HELPING KIDS UNDERSTAND
The Big Listen

Helping Children Understand Their Diagnoses

Trust, emotional support and love all are part of a diagnosis conversation. Noah Spencer, 8, who has spinal muscular atrophy, connects with a fuzzy friend in a wheelchair. Photos of Noah by Tim Fuller.

by Christina Medvescek

Joanne Wechsler remembers when she first formally told her preschool-age son Adam that he “had muscular dystrophy.”

“I don’t have that!” he protested loudly. “I don’t have muscular dystrophy!”

Adam had been going to doctors since he was 2 and his parents learned he had Duchenne muscular dystrophy (DMD) when he was 3. So far Adam was satisfied with minimal explanations for the doctor’s visits and uncomfortable procedures. When he was about 5, his parents decided to talk to him about his diagnosis more formally.

Wechsler and her husband, David, of Jericho, Vt., realized pretty quickly that Adam “hated the idea that he had something,” she says. “We needed to change our language and not say, ‘You have something,’ but say, ‘there’s a problem with your muscles. Your body doesn’t make the glue that holds the muscles together.’”

Opportunities to discuss the disease come up naturally during active family life, says Joanne Wechsler, shown with husband David and sons Adam (left), now 10, and Jacob.

An Ongoing Process

Telling children about their neuromuscular diseases is a necessary, delicate conversation. Parents rarely feel con-fident going in. How much information? Is this the right time? How do I explain this simply? Will this cause worry and fear?

There isn’t a single “Big Talk” about the diagnosis, when all information is transmitted and everyone goes forth enlightened. Rather, ideally, a conversation develops in little bursts over time, as the child matures, life happens and questions come up. Rather than a Big Talk, experts say this conversation is a Big Listen.

“I would suggest you never ‘tell’ a child they have a muscle disease. You just set the stage,” says Charlotte Thompson, a pediatrician with 50 years experience caring for children with muscle diseases, and author of Raising a Child with a Neuromuscular Disorder (Oxford University Press, 1999).

Setting the stage means to go someplace quiet, like for a walk or an ice cream. Turn off the cell phone. Create openings for conversation, such as, “You’re probably wondering about all the medical tests you’ve taken recently.” Then be quiet, listen and answer questions as they come up.

If you try to “tell” kids rather than following their lead, you may use the wrong language, say something inappro-priate for the child’s age or answer the wrong question, Thompson says. “You want to come from the kid’s perspec-tive. Kids are very smart. They may not be worried at all. They may already know. He or she may have overheard something or talked to other kids at clinic or picked up a brochure.”

These occasional, usually brief conversations are part of building a child’s resiliency — their capacity to face, overcome and even be strengthened by adversity. The elements that build resiliency — trust, emotional support, self-esteem, hope, achievement, unconditional love — all come up in these conversations.

How to Build Resiliency

Brief, occasional talks about the disease as issues arise can help children become stronger in the face of adversity.

Parents and professionals suggest these guidelines:

Be honest, but don’t talk too much.

“Provide the information the child is asking for, in a way that says, ‘this is just how it is,’” advises Joanne Wechsler, who’s also a family support coordinator for Vermont Parent to Parent, an organization for parents of chil-dren with disabilities. Children “just want knowledge: ‘Tell me why this is happening. Oh, that’s why. OK, bye.’”

Be upbeat. Focus on hope. Provide reassurance.

Two years ago, Sheri Dede’s daughter, Kaitlyn, 9, received a diagnosis of desmin storage myopathy, a rare condi-tion that causes muscle and organ damage.

“I sat her down and told her they have a name for this,” recalls Dede, of Billings, Mont. “I said, right now there’s not a cure for it, but there are things we can do to make things better for you. We have all these doctors that are going to help, and we’re going to get you what you need.”

It’s OK to be a little sad.

It’s best to be matter-of-fact and neutral in tone, but sometimes emotions just well up. Although some experts say parents should never cry in front of their children, others say that, within limits, it’s OK. When emotions come up, parents can admit they’re sad, and also let children know that sadness won’t break them.

“Sometimes it makes it easier for (children) to feel their own emotions,” Dede says. But don’t go too far — the conversation isn’t about the adult. Children shouldn’t be afraid to talk about their diagnoses for fear of upsetting their parents.

Acknowledge their emotions without trying to “fix” them.

Anger, grief, fear and other negative emotions are reasonable responses to a muscle disease, Thompson says. Rather than trying to make the feelings go away, parents can lovingly accept and show that they understand.

