Energetic Chiari Patient Inspires Sculptor's Work

Toddler's Image Displayed in Comer Children's Hospital

January 2007

To an acquaintance, Riley Johnson appears to be a healthy, energetic, two-and-a-half-year-old boy. His nickname is Smiley Riley. And like many his age, he runs as soon as he's out of his stroller.

"Riley never stops," his mother Rachel Johnson said with a laugh. "We believe that's why he's so thin. He takes off. It's like Forrest Gump."

Riley is so active that in order to create a sculpture of the toddler, artist Jeffrey Breslow had to first take a series of photographs and work from the still images. But Riley's activity level is deceiving--sometimes making it difficult to believe that he has Chiari malformation and pseudotumor cerebri.

He runs because it's easier than walking, Rachel Johnson explained. "He only runs. He doesn't walk because he can't control himself."

Chiari malformation is a structural abnormality in the cerebellum. The cerebellum extends from the skull, places pressure on both the brain and spine, and impedes the flow of cerebrospinal fluid. Pseudotumor cerebri, high intracranial pressure, sometimes accompanies Chiari.

Riley has already undergone five surgeries to relieve the pressure, which causes excruciating headaches. His mom likened his condition to having a 10-pound brain in a skull that houses a nine-pound brain. Possible Chiari symptoms include headaches caused by coughing or laughing, muscle weakness, eye dysfunctions, numbness, difficulty swallowing, difficulty articulating words, and loss of consciousness because of lack of blood flow to the brain--symptoms that disrupt daily life.

"We're a three-generational Chiari family," she said. After Riley's diagnosis, his five-year-old brother Alex also was diagnosed with Chiari. Later, their mom and grandmother learned the cause of their life-long headaches and other health issues that had gone unrecognized as Chiari symptoms for a long time. Upon review of previous MRI films, doctors told Rachel Johnson and her mother that they--like many Chiari patients--had been misdiagnosed for years.

The Johnson's first noticed something awry when Riley was two months old. He couldn't move his head, which eventually led to plagiocephaly--flattening of the skull. With time, physical therapy and a band helmet helped strengthen Riley's muscles and reshape his head.

All seemed on the right path until Riley became more mobile. When he was 16 months old, they began to notice he crossed his eyes and had difficulty walking. After a series of doctor's appointments and an MRI, Riley was diagnosed with Chiari malformation. As Riley grew, the symptoms worsened.

"He would scream for hours, reaching for the back of his head, and his eyes were almost always crossed," said Riley's father, Kurt Johnson. "He rarely slept through the night. Our search for someone who was experienced with Chiari in children brought us to Dr. David Frim and the University of Chicago Comer Children's Hospital."

Since then, Riley has undergone one brain surgery and four procedures related to a shunt that drains excess spinal fluid.

"He has high pressure due to too much spinal fluid," Rachel Johnson said. "It's like shaking up a pop bottle. It has nowhere to go."

Since Riley can't express himself verbally, his family looks at his eyes to gauge his level of discomfort. The worse the pressure gets, the more his eyes cross.

"Riley's in uncharted territory," his mom said. "There's no cure. They try to alleviate the symptoms."

Artist Jeffrey Breslow working on a sculpture of Riley Johnson

The Riley sculpture was the signature piece at Breslow's Forms from Nature exhibit and auction in September 2006. Riley was cast in a series of nine sculptures, with the first edition being donated by Breslow and permanently installed in Comer Children's Hospital. Half the proceeds from the exhibit were donated to the hospital as well.

Rachel Johnson was thankful for the opportunity to support Comer Children's Hospital. "We want to give back to the hospital that has given us so much. And we're grateful for the efforts of Jeffrey and his wife to raise funds to help Comer Children's Hospital."