WEST PEORIA, Illinois — At 16, Maddison Harmon knows that normalcy isn't easily defined.
Diagnosed with a rare disease at age 2, she's spent her adolescence dealing with the neurodegenerative effects of Langerhans cell histiocytosis and an accompanying 10 months of chemotherapy. The long-term effects of the disease include hearing loss and poor coordination.
She wore a hearing aid, but stopped because of teasing, and struggles with basic motor skills such as catching a ball.
"I thought that was just the normal side of things," she said. "I just tried not to think about it."
Her mother, Melissa Ruhl, said those side effects got progressively worse — Maddison couldn't ride a bike or pass a driving simulator to get her license — until a failed attempt at a handstand landed them in the emergency room with a concussion and discomforting news: The symptoms weren't just caused by the treatments she had as a baby.
Maddison had neurodegenerative central nervous system LCH active in her cerebellum, a diagnosis Ruhl said was disappointing, though not altogether surprising.
"As much as I hate being the mom that's critical of my child, I always knew there had to be something we could do to help her," Ruhl said. "If we do nothing ... five to 10 years. Her life would be of no function. So we say look at that bright, beautiful face. How could you give up on her?"
After transferring from Farmington to Limestone Community High School when she moved with her mother and sister to West Peoria to be closer to the hospital, Maddison found making friends difficult, and missing school for cancer treatments didn't help.
"At the time, I wanted to keep it quiet. I didn't want to be treated differently," Maddison said. "So I would tell them I got dehydrated and had to go to the hospital, which wasn't technically lying that much because I did go to the hospital and I did get dehydrated."
In the fall she would miss weeks at a time for in-patient chemotherapy treatments that wiped out her energy and immune system. She brushed off questions from inquisitive peers.
During the spring semester, Maddison was on a new drug, one that decimated her immune system, so she finished the 10th grade watching geometry lessons her teacher posted on YouTube and sending homework with her mom to drop off at the school.
Maddison took several different chemotherapy drugs through treatments at St. Jude Midwest Affiliate, some with harsh side effects, but doctors weren't seeing the progress they'd hoped for.
"There's a group of patients that tend to have more difficult and recurring disease. Those are the patients that we're still struggling to find the best solutions for. I don't think we have the best answer yet," said Dr. Mohamad Al-Rahawan, the pediatric hematologist oncologist who has treated Maddison in Peoria.
LCH isn't considered a true cancer, he said, though in some ways the disease behaves like one, and it's often treated with chemotherapy by oncologists.
The type of neurodegenerative LCH Maddison has is especially rare. While St. Jude treats patients with Langerhans cell histiocytosis, the organization doesn't have an open trial for neurodegenerative LCH, and Maddison isn't eligible for a multi-institutional trial for patients with recurrent LCH and a certain genetic mutation.
"If (patients) had disease that affected their skull in the beginning and were treated, many times successfully, a few, a very rare portion of them, come back with neurodegenerative effects," Al-Rahawan said. "I think there are one or two experts in the country that would claim knowledge on how to treat it."
One such expert is Dr. Kenneth McClain, director of the histiocytosis program at Texas Children's Cancer Center, which has a new course of treatment.
While the treatment at St. Jude is free and the family is insured, travel expenses have started to mount. With four trips to Houston before the end of summer, the family is bracing for increased expenses.
The 1,000-mile trek can be completed with three short flights from volunteer pilots with Angel Flight, LifeLine and compassion flights. But, as the family learned earlier this month, the system has gaps.
Two days before their scheduled departure to begin Maddison's treatments, one of the donated flights was canceled because of an impending storm.
Panicked, the family started planning a two-day road trip to keep the appointment before receiving an unexpected gift of airfare and hotel accommodations from the Dax Locke Foundation.
With the first week of treatment complete and just more than two weeks before the next trip, the family is encouraged by Maddison's reaction to the drug, especially compared to the harsh chemotherapies. But it is too soon to tell whether the treatment will slow or stop the disease's progress.
"What she's experiencing herself and outward function, this is better than anything we've ever done," Ruhl said. "Unfortunately, in the long term, we'll have to wait and see what happens."
Source: (Peoria) Journal Star, http://bit.ly/1iGLi8e