Interviewed by Tom Mangan

Ralph Becker, Julia's dad, explaining her rare ailment.

Ralph and Julia's JDMS diary is here.

Describe one of those episodes that amazed you at how well your daughter could cope with a strange, instrusive disease.

Julia has shown enough strength and courage to be called heroic. One great example of this happened last spring. She was in the midst of a long, slow relapse and was feeling weaker and more fatigued with almost every passing day, but she was determined to finish her swimming lessons. She'd been taking lessons for the past 4 or 5 years, but her disease had really slowed her down. It was to the point where she could barely climb out of the pool because her arm muscles were so weak. But she only rarely missed a lesson, and she worked hard every week to keep up with the other kids. She finished lessons at the end of May, less than four weeks away from being hospitalized, and she'd passed the next level of certification! We were so proud of her, and so happy for her, like a coach might feel when the team wins a championship.

She'd earned that certificate, and it represented a level of determination I don't think many adults have.

What's the range of symptoms, from minor to serious, of your daughter's case of juvenile dermatomyositis?

JDMS manifests itself primarily in a skin rash and muscle weakness. In Julia's case, we first noticed a rash on her face a few days after she got over a cold. Her eyelids had turned pink-purplish, and the rest of her face looked flushed all the time.

Soon, the rash also appeared as red spots on her joints. No creams had any effect on the rash. Within a few weeks, it became so pronounced that we began getting questions from people who saw her if she had a sunburn, despite it being in the middle of winter in New England. She still has the rash to this day, and our doctors tell us it's the most persistent JDMS rash they've ever seen.

After several weeks, we began to notice that Julia was becoming more easily tired -- she takes dance and swimming lessons, and was an otherwise normal, active 7-year-old. We didn't connect it to the rash until after we received a diagnosis, but fatigue is an early symptom of muscle inflammation. Once we received a diagnosis, the doctors showed us where her muscles were inflamed with an MRI and through clinical tests.

The muscle weakness is the much more serious symptom of JDMS. Julia had a relapse in 1999 that left her barely able to climb out of a swimming pool or ascend a flight of stairs. She didn't have the energy to go to an all-day event, like a trip to an amusement park.

We were lucky, though; careful monitoring prevented it from getting any worse or causing permanent damage. Some kids progress to the point where they have breathing or swallowing difficulties, and need to use a wheelchair or braces. JDMS even has a small fatality rate, in kids who don't respond to the available treatments.

How have Julia's friends and schoolmates reacted to her illness?

For the most part, remarkably well. Julia is a third-grader at a Catholic school and everyone, from the principal and staff, to the teachers, to her classmates, has been extremely accepting and understanding.

We believe this stems from the fact that religion and values are part of the curriculum. Also, we have made an effort to keep the staff up-to-date on Julia's medical progress, and some of them even read her diary regularly.

That said, I do believe that the situation would be different if Julia were in the public school system. This opinion stems from actual experience. At the start of the 1998 school year (Julia was in second grade), the bus schedule had Julia being picked up by a bus that was carrying both public school and Catholic school kids. At that point, Julia's rash was in full bloom, and she was on a high dose of Prednisone, which made her put on a significant amount of weight and gave her "Cushingoid Syndrome," better known as "moon face".

[ For an idea of what she looked like, see her school picture from that time at: http://www.ralphb.net/juliafb/jfb98.gif ]

The public school kids, almost daily, would rudely ask Julia about her appearance, make comments to her or each other, or just stare at her during their time together on the bus. Kids can be cruel, of course, and there's little that Julia could do about it except ignore them. This really hurt Julia, to the point where she just broke down and cried about it one day while we were out shopping. It was heartbreaking as a parent to see that.

Fortunately, soon after that, the town saw fit to shuffle the bus schedule so that the Catholic school kids were on a separate bus. It's been that way ever since, and there haven't been any further problems.

How has this ordeal changed your perception of the medical profession?

I'd have to say that it's gone from a distant awe, due to a lack of personal experience with serious medical problems, to a deep personal respect. Julia is treated at Children's Hospital in Boston, which has a reputation for being one of the best such hospitals in the world. That reputation is well earned. Practically every person we have encountered at that hospital has shown a singular dedication to helping sick kids get well, and helping their parents deal with it. They are simply the best at what they do, and they seem to love doing it.

Children's is the exceptional case, and we understand how truly lucky we are to have such easy access to it. Our perception would surely be different if we were in different circumstances. However, that doesn't diminish the respect we have for the professionals we've dealt with.

Can you say the same for your health insurance provider?

We are equally fortunate to have one of the best HMOs in the country. They always rate very high in the annual consumer surveys. [It's Harvard-Pilgrim HMO]. They also have a well-earned reputation for their handling of kids' diseases. I'm sure no insurance company wants bad publicity because they're denying coverage to a sick child, but they have been great.

From day 1, they have been helpful and informative, handling all the paperwork, and not a whimper when tests and office visits are called for. We've only had a couple of minor snafus that were eventually resolved to everyone's satisfaction. Again, I realize how lucky we are to not have to deal with an insurance company that's fighting you at every step of the way.

Is there any hope for a cure for Julia?

No. In fact, there's not even a treatment specific to the disease. All the treatments that are given to JDMS either treat the symptoms (anti-inflammatories like Prednisone, and drugs like Plaquenil for the rash), or generally suppress the immune system (Prednisone again, and Methotrexate, among others). The fact is that this is so rare, that no drug cmpany would likely ever spend the money to develop a specific treatment.

The news is not all bad, however. The vast majority of kids (over 95%, in fact) who get JDMS before puberty eventually achieve a state of "permanent remission," which means symptom-free and drug-free for at least a year. This fact, plus the fact that Julia's case was caught very early and is relatively mild, gives us a lot of hope. There's always a chance that Julia could become chronic and have to deal with it into her adulthood, but we're confident that she (and we) could handle that.

What are a few specific tips you'd offer to parents who find themselves in a similar situation?

One thing I always suggest is to keep a notebook. Write down exactly what the doctor says, because you won't remember later if you don't. Keep the notebook handy all the time, and write down any questions that occur to you. Write down the lab test results. Jot down events that occur (like falls or colds or whatever) - they may seem insignificant alone, but a string of them can indicate a trend or a symptom. Naturally, your notebook doesn't have to be published on the Internet, but you never know.

It's a good idea to be informed. Read some books, scan the Web sites, ask a lot of questions and pay attention to the answers. Be active in the decision making process - don't just do what the doctors say. Using a notebook can go a long way here, too. Most doctors appreciate a well-informed patient.

Another thought is to seek help and support. Don't try to go it alone. Your doctor can tell you where local support for JDMS is - usually rolled into another disease's support because there are never enough JDMS patients in one place to have an actual support group. Seek help from relatives, friends, and neighbors, as well as medical professionals, particularly if your child is disabled by the disease. There are a number of Web sites that offer support for the Myositis diseases, and you should take advantage of them. The people there understand what you're going through because they've been there.

Finally, always show appreciation to caregivers and medical professionals. They are often taken for granted and genuinely appreciate a personal thank you, general greeting, or a small present at holiday time. One of Julia's doctors is doing some medical research with mice, and Julia made her a booklet of hand rubber-stamped and colored mouse pictures. The doctor loved it, and talked about it for months after getting it. Remember your doctors, nurses, physical therapists, home health aides, administrators, and even your pharmacist - anyone who has made your life a little easier.

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