Girl battles neuromuscular disease

Published Thursday, April 14, 2005 1:18:01 PM Central Time

EagleHerald senior staff writer


Jerika Bolen, 3, pulls her mother, Jennifer, behind her motorized "wheels" at their home in Menominee. Jerika has spinal muscular atrophy, and her mother is working to raise money to buy specialized equipment to keep her muscles strong.
EagleHerald/Rick Gebhard



MENOMINEE -- Jerika Bolen has never taken a step in her young three years of life, yet she already has taken many journeys.

Her latest journey is attending school. The bright and energetic little girl is full of life and excitement as she recites sayings and shares songs she has learned with her classmates.

Of course, all of this is done in the front rooms of the home she lives in with her mother and aunt in Menominee, and it is done while she streaks around the rooms in her motorized wheelchair she simply refers to as her "wheels."

She can zip right up to someone and stop on a dime. The chair turns in a tight circle to allow her a quick escape and is small enough to fit through spaces not wide enough for a conventional wheelchair.

But it places the fragile child in a sitting position for much of her day, and with a disease that weakens her muscles and eventually, her bones, Jerika will lose the ability to sit up straight in her chair as time goes on.

She has spinal muscular atrophy, a progressive neuromuscular disease that generally leads to the loss of function in voluntary muscles. As a result, children affected with the disease can lose their ability to sit, stand, walk, control their head, swallow or breathe.

Jerika was diagnosed with SMA when she was about a year old. Her mother, Jennifer, has maintained Jerika's strict amino acid diet, which includes extra vitamins and calcium, through a feeding tube she has had since diagnosis. Jerika can eat some regular foods, but not enough to sustain her.

She also uses a bipap machine, which controls oxygen flow into her body, for certain periods each day. Breathing is often difficult for children with SMA, since their muscles are not strong enough to take deep breaths.

Without the ability to fully breathe in and out on her own, Jerika also uses a machine to help her cough. It expands her rib cage and breaks up mucus that collects in her lungs, which can then be suctioned out.

Every day is a lot of work for Jennifer, a single mom. She gets help from her sister, Lisa Bolen, and her mother, Sue Bolen, as well as a physical therapist who works with Jerika every week.

But when it comes to finding out about new treatments, therapies or the best way to care for her daughter, Jennifer has learned to become Jerika's strongest advocate.

She communicates regularly with other parents of children with SMA, and has tragically seen many of them succumb to the disease. She and Jerika attend annual conferences where they can learn about new medical advances, and she is constantly on the Internet doing research.

"Five children we knew well have passed away recently," said Jennifer, "including one little boy who fought so hard."

She estimates at least 30 children she has come to know through a Web-based support group or the conferences have died since Jerika was diagnosed. "It is so hard. When Jerika is 10, what are the numbers going to be?"

What she has learned is that Jerika could benefit from using a device called a Sprout dynamic wheelstand, which would place her in a standing position, with support, to allow her body to stretch out and build some lower bone strength.

"Contractures on her hips and knees affect her bone density," Jennifer said. "She easily breaks bones all the time." The stand also will help slow the progression of scoliosis, or curvature of the spine.

According to her physical therapist, it also will allow her to interact with people at eye level.

Jennifer wants Jerika to have the special wheel stand, but funding was denied by the state agency that funds her care. For Jennifer, it's all about doing everything she can to enhance her daughter's quality of life for however long she can.

"Losing all those other children is a constant reminder that she could be gone tomorrow," Jennifer said. "One day is good, the next is bad."

Even sending Jerika to school in the Early Childhood Program at Central Elementary has its risks.

Her resistance to infection is low, and she's around other children with the usual litany of colds, coughs and viruses. She has brought some of them home, which can be catastrophic for her. A cold she contracted in June 2004, before she attended school, sent her to Madison, Wis., in an ambulance. Her local pediatrician, Sandy MacArthur, accompanied Jerika all the way there.

But despite the risks, Jennifer said she wants Jerika to attend school like any other child. "She enjoys it so much that unless it becomes a serious threat, she'll stay in school," she said.

Jerika's "wheels" were paid for through a state fund, Children's Special Health Care, which also helped Jennifer purchase a van and ramp a year ago.

But they denied her request for a power stand, saying that her existing powerized wheelchair provides her with enough independence.

As a result, Jennifer has taken it upon herself to raise the $13,000 needed for the stand, and is planning a fund-raising walk at Menominee High School later this year. She also is working with her employer to sell candles, proceeds of which would go to a fund being set up at Niagara Area Credit Union in Marinette.

"The SMA support group has some funds available to cover funeral and burial costs and for conference attendance, but I don't want to take that money away from other families," said Jennifer.

As she works toward raising enough money for Jerika's wheelstand, Jennifer also looks at making Jerika's life as happy and normal as possible.

"As she's getting older, she sees her younger cousins walking, and says 'I'm getting bigger, but I'm not a walker.' We never lose hope that they will find a cure. They have cured the disease in mice. I never said 'never' to her."

But Jerika realizes that she, like so many children she has met with her disease, may die some day. "She asks about heaven, because we've sent up balloons for the children we know who have died. But she says that she can walk when she goes to heaven."

Jennifer said she never looks at her own role as one of hardship.

"I think of them as angels and feel blessed that someone trusted you with them. Nobody in Jerika's presence will ever be sad."

Jennifer can be reached at for more information about the upcoming fund-raising events. More information will also be printed in the EagleHerald as it becomes available.