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Recovery from a rare disease, thanks to blood donors

July 06, 2017 | Lindsay Carrillo
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Vivian Nguyen during her 56-day hospitalization. The blood of dozens of donors saved her from a rare disease.

Vivian Nguyen had been feeling lousy for three weeks. At first, she figured she had a cold, but it just wouldn’t go away. On the advice of her aunt, she decided to get checked out at UC Irvine Medical Center, where her aunt – a pharmacist – works.

She was discharged from the hospital nearly two months later, after a team of UC Irvine Health physicians worked together to diagnose and treat what turned out to be a rare disease, atypical hemolytic uremic syndrome (aHUS). In Nguyen’s case, it was triggered by lupus nephritis, which she did not know she had.

“I never had any health problems,” Nguyen said. “I would get sick easily but always got better. I was never so sick that I had to go to the hospital.”

Owing recovery to strangers

Nguyen owes her recovery, in large part, to people she never met – people who donated blood at the UC Irvine Health Blood Donor Center, which provided plasma and red blood cells that were essential in her treatment.

At the time of her diagnosis in 2015, Nguyen was 25. She had been employed full time as a behavioral technician, working with children with learning disabilities, autism or ADHD. She was also attending college full-time, pursuing a master’s degree in biochemistry. Juggling work and school had been a challenge for Nguyen, and she considers stress a major factor in her illness.

A rare diagnosis

“We were faced with a young person with recalcitrant multiorgan disease, and the team was very concerned for her,” said Dr. Minh-Ha Tran, UC Irvine Health director of transfusion medicine services who, in conjunction with colleagues in hematology, diagnosed Nguyen with aHUS. This rare disease is characterized by immune activation – in Nguyen’s case, by lupus activity – characterized by kidney failure, low platelets, and a unique form of anemia (low blood count). Under the microscope, some of her red blood cells had a “broken” appearance. 

In addition to receiving standard treatments for lupus and lupus nephritis, Nguyen underwent plasma exchange treatments. Plasma, the non-cellular portion of human blood, comes from blood donations. The treatments however, were only partially effective as her red cell transfusion needs persisted.    

vivian nguyen and dr. minh ha tran
Vivian Nguyen and Dr. Minh-Ha Tran.

Impact of blood donation

The impact of blood donation can be far-reaching, said Tran, who oversees the UC Irvine Health Blood Bank, the UC Irvine Therapeutic Apheresis Service, and the UC Irvine Health Blood Donor Program, which collects more than 16,000 blood products per year.

Blood products collected by the blood donor program are used in the treatment of many UC Irvine Health patients including trauma injury victims, cancer patients, newborns in the neonatal intensive care unit and their mothers, and other patients facing a variety of diseases.

Blood bank on site

The blood bank is crucial because critically ill and injured patients can need blood at a moment’s notice. UC Irvine Medical Center is the region’s only Level I trauma center, annually treating more than half of Orange County’s trauma victims. The UC Irvine Health Chao Family Comprehensive Cancer Center is Orange County’s only National Cancer Institute-designated comprehensive cancer center.

Treatment of aHUS presents its own challenges. As a rare disease, annually affecting only one to two people per million, it has not been the subject of any large-scale, multicenter trials to determine the best course, Tran said.

Life after recovery

Tran advocated for the use of eculizumab, approved in 2011 as an orphan drug to treat patients with aHUS.  The rarity of aHUS made Nguyen one of the first patients at UC Irvine Health to be treated with eculizumab, which produced immediate effects allowing Nguyen’s hematologic picture to quickly improve.  She was discharged a week later, after spending 56 days in the hospital.

Nguyen said she took some time off from work and school to evaluate her life. She had always loved working with kids and parents to teach them behavioral skills, and decided to make that her long-term career. She is now enrolled in a master’s program at Arizona State University in applied behavioral analysis, and she is scheduled to graduate in March 2018. After that, she plans to get her PhD.

Nguyen’s case was so compelling that the team involved in her care wrote it up as a case report; it was published in Hematology Reports in September 2016. Tran said the team hoped that their experience would raise awareness among other clinicians who might be faced with treating this rare disorder. Her case was also notable as it was her lupus activity that appeared to be the primary driver for Nguyen’s aHUS. 

'Important need for blood donation'

During her stay, Nguyen’s transfusion requirements amounted to 12 units of blood, two units of platelets, and more than 200 units of plasma. UC Irvine Health blood donors provided 97 percent of the transfusion products Nguyen received.

“Stories like Vivian’s really help to connect blood donors to the impact of what they are doing,” Tran said. “There’s a very important need for blood donation.”

Donate blood and get Angels tickets

UC Irvine Health is teaming up with the Los Angeles Angels to provide two free baseball tickets to those who donate blood at one of our upcoming blood drives. Make an appointment ›

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