medications used for juvenile dermatomyositis (JDM) than those typically used for his first diagnosis of enthesitis-related JIA, despite the absence of a JDM diagnosis! The earlier the specific type is determined, the better chance the medical team has to limit or even reverse possible damage to the parts of the body that are under attack.
The Big Picture
In the beginning, I just wanted a firm diagnosis. When the doctors felt that it was enthesitis-related JIA, I thought, “Okay, now we can make a plan.” But that diagnosis morphed into probable spondyloarthropathy because Grant was showing signs of bowel disease (a condition that is commonly related to spondyloarthropathy). So, we mentally shifted gears. From there, we heard rumblings of systemic arthritis, psoriatic arthritis, urticaria, autoimmune diabetes, episcleritis, and uveitis, to name a few medical conditions. Some of the symptoms related to these other conditions materialized, while others didn’t. Certain symptoms seemed to appear out of the blue, each requiring the opinion of a specialist. As a matter of fact, our son was collecting specialists faster than a 12-year-old boy collects baseball cards! He was seen by doctors in rheumatology, allergy, hematology/oncology, infectious disease, immunology, orthopedic surgery, dermatology, gastroenterology, cardiology, ophthalmology, endocrinology, neurology, psychology, pain management, and ear, nose and throat! It was overwhelming.
Eventually, I learned to focus less on the specific diagnoses (that is the doctor’s job) and more on getting Grant the treatment he needed. Worrying about this syndrome or that subtype changed nothing. Simply put, no matter how we decided to define it to the world, his body was attacking itself. In the beginning, the where, when, and how seemed to change with the direction of the wind. My job was to help the doctors pin down what was going on when they couldn’t observe him, and then help get him the right treatments and the right care at the right time. In comparison, my younger son Evan’s case has been much less complicated, involving a few issues that have been easily managed on the first attempt, with just a couple of doctors in the mix. Like I said, every case is different.
Educate to Communicate
Nevertheless, educating yourself about the different types of pediatric autoimmune conditions can be helpful to communicate with the doctors. One of the best resources I have found is Dr. Thomas Lehman’s book It’s Not Just Growing Pains, which covers all types of JA and related conditions. Keep in mind, there can be quite a bit of overlap from one type to the next, so just because you may see similar symptoms don’t panic and don’t self-diagnose! Use that information to help the doctor.
As an example, lupus runs in our family, and many of Grant’s symptoms (after his first hospital discharge) seemed to mirror cases of lupus that I had researched. Grant did not have lupus, but framing his symptoms in a context that was well known to the doctor helped me effectively communicate what was happening to Grant on a cyclical basis. Doing research, reading books authored by recognized experts, and familiarizing myself with other cases gave me the tools I needed to better convey my child’s situation. Using terms and contexts common to autoimmune disease put me on the same page as the doctors, and we started speaking more of the same language.
Know Your Adversary
Battling an unpredictable opponent is tough. It’s difficult to make a game plan and stick to it. As the enemy changes, so must the plan! One of the best ways to prepare is to learn as much as possible so that you can be ready for any situation. When my husband coached youth and high school football, one of the ways he prepared his team for upcoming games was to watch film taken of the opponent. He didn’t just watch the prior week’s game; he watched all the film he could get his hands on. He observed how they handled each of their opponents, because each game was different.
Juvenile arthritis is different in every child. By observing how other kids act and react to JA, it can give you snippets of the opponent your child will face in his or her battle. Reading about their lives and their struggles is like watching game film for your child. It may not be your particular game, but it shows you what that opponent is capable of and how to prepare against it.
Meet Some of the Other Players
The following are actual accounts shared by families living with different types of JA. Their stories illustrate how diverse the paths to diagnosis and treatment can be, as well as the unpredictable nature of this disease. However, while their stories are different, there is a common thread—hope.
Connor’s Journey with JDM by Anke
In 2005, my husband and I were thrilled to become parents by adopting one-year-old identical twin boys from Russia. The adoption of our boys was such a gift, and they have brought us immeasurable joy, despite the medical challenges we’ve faced. Because our sons were adopted from a foreign country, we did not receive any medical history on the boys or their parents. As we soon learned, this lack of information can make diagnosing health problems more difficult.
Shortly after we arrived home with our sons, the “younger” twin, Alexander, was diagnosed with a tethered spinal cord, which required corrective surgery when he was just 18 months old. He had a dimple on his back that led to this diagnosis, but his brother, Connor, had no outward signs of the condition. However, a few months later, as I was watching a documentary on syndromes that can develop during pregnancy, a warning bell went off in my head.
Since our boys were identical twins, I took Connor in for an assessment. Sure enough, an MRI revealed he also had a tethered spinal cord. Like his brother, he underwent surgery shortly after he turned two. Though the surgeries were stressful, we thought the worst was behind us.
In 2009, as we were preparing for our yearly trip to Germany (where I was born and raised), warnings about the swine flu filled the news. We were told it was even more widespread in Europe and, as a precaution, the boys should receive the H1N1 flu shot. They were vaccinated about three days before our flight, and while we were in the airport Connor started complaining about pain in his legs. We brushed it off as growing pains or the normal aches of a very active five-year-old.
However, during our stay in Germany the pain got progressively worse. I was so concerned that I changed our travel plans to come home early. During the return trip, Connor’s legs were so sore that I had to carry him most of the time. Our first stop upon arrival home was the doctor’s office.
The first diagnosis we received was mononucleosis. We were told to take him home and give him Motrin for the discomfort. But, Connor did not get better. In fact, his condition worsened, and that’s when the “Mommy Bear” instinct kicked in. I took him back to the doctor three more times, until finally a physician’s assistant put me in touch with a neurologist.
Initially, we were worried about a reattachment of the spinal cord, but the MRI came back clear. The next step was a spinal tap to rule out Guillain-Barré syndrome, which can occur after receiving a vaccination. When this test came back negative, Connor had a muscle biopsy done, which confirmed that his muscles were inflamed and he was put on a steroid. We had to wait weeks for the complete results, but in January 2011, we were told that our son had juvenile dermatomyositis (JDM), a rare form of juvenile arthritis. More specifically, JDM is a systemic autoimmune disease that causes inflamed and weakened muscles, among other things.
We had a diagnosis, but we did not have the answers we needed to make Connor better. The steroids didn’t make a dent in his condition, and Connor declined at a rapid pace. Taking matters into my own hands, I began to research this disease. My husband and I agreed that we would take Connor anywhere in the world to find the help he needed.
I have to admit, the more I learned about JDM, the more fearful I became. But, then I found a ray of hope: the leading doctor for JDM, Dr. Lauren Pachman, was
