Every six weeks, Koryn Bakken comes to the Winneshiek Medical Center and receives the drip that protects her from forever-present germs.
The little angel that could
Five-year old Koryn Bakken settles on her mother’s lap dragging along a cozy fleece blanket covered in colorful ladybugs, and tucks her red hair behind her ears. Cinderella’s silhouette smiles on Koryn’s lavender shirt, and she hands her mother a book: Dora the Explorer, a tale of a princess with a flowing dress and sparkling tiara.
Koryn is like other five-year old girls: she wishes she was a princess (though her parents tell her she already is), loves tumbling, the color pink and snow angels. Yet something separates Koryn from most other little girls: two faded scars – one on her lower neck, the other on her upper left chest – marks of a past filled with hospital stays, blood transfusions and endless doctor visits.
Koryn was born a healthy girl on August 7, 2003 at Winneshiek Medical Center, and under the watchful eyes of first-time parents, Karla and Carlton, the usual baby activities filled her first two months of life. But one evening, amid new smiles and Koryn’s wonderful discovery of her ability to suck on her toes, Koryn’s temperature began to rise. It spiked to 103°, and Koryn parents rushed her to the emergency room. Once under emergency care, the medical staff stabilized Koryn’s fever and the physician ordered blood samples, which came back abnormal; an ambulance transported Koryn to La Crosse.
“Koryn was in La Crosse for two weeks but they could not determine why her blood levels were abnormal. We turned to Iowa City,” says Karla. On the first day at the University of Iowa Hospital and Clinics in Iowa City, the specialists gave Koryn her diagnosis: HLH (Hemophagocytic Lymphohistiocytosis). The Histiocytosis Association of America explains that HLH is a rare, possibly life-threatening, genetic blood disease caused by too many white blood cells.
And thus began Koryn’s journey through chemotherapy, blood transfusions, a stem cell transplant and countless medications and the ups and downs and highs and lows that were her illness. “My world crashed down around me,” says Karla. “Our baby could die without a transplant, and the only thing I could do for her was hold her – pray for her and pray that we would make the right choices for her to survive.”
Histiocytosis is similar to cancer because oncologists can treat it with chemotherapy and radiation. The disease affects approximately one in 200,000 children born each year in the United States, and since this illness is so rare there is little research into its cause and treatment.
“Koryn was the second child ever diagnosed with HLH at the University of Iowa Hospital and Clinics; they figured out how to treat her as they went,” says Karla.
Through a central line catheter, Koryn received chemotherapy to rid her body of all white blood cells, medication to stabilize her blood pressure, morphine to control her pain. Her little body swelled with retained fluid from steroids, giving her the appearance of a healthy, chubby baby, not the sick child she really was. “And through it all, she smiled,” says Karla. “But not at the doctors, only the nurses. As soon as her doctors came in the room, she became very serious, but would then start babbling as soon as they left.”
Koryn received an umbilical cord stem cell transplant to rid her body of HLH, a process that, on the outside, appeared to be a normal blood transfusion. The hope was that the new cells would find their place in Koryn and begin making healthy white blood cells. Karla says, “Her father held her through the transplant. That was day zero. From there, we went forward; held on to the hope that the transplant would work – that our little girl would be cured of this awful disease.”
Koryn and Karla lived in Iowa City for three months, joined by Carlton whenever possible. The family moved to Iowa City’s Ronald MacDonald House during the last few weeks of care. “She was slowly progressing, her white count would go up, then back down, then her hemoglobin would rise, and fall again,” says Karla. The family knew inconsistencies were to be expected, and the doctors altered her medications as the days went on. “She baffled them,” says Karla. “Koryn did not follow the known progression of the disease. When medication was supposed to make her uncomfortable, she tolerated it. She was just this little body – that took so much. She was so strong; she always smiled.”
But then, after an approved weekend out of the Mac House, Koryn seemed weak and her skin took on a yellowish tinge. Karla says, “I thought her bilirubin was just too high, but the doctors seemed to think it was more serious – they ran tests at our appointment the next day.” Koryn’s specialists determined her antibodies were attacking the donor’s red blood cells, a condition called Autoimmune Hemolytic Anemia: Koryn became a patient in the Pediatric Intensive Care Unit for the first time. “The doctors said that this was a rare side effect of her transplant. Why not? She had a rare disease, so why not a rare side effect?” says Karla.
The anemia resulting from her transplant required the use of immunoglobulin, a substance that binds up antibodies that are damaging the red blood cells and rids them from the body, and as Koryn recovered from the anemia – first in Iowa City, then back in Decorah – she needed continued infusions of immunoglobulin to keep her healthy. “Without the immunoglobulin, Koryn’s immune system is not up to par – she is very susceptible to all illnesses,” says Karla.
Weekly check-ups in Iowa City turned into every other week, then monthly visits. An infection in her central line catheter led to its removal. Karla says, “When we were going to Iowa City only for Koryn’s infusion, I began to wonder why the infusion couldn’t be done at Winneshiek Medical Center in Decorah.” Karla worked with the specialists in Iowa City and Koryn’s primary care physician, Janet Ryan, M.D., of Gundersen Lutheran – Decorah Clinic, to arrange the administration of her daughter’s infusions in the Same Day Services department at the medical center.
Koryn now receives treatments here at home, in Decorah. Every six weeks, Koryn comes to the medical center, chooses toys off the pediatric toy cart, races around the department on a rolling chair (pushed by playful nurses) and receives the drip that protects her from forever-present germs. Infusion treatments are available for patients who struggle with chronic illness, such as cancer – or in Koryn’s case, the anemia resulting from HLH – at the Winneshiek Medical Center Same Day Services Department. Deb Tekippe, Assistant Nurse Manager of Same Day Services/Surgery (and now, close friend of Koryn) says, “Patients come to us with a variety of illnesses and we take referrals from many clinics. This service makes chronic illness treatments more bearable for patients because they don’t have to travel when they may not be feeling well – we are close to home.”
Karla says, “I don’t know how long Koryn will have to receive immunoglobulin to counteract her anemia – it may be for the rest of her life – but this March, she will celebrate her five-year anniversary since the transplant. Koryn will be cured of HLH.”