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Aplastic Anemia News - Return to News Menu
Q-C boy has rare blood disease
By Rachelle Treiber
ELDRIDGE, Iowa — April 6th, 2005 - Paul and Mollie Strief were not sure what to think when they noticed a number of bruises and little red spots covering their 2-year-old son.
Until that day in February, the couple had a healthy child.
Friends and family say Deven Strief always has been happy-go-lucky. And to see him today, smiling and playing with his favorite toys at the Children’s Hospital of Wisconsin in Milwaukee, Mollie Strief says it is still difficult to believe her little boy is fighting for his life.
“He’s been diagnosed with very severe aplastic anemia, a serious and rare blood disorder,” she said. “But he’s very happy and playful. So long as he feels good, he is excellent.”
In a telephone interview from Milwaukee, where she has spent every day with her son since he was flown there for treatment March 6, she said Deven’s life-threatening condition affects less than 1,000 people in the United States each year.
According to officials with the National Marrow Donor Program, or NMDP (www.marrow.org), aplastic anemia is not a single disease, but a group of closely related disorders characterized by the failure of the bone marrow to produce all three types of blood cells: red, white and platelets.
The exact cause of aplastic anemia is unknown, although it has been linked to exposure to chemicals and radiation. It also is believed that some cases are inherited and that some are due to a viral infection.
Aplastic anemia means the bone marrow is failing and not making any blood cells at all, said Dr. Gregory Garvin, a Genesis Health Group pediatrician in Davenport.
“There are different types of aplastic anemia and they are all serious. This is not a good disease. It’s higher than a 50 percent mortality rate over six months if it is untreated,” he said. “Bone marrow transplants are the main treatment, and they are much more likely to be successful if there is a sibling match. It’s much harder to find a match who is not a sibling.”
If the marrow is taken from a matched sibling, transplants are between 80 percent and 90 percent effective. With a matched, unrelated donor, there is a 70 percent survival rate after one year. Deven is an only child, although Strief is due to have a second child in July. She and her husband have been tested and are not a good bone marrow match for their son.
So far, she said, he has had at least 12 transfusions to make up for the lack of blood production.
She said her son has little ability to fight infections and is undergoing immunosuppressive therapy called ATG, which is sometimes successful at inducing remission of the disease. If the therapy fails, his only chance would be a bone marrow or blood-cell transplant.
While Deven’s doctors are continually checking the national donor registry, as well as the umbilical cord blood bank (another transplant option for those with aplastic anemia, although not as common), a match has yet to be found.
Because locating a match can take a lot of time, the Striefs and their friends and family have planned a blood marrow donor drive in Deven’s name for 3-7 p.m. Thursday at the Mississippi Valley Regional Blood Center, or MVRBC, in Davenport. Officials at the blood center, said volunteer donors will sign a consent form and give a small blood sample to have their bone marrow type registered.
The MVRBC, 5500 Lakeview Park, just north of 53rd Street, is a host site for the drive, which is being conducted by officials with University Hospitals in Iowa City.
A marrow or blood-cell transplant cannot take place unless a matched donor can be located. About one- third of patients can find a compatible family member, typically a sibling. Patients without a matching related donor can search for an unrelated donor among the more than 5 million volunteers registered with the NMDP. Donors have about a 2 percent chance of matching someone on the registry, according to its Web site.
Strief said her second child has a 25 percent chance of being a match for Deven. And although doctors ideally would like to wait until after they check the baby’s type, Deven may need the transplant sooner rather than later.
“They’d have to wait until the baby is 11 months old to do a transplant. And because Deven has very severe aplastic anemia and has little ability to fight infection, if he gets an infection, he could need an emergency transplant,” she said. “And that’s why we’re doing the marrow drive.”
Strief said so many people have expressed a desire to be tested that they thought the drive was the best option. And although she is hoping and praying for a match for her son, she said people should donate for the benefit of their relatives as well.
“That way, if something happens to your family member, you will already be in the system,” she said.
Strief also encourages minorities to get registered because the registry is 75 percent caucasian.
“The most likely match for caucasians is other caucasians and so on. I’ve talked to families of other ethnicities who have even worse odds of finding a match,” she said. “They really need more minorities in the registry.”
Strief said doctors also plan to keep her umbilical cord blood when she gives birth because, even if Deven’s drug therapy works and he goes into remission, he could need it at some future date.
Over the past month, Strief said she and Deven have gone back and forth between the hospital and a family suite at the nearby Ronald McDonald House in Milwaukee.
Whenever Deven does not have a fever, he is able to leave the hospital for a while.
But he cannot be around other children due to the possibility of infection, so his family is keeping him entertained in other ways.
“He misses his friends and his baby sitter so much,” Strief said. “And even at the Ronald McDonald House, they have an isolation area, so he cannot see other children at all. But our friends and relatives and the hospital staff are giving him a lot of attention to make up for it.”
She is particularly grateful for the recent spate of warm weather, which has allowed her to take her son to the park — away from the hospital and the treatment he has been facing so bravely.
Strief said Deven seems to know much more about his illness than she would have expected at his age.
“When we leave the hospital and he’s not on antibiotics, he gets fevers. He knows when he starts to get a fever and will have to go back to the hospital,” she said. “He picks up the phone and says, ‘Margolis,’ which is the name of his doctor.”
The family is trying to remain optimistic.
“We have one of the best doctors in the world for this disease, Dr. David Margolis, and we’re at a great hospital,” she said. “Deven’s outlook is good, and the support from everyone has been great.”
Rachelle Treiber can be contacted at (563) 383-2363 or rtreiber@qctimes.com.
TO HELP OUT
The Deven Strief Marrow Donor Drive will be 3-7 p.m. Thursday at Mississippi Valley Regional Blood Center, 5500 Lakeview Parkway in Davenport. People age 18 to 60 can sign a consent form and give a small blood donation to have their marrow type registered with the National Marrow Donor Program.
Visit www.devenstrief.com for more information on Deven’s progress and how to be a donor.
A Scrapbooking Benefit is planned for May 14 at North Scott High School, Eldridge; contact Penny Beem at (563) 285-2356.
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