Creve Coeur 8-year-old struggles with severe immune system deficiency
Wednesday, May 23, 2007
Kim Freeman is accustomed to seeing her daughter, Sydnee, in poor health. The only thing worse is the idea of not seeing her at all.
Those are the possibilities for the mother of Sydnee Freeman, an 8-year-old Creve Coeur girl who is afflicted with severe combined immunodeficiency. The rare syndrome prevents the immune system from performing properly.
Sydnee is scheduled to receive a stem-cell transplant tomorrow at Children’s Memorial Hospital in Chicago.
“I’m going around every single day wondering if she’s going to catch something,” said Kim Freeman, 27, a divorced mother of three. “It’s like a time bomb. I always wonder, ‘Is this going to be the last (illness) she gets?’ On the other hand, I don’t know how I’m going to go up there and hand my daughter over to these doctors, knowing I could walk back out of the hospital without her. That’s what took me a year to finally come to the decision.
“She could catch something tomorrow and be gone. She could do the transplant and make it, or not. It’s a game of luck.”
Sydnee’s variation of SCID is deficiency of adenosine deaminase, which results from genetic mutations. Both parents, Kim and Brett Freeman, carry the recessive gene.
One of every 50,000 to 70,000 children born in the United States has SCID, with just 15 percent of those ADA deficient.
Insufficient ADA enzymes lead to low lymphocyte counts, thus preventing the body from fighting off everyday illnesses.
Sydnee receives bovine enzyme injections twice a week. For much of her life, she has ingested four pills a week to counteract a severely underactive immune system.
Medication alone costs thousands of dollars a week, and routine childhood illnesses such as chicken pox can lead to an extended hospital stay. Medical insurance held by Sydnee’s father long ago passed the $1 million lifetime cap, and Kim Freeman – an unemployed student at Illinois Central College – depends on public aid and ongoing fundraising events to try to keep up with mounting expenses. CEFCU locations collect donations for the Sydnee Freeman Benefit Fund.
Some SCID patients wear masks and are home-schooled to limit contact with other children. Freeman opts to send her daughter to Shute School in East Peoria, where she is in first grade.
“I choose to let her do a lot more than she should, because I would rather she enjoy the time she has,” Kim Freeman said. “Eight years ago, (doctors) told me I’d probably have my daughter until she was 3. Now she’s 8. I feel she should live – not be cooped up in the house and isolated.”
School personnel inform Sydnee’s mother of any outbreak of illnesses among students. A recent virus kept her out of classes for nearly two weeks.
“The precautions are up to me,” Kim Freeman said. “Their suggestions are, no Chuck E. Cheese’s, no public pools, no McDonald’s Playlands. They throw out the option of home-schooling. In the end, it’s my choice.
“I know one kid who’s home-schooled and he can’t go to the store or anywhere. But he’s like a baby. He’s so behind.”
Sydnee is behind schedule in her growth – physically, socially and mentally. She has learning disabilities, and times of isolation during illness have created a shyness around groups of kids.
But with maturity, Sydnee has become more aware of her condition – especially after Freeman gave birth to healthy twins nearly two years ago.
“Her quality of life is bad right now, because she’s getting to the age where she’s realizing what’s wrong with her,” Freeman said. “When I had the twins, she asked,
‘Mommy, why don’t they take medicine? Why don’t they get shots?’ At school, one of the mothers told her daughter what’s going on with Sydnee. The little girl went back to school and said ‘Sydnee’s dying.’ How do you fix that? She said, ‘I’m the sickest girl in the whole world.’ ”
Dr. Reggie Duerst, clinical director of the stem-cell transplant program at Children’s Memorial Hospital, aims to change that.
But nothing is guaranteed.
In 1999, Sydnee received a stem-cell transplant taken from the bone marrow of her mother at Duke University Medical Center in Durham, N.C. That transplant failed.
This time, Sydnee is matched with an anonymous marrow donor. In another variation from ’99, doctors administered chemotherapy for 10 days preceding the transplant. Chemo is used to reduce her blood-cell count before the donor’s cells are introduced.
Using a national database, an anonymous donor was found with nine of 10 matching points, or alleals. His blood cells will be introduced into Sydnee’s blood stream intravenously. Ideally, those blood cells will settle in her blood stream and begin forming more healthy blood cells.
“That is essentially giving Sydnee a new immune system,” said Jessica Guarino, a pediatric nurse practitioner at Children’s Memorial.
Stem-cell transplants can have a wide range of outcomes, ranging from successful development of a new immune system, to graft vs. host disease – in which the new immune system attacks Sydney’s cells and creates a host of new problems – to death.
Should a second stem-cell transplant fail, Freeman will consider the experimental, unproven route of gene therapy.
“I’m scared,” Sydnee said before leaving home. “I get needles. They’re going to make me better, so I won’t have to get shots.”
If all goes well, Sydnee and her mother will spend the next three months living in Chicago before moving back home to begin a new, normal life.
“I think she’s handling it great,” Guarino said. “She’s strong. I think she misses home, but she’s positive and she’s funny. Our nurses love her.”
Ryan Ori can be reached at 686-3264 or firstname.lastname@example.org.