But for her power wheelchair, Jerika Bolen is every bit an active 14-year-old girl — a hopeless romantic with shiny purple hair, a love of alternative music and an addiction to Facebook.
She has a maturity and wisdom that belies her age, and on a recent spring day, as other 14-year-olds were finishing their final year of middle school and making summer plans, Jerika told her mother she was ready to die.
The Appleton teen has Spinal Muscular Atrophy Type 2, an incurable genetic disease that often claims lives before adolescence. She’s mostly immobile and knows her already chronic pain — a seven on a scale of one to 10 on her best days, she said — will only grow worse.
She's at peace with her decision, she said, but heartbroken for those who’ll grieve her death, expected to come by late summer.
“When I decided, I felt extremely happy and sad at the same time,” she said. “There were a lot of tears, but then I realized I’m going to be in a better place, and I’m not going to be in this terrible pain. I’ve been working on it and thinking about it for way longer than anyone else has.”
Jerika and her mother, Jen Bolen, made arrangements for hospice care in early June, and Jerika will go without the ventilator that now assists her breathing for 12 hours each day. Jerika wanted a full summer, so the ventilator won't be pulled until the end of August.
Once the ventilator is shut down, there's no knowing how soon the end will come. Jen knows Jerika has a certain stubbornness built into her psyche — and though it's long been to her benefit, she hopes her daughter's fighting spirit will relent when it's time.
"I hope it's not more than a few days," Jen said.
Jerika's pain is mostly felt in her hips and back. She gets nerve spasms and her bones are weak because she's never been able to get up and move. Jerika said the aching is persistent, and she feels sharp, sudden pains. Though painkillers ratchet down the intensity, she only takes them when the pain moves into the eight-to-10 range on the scale.
The medications that keep the pain at bay have damaged her body; the really tough days are becoming more frequent.
Jerika said there’s peace in being able to confront the inevitable on her own terms. Is she scared?
“A little bit,” she said, “but in knowing my doctors for so long, I’m pretty sure they’ll take good care of me.”
Jen, a single mom who works as a nurse for ManorCare Health Services in Appleton, had long promised to stand behind Jerika’s wishes when the pain became too much. Knowing the conversation would eventually arrive made the words no easier to process — but a promise is a promise.
“If she’s at peace with it, I have to find a way to make peace with it,” Jen said.
‘Maybe because I’m strong’
Spinal muscular atrophy causes the muscles to waste away, and Jerika lost much of her physical strength as a baby. Since then, there had been times of plateau between periods of muscle loss, but the losses always returned. And the pain always built.
There was a time when Jerika could raise her arms above her head. Today, her strength is limited to her hands. It’s sufficient to control her power wheelchair and entertainment sources — usually a computer mouse or smart phone.
She said she wants to die at home, surrounded by her mom, extended family and her two dogs, Tupelo and Julie.
For now, she’s intent on making the most of this final summer. She’s hosted sleepovers with friends, and for her final wish, she’ll be going to a prom. It's July 22, and the community is invited. It’ll be a night for friends at Appleton’s Grand Meridian, with lights flashing and a disco ball spinning. Guests will enjoy candy, cake and hors d'oeuvres while the music fuels the night’s most important element — "People getting crazy on the dance floor; she loves that,” Jen said.
Jerika picked out a greenish blue dress for her dream prom.
“I’m super happy,” she said of the prom preparations, “and I don’t have to think about anything bad at the moment.”
On a recent Saturday, Jerika maneuvered her chair into a shady spot in the yard and watched as a friend danced through the prom choreography they worked out together. She’s never been jealous of other kids’ physical abilities — dance or otherwise.
Her grandmother, Sue Bolen, laughed while talking about Jerika imploring her to do the splits.
“She gets a kick out of watching us try,” she said.
Jerika and her mom made a recent day trip to the Muscular Dystrophy Association summer camp that has long been a summer highlight. There also was a night of fireworks with her grandparents and a trip to the movies.
For all she’s lost, Jerika has maintained control of her facial muscles. She loves to smile.
“I try to be as happy as possible,” Jerika said. “I know I can’t always be happy every day. I still wonder why God picked me to have this disease, and I know I can never know the reason. Maybe because I’m strong, I guess.”
‘I was ready a long time ago’
Jen was encouraged by doctors to prepare for the end almost from the beginning — but 13 years ago she wanted no part of defeatist talk.
She knew something was wrong when her baby went from nearly crawling to an inability to hold herself up. A devastating diagnosis, however, served as a call to action, and Jen bolstered herself to do anything for Jerika not only to survive, but also to live to her fullest while praying for scientists to make breakthroughs.
Spinal muscular atrophy destroys nerve cells in the brain stem and spinal cord that control voluntary muscle activity.Though breakthroughs have happened in Jerika’s lifetime, “there’s still so much about this disease that we don’t know,” Jen said.
Kari Stampfli, director of the pediatric palliative care program for UW Health in Madison, said both mom and daughter have been nothing short of courageous.
“There is no doubt they’ve turned over every stone and tried every treatment to make things better for her, but we really haven’t been able to help her pain,” Stampfli said.
They were more aggressive in attempting treatments than some families, and neither Jen nor Jerika have regrets. Jerika, they believe, has lived longer than she otherwise would have.
Still, it's been a difficult fight. Jerika has been in and out of operating rooms more than 30 times. Last year, she had the heads of her femurs removed in hopes of bringing comfort to her aching hips. In 2013, she had her spine fused.
Last year’s surgery was a turning point that guided her hospice decision.
“I was ready then,” Jerika said. “I was ready a long time ago, but I kept going. After that surgery — it didn’t work and my pain got worse — I kind of sat down and thought, ‘Am I doing this for me or for my family?’ I kind of realized I was doing it for my family.”
'How do you not be bitter?'
Those who know Jerika best are no less amazed by her attitude.
Her aunt, Lisa Wilson — affectionately known as Auntie Bean — went to say good night to her niece during a recent weekend visit, and emotions caught up to her in realizing their remaining moments are few.
"I'm sitting on the bed next to her crying, and she ... told me all these things to make me feel better," Wilson said. "That's Jerika."
Jerika is often at the computer for sessions of “The Sims.” It’s a life simulation game that allows players to take on a character and engage in activities and relationships. She said it’s allowed her to better imagine a more typical existence.
When not in her chair, Jerika lies on the couch with sunshine pouring through the south window with her Chihuahua, Julie, cuddled up next to her. Mom and daughter have a lot of time to talk as Jen tends to Jerika's needs.
Jen said she’s always done her best to keep things “fun and exciting” and as typical as their atypical journey could allow.
Jerika went to school at Janet Berry Elementary School and spent a year at Valley New School. She recently completed eighth grade through the online Wisconsin Connections Academy.
When Jerika was little, Jen would hold her on the playground swings and take her down the slide. Jerika’s grandfather, Mike, attached a laundry basket to a saucer sled so Jerika — held tight and warm in blankets — could sit upright while being pulled through the snow.
The Bolens are afforded home nurses for up to 18 hours a day, which allows Jen to work and catch some sleep. Their nurses have been like family, and members of her care team said Jerika has been more than a patient.
Jerika is quick with humor and skilled at employing a little manipulation when she wants something, nurse Alyssa Dahlen said.
But she’s as sweet as she is strong, Dahlen said. Dahlen talked about Jerika’s interest in looking at pictures of Dahlen's baby. She’s a girl who’s never walked, much less hopped on a bicycle. She’s mourned the loss of friends who have the same disease.
“How do you not be bitter?” Dahlen asked. “It’s amazing.”
‘It’s her pain’
Jen has kept her mind occupied by making sure prom will go beyond her daughter’s expectations. Recent weeks have brought a mix of emotions, and she attributes the best moments to her daughter setting the example.
“I’m strong because of her, that’s for sure,” Jen said. “It’s just we know we’ve literally done everything we can do.”
Jen knows many struggle to understand Jerika's journey, including her decision to bring it to an end. She’s taken questions on how Jerika’s decision to die differs from assisted suicide.
“People don’t realize what it takes to keep her alive," Jen said.
She recognizes many people have discomfort with the idea of someone so young making such a huge decision.
“I know she’s only 14, but she’s old enough to decide,” Jen said. “It’s her body and it’s her pain.”
Stampfli dismissed all the notions of those who'd question Jerika's decision. For kids with spinal muscular atrophy, “the standard of care is often comfort measures from the beginning," she said. When taking on any course of treatment, there’s always the option to stop if it isn’t offering the quality of life that was hoped, she said.
“They did it all,” she said.
As both a mom and a nurse, Jen rationally knows it would still only be a matter of time. The heavy doses of painkillers are hurting Jerika’s body, but it’s the only way to give her any comfort.
Logic hasn't precluded emotion, and Jen has done a lot of crying. There have been times of anger and numb feelings.
“I’ll still have you,” she said while petting Tupelo, their golden retriever.
Jen entertains fleeting thoughts that a perfect combination of medications might be found to restore her daughter’s comfort, but she knows it's fantasy.
“I don’t think she’s changing her mind,” Jen said.
Jerika already uses her ventilator on its highest settings. When taken off, she’ll be put on morphine to calm the feeling of being starved for air.
But for now, as she braces herself for those final, heartbreaking events to unfold, Jen is determined to provide one last summer of joy for a girl who’s given and endured more at 14 than many will in full, long lives.
“She’s a very unselfish little girl,” grandma Sue Bolen said, “and she’s tougher than anyone I’ve ever known.”
SEND JERIKA YOUR MESSAGE
We're collecting wishes and messages for Jerika and will publish them next Sunday. Email them to firstname.lastname@example.org or post them on our Facebook page at facebook.com/postcrescent at 8 p.m. Sunday.