Kaitlyn Dede	Adam Wechsler

Don’t predict the future.

If children don’t ask about the future, don’t bring it up. If they do ask, answer honestly but keep the door open to possibility.

Rhonda Hillman of Fenton, Mich., recalls that when her daughter, Gabrielle Ford, asked if it was true that serious heart problems could develop in Friedreich’s ataxia, “I just told her, well, the heart is a muscle. But if you’re asking me to put a time line on you, I refuse to do that. Every case is different.”

When increasing hand weakness led Dede’s daughter to ask if her condition would get worse, Dede responded, “Yes, but we don’t know for sure what’s going to happen. We’ll make sure we do what we can when things happen.” Dede then contacted the school about accommodating Kaitlyn’s hand weakness, offer-ing a concrete response to the situation.

The Quiet Child

Some children — especially teens — may refuse to be drawn into discussions about their diseases and may even seem to be in denial. This may be their personal style or a symptom of their age and no cause for concern — or it could be a mask for emotional difficulties. Thompson advises parents to watch for signs of depression.

If children are more withdrawn than normal, or have changes in eating, sleeping, friends or school, parents should consult a family therapist, social worker or school counselor, who may even pay a house call. Counseling also can be valuable for the parents alone, if the child refuses to participate.

A lack of questions about the disease may simply mean the child is not focusing on it.

Parents should let quiet kids know they’re available to talk if the child wants, and create opportunities for conversation to happen. Just knowing the door is open is enough for some children.

Nancy Nereo, a clinical psychologist at the Hospital for Special Surgery in New York, says a lack of questions about the disease may simply mean the child isn’t focusing on it.

In 2003 she published a study in which boys with Duchenne MD ages 6-12 were asked what three wishes they would make if they could wish for anything at all (see Quest, November-December 2003).

Surprisingly, the boys with DMD were no more likely to make health-related wishes than were boys in the control group. In fact, the siblings of the boys with DMD were more likely to make health wishes, especially for the health of their brothers.

Adam Wechsler, now 10, still doesn’t want to talk about his DMD. “He’s a typical guy. He doesn’t talk a lot and he doesn’t want to talk about his emotions,” his mother says.

Adam has never asked what his mom calls “the really hard questions” — Will I need a wheelchair? Am I going to die? (see “The Hard Questions”). Although he’s sometimes frustrated by his declining abilities, “he’s generally a happy kid and doesn’t see himself as different,” Joanne says.

The Wechslers haven’t shielded Adam from his DMD. He recently was appointed MDA Goodwill Ambassador for Vermont, a position that requires talking about his diagnosis. Making friends at MDA summer camp is another way he’s exposed to information about his disease and the future, says Joanne, who hopes experiences like these will help him become a good self-advocate.

Talking with Siblings — and Others

A neuromuscular diagnosis affects the entire family. All the tips for talking with affected children about the diagno-sis hold true for their brothers and sisters.

In addition to love and concern, siblings may feel guilt, jealousy, anger or embarrassment about having a sibling with a disability. These aren’t comfortable feelings for parents to hear about, but kids need to know they can talk about them, Nereo says. Some siblings also benefit from talking with a counselor.

Outside the immediate family, many parents talk about the diagnosis with the affected child’s classmates at school. Sharing information about the disease and its effects cuts down on bullying and facilitates friendships.

Just like everyone else in the family, parents need someone supportive to talk to about the diagnosis. Extended family may or may not fulfill this need.

If family members don’t seem to get it, and talking with them is more draining than supportive, just drop it, advises Joanne Wechsler, who offers information only when her relatives ask her. “True support comes from other parents of children with the same diagnosis, who understand.”

Seize the Moment

This spring, Adam Wechsler wanted to go out for Little League but his parents weren’t convinced it was a good idea. In the course of negotiating a solution, they discussed his muscle disease.

The conversation was realistic about his abilities, creative in looking for solutions and supportive of his interests in other areas. These little opportunities come up regularly and naturally during family activities, Joanne says.

“He’s OK now with saying, ‘I have Duchenne muscular dystrophy,’ whereas at age 5 he couldn’t say it.

He knows that’s just part of who he is.”

HELPING KIDS UNDERSTAND

I DON'T WANNA!

Getting Your Child to Go With the Flow

by Kathy Wechsler

A wise teenager once said, “I think in order to motivate people, you have to kind of know how they work, and it’s different for every person.”

