BY LINDA KOBERT 

When Joseph Berretone III went off to Duquesne University last fall, he and his mom, Monica Pedano, barely gave his health a second thought. Joseph had been diagnosed as a toddler with juvenile dermatomyositis (JDM), a rare autoimmune disease of the muscles that causes weakness, extreme fatigue, debilitating skin rashes, and other symptoms. His disease had been in remission for ten years, though. He was healthy. He was strong. And he was doing what kids do: growing up and leaving home.

The goal is to help the young person to gradually assume more responsibility for their own healthcare management and develop a greater sense of confidence.

Parents must also make their own transition.

But it was not a good year for Joseph. While his JDM stayed in remission, his immune system was still compromised, leaving him more vulnerable than most young people to a variety of ailments. He spent a good part of the school year being sick. 

"I was living in the dorms where there was always someone sick," Joseph says. "The first time I got sick, I came down with both strep throat and mono, that triggered it all. After that I wasn't able to fully recover."

It took four weeks for the providers at the student health center to accurately diagnose and treat Joseph's sore throat. When they did, he had a bad reaction to the medication. His throat was so sore he couldn't swallow food, so he lost a lot of weight. He was staying up late to study and go out with friends and working and traveling with the football team as equipment manager, so he wasn't getting enough sleep. After one rainy football game where Joseph sat on the field, soaking wet and freezing, he ended up in the hospital with pneumonia. "I actually had no idea that just being cold and out in the elements would contribute to me getting sick so easily," Joseph says. Pedano, who spent much of the semester driving back and forth across the state to check on and care for her son says, "It was a nightmare."

A young person first

Dr. Richard Chung, an internist and pediatrician who specializes in adolescent medicine at Duke University School of Medicine, says it's not surprising that young people with chronic conditions like myositis might have some difficulty when they take those first steps away from home and family support. "The 18-to-22-year age range is a time of a lot of change on every level," Chung says. It's a time when kids are graduating from high school or college. They're leaving home to continue their education or entering the workforce. They're moving away from a family unit to a more peer-oriented social and living situation. They're making the leap to financial independence, which can also cause changes in health insurance. And because they are no longer children, their healthcare must transition from pediatric to adult care. "It's a perfect storm," Chung says. "We have a lot of examples of young people with chronic illnesses who do reasonably well through their 18th birthday and then dramatically fall off, not because their disease has evolved or their treatments have changed, but because the supports that have propped them up over time fall away."

On top of all that, while young people in their late teens and early 20s are physically mature, their mental and emotional maturity is still in flux. Their cognitive and decision-making skills may not be fully developed until the mid-20s, in most cases. Young adults may have more trouble than older adults with things like self-management, decision making, and executive functioning— the ability to plan, organize, and complete tasks.

"It's not that young people aren't smart or talented, but they don't always have the ability to comprehend difficult medical concepts and make life decisions around those concepts," says Dr. Ann Reed, a pediatric rheumatologist and myositis specialist at Duke. "That leaves young people very vulnerable during a time when they're trying to be independent and make their own decisions. All of a sudden they are legal, and their parents aren't there to help them." For the young person who is living in a new place and trying to adjust to all the other demands of work or school and social life, their health may not be at the top of their list of concerns. Especially if their disease has been well controlled, it stands to reason that finding a new doctor or getting their medications refilled may not be the 19-year-old's first priority.

"We have to remember that a young person is a young person first, with all sorts of interests and priorities, and being a patient is only part of that," Chung says.

Youth vs. reality

N. Linda Iferika didn't want to have anything to do with her disease during her late teens and early 20s. Like Joseph, she was diagnosed at an early age with JDM. While she had periods where she experienced only mild muscle weakness, she had been taking prednisone and other medications on and off to control her symptoms. When she was finishing high school and starting college, however, she began to take her condition less seriously and sometimes stopped taking her meds.

"My symptoms weren't that bad, so I thought how bad could it be," she says. "At the time, I was extremely angry with the whole situation. I didn't want to be different. I didn't want to take the medicine. There were times when I had doctor's appointments, and I just wouldn't go. I didn't want to deal with it, so I just said, whatever."

Iferika now understands her reaction was a normal teenage response to an extremely overwhelming situation. Interestingly, the doctor who diagnosed her told her mom that Linda would outgrow the disease, an assertion that turned out to be inaccurate. Now in her late 30s, Iferika wishes someone would have corrected that misinformation, stressing the importance of following through with treatment recommendations. She now feels that some of her current physical challenges are probably related to that time in her life when she ignored the responsibilities of her treatment because she just wanted her disease to go away. 

For Joseph, on the other hand, it wasn't so much that he was inconsistent with his treatments as that he wasn't prepared for the health crises he encountered.

