietnam, Lani Nguyen, 36, moved to Hawai'i 12 years ago to be closer to her father. She has three sons and one daughter, 8-year-old Natalie. The children are bright, loving and hopeful that a transplant can give them a chance for a normal life.

Tuan, a freshman at McKinley High School, likes to break dance, watch action movies and hang out with his friends. In school, he likes Junior ROTC. He's looking forward to his 15th birthday next month, Oct. 2. "My friends call me Tuna," he said.

He has the brash energy of a teenaged boy, bouncing into the room. He and his friends have a dance group that goes by BWS for "Breakers With Style." He'd like to be a doctor. Or sometimes an ice cream man, or maybe a doctor who gives away ice cream on Sundays.

Brother Kevin is more low-key. His favorite subject in school is math. He likes to play computer games and watch TV. He hopes a transplant will give him the chance to play soccer.

The effects of the disease are most obvious for Kevin. He has had an enlarged spleen and suffered liver damage. A seventh-grader at Kawananakoa Middle School, he weighs 57 pounds.

His short-term goal is to get better. Long-term? "I want to be a chef," he says with a big smile.

Their apartment at Kukui Gardens is filled with bicycles, homework and family photos. They bicker like all siblings but show pride in each other and their single mother, helping them cope with the overwhelming disease. "It's a great family," Tuan says with conviction.

All of the boys bruise easily, take longer to fight off colds and have to stay away from most contact sports because of the risk of injury. They visit the doctor at least weekly and take several medications every day.

As the eldest, Tuan is keenly aware of the disease. He hopes the transplant will make them stronger. "I want to learn how to swim. I go to the beach a lot, but I don't know how to swim."

Since learning more about the disease, Lani Nguyen has signed up as a potential donor. She knows what it's like to have a doctor shake his head over the long-term survival chances of a child. "I pray every day," she said.

And she's faced critical times. Like in 1998 in Louisiana, when Kevin was hospitalized for two months. Or when Tuan had a fever for two weeks and had to be taken by ambulance to Kapi'olani Medical Center.

Anthony, 11, also is in seventh grade at Kawananakoa, and wants to join the military when he grows up.

The boys are among about two to three dozen patients in Hawai'i each year who are looking for a transplant. About 3,000 across the country are seeking a match, Wada said.

"Without a stem cell transplant, these boys will be left without a means to overcome the infections that they presently suffer from," he said. "Eventually, the cumulative effects will be too much for them to handle."

Wada said Hawai'i's strong sense of community helps make the state's donor registry especially effective because "so many people feel a part of each other's welfare."

"The Hawai'i registry has one of the best records in the country for volunteers who remain committed to being donors. It's a credit to the people who live in Hawai'i."

Wada said dozens of people's lives have been saved by the thousands who signed up during the Alana Dung drives. Tuan is counting on finding helpers out there.

"We hope we'll find a donor so we're going to make it," Tuan said. Then he stopped and started again, his voice stronger: "We ARE going to make it."

Reach Robbie Dingeman at rdingeman@honoluluadvertiser.com or 535-2429.


Correction: Bone marrow donors must be between the ages of 18 and 60. Information with a previous version of this story was incorrect.