Krista Fincke of Tarpon Springs, Fla., has experience with not wanting to do physical therapy (PT), take her medications or wear her ankle-foot orthotics (AFOs).

A bright 15-year-old with Charcot-Marie-Tooth disease (CMT), Krista offers Quest readers some suggestions for motivating your children to keep up their health care routines. Other tips are provided by Anne Connolly, a pediatric neurologist at Washington University in St. Louis, and parents of young children with neuromuscular diseases.

Be Consistent

If your child takes his or her medication every morning after breakfast, it becomes a comfortable routine. Skipping a day sometimes makes it harder to get back into the habit.

Consistent stretching, once or twice a day, is the key to getting your child to like being stretched, says Connolly, an MDA research grantee who sees patients at MDA’s clinic at the university. If limbs are stretched intermittently, it’s more painful because the tendons lose their elasticity, and you’re fighting against resistance, she adds.

“The nighttime AFOs (night splints) give 10 hours of ‘free stretch’ time while sleeping, and mean that the ankles will stay looser longer,” Connolly says. “Without stretching, the stronger muscle group ‘wins’ and the contracture will develop.”

Most children don’t mind wearing night splints because they help them function better. The main complaint is that they’re big and bulky and make it difficult to change positions in bed. For these reasons, Connolly recommends having children begin wearing night splints at an early age so they’ll get used to the way they feel.

“Some parents say that they give their kids a couple of nights off a week,” Connolly says. “Usually that’s not a great idea, because then they’re much more likely to compare that night with the next night. It’s better to just wear them all the time.”

Make It Fun

It’s much easier to get your child to comply with a medical regimen when you make the activity pleasant. Parents can be pretty creative in distracting their children from monotonous tasks such as stretching and other PT, Connolly says.

Rock Cottone stretches the leg muscles of his son, Torre, while mom Molly reads a story aloud.

R. Rocco “Rock” Cottone, a professor of psychology at the University of Missouri in St. Louis and the father of Torre, a 9-year-old with Duchenne muscular dystrophy (DMD), says he and his wife, Molly, take turns with Torre’s care and work as a team whenever possible. They try to stretch his heel cords and hip flexor muscles in the mornings and evenings.

“We try to make his stretching and the time we put on his AFOs ‘fun time’ — we stretch him at night while we watch TV or one of us reads stories,” Cottone says. “It’s best not to make stretching the focus, so that it’s associated with some fun family time.”

This “fun time,” along with a brief explanation of why stretching and wearing his AFOs are important, keeps Torre from complaining about his nightly routine, as long as the stretching doesn’t get too aggressive.

Occupational therapist Michelle Bragdon instructs Joseph Miller on Nomoe in the Carriage Barn Equestrian Center’s therapeutic riding program.

Dena Miller of Haverhill, Mass., says her son, Joseph, 3, loves going to PT at his preschool twice a week.

In the school’s gym, Joseph, who has spinal muscular atrophy (SMA) and uses a power wheelchair, enjoys playing in the large ball pit and the wheelchair-accessible playhouse. His physical therapist also uses balance boards and big exercise balls to help with motor skills, and has him play basketball and throw Beanie Babies to work on his arm strength.

In occupational therapy (OT), which he attends once a week at school, Joseph makes puzzles and plays with Play Doh while lying on the floor and propping himself on his elbows to strengthen his chest muscles.

Joseph also does hippotherapy (therapeutic horseback riding) and aqua therapy once a week.

His favorite parts of hippotherapy are “when the horse makes noises” and “when I get to say ‘walk on.’” He enjoys spending time at the barn, helping clean out the stalls and brush the horses.

In addition to daily stretching and using his Standing Dani, Joseph works each of his muscle groups with games and activities his mother has invented.

“If I want to work on his legs we’ll do something where he’s kneeling to play the game,” says Dena, who has a basic knowledge of PT and OT skills from earlier work with children with brain injuries. “Mostly all his toys that I’ve purchased have a therapeutic value.”

Joseph likes to play with his collection of rocks of different sizes and weights. Dena turns this hobby into exercise by arranging several storage buckets of different heights. He sorts his rocks and puts them in various buckets, working his arms.

Communicate

Telling children why they need to do PT, stretch, wear AFOs or have surgery helps to motivate them, especially when parents let the child have a say in whether or not to proceed.

Krista Fincke enjoys her sessions with her physical therapist, Nicole Baisley.

When she was 12, Krista Fincke, now 15, stopped going to PT (for insurance reasons), but she also stopped doing her exercises at home.