"He did the right thing and went to the doctor, but they didn't know how to deal with someone with such a compromised immune system," Pedano says. "There were so many things we needed to think about that we didn't even consider. I wish somebody had prepared us and said, hey look, this is going to be tough. You need to watch out for these things."

ABOUT THE MYOSITIS ASSOCIATION:

The Myositis Association is the leading international nonprofit organization committed to the global community of people living with myositis, their care partners, family members, and the medical community. TMA provides patient education and support, advocacy, physician education, and research funding for myositis diseases. To learn more about myositis go to myositis.org

Taking on adulthood

Making the transition into adulthood, however, doesn't have to be difficult for children with chronic or disabling conditions. There are steps families can take to help the young person move from reliance on mom and dad to taking responsibility for their own care. This transition flows more seamlessly when there is coordination and planning among the healthcare team, the parents/family, and the young person.

Experts like Chung and Reed often begin by assessing how ready the family is for this process. Children vary considerably in their ability to comprehend complex medical concepts as well as how comfortable they are with being more involved in their own healthcare. Parents also vary in how ready they may be to turn over the reins and let their child take on more responsibility. 

This assessment is especially important when the young person has a developmental, cognitive, or learning disability. Someone who has autism, for example, will need a very different plan for transitioning to adult care than someone like Joseph who has dermatomyositis but does not have the cognitive or emotional challenges other young people may face.

While every family is different in terms of readiness, laying the groundwork for this transition could start as early as age 12. For Chung, this means simply talking with the family about the idea that this transition is coming and that it is important to start thinking about it well in advance.

"The biggest thing is articulating the importance of transition," Chung says, "convincing everybody in the room that this is crucial, and then planning well in advance for what that transition is going to look like." The goal of transition is to help the young person to gradually assume more responsibility for their own healthcare management and develop a greater sense of confidence. Parents must also make their own transition. As they start encouraging their child to be more independent, the parents can back away from complete control over their child's healthcare and become more of a supportive backup.

Some of the things that will facilitate this independence are empowering the young person to schedule their own appointments and refill prescriptions. When they see the doctor, encourage the young person to be the one to respond to the doctor's questions and ask their own questions. And even younger children can be permitted to take their own meds every day.

When it comes to medications, it is important for the young person to know the names of all the drugs they take, why they are taking them, what the doses are, and what side effects to look out for. Prednisone, for example, should never be stopped suddenly without tapering the dose. It's also important that the young person especially understand any possible drug interactions between their medications and alcohol. For example, alcohol significantly increases the risk for liver damage while taking methotrexate, another medication often prescribed for dermatomyositis.

GO TO THE SOURCE: Many university medical centers have programs designed to facilitate transition for young people with chronic health needs.

Taking advantage of resources

No matter how much parents prepare their child, releasing them into the world can still be worrisome for families.

"You need to be realistic about how you can help your child once they leave home," says Pedano. "When they are four or five hours away, you have no control over their eating habits, their sleeping habits, their schedule, what kinds of medicines they're taking, and whether or not they're taking care of themselves. And you forget they are 18 years old and aren't going to listen to you anyway."

Healthcare providers can be a family's greatest resource in helping them make this transition. Many university medical centers like Duke have physicians who specialize in both internal medicine and pediatrics, and many have programs designed specifically to help facilitate medical transition for young people with chronic health needs.

Even if your health care provider is not associated with such a program, they or their staff can often help prepare the way for young people who are heading off into the world, providing referrals and facilitating communication with local resources.

Transitioning well does not require medical intervention, however. Other resources are available, including disease-specific patient organizations like The Myositis Association ( myositis.org), that provide the chance to meet and chat with other patients and families who are going through similar challenges. Got Transition ( gottransition.org) is a comprehensive resource for parents, young people, and providers, offering insights and tools for all kinds of issues that families may never have thought about before.

Linda Iferika also suggests looking for resources close to home. "Reach out to others," she says. "Find a support group. Tap into friends. Find some other outlet for you anger. And remember: This diagnosis is not who you are."

lessons learned

Last year was a big learning experience for Joseph. When he heads back to school this year, however, things will be different. "The biggest thing is I'm going to stay on top of my health," Joseph says. "Sometimes you forget about it. With everything going on, your health is not your number one concern, but it really needs to be." Joseph will pack his vitamins and protein shakes this year, and make sure he's eating and sleeping enough. And when he starts feeling like something isn't quite right, he promises to be more proactive about getting the medical attention he needs.

Pedano feels better about the people who will care for her son, too. "We know everyone in student health services by first name now, and they understand Joseph's disease and what it has done to his immune system. They know they need to take it more seriously. If he comes in, they're going to treat it differently."•

ABOUT THE AUTHOR:

Linda Kobert is the Research and Communications Director of The Myositis Association.