Fighting immune deficiency, Josie Fesperman and her family seek cure

Published 12:00 am Saturday, August 20, 2011

By Katie Scarvey When you see little Josie Fesperman bopping around her house, snapping pictures of big brother Warren on her toy camera, playing with the family dog, Ace, nothing hints to the major health problem she’s facing. Any worries she has are certainly not etched on her grinning face.
But at 21/2, Josie — the daughter of Alan and Missy Fesperman of Salisbury — has already spent more time in hospitals than most people will in a lifetime.
Concerns began to surface when she was about six weeks old — not coincidentally, the time that protection from a mother’s antibodies wears off.
Josie began having a string of fevers, ear infections and viruses.
“She’d get better, then she’d get sick again,” Alan said. “We just had a bad feeling about it.”
When she was around three months old, she developed a fever of more than 101 and kept it for 16 straight days — troubling, to be sure.
She didn’t want to eat, and she wasn’t gaining weight.
Finally, Josie was referred to Presbyterian Hospital, weighing only 8 pounds, slightly less than when she was born.
Testing determined that Josie could not produce her own antibodies. The life-threatening condition she has is called agammaglobulinemia. In the hospital for about three weeks, Josie was given a feeding tube for nourishment since she would not eat on her own.
Her fever finally subsided and doctors allowed her to go home, but she would have to return for weekly visits to check her IgG (immunoglobulin G) levels.
Every three or four weeks she would receive intravenous immunoglobulins, which are made from donated human plasma. These would help her, temporarily, be able to fight off disease.
Even so, Alan and Missy realized they would have to keep Josie isolated from excessive human contact, which could cause her to become ill. That meant no church, pre-school or parties.
“She went to one family gathering at Christmas and got sick,” Missy says.
They wanted Warren to be able to continue leading a normal life, so one parent began staying with Josie while the other parent took Warren places. Missy’s mother helped out a lot, coming in three or four days a week to stay with Josie.
When she was nine months old, Josie came down with a severe case of pneumonia and RSV, a respiratory virus. That led to a grueling nine-week stay in the hospital. Her pneumonia was complicated when she aspirated on her feeding tube and had to be on a ventilator for 11 days.
“It was a pretty tough two weeks,” Alan said.
Last June, Josie’s parents began giving her weekly plasma injections at home. She had also been diagnosed with neutropenia, another type of immune deficiency.
Things began to look up in 2010. With the help of the injections, Josie didn’t have any hospital stays longer than three days. She began to gain weight, and developmentally, she caught up to where she was supposed to be.
Then, in January of this year, she got sick again— PCP pneumonia, the sort that end-stage AIDS patients often get because of their severe immune deficiencies. That kept her in the hospital for more than a month.
“If you have normally functioning T-cells (a type of white blood cell important to immune function) you would not get PCP pneumonia,” Alan explains.
But it was worse than that. An MRI showed that an infection had attacked the basal ganglia area of her brain, which controls motor skills. Josie went from being a normal 2-year-old, walking and talking, to not being able to sit up without falling over.
That infection is finally almost gone, and Josie today looks and acts like a normal 21/2 year old.
But of course with her severe immune deficiencies, she’s not. The Fespermans realized that if they did nothing, they would always be in the position of wondering when Josie would get sick again and land in the hospital. It’s a frightening way to live, just waiting for the next bad thing to happen.
As if these challenges weren’t enough for the Fespermans, Missy has also been dealing with a debilitating condition.
Hers began years ago when she was very young and living in Ohio. Prone to severe bronchitis, she would wind up in the hospital every winter, she says. In middle school, she moved to Rowan County and attended East Rowan High School, where she met her future husband. At 15, she began having back problems, headaches and dizziness and would go through spells that resembled seizures. The spells would leave her walking like a baby for a while, she says.
Her father took her to the Cleveland Clinic in Ohio, where doctors discovered a spinal tumor. Surgeons removed it, along with a few vertebrae, but it grew back. A second surgery at New York University Medical Center with a world-renowned surgeon when she was 18 got things under control, and Missy went on to college.
For many years, she was fine, she says, but about a year ago she began having “little spells,” which prompted more testing. She was having hemiplegic migraines, a serious neurological disorder that would give her sharp head pains, dizziness, and most troubling, a stroke-like paralysis on one side of the body that would temporarily take away her ability to walk. She began having them so frequently that she finally bought a wheelchair and a walker.
Her condition has improved, she says, but she still has about five episodes a month. Her disability has meant that she can no longer work at her job as a speech therapist with the school system.
It’s been difficult on Warren and Josie, to see their mother incapacitated at times and for her not to be able to hold them.
“It’s been a strange year at our house,” Alan says.
But the Fespermans are looking at some good news in the form of a bone marrow transplant for Josie.
Although bone marrow transplants are a fairly new treatment for conditions like Josie’s, they have been used quite successfully. Soon, Josie will be traveling to Cincinnati Children’s Hospital for the procedure.
The donor?
Her big brother. Although 5-year-old Warren doesn’t know all the details of the surgery, he has been told that he can “fix” Josie’s troubles by giving her some of his “soldiers.”
The healthy marrow from Warren will blend with Josie’s marrow and odds are, she will begin to produce on her own the cells she needs to protect her body from disease.
Although donating marrow will be a fairly easy procedure for Warren, Josie will have to remain in the hospital for perhaps three to four months to be monitored.
Missy will stay with Josie the whole time she’s there; Alan will stay for about a month before returning to his job as a pilot for Food Lion and then traveling to Cincinnati to visit when he can. After Warren finishes his part as a marrow donor, he will return to stay with Alan’s sister in Charlotte until Josie and Missy return from Cincinnati.
The date for the transplant hasn’t been set yet, but the Fespermans have a preliminary meeting with doctors this Wednesday in Cincinnati, and they feel very hopeful that Josie has a good chance of being cured and leading a normal life. Alleviating the stress associated with Josie’s condition might also help Missy with hers.
Now, it’s up to the soldiers.