Krista’s mother, Helene, wasn’t able to monitor Krista’s exercises because of her work schedule. It took a neurology appointment in October to motivate her to make some changes.

“What really worked best for us is to get a third party to express how critical treatment is,” Helene says. “I wanted the doctors to be very straight with Krista, and she needed tough love. They needed to stress how important physical therapy was for her so she could remain strong and mobile.”

Give Your Child a Voice

Make sure your child is involved in the important decisions, Connolly says. She never recommends forcing a child to have surgery or do PT.

Torre Cottone, who’ll be attending fourth grade at St. Joseph School in Cottleville, Mo., recently had surgery on his heel cords and hip abductor muscles.

“The goal of the surgery is to give him the stability to walk with full leg braces, and he ultimately made the decision [to have it],” his mother, Molly, says. “We told him he didn’t have to do it, that it was totally up to him.”

The weight a family gives to the child’s opinion depends on the maturity of the child and the nature of the medical treatment. Parents should also be sure the child understands all aspects of the choice being made.

When asked how he felt about having the surgery, Torre responds, “Kind of excited.”

Why “excited?”

“So I can walk again.”

Agnes Jani Acsadi, a neuromuscular physician doing clinical and research studies at Wayne State University’s Detroit Medical Center, describes a possible approach to make children more willing to undergo a nerve conduction velocity (NCV) test, which tests nerve function and is sometimes used in diagnosing a neuromuscular disease. An NCV is uncomfortable, even painful, because electricity is sent through a patchlike electrode placed on the skin.

Acsadi finds that some children are more comfortable if they can participate in the testing.

“It takes the fear away if I tell them that they can do it to me, too, if they want, and look at the screen,” Acsadi says. “Older kids can compare what they see on the screen or just kind of have them see that this is something other people need done, and I don’t have any problem taking it on myself.”

Justin Wilton, 6, of Warren, Mich., was cautious of being hooked up to the big machine. Justin, who has CMT, wanted to operate the machine and opted to conduct the NCV on Acsadi after his own test.

Justin thinks “it’s cool” that he could do “the zapping test” on the doctor. Kids might also be reassured by acting out such tests with a stuffed animal or doll.

Keep It Positive

To get your child to accept changes and new equipment, Molly Cottone recommends being excited about it yourself. She says that when parents dread getting a wheelchair, so does the child.

Torre loves his power chair because it gives him freedom.

“We’ve always been open with equipment when he needed it. We had it there for him, sometimes before he’s even needed it,” his mother says. “The chair was a big, exciting thing for him and we kept it very positive.”

A huge motivator for Krista Fincke was to find a physical therapist she liked.

Her current physical therapist “made it a lot more fun, and she has a more outgoing personality,” says Krista, a freshman at Eastlake High School.

“She’s a really happy person. Honestly, I think that if I didn’t do the exercises and she said, ‘So how have you been doing?’ and I say, ‘I didn’t do anything,’ she’d be so sad. I think I’d be sad with her. That kind of makes me do it, too.”

Her mother comments, “I think that physical therapy not only is good for her physically but psychologically, too, because she is very connected to her physical therapist and her physical therapist does give her positive feedback.”

Create Camaraderie

“On some level, both the parent and the child have to be motivated [to do PT, stretch, diet, etc.], because if it’s only the child, it won’t work and if it’s only the parent who’s motivated, it also won’t work,” Connolly says.

It also helps children to know that they’re not alone and to learn from other children or adults who’ve been through or are go-ing through the same thing.

If your child needs spinal-fusion surgery, it may be helpful for him or her to talk to someone who’s had that surgery.

This works for painful tests, too.

Offer Incentives

Depending on the age of the child, incentives sometimes work as a motivator, if they’re appropriate. You don’t want to reward a child for taking prednisone, a “yucky”-tasting drug that generally causes weight gain, with a Twinkie.

Connolly suggests, “Maybe it means, ‘Listen, if you keep your weight at the same target weight, we’ll get to go to the zoo’ or something different or fun.”

With teenagers, it may be a different story.

Krista sums it up with a metaphor:

“Positive reinforcement is good, but that doesn’t really work on me too much, because I’m kind of a lazy person.

“If you come up to me and say, ‘Krista, if you vacuum for the week, I’ll give you $5 at the end of the week,’ I’ll say, ‘I’d rather watch TV than get $5.’ But if you say, ‘Vacuum or you’re not going anywhere this weekend,’ I’ll definitely vacuum for you.”