'Arlene' highlights rare disease

Posted 4/17/13

Arlene, the heroine of “Arlene on the Scene,” a children’s novel about a Rhode Island fourth grader with a debilitating muscle disorder, is a girl trying to fit in with the kids in …

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'Arlene' highlights rare disease


Arlene, the heroine of “Arlene on the Scene,” a children’s novel about a Rhode Island fourth grader with a debilitating muscle disorder, is a girl trying to fit in with the kids in school. In the real world, she represents about one in 2,500 people in America who suffer from Charcot-Marie-Tooth disease (CMT), a neurological condition that progressively weakens muscles. 

“It was in the fourth grade that I first arrived on the scene wearing my custom-made, bright purple leg braces decorated with floating butterflies,” is the way Arlene introduces herself in the novel that was first published in 2010. “Kids, teachers, even the janitors sure took long stares at me with these new things on my legs. Maybe the purple was just too much.”

The diagnosis of CMT came over the summer and, like most kids, Arlene is more worried about how her classmates will react to her braces than she is about the disease itself. She has had the summer to resign herself to the condition, comforted so to speak, by the fact that her mother suffers from the same disease. But this is her first exposure to the world at large since the diagnosis and, like one of the authors of the tale, she has a lot of explaining to do. She does so admirably.

“In fact, there really is a 9-year-old girl living with CMT in Rhode Island,” wrote Carol Liu, a former Warwick resident who was at her old school last Friday, talking about the book and the disease.

“This is my old school,” she told an assembly of fifth graders at the Sherman School. “You know that bench outside the principal’s office? Where you wait to see the principal? Well, I spent some time sitting on that bench myself.”

Liu was in town promoting the sequel to the 2010 book, “Arlene, the Rebel Queen,” which was released in March. As she did in the first book, Liu doesn’t allow Arlene to remain on the sidelines and this time she and her mischievous school chums take on climate change and set out to reduce the fictional Greenwood School’s carbon footprint. Along the way, we see Arlene coping with the advance of CMT with the same determination she showed in the first book. But Liu was at the Sherman School explaining why she wrote the first book, which was to introduce the kids to CMT and to promote empathy among the kids for people who are different.

“Sympathy is from a distance,” she explained. “Empathy is up close.”

She explained how it is easy to feel sorry for people afflicted with various diseases but more difficult, and more important, to share their feelings. In “Arlene on the Scene,” Liu creates a character that has much more in common with her peers than differences. Arlene is petty, petulant, insecure and vengeful but she is also learning to understand how rough things can be for all kids, even the ones she doesn’t like. In short, what Arlene discovers in the first book is that she, too, needs a lesson in empathy and just facing a frightening and frustrating future coping with CMT does not excuse her from her responsibility to the world.

But CMT is a large part of Arlene’s and a lot of other peoples’ world. It is one of the most common inherited neurological disorders. CMT is also known as hereditary motor and sensory neuropathy (HMSN), a group of disorders that affect peripheral nerves, the nerves outside the brain and spinal cord in the muscles and limbs. CMT affects both motor and sensory nerves. It weakens foot and lower leg muscles, which causes foot drop, a high-stepped gait and frequent falling. Later in the disease, weakness and muscle atrophy may occur in the hands.

Liu’s co-author, Marybeth Sidoti Caldarone, has CMT and is confined to a wheelchair. Unlike most people, Caldarone developed the disease even though there was no history of it in her family. That is one of the many puzzles surrounding CMT that research hopes to resolve.

According to the National Institute of Neurological Disorders and Stroke, trauma or some other illness can prompt the onset of symptoms, most often in adolescence or early adulthood, but some individuals develop symptoms in mid-adulthood. The severity varies greatly even among family members and progression is gradual. In rare cases, individuals may have respiratory muscle weakness but CMT is not considered fatal and people with CMT have a normal life expectancy.

CMT is caused by mutations in genes that produce proteins involved in the structure and function of nerves. Nerves slowly degenerate and lose the ability to feel heat, cold and pain.

There is no cure for CMT, but physical therapy, occupational therapy, orthopedic devices and surgery can help symptoms.

Entering a treatment program early delays or reduces muscle atrophy if it begins before nerve degeneration and muscle weakness get to the point of disability.

Arlene wears braces on her lower legs but she insists on at least trying to do everything the other kids are doing and when she can’t, she tries to hide that from the other kids.

“I hesitated outside the door for a second, worried that I might get knocked down in that tornado of kids,” says Arlene. “I mean, even without a tornado, I fall a lot. I’ve got so many scrapes and bruises; my knees look like they’ve been pecked by an army of ducks! Mostly it happens at home, but sometimes my legs just give way at school. I’ve learned to play it cool by pretending that I’m examining some ladybug crawling by or that I just feel like rolling in the grass. Sure, I laugh, but what I really want to do is scream…”

The neat trick that Liu achieves with Arlene is creating a protagonist that is a kid first; a kid who just happens to have CMT. And, like most kids, Arlene has a distance to go before she gains a more realistic worldview. At first, Arlene insists that she can do anything that other kids do. The “mean girl” in the narrative is the one that delivers the message that Arlene needs to hear but refuses to listen to.

“You proved that you can do just about anything you want. I was just upset, that’s all. I mean, you came back to school this year and everybody loved you, everybody paid so much attention to you. And you let that all happen, sometimes even when it was just because people were feeling sorry for you. Then you kept acting as if you were just like everybody else. But you’re not.”

The mean girl delivered this critique after Arlene won the presidency of the school as a write-in candidate and as a fourth grader who got special permission to run for the office. The mean girl is just telling Arlene to think about the difference between what you earn, like respect, and what you are given, like sympathy. Whether Arlene gave more than she took is up to the reader to decide, but the lesson Arlene learns on top of all that is that she needs to put herself in other peoples’ shoes, at least metaphorically, and that CMT is only one of the things she’ll have to deal with in her life. As she confesses to the mean girl, after the mean girl says Arlene really is just like everybody else: “Nah, I’m just me, doing my best. It’s all I can do.”

 “Arlene on the Scene” is available in bookstores, at Amazon.com and on Kindle. “Arlene, the Rebel Queen” is in bookstores and available at amazon.com. For more information about CMT, visit the Hereditary Neuropathy Foundation at www.hnf-cure.org.


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