Lifelines (Spring 2002) - Celebrating Life at the Thalassemia Center

Celebrating Life at the Thalassemia Center
By Angela Woon

Alfred, Kim, Minh and Hay. They are among a group of young people who meet at the same time and place every three weeks. Observing their friendly smiles and hearing their laughter and chatter, one would think they were having a party. In a way, they are. They are celebrating the gift that blood donors continually give them – the gift of life. Meeting place? The Northern California Comprehensive Thalassemia Center in Children’s Hospital, Oakland (CHO).

Minh receiving a blood transfusion.

Thalassemia is a genetic disease that inhibits a patient’s ability to produce hemoglobin, which is the protein that carries oxygen and nutrients in the body. Both parents must possess the thalassemia trait in order for it to be passed on to their children, said Dr. Keith Quirolo, Outpatient Director of the Sickle Cell and Thalassemia Clinics at CHO.

“These thalassemia patients have been transfused their whole lives, most starting from about six months of age,” said Dr. Quirolo. “As a result, we see each other so often that we’ve become a family.”

“I can’t remember how old I was when
I first began transfusions, it was probably
when I was six months old or something.”
-20-year-old Hay

Alfred, a 22 year-old UC Berkeley Biology student, agreed. “It’s like a family gathering,” he said. Alfred, who is originally from Hong Kong, has thalassemia, which means every three weeks he needs to be transfused with two to three units of blood at the center.

Kim, also a thalassemia patient and aspiring biotechnologist, said, “We sometimes have mini parties and even pot lucks during the holidays.” This 17 year-old Bay Area native needs about one to two units of blood per visit.

“Fortunately in this day, every baby is screened at birth for thalassemia in California,” said Dr. Quirolo. “If, however, a baby is somehow not tested and does have the disease, he/she will develop significant anemia by nine months of age and will develop severe symptoms if left untreated. Prior to blood transfusions the life expectancy for a baby with thalassemia was about five years.”

Children who have thalassemia and are left untreated, can experience some or all of the following: severe anemia, jaundice, an enlarged spleen, identifiable facial malformations and growth problems, among other problems. However, those who receive early transfusion regimens and iron removal therapy can avoid these symptoms and lead a normal life.

Hay, a 20-year-old UC Berkeley Biology junior, is one of the fortunate ones who was diagnosed at birth and has been receiving transfusions since he was a baby. Originally from Hong Kong as well, Hay said, “I can’t remember how old I was when I first began transfusions, it was probably when I was six months old or something.” Minh, a Thai-born 17-year-old, who receives about two units of blood each visit, can’t remember when he began transfusions either.

Looking at the patients in the center, one obvious common factor is that they are mostly, if not all, of Asian descent. “The disease is prevalent in the Bay Area because of the migration of South East Asians after the Viet Nam War and recently since the Chinese take-over of Hong Kong,” said Dr. Quirolo. “There are about 2,000 blood-transfused thalassemia patients in the U.S., approximately 2 percent of whom come to Children’s Hospital.” Adult thalassemia patients come to CHO for their transfusions too, as it is the only other Thalassemia center in Northern California apart from UCSF.

People of Asia, Africa and Mediterranean decent are at a higher risk of thalassemia, explained Dr. Quirolo.

Transfusion & Deferral
Because thalassemia patients receive so much blood, there is a risk of receiving too much iron. Desferal is an essential medication that removes iron from the patient’s blood. “Desferal has to be administered at least five times a week for the rest of the patient’s life,” said Dr. Quirolo. “It gets rid of the iron and can be administered with a little pump at home.” According to the Children's Hospital Thalassemia website, individuals only lived to their late teens and early 20’s prior to the introduction of Desferal, due to the overloading of iron.

On a “typical” transfusion day, Alfred, Kim, Minh and Hay start their morning at about 8:30am at the center and remain there till about 2:30pm, depending on the requirements of each individual. The better part of the morning is spent preparing for the transfusion. It takes approximately one and a half hours to transfuse a unit of blood.

The Need
Thalassemia is a life-long disease and blood will always be needed for survival by these patients. “It is important to stress the need for blood,” said Dr. Quirolo. “As individuals, each patient may require one or two units of blood every three weeks or so, but collectively as a whole group, the amount of blood needed is quite considerable.”

Dr. Quirolo urges more Asians to give blood. “The blood our (Asian) patients get is probably donated from Asians themselves, as most Caucasians’ blood do not match with theirs.” One’s blood type is inherited the same way as the color of one’s eye or hair. As a result many blood phenotypes are found only in specific racial or ethnic groups. Most people think about blood as being A, B, O, and either Rh positive or negative. Actually, there are other antigens on blood that can cause transfusion reactions in chronically transfused patients. All of these patients at CHO receive “phenotypically matched” blood products.

All four of Dr. Quirolo’s patients agree that more people should donate blood. “To be honest, I don’t know why people don’t give blood,” said Hay. “After all, their bodies replace it every two months.” Kim meanwhile encourages her classmates to give blood at their annual high school drive. “Most of the time, they are afraid of the needles,” she said. “But I try to encourage them and tell them it’s ok and that it’s a good thing.” Alfred and Minh also echo similar sentiments. “Donate blood,” said Minh. “It’s important to give blood as it saves lives,” said Alfred.

According to BCP’s 1999 statistics, Asian Americans only make up 6.76 percent of our blood donors. This is in stark contrast to the fact that 30.7 percent of the San Francisco population is Asian American. To donate blood, please call 1-888-393-GIVE, or make an appointment online at www.bloodcenters.org.

For more information about thalassemia, visit the Northern California Thalassemia Center’s website at www.thalassemia.org or call 510-428-3885 ext. 